Case Reports

Case Report

A 52-year-old black woman presented to our dermatology clinic for evaluation of a generalized pruritic rash of 5 days’ duration. The eruption had started on the trunk and subsequently spread to the face, legs, and arms, including the dorsal surfaces of the hands (Figure 1). The patient reported that she had developed a similar rash 4 years prior. She recalled no sick contacts but had occupational exposure to many people as a food service worker. Two days prior, the referring physician had initiated treatment with oral acyclovir 400 mg every 6 hours. The patient was in otherwise good health and reported no fever, chills, diaphoresis, or fatigue. She did not recall any recent insect bites, and a review of systems was negative.

The patient’s medical history was remarkable for 2 cases of varicella: the first, which occurred at 5 years of age, was diagnosed by a pediatrician and manifested as diffuse papules, vesicles, and crusts with concurrent mild fever. The infection followed a typical clinical course and resolved without complications after 1 week. The second case of varicella was diagnosed clinically at our dermatology clinic approximately 4 years prior to the current presentation and manifested as widespread pruritic lesions that were too numerous to count. Given her history of varicella in childhood, a punch biopsy specimen was taken from a lesion on the left trunk and a dermatopathologist confirmed the diagnosis of a herpesvirus infection. The second infection also resolved without sequelae after 12 days. Her medical history was otherwise unremarkable, revealing no exceptional sinopulmonary or gastrointestinal infections. The patient was not currently taking any medications or supplements and reported no known drug allergies.

Physical examination at the current presentation revealed a well-nourished, afebrile woman with vesicles and papules on the hands, arms, and legs along with vesicular and crusted papules in various stages of healing distributed on the chest, abdomen, and back. Lesions on the legs and feet were present but scant. The eruption was not confined to a single dermatome. No lesions were noted on the palms, soles, or oral mucosa and no epitrochlear, axillary, or supraclavicular lymphadenopathy was noted.

Initial laboratory values were obtained. A complete blood count demonstrated a normal leukocyte number of 5700 cells/μL (reference range, 4500–11,000 cells/μL) and mild anemia with a hemoglobin level of 10.3 g/dL (reference range, 14.0–17.5 g/dL). Monocytes were mildly elevated at 11% (reference range, 1%–9%). Serologic tests showed positive titers for varicella-zoster virus (VZV) IgM at 1.64 (negative, <0.91) and VZV IgG at 1.72 (negative, <0.91), indicating current and past VZV infection, respectively. Antibodies against herpes simplex virus (HSV) types 1 and 2 were negative for IgM and positive for IgG at >5.00 (negative, <0.90), indicating a remote HSV infection. Furthermore, results from a culture of a lesion on the left hand were negative for HSV.

After consultation with the Department of Infectious Diseases, further laboratory studies were performed. The absolute lymphocyte number was within normal range at 1600 cells/μL (reference range, 850–3900 cells/μL). Likewise, CD4⁺ T lymphocytes were normal at 618 cells/μL (reference range, 490–1740 cells/μL) or 39% of total lymphocytes (reference range, 30%–61%). Screening results were negative for human immunodeficiency virus types 1 and 2. Immunoglobulin subtype analysis revealed slightly elevated IgG at 1709 mg/dL (reference range, 723–1685 mg/dL), elevated IgA at 487 mg/dL (reference range, 65–382 mg/dL), and normal IgM at 238 mg/dL (reference range, 63–277 mg/dL).

Consistent with the clinical presentation and serologic studies, recurrent varicella was accepted as the most plausible diagnosis. Over the next 2 weeks, the eruption resolved with postinflammatory hyperpigmentation (Figure 2). The patient returned to work without further incident.

Comment

As denoted by its hyphenated name, VZV infection can cause 2 distinct disease processes.^1,2 Varicella, the generalized initial exanthem known as chickenpox, appears predominantly in childhood. With resolution of this primary infection, the virus lies dormant in sensory ganglia, persisting in neurons. Stress, advanced age, and/or compromised immunity may reactivate latent VZV. This secondary expression is known as herpes zoster (shingles), a unilateral eruption of lesions localized to a single dermatome.

In most cases, morphology of the varicella eruption confirms the diagnosis. Lesions evolve through stages from macules and papules to vesicles and pustules and then to crusts. This evolution typically takes 24 to 48 hours.² The varicella eruption contains an admixture of elements from each stage simultaneously. Crusts usually resolve over an average of 14 days. Serologically, IgM is measurable as early as 1 to 2 days after appearance of the eruption.^3,4 Next to appear are IgG antibodies, which generally remain detectable for life. With more than 90% of the US population being seropositive for VZV,⁵ diagnosis and management of varicella and herpes zoster usually are straightforward; however, there have been unusual variations on this classic sequence of pathogenesis.

In disseminated zoster, the clinical presentation includes more than 20 lesions outside the dermatome primarily affected.⁶ Another permutation of VZV infection is zoster sine herpete, which causes the characteristic dermatomal pain of herpes zoster but without the rash.⁷ Occasionally, 2 cases of chickenpox occur in the same person, usually indicating an underlying immune deficiency. Recurrent varicella in those with intact immunity is purportedly rare. A PubMed search of articles indexed for MEDLINE using the search terms recurrent varicella, chickenpox reinfection, and immunocompetent revealed 41 cases of recurrent varicella in immunocompetent patients in the English language literature occurring among children,^1,8-11 adults,⁸ the elderly,¹² health care workers,^13-15 and pregnant women¹⁶ (Table).

Surveillance studies, however, have challenged the apparent rarity of recurrent varicella, asserting that varicella may recur more frequently than is generally recognized.^17,18 Hall et al¹⁷ described 9947 cases of varicella, with nearly 6.9% reporting prior varicella infection. Another surveillance report by Marin et al¹⁸ evaluating data from 1047 adults with varicella noted that 21% of participants reported prior VZV infections. Both of these studies defined varicella by clinical parameters as a condition with acute onset of generalized maculopapulovesicular rash without other known cause. Although laboratory confirmation of VZV infection was not documented in either study, a history of varicella is considered a reliable indicator of immunity. In fact, studies show that a history of varicella is associated with serologic evidence of immunity 97% to 100% of the time.^19,20

Immunity against VZV in humans is not well understood. Although both humoral and cellular factors play a role, cell-mediated immunity may be more important in suppressing primary infection and defending against reinfection. Varicella is more likely to disseminate in lymphopenic patients,^21,22 while its course is uninfluenced by hypogammaglobulinemia.^1,23 One study of simian varicella virus, which demonstrates 75% genetic homology with VZV, noted that simian varicella virus–infected rhesus macaques without CD4⁺ T lymphocyte response experienced higher viral loads, prolonged viremia, and disseminated varicella.²⁴ The loss of CD20⁺ B lymphocytes did not intensify the severity of varicella in the primate model. It is accepted, however, that waning humoral immunity and lower antibody levels correlate with varicella recurrence.²⁵ Ethnicity may impact immunoglobulin persistence. One investigation postulated that individuals with darker skin types experience reduced viral shedding and therefore less antigenic boosting from secondary VZV infections, as they may less readily maintain protective levels of VZV-specific immunoglobulins.²⁵ This phenomenon may have contributed to the 3 episodes of varicella in our patient.

Virulence factors that are intrinsic to VZV may also prompt reinfection. Although taxonomy is still in flux, 3 to 5 major genotypes of VZV have been recognized to date, categorized into European (Dumas), Japanese (Oka), and mosaic clades.^26-28 In one study population, approximately 80% of the VZV strains isolated in the United States were of the European variety.²⁶ It is unclear whether infection with one strain of VZV affords immunoprotection against the other strains. Interestingly, one report documented recurrent herpes zoster caused by 2 distinct VZV strains in the same individual.²⁹ Since subtypes of VZV vary geographically, it is possible that increasing global travel may correlate with increased incidence and reporting of varicella reinfection, particularly in cosmopolitan centers. In patients with recurrent varicella, a careful investigation of their international travel history may be necessary.

Case Report

A 52-year-old black woman presented to our dermatology clinic for evaluation of a generalized pruritic rash of 5 days’ duration. The eruption had started on the trunk and subsequently spread to the face, legs, and arms, including the dorsal surfaces of the hands (Figure 1). The patient reported that she had developed a similar rash 4 years prior. She recalled no sick contacts but had occupational exposure to many people as a food service worker. Two days prior, the referring physician had initiated treatment with oral acyclovir 400 mg every 6 hours. The patient was in otherwise good health and reported no fever, chills, diaphoresis, or fatigue. She did not recall any recent insect bites, and a review of systems was negative.

The patient’s medical history was remarkable for 2 cases of varicella: the first, which occurred at 5 years of age, was diagnosed by a pediatrician and manifested as diffuse papules, vesicles, and crusts with concurrent mild fever. The infection followed a typical clinical course and resolved without complications after 1 week. The second case of varicella was diagnosed clinically at our dermatology clinic approximately 4 years prior to the current presentation and manifested as widespread pruritic lesions that were too numerous to count. Given her history of varicella in childhood, a punch biopsy specimen was taken from a lesion on the left trunk and a dermatopathologist confirmed the diagnosis of a herpesvirus infection. The second infection also resolved without sequelae after 12 days. Her medical history was otherwise unremarkable, revealing no exceptional sinopulmonary or gastrointestinal infections. The patient was not currently taking any medications or supplements and reported no known drug allergies.

Physical examination at the current presentation revealed a well-nourished, afebrile woman with vesicles and papules on the hands, arms, and legs along with vesicular and crusted papules in various stages of healing distributed on the chest, abdomen, and back. Lesions on the legs and feet were present but scant. The eruption was not confined to a single dermatome. No lesions were noted on the palms, soles, or oral mucosa and no epitrochlear, axillary, or supraclavicular lymphadenopathy was noted.

Initial laboratory values were obtained. A complete blood count demonstrated a normal leukocyte number of 5700 cells/μL (reference range, 4500–11,000 cells/μL) and mild anemia with a hemoglobin level of 10.3 g/dL (reference range, 14.0–17.5 g/dL). Monocytes were mildly elevated at 11% (reference range, 1%–9%). Serologic tests showed positive titers for varicella-zoster virus (VZV) IgM at 1.64 (negative, <0.91) and VZV IgG at 1.72 (negative, <0.91), indicating current and past VZV infection, respectively. Antibodies against herpes simplex virus (HSV) types 1 and 2 were negative for IgM and positive for IgG at >5.00 (negative, <0.90), indicating a remote HSV infection. Furthermore, results from a culture of a lesion on the left hand were negative for HSV.

After consultation with the Department of Infectious Diseases, further laboratory studies were performed. The absolute lymphocyte number was within normal range at 1600 cells/μL (reference range, 850–3900 cells/μL). Likewise, CD4⁺ T lymphocytes were normal at 618 cells/μL (reference range, 490–1740 cells/μL) or 39% of total lymphocytes (reference range, 30%–61%). Screening results were negative for human immunodeficiency virus types 1 and 2. Immunoglobulin subtype analysis revealed slightly elevated IgG at 1709 mg/dL (reference range, 723–1685 mg/dL), elevated IgA at 487 mg/dL (reference range, 65–382 mg/dL), and normal IgM at 238 mg/dL (reference range, 63–277 mg/dL).

Consistent with the clinical presentation and serologic studies, recurrent varicella was accepted as the most plausible diagnosis. Over the next 2 weeks, the eruption resolved with postinflammatory hyperpigmentation (Figure 2). The patient returned to work without further incident.

Comment

As denoted by its hyphenated name, VZV infection can cause 2 distinct disease processes.^1,2 Varicella, the generalized initial exanthem known as chickenpox, appears predominantly in childhood. With resolution of this primary infection, the virus lies dormant in sensory ganglia, persisting in neurons. Stress, advanced age, and/or compromised immunity may reactivate latent VZV. This secondary expression is known as herpes zoster (shingles), a unilateral eruption of lesions localized to a single dermatome.

In most cases, morphology of the varicella eruption confirms the diagnosis. Lesions evolve through stages from macules and papules to vesicles and pustules and then to crusts. This evolution typically takes 24 to 48 hours.² The varicella eruption contains an admixture of elements from each stage simultaneously. Crusts usually resolve over an average of 14 days. Serologically, IgM is measurable as early as 1 to 2 days after appearance of the eruption.^3,4 Next to appear are IgG antibodies, which generally remain detectable for life. With more than 90% of the US population being seropositive for VZV,⁵ diagnosis and management of varicella and herpes zoster usually are straightforward; however, there have been unusual variations on this classic sequence of pathogenesis.

In disseminated zoster, the clinical presentation includes more than 20 lesions outside the dermatome primarily affected.⁶ Another permutation of VZV infection is zoster sine herpete, which causes the characteristic dermatomal pain of herpes zoster but without the rash.⁷ Occasionally, 2 cases of chickenpox occur in the same person, usually indicating an underlying immune deficiency. Recurrent varicella in those with intact immunity is purportedly rare. A PubMed search of articles indexed for MEDLINE using the search terms recurrent varicella, chickenpox reinfection, and immunocompetent revealed 41 cases of recurrent varicella in immunocompetent patients in the English language literature occurring among children,^1,8-11 adults,⁸ the elderly,¹² health care workers,^13-15 and pregnant women¹⁶ (Table).

Surveillance studies, however, have challenged the apparent rarity of recurrent varicella, asserting that varicella may recur more frequently than is generally recognized.^17,18 Hall et al¹⁷ described 9947 cases of varicella, with nearly 6.9% reporting prior varicella infection. Another surveillance report by Marin et al¹⁸ evaluating data from 1047 adults with varicella noted that 21% of participants reported prior VZV infections. Both of these studies defined varicella by clinical parameters as a condition with acute onset of generalized maculopapulovesicular rash without other known cause. Although laboratory confirmation of VZV infection was not documented in either study, a history of varicella is considered a reliable indicator of immunity. In fact, studies show that a history of varicella is associated with serologic evidence of immunity 97% to 100% of the time.^19,20

Immunity against VZV in humans is not well understood. Although both humoral and cellular factors play a role, cell-mediated immunity may be more important in suppressing primary infection and defending against reinfection. Varicella is more likely to disseminate in lymphopenic patients,^21,22 while its course is uninfluenced by hypogammaglobulinemia.^1,23 One study of simian varicella virus, which demonstrates 75% genetic homology with VZV, noted that simian varicella virus–infected rhesus macaques without CD4⁺ T lymphocyte response experienced higher viral loads, prolonged viremia, and disseminated varicella.²⁴ The loss of CD20⁺ B lymphocytes did not intensify the severity of varicella in the primate model. It is accepted, however, that waning humoral immunity and lower antibody levels correlate with varicella recurrence.²⁵ Ethnicity may impact immunoglobulin persistence. One investigation postulated that individuals with darker skin types experience reduced viral shedding and therefore less antigenic boosting from secondary VZV infections, as they may less readily maintain protective levels of VZV-specific immunoglobulins.²⁵ This phenomenon may have contributed to the 3 episodes of varicella in our patient.

Virulence factors that are intrinsic to VZV may also prompt reinfection. Although taxonomy is still in flux, 3 to 5 major genotypes of VZV have been recognized to date, categorized into European (Dumas), Japanese (Oka), and mosaic clades.^26-28 In one study population, approximately 80% of the VZV strains isolated in the United States were of the European variety.²⁶ It is unclear whether infection with one strain of VZV affords immunoprotection against the other strains. Interestingly, one report documented recurrent herpes zoster caused by 2 distinct VZV strains in the same individual.²⁹ Since subtypes of VZV vary geographically, it is possible that increasing global travel may correlate with increased incidence and reporting of varicella reinfection, particularly in cosmopolitan centers. In patients with recurrent varicella, a careful investigation of their international travel history may be necessary.

Case Report

A 52-year-old black woman presented to our dermatology clinic for evaluation of a generalized pruritic rash of 5 days’ duration. The eruption had started on the trunk and subsequently spread to the face, legs, and arms, including the dorsal surfaces of the hands (Figure 1). The patient reported that she had developed a similar rash 4 years prior. She recalled no sick contacts but had occupational exposure to many people as a food service worker. Two days prior, the referring physician had initiated treatment with oral acyclovir 400 mg every 6 hours. The patient was in otherwise good health and reported no fever, chills, diaphoresis, or fatigue. She did not recall any recent insect bites, and a review of systems was negative.

The patient’s medical history was remarkable for 2 cases of varicella: the first, which occurred at 5 years of age, was diagnosed by a pediatrician and manifested as diffuse papules, vesicles, and crusts with concurrent mild fever. The infection followed a typical clinical course and resolved without complications after 1 week. The second case of varicella was diagnosed clinically at our dermatology clinic approximately 4 years prior to the current presentation and manifested as widespread pruritic lesions that were too numerous to count. Given her history of varicella in childhood, a punch biopsy specimen was taken from a lesion on the left trunk and a dermatopathologist confirmed the diagnosis of a herpesvirus infection. The second infection also resolved without sequelae after 12 days. Her medical history was otherwise unremarkable, revealing no exceptional sinopulmonary or gastrointestinal infections. The patient was not currently taking any medications or supplements and reported no known drug allergies.

Physical examination at the current presentation revealed a well-nourished, afebrile woman with vesicles and papules on the hands, arms, and legs along with vesicular and crusted papules in various stages of healing distributed on the chest, abdomen, and back. Lesions on the legs and feet were present but scant. The eruption was not confined to a single dermatome. No lesions were noted on the palms, soles, or oral mucosa and no epitrochlear, axillary, or supraclavicular lymphadenopathy was noted.

Initial laboratory values were obtained. A complete blood count demonstrated a normal leukocyte number of 5700 cells/μL (reference range, 4500–11,000 cells/μL) and mild anemia with a hemoglobin level of 10.3 g/dL (reference range, 14.0–17.5 g/dL). Monocytes were mildly elevated at 11% (reference range, 1%–9%). Serologic tests showed positive titers for varicella-zoster virus (VZV) IgM at 1.64 (negative, <0.91) and VZV IgG at 1.72 (negative, <0.91), indicating current and past VZV infection, respectively. Antibodies against herpes simplex virus (HSV) types 1 and 2 were negative for IgM and positive for IgG at >5.00 (negative, <0.90), indicating a remote HSV infection. Furthermore, results from a culture of a lesion on the left hand were negative for HSV.

After consultation with the Department of Infectious Diseases, further laboratory studies were performed. The absolute lymphocyte number was within normal range at 1600 cells/μL (reference range, 850–3900 cells/μL). Likewise, CD4⁺ T lymphocytes were normal at 618 cells/μL (reference range, 490–1740 cells/μL) or 39% of total lymphocytes (reference range, 30%–61%). Screening results were negative for human immunodeficiency virus types 1 and 2. Immunoglobulin subtype analysis revealed slightly elevated IgG at 1709 mg/dL (reference range, 723–1685 mg/dL), elevated IgA at 487 mg/dL (reference range, 65–382 mg/dL), and normal IgM at 238 mg/dL (reference range, 63–277 mg/dL).

Consistent with the clinical presentation and serologic studies, recurrent varicella was accepted as the most plausible diagnosis. Over the next 2 weeks, the eruption resolved with postinflammatory hyperpigmentation (Figure 2). The patient returned to work without further incident.

Comment

As denoted by its hyphenated name, VZV infection can cause 2 distinct disease processes.^1,2 Varicella, the generalized initial exanthem known as chickenpox, appears predominantly in childhood. With resolution of this primary infection, the virus lies dormant in sensory ganglia, persisting in neurons. Stress, advanced age, and/or compromised immunity may reactivate latent VZV. This secondary expression is known as herpes zoster (shingles), a unilateral eruption of lesions localized to a single dermatome.

In most cases, morphology of the varicella eruption confirms the diagnosis. Lesions evolve through stages from macules and papules to vesicles and pustules and then to crusts. This evolution typically takes 24 to 48 hours.² The varicella eruption contains an admixture of elements from each stage simultaneously. Crusts usually resolve over an average of 14 days. Serologically, IgM is measurable as early as 1 to 2 days after appearance of the eruption.^3,4 Next to appear are IgG antibodies, which generally remain detectable for life. With more than 90% of the US population being seropositive for VZV,⁵ diagnosis and management of varicella and herpes zoster usually are straightforward; however, there have been unusual variations on this classic sequence of pathogenesis.

In disseminated zoster, the clinical presentation includes more than 20 lesions outside the dermatome primarily affected.⁶ Another permutation of VZV infection is zoster sine herpete, which causes the characteristic dermatomal pain of herpes zoster but without the rash.⁷ Occasionally, 2 cases of chickenpox occur in the same person, usually indicating an underlying immune deficiency. Recurrent varicella in those with intact immunity is purportedly rare. A PubMed search of articles indexed for MEDLINE using the search terms recurrent varicella, chickenpox reinfection, and immunocompetent revealed 41 cases of recurrent varicella in immunocompetent patients in the English language literature occurring among children,^1,8-11 adults,⁸ the elderly,¹² health care workers,^13-15 and pregnant women¹⁶ (Table).

Surveillance studies, however, have challenged the apparent rarity of recurrent varicella, asserting that varicella may recur more frequently than is generally recognized.^17,18 Hall et al¹⁷ described 9947 cases of varicella, with nearly 6.9% reporting prior varicella infection. Another surveillance report by Marin et al¹⁸ evaluating data from 1047 adults with varicella noted that 21% of participants reported prior VZV infections. Both of these studies defined varicella by clinical parameters as a condition with acute onset of generalized maculopapulovesicular rash without other known cause. Although laboratory confirmation of VZV infection was not documented in either study, a history of varicella is considered a reliable indicator of immunity. In fact, studies show that a history of varicella is associated with serologic evidence of immunity 97% to 100% of the time.^19,20

Immunity against VZV in humans is not well understood. Although both humoral and cellular factors play a role, cell-mediated immunity may be more important in suppressing primary infection and defending against reinfection. Varicella is more likely to disseminate in lymphopenic patients,^21,22 while its course is uninfluenced by hypogammaglobulinemia.^1,23 One study of simian varicella virus, which demonstrates 75% genetic homology with VZV, noted that simian varicella virus–infected rhesus macaques without CD4⁺ T lymphocyte response experienced higher viral loads, prolonged viremia, and disseminated varicella.²⁴ The loss of CD20⁺ B lymphocytes did not intensify the severity of varicella in the primate model. It is accepted, however, that waning humoral immunity and lower antibody levels correlate with varicella recurrence.²⁵ Ethnicity may impact immunoglobulin persistence. One investigation postulated that individuals with darker skin types experience reduced viral shedding and therefore less antigenic boosting from secondary VZV infections, as they may less readily maintain protective levels of VZV-specific immunoglobulins.²⁵ This phenomenon may have contributed to the 3 episodes of varicella in our patient.

Virulence factors that are intrinsic to VZV may also prompt reinfection. Although taxonomy is still in flux, 3 to 5 major genotypes of VZV have been recognized to date, categorized into European (Dumas), Japanese (Oka), and mosaic clades.^26-28 In one study population, approximately 80% of the VZV strains isolated in the United States were of the European variety.²⁶ It is unclear whether infection with one strain of VZV affords immunoprotection against the other strains. Interestingly, one report documented recurrent herpes zoster caused by 2 distinct VZV strains in the same individual.²⁹ Since subtypes of VZV vary geographically, it is possible that increasing global travel may correlate with increased incidence and reporting of varicella reinfection, particularly in cosmopolitan centers. In patients with recurrent varicella, a careful investigation of their international travel history may be necessary.

PMP22 is a tetra-span membrane protein primarily expressed in myelinating Schwann cells. Heterozygous deletion of the PMP22 gene (1 copy) causes HNPP (hereditary neuropathy with liability to pressure palsies).¹ Interestingly, a reciprocal genetic disorder with 3 copies of human PMP22 causes the most common inherited neuropathy, Charcot-Marie-Tooth disease type 1A (CMT1A).^2,3 As the reciprocal mutations occur at initiation of gestation, it is expected that HNPP and CMT1A have a similar prevalence. However, studies have shown HNPP prevalence of 2 to 5 cases per 100,000, far below the CMT1A prevalence of 1:5000.⁴ This finding prompted speculation that many patients with HNPP may be undiagnosed because of the subtlety of the phenotypes.⁵

Patients with HNPP typically present with focal sensory loss and muscle weakness related to mechanical stress–induced failure of action potential propagation.^6,7 In this article, we report the case of an asymptomatic woman with the HNPP mutation. Her focal neurologic deficits occurred only after total knee arthroplasty (TKA), which in healthy patients is not expected to induce focal sensory and motor symptoms. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

The patient, a healthy 57-year-old woman, had a normal developmental history. For decades, she had practiced ballet without any physical difficulties. She underwent left TKA and woke up with a footdrop on the left side. The left foot was less sensitive to temperature. Ankle strength returned 2 months later. There was no family history of HNPP.

The patient was examined by a local neurologist, who found steppage gait, weak ankle dorsiflexion (4 on Medical Research Council scale), and diminished touch on the lateral aspect of the left leg. Deep tendon reflexes were present in the arms but not the legs.

A nerve conduction study (NCS) performed after the footdrop revealed prolonged distal latency and decreased amplitude in the left peroneal and tibial nerves. The left sural nerve was normal. Needle electromyogram revealed denervation changes in the muscles innervated by the left peroneal nerve (Table). In addition, we also performed an NCS on the arm (Table), which was unaffected by the surgical procedure. This NCS revealed severely prolonged distal latency across the left wrist in the median nerve and focal slowing of conduction velocity of the ulnar nerve across the left elbow. These changes provide evidence of asymptomatic carpal tunnel syndrome and ulnar nerve entrapment, typical electrophysiologic abnormalities of HNPP.8As there was no explanation for the footdrop from the surgery, we had a DNA test performed (Athena Diagnostics). This test identified a heterozygous deletion of chromosome 17p12 containing the PMP22 gene, the HNPP mutation.

Discussion

This case had several important features. First, though the patient developed an electrophysiologic phenotype of HNPP, she was completely asymptomatic clinically and very athletic before her medical procedure. She would not have been diagnosed with HNPP if her clinical deficits had not been induced by TKA. Therefore, the prevalence of HNPP is likely underestimated. Second, for patients with the HNPP mutation, there may be serious neurologic consequences of certain medical procedures. The diagnosis of HNPP should be pursued if there is no explanation from the medical procedure per se. In addition, patients with a family history of HNPP should be carefully evaluated before any procedure that may put them at risk for severe peripheral nerve damage, and they should be counseled regarding the risks. It is important to determine the prevalence of HNPP among patients who develop footdrop after knee arthroplasty, as this information could potentially be used to revise ideas about the etiology of peripheral nerve complications of knee arthroplasty. We now describe possible revisions of these ideas.

Footdrop is a rare complication of TKA. Retrospective studies have found its incidence ranging from 0.3% to 1.3%.^9-11 The investigators in those studies postulated 3 main causes for peroneal nerve palsy. First, traction may put pressure on the peroneal nerve during normalization of the mechanical axis of a valgus knee. Our patient did not have a valgus knee. Second, epidural hematoma by anesthetic procedure may compress the spinal roots. Our patient received general anesthesia during the procedure; epidural or spinal anesthesia was not used. Third, postoperative dressing may compress the nerve. Our patient did not develop any signs of constrictive dressing, such as inordinate pain, which can be relieved by removing the dressing, and swelling of the leg distal to the dressing. Therefore, her footdrop likely was not a complication of surgery.

This case demonstrates how a patient with undiagnosed HNPP can manifest the HNPP phenotype only after undergoing a particular surgical procedure. HNPP is unfamiliar to most orthopedic surgeons.

PMP22 is a tetra-span membrane protein primarily expressed in myelinating Schwann cells. Heterozygous deletion of the PMP22 gene (1 copy) causes HNPP (hereditary neuropathy with liability to pressure palsies).¹ Interestingly, a reciprocal genetic disorder with 3 copies of human PMP22 causes the most common inherited neuropathy, Charcot-Marie-Tooth disease type 1A (CMT1A).^2,3 As the reciprocal mutations occur at initiation of gestation, it is expected that HNPP and CMT1A have a similar prevalence. However, studies have shown HNPP prevalence of 2 to 5 cases per 100,000, far below the CMT1A prevalence of 1:5000.⁴ This finding prompted speculation that many patients with HNPP may be undiagnosed because of the subtlety of the phenotypes.⁵

Patients with HNPP typically present with focal sensory loss and muscle weakness related to mechanical stress–induced failure of action potential propagation.^6,7 In this article, we report the case of an asymptomatic woman with the HNPP mutation. Her focal neurologic deficits occurred only after total knee arthroplasty (TKA), which in healthy patients is not expected to induce focal sensory and motor symptoms. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

The patient, a healthy 57-year-old woman, had a normal developmental history. For decades, she had practiced ballet without any physical difficulties. She underwent left TKA and woke up with a footdrop on the left side. The left foot was less sensitive to temperature. Ankle strength returned 2 months later. There was no family history of HNPP.

The patient was examined by a local neurologist, who found steppage gait, weak ankle dorsiflexion (4 on Medical Research Council scale), and diminished touch on the lateral aspect of the left leg. Deep tendon reflexes were present in the arms but not the legs.

A nerve conduction study (NCS) performed after the footdrop revealed prolonged distal latency and decreased amplitude in the left peroneal and tibial nerves. The left sural nerve was normal. Needle electromyogram revealed denervation changes in the muscles innervated by the left peroneal nerve (Table). In addition, we also performed an NCS on the arm (Table), which was unaffected by the surgical procedure. This NCS revealed severely prolonged distal latency across the left wrist in the median nerve and focal slowing of conduction velocity of the ulnar nerve across the left elbow. These changes provide evidence of asymptomatic carpal tunnel syndrome and ulnar nerve entrapment, typical electrophysiologic abnormalities of HNPP.8As there was no explanation for the footdrop from the surgery, we had a DNA test performed (Athena Diagnostics). This test identified a heterozygous deletion of chromosome 17p12 containing the PMP22 gene, the HNPP mutation.

Discussion

This case had several important features. First, though the patient developed an electrophysiologic phenotype of HNPP, she was completely asymptomatic clinically and very athletic before her medical procedure. She would not have been diagnosed with HNPP if her clinical deficits had not been induced by TKA. Therefore, the prevalence of HNPP is likely underestimated. Second, for patients with the HNPP mutation, there may be serious neurologic consequences of certain medical procedures. The diagnosis of HNPP should be pursued if there is no explanation from the medical procedure per se. In addition, patients with a family history of HNPP should be carefully evaluated before any procedure that may put them at risk for severe peripheral nerve damage, and they should be counseled regarding the risks. It is important to determine the prevalence of HNPP among patients who develop footdrop after knee arthroplasty, as this information could potentially be used to revise ideas about the etiology of peripheral nerve complications of knee arthroplasty. We now describe possible revisions of these ideas.

Footdrop is a rare complication of TKA. Retrospective studies have found its incidence ranging from 0.3% to 1.3%.^9-11 The investigators in those studies postulated 3 main causes for peroneal nerve palsy. First, traction may put pressure on the peroneal nerve during normalization of the mechanical axis of a valgus knee. Our patient did not have a valgus knee. Second, epidural hematoma by anesthetic procedure may compress the spinal roots. Our patient received general anesthesia during the procedure; epidural or spinal anesthesia was not used. Third, postoperative dressing may compress the nerve. Our patient did not develop any signs of constrictive dressing, such as inordinate pain, which can be relieved by removing the dressing, and swelling of the leg distal to the dressing. Therefore, her footdrop likely was not a complication of surgery.

This case demonstrates how a patient with undiagnosed HNPP can manifest the HNPP phenotype only after undergoing a particular surgical procedure. HNPP is unfamiliar to most orthopedic surgeons.

PMP22 is a tetra-span membrane protein primarily expressed in myelinating Schwann cells. Heterozygous deletion of the PMP22 gene (1 copy) causes HNPP (hereditary neuropathy with liability to pressure palsies).¹ Interestingly, a reciprocal genetic disorder with 3 copies of human PMP22 causes the most common inherited neuropathy, Charcot-Marie-Tooth disease type 1A (CMT1A).^2,3 As the reciprocal mutations occur at initiation of gestation, it is expected that HNPP and CMT1A have a similar prevalence. However, studies have shown HNPP prevalence of 2 to 5 cases per 100,000, far below the CMT1A prevalence of 1:5000.⁴ This finding prompted speculation that many patients with HNPP may be undiagnosed because of the subtlety of the phenotypes.⁵

Patients with HNPP typically present with focal sensory loss and muscle weakness related to mechanical stress–induced failure of action potential propagation.^6,7 In this article, we report the case of an asymptomatic woman with the HNPP mutation. Her focal neurologic deficits occurred only after total knee arthroplasty (TKA), which in healthy patients is not expected to induce focal sensory and motor symptoms. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

The patient, a healthy 57-year-old woman, had a normal developmental history. For decades, she had practiced ballet without any physical difficulties. She underwent left TKA and woke up with a footdrop on the left side. The left foot was less sensitive to temperature. Ankle strength returned 2 months later. There was no family history of HNPP.

The patient was examined by a local neurologist, who found steppage gait, weak ankle dorsiflexion (4 on Medical Research Council scale), and diminished touch on the lateral aspect of the left leg. Deep tendon reflexes were present in the arms but not the legs.

A nerve conduction study (NCS) performed after the footdrop revealed prolonged distal latency and decreased amplitude in the left peroneal and tibial nerves. The left sural nerve was normal. Needle electromyogram revealed denervation changes in the muscles innervated by the left peroneal nerve (Table). In addition, we also performed an NCS on the arm (Table), which was unaffected by the surgical procedure. This NCS revealed severely prolonged distal latency across the left wrist in the median nerve and focal slowing of conduction velocity of the ulnar nerve across the left elbow. These changes provide evidence of asymptomatic carpal tunnel syndrome and ulnar nerve entrapment, typical electrophysiologic abnormalities of HNPP.8As there was no explanation for the footdrop from the surgery, we had a DNA test performed (Athena Diagnostics). This test identified a heterozygous deletion of chromosome 17p12 containing the PMP22 gene, the HNPP mutation.

Discussion

This case had several important features. First, though the patient developed an electrophysiologic phenotype of HNPP, she was completely asymptomatic clinically and very athletic before her medical procedure. She would not have been diagnosed with HNPP if her clinical deficits had not been induced by TKA. Therefore, the prevalence of HNPP is likely underestimated. Second, for patients with the HNPP mutation, there may be serious neurologic consequences of certain medical procedures. The diagnosis of HNPP should be pursued if there is no explanation from the medical procedure per se. In addition, patients with a family history of HNPP should be carefully evaluated before any procedure that may put them at risk for severe peripheral nerve damage, and they should be counseled regarding the risks. It is important to determine the prevalence of HNPP among patients who develop footdrop after knee arthroplasty, as this information could potentially be used to revise ideas about the etiology of peripheral nerve complications of knee arthroplasty. We now describe possible revisions of these ideas.

Footdrop is a rare complication of TKA. Retrospective studies have found its incidence ranging from 0.3% to 1.3%.^9-11 The investigators in those studies postulated 3 main causes for peroneal nerve palsy. First, traction may put pressure on the peroneal nerve during normalization of the mechanical axis of a valgus knee. Our patient did not have a valgus knee. Second, epidural hematoma by anesthetic procedure may compress the spinal roots. Our patient received general anesthesia during the procedure; epidural or spinal anesthesia was not used. Third, postoperative dressing may compress the nerve. Our patient did not develop any signs of constrictive dressing, such as inordinate pain, which can be relieved by removing the dressing, and swelling of the leg distal to the dressing. Therefore, her footdrop likely was not a complication of surgery.

This case demonstrates how a patient with undiagnosed HNPP can manifest the HNPP phenotype only after undergoing a particular surgical procedure. HNPP is unfamiliar to most orthopedic surgeons.

Tibial tubercle fractures are rare injuries accounting for less than 1% of all pediatric physeal injuries.¹ The original classification scheme for such fractures was proposed by Watson-Jones.² Initially modified by Ogden and colleagues,³ the classification system has had numerous additions and modifications as new patterns of injury have been identified.^4-6 Patellar fractures are also rare in children, making up 1% of all pediatric fractures, with less than 2% of these occurring in skeletally immature children.⁷

We present a case of an unreported combined tibial tubercle avulsion fracture and patellar avulsion fracture in an adolescent boy. The patient and his guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-year-old boy presented to the emergency department with acute onset of right-knee pain and inability to ambulate after falling off a skateboard on the day of the injury. The patient was otherwise healthy and had no noteworthy medical or surgical history, including no prior fractures. On physical examination, he was noted to have a large right-knee effusion presumed to be hemarthrosis, and inability to perform a straight-leg raise against gravity. There were no neurologic deficits and his leg compartments were soft. Plain radiographs showed patella alta and numerous bony fragments believed to represent a complex tibial tubercle fracture. One bony fragment was identified closer to the patella, suggesting a possible concurrent patellar fracture (Figures 1A, 1B). A computed tomography (CT) scan further characterized both the tibial tubercle avulsion fracture and the lateral inferior pole patellar avulsion fracture (Figures 2A, 2B). The patient’s knee was immobilized, and he was admitted for soft-tissue rest and overnight observation to ensure that compartment syndrome did not develop.

Five days after injury, open reduction and internal fixation were performed. After limb exsanguination and tourniquet insufflation, the fracture was visualized through a direct midline approach. The patient was found to have a Z-type injury pattern to the extensor mechanism: an inferior lateral patellar avulsion fracture, longitudinal splits of the patellar tendon, and 2 large, mainly cartilaginous tibial tubercle fracture fragments, 1 of which extended into the proximal tibial epiphysis (Ogden type III) (Figures 3A-3C). Under direct visualization, the tibial tubercle fragments were reduced and stabilized with 3 cannulated 3.5-mm titanium, partially threaded screws with washers. Smaller screws were used to prevent fragmentation of these mostly cartilaginous fragments. Anatomic reduction was ensured along the articular surface, visualized through an arthrotomy, as well as on the distal cortex (Figures 4A, 4B). The patellar avulsion fracture included a very small section of articular surface and the decision was made to preserve the fragment. Because the patellar fragment was too small for screw fixation, the fracture was secured with suture fixation through bone tunnels over a patellar bony bridge using size 2 Phantom Fiber suture (Tornier) (Figure 5). Vicryl was used to repair the longitudinal patellar tendon split as well as the capsular and paratenon traumatic tears. Layered closure was completed and intraoperative radiographs were obtained (Figures 6A, 6B) prior to placement of a cylinder cast in full extension. Postoperatively, the patient remained overnight for observation and physical therapy evaluation. He was encouraged to bear weight in his cylinder cast as tolerated with crutches to assist with ambulation.

Postoperatively, the patient was maintained in full extension in the cylinder cast for 4 weeks. After cast removal, the patient was placed in a range-of-motion brace locked in extension for ambulation. He started physical therapy and was allowed to perform prone active-knee flexion limited to 90º, with passive extension, for an additional 4 weeks. At 8 weeks, the patient was allowed full-knee motion both active and passive, and the brace was discontinued. At his 18-week follow-up appointment, the patient reported successful return to all his normal activities, including skateboarding, with no apparent limitation in motion or weight-bearing. Examination at that time demonstrated knee range of motion from 5º in hyperextension to 135º in flexion, with his left knee having 5º in hyperextension and 145º in flexion. The patient appeared to have no gait abnormalities, and radiographs showed healed fractures. Because of a concern that continued compression across his tibial physis could lead to greater risk of growth arrest, the decision was made to remove the implants when radiographs showed healing. The patient returned to surgery at 20 weeks for implant removal. At 6 weeks after implant removal, the patient had returned to full activity with no residual pain and full-knee flexion equal to the uninvolved left knee. He was able to perform a stable single-leg squat on his affected leg, and his single-leg hop for distance was the same as his uninvolved leg. He was allowed to return to full sports activity. The patient will be followed with serial radiographs at 4 months, 8 months, and 12 months to look for premature physeal arrest. If an arrest occurs, treatment will be dictated by the extent of the arrest and the potential to cause either limb-length difference or angular deformity.

Discussion

Tibial tubercle fractures typically result from quadriceps contraction during sporting activities, predominantly in adolescent boys with open physes. Numerous modifications and additions have been made to the original classification of such fractures by Watson-Jones,² most notably by Ogden and colleagues³ in 1980. These additions have included combined tendon avulsions and tubercle fractures as described by Frankl and coauthors,⁴ complete proximal tibial physeal separation now classified as type 4 by Ryu and Debenham,⁵ and a “Y” fracture configuration now termed type 5 by McKoy and Stanitski.⁶ Pandya and colleagues⁸ reported on 41 tibial tubercle fractures and described a new classification scheme based on the known anatomical closure pattern of the proximal tibial physis and tibial tubercle apophysis. The authors stressed the role of advanced imaging, such as CT or magnetic resonance imaging, in preoperative management of these complex high-energy fractures in adolescents, and the need for intraoperative arthroscopy or arthrotomy to ensure anatomical reduction of the articular involvement.

Tibial tubercle fractures and extensor mechanism injuries that do not fit these classification patterns have also been described. In 1979, Houghton and Ackroyd⁹ reported 3 cases of acute loss of extensor mechanism secondary to a traumatic patellar sleeve avulsion. In 1995, Berg¹⁰ described an ipsilateral inferior pole osteochondral patellar avulsion fracture with patellar tendon avulsion without fracture at the tubercle in a 12-year-old boy. Another variant was described in a 2002 case series of 3 adolescent boys who underwent operative fixation for tibial metaphyseal partial-sleeve avulsion injuries.¹¹

Conclusion

We report a case of combined ipsilateral inferior lateral patellar avulsion fracture and an intra-articular tibial tubercle avulsion fracture with intervening longitudinal patellar tendon split. Preoperative standard radiographs were confusing, given the bony fragment high up by the patella, but use of advanced imaging, in this case CT, allowed us to fully characterize the origin of fracture fragments and realize we were dealing with a unique fracture pattern previously unreported in a pediatric patient. The CT findings allowed us to be better prepared preoperatively by having options for fixation of the patellar fracture, and the extent of articular involvement led us to decide that intra-articular evaluation would be required. Through the use of an open arthrotomy, anatomical articular reduction could be visualized and stabilized with screw fixation of the large, mostly cartilaginous tubercle fracture. Following the principles described by Pandya and colleagues,8 anatomical reduction was achieved, and, 6 months after the original surgery, the patient had return of full motion, clinical and radiographic union, and no clinical pain or limp, with no retained metallic implants across the tibial apophysis. Longer-term follow-up as planned will demonstrate any growth abnormality that would require further surgical intervention.

Tibial tubercle fractures are rare injuries accounting for less than 1% of all pediatric physeal injuries.¹ The original classification scheme for such fractures was proposed by Watson-Jones.² Initially modified by Ogden and colleagues,³ the classification system has had numerous additions and modifications as new patterns of injury have been identified.^4-6 Patellar fractures are also rare in children, making up 1% of all pediatric fractures, with less than 2% of these occurring in skeletally immature children.⁷

We present a case of an unreported combined tibial tubercle avulsion fracture and patellar avulsion fracture in an adolescent boy. The patient and his guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-year-old boy presented to the emergency department with acute onset of right-knee pain and inability to ambulate after falling off a skateboard on the day of the injury. The patient was otherwise healthy and had no noteworthy medical or surgical history, including no prior fractures. On physical examination, he was noted to have a large right-knee effusion presumed to be hemarthrosis, and inability to perform a straight-leg raise against gravity. There were no neurologic deficits and his leg compartments were soft. Plain radiographs showed patella alta and numerous bony fragments believed to represent a complex tibial tubercle fracture. One bony fragment was identified closer to the patella, suggesting a possible concurrent patellar fracture (Figures 1A, 1B). A computed tomography (CT) scan further characterized both the tibial tubercle avulsion fracture and the lateral inferior pole patellar avulsion fracture (Figures 2A, 2B). The patient’s knee was immobilized, and he was admitted for soft-tissue rest and overnight observation to ensure that compartment syndrome did not develop.

Five days after injury, open reduction and internal fixation were performed. After limb exsanguination and tourniquet insufflation, the fracture was visualized through a direct midline approach. The patient was found to have a Z-type injury pattern to the extensor mechanism: an inferior lateral patellar avulsion fracture, longitudinal splits of the patellar tendon, and 2 large, mainly cartilaginous tibial tubercle fracture fragments, 1 of which extended into the proximal tibial epiphysis (Ogden type III) (Figures 3A-3C). Under direct visualization, the tibial tubercle fragments were reduced and stabilized with 3 cannulated 3.5-mm titanium, partially threaded screws with washers. Smaller screws were used to prevent fragmentation of these mostly cartilaginous fragments. Anatomic reduction was ensured along the articular surface, visualized through an arthrotomy, as well as on the distal cortex (Figures 4A, 4B). The patellar avulsion fracture included a very small section of articular surface and the decision was made to preserve the fragment. Because the patellar fragment was too small for screw fixation, the fracture was secured with suture fixation through bone tunnels over a patellar bony bridge using size 2 Phantom Fiber suture (Tornier) (Figure 5). Vicryl was used to repair the longitudinal patellar tendon split as well as the capsular and paratenon traumatic tears. Layered closure was completed and intraoperative radiographs were obtained (Figures 6A, 6B) prior to placement of a cylinder cast in full extension. Postoperatively, the patient remained overnight for observation and physical therapy evaluation. He was encouraged to bear weight in his cylinder cast as tolerated with crutches to assist with ambulation.

Postoperatively, the patient was maintained in full extension in the cylinder cast for 4 weeks. After cast removal, the patient was placed in a range-of-motion brace locked in extension for ambulation. He started physical therapy and was allowed to perform prone active-knee flexion limited to 90º, with passive extension, for an additional 4 weeks. At 8 weeks, the patient was allowed full-knee motion both active and passive, and the brace was discontinued. At his 18-week follow-up appointment, the patient reported successful return to all his normal activities, including skateboarding, with no apparent limitation in motion or weight-bearing. Examination at that time demonstrated knee range of motion from 5º in hyperextension to 135º in flexion, with his left knee having 5º in hyperextension and 145º in flexion. The patient appeared to have no gait abnormalities, and radiographs showed healed fractures. Because of a concern that continued compression across his tibial physis could lead to greater risk of growth arrest, the decision was made to remove the implants when radiographs showed healing. The patient returned to surgery at 20 weeks for implant removal. At 6 weeks after implant removal, the patient had returned to full activity with no residual pain and full-knee flexion equal to the uninvolved left knee. He was able to perform a stable single-leg squat on his affected leg, and his single-leg hop for distance was the same as his uninvolved leg. He was allowed to return to full sports activity. The patient will be followed with serial radiographs at 4 months, 8 months, and 12 months to look for premature physeal arrest. If an arrest occurs, treatment will be dictated by the extent of the arrest and the potential to cause either limb-length difference or angular deformity.

Discussion

Tibial tubercle fractures typically result from quadriceps contraction during sporting activities, predominantly in adolescent boys with open physes. Numerous modifications and additions have been made to the original classification of such fractures by Watson-Jones,² most notably by Ogden and colleagues³ in 1980. These additions have included combined tendon avulsions and tubercle fractures as described by Frankl and coauthors,⁴ complete proximal tibial physeal separation now classified as type 4 by Ryu and Debenham,⁵ and a “Y” fracture configuration now termed type 5 by McKoy and Stanitski.⁶ Pandya and colleagues⁸ reported on 41 tibial tubercle fractures and described a new classification scheme based on the known anatomical closure pattern of the proximal tibial physis and tibial tubercle apophysis. The authors stressed the role of advanced imaging, such as CT or magnetic resonance imaging, in preoperative management of these complex high-energy fractures in adolescents, and the need for intraoperative arthroscopy or arthrotomy to ensure anatomical reduction of the articular involvement.

Tibial tubercle fractures and extensor mechanism injuries that do not fit these classification patterns have also been described. In 1979, Houghton and Ackroyd⁹ reported 3 cases of acute loss of extensor mechanism secondary to a traumatic patellar sleeve avulsion. In 1995, Berg¹⁰ described an ipsilateral inferior pole osteochondral patellar avulsion fracture with patellar tendon avulsion without fracture at the tubercle in a 12-year-old boy. Another variant was described in a 2002 case series of 3 adolescent boys who underwent operative fixation for tibial metaphyseal partial-sleeve avulsion injuries.¹¹

Conclusion

We report a case of combined ipsilateral inferior lateral patellar avulsion fracture and an intra-articular tibial tubercle avulsion fracture with intervening longitudinal patellar tendon split. Preoperative standard radiographs were confusing, given the bony fragment high up by the patella, but use of advanced imaging, in this case CT, allowed us to fully characterize the origin of fracture fragments and realize we were dealing with a unique fracture pattern previously unreported in a pediatric patient. The CT findings allowed us to be better prepared preoperatively by having options for fixation of the patellar fracture, and the extent of articular involvement led us to decide that intra-articular evaluation would be required. Through the use of an open arthrotomy, anatomical articular reduction could be visualized and stabilized with screw fixation of the large, mostly cartilaginous tubercle fracture. Following the principles described by Pandya and colleagues,8 anatomical reduction was achieved, and, 6 months after the original surgery, the patient had return of full motion, clinical and radiographic union, and no clinical pain or limp, with no retained metallic implants across the tibial apophysis. Longer-term follow-up as planned will demonstrate any growth abnormality that would require further surgical intervention.

Tibial tubercle fractures are rare injuries accounting for less than 1% of all pediatric physeal injuries.¹ The original classification scheme for such fractures was proposed by Watson-Jones.² Initially modified by Ogden and colleagues,³ the classification system has had numerous additions and modifications as new patterns of injury have been identified.^4-6 Patellar fractures are also rare in children, making up 1% of all pediatric fractures, with less than 2% of these occurring in skeletally immature children.⁷

We present a case of an unreported combined tibial tubercle avulsion fracture and patellar avulsion fracture in an adolescent boy. The patient and his guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A 12-year-old boy presented to the emergency department with acute onset of right-knee pain and inability to ambulate after falling off a skateboard on the day of the injury. The patient was otherwise healthy and had no noteworthy medical or surgical history, including no prior fractures. On physical examination, he was noted to have a large right-knee effusion presumed to be hemarthrosis, and inability to perform a straight-leg raise against gravity. There were no neurologic deficits and his leg compartments were soft. Plain radiographs showed patella alta and numerous bony fragments believed to represent a complex tibial tubercle fracture. One bony fragment was identified closer to the patella, suggesting a possible concurrent patellar fracture (Figures 1A, 1B). A computed tomography (CT) scan further characterized both the tibial tubercle avulsion fracture and the lateral inferior pole patellar avulsion fracture (Figures 2A, 2B). The patient’s knee was immobilized, and he was admitted for soft-tissue rest and overnight observation to ensure that compartment syndrome did not develop.

Five days after injury, open reduction and internal fixation were performed. After limb exsanguination and tourniquet insufflation, the fracture was visualized through a direct midline approach. The patient was found to have a Z-type injury pattern to the extensor mechanism: an inferior lateral patellar avulsion fracture, longitudinal splits of the patellar tendon, and 2 large, mainly cartilaginous tibial tubercle fracture fragments, 1 of which extended into the proximal tibial epiphysis (Ogden type III) (Figures 3A-3C). Under direct visualization, the tibial tubercle fragments were reduced and stabilized with 3 cannulated 3.5-mm titanium, partially threaded screws with washers. Smaller screws were used to prevent fragmentation of these mostly cartilaginous fragments. Anatomic reduction was ensured along the articular surface, visualized through an arthrotomy, as well as on the distal cortex (Figures 4A, 4B). The patellar avulsion fracture included a very small section of articular surface and the decision was made to preserve the fragment. Because the patellar fragment was too small for screw fixation, the fracture was secured with suture fixation through bone tunnels over a patellar bony bridge using size 2 Phantom Fiber suture (Tornier) (Figure 5). Vicryl was used to repair the longitudinal patellar tendon split as well as the capsular and paratenon traumatic tears. Layered closure was completed and intraoperative radiographs were obtained (Figures 6A, 6B) prior to placement of a cylinder cast in full extension. Postoperatively, the patient remained overnight for observation and physical therapy evaluation. He was encouraged to bear weight in his cylinder cast as tolerated with crutches to assist with ambulation.

Postoperatively, the patient was maintained in full extension in the cylinder cast for 4 weeks. After cast removal, the patient was placed in a range-of-motion brace locked in extension for ambulation. He started physical therapy and was allowed to perform prone active-knee flexion limited to 90º, with passive extension, for an additional 4 weeks. At 8 weeks, the patient was allowed full-knee motion both active and passive, and the brace was discontinued. At his 18-week follow-up appointment, the patient reported successful return to all his normal activities, including skateboarding, with no apparent limitation in motion or weight-bearing. Examination at that time demonstrated knee range of motion from 5º in hyperextension to 135º in flexion, with his left knee having 5º in hyperextension and 145º in flexion. The patient appeared to have no gait abnormalities, and radiographs showed healed fractures. Because of a concern that continued compression across his tibial physis could lead to greater risk of growth arrest, the decision was made to remove the implants when radiographs showed healing. The patient returned to surgery at 20 weeks for implant removal. At 6 weeks after implant removal, the patient had returned to full activity with no residual pain and full-knee flexion equal to the uninvolved left knee. He was able to perform a stable single-leg squat on his affected leg, and his single-leg hop for distance was the same as his uninvolved leg. He was allowed to return to full sports activity. The patient will be followed with serial radiographs at 4 months, 8 months, and 12 months to look for premature physeal arrest. If an arrest occurs, treatment will be dictated by the extent of the arrest and the potential to cause either limb-length difference or angular deformity.

Discussion

Tibial tubercle fractures typically result from quadriceps contraction during sporting activities, predominantly in adolescent boys with open physes. Numerous modifications and additions have been made to the original classification of such fractures by Watson-Jones,² most notably by Ogden and colleagues³ in 1980. These additions have included combined tendon avulsions and tubercle fractures as described by Frankl and coauthors,⁴ complete proximal tibial physeal separation now classified as type 4 by Ryu and Debenham,⁵ and a “Y” fracture configuration now termed type 5 by McKoy and Stanitski.⁶ Pandya and colleagues⁸ reported on 41 tibial tubercle fractures and described a new classification scheme based on the known anatomical closure pattern of the proximal tibial physis and tibial tubercle apophysis. The authors stressed the role of advanced imaging, such as CT or magnetic resonance imaging, in preoperative management of these complex high-energy fractures in adolescents, and the need for intraoperative arthroscopy or arthrotomy to ensure anatomical reduction of the articular involvement.

Tibial tubercle fractures and extensor mechanism injuries that do not fit these classification patterns have also been described. In 1979, Houghton and Ackroyd⁹ reported 3 cases of acute loss of extensor mechanism secondary to a traumatic patellar sleeve avulsion. In 1995, Berg¹⁰ described an ipsilateral inferior pole osteochondral patellar avulsion fracture with patellar tendon avulsion without fracture at the tubercle in a 12-year-old boy. Another variant was described in a 2002 case series of 3 adolescent boys who underwent operative fixation for tibial metaphyseal partial-sleeve avulsion injuries.¹¹

Conclusion

We report a case of combined ipsilateral inferior lateral patellar avulsion fracture and an intra-articular tibial tubercle avulsion fracture with intervening longitudinal patellar tendon split. Preoperative standard radiographs were confusing, given the bony fragment high up by the patella, but use of advanced imaging, in this case CT, allowed us to fully characterize the origin of fracture fragments and realize we were dealing with a unique fracture pattern previously unreported in a pediatric patient. The CT findings allowed us to be better prepared preoperatively by having options for fixation of the patellar fracture, and the extent of articular involvement led us to decide that intra-articular evaluation would be required. Through the use of an open arthrotomy, anatomical articular reduction could be visualized and stabilized with screw fixation of the large, mostly cartilaginous tubercle fracture. Following the principles described by Pandya and colleagues,8 anatomical reduction was achieved, and, 6 months after the original surgery, the patient had return of full motion, clinical and radiographic union, and no clinical pain or limp, with no retained metallic implants across the tibial apophysis. Longer-term follow-up as planned will demonstrate any growth abnormality that would require further surgical intervention.

Case

A 76-year-old woman presented to the ED with right rib pain after tripping on a rug and sustaining a fall down the stairs in her home. The patient’s chart review showed a history of multiple falls over the past year, with injuries including left rib fracture, right distal radius fracture, ankle sprain, forehead contusion, and left hip contusion. Regarding her social history, the patient denied any alcohol or drug use. She was not on any prescription medications and had no known medication or food allergies.

Introduction

Geriatric patients aged 65 years and older represent a large, growing segment of the US population and, according to US Census Bureau data, represent an estimated 14% of the  population.¹ Moreover, this population accounts for 36% of all ambulance transports, 25% of hospitalizations, and 25% of total trauma costs.² Although geriatric patients are less likely to be involved in trauma compared with other age groups, they are more likely to have fatal outcomes when injured. Approximately 28% of deaths due to accidental causes involve persons aged 65 and older. The highest mortality rates from trauma are noted in patients in the 8th decade and older.³

Mechanism of Injury and Preexisting Conditions

The presence of preexisting conditions, which affect a patient’s physiological age, is associated with increased mortality rates.^7,8 As with other age groups, outcomes for geriatric trauma patients can also be predicted using the Injury Severity Score.⁹ Conditions associated with altered mental status in the geriatric trauma population and are listed in Table 1.

Review Data

The issue of traumatic injury in the aging population was studied at the authors’ institution through a retrospective chart review at the ED of Miami Valley Hospital, Dayton, Ohio, an urban hospital with an annual patient census of 95,000 visits.¹⁰ This study was approved by the Wright State University Institutional Review Board (IRB) and the Miami Valley Hospital Human Investigation and Research Committee (HIRC).

Laboratory Findings

Mortality

Although traumatic injury is a common presentation among geriatric emergency patients, this population is overall less likely to be involved in a traumatic event compared to other age groups. However, when injured, geriatric trauma patients are more likely to have fatal outcomes.

As previously noted, falls are the most common mechanism of injury in patients older than age 65 years. The trend of fall-related mortality increases with advanced age. It has been estimated that 36% of geriatric patients who fall will require a repeat ED visit or will die within 1 year following the fall.¹¹ Previous reports have demonstrated that mortality is associated with advanced age, injury severity score, shock index, transfusion, head injury, hypotension, and treatment site.^12-16

Cerebral Hemorrhage

In the study conducted at the authors’ institution, most patients receiving a head CT scan had at least one abnormality.¹⁰ Subdural hemorrhage was the most commonly reported abnormality followed by subarachnoid and intraparenchymal hemorrhages, respectively.¹⁰

Falls are a common cause of intracranial hemorrhage, and 30% to 40% of patients over age 65 years will experience at least one fall each year.¹⁷ Consistent with these statistics, fall was the most common mechanism of injury in the patient population at the authors’ institution. Intracranial hemorrhage can cause altered mental status by increasing the intracranial pressure and decreasing the cerebral perfusion pressure. These abnormalities are often amenable to medical and/or surgical treatment if identified in time.¹⁸

Hyperglycemia

Hyperglycemia was one of the most common diagnostic test abnormalities associated with altered mental status in the authors' study.¹⁰ Although increased blood glucose is part of the stress response to injury, geriatric patients experience a higher incidence of stress hyperglycemia and are unable to mount an adequate insulin response in trauma.^19,20 High-glucose levels are associated with significantly higher mortality rates among trauma patients.^21-24

Alcohol Intoxication

Alcohol intoxication was common among the patients in the author’s study.¹⁰ In contrast, a smaller percentage of patients were tested and found to be positive for opioids or benzodiazepines. The risk of a traumatic brain injury (TBI) increases significantly if the patient sustained the injury while under the influence of alcohol.²⁵ Alcohol increases the mortality after trauma especially in patients over the age of 40.²⁶ Alcohol-related TBIs are associated with poorer outcomes with increasing age.²⁷ Falls at ground level after alcohol consumption are associated with more casualties than nonalcohol-related falls.^28,29

Differential Diagnosis

As the case in this review illustrates, among geriatric trauma patients with altered mental status, the most common mechanism of injury is fall. The differential diagnosis should be considered, including intracranial hemorrhage, alcohol intoxication, nonprescription drug use, prescription-drug effects, infection, and/or metabolic or endocrine disorders. Appropriate laboratory and radiographic tests should be obtained, and may include CT of the brain and cervical spine, chemistry profile, complete blood count, chest X-ray, urinalysis, alcohol level, and toxicology screen.

Conclusion

This case represents one of many common presentations of trauma among geriatric patients. There was evidence of multiple falls by chart review and physical examination. Evidence of multiple traumatic events of various stages should raise the suspicion of neurological deficits, substance or prescription-medication effects, or physical abuse of the elderly patient. The ED workup should include brain CT, electrolytes, complete blood count, chest radiograph, and urinalysis. The patient should be admitted for observation and workup for medical and traumatic etiologies of multiple falls. When discharged, home-health services or rehabilitation services should be considered.

The results of the authors’ chart-review study confirmed that falls are the most common mechanism of injury in geriatric trauma patients presenting to the ED with altered mental status.¹⁰ The most common diagnostic test abnormalities associated with altered mental status in this study included hyperglycemia, abnormal CT results, anemia, and alcohol intoxication. Future studies are needed to access relations between ethanol or opioid intoxication and the presence of positive CT findings to guide clinicians’ judgment when ordering CT scans and other tests.

Dr Marco is a professor of emergency medicine and surgery, Wright State University Boonshoft School of Medicine, Kettering, Ohio; and an emergency physician at Miami Valley Hospital, Dayton, Ohio. Ms Edgell, Ms Eggers, and Mr Fagan are students at Wright State University Boonshoft School of Medicine, Dayton, Ohio. Dr Olson is the director of the research laboratory and professor of emergency medicine at Wright State University Boonshoft School of Medicine, Kettering, Ohio.

Case

A 76-year-old woman presented to the ED with right rib pain after tripping on a rug and sustaining a fall down the stairs in her home. The patient’s chart review showed a history of multiple falls over the past year, with injuries including left rib fracture, right distal radius fracture, ankle sprain, forehead contusion, and left hip contusion. Regarding her social history, the patient denied any alcohol or drug use. She was not on any prescription medications and had no known medication or food allergies.

Introduction

Geriatric patients aged 65 years and older represent a large, growing segment of the US population and, according to US Census Bureau data, represent an estimated 14% of the  population.¹ Moreover, this population accounts for 36% of all ambulance transports, 25% of hospitalizations, and 25% of total trauma costs.² Although geriatric patients are less likely to be involved in trauma compared with other age groups, they are more likely to have fatal outcomes when injured. Approximately 28% of deaths due to accidental causes involve persons aged 65 and older. The highest mortality rates from trauma are noted in patients in the 8th decade and older.³

Mechanism of Injury and Preexisting Conditions

The presence of preexisting conditions, which affect a patient’s physiological age, is associated with increased mortality rates.^7,8 As with other age groups, outcomes for geriatric trauma patients can also be predicted using the Injury Severity Score.⁹ Conditions associated with altered mental status in the geriatric trauma population and are listed in Table 1.

Review Data

The issue of traumatic injury in the aging population was studied at the authors’ institution through a retrospective chart review at the ED of Miami Valley Hospital, Dayton, Ohio, an urban hospital with an annual patient census of 95,000 visits.¹⁰ This study was approved by the Wright State University Institutional Review Board (IRB) and the Miami Valley Hospital Human Investigation and Research Committee (HIRC).

Laboratory Findings

Mortality

Although traumatic injury is a common presentation among geriatric emergency patients, this population is overall less likely to be involved in a traumatic event compared to other age groups. However, when injured, geriatric trauma patients are more likely to have fatal outcomes.

As previously noted, falls are the most common mechanism of injury in patients older than age 65 years. The trend of fall-related mortality increases with advanced age. It has been estimated that 36% of geriatric patients who fall will require a repeat ED visit or will die within 1 year following the fall.¹¹ Previous reports have demonstrated that mortality is associated with advanced age, injury severity score, shock index, transfusion, head injury, hypotension, and treatment site.^12-16

Cerebral Hemorrhage

In the study conducted at the authors’ institution, most patients receiving a head CT scan had at least one abnormality.¹⁰ Subdural hemorrhage was the most commonly reported abnormality followed by subarachnoid and intraparenchymal hemorrhages, respectively.¹⁰

Falls are a common cause of intracranial hemorrhage, and 30% to 40% of patients over age 65 years will experience at least one fall each year.¹⁷ Consistent with these statistics, fall was the most common mechanism of injury in the patient population at the authors’ institution. Intracranial hemorrhage can cause altered mental status by increasing the intracranial pressure and decreasing the cerebral perfusion pressure. These abnormalities are often amenable to medical and/or surgical treatment if identified in time.¹⁸

Hyperglycemia

Hyperglycemia was one of the most common diagnostic test abnormalities associated with altered mental status in the authors' study.¹⁰ Although increased blood glucose is part of the stress response to injury, geriatric patients experience a higher incidence of stress hyperglycemia and are unable to mount an adequate insulin response in trauma.^19,20 High-glucose levels are associated with significantly higher mortality rates among trauma patients.^21-24

Alcohol Intoxication

Alcohol intoxication was common among the patients in the author’s study.¹⁰ In contrast, a smaller percentage of patients were tested and found to be positive for opioids or benzodiazepines. The risk of a traumatic brain injury (TBI) increases significantly if the patient sustained the injury while under the influence of alcohol.²⁵ Alcohol increases the mortality after trauma especially in patients over the age of 40.²⁶ Alcohol-related TBIs are associated with poorer outcomes with increasing age.²⁷ Falls at ground level after alcohol consumption are associated with more casualties than nonalcohol-related falls.^28,29

Differential Diagnosis

As the case in this review illustrates, among geriatric trauma patients with altered mental status, the most common mechanism of injury is fall. The differential diagnosis should be considered, including intracranial hemorrhage, alcohol intoxication, nonprescription drug use, prescription-drug effects, infection, and/or metabolic or endocrine disorders. Appropriate laboratory and radiographic tests should be obtained, and may include CT of the brain and cervical spine, chemistry profile, complete blood count, chest X-ray, urinalysis, alcohol level, and toxicology screen.

Conclusion

This case represents one of many common presentations of trauma among geriatric patients. There was evidence of multiple falls by chart review and physical examination. Evidence of multiple traumatic events of various stages should raise the suspicion of neurological deficits, substance or prescription-medication effects, or physical abuse of the elderly patient. The ED workup should include brain CT, electrolytes, complete blood count, chest radiograph, and urinalysis. The patient should be admitted for observation and workup for medical and traumatic etiologies of multiple falls. When discharged, home-health services or rehabilitation services should be considered.

The results of the authors’ chart-review study confirmed that falls are the most common mechanism of injury in geriatric trauma patients presenting to the ED with altered mental status.¹⁰ The most common diagnostic test abnormalities associated with altered mental status in this study included hyperglycemia, abnormal CT results, anemia, and alcohol intoxication. Future studies are needed to access relations between ethanol or opioid intoxication and the presence of positive CT findings to guide clinicians’ judgment when ordering CT scans and other tests.

Dr Marco is a professor of emergency medicine and surgery, Wright State University Boonshoft School of Medicine, Kettering, Ohio; and an emergency physician at Miami Valley Hospital, Dayton, Ohio. Ms Edgell, Ms Eggers, and Mr Fagan are students at Wright State University Boonshoft School of Medicine, Dayton, Ohio. Dr Olson is the director of the research laboratory and professor of emergency medicine at Wright State University Boonshoft School of Medicine, Kettering, Ohio.

Case

A 76-year-old woman presented to the ED with right rib pain after tripping on a rug and sustaining a fall down the stairs in her home. The patient’s chart review showed a history of multiple falls over the past year, with injuries including left rib fracture, right distal radius fracture, ankle sprain, forehead contusion, and left hip contusion. Regarding her social history, the patient denied any alcohol or drug use. She was not on any prescription medications and had no known medication or food allergies.

Introduction

Geriatric patients aged 65 years and older represent a large, growing segment of the US population and, according to US Census Bureau data, represent an estimated 14% of the  population.¹ Moreover, this population accounts for 36% of all ambulance transports, 25% of hospitalizations, and 25% of total trauma costs.² Although geriatric patients are less likely to be involved in trauma compared with other age groups, they are more likely to have fatal outcomes when injured. Approximately 28% of deaths due to accidental causes involve persons aged 65 and older. The highest mortality rates from trauma are noted in patients in the 8th decade and older.³

Mechanism of Injury and Preexisting Conditions

The presence of preexisting conditions, which affect a patient’s physiological age, is associated with increased mortality rates.^7,8 As with other age groups, outcomes for geriatric trauma patients can also be predicted using the Injury Severity Score.⁹ Conditions associated with altered mental status in the geriatric trauma population and are listed in Table 1.

Review Data

The issue of traumatic injury in the aging population was studied at the authors’ institution through a retrospective chart review at the ED of Miami Valley Hospital, Dayton, Ohio, an urban hospital with an annual patient census of 95,000 visits.¹⁰ This study was approved by the Wright State University Institutional Review Board (IRB) and the Miami Valley Hospital Human Investigation and Research Committee (HIRC).

Laboratory Findings

Mortality

Although traumatic injury is a common presentation among geriatric emergency patients, this population is overall less likely to be involved in a traumatic event compared to other age groups. However, when injured, geriatric trauma patients are more likely to have fatal outcomes.

As previously noted, falls are the most common mechanism of injury in patients older than age 65 years. The trend of fall-related mortality increases with advanced age. It has been estimated that 36% of geriatric patients who fall will require a repeat ED visit or will die within 1 year following the fall.¹¹ Previous reports have demonstrated that mortality is associated with advanced age, injury severity score, shock index, transfusion, head injury, hypotension, and treatment site.^12-16

Cerebral Hemorrhage

In the study conducted at the authors’ institution, most patients receiving a head CT scan had at least one abnormality.¹⁰ Subdural hemorrhage was the most commonly reported abnormality followed by subarachnoid and intraparenchymal hemorrhages, respectively.¹⁰

Falls are a common cause of intracranial hemorrhage, and 30% to 40% of patients over age 65 years will experience at least one fall each year.¹⁷ Consistent with these statistics, fall was the most common mechanism of injury in the patient population at the authors’ institution. Intracranial hemorrhage can cause altered mental status by increasing the intracranial pressure and decreasing the cerebral perfusion pressure. These abnormalities are often amenable to medical and/or surgical treatment if identified in time.¹⁸

Hyperglycemia

Hyperglycemia was one of the most common diagnostic test abnormalities associated with altered mental status in the authors' study.¹⁰ Although increased blood glucose is part of the stress response to injury, geriatric patients experience a higher incidence of stress hyperglycemia and are unable to mount an adequate insulin response in trauma.^19,20 High-glucose levels are associated with significantly higher mortality rates among trauma patients.^21-24

Alcohol Intoxication

Alcohol intoxication was common among the patients in the author’s study.¹⁰ In contrast, a smaller percentage of patients were tested and found to be positive for opioids or benzodiazepines. The risk of a traumatic brain injury (TBI) increases significantly if the patient sustained the injury while under the influence of alcohol.²⁵ Alcohol increases the mortality after trauma especially in patients over the age of 40.²⁶ Alcohol-related TBIs are associated with poorer outcomes with increasing age.²⁷ Falls at ground level after alcohol consumption are associated with more casualties than nonalcohol-related falls.^28,29

Differential Diagnosis

As the case in this review illustrates, among geriatric trauma patients with altered mental status, the most common mechanism of injury is fall. The differential diagnosis should be considered, including intracranial hemorrhage, alcohol intoxication, nonprescription drug use, prescription-drug effects, infection, and/or metabolic or endocrine disorders. Appropriate laboratory and radiographic tests should be obtained, and may include CT of the brain and cervical spine, chemistry profile, complete blood count, chest X-ray, urinalysis, alcohol level, and toxicology screen.

Conclusion

This case represents one of many common presentations of trauma among geriatric patients. There was evidence of multiple falls by chart review and physical examination. Evidence of multiple traumatic events of various stages should raise the suspicion of neurological deficits, substance or prescription-medication effects, or physical abuse of the elderly patient. The ED workup should include brain CT, electrolytes, complete blood count, chest radiograph, and urinalysis. The patient should be admitted for observation and workup for medical and traumatic etiologies of multiple falls. When discharged, home-health services or rehabilitation services should be considered.

The results of the authors’ chart-review study confirmed that falls are the most common mechanism of injury in geriatric trauma patients presenting to the ED with altered mental status.¹⁰ The most common diagnostic test abnormalities associated with altered mental status in this study included hyperglycemia, abnormal CT results, anemia, and alcohol intoxication. Future studies are needed to access relations between ethanol or opioid intoxication and the presence of positive CT findings to guide clinicians’ judgment when ordering CT scans and other tests.

Dr Marco is a professor of emergency medicine and surgery, Wright State University Boonshoft School of Medicine, Kettering, Ohio; and an emergency physician at Miami Valley Hospital, Dayton, Ohio. Ms Edgell, Ms Eggers, and Mr Fagan are students at Wright State University Boonshoft School of Medicine, Dayton, Ohio. Dr Olson is the director of the research laboratory and professor of emergency medicine at Wright State University Boonshoft School of Medicine, Kettering, Ohio.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

Discussion

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.² As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant² described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.³

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.² In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,⁴ PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

Discussion

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.² As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant² described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.³

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.² In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,⁴ PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.

Case

The mother of a 3-year-old boy presented her son to the ED for evaluation after she noticed peeling of the skin in his perianal region. She stated that the peeling had started 1 day prior to presentation. Two days earlier, the mother had brought the same patient to the ED for evaluation of a fever, sore throat, and a slight rash over his face. The boy’s vital signs at the initial presentation were: temperature, 101.8°F; heart rate, 102 beats/minute; and respiratory rate, 28 breaths/minute. Oxygen saturation was 98% on room air.

During this first visit, the mother denied the child having had any fever, chills, headache, sore throat, facial rash, joint pain, or pain on defecation. He had no significant medical history and no known drug allergies. After examination, a throat culture was taken, and the patient was given acetaminophen and discharged home with a diagnosis of viral syndrome.

At the second presentation, physical examination revealed a well-developed child in no distress. The examination was negative except for a 4 x 2 cm area of desquamation present over the perianal region (Figure).

The area of desquamation was dry, mildly erythematous without discharge, and nontender. The patient’s vital signs at this presentation were stable, and he was afebrile. The remaining physical examination findings were normal. The throat culture taken during the first ED presentation was reported as negative. A perianal swab was sent for culture and sensitivity. This was later reported to be positive for group A β-hemolytic streptococci (GABHS), which is sensitive to penicillin. The patient was discharged home in the care of his mother with a prescription of penicillin.  A 10-day follow-up showed complete resolution of the skin rash.

Discussion

The rash in this case was most likely the result of scarlet fever with an unusual presentation of PSD; the signs and symptoms of which include perianal erythema, itching, rectal pain, sometimes blood-streaked stools, rectal bleeding, irritation or pruritus, tissue loss and exudation, secondary constipation, and cellulitis. Perianal streptococcal dermatitis has also been described in the adult literature.² As with pediatric cases, PSD in adults is usually caused by GABHS.

Evaluation and Diagnosis

A rapid streptococcal test of suspicious areas can confirm the diagnosis. Fever, sore throat, and arthralgia are rare; however, culture from the perianal region grows GABHS. Titers are usually not elevated in laboratory evaluation. A routine skin culture is an alternative diagnostic aid.

Brilliant² described the bright red color of PSD as a sharply demarcated rash that is caused by GABHS. As previously stated, symptoms include perianal rash, itching, and rectal pain; blood-streaked stools may also be seen in one-third of patients. It primarily occurs in children between 6 months and 10 years of age and is often misdiagnosed and treated inappropriately.³

Prompt diagnosis of GABHS is important. If untreated, it can cause serious systemic infections, especially in elderly and in newborn patients. Treatment with antibiotics resolves the condition in the majority of patients.² In the acute stage, a white pseudomembrane may be present. As the rash becomes more chronic, the perianal eruption may consist of painful fissures, a dry mucoid discharge, or psoriasiform plaques. Perianal dermatitis can also be caused by Staphylococcus aureus or Candida. Confirmation of the diagnosis is accomplished by culturing a moderate-to-heavy growth of GABHS on 5% sheep-blood agar.

Treatment

A 10-day course of oral penicillin produces resolution of the dermatitis and other symptoms in most patients, but a relapse rate as high as 39% has been reported. Other treatment plans include amoxicillin, 40 mg/kg per day, divided into three doses, and/or topical applications of mupirocin 2% three times per day for 10 days. Penicillin, clindamycin phosphate, and erythromycin have also been used.

Although penicillin is generally recommended for treatment of GABHS infection, amoxicillin is often better tolerated in the pediatric population due to its superior palatability. Early antibiotic treatment causes a dramatic and rapid improvement of symptoms. However, according to Olson et al,⁴ PSD initially treated with amoxicillin or penicillin is consistently associated with a high risk of clinical recurrence. Whether treatment with a β-lactamase–resistant agent reduces this risk is uncertain.

Conclusion

This case represents an unusual presentation of scarlet fever manifesting as perianal dermatitis caused by GABHS. Although more common in the pediatric population, adult cases have been documented in the literature. As this case illustrates, early recognition and treatment with penicillin (or amoxicillin) produces rapid improvement and resolution of symptoms. 

Dr Nibhanipudi is a professor of clinical emergency medicine at New York Medical College - Metropolitan Hospital Center, New York.