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Neonatal Hemangiomatosis Presenting as Infantile Spasms By M. A. McShane 1,]. P. Finn2, M. A. Hall-Craggs 2, O. Hanmer3 and]. Harper4 1Department of Neurology, 2NMR Unit, Department of Radiology and 4Department of Dermatology, Hospital for Siek Children, Great Ormond Street, London WC1, and 3Department of Paediatries, Newham General Hospital, London E13, U.K.
Case report
The previously unreported association of neonatal hemangiomatosis and infantile spasms is described in a 4-month-old female. The high mortality and morbidity of this neurocutaneous syndrome necessitates prompt recognition and investigation, to enable identification of associated internalIesions.
Keywords Neonatal hemangiomatosis spasms - Magnetic resonance imaging
-
Infantile
Zusammenfassung Eine bisher unveröffentlichte Assoziation von neonataler Hämangiomatose und Blick-Nick-SalaamKrämpfen wird an einem 4 Monate alten Baby beschrieben. Die hohe Sterblichkeit und Morbidität dieses neurokutanen Syndroms erfordert eine schnelle Beachtung und eingehende Untersuchung, damit eventuelle innere Fehlbildungen schnell erkannt werden können.
Our patient is a black, female infant born by normal delivery at 39 weeks gestation, following an uneventful pregnancy. There were no perinatal problems although several small cutaneous hemangiomata were noted at birth. At 4 months, she presented with typical "infantile spasms". On clinical examination, there were several small protuberant, dusky purpIe hemangiomata on the skin (5-10 mm in diameter) (Fig. 1), and on the buccal mucouS membrane. Developmental parameters were normal and there were no abnormal findings on neurologieal, ophthalmological or abdominal examination. Typical "infantile spasms" were observed in hospital. Basic haematological and biochemical investigations were normal and metabolie screening was negative. Electroencephalography showed the features of hypsarrhythmia. CT head scan, abdominal ultrasound, and Magnetic Resonance (MR) Imaging of the head and abdomen suggested the presence of vascular malformations in the brain and liver (Figs. 2, 3). Histological examination confirmed the hemangiomatous nature of the skin lesions, and all the vessels labelled strongly with monoclonal antibodies to vascular membrane antigens. The seizures responded rapidly to initial treatment with ACTH and clonazepam. ACTH was discontinued at 5 months and seizures recurred at 7 months. Increasing doses of
Introduction Neonatal hemangiomatosis is a rare condition characterised by the presence of multiple button-shaped, purpIe, hemangiomata present on the skin from birth, with involvement of internaIorgans. We describe the previously unreported association of neonatal hemangiomatosis and infantile spasms in a 4-month-old female. When vital organs are involved, the condition can be lethaI, and should be distinguished from the more common "strawberry naevi". The high mortality and morbidity of this neurocutaneous syndrome necessitates prompt recognition and investigation, to enable identification of associated internalIesions.
Received January 22, 1990; aeeepted February 16, 1990 Neuropediatries21 (1990) 211-212 © Hippokrates Verlag Stuttgart
Fig. 1 A typical cutaneous hemangioma.
Downloaded by: National University of Singapore. Copyrighted material.
Abstract
M. A. McShane et al
Neuropediatrics 21 (1990)
Discussion The cutaneous hemangiomata in this case were inconspicuous and involvement of internaiorgans would not have been suspected from routine clinical examination. The development of infantile spasms led to the recognition of the disorder and radiological investigation confirmed the presence of internallesions.
Fig. 2 CT scan showed a calcified lesion in the right tempero-parietal region, which enhanced with intravenous contrast (arrowed).
Neonatal hemangiomatosis is a rare disorder and is characterised by multiple hemangiomata usually involving three body organs. The skin lesions have a characteristic appearance and are typically observed at birth. They tend to be smalI, button-like, raised purpie lesions which are usually multiple. These features distinguish this condition from the more common strawberry naevi (1). Internaliesions can be large and critically placed. Mortality can be as high as 56 %, due to complications such as congestive cardiac failure, haemorrhage, and pressure effects on vital organs (2). The presence of multiple cutaneous hemangiomata in the newborn should stimulate a search for visceraliesions. Computerised tomography, ultrasound and now, MR, give useful diagnostic information without heing invasive. MR offers an excellent method of monitoring internallesions. Prednisolone in doses of 2-4 mg per kg per day, has been recommended for infants with evidence of visceral involvement and high output cardiac failure. The mechanism of action of corticosteroids in the treatment of hemangiomas is not known. In our case there is clear evidence of involution of internaliesions which may be related to corticosteroid therapy, although spontaneous change may occur.
Acknowledgments The authors wish to express their thanks to Dr.
R. Cerio and Professor E. Wilson-]ones, St. John's Hospital for Diseases of the Skin, for performing the immunohistochemical examination. MAH-C is a Research Fellow supported by Action Research for the Crippled Child.
Fig. 3 Coronal short TRjshort TE spin-echo sequence (TR = 500msec, TE = 15msec), through the upper abdomen. A weil defined low intensity, vascular malformation (arrowed) is shown in the lett lobe of the liver.
clonazepam, and then carbamazepine, proved ineffective. At 9 months, high-dose prednisolone (3 mg/kg/day) produced rapid seizure control and other anticonvulsant treatment was withdrawn. The patient remained seizure free and the steroid dosage was gradually reduced, then stopped at 13 months. Repeat MR scan of the brain and abdomen showed a reduction in the size (approximately 25 %) of the hemangiomatous lesions. Repeat EEG was normal. At 24 months she remains seizure free and development is appropriate for age.
References Golitz, L. E., et al: Diffuse neonatal hemangiomatosis. Pediatr. Dermato!. 3 (1986) 145 2 ]acobs, A. H.: Vascular Malformations. In: Schachner, L. A., Hansen, R. C. (Eds.) Pediatric Dermatology. London - New York, Churchill Livingstone (1988) 1017-1054 1
Dr. M. A. McShane
Department of Neurology Hospital for Siek Children Great Ormond Street London WCl, U.K.
Downloaded by: National University of Singapore. Copyrighted material.
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Neonatal Hemangiomatosis Presenting as Infantile Spasms By M. A. McShane 1,]. P. Finn2, M. A. Hall-Craggs 2, O. Hanmer3 and]. Harper4 1Department of Neurology, 2NMR Unit, Department of Radiology and 4Department of Dermatology, Hospital for Siek Children, Great Ormond Street, London WC1, and 3Department of Paediatries, Newham General Hospital, London E13, U.K.
Case report
The previously unreported association of neonatal hemangiomatosis and infantile spasms is described in a 4-month-old female. The high mortality and morbidity of this neurocutaneous syndrome necessitates prompt recognition and investigation, to enable identification of associated internalIesions.
Keywords Neonatal hemangiomatosis spasms - Magnetic resonance imaging
-
Infantile
Zusammenfassung Eine bisher unveröffentlichte Assoziation von neonataler Hämangiomatose und Blick-Nick-SalaamKrämpfen wird an einem 4 Monate alten Baby beschrieben. Die hohe Sterblichkeit und Morbidität dieses neurokutanen Syndroms erfordert eine schnelle Beachtung und eingehende Untersuchung, damit eventuelle innere Fehlbildungen schnell erkannt werden können.
Our patient is a black, female infant born by normal delivery at 39 weeks gestation, following an uneventful pregnancy. There were no perinatal problems although several small cutaneous hemangiomata were noted at birth. At 4 months, she presented with typical "infantile spasms". On clinical examination, there were several small protuberant, dusky purpIe hemangiomata on the skin (5-10 mm in diameter) (Fig. 1), and on the buccal mucouS membrane. Developmental parameters were normal and there were no abnormal findings on neurologieal, ophthalmological or abdominal examination. Typical "infantile spasms" were observed in hospital. Basic haematological and biochemical investigations were normal and metabolie screening was negative. Electroencephalography showed the features of hypsarrhythmia. CT head scan, abdominal ultrasound, and Magnetic Resonance (MR) Imaging of the head and abdomen suggested the presence of vascular malformations in the brain and liver (Figs. 2, 3). Histological examination confirmed the hemangiomatous nature of the skin lesions, and all the vessels labelled strongly with monoclonal antibodies to vascular membrane antigens. The seizures responded rapidly to initial treatment with ACTH and clonazepam. ACTH was discontinued at 5 months and seizures recurred at 7 months. Increasing doses of
Introduction Neonatal hemangiomatosis is a rare condition characterised by the presence of multiple button-shaped, purpIe, hemangiomata present on the skin from birth, with involvement of internaIorgans. We describe the previously unreported association of neonatal hemangiomatosis and infantile spasms in a 4-month-old female. When vital organs are involved, the condition can be lethaI, and should be distinguished from the more common "strawberry naevi". The high mortality and morbidity of this neurocutaneous syndrome necessitates prompt recognition and investigation, to enable identification of associated internalIesions.
Received January 22, 1990; aeeepted February 16, 1990 Neuropediatries21 (1990) 211-212 © Hippokrates Verlag Stuttgart
Fig. 1 A typical cutaneous hemangioma.
Downloaded by: National University of Singapore. Copyrighted material.
Abstract
M. A. McShane et al
Neuropediatrics 21 (1990)
Discussion The cutaneous hemangiomata in this case were inconspicuous and involvement of internaiorgans would not have been suspected from routine clinical examination. The development of infantile spasms led to the recognition of the disorder and radiological investigation confirmed the presence of internallesions.
Fig. 2 CT scan showed a calcified lesion in the right tempero-parietal region, which enhanced with intravenous contrast (arrowed).
Neonatal hemangiomatosis is a rare disorder and is characterised by multiple hemangiomata usually involving three body organs. The skin lesions have a characteristic appearance and are typically observed at birth. They tend to be smalI, button-like, raised purpie lesions which are usually multiple. These features distinguish this condition from the more common strawberry naevi (1). Internaliesions can be large and critically placed. Mortality can be as high as 56 %, due to complications such as congestive cardiac failure, haemorrhage, and pressure effects on vital organs (2). The presence of multiple cutaneous hemangiomata in the newborn should stimulate a search for visceraliesions. Computerised tomography, ultrasound and now, MR, give useful diagnostic information without heing invasive. MR offers an excellent method of monitoring internallesions. Prednisolone in doses of 2-4 mg per kg per day, has been recommended for infants with evidence of visceral involvement and high output cardiac failure. The mechanism of action of corticosteroids in the treatment of hemangiomas is not known. In our case there is clear evidence of involution of internaliesions which may be related to corticosteroid therapy, although spontaneous change may occur.
Acknowledgments The authors wish to express their thanks to Dr.
R. Cerio and Professor E. Wilson-]ones, St. John's Hospital for Diseases of the Skin, for performing the immunohistochemical examination. MAH-C is a Research Fellow supported by Action Research for the Crippled Child.
Fig. 3 Coronal short TRjshort TE spin-echo sequence (TR = 500msec, TE = 15msec), through the upper abdomen. A weil defined low intensity, vascular malformation (arrowed) is shown in the lett lobe of the liver.
clonazepam, and then carbamazepine, proved ineffective. At 9 months, high-dose prednisolone (3 mg/kg/day) produced rapid seizure control and other anticonvulsant treatment was withdrawn. The patient remained seizure free and the steroid dosage was gradually reduced, then stopped at 13 months. Repeat MR scan of the brain and abdomen showed a reduction in the size (approximately 25 %) of the hemangiomatous lesions. Repeat EEG was normal. At 24 months she remains seizure free and development is appropriate for age.
References Golitz, L. E., et al: Diffuse neonatal hemangiomatosis. Pediatr. Dermato!. 3 (1986) 145 2 ]acobs, A. H.: Vascular Malformations. In: Schachner, L. A., Hansen, R. C. (Eds.) Pediatric Dermatology. London - New York, Churchill Livingstone (1988) 1017-1054 1
Dr. M. A. McShane
Department of Neurology Hospital for Siek Children Great Ormond Street London WCl, U.K.
Downloaded by: National University of Singapore. Copyrighted material.
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