DIFFUSE NEONATAL HEMANGIOMATOSIS PRESENTING AS BILATERAL IRIS HEMANGIOMAS IN AN INFANT Stephen S. Couvillion, MD,* Charles C. Wykoff, MD, PHD,* Jacqueline L. Tutiven, MD,† Anne M. Schaefer, MD,‡ Timothy G. Murray, MD, FACS*

Purpose: To describe a case of diffuse neonatal hemangiomatosis presenting as bilateral iris hemangiomas. Method: Case report. Patient: A 2-month-old girl presented with bilateral enlarging red masses of her irises, congestive heart failure, and abdominal distension. The iris masses were diagnosed as hemangiomas, and she was found to have diffuse neonatal hemangiomatosis also involving her skin, liver, heart, and cerebellum. Results: The patient’s systemic hemangiomas were successfully treated with four cycles of weekly intravenous vincristine (0.05 mg/kg/dose) with concurrent resolution of her right iris hemangioma within 6 weeks. The left iris lesion then regressed within 1 month following 1 subTenon injection of 20 mg of triamcinolone acetonide with residual iridocorneal adhesions at the site. Conclusion: Diffuse neonatal hemangiomatosis should be considered in the setting of bilateral iris hemangiomas. Also, this case demonstrates that iris hemangiomas associated with diffuse neonatal hemangiomatosis may respond to systemic vincristine, and that periocular steroids may be useful for treating iris hemangiomas that are unresponsive to systemic vincristine alone. RETINAL CASES & BRIEF REPORTS 3:279 –282, 2009

From the *Department of Ophthalmology, Bascom Palmer Eye Institute, and †Department of Anesthesiology, University of Miami Miller School of Medicine; and ‡Department of Hematology and Oncology, Joe DiMaggio Childrens Hospital, Hollywood, Florida.

may number in the hundreds. The lesions may be asymptomatic or lead to complications including congestive heart failure, hepatomegaly, and thrombocytopenia. Mortality rates have been reported to range from 60 –90% within the first few months of life.1–3 Ocular manifestations include hemangiomas of the eyelid, conjunctiva, and uveal tract,4 and iris involvement has been associated with glaucoma.5 To the authors’ knowledge, this is the first reported case of DNH in which the initial clinical presentation was bilateral iris hemangiomas.

D

iffuse neonatal hemangiomatosis (DNH) is a rare condition characterized by multiple hemangiomas involving at least three organs, most commonly the skin, gastrointestinal tract, lungs, and central nervous system. The hemangiomas are often small and

Supported by the National Institutes of Health and an unrestricted grant to the University of Miami from Research to Prevent Blindness. Reprint requests: Timothy G. Murray, MD, FACS, Bascom Palmer Eye Institute, 900 N.W. 17th Street, Miami, FL 33136; e-mail: [email protected]

Case Report A 2-month-old full-term, 6 kg girl presented with a 1-day history of an enlarging red mass in her right eye and a 2-week history of an enlarging red mass in her left eye. There was no significant

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Fig. 1. Photographs of iris hemangiomas before and after treatment. A, Right iris with small lesion located temporally. B, Left iris vascular lesion with an appearance similar to juvenile xanthogranuloma. C, Excellent involutional response of right eye lesion 6 weeks after initiation of systemic vincristine and of the left eye lesion (D) 4 weeks after posterior sub-Tenon injection of 20 mg of triamcinolone acetonide with iridocorneal adhesions.

family history and no history of trauma. Ocular examination revealed apparent bilateral hyphema believed to represent atypical juvenile xanthogranulomatosis (Figure 1A, B). Preoperative evaluation for examination under anesthesia revealed high output congestive heart failure and abdominal distension. Subsequent imaging studies revealed extensive liver lesions consistent with hemangiomas, cardiomegaly, and a probable right cerebellar hemangioma (Figure 2). The patient also had cutaneous lesions consistent with hemangiomas on her abdomen in the epigastric region, on her left arm and on her left inner thigh just below her groin. The patient was diagnosed with DNH without systemic evidence of Kasabach-Merritt syndrome. She was hospitalized and her cardiac performance was optimized using digoxin, furosemide, and captopril. The patient was initially treated with prednisone 4 mg/kg/d but after 1 month of therapy her hemangiomas were unchanged. Therefore, she was treated with 4 cycles of weekly intravenous vincristine at 0.05 mg/kg/dose without toxicity. Her hepatic, cerebellar, and cutaneous hemangiomas dramatically involuted with eventually near complete resolution of the lesions. Examination of the eyes 6 weeks after initiation of vincristine revealed the right iris hemangioma to have completely regressed (Figure 1C) and the left iris lesion to be unchanged. At the time of examination under anesthesia, there was no evidence of glaucoma in either eye. The left iris lesion was then managed with a posterior subTenon

injection of 20 mg of triamcinolone acetonide. One month follow-up revealed complete regression of the left iris lesions with residual iridocorneal adhesions at the site (Figure 1D). One- and 2-year follow-up examinations were unchanged.

Discussion The differential diagnosis of apparent bilateral hyphema in a neonate includes trauma, inflammation, rubeosis, blood dyscrasias, vascular abnormalities, and neoplasms such as juvenile xanthogranuloma, medulloepitheliomas, and retinoblastoma. DNH is a rare entity with approximately 67 previously reported cases, 8 of which have had documented unilateral iris hemangiomas.1,2,4 –9 To our knowledge, this represents the first case of DNH which presented clinically as bilateral iris hemangiomas. Our patient’s visceral and right iris hemangiomas responded rapidly to systemic vincristine and the left iris hemangioma responded to the combination of vincristine and a posterior subTenon injection of triamcinolone.

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Fig. 2. A, Computed tomography (CT) imaging revealed hepatomegaly with multiple hepatic lesions consistent with hemangiomas as well as splenomegaly. B, CT imaging of the chest revealed cardiomegaly consistent with the patient’s high output cardiac failure. C, CT imaging of the brain revealed a right cerebellar vascular lesion consistent with a hemangioma.

Surgical management of patients with DNH requires multidisciplinary perioperative care and close follow-up. When inhalation anesthesia is required, it should be used with caution due to its potential depressive effects on the myocardium. Treatment of DNH-associated hemangiomas is indicated for tumors that threaten vision or life. Corticosteroids are the most commonly employed agents for treatment of hemangiomas. Response to systemic administration is highly variable and prolonged therapy beyond 8 to 12 weeks is common which may lead to significant side effects such as growth delay, insomnia, and adrenocortical imbalances. While fewer than half of hemangiomas shrink in response to treatment, if the tumor is responsive, cessation of growth or onset of involution is expected within 2 weeks.10,11 Of note, hepatic lesions as seen in the current patient are generally less steroid responsive.10 In comparison, corticosteroid administration locally is often effective for well-defined cutaneous and anterior orbital lesions; in a series of 25 patients, 84% showed a moderate or marked response.12 Potential complications include hypopigmentation, local fat atrophy or eyelid necrosis, and central retinal artery embolization. Interferon alpha 2a (IFN␣2a), with antiangiogenic and antineoplastic properties, is effective and may result in a higher rate of actual shrinkage of lesions compared to corticosteroids. But potential severe side effects limit their use. For example, spastic diplegia was seen in 5 of 26 children treated with IFN␣2a and in 3 of these patients’ paralysis was permanent.13 Other reported treatments include cyclophosphamide, limited IFN␣2a, surgical excision, embolization, laser photocoagulation, and radiotherapy.10,14 Systemic vincristine has emerged as a new potential therapy for hemangiomas. Its efficacy has been re-

ported in the setting of the Kasabach-Merritt syndrome15 and various reports have found it to be efficacious for large, endangering hemangiomas.16,17 The usual dosage is 0.05 mg/kg in children less than 10 kg, or 1.5 mg/m2 in infants greater than 10 kg, given intravenously on a weekly basis.10 Unfortunately, central venous access is often necessary given its highly caustic nature. Potential complications include seizures, hemorrhage, and respiratory and biliary tract obstruction.10,16 Further study is needed to determine the optimal treatment regimen for managing life- or function-threatening hemangiomas. This case demonstrates that iris hemangiomas associated with DNH may respond to systemic vincristine, and that periocular steroids may be useful for treating iris hemangiomas that are unresponsive to systemic vincristine alone. References 1.

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Stratte EG, Tope WD, Johnson CL, Swanson NA. Multimodal management of diffuse neonatal hemangiomatosis. J Am Acad Dermatol 1996;34:337–342. Haik BG, Clancy P, Ellsworth RM, et al. Ocular manifestations in diffuse neonatal hemangiomatosis. J Pediatr Ophthalmol Strabismus 1983;20:101–105. Golitz LE, Rudikoff J, O’Meara OP. Diffuse neonatal hemangiomatosis. Pediatr Dermatol 1986;3:145–152. Chang CW, Rao NA, Stout JT. Histopathology of the eye in diffuse neonatal hemangiomatosis. Am J Ophthalmol 1998; 125:868–870. Weiss MJ, Ernest JT. Diffuse congenital hemangiomatosis with infantile glaucoma. Am J Ophthalmol 1976;81:216–218. Bryce IG, Pai V, Bradbury JA. Spontaneous resolution of iris and cutaneous haemangiomata in diffuse neonatal haemangiomatosis. Eye 1999;13:388–390. Naidoff MA, Kenyon KR, Green WR. Iris hemangioma and abnormal retinal vasculature in a case of diffuse congenital hemangiomatosis. Am J Ophthalmol 1971;72:633–644.

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RETINAL CASES & BRIEF REPORTSℜ Fryns JP, Eggermont E, Eeckels R. Multiple diffuse hemangiomatosis. Case report and review of the literature. Z Kinderheilkd 1974;117:115–119. Burke EC, Winkelmann RK, Strickland MK. Disseminated hemangiomatosis. The newborn with central nervous system involvement. Am J Dis Child 1964;108:418–424. Bruckner AL, Frieden IJ. Hemangiomas of infancy. J Am Acad Dermatol 2003;48:477–493. Enjolras O, Riche MC, Merland JJ, Escande JP. Management of alarming hemangiomas in infancy: a review of 25 cases. Pediatrics 1990;85:491–498. Kushner BJ. The treatment of periorbital infantile hemangioma with intralesional corticosteroid. Plast Reconstr Surg 1985;76:517–526. Barlow CF, Priebe CJ, Mulliken JB, et al. Spastic diplegia as

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a complication of interferon alfa-2a treatment of hemangiomas of infancy. J Pediatr 1998;132:527–530. Wilson MW, Hoehn ME, Haik BG, et al. Low-dose cyclophosphamide and interferon alfa 2a for the treatment of capillary hemangioma of the orbit. Ophthalmology 2007;114: 1007–1011. Haisley-Royster C, Enjolras O, Frieden IJ, et al. KasabachMerritt phenomenon: a retrospective study of treatment with vincristine. J Pediatr Hematol Oncol 2002;24:459–462. Moore J, Lee M, Garzon M, et al. Effective therapy of a vascular tumor of infancy with vincristine. J Pediatr Surg 2001;36:1273–1276. Perez Payarols J, Pardo Masferrer J, Gomez Bellvert C. Treatment of life-threatening infantile hemangiomas with vincristine. N Engl J Med 1995;333:69.

Diffuse neonatal hemangiomatosis presenting as bilateral iris hemangiomas in an infant.

To describe a case of diffuse neonatal hemangiomatosis presenting as bilateral iris hemangiomas...
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