Case Reports

Isolated dislocation of the carpometacarpal (CMC) joint of the hand is a rare injury. While the dislocation can be dorsal or palmar, dorsal dislocation is more common. Palmar dislocations can be either ulnopalmar or radiopalmar. There are very few reports of isolated radiopalmar dislocations of the fifth CMC joint in the English-language literature.^1-3 We present a case of delayed presentation and management of radiopalmar dislocation of the fifth CMC joint. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 42-year-old man presented with polytrauma to our emergency department. He was stabilized initially, and open fractures were treated by débridement and external fixator application. During an examination 3 days after admission, swelling was noted in the right hand. On further study, there was splaying of the fifth digit and tenderness over the fourth and fifth CMC joints (Figure 1). No abnormal mobility or crepitus could be elicited. Plain radiographs of the right hand in anteroposterior and lateral views revealed radiopalmar dislocation of the fifth CMC joint (Figure 2). It was decided to reduce the dislocation immediately after the patient was declared fit for surgery.

Under axillary block, closed reduction was unsuccessful. Open reduction of the fifth CMC joint was performed through a dorsal incision. The base of the fifth metacarpal bone was found to be stripped of soft-tissue attachments and lying in a radiopalmar location. Reduction, which was checked under image intensifier, was found to be satisfactory (Figure 3). Reduction was stabilized by passing a smooth Kirschner wire (K-wire) from the fifth metacarpal to the hamate bone. After achieving hemostasis, the wound was closed in layers and a below-elbow splint was applied. The perioperative period was uneventful, and sutures were removed on postoperative day 10. The K-wire was removed after 4 weeks, and radiographs showed satisfactory position of the fifth CMC joint. Gentle active and passive mobilization of fingers and wrist were started. The patient had regained good function of the wrist and fingers 2 months after surgery (Figure 4).

Discussion

Carpometacarpal joint dislocations are uncommon injuries and account for less than 1% of hand injuries.⁴ They are classified as dorsal and volar (palmar) dislocations. Dorsal dislocations of the CMC joints occur more frequently than do volar dislocations, mainly affecting the fourth and fifth digits.⁵ Isolated volar or palmar dislocation of the fifth CMC joint is an uncommon injury that was first reported in 1918 by McWhorter.⁶ In 1968, Nalebuff⁷ classified the volar dislocations into 2 groups according to the direction of the displacement of the fifth metacarpal base: radiopalmar and ulnopalmar. Berg and Murphy⁸ found the hook of the hamate to deviate the metacarpal bone to either the ulnar or radial side. Tearing of all ligament and tendon attachments of the base of the fifth metacarpal results in radiopalmar dislocation.⁷ The attachments of ligaments and tendons remain intact in the ulnopalmar dislocation.⁷

The clinical features of this injury are pain and swelling about the base of the fifth metacarpal and axial deformity of the little finger with apparent shortening. The deep motor branch of the ulnar nerve lies volar to the fifth CMC joint as it courses around the hook of the hamate. It is vulnerable to injury in both dorsal^9,10 and volar¹¹ CMC dislocations. For radiologic evaluation, in addition to standard anteroposterior and lateral radiographs, a lateral view in 30º pronation of the hand can provide an improved view of the fifth CMC joint, as suggested by Bora and Didizian.¹²

The treatment of ulnopalmar dislocation has evolved. Ulnopalmar dislocations have been successfully treated by closed reduction without fixation,⁸ and by open reduction and K-wire fixation.^3,7,13

Radiopalmar dislocations are inherently unstable because of the tearing of all ligament and tendon attachments of the base of the fifth metacarpal.⁷ In our case of radiopalmar dislocation, diagnosis was delayed and attempts at closed reduction were unsuccessful. Therefore, it was treated by open reduction and K-wire fixation. In our case, open reduction and K-wire fixation for radiopalmar dislocation of the fifth CMC joint provided promising results.

Conclusion

Radiopalmar dislocation of the fifth CMC joint is a rare injury, and very few cases have been reported in the English-language literature. We report one such case, which was successfully treated with open reduction and K-wire fixation.

Isolated dislocation of the carpometacarpal (CMC) joint of the hand is a rare injury. While the dislocation can be dorsal or palmar, dorsal dislocation is more common. Palmar dislocations can be either ulnopalmar or radiopalmar. There are very few reports of isolated radiopalmar dislocations of the fifth CMC joint in the English-language literature.^1-3 We present a case of delayed presentation and management of radiopalmar dislocation of the fifth CMC joint. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 42-year-old man presented with polytrauma to our emergency department. He was stabilized initially, and open fractures were treated by débridement and external fixator application. During an examination 3 days after admission, swelling was noted in the right hand. On further study, there was splaying of the fifth digit and tenderness over the fourth and fifth CMC joints (Figure 1). No abnormal mobility or crepitus could be elicited. Plain radiographs of the right hand in anteroposterior and lateral views revealed radiopalmar dislocation of the fifth CMC joint (Figure 2). It was decided to reduce the dislocation immediately after the patient was declared fit for surgery.

Under axillary block, closed reduction was unsuccessful. Open reduction of the fifth CMC joint was performed through a dorsal incision. The base of the fifth metacarpal bone was found to be stripped of soft-tissue attachments and lying in a radiopalmar location. Reduction, which was checked under image intensifier, was found to be satisfactory (Figure 3). Reduction was stabilized by passing a smooth Kirschner wire (K-wire) from the fifth metacarpal to the hamate bone. After achieving hemostasis, the wound was closed in layers and a below-elbow splint was applied. The perioperative period was uneventful, and sutures were removed on postoperative day 10. The K-wire was removed after 4 weeks, and radiographs showed satisfactory position of the fifth CMC joint. Gentle active and passive mobilization of fingers and wrist were started. The patient had regained good function of the wrist and fingers 2 months after surgery (Figure 4).

Discussion

Carpometacarpal joint dislocations are uncommon injuries and account for less than 1% of hand injuries.⁴ They are classified as dorsal and volar (palmar) dislocations. Dorsal dislocations of the CMC joints occur more frequently than do volar dislocations, mainly affecting the fourth and fifth digits.⁵ Isolated volar or palmar dislocation of the fifth CMC joint is an uncommon injury that was first reported in 1918 by McWhorter.⁶ In 1968, Nalebuff⁷ classified the volar dislocations into 2 groups according to the direction of the displacement of the fifth metacarpal base: radiopalmar and ulnopalmar. Berg and Murphy⁸ found the hook of the hamate to deviate the metacarpal bone to either the ulnar or radial side. Tearing of all ligament and tendon attachments of the base of the fifth metacarpal results in radiopalmar dislocation.⁷ The attachments of ligaments and tendons remain intact in the ulnopalmar dislocation.⁷

The clinical features of this injury are pain and swelling about the base of the fifth metacarpal and axial deformity of the little finger with apparent shortening. The deep motor branch of the ulnar nerve lies volar to the fifth CMC joint as it courses around the hook of the hamate. It is vulnerable to injury in both dorsal^9,10 and volar¹¹ CMC dislocations. For radiologic evaluation, in addition to standard anteroposterior and lateral radiographs, a lateral view in 30º pronation of the hand can provide an improved view of the fifth CMC joint, as suggested by Bora and Didizian.¹²

The treatment of ulnopalmar dislocation has evolved. Ulnopalmar dislocations have been successfully treated by closed reduction without fixation,⁸ and by open reduction and K-wire fixation.^3,7,13

Radiopalmar dislocations are inherently unstable because of the tearing of all ligament and tendon attachments of the base of the fifth metacarpal.⁷ In our case of radiopalmar dislocation, diagnosis was delayed and attempts at closed reduction were unsuccessful. Therefore, it was treated by open reduction and K-wire fixation. In our case, open reduction and K-wire fixation for radiopalmar dislocation of the fifth CMC joint provided promising results.

Conclusion

Radiopalmar dislocation of the fifth CMC joint is a rare injury, and very few cases have been reported in the English-language literature. We report one such case, which was successfully treated with open reduction and K-wire fixation.

Isolated dislocation of the carpometacarpal (CMC) joint of the hand is a rare injury. While the dislocation can be dorsal or palmar, dorsal dislocation is more common. Palmar dislocations can be either ulnopalmar or radiopalmar. There are very few reports of isolated radiopalmar dislocations of the fifth CMC joint in the English-language literature.^1-3 We present a case of delayed presentation and management of radiopalmar dislocation of the fifth CMC joint. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 42-year-old man presented with polytrauma to our emergency department. He was stabilized initially, and open fractures were treated by débridement and external fixator application. During an examination 3 days after admission, swelling was noted in the right hand. On further study, there was splaying of the fifth digit and tenderness over the fourth and fifth CMC joints (Figure 1). No abnormal mobility or crepitus could be elicited. Plain radiographs of the right hand in anteroposterior and lateral views revealed radiopalmar dislocation of the fifth CMC joint (Figure 2). It was decided to reduce the dislocation immediately after the patient was declared fit for surgery.

Under axillary block, closed reduction was unsuccessful. Open reduction of the fifth CMC joint was performed through a dorsal incision. The base of the fifth metacarpal bone was found to be stripped of soft-tissue attachments and lying in a radiopalmar location. Reduction, which was checked under image intensifier, was found to be satisfactory (Figure 3). Reduction was stabilized by passing a smooth Kirschner wire (K-wire) from the fifth metacarpal to the hamate bone. After achieving hemostasis, the wound was closed in layers and a below-elbow splint was applied. The perioperative period was uneventful, and sutures were removed on postoperative day 10. The K-wire was removed after 4 weeks, and radiographs showed satisfactory position of the fifth CMC joint. Gentle active and passive mobilization of fingers and wrist were started. The patient had regained good function of the wrist and fingers 2 months after surgery (Figure 4).

Discussion

Carpometacarpal joint dislocations are uncommon injuries and account for less than 1% of hand injuries.⁴ They are classified as dorsal and volar (palmar) dislocations. Dorsal dislocations of the CMC joints occur more frequently than do volar dislocations, mainly affecting the fourth and fifth digits.⁵ Isolated volar or palmar dislocation of the fifth CMC joint is an uncommon injury that was first reported in 1918 by McWhorter.⁶ In 1968, Nalebuff⁷ classified the volar dislocations into 2 groups according to the direction of the displacement of the fifth metacarpal base: radiopalmar and ulnopalmar. Berg and Murphy⁸ found the hook of the hamate to deviate the metacarpal bone to either the ulnar or radial side. Tearing of all ligament and tendon attachments of the base of the fifth metacarpal results in radiopalmar dislocation.⁷ The attachments of ligaments and tendons remain intact in the ulnopalmar dislocation.⁷

The clinical features of this injury are pain and swelling about the base of the fifth metacarpal and axial deformity of the little finger with apparent shortening. The deep motor branch of the ulnar nerve lies volar to the fifth CMC joint as it courses around the hook of the hamate. It is vulnerable to injury in both dorsal^9,10 and volar¹¹ CMC dislocations. For radiologic evaluation, in addition to standard anteroposterior and lateral radiographs, a lateral view in 30º pronation of the hand can provide an improved view of the fifth CMC joint, as suggested by Bora and Didizian.¹²

The treatment of ulnopalmar dislocation has evolved. Ulnopalmar dislocations have been successfully treated by closed reduction without fixation,⁸ and by open reduction and K-wire fixation.^3,7,13

Radiopalmar dislocations are inherently unstable because of the tearing of all ligament and tendon attachments of the base of the fifth metacarpal.⁷ In our case of radiopalmar dislocation, diagnosis was delayed and attempts at closed reduction were unsuccessful. Therefore, it was treated by open reduction and K-wire fixation. In our case, open reduction and K-wire fixation for radiopalmar dislocation of the fifth CMC joint provided promising results.

Conclusion

Radiopalmar dislocation of the fifth CMC joint is a rare injury, and very few cases have been reported in the English-language literature. We report one such case, which was successfully treated with open reduction and K-wire fixation.

Ollier disease, or multiple enchondromatosis, is a rare nonfamilial condition characterized by multiple cartilaginous tumors often beginning in early childhood. There is significant variation in disease distribution, location, size, number of lesions, and behavior, but the tumors are often located unilaterally.¹ Enchondromas are most commonly found in the metacarpals, metatarsals, and phalanges, and develop from metaphyseal bone in close proximity to the physis. They frequently present as painless masses or are incidentally noted during the evaluation of another musculoskeletal condition. Radiographically, enchondromas of the hands and feet appear as oval radiolucencies with thinned, sclerotic rims. The lesions have varying degrees of mineralization and endosteal scalloping, and may expand the bone.² Enchondromas usually enlarge until skeletal maturity and have been observed to ossify spontaneously.^1,3 The clinical course of Ollier disease is variable, and a number of cases of significant hand deformity and malignant transformation have been reported.^4-6

In this case report, we present a mild form of Ollier disease isolated to the patient’s left hand, which we followed for 8 years, demonstrating part of the natural history of these lesions. We discuss the patient’s clinical features, radiologic findings, diagnosis, treatment, prognosis, and follow-up, as well as review the literature. The patient and the patient’s family provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old, right-handed girl was referred to our department for the evaluation of left-hand masses. At age 3 years, the patient underwent a chondroma excision from the middle phalanx of her middle finger on her left hand. No operative or pathology report was available from this surgery, and the patient tolerated the procedure well without any complications. At the time of presentation, the masses did not cause any pain, motor or sensory dysfunction, or any systemic symptoms. No history of recent or distant trauma was elicited. The patient’s medical and family history was unremarkable.

On physical examination, there was a firm, immobile, nontender palpable mass over the dorsal aspect of the distal second metacarpal bone of the left hand. The mass extended medially between the second and third metacarpals. A second small, firm, nontender left-hand mass was palpated over the volar aspect of her proximal phalanx on her index finger. She was neurovascularly intact with full active range of motion of the metacarpophalangeal and proximal and distal interphalangeal joints. There was no angular deformity of the digits. Plain radiographs taken at the time of initial presentation showed a 2.3×1.7-cm radiolucent lesion located in the metaphysis and diaphysis of the second metacarpal of the left hand (Figures 1A-1C). The lesion had varying degrees of mineralization with cortical thinning and expansion in the volar, dorsal, radial, and ulnar directions, consistent with a chondroid lesion. The second and third lesions were oval radiolucencies with sclerotic rims located at the metaphyseal-diaphyseal junction of the proximal phalanx of the index finger and middle phalanx of the middle finger, respectively. No fractures were identified in the radiographs, and the physes were open at this time. The patient was diagnosed with multiple enchondromatosis, or Ollier disease.

Our case showed 1 episode of pain and tenderness to palpation at the second proximal phalanx approximately 6 months after initial presentation. We attributed the pain and tenderness to a small pathologic fracture but did not see radiographic evidence of this. We elected to provide a trial of supportive measures, such as splinting and buddy taping, and to monitor the pain with a tentative plan of open biopsy with curettage and bone grafting if the pain persisted or evidence of fracture was seen on radiographs. The pain and tenderness to palpation resolved at a follow-up visit, and the surgery was deferred.

The patient was treated nonoperatively at initial presentation given the lack of significant cosmetic deformity or functional compromise and was advised close follow-up at 3 and 6 months. Given the absence of disease progression, annual checks (ie, clinical examination and radiographs) in a skeletally immature patient were decided on after consultation with the patient and parent. The family was educated about the possibility of pathologic fracture from minimal trauma to the hand versus the small risk of iatrogenic physeal injury with surgical curettage and bone grafting. No protective splinting was offered. A favorable prognosis and reassurance was provided to the patient and family, given the absence of symptoms, low suspicion and risk of malignant transformation, and stability of the lesion. Serial radiographs showed gradual increases in the lesions’ sizes but were consistent with the stable growth of the metacarpal and phalanges. With the patient nearing skeletal maturity, no pathologic fractures were identified on radiography during follow-up, and the risks of surgery lessened with growth; however, the continued absence of symptoms led to the mutual decision to continue observation.

Nearly 8 years after initial presentation, plain radiographs showed closed physes and partially ossified bone masses (Figures 2A-2C). The metacarpal lesion measured 3.2×1.5 cm, and the cortex appeared thickened and regular. The proximal phalanx lesion had a thickened cortex without periosteal reaction, and the middle phalanx lesion appeared to be completely healed. The patient has been asymptomatic for many years, and she has retained complete function of her left hand without any growth retardation, angular deformity, or pathologic fracture. A small but potential risk of malignant transformation was discussed with the patient and her family, as was the need for lifetime follow-up. We intend to follow the enchondromas clinically and radiographically every 2 years and obtain new radiographs if the mass presents with new clinical findings, such as enlargement or pain, for surveillance of tumor transformation. If the patient desired or symptoms developed, curettage and bone grafting would be offered, and the surgical tissue would be sent for pathologic analysis. A bone scan that was obtained at the request of the patient, when she was 21 years old, showed no other sites of disease besides the fingers.

Discussion

Multiple enchondromatosis was first described by Ollier at the turn of the 19th century and has been estimated to affect one in every 100,000 persons.¹ The low prevalence and variable manifestations of Ollier disease lead clinicians to handle the disease and its complications, namely skeletal deformity and malignant transformation, on a case-by-case approach. Additionally, the prognosis of Ollier disease with malignant transformation is quite variable, with studies reporting the estimated incidence as 5% to 50%.⁷ Muramatsu and colleagues⁶ reported that the occurrence of malignant transformation of multiple enchondromas limited to the bones of the hand was extremely rare, with only 12 cases of malignant transformation. Enchondromas of the pelvis, scapula, and long bones of the extremities have increased risks and rates of secondary transformation to chondrosarcoma.⁸

A recent large European multicenter retrospective study investigating the clinical characteristics and behavior of enchondromas in 144 patients with Ollier disease has provided new information regarding this rare disease.⁷ Verdegaal and colleagues⁷ divided patients into 3 categories depending on their distribution of enchondromas. The development of chondrosarcoma was notably different between individuals with enchondromas limited to the small bones of the hands and feet (15%, group I) versus individuals with enchondromas limited to the long bones and flat bones (43%, group II) or individuals with enchondromas of the short, long, and flat bones (46%, group III).⁷ The only location found to be statistically significant for the development of chondrosarcoma was the pelvis.

The clinical findings associated with risk of malignant transformation of enchondromas are increasing size of the lesion and onset of pain and tenderness. Dahlin and Salvador⁹ reported that only 60% of patients with chondrosarcoma of the hand experience pain. The absence of pain may lead to a delay in patient presentation to the clinician.^5,6 Radiographic findings of malignant transformation include the classic features of temporal increases in the lesion’s size after skeletal maturity and cortical destruction associated with soft-tissue invasion. However, both findings are nonspecific for differentiating enchondromas from grade 1 chondrosarcomas as described by Geirnaerdt and colleagues.¹⁰

Sassoon and colleagues¹¹ reported on a series of hand enchondromas treated operatively. Subgroup analysis between pathologic fractures treated primarily or in delayed fashion showed similar outcomes for achieving full motion and similar number of complications; however, they noted that the delayed group required 7 more weeks of immobilization. Additionally, review of the whole series showed 1 episode of metacarpal shortening and 1 occurrence of angular malalignment. In our patient, we were concerned about introducing an iatrogenic cosmetic deformity, and we believed a pathologic fracture could be managed expectantly. Overall, patients without pathologic fracture treated surgically experienced a complication rate of 12%, whereas patients with a fracture had a complication rate of 20%.¹¹ The majority of patients with multiple enchondromatosis treated with surgical curettage and grafting had successful outcomes, with 86% of patients regaining full motion, but the recurrence rate was 21%.¹¹ Patients with expansile lesions regained less motion than patients with nonexpansile lesions. There was a single lesion believed preoperatively to be an enchondroma, but it underwent malignant transformation, as confirmed on intraoperative pathology. This patient had Maffucci syndrome and was treated with an amputation through the metacarpophalangeal joint.

There are 3 options for treating hand enchondromas: observation, curettage alone, or curettage with bone grafting. There is no consensus about conservative management, timing of intervention, or risk of pathologic fracture. Each patient is treated individually with attention to reason for presentation, number of lesions, associated pain, deformity, or pathologic fracture. Operative criteria include high risk of pathologic fracture based on location of enchondroma, cortical thinning, and previous pathologic fracture with resulting angular deformity. Nonoperative management may increase the risk of pathologic fracture, particularly in patients involved in aggressive contact sports, but the physician may offer protective splinting or counsel the patient on activity modification. Our case provides a study of the natural history of multiple enchondromatosis and shows mild increases in the lesions’ sizes during the 8-year follow-up. This was an expected finding given the patient’s immature skeleton. The lesions’ cortices continued to ossify after the physes closed and now provides an excellent comparison for the identification of future malignant changes. 

Histologic analysis of biopsied or surgically treated lesions contributes to the differentiation between benign hand enchondromas and chondrosarcoma. Pathologic findings must be correlated with clinical and radiographic findings because hand enchondromas contain cytologic features of chondrosarcoma.¹² In a series of 55 patients with chondrosarcoma, Liu and colleagues⁸ reported no cases from the hand. Verdegaal and colleagues⁷ reported a total of 13 chondrosarcomas in the metacarpals and hand phalanges in 97 group I and III patients. Five of these lesions were grade 1, 2 were grade 2, 1 was grade 3, and 5 lesions were unknown.

For patients with multiple enchondromatosis limited to the hands, prognosis is relatively good with respect to risk of secondary chondrosarcoma transformation, metastasis of secondary chondrosarcoma, and death. Verdegaal and colleagues⁷ reported the rate of secondary transformation in the hand to be 15%. Patil and colleagues¹³ reported no distant metastases in 23 patients with hand chondrosarcoma at mean follow-up of 8.5 years (range, 2-19 years), although none of their patients had Ollier disease. Verdegaal and colleagues⁷ reported 7 of the 8 deaths in their study were related to development of pulmonary metastases; however, none originated from chondrosarcomas in the hand. Additionally, there were no disease-related deaths in 29 group I patients. Herget and colleagues,¹⁴ in summarizing the literature, postulated that the overall survival rate of patients with secondary chondrosarcoma at 5 years is approximately 90%.

In our case, the patient, who had 3 enchondromas isolated to the left hand, can be categorized in group I. Thus, this case highlights the natural history of a patient with hand enchondromas and demonstrates that enchondromatosis of the short tubular bones of the hands can mature and ossify.

Ollier disease, or multiple enchondromatosis, is a rare nonfamilial condition characterized by multiple cartilaginous tumors often beginning in early childhood. There is significant variation in disease distribution, location, size, number of lesions, and behavior, but the tumors are often located unilaterally.¹ Enchondromas are most commonly found in the metacarpals, metatarsals, and phalanges, and develop from metaphyseal bone in close proximity to the physis. They frequently present as painless masses or are incidentally noted during the evaluation of another musculoskeletal condition. Radiographically, enchondromas of the hands and feet appear as oval radiolucencies with thinned, sclerotic rims. The lesions have varying degrees of mineralization and endosteal scalloping, and may expand the bone.² Enchondromas usually enlarge until skeletal maturity and have been observed to ossify spontaneously.^1,3 The clinical course of Ollier disease is variable, and a number of cases of significant hand deformity and malignant transformation have been reported.^4-6

In this case report, we present a mild form of Ollier disease isolated to the patient’s left hand, which we followed for 8 years, demonstrating part of the natural history of these lesions. We discuss the patient’s clinical features, radiologic findings, diagnosis, treatment, prognosis, and follow-up, as well as review the literature. The patient and the patient’s family provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old, right-handed girl was referred to our department for the evaluation of left-hand masses. At age 3 years, the patient underwent a chondroma excision from the middle phalanx of her middle finger on her left hand. No operative or pathology report was available from this surgery, and the patient tolerated the procedure well without any complications. At the time of presentation, the masses did not cause any pain, motor or sensory dysfunction, or any systemic symptoms. No history of recent or distant trauma was elicited. The patient’s medical and family history was unremarkable.

On physical examination, there was a firm, immobile, nontender palpable mass over the dorsal aspect of the distal second metacarpal bone of the left hand. The mass extended medially between the second and third metacarpals. A second small, firm, nontender left-hand mass was palpated over the volar aspect of her proximal phalanx on her index finger. She was neurovascularly intact with full active range of motion of the metacarpophalangeal and proximal and distal interphalangeal joints. There was no angular deformity of the digits. Plain radiographs taken at the time of initial presentation showed a 2.3×1.7-cm radiolucent lesion located in the metaphysis and diaphysis of the second metacarpal of the left hand (Figures 1A-1C). The lesion had varying degrees of mineralization with cortical thinning and expansion in the volar, dorsal, radial, and ulnar directions, consistent with a chondroid lesion. The second and third lesions were oval radiolucencies with sclerotic rims located at the metaphyseal-diaphyseal junction of the proximal phalanx of the index finger and middle phalanx of the middle finger, respectively. No fractures were identified in the radiographs, and the physes were open at this time. The patient was diagnosed with multiple enchondromatosis, or Ollier disease.

Our case showed 1 episode of pain and tenderness to palpation at the second proximal phalanx approximately 6 months after initial presentation. We attributed the pain and tenderness to a small pathologic fracture but did not see radiographic evidence of this. We elected to provide a trial of supportive measures, such as splinting and buddy taping, and to monitor the pain with a tentative plan of open biopsy with curettage and bone grafting if the pain persisted or evidence of fracture was seen on radiographs. The pain and tenderness to palpation resolved at a follow-up visit, and the surgery was deferred.

The patient was treated nonoperatively at initial presentation given the lack of significant cosmetic deformity or functional compromise and was advised close follow-up at 3 and 6 months. Given the absence of disease progression, annual checks (ie, clinical examination and radiographs) in a skeletally immature patient were decided on after consultation with the patient and parent. The family was educated about the possibility of pathologic fracture from minimal trauma to the hand versus the small risk of iatrogenic physeal injury with surgical curettage and bone grafting. No protective splinting was offered. A favorable prognosis and reassurance was provided to the patient and family, given the absence of symptoms, low suspicion and risk of malignant transformation, and stability of the lesion. Serial radiographs showed gradual increases in the lesions’ sizes but were consistent with the stable growth of the metacarpal and phalanges. With the patient nearing skeletal maturity, no pathologic fractures were identified on radiography during follow-up, and the risks of surgery lessened with growth; however, the continued absence of symptoms led to the mutual decision to continue observation.

Nearly 8 years after initial presentation, plain radiographs showed closed physes and partially ossified bone masses (Figures 2A-2C). The metacarpal lesion measured 3.2×1.5 cm, and the cortex appeared thickened and regular. The proximal phalanx lesion had a thickened cortex without periosteal reaction, and the middle phalanx lesion appeared to be completely healed. The patient has been asymptomatic for many years, and she has retained complete function of her left hand without any growth retardation, angular deformity, or pathologic fracture. A small but potential risk of malignant transformation was discussed with the patient and her family, as was the need for lifetime follow-up. We intend to follow the enchondromas clinically and radiographically every 2 years and obtain new radiographs if the mass presents with new clinical findings, such as enlargement or pain, for surveillance of tumor transformation. If the patient desired or symptoms developed, curettage and bone grafting would be offered, and the surgical tissue would be sent for pathologic analysis. A bone scan that was obtained at the request of the patient, when she was 21 years old, showed no other sites of disease besides the fingers.

Discussion

Multiple enchondromatosis was first described by Ollier at the turn of the 19th century and has been estimated to affect one in every 100,000 persons.¹ The low prevalence and variable manifestations of Ollier disease lead clinicians to handle the disease and its complications, namely skeletal deformity and malignant transformation, on a case-by-case approach. Additionally, the prognosis of Ollier disease with malignant transformation is quite variable, with studies reporting the estimated incidence as 5% to 50%.⁷ Muramatsu and colleagues⁶ reported that the occurrence of malignant transformation of multiple enchondromas limited to the bones of the hand was extremely rare, with only 12 cases of malignant transformation. Enchondromas of the pelvis, scapula, and long bones of the extremities have increased risks and rates of secondary transformation to chondrosarcoma.⁸

A recent large European multicenter retrospective study investigating the clinical characteristics and behavior of enchondromas in 144 patients with Ollier disease has provided new information regarding this rare disease.⁷ Verdegaal and colleagues⁷ divided patients into 3 categories depending on their distribution of enchondromas. The development of chondrosarcoma was notably different between individuals with enchondromas limited to the small bones of the hands and feet (15%, group I) versus individuals with enchondromas limited to the long bones and flat bones (43%, group II) or individuals with enchondromas of the short, long, and flat bones (46%, group III).⁷ The only location found to be statistically significant for the development of chondrosarcoma was the pelvis.

The clinical findings associated with risk of malignant transformation of enchondromas are increasing size of the lesion and onset of pain and tenderness. Dahlin and Salvador⁹ reported that only 60% of patients with chondrosarcoma of the hand experience pain. The absence of pain may lead to a delay in patient presentation to the clinician.^5,6 Radiographic findings of malignant transformation include the classic features of temporal increases in the lesion’s size after skeletal maturity and cortical destruction associated with soft-tissue invasion. However, both findings are nonspecific for differentiating enchondromas from grade 1 chondrosarcomas as described by Geirnaerdt and colleagues.¹⁰

Sassoon and colleagues¹¹ reported on a series of hand enchondromas treated operatively. Subgroup analysis between pathologic fractures treated primarily or in delayed fashion showed similar outcomes for achieving full motion and similar number of complications; however, they noted that the delayed group required 7 more weeks of immobilization. Additionally, review of the whole series showed 1 episode of metacarpal shortening and 1 occurrence of angular malalignment. In our patient, we were concerned about introducing an iatrogenic cosmetic deformity, and we believed a pathologic fracture could be managed expectantly. Overall, patients without pathologic fracture treated surgically experienced a complication rate of 12%, whereas patients with a fracture had a complication rate of 20%.¹¹ The majority of patients with multiple enchondromatosis treated with surgical curettage and grafting had successful outcomes, with 86% of patients regaining full motion, but the recurrence rate was 21%.¹¹ Patients with expansile lesions regained less motion than patients with nonexpansile lesions. There was a single lesion believed preoperatively to be an enchondroma, but it underwent malignant transformation, as confirmed on intraoperative pathology. This patient had Maffucci syndrome and was treated with an amputation through the metacarpophalangeal joint.

There are 3 options for treating hand enchondromas: observation, curettage alone, or curettage with bone grafting. There is no consensus about conservative management, timing of intervention, or risk of pathologic fracture. Each patient is treated individually with attention to reason for presentation, number of lesions, associated pain, deformity, or pathologic fracture. Operative criteria include high risk of pathologic fracture based on location of enchondroma, cortical thinning, and previous pathologic fracture with resulting angular deformity. Nonoperative management may increase the risk of pathologic fracture, particularly in patients involved in aggressive contact sports, but the physician may offer protective splinting or counsel the patient on activity modification. Our case provides a study of the natural history of multiple enchondromatosis and shows mild increases in the lesions’ sizes during the 8-year follow-up. This was an expected finding given the patient’s immature skeleton. The lesions’ cortices continued to ossify after the physes closed and now provides an excellent comparison for the identification of future malignant changes. 

Histologic analysis of biopsied or surgically treated lesions contributes to the differentiation between benign hand enchondromas and chondrosarcoma. Pathologic findings must be correlated with clinical and radiographic findings because hand enchondromas contain cytologic features of chondrosarcoma.¹² In a series of 55 patients with chondrosarcoma, Liu and colleagues⁸ reported no cases from the hand. Verdegaal and colleagues⁷ reported a total of 13 chondrosarcomas in the metacarpals and hand phalanges in 97 group I and III patients. Five of these lesions were grade 1, 2 were grade 2, 1 was grade 3, and 5 lesions were unknown.

For patients with multiple enchondromatosis limited to the hands, prognosis is relatively good with respect to risk of secondary chondrosarcoma transformation, metastasis of secondary chondrosarcoma, and death. Verdegaal and colleagues⁷ reported the rate of secondary transformation in the hand to be 15%. Patil and colleagues¹³ reported no distant metastases in 23 patients with hand chondrosarcoma at mean follow-up of 8.5 years (range, 2-19 years), although none of their patients had Ollier disease. Verdegaal and colleagues⁷ reported 7 of the 8 deaths in their study were related to development of pulmonary metastases; however, none originated from chondrosarcomas in the hand. Additionally, there were no disease-related deaths in 29 group I patients. Herget and colleagues,¹⁴ in summarizing the literature, postulated that the overall survival rate of patients with secondary chondrosarcoma at 5 years is approximately 90%.

In our case, the patient, who had 3 enchondromas isolated to the left hand, can be categorized in group I. Thus, this case highlights the natural history of a patient with hand enchondromas and demonstrates that enchondromatosis of the short tubular bones of the hands can mature and ossify.

Ollier disease, or multiple enchondromatosis, is a rare nonfamilial condition characterized by multiple cartilaginous tumors often beginning in early childhood. There is significant variation in disease distribution, location, size, number of lesions, and behavior, but the tumors are often located unilaterally.¹ Enchondromas are most commonly found in the metacarpals, metatarsals, and phalanges, and develop from metaphyseal bone in close proximity to the physis. They frequently present as painless masses or are incidentally noted during the evaluation of another musculoskeletal condition. Radiographically, enchondromas of the hands and feet appear as oval radiolucencies with thinned, sclerotic rims. The lesions have varying degrees of mineralization and endosteal scalloping, and may expand the bone.² Enchondromas usually enlarge until skeletal maturity and have been observed to ossify spontaneously.^1,3 The clinical course of Ollier disease is variable, and a number of cases of significant hand deformity and malignant transformation have been reported.^4-6

In this case report, we present a mild form of Ollier disease isolated to the patient’s left hand, which we followed for 8 years, demonstrating part of the natural history of these lesions. We discuss the patient’s clinical features, radiologic findings, diagnosis, treatment, prognosis, and follow-up, as well as review the literature. The patient and the patient’s family provided written informed consent for print and electronic publication of this case report.

Case Report

A 10-year-old, right-handed girl was referred to our department for the evaluation of left-hand masses. At age 3 years, the patient underwent a chondroma excision from the middle phalanx of her middle finger on her left hand. No operative or pathology report was available from this surgery, and the patient tolerated the procedure well without any complications. At the time of presentation, the masses did not cause any pain, motor or sensory dysfunction, or any systemic symptoms. No history of recent or distant trauma was elicited. The patient’s medical and family history was unremarkable.

On physical examination, there was a firm, immobile, nontender palpable mass over the dorsal aspect of the distal second metacarpal bone of the left hand. The mass extended medially between the second and third metacarpals. A second small, firm, nontender left-hand mass was palpated over the volar aspect of her proximal phalanx on her index finger. She was neurovascularly intact with full active range of motion of the metacarpophalangeal and proximal and distal interphalangeal joints. There was no angular deformity of the digits. Plain radiographs taken at the time of initial presentation showed a 2.3×1.7-cm radiolucent lesion located in the metaphysis and diaphysis of the second metacarpal of the left hand (Figures 1A-1C). The lesion had varying degrees of mineralization with cortical thinning and expansion in the volar, dorsal, radial, and ulnar directions, consistent with a chondroid lesion. The second and third lesions were oval radiolucencies with sclerotic rims located at the metaphyseal-diaphyseal junction of the proximal phalanx of the index finger and middle phalanx of the middle finger, respectively. No fractures were identified in the radiographs, and the physes were open at this time. The patient was diagnosed with multiple enchondromatosis, or Ollier disease.

Our case showed 1 episode of pain and tenderness to palpation at the second proximal phalanx approximately 6 months after initial presentation. We attributed the pain and tenderness to a small pathologic fracture but did not see radiographic evidence of this. We elected to provide a trial of supportive measures, such as splinting and buddy taping, and to monitor the pain with a tentative plan of open biopsy with curettage and bone grafting if the pain persisted or evidence of fracture was seen on radiographs. The pain and tenderness to palpation resolved at a follow-up visit, and the surgery was deferred.

The patient was treated nonoperatively at initial presentation given the lack of significant cosmetic deformity or functional compromise and was advised close follow-up at 3 and 6 months. Given the absence of disease progression, annual checks (ie, clinical examination and radiographs) in a skeletally immature patient were decided on after consultation with the patient and parent. The family was educated about the possibility of pathologic fracture from minimal trauma to the hand versus the small risk of iatrogenic physeal injury with surgical curettage and bone grafting. No protective splinting was offered. A favorable prognosis and reassurance was provided to the patient and family, given the absence of symptoms, low suspicion and risk of malignant transformation, and stability of the lesion. Serial radiographs showed gradual increases in the lesions’ sizes but were consistent with the stable growth of the metacarpal and phalanges. With the patient nearing skeletal maturity, no pathologic fractures were identified on radiography during follow-up, and the risks of surgery lessened with growth; however, the continued absence of symptoms led to the mutual decision to continue observation.

Nearly 8 years after initial presentation, plain radiographs showed closed physes and partially ossified bone masses (Figures 2A-2C). The metacarpal lesion measured 3.2×1.5 cm, and the cortex appeared thickened and regular. The proximal phalanx lesion had a thickened cortex without periosteal reaction, and the middle phalanx lesion appeared to be completely healed. The patient has been asymptomatic for many years, and she has retained complete function of her left hand without any growth retardation, angular deformity, or pathologic fracture. A small but potential risk of malignant transformation was discussed with the patient and her family, as was the need for lifetime follow-up. We intend to follow the enchondromas clinically and radiographically every 2 years and obtain new radiographs if the mass presents with new clinical findings, such as enlargement or pain, for surveillance of tumor transformation. If the patient desired or symptoms developed, curettage and bone grafting would be offered, and the surgical tissue would be sent for pathologic analysis. A bone scan that was obtained at the request of the patient, when she was 21 years old, showed no other sites of disease besides the fingers.

Discussion

Multiple enchondromatosis was first described by Ollier at the turn of the 19th century and has been estimated to affect one in every 100,000 persons.¹ The low prevalence and variable manifestations of Ollier disease lead clinicians to handle the disease and its complications, namely skeletal deformity and malignant transformation, on a case-by-case approach. Additionally, the prognosis of Ollier disease with malignant transformation is quite variable, with studies reporting the estimated incidence as 5% to 50%.⁷ Muramatsu and colleagues⁶ reported that the occurrence of malignant transformation of multiple enchondromas limited to the bones of the hand was extremely rare, with only 12 cases of malignant transformation. Enchondromas of the pelvis, scapula, and long bones of the extremities have increased risks and rates of secondary transformation to chondrosarcoma.⁸

A recent large European multicenter retrospective study investigating the clinical characteristics and behavior of enchondromas in 144 patients with Ollier disease has provided new information regarding this rare disease.⁷ Verdegaal and colleagues⁷ divided patients into 3 categories depending on their distribution of enchondromas. The development of chondrosarcoma was notably different between individuals with enchondromas limited to the small bones of the hands and feet (15%, group I) versus individuals with enchondromas limited to the long bones and flat bones (43%, group II) or individuals with enchondromas of the short, long, and flat bones (46%, group III).⁷ The only location found to be statistically significant for the development of chondrosarcoma was the pelvis.

The clinical findings associated with risk of malignant transformation of enchondromas are increasing size of the lesion and onset of pain and tenderness. Dahlin and Salvador⁹ reported that only 60% of patients with chondrosarcoma of the hand experience pain. The absence of pain may lead to a delay in patient presentation to the clinician.^5,6 Radiographic findings of malignant transformation include the classic features of temporal increases in the lesion’s size after skeletal maturity and cortical destruction associated with soft-tissue invasion. However, both findings are nonspecific for differentiating enchondromas from grade 1 chondrosarcomas as described by Geirnaerdt and colleagues.¹⁰

Sassoon and colleagues¹¹ reported on a series of hand enchondromas treated operatively. Subgroup analysis between pathologic fractures treated primarily or in delayed fashion showed similar outcomes for achieving full motion and similar number of complications; however, they noted that the delayed group required 7 more weeks of immobilization. Additionally, review of the whole series showed 1 episode of metacarpal shortening and 1 occurrence of angular malalignment. In our patient, we were concerned about introducing an iatrogenic cosmetic deformity, and we believed a pathologic fracture could be managed expectantly. Overall, patients without pathologic fracture treated surgically experienced a complication rate of 12%, whereas patients with a fracture had a complication rate of 20%.¹¹ The majority of patients with multiple enchondromatosis treated with surgical curettage and grafting had successful outcomes, with 86% of patients regaining full motion, but the recurrence rate was 21%.¹¹ Patients with expansile lesions regained less motion than patients with nonexpansile lesions. There was a single lesion believed preoperatively to be an enchondroma, but it underwent malignant transformation, as confirmed on intraoperative pathology. This patient had Maffucci syndrome and was treated with an amputation through the metacarpophalangeal joint.

There are 3 options for treating hand enchondromas: observation, curettage alone, or curettage with bone grafting. There is no consensus about conservative management, timing of intervention, or risk of pathologic fracture. Each patient is treated individually with attention to reason for presentation, number of lesions, associated pain, deformity, or pathologic fracture. Operative criteria include high risk of pathologic fracture based on location of enchondroma, cortical thinning, and previous pathologic fracture with resulting angular deformity. Nonoperative management may increase the risk of pathologic fracture, particularly in patients involved in aggressive contact sports, but the physician may offer protective splinting or counsel the patient on activity modification. Our case provides a study of the natural history of multiple enchondromatosis and shows mild increases in the lesions’ sizes during the 8-year follow-up. This was an expected finding given the patient’s immature skeleton. The lesions’ cortices continued to ossify after the physes closed and now provides an excellent comparison for the identification of future malignant changes. 

Histologic analysis of biopsied or surgically treated lesions contributes to the differentiation between benign hand enchondromas and chondrosarcoma. Pathologic findings must be correlated with clinical and radiographic findings because hand enchondromas contain cytologic features of chondrosarcoma.¹² In a series of 55 patients with chondrosarcoma, Liu and colleagues⁸ reported no cases from the hand. Verdegaal and colleagues⁷ reported a total of 13 chondrosarcomas in the metacarpals and hand phalanges in 97 group I and III patients. Five of these lesions were grade 1, 2 were grade 2, 1 was grade 3, and 5 lesions were unknown.

For patients with multiple enchondromatosis limited to the hands, prognosis is relatively good with respect to risk of secondary chondrosarcoma transformation, metastasis of secondary chondrosarcoma, and death. Verdegaal and colleagues⁷ reported the rate of secondary transformation in the hand to be 15%. Patil and colleagues¹³ reported no distant metastases in 23 patients with hand chondrosarcoma at mean follow-up of 8.5 years (range, 2-19 years), although none of their patients had Ollier disease. Verdegaal and colleagues⁷ reported 7 of the 8 deaths in their study were related to development of pulmonary metastases; however, none originated from chondrosarcomas in the hand. Additionally, there were no disease-related deaths in 29 group I patients. Herget and colleagues,¹⁴ in summarizing the literature, postulated that the overall survival rate of patients with secondary chondrosarcoma at 5 years is approximately 90%.

In our case, the patient, who had 3 enchondromas isolated to the left hand, can be categorized in group I. Thus, this case highlights the natural history of a patient with hand enchondromas and demonstrates that enchondromatosis of the short tubular bones of the hands can mature and ossify.

THE CASE

A 72-year-old woman came to our internal medicine department clinic for a follow-up appointment for her fibromyalgia. Thirteen months earlier, she had sought care at our facility not only for fibromyalgia, but for insomnia, anxiety, depression, and urinary incontinence. At the time, we prescribed amitriptyline 10 mg/d—for her pain and depression—as well as clonazepam 10 mg/d and paracetamol 650 mg, as needed.

When she came in for the follow-up, she indicated that for the past 8 months, she’d been experiencing urinary retention that required her to self-catheterize 2 to 3 times a day. She said she hadn’t used other medicines or herbal products during this time.

The patient had visited her family physician several times over the previous few months, and had been referred to a urologist. During an episode of acute urinary retention, she went to the emergency department (ED), where the ED physician performed urinary catheterization and referred her to the hospital’s Urology Department. After 48 hours, she was evaluated by a urologist, who diagnosed chronic urinary retention related to a hypercontractile bladder, without any particular cause. She was advised to continue to catheterize herself when needed. She was also prescribed pyridostigmine bromide, but she stopped taking it because of abdominal pain and bloating.

Two months prior to her visit with us, the patient suffered a second acute urinary retention episode and returned to the ED. Urinary catheterization was performed for 72 hours. At her next visit to her urologist, she was told to continue self-catheterization and was prescribed silodosin 8 mg/d.

THE DIAGNOSIS

Based on the patient’s history, we suspected the urinary retention was secondary to the anticholinergic effects of amitriptyline. We were able to determine that the patient’s urinary retention was likely the result of an adverse drug reaction (ADR) by using the causality algorithm of the Spanish Pharmacovigilance System, which suggests the following criteria:¹ a) a positive time sequence (ie, onset of symptoms closely followed administration of the medication), b) the existence of an ADR that is well known and consistent with the mechanism of action of the drug,² c) symptoms that resolve after suspending the drug; d) no repeat exposure (to the adverse effects of amitriptyline) due to ethical reasons; and e) the absence of an alternative explanation for the symptoms.³

DISCUSSION

Although indicated for depression, amitriptyline is also used for other conditions, including nocturnal enuresis and chronic neuropathic pain.⁴ Amitriptyline exhibits anticholinergic effects that can cause symptoms related to the nervous system (agitation, disorientation, sleepiness, delirium, cognitive impairment), ocular system (blurred vision, dry eye, accommodation disturbances, increased intraocular pressure), cardiovascular system (tachycardia), gastrointestinal tract (dry mouth, paralytic ileus, constipation), urinary system (urinary retention); and skin and mucosal membranes (dryness).^5,6 Anticholinergic effects can also induce hyperthermia or increase the risk of falls.^5,6

Four other physicians had seen our patient, and none had considered the possibility that this was an adverse drug effect.

Anticholinergic medications can cause ADRs in high-risk older patients and thus are usually considered inappropriate for this patient population.⁶ The Anticholinergic Risk Scale (ARS) can be used to categorize medications based on their potential for anticholinergic adverse effects (TABLE).⁷ Amitriptyline is included in the group with the highest risk of ADRs. Amitriptyline is also included in the list of drugs that should be avoided in older adults, according to the 2012 American Geriatrics Society Beers Criteria.⁸

Our patient. We instructed her to stop taking amitriptyline, and her urinary retention disappeared within 48 hours. Two months later, she remained asymptomatic.

THE TAKEAWAY

Although many medications are known to cause adverse events, they can be missed when clinicians fail to pinpoint exactly when a new sign, symptom, or health problem appeared. This often leads to a chain reaction of unnecessary explorations, harmful treatment, patient suffering, and unjustified costs.^9-11 Our patient had seen 4 different health care providers (a family physician, urologist, and 2 ED physicians) before we saw her and ultimately made the diagnosis. Family physicians can prevent anticholinergic ADRs by using a scale, such as the ARS, before prescribing a medication.

THE CASE

A 72-year-old woman came to our internal medicine department clinic for a follow-up appointment for her fibromyalgia. Thirteen months earlier, she had sought care at our facility not only for fibromyalgia, but for insomnia, anxiety, depression, and urinary incontinence. At the time, we prescribed amitriptyline 10 mg/d—for her pain and depression—as well as clonazepam 10 mg/d and paracetamol 650 mg, as needed.

When she came in for the follow-up, she indicated that for the past 8 months, she’d been experiencing urinary retention that required her to self-catheterize 2 to 3 times a day. She said she hadn’t used other medicines or herbal products during this time.

The patient had visited her family physician several times over the previous few months, and had been referred to a urologist. During an episode of acute urinary retention, she went to the emergency department (ED), where the ED physician performed urinary catheterization and referred her to the hospital’s Urology Department. After 48 hours, she was evaluated by a urologist, who diagnosed chronic urinary retention related to a hypercontractile bladder, without any particular cause. She was advised to continue to catheterize herself when needed. She was also prescribed pyridostigmine bromide, but she stopped taking it because of abdominal pain and bloating.

Two months prior to her visit with us, the patient suffered a second acute urinary retention episode and returned to the ED. Urinary catheterization was performed for 72 hours. At her next visit to her urologist, she was told to continue self-catheterization and was prescribed silodosin 8 mg/d.

THE DIAGNOSIS

Based on the patient’s history, we suspected the urinary retention was secondary to the anticholinergic effects of amitriptyline. We were able to determine that the patient’s urinary retention was likely the result of an adverse drug reaction (ADR) by using the causality algorithm of the Spanish Pharmacovigilance System, which suggests the following criteria:¹ a) a positive time sequence (ie, onset of symptoms closely followed administration of the medication), b) the existence of an ADR that is well known and consistent with the mechanism of action of the drug,² c) symptoms that resolve after suspending the drug; d) no repeat exposure (to the adverse effects of amitriptyline) due to ethical reasons; and e) the absence of an alternative explanation for the symptoms.³

DISCUSSION

Although indicated for depression, amitriptyline is also used for other conditions, including nocturnal enuresis and chronic neuropathic pain.⁴ Amitriptyline exhibits anticholinergic effects that can cause symptoms related to the nervous system (agitation, disorientation, sleepiness, delirium, cognitive impairment), ocular system (blurred vision, dry eye, accommodation disturbances, increased intraocular pressure), cardiovascular system (tachycardia), gastrointestinal tract (dry mouth, paralytic ileus, constipation), urinary system (urinary retention); and skin and mucosal membranes (dryness).^5,6 Anticholinergic effects can also induce hyperthermia or increase the risk of falls.^5,6

Four other physicians had seen our patient, and none had considered the possibility that this was an adverse drug effect.

Anticholinergic medications can cause ADRs in high-risk older patients and thus are usually considered inappropriate for this patient population.⁶ The Anticholinergic Risk Scale (ARS) can be used to categorize medications based on their potential for anticholinergic adverse effects (TABLE).⁷ Amitriptyline is included in the group with the highest risk of ADRs. Amitriptyline is also included in the list of drugs that should be avoided in older adults, according to the 2012 American Geriatrics Society Beers Criteria.⁸

Our patient. We instructed her to stop taking amitriptyline, and her urinary retention disappeared within 48 hours. Two months later, she remained asymptomatic.

THE TAKEAWAY

Although many medications are known to cause adverse events, they can be missed when clinicians fail to pinpoint exactly when a new sign, symptom, or health problem appeared. This often leads to a chain reaction of unnecessary explorations, harmful treatment, patient suffering, and unjustified costs.^9-11 Our patient had seen 4 different health care providers (a family physician, urologist, and 2 ED physicians) before we saw her and ultimately made the diagnosis. Family physicians can prevent anticholinergic ADRs by using a scale, such as the ARS, before prescribing a medication.

THE CASE

A 72-year-old woman came to our internal medicine department clinic for a follow-up appointment for her fibromyalgia. Thirteen months earlier, she had sought care at our facility not only for fibromyalgia, but for insomnia, anxiety, depression, and urinary incontinence. At the time, we prescribed amitriptyline 10 mg/d—for her pain and depression—as well as clonazepam 10 mg/d and paracetamol 650 mg, as needed.

When she came in for the follow-up, she indicated that for the past 8 months, she’d been experiencing urinary retention that required her to self-catheterize 2 to 3 times a day. She said she hadn’t used other medicines or herbal products during this time.

The patient had visited her family physician several times over the previous few months, and had been referred to a urologist. During an episode of acute urinary retention, she went to the emergency department (ED), where the ED physician performed urinary catheterization and referred her to the hospital’s Urology Department. After 48 hours, she was evaluated by a urologist, who diagnosed chronic urinary retention related to a hypercontractile bladder, without any particular cause. She was advised to continue to catheterize herself when needed. She was also prescribed pyridostigmine bromide, but she stopped taking it because of abdominal pain and bloating.

Two months prior to her visit with us, the patient suffered a second acute urinary retention episode and returned to the ED. Urinary catheterization was performed for 72 hours. At her next visit to her urologist, she was told to continue self-catheterization and was prescribed silodosin 8 mg/d.

THE DIAGNOSIS

Based on the patient’s history, we suspected the urinary retention was secondary to the anticholinergic effects of amitriptyline. We were able to determine that the patient’s urinary retention was likely the result of an adverse drug reaction (ADR) by using the causality algorithm of the Spanish Pharmacovigilance System, which suggests the following criteria:¹ a) a positive time sequence (ie, onset of symptoms closely followed administration of the medication), b) the existence of an ADR that is well known and consistent with the mechanism of action of the drug,² c) symptoms that resolve after suspending the drug; d) no repeat exposure (to the adverse effects of amitriptyline) due to ethical reasons; and e) the absence of an alternative explanation for the symptoms.³

DISCUSSION

Although indicated for depression, amitriptyline is also used for other conditions, including nocturnal enuresis and chronic neuropathic pain.⁴ Amitriptyline exhibits anticholinergic effects that can cause symptoms related to the nervous system (agitation, disorientation, sleepiness, delirium, cognitive impairment), ocular system (blurred vision, dry eye, accommodation disturbances, increased intraocular pressure), cardiovascular system (tachycardia), gastrointestinal tract (dry mouth, paralytic ileus, constipation), urinary system (urinary retention); and skin and mucosal membranes (dryness).^5,6 Anticholinergic effects can also induce hyperthermia or increase the risk of falls.^5,6

Four other physicians had seen our patient, and none had considered the possibility that this was an adverse drug effect.

Anticholinergic medications can cause ADRs in high-risk older patients and thus are usually considered inappropriate for this patient population.⁶ The Anticholinergic Risk Scale (ARS) can be used to categorize medications based on their potential for anticholinergic adverse effects (TABLE).⁷ Amitriptyline is included in the group with the highest risk of ADRs. Amitriptyline is also included in the list of drugs that should be avoided in older adults, according to the 2012 American Geriatrics Society Beers Criteria.⁸

Our patient. We instructed her to stop taking amitriptyline, and her urinary retention disappeared within 48 hours. Two months later, she remained asymptomatic.

THE TAKEAWAY

Although many medications are known to cause adverse events, they can be missed when clinicians fail to pinpoint exactly when a new sign, symptom, or health problem appeared. This often leads to a chain reaction of unnecessary explorations, harmful treatment, patient suffering, and unjustified costs.^9-11 Our patient had seen 4 different health care providers (a family physician, urologist, and 2 ED physicians) before we saw her and ultimately made the diagnosis. Family physicians can prevent anticholinergic ADRs by using a scale, such as the ARS, before prescribing a medication.

THE CASE

A 25-year-old G2P2 woman came to our family practice clinic because she had multiple positive home pregnancy test results despite having undergone a sterilization procedure 4 years earlier. She said that 9 months ago, she had begun to experience hot flashes and night sweats that were getting progressively worse. Her menstrual cycles had been regular until 6 months earlier, when her bleeding became very light and irregular (2- to 6-week cycles with only one day of menstruation). Then 3 months ago, she stopped menstruating.

She’d had 2 uncomplicated pregnancies with normal vaginal deliveries 3 and 4 years ago, and had undergone a transcervical sterilization procedure after delivering her second child. Her medical history included hypothyroidism diagnosed at age 15, moderate persistent asthma, and seasonal allergies. She was taking levothyroxine 250 mcg/d, inhaled fluticasone/salmeterol, albuterol, and intranasal mometasone.

Transvaginal ultrasound failed to identify an intrauterine or ectopic pregnancy, and the patient’s ovaries were not visualized (uterine anatomy was normal with an endometrial stripe of 5.7 mm). The result of a serum human chorionic gonadotropin (hCG) test was 6.73 mIU/ mL. (In a nonpregnant, premenopausal woman, hCG is typically undetectable.) Subsequent serial hCG measurements remained low (6.72-7.09 mIU/mL), but persistent. Given these low hCG levels, it was imperative to rule out an intrauterine or ectopic pregnancy. A urine hCG was negative.

THE DIAGNOSIS

Because of our patient’s vasomotor symptoms, we ordered additional laboratory studies, which revealed an elevated follicle-stimulating hormone (FSH) level (66.08 mIU/mL and 42.2 mIU/mL taken one year apart; normal, 1.98-9.58 mIU/mL in a premenopausal female), an elevated luteinizing hormone (LH) level (46.1 mIU/mL; normal, 2.58-15.5 mIU/mL in a premenopausal female), a low thyroid-stimulating hormone (TSH) level (0.445 mIU/mL; normal, 0.465-4.65 mIU/mL), and a normal prolactin level (12.5 mIU/mL). Based on these results, we diagnosed primary ovarian insufficiency (POI).

DISCUSSION

POI, formerly known as premature ovarian failure, is defined as 4 to 6 months of amenorrhea or oligomenorrhea in a woman younger than 40 with an elevated FSH on 2 occasions, at least 4 weeks apart.^1-3

The etiology of POI is broad. It can be caused by a failure of the pituitary gland or hypothalamus to secrete regulating hormones to stimulate the ovaries. Possible genetic causes include Turner’s syndrome, fragile X permutation, and other autosomal disorders that cause follicle dysfunction or destruction.¹ Infections such as mumps, varicella, and tuberculosis are known to affect ovary function, as well.^1,4 In addition, women who are exposed to chemotherapy or radiation are at higher risk for developing POI.¹

Because POI and autoimmune disorders tend to occur together, consider screening any patient with POI for disorders such as hypothyroidism and Addison’s disease. A serum analysis to evaluate for autoantibodies against steroid-producing cells may be a potential marker for POI in patients with an autoimmune disease that affects the adrenal glands or thyroid. However, patients with isolated Addison’s disease, autoimmune hypothyroidism, or diabetes mellitus in the absence of POI do not appear to have steroid-specific antibodies.² In our patient’s case, her hypothyroidism may have placed her at higher risk of having a second organ system adversely affected by her immune system.

What causes a false-positive pregnancy test? This case is unique because our patient reported multiple positive home pregnancy test results and had persistently low serum hCG levels. While she had symptoms that suggested menopause (hot flashes, oligomenorrhea that progressed to amenorrhea), she believed these symptoms were related to pregnancy. In addition to pregnancy, an elevated serum hCG measurement can be due to various malignancies, molar pregnancy, pituitary production of hCG, elevated LH, cross-reactivity with multiple animal exposures (due to the production of human anti-animal antibodies that react with testing), and recent mononucleosis infection.⁵

Other potential causes for false-positive urine pregnancy test results include tuboovarian abscess,⁶ adenomyosis,⁷ and cancers that produce hCG, such as colon, pancreatic, lung, liver, and urothelial bladder carcinoma.^8,9 Urine with significant proteinuria can also cause a positive pregnancy test result.¹⁰

Our patient likely had a false-positive hCG due to elevated LH, secondary to POI, that demonstrated cross-reactivity on the hCG assay. The similarity in the chemical structure of the beta subunits of hCG and LH have been reported as false-positive tests in the absence of pregnancy.⁵

Hormone therapy and supplemental calcium and vitamin D are recommended for women with primary ovarian insufficiency to help prevent loss of bone density and other negative effects of low estrogen.

Because home pregnancy tests are designed to detect pregnancy as early as possible, they typically feature a high sensitivity by detecting very low levels of hCG, which leads to more frequent false-positive results. It is possible that different assay methods could account for the discrepancy between our patient’s positive home pregnancy tests and our negative laboratory urine pregnancy test.

Our patient and her husband were both counseled regarding her POI diagnosis. We conducted further studies to establish a possible etiology. She was found to have a normal karyotype of 46, XX, which ruled out Turner’s syndrome. Testing for permutations of the FMR1 gene was negative for fragile X syndrome, and antibody testing for thyroid and adrenal glands was negative for autoimmune disease.

Hormone therapy and supplemental calcium and vitamin D are recommended for women with POI to help prevent bone loss and other negative effects of low estrogen.¹¹ We did not take this tack with our patient, however, because she decided she wanted to pursue a tubal ligation reversal in order to get pregnant. So instead, we decreased her dose of levothyroxine to 150 mcg (since her TSH was low) and we referred her to the Reproductive Endocrinology Department.

THE TAKEAWAY

Although many cases of POI have no discernible etiology, it is important to rule out malignancies, failure of pituitary production, genetic causes, infections, and other possible causes. Hormone therapy and prophylactic doses of calcium and vitamin D are recommended for patients diagnosed with POI.

THE CASE

A 25-year-old G2P2 woman came to our family practice clinic because she had multiple positive home pregnancy test results despite having undergone a sterilization procedure 4 years earlier. She said that 9 months ago, she had begun to experience hot flashes and night sweats that were getting progressively worse. Her menstrual cycles had been regular until 6 months earlier, when her bleeding became very light and irregular (2- to 6-week cycles with only one day of menstruation). Then 3 months ago, she stopped menstruating.

She’d had 2 uncomplicated pregnancies with normal vaginal deliveries 3 and 4 years ago, and had undergone a transcervical sterilization procedure after delivering her second child. Her medical history included hypothyroidism diagnosed at age 15, moderate persistent asthma, and seasonal allergies. She was taking levothyroxine 250 mcg/d, inhaled fluticasone/salmeterol, albuterol, and intranasal mometasone.

Transvaginal ultrasound failed to identify an intrauterine or ectopic pregnancy, and the patient’s ovaries were not visualized (uterine anatomy was normal with an endometrial stripe of 5.7 mm). The result of a serum human chorionic gonadotropin (hCG) test was 6.73 mIU/ mL. (In a nonpregnant, premenopausal woman, hCG is typically undetectable.) Subsequent serial hCG measurements remained low (6.72-7.09 mIU/mL), but persistent. Given these low hCG levels, it was imperative to rule out an intrauterine or ectopic pregnancy. A urine hCG was negative.

THE DIAGNOSIS

Because of our patient’s vasomotor symptoms, we ordered additional laboratory studies, which revealed an elevated follicle-stimulating hormone (FSH) level (66.08 mIU/mL and 42.2 mIU/mL taken one year apart; normal, 1.98-9.58 mIU/mL in a premenopausal female), an elevated luteinizing hormone (LH) level (46.1 mIU/mL; normal, 2.58-15.5 mIU/mL in a premenopausal female), a low thyroid-stimulating hormone (TSH) level (0.445 mIU/mL; normal, 0.465-4.65 mIU/mL), and a normal prolactin level (12.5 mIU/mL). Based on these results, we diagnosed primary ovarian insufficiency (POI).

DISCUSSION

POI, formerly known as premature ovarian failure, is defined as 4 to 6 months of amenorrhea or oligomenorrhea in a woman younger than 40 with an elevated FSH on 2 occasions, at least 4 weeks apart.^1-3

The etiology of POI is broad. It can be caused by a failure of the pituitary gland or hypothalamus to secrete regulating hormones to stimulate the ovaries. Possible genetic causes include Turner’s syndrome, fragile X permutation, and other autosomal disorders that cause follicle dysfunction or destruction.¹ Infections such as mumps, varicella, and tuberculosis are known to affect ovary function, as well.^1,4 In addition, women who are exposed to chemotherapy or radiation are at higher risk for developing POI.¹

Because POI and autoimmune disorders tend to occur together, consider screening any patient with POI for disorders such as hypothyroidism and Addison’s disease. A serum analysis to evaluate for autoantibodies against steroid-producing cells may be a potential marker for POI in patients with an autoimmune disease that affects the adrenal glands or thyroid. However, patients with isolated Addison’s disease, autoimmune hypothyroidism, or diabetes mellitus in the absence of POI do not appear to have steroid-specific antibodies.² In our patient’s case, her hypothyroidism may have placed her at higher risk of having a second organ system adversely affected by her immune system.

What causes a false-positive pregnancy test? This case is unique because our patient reported multiple positive home pregnancy test results and had persistently low serum hCG levels. While she had symptoms that suggested menopause (hot flashes, oligomenorrhea that progressed to amenorrhea), she believed these symptoms were related to pregnancy. In addition to pregnancy, an elevated serum hCG measurement can be due to various malignancies, molar pregnancy, pituitary production of hCG, elevated LH, cross-reactivity with multiple animal exposures (due to the production of human anti-animal antibodies that react with testing), and recent mononucleosis infection.⁵

Other potential causes for false-positive urine pregnancy test results include tuboovarian abscess,⁶ adenomyosis,⁷ and cancers that produce hCG, such as colon, pancreatic, lung, liver, and urothelial bladder carcinoma.^8,9 Urine with significant proteinuria can also cause a positive pregnancy test result.¹⁰

Our patient likely had a false-positive hCG due to elevated LH, secondary to POI, that demonstrated cross-reactivity on the hCG assay. The similarity in the chemical structure of the beta subunits of hCG and LH have been reported as false-positive tests in the absence of pregnancy.⁵

Hormone therapy and supplemental calcium and vitamin D are recommended for women with primary ovarian insufficiency to help prevent loss of bone density and other negative effects of low estrogen.

Because home pregnancy tests are designed to detect pregnancy as early as possible, they typically feature a high sensitivity by detecting very low levels of hCG, which leads to more frequent false-positive results. It is possible that different assay methods could account for the discrepancy between our patient’s positive home pregnancy tests and our negative laboratory urine pregnancy test.

Our patient and her husband were both counseled regarding her POI diagnosis. We conducted further studies to establish a possible etiology. She was found to have a normal karyotype of 46, XX, which ruled out Turner’s syndrome. Testing for permutations of the FMR1 gene was negative for fragile X syndrome, and antibody testing for thyroid and adrenal glands was negative for autoimmune disease.

Hormone therapy and supplemental calcium and vitamin D are recommended for women with POI to help prevent bone loss and other negative effects of low estrogen.¹¹ We did not take this tack with our patient, however, because she decided she wanted to pursue a tubal ligation reversal in order to get pregnant. So instead, we decreased her dose of levothyroxine to 150 mcg (since her TSH was low) and we referred her to the Reproductive Endocrinology Department.

THE TAKEAWAY

Although many cases of POI have no discernible etiology, it is important to rule out malignancies, failure of pituitary production, genetic causes, infections, and other possible causes. Hormone therapy and prophylactic doses of calcium and vitamin D are recommended for patients diagnosed with POI.

THE CASE

A 25-year-old G2P2 woman came to our family practice clinic because she had multiple positive home pregnancy test results despite having undergone a sterilization procedure 4 years earlier. She said that 9 months ago, she had begun to experience hot flashes and night sweats that were getting progressively worse. Her menstrual cycles had been regular until 6 months earlier, when her bleeding became very light and irregular (2- to 6-week cycles with only one day of menstruation). Then 3 months ago, she stopped menstruating.

She’d had 2 uncomplicated pregnancies with normal vaginal deliveries 3 and 4 years ago, and had undergone a transcervical sterilization procedure after delivering her second child. Her medical history included hypothyroidism diagnosed at age 15, moderate persistent asthma, and seasonal allergies. She was taking levothyroxine 250 mcg/d, inhaled fluticasone/salmeterol, albuterol, and intranasal mometasone.

Transvaginal ultrasound failed to identify an intrauterine or ectopic pregnancy, and the patient’s ovaries were not visualized (uterine anatomy was normal with an endometrial stripe of 5.7 mm). The result of a serum human chorionic gonadotropin (hCG) test was 6.73 mIU/ mL. (In a nonpregnant, premenopausal woman, hCG is typically undetectable.) Subsequent serial hCG measurements remained low (6.72-7.09 mIU/mL), but persistent. Given these low hCG levels, it was imperative to rule out an intrauterine or ectopic pregnancy. A urine hCG was negative.

THE DIAGNOSIS

Because of our patient’s vasomotor symptoms, we ordered additional laboratory studies, which revealed an elevated follicle-stimulating hormone (FSH) level (66.08 mIU/mL and 42.2 mIU/mL taken one year apart; normal, 1.98-9.58 mIU/mL in a premenopausal female), an elevated luteinizing hormone (LH) level (46.1 mIU/mL; normal, 2.58-15.5 mIU/mL in a premenopausal female), a low thyroid-stimulating hormone (TSH) level (0.445 mIU/mL; normal, 0.465-4.65 mIU/mL), and a normal prolactin level (12.5 mIU/mL). Based on these results, we diagnosed primary ovarian insufficiency (POI).

DISCUSSION

POI, formerly known as premature ovarian failure, is defined as 4 to 6 months of amenorrhea or oligomenorrhea in a woman younger than 40 with an elevated FSH on 2 occasions, at least 4 weeks apart.^1-3

The etiology of POI is broad. It can be caused by a failure of the pituitary gland or hypothalamus to secrete regulating hormones to stimulate the ovaries. Possible genetic causes include Turner’s syndrome, fragile X permutation, and other autosomal disorders that cause follicle dysfunction or destruction.¹ Infections such as mumps, varicella, and tuberculosis are known to affect ovary function, as well.^1,4 In addition, women who are exposed to chemotherapy or radiation are at higher risk for developing POI.¹

Because POI and autoimmune disorders tend to occur together, consider screening any patient with POI for disorders such as hypothyroidism and Addison’s disease. A serum analysis to evaluate for autoantibodies against steroid-producing cells may be a potential marker for POI in patients with an autoimmune disease that affects the adrenal glands or thyroid. However, patients with isolated Addison’s disease, autoimmune hypothyroidism, or diabetes mellitus in the absence of POI do not appear to have steroid-specific antibodies.² In our patient’s case, her hypothyroidism may have placed her at higher risk of having a second organ system adversely affected by her immune system.

What causes a false-positive pregnancy test? This case is unique because our patient reported multiple positive home pregnancy test results and had persistently low serum hCG levels. While she had symptoms that suggested menopause (hot flashes, oligomenorrhea that progressed to amenorrhea), she believed these symptoms were related to pregnancy. In addition to pregnancy, an elevated serum hCG measurement can be due to various malignancies, molar pregnancy, pituitary production of hCG, elevated LH, cross-reactivity with multiple animal exposures (due to the production of human anti-animal antibodies that react with testing), and recent mononucleosis infection.⁵

Other potential causes for false-positive urine pregnancy test results include tuboovarian abscess,⁶ adenomyosis,⁷ and cancers that produce hCG, such as colon, pancreatic, lung, liver, and urothelial bladder carcinoma.^8,9 Urine with significant proteinuria can also cause a positive pregnancy test result.¹⁰

Our patient likely had a false-positive hCG due to elevated LH, secondary to POI, that demonstrated cross-reactivity on the hCG assay. The similarity in the chemical structure of the beta subunits of hCG and LH have been reported as false-positive tests in the absence of pregnancy.⁵

Hormone therapy and supplemental calcium and vitamin D are recommended for women with primary ovarian insufficiency to help prevent loss of bone density and other negative effects of low estrogen.

Because home pregnancy tests are designed to detect pregnancy as early as possible, they typically feature a high sensitivity by detecting very low levels of hCG, which leads to more frequent false-positive results. It is possible that different assay methods could account for the discrepancy between our patient’s positive home pregnancy tests and our negative laboratory urine pregnancy test.

Our patient and her husband were both counseled regarding her POI diagnosis. We conducted further studies to establish a possible etiology. She was found to have a normal karyotype of 46, XX, which ruled out Turner’s syndrome. Testing for permutations of the FMR1 gene was negative for fragile X syndrome, and antibody testing for thyroid and adrenal glands was negative for autoimmune disease.

Hormone therapy and supplemental calcium and vitamin D are recommended for women with POI to help prevent bone loss and other negative effects of low estrogen.¹¹ We did not take this tack with our patient, however, because she decided she wanted to pursue a tubal ligation reversal in order to get pregnant. So instead, we decreased her dose of levothyroxine to 150 mcg (since her TSH was low) and we referred her to the Reproductive Endocrinology Department.

THE TAKEAWAY

Although many cases of POI have no discernible etiology, it is important to rule out malignancies, failure of pituitary production, genetic causes, infections, and other possible causes. Hormone therapy and prophylactic doses of calcium and vitamin D are recommended for patients diagnosed with POI.

High tibial osteotomy has proved successful in treating unicompartmental arthritis in young, active patients.^1-3 However, this procedure fails over time because the other compartments deteriorate.⁴ The next step is conversion of the osteotomy to total knee arthroplasty (TKA). Conversion results vary, with several authors reporting poor outcomes^5-9 and others reporting outcomes equal to those of primary TKA.^10-14

The long-term success of TKA depends on proper restoration of the mechanical axis and soft-tissue balancing.¹⁵ Preexisting extra-articular deformity may adversely affect outcomes. A deformity of more than 15° may make it difficult to obtain intra-articular correction of an extra-articular deformity through soft-tissue balancing alone.¹⁶

In this article, we report the unique case of a patient whose bilateral high tibial osteotomies failed because of excessive extra-articular deformity. TKAs were performed consecutively, in 2 separate settings. Each TKA was combined with a recorrection tibial osteotomy in a single operation, allowing for re-creation of normal knee alignment with ligament balance. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 58-year-old man (weight, 250 pounds; body mass index, 30) underwent staged bilateral medial opening wedge osteotomies using distraction osteogenesis. A uniplanar external fixator was used for fixation on each knee. Before surgery, anatomical axis was 2° (right knee) and –1° (left knee) (Figure 1A), and tibial slope was 9° (right) and 8° (left) (Figures 1B, 1C). The procedures were performed 10 months apart. After surgery, anatomical alignment was 17° valgus (right knee) and 12° valgus (left knee) (Figure 2), and tibial slope was 20° (right) and 13° (left).

The patient received mild relief of his arthritis symptoms. Fifty-six months after the index operation, he decided to undergo conversion of the right high tibial osteotomy to TKA because of progressive painful arthritis of the knee. Excessive valgus alignment caused by the initial osteotomy raised concerns about being able to correct the extra-articular deformity intra-articularly while maintaining kinematic ligament balance. For this reason, a recorrection osteotomy was performed concurrently with the TKA. A posterior cruciate ligament–retaining (PCL-retaining) knee design (NexGen, Zimmer) was selected.

The procedure began with bone cuts for the TKA. Initial cuts were made on the femur. The tibial cut was made in valgus corresponding to the preoperative valgus deformity. The tibial recorrection osteotomy was made at the level of the original osteotomy site. A stemmed tibial component was used to cross the osteotomy site, correcting the valgus deformity and providing stability at the osteotomy site. A 3.5-mm locking compression T-plate (Synthes) was medially placed to prevent loss of correction and control rotation of the osteotomy during healing. The patient began range of motion on postoperative day 1. Continuous passive motion was not used. Protective weight-bearing continued for 6 weeks. After 6 weeks, and once there was radiograph evidence of healing at the osteotomy site, full weight-bearing was allowed.

After 4 months, the patient decided to undergo a similar procedure on the left knee. Postoperative rehabilitation was the same. A year after the bilateral TKAs, the patient maintained a Knee Society Score of 95 and a functional score of 90. After surgery, anatomical alignment was 6° (right knee) and 3° (left knee) (Figure 3), and tibial slope was 6° (right) and 7° (left) (Figures 4A, 4B). In each knee, the PCL was preserved with ligament balance.

Discussion

Clinical outcomes of TKA after high tibial osteotomy vary. Windsor and colleagues⁹ reported that knee arthroplasties after tibial osteotomy were less successful than primary TKAs. In small studies, both Staeheli and colleagues¹⁷ and Katz and colleagues⁵ found that TKA outcomes after osteotomy were satisfactory compared with outcomes of primary TKA without previous osteotomy. A meta-analysis by Ramappa and colleagues¹⁸ showed no difference in outcomes between TKAs with and without previous osteotomy. In addition, there were no differences in outcomes between TKAs performed after opening wedge versus closing wedge osteotomies.¹⁹

An arthritic knee compartment is unloaded when a high tibial osteotomy produces an extra-articular deformity. Neyret and colleagues⁷ reported difficulties in correcting angulations of 9° or more through soft-tissue release. Cameron and Welsh¹⁶ suggested pre–knee arthroplasty correction of the extra-articular deformity for malalignments of more than 15°. In cases of severe malalignment produced by an osteotomy, Katz and colleagues⁵ also suggested that a second osteotomy be performed to correct alignment before TKA.

For TKA after high tibial osteotomy, a neutral plateau resection removes more bone medially than laterally, creating medial laxity. Without correction of the tibial deformity, lateral release (or, as Krackow and Holtgrewe²⁰ advocated, medial advancement) is required for ligament stability. Both technically demanding options may not provide complete stability throughout the arc of motion. In addition, neither corrects for rotational or sagittal deformities (the concern with correcting an extra-articular deformity with intra-articular ligament balancing).

Another option is valgus tibial resection, which maintains native ligament balance at the cost of excessive valgus alignment. In the low-demand patient, a condylar constrained implant provides a means of correcting the malalignment with knee stability.^8,13,17 The increased restraint produces greater forces at the implant–bone interface and may risk early loosening.

The case presented here represents a unique situation of failed bilateral high tibial osteotomies with excessive valgus malalignment. In a similar situation, Papagelopoulos and colleagues²¹ suggested correcting fracture deformities before or at time of knee arthroplasty. Yoshina and colleagues²² reported using a stemmed tibial component with TKA in treating nonunion of a high tibial osteotomy. As mentioned, Katz and colleagues⁵ and Neyret and colleagues⁷ suggested preoperative correction of the osteotomy in cases of severe malalignment. Others have suggested combining recorrection osteotomy and knee arthroplasty in either consecutive operations or a single operation.^23-26 Wolff and colleagues²⁷ and Uchinou and colleagues²⁸ described recorrection osteotomy performed concurrent with TKA. The present article is the first to report a case involving concurrent bilateral recorrection osteotomy and TKA.

In one setting, the recorrection osteotomy is performed after the bony cuts are made for the TKA. The initial tibial plateau resection is performed in valgus at the same degree of malalignment as the osteotomy. This allows the plane of the tibial resection to parallel the floor once the recorrection is finished. With use of a tibial stem crossing the osteotomy site and a derotation plate, adequate fixation of the osteotomy is obtained. The recorrection osteotomy prevents the ligaments from overlengthening, allows the native ligament balance of the knee, and preserves the PCL—which lets the surgeon obtain ligament balance for the TKA throughout the arc of motion, avoiding midstance instabilities and achieving knee alignment rotationally and in the coronal and sagittal planes.

The TKA used in the present case was a PCL-retaining design. Both posterior-stabilized and PCL-retaining designs are reasonable options for use in combination with recorrection osteotomy. A stemmed tibial component is needed to cross the osteotomy site. In our patient’s case, use of a PCL-retaining design was based on surgeon preference and experience.

Patella infera has been noted as a problem in studies on converting high tibial osteotomy to TKA.^9,12,29 A postulated cause is scarring of the infrapatellar tendon after high tibial osteotomy. In addition, a higher incidence of lateral retinacular release has been identified.^9-11 Patella infera did not occur in either knee in the present case, and lateral release was not required.

Our patient’s lateral radiographs (Figures 4A, 4B) showed persistence of the osteotomy plane anterior to the tibia. The osteotomy healed posteriorly but not completely anteriorly. This raises the issue of risk for nonunion when recorrection osteotomy is performed with TKA. Use of a stemmed tibial implant with a derotation plate provides the benefit of intramedullary fixation for the recorrection osteotomy. If the recorrection osteotomy were performed in a separate setting before TKA, plate fixation would be the primary fixation option. Should nonunion occur at the recorrection osteotomy site, revision of the tibial plateau with a new stemmed implant would be required in combination with plate fixation. Madelaine and colleagues³⁰ reported on a series of 15 severe varus knees treated with both osteotomy and TKA. Two nonunions occurred. Fixation was a staple in one case and a cement wedge in the other. Risk for nonunion may be reduced with the combination of stemmed tibial implant and internal fixation with a derotation plate. Protective weight-bearing is recommended for the first 6 postoperative weeks.

Conclusion

Ligament imbalances produced by high tibial osteotomy and exacerbated by conversion to TKA are difficult to address. In this report, we have described successful single-stage high tibial osteotomy recorrection and TKA performed bilaterally in separate settings. With use of a stemmed tibial component and a derotation plate, solid fixation was obtained with an excellent clinical outcome. The malalignment was corrected while ligament balance was maintained for a PCL-retaining TKA design.

High tibial osteotomy has proved successful in treating unicompartmental arthritis in young, active patients.^1-3 However, this procedure fails over time because the other compartments deteriorate.⁴ The next step is conversion of the osteotomy to total knee arthroplasty (TKA). Conversion results vary, with several authors reporting poor outcomes^5-9 and others reporting outcomes equal to those of primary TKA.^10-14

The long-term success of TKA depends on proper restoration of the mechanical axis and soft-tissue balancing.¹⁵ Preexisting extra-articular deformity may adversely affect outcomes. A deformity of more than 15° may make it difficult to obtain intra-articular correction of an extra-articular deformity through soft-tissue balancing alone.¹⁶

In this article, we report the unique case of a patient whose bilateral high tibial osteotomies failed because of excessive extra-articular deformity. TKAs were performed consecutively, in 2 separate settings. Each TKA was combined with a recorrection tibial osteotomy in a single operation, allowing for re-creation of normal knee alignment with ligament balance. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 58-year-old man (weight, 250 pounds; body mass index, 30) underwent staged bilateral medial opening wedge osteotomies using distraction osteogenesis. A uniplanar external fixator was used for fixation on each knee. Before surgery, anatomical axis was 2° (right knee) and –1° (left knee) (Figure 1A), and tibial slope was 9° (right) and 8° (left) (Figures 1B, 1C). The procedures were performed 10 months apart. After surgery, anatomical alignment was 17° valgus (right knee) and 12° valgus (left knee) (Figure 2), and tibial slope was 20° (right) and 13° (left).

The patient received mild relief of his arthritis symptoms. Fifty-six months after the index operation, he decided to undergo conversion of the right high tibial osteotomy to TKA because of progressive painful arthritis of the knee. Excessive valgus alignment caused by the initial osteotomy raised concerns about being able to correct the extra-articular deformity intra-articularly while maintaining kinematic ligament balance. For this reason, a recorrection osteotomy was performed concurrently with the TKA. A posterior cruciate ligament–retaining (PCL-retaining) knee design (NexGen, Zimmer) was selected.

The procedure began with bone cuts for the TKA. Initial cuts were made on the femur. The tibial cut was made in valgus corresponding to the preoperative valgus deformity. The tibial recorrection osteotomy was made at the level of the original osteotomy site. A stemmed tibial component was used to cross the osteotomy site, correcting the valgus deformity and providing stability at the osteotomy site. A 3.5-mm locking compression T-plate (Synthes) was medially placed to prevent loss of correction and control rotation of the osteotomy during healing. The patient began range of motion on postoperative day 1. Continuous passive motion was not used. Protective weight-bearing continued for 6 weeks. After 6 weeks, and once there was radiograph evidence of healing at the osteotomy site, full weight-bearing was allowed.

After 4 months, the patient decided to undergo a similar procedure on the left knee. Postoperative rehabilitation was the same. A year after the bilateral TKAs, the patient maintained a Knee Society Score of 95 and a functional score of 90. After surgery, anatomical alignment was 6° (right knee) and 3° (left knee) (Figure 3), and tibial slope was 6° (right) and 7° (left) (Figures 4A, 4B). In each knee, the PCL was preserved with ligament balance.

Discussion

Clinical outcomes of TKA after high tibial osteotomy vary. Windsor and colleagues⁹ reported that knee arthroplasties after tibial osteotomy were less successful than primary TKAs. In small studies, both Staeheli and colleagues¹⁷ and Katz and colleagues⁵ found that TKA outcomes after osteotomy were satisfactory compared with outcomes of primary TKA without previous osteotomy. A meta-analysis by Ramappa and colleagues¹⁸ showed no difference in outcomes between TKAs with and without previous osteotomy. In addition, there were no differences in outcomes between TKAs performed after opening wedge versus closing wedge osteotomies.¹⁹

An arthritic knee compartment is unloaded when a high tibial osteotomy produces an extra-articular deformity. Neyret and colleagues⁷ reported difficulties in correcting angulations of 9° or more through soft-tissue release. Cameron and Welsh¹⁶ suggested pre–knee arthroplasty correction of the extra-articular deformity for malalignments of more than 15°. In cases of severe malalignment produced by an osteotomy, Katz and colleagues⁵ also suggested that a second osteotomy be performed to correct alignment before TKA.

For TKA after high tibial osteotomy, a neutral plateau resection removes more bone medially than laterally, creating medial laxity. Without correction of the tibial deformity, lateral release (or, as Krackow and Holtgrewe²⁰ advocated, medial advancement) is required for ligament stability. Both technically demanding options may not provide complete stability throughout the arc of motion. In addition, neither corrects for rotational or sagittal deformities (the concern with correcting an extra-articular deformity with intra-articular ligament balancing).

Another option is valgus tibial resection, which maintains native ligament balance at the cost of excessive valgus alignment. In the low-demand patient, a condylar constrained implant provides a means of correcting the malalignment with knee stability.^8,13,17 The increased restraint produces greater forces at the implant–bone interface and may risk early loosening.

The case presented here represents a unique situation of failed bilateral high tibial osteotomies with excessive valgus malalignment. In a similar situation, Papagelopoulos and colleagues²¹ suggested correcting fracture deformities before or at time of knee arthroplasty. Yoshina and colleagues²² reported using a stemmed tibial component with TKA in treating nonunion of a high tibial osteotomy. As mentioned, Katz and colleagues⁵ and Neyret and colleagues⁷ suggested preoperative correction of the osteotomy in cases of severe malalignment. Others have suggested combining recorrection osteotomy and knee arthroplasty in either consecutive operations or a single operation.^23-26 Wolff and colleagues²⁷ and Uchinou and colleagues²⁸ described recorrection osteotomy performed concurrent with TKA. The present article is the first to report a case involving concurrent bilateral recorrection osteotomy and TKA.

In one setting, the recorrection osteotomy is performed after the bony cuts are made for the TKA. The initial tibial plateau resection is performed in valgus at the same degree of malalignment as the osteotomy. This allows the plane of the tibial resection to parallel the floor once the recorrection is finished. With use of a tibial stem crossing the osteotomy site and a derotation plate, adequate fixation of the osteotomy is obtained. The recorrection osteotomy prevents the ligaments from overlengthening, allows the native ligament balance of the knee, and preserves the PCL—which lets the surgeon obtain ligament balance for the TKA throughout the arc of motion, avoiding midstance instabilities and achieving knee alignment rotationally and in the coronal and sagittal planes.

The TKA used in the present case was a PCL-retaining design. Both posterior-stabilized and PCL-retaining designs are reasonable options for use in combination with recorrection osteotomy. A stemmed tibial component is needed to cross the osteotomy site. In our patient’s case, use of a PCL-retaining design was based on surgeon preference and experience.

Patella infera has been noted as a problem in studies on converting high tibial osteotomy to TKA.^9,12,29 A postulated cause is scarring of the infrapatellar tendon after high tibial osteotomy. In addition, a higher incidence of lateral retinacular release has been identified.^9-11 Patella infera did not occur in either knee in the present case, and lateral release was not required.

Our patient’s lateral radiographs (Figures 4A, 4B) showed persistence of the osteotomy plane anterior to the tibia. The osteotomy healed posteriorly but not completely anteriorly. This raises the issue of risk for nonunion when recorrection osteotomy is performed with TKA. Use of a stemmed tibial implant with a derotation plate provides the benefit of intramedullary fixation for the recorrection osteotomy. If the recorrection osteotomy were performed in a separate setting before TKA, plate fixation would be the primary fixation option. Should nonunion occur at the recorrection osteotomy site, revision of the tibial plateau with a new stemmed implant would be required in combination with plate fixation. Madelaine and colleagues³⁰ reported on a series of 15 severe varus knees treated with both osteotomy and TKA. Two nonunions occurred. Fixation was a staple in one case and a cement wedge in the other. Risk for nonunion may be reduced with the combination of stemmed tibial implant and internal fixation with a derotation plate. Protective weight-bearing is recommended for the first 6 postoperative weeks.

Conclusion

Ligament imbalances produced by high tibial osteotomy and exacerbated by conversion to TKA are difficult to address. In this report, we have described successful single-stage high tibial osteotomy recorrection and TKA performed bilaterally in separate settings. With use of a stemmed tibial component and a derotation plate, solid fixation was obtained with an excellent clinical outcome. The malalignment was corrected while ligament balance was maintained for a PCL-retaining TKA design.

High tibial osteotomy has proved successful in treating unicompartmental arthritis in young, active patients.^1-3 However, this procedure fails over time because the other compartments deteriorate.⁴ The next step is conversion of the osteotomy to total knee arthroplasty (TKA). Conversion results vary, with several authors reporting poor outcomes^5-9 and others reporting outcomes equal to those of primary TKA.^10-14

The long-term success of TKA depends on proper restoration of the mechanical axis and soft-tissue balancing.¹⁵ Preexisting extra-articular deformity may adversely affect outcomes. A deformity of more than 15° may make it difficult to obtain intra-articular correction of an extra-articular deformity through soft-tissue balancing alone.¹⁶

In this article, we report the unique case of a patient whose bilateral high tibial osteotomies failed because of excessive extra-articular deformity. TKAs were performed consecutively, in 2 separate settings. Each TKA was combined with a recorrection tibial osteotomy in a single operation, allowing for re-creation of normal knee alignment with ligament balance. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 58-year-old man (weight, 250 pounds; body mass index, 30) underwent staged bilateral medial opening wedge osteotomies using distraction osteogenesis. A uniplanar external fixator was used for fixation on each knee. Before surgery, anatomical axis was 2° (right knee) and –1° (left knee) (Figure 1A), and tibial slope was 9° (right) and 8° (left) (Figures 1B, 1C). The procedures were performed 10 months apart. After surgery, anatomical alignment was 17° valgus (right knee) and 12° valgus (left knee) (Figure 2), and tibial slope was 20° (right) and 13° (left).

The patient received mild relief of his arthritis symptoms. Fifty-six months after the index operation, he decided to undergo conversion of the right high tibial osteotomy to TKA because of progressive painful arthritis of the knee. Excessive valgus alignment caused by the initial osteotomy raised concerns about being able to correct the extra-articular deformity intra-articularly while maintaining kinematic ligament balance. For this reason, a recorrection osteotomy was performed concurrently with the TKA. A posterior cruciate ligament–retaining (PCL-retaining) knee design (NexGen, Zimmer) was selected.

The procedure began with bone cuts for the TKA. Initial cuts were made on the femur. The tibial cut was made in valgus corresponding to the preoperative valgus deformity. The tibial recorrection osteotomy was made at the level of the original osteotomy site. A stemmed tibial component was used to cross the osteotomy site, correcting the valgus deformity and providing stability at the osteotomy site. A 3.5-mm locking compression T-plate (Synthes) was medially placed to prevent loss of correction and control rotation of the osteotomy during healing. The patient began range of motion on postoperative day 1. Continuous passive motion was not used. Protective weight-bearing continued for 6 weeks. After 6 weeks, and once there was radiograph evidence of healing at the osteotomy site, full weight-bearing was allowed.

After 4 months, the patient decided to undergo a similar procedure on the left knee. Postoperative rehabilitation was the same. A year after the bilateral TKAs, the patient maintained a Knee Society Score of 95 and a functional score of 90. After surgery, anatomical alignment was 6° (right knee) and 3° (left knee) (Figure 3), and tibial slope was 6° (right) and 7° (left) (Figures 4A, 4B). In each knee, the PCL was preserved with ligament balance.

Discussion

Clinical outcomes of TKA after high tibial osteotomy vary. Windsor and colleagues⁹ reported that knee arthroplasties after tibial osteotomy were less successful than primary TKAs. In small studies, both Staeheli and colleagues¹⁷ and Katz and colleagues⁵ found that TKA outcomes after osteotomy were satisfactory compared with outcomes of primary TKA without previous osteotomy. A meta-analysis by Ramappa and colleagues¹⁸ showed no difference in outcomes between TKAs with and without previous osteotomy. In addition, there were no differences in outcomes between TKAs performed after opening wedge versus closing wedge osteotomies.¹⁹

An arthritic knee compartment is unloaded when a high tibial osteotomy produces an extra-articular deformity. Neyret and colleagues⁷ reported difficulties in correcting angulations of 9° or more through soft-tissue release. Cameron and Welsh¹⁶ suggested pre–knee arthroplasty correction of the extra-articular deformity for malalignments of more than 15°. In cases of severe malalignment produced by an osteotomy, Katz and colleagues⁵ also suggested that a second osteotomy be performed to correct alignment before TKA.

For TKA after high tibial osteotomy, a neutral plateau resection removes more bone medially than laterally, creating medial laxity. Without correction of the tibial deformity, lateral release (or, as Krackow and Holtgrewe²⁰ advocated, medial advancement) is required for ligament stability. Both technically demanding options may not provide complete stability throughout the arc of motion. In addition, neither corrects for rotational or sagittal deformities (the concern with correcting an extra-articular deformity with intra-articular ligament balancing).

Another option is valgus tibial resection, which maintains native ligament balance at the cost of excessive valgus alignment. In the low-demand patient, a condylar constrained implant provides a means of correcting the malalignment with knee stability.^8,13,17 The increased restraint produces greater forces at the implant–bone interface and may risk early loosening.

The case presented here represents a unique situation of failed bilateral high tibial osteotomies with excessive valgus malalignment. In a similar situation, Papagelopoulos and colleagues²¹ suggested correcting fracture deformities before or at time of knee arthroplasty. Yoshina and colleagues²² reported using a stemmed tibial component with TKA in treating nonunion of a high tibial osteotomy. As mentioned, Katz and colleagues⁵ and Neyret and colleagues⁷ suggested preoperative correction of the osteotomy in cases of severe malalignment. Others have suggested combining recorrection osteotomy and knee arthroplasty in either consecutive operations or a single operation.^23-26 Wolff and colleagues²⁷ and Uchinou and colleagues²⁸ described recorrection osteotomy performed concurrent with TKA. The present article is the first to report a case involving concurrent bilateral recorrection osteotomy and TKA.

In one setting, the recorrection osteotomy is performed after the bony cuts are made for the TKA. The initial tibial plateau resection is performed in valgus at the same degree of malalignment as the osteotomy. This allows the plane of the tibial resection to parallel the floor once the recorrection is finished. With use of a tibial stem crossing the osteotomy site and a derotation plate, adequate fixation of the osteotomy is obtained. The recorrection osteotomy prevents the ligaments from overlengthening, allows the native ligament balance of the knee, and preserves the PCL—which lets the surgeon obtain ligament balance for the TKA throughout the arc of motion, avoiding midstance instabilities and achieving knee alignment rotationally and in the coronal and sagittal planes.

The TKA used in the present case was a PCL-retaining design. Both posterior-stabilized and PCL-retaining designs are reasonable options for use in combination with recorrection osteotomy. A stemmed tibial component is needed to cross the osteotomy site. In our patient’s case, use of a PCL-retaining design was based on surgeon preference and experience.

Patella infera has been noted as a problem in studies on converting high tibial osteotomy to TKA.^9,12,29 A postulated cause is scarring of the infrapatellar tendon after high tibial osteotomy. In addition, a higher incidence of lateral retinacular release has been identified.^9-11 Patella infera did not occur in either knee in the present case, and lateral release was not required.

Our patient’s lateral radiographs (Figures 4A, 4B) showed persistence of the osteotomy plane anterior to the tibia. The osteotomy healed posteriorly but not completely anteriorly. This raises the issue of risk for nonunion when recorrection osteotomy is performed with TKA. Use of a stemmed tibial implant with a derotation plate provides the benefit of intramedullary fixation for the recorrection osteotomy. If the recorrection osteotomy were performed in a separate setting before TKA, plate fixation would be the primary fixation option. Should nonunion occur at the recorrection osteotomy site, revision of the tibial plateau with a new stemmed implant would be required in combination with plate fixation. Madelaine and colleagues³⁰ reported on a series of 15 severe varus knees treated with both osteotomy and TKA. Two nonunions occurred. Fixation was a staple in one case and a cement wedge in the other. Risk for nonunion may be reduced with the combination of stemmed tibial implant and internal fixation with a derotation plate. Protective weight-bearing is recommended for the first 6 postoperative weeks.

Conclusion

Ligament imbalances produced by high tibial osteotomy and exacerbated by conversion to TKA are difficult to address. In this report, we have described successful single-stage high tibial osteotomy recorrection and TKA performed bilaterally in separate settings. With use of a stemmed tibial component and a derotation plate, solid fixation was obtained with an excellent clinical outcome. The malalignment was corrected while ligament balance was maintained for a PCL-retaining TKA design.