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Varicella gangrenosa in the newborn upper extremity: A case report
Varicella Gangrenosa in the Newborn Upper Extremity: A Case Report Ronald M. Friedman, MD, Virchel E. Wood, MD, Loma Linda, CA
Chickenpox, an acute communicable disease caused by the varicella-zoster virus, is a common and generally benign childhood exanthem. In rare instances, however, varicella may be complicated by gangrene of the extremities, more commonly the lower than the upper, secondary to arterial thrombosis or bacterial superinfection.l.2 A review of the literature demonstrates that in every child reported, varicella gangrenosa resulted from a postnatal infection with chickenpox.'-7 We describe a case of extremity gangrene due to an in utero varicella infection.
Case Report An infant boy weighing 2.8 kg was born at 40 weeks gestation to a 17-year-old mother. At 6 weeks of pregnancy, the mother developed clinically documented chickenpox and experienced transient spotty vaginal bleeding. The pregnancy and delivery were otherwise uncomplicated except for meconium staining at birth. At birth, the baby's left upper extremity was noted to be markedly hypoplastic and gangrenous just distal to the elbow (Fig. 1). The fingers were minimally developed, fused, and devoid of skin. Multiple draining, yellow vesicular lesions were seen in the forearm, arm, and the axilla displayed bullae. Ipsilateral
From the Department of Orthopaedic Surgery, Loma Linda University School of Medicine, Loma Linda, CA. Received for publication Feb. 1, 1995; accepted in revised form July 9, 1995. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Virchel E. Wood, MD, Department of Orthopaedic Surgery, Loma Linda University Medical Center, 11234 Anderson Street, Room A519, Loma Linda, CA 92354.
pectoralis hypoplasia and axillary contracture were present. Examination demonstrated no constricting bands and no other congenital anomalies, and x-ray films of the left upper limb revealed hypoplastic tapering of the distal radius and ulna (Fig. 2). The hemogram was normal; vesicular and blood cultures were negative for organisms, including varicella; antibody titers were negative for toxoplasmosis, rubella, herpes simplex, and h u m a n immunodeficiency virus (HIV). Varicella-zoster IgM antibody titers obtained at 1 week of life were strongly positive (enzyme immunoassay index 0.85). Acyclovir 30 mg/kg/d, ampicillin, and cefotaxime were administered empirically. An above-elbow amputation was performed at the age of 13 days, after demarcation of the necrotic distal limb. Histologic examination demonstrated extensive ulceration with an acute inflammatory exudate. The patient recovered uneventfully and subsequently underwent axillary contracture release and amputation revision at 7 months of age. The axillary contracture was secondary to scarring from prior chickenpox vesicular eruptions. Humerus shortening due to bony overgrowth was performed at the age of 3 years. The child currently uses an above-elbow prosthesis.
Discussion Although the gangrenous complications of varicella were first described by Stokes 8 in 1807, the term varicella gangrenosa was coined by Hutchinson 9 in 1881. Gangrenous changes occur in 0.05% to 0.16% of patients with varicella infections? In most cases, the necrotic area is confined to the site of a previous vesicle. Extensive extremity involvement is exceedingly rare and is thought to be due to arterial thrombosis? To our knowledge, extremity gangrene The Journal of Hand Surgery 487
488
Friedman and Wood / Varicella Gangrenosa in Newborn
Figure 1. The upper extremity was markedly hypoplastic and gangrenous just distal to the elbow. Multiple draining lesions were present in the forearm and hand. Note the vesicular lesions on the upper arm, which are characteristic of varicella infection.
due to varicella has not previously been described in the newborn. In fact, Shaffer et al. 1~did not implicate a viral etiology in any of 64 cases of neonatal extremity gangrene reviewed over a 40-year period. Extremity hypoplasia, conversely, is a known sequela of varicella infection in early pregnancy. H ~6 Maternal chickenpox during the first trimester is associated with a 1% to 2.2% risk of fetal malformations, u,12 This fetal varicella syndrome frequently includes limb hypoplasia, paralysis, rudimentary digits, and dermatomal scarring, all of which were present in the patient reported. Savage et al. 15 attributed these manifestations to viral-mediated limb denervation during early development. Other muscu-
Figure 2. In this x-ray film of the upper extremity, note the distal tapering of the radius and ulna.
loskeletal abnormalities seen in fetal varicella syndrome include clubfoot, scoliosis, scapular hypoplasia, and clavicular hypoplasia.'6 The diagnosis of congenital varicella syndrome is based on clinical suspicion. It should be considered whenever the characteristic congenital anomalies occur following a first-trimester maternal varicella infection. 11a2The diagnosis m a y be confirmed by the presence of varicella in the vesicular eruptions or a positive varicella I g M titer in the infant's serum, although neither of these is a constant finding. 11,12,~7 Congenital varicella infection should be treated with high-dose acyclovir and supportive care. ~* When varicella is complicated by gangrene, antibiotic therapy directed toward Streptococcus and Staphylococcus should be added until vesicular culture results are available. 2 As with any other etiology, the presence of infectious gangrene must be adequately addressed by aggressive surgical debridement and amputation when indicated.
References 1. Bogumill GP. Bilateral above the knee amputations: a complication of chickenpox. J Bone Joint Surg 1965;47: 371-374. 2. Smith EWP, Garson A, Boyleston JA, Katz SL, Wilfert CM. Varicella gangrenosa due to group A B-hemolytic streptococcus. Pediatrics 1976;57:306-310. 3. Barenberg LH, Lewis JM. Gangrenous varicella. Arch Pediatr 1927;44:653-654. 4. Watson TM. Massive skin gangrene complicating chickenpox. JAMA 1934;102:2179-2180. 5. Crocker AM, Middlekamp JN. Varicella gangrenosa. J Pediatr 1959;54:104-108. 6. John J. Varieties of varicella gangrenosa. Pediatrics 1977; 60:384. 7. Kidney DD, Watson JBG, Nisar N. Varicella gangrenosa. Arch Dis Child 1988;63:444-445. 8. Stokes W. An eruptive disease of children. Dublin Med Phys Essays 1807;1:146. 9. Hutchinson J. On gangrenous eruptions in connection with vaccination and chickenpox. Lancet 1881 ;2:751-753. 10. Shaffer WO, Aulicino PL, DuPuy TE. Upper extremity gangrene of the newborn infant. J Hand Surg 1984;9A: 88-90. 11. Enders G, Miller E, Cradock-Watson J, Bolley I, Ridehalgh M. Consequences of varicella and herpes zoster in pregnancy: prospective study of 1739 cases. Lancet 1994;343:1548-1551. 12. Pastuszak AL, Levy M, Schick B, Zuber C, Feldkamp M, Gladstone Jet al. Outcome after maternal varicella infection in the first 20 weeks of pregnancy. N Engl J Med 1994;330:901-905. 13. Magliocco AM, Demetrick DJ, Sarnat HB, Hwang W. Varicella embryopathy. Arch Pathol Lab Med 1992; 116:181-186.
The Journal of Hand Surgery/Vol. 21A No. 3 May 1996 14. Laforet EG, Lynch CL. Multiple congenital defects following maternal varicella. N Eng J Med 1947;236:534-537. 15. Savage MO, Moosa A, Gordon RR. Maternal varicella infection as a cause of fetal malformations. Lancet 1973; 1:352-354. 16. Higa K, Dan K, Manabe H. Varicella-zoster virus infections during pregnancy: hypothesis concerning the mecha-
489
nisms of congenital malformations. Obstet Gynecol 1987; 69:214-222. 17. DaSilva O, Hammerberg O, Chance GM. Fetal varicella syndrome. Pediatr Infect Dis J 1990;9: 854-855. 18. Brunell PA. Varicella in pregnancy, the fetus, and the newborn: problems in management. J Infect Dis 1992;166: $42-$47.
Chickenpox, an acute communicable disease caused by the varicella-zoster virus, is a common and generally benign childhood exanthem. In rare instances, however, varicella may be complicated by gangrene of the extremities, more commonly the lower than the upper, secondary to arterial thrombosis or bacterial superinfection.l.2 A review of the literature demonstrates that in every child reported, varicella gangrenosa resulted from a postnatal infection with chickenpox.'-7 We describe a case of extremity gangrene due to an in utero varicella infection.
Case Report An infant boy weighing 2.8 kg was born at 40 weeks gestation to a 17-year-old mother. At 6 weeks of pregnancy, the mother developed clinically documented chickenpox and experienced transient spotty vaginal bleeding. The pregnancy and delivery were otherwise uncomplicated except for meconium staining at birth. At birth, the baby's left upper extremity was noted to be markedly hypoplastic and gangrenous just distal to the elbow (Fig. 1). The fingers were minimally developed, fused, and devoid of skin. Multiple draining, yellow vesicular lesions were seen in the forearm, arm, and the axilla displayed bullae. Ipsilateral
From the Department of Orthopaedic Surgery, Loma Linda University School of Medicine, Loma Linda, CA. Received for publication Feb. 1, 1995; accepted in revised form July 9, 1995. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Virchel E. Wood, MD, Department of Orthopaedic Surgery, Loma Linda University Medical Center, 11234 Anderson Street, Room A519, Loma Linda, CA 92354.
pectoralis hypoplasia and axillary contracture were present. Examination demonstrated no constricting bands and no other congenital anomalies, and x-ray films of the left upper limb revealed hypoplastic tapering of the distal radius and ulna (Fig. 2). The hemogram was normal; vesicular and blood cultures were negative for organisms, including varicella; antibody titers were negative for toxoplasmosis, rubella, herpes simplex, and h u m a n immunodeficiency virus (HIV). Varicella-zoster IgM antibody titers obtained at 1 week of life were strongly positive (enzyme immunoassay index 0.85). Acyclovir 30 mg/kg/d, ampicillin, and cefotaxime were administered empirically. An above-elbow amputation was performed at the age of 13 days, after demarcation of the necrotic distal limb. Histologic examination demonstrated extensive ulceration with an acute inflammatory exudate. The patient recovered uneventfully and subsequently underwent axillary contracture release and amputation revision at 7 months of age. The axillary contracture was secondary to scarring from prior chickenpox vesicular eruptions. Humerus shortening due to bony overgrowth was performed at the age of 3 years. The child currently uses an above-elbow prosthesis.
Discussion Although the gangrenous complications of varicella were first described by Stokes 8 in 1807, the term varicella gangrenosa was coined by Hutchinson 9 in 1881. Gangrenous changes occur in 0.05% to 0.16% of patients with varicella infections? In most cases, the necrotic area is confined to the site of a previous vesicle. Extensive extremity involvement is exceedingly rare and is thought to be due to arterial thrombosis? To our knowledge, extremity gangrene The Journal of Hand Surgery 487
488
Friedman and Wood / Varicella Gangrenosa in Newborn
Figure 1. The upper extremity was markedly hypoplastic and gangrenous just distal to the elbow. Multiple draining lesions were present in the forearm and hand. Note the vesicular lesions on the upper arm, which are characteristic of varicella infection.
due to varicella has not previously been described in the newborn. In fact, Shaffer et al. 1~did not implicate a viral etiology in any of 64 cases of neonatal extremity gangrene reviewed over a 40-year period. Extremity hypoplasia, conversely, is a known sequela of varicella infection in early pregnancy. H ~6 Maternal chickenpox during the first trimester is associated with a 1% to 2.2% risk of fetal malformations, u,12 This fetal varicella syndrome frequently includes limb hypoplasia, paralysis, rudimentary digits, and dermatomal scarring, all of which were present in the patient reported. Savage et al. 15 attributed these manifestations to viral-mediated limb denervation during early development. Other muscu-
Figure 2. In this x-ray film of the upper extremity, note the distal tapering of the radius and ulna.
loskeletal abnormalities seen in fetal varicella syndrome include clubfoot, scoliosis, scapular hypoplasia, and clavicular hypoplasia.'6 The diagnosis of congenital varicella syndrome is based on clinical suspicion. It should be considered whenever the characteristic congenital anomalies occur following a first-trimester maternal varicella infection. 11a2The diagnosis m a y be confirmed by the presence of varicella in the vesicular eruptions or a positive varicella I g M titer in the infant's serum, although neither of these is a constant finding. 11,12,~7 Congenital varicella infection should be treated with high-dose acyclovir and supportive care. ~* When varicella is complicated by gangrene, antibiotic therapy directed toward Streptococcus and Staphylococcus should be added until vesicular culture results are available. 2 As with any other etiology, the presence of infectious gangrene must be adequately addressed by aggressive surgical debridement and amputation when indicated.
References 1. Bogumill GP. Bilateral above the knee amputations: a complication of chickenpox. J Bone Joint Surg 1965;47: 371-374. 2. Smith EWP, Garson A, Boyleston JA, Katz SL, Wilfert CM. Varicella gangrenosa due to group A B-hemolytic streptococcus. Pediatrics 1976;57:306-310. 3. Barenberg LH, Lewis JM. Gangrenous varicella. Arch Pediatr 1927;44:653-654. 4. Watson TM. Massive skin gangrene complicating chickenpox. JAMA 1934;102:2179-2180. 5. Crocker AM, Middlekamp JN. Varicella gangrenosa. J Pediatr 1959;54:104-108. 6. John J. Varieties of varicella gangrenosa. Pediatrics 1977; 60:384. 7. Kidney DD, Watson JBG, Nisar N. Varicella gangrenosa. Arch Dis Child 1988;63:444-445. 8. Stokes W. An eruptive disease of children. Dublin Med Phys Essays 1807;1:146. 9. Hutchinson J. On gangrenous eruptions in connection with vaccination and chickenpox. Lancet 1881 ;2:751-753. 10. Shaffer WO, Aulicino PL, DuPuy TE. Upper extremity gangrene of the newborn infant. J Hand Surg 1984;9A: 88-90. 11. Enders G, Miller E, Cradock-Watson J, Bolley I, Ridehalgh M. Consequences of varicella and herpes zoster in pregnancy: prospective study of 1739 cases. Lancet 1994;343:1548-1551. 12. Pastuszak AL, Levy M, Schick B, Zuber C, Feldkamp M, Gladstone Jet al. Outcome after maternal varicella infection in the first 20 weeks of pregnancy. N Engl J Med 1994;330:901-905. 13. Magliocco AM, Demetrick DJ, Sarnat HB, Hwang W. Varicella embryopathy. Arch Pathol Lab Med 1992; 116:181-186.
The Journal of Hand Surgery/Vol. 21A No. 3 May 1996 14. Laforet EG, Lynch CL. Multiple congenital defects following maternal varicella. N Eng J Med 1947;236:534-537. 15. Savage MO, Moosa A, Gordon RR. Maternal varicella infection as a cause of fetal malformations. Lancet 1973; 1:352-354. 16. Higa K, Dan K, Manabe H. Varicella-zoster virus infections during pregnancy: hypothesis concerning the mecha-
489
nisms of congenital malformations. Obstet Gynecol 1987; 69:214-222. 17. DaSilva O, Hammerberg O, Chance GM. Fetal varicella syndrome. Pediatr Infect Dis J 1990;9: 854-855. 18. Brunell PA. Varicella in pregnancy, the fetus, and the newborn: problems in management. J Infect Dis 1992;166: $42-$47.