Surgical treatment of a newborn child with congenital aplasia of the scalp

Abstract

Introduction. Skin aplasia is a developmental defect represented by a skin defect of varying extent, often in combination with the defect of underlying tissues – subcutaneous fat, muscles and with various forms of dysraphy - non-closured embryonic slits. By various authors, defect frequency ranges from 1 per 1 000 to 1 per 10 000 newborns. Congenital focal aplasia of the skin and subcutaneous tissues can be located in limited areas; most often it is diagnosed on the scalp looking like single or multiple focal skin defects. Quite often, focal aplasia also affects the skin on the trunk and limbs.

Description of observation. The article presents a clinical case of congenital focal aplasia of the scalp and skull bones in a newborn child. This type of skin congenital aplasia by I. Frieden classification corresponds to Group I of congenital aplasia of the skin with lesions on the scalp. At the expert examination with multispiral computed tomography, the absence of parietal bone was diagnosed. So, the defect bottom was dura mater. In this case, the authors considered conservative approach to be unacceptable because of long period of healing and high risk of secondary infection. The performed surgery has led to a satisfactory cosmetic result. The state of bone structures was assessed catamnetically.

Conclusion. Modern diagnostic techniques have proven that an active surgical tactics in children with congenital aplasia of the scalp is justified.

Keywords

scalp aplasia / newborns / multispiral computed tomography / surgical management

References

[1] Хегер П.Г. Детская дерматология. Дифференциальная диагностика и лечение у детей и подростков. М.: Изд-во Панфилова: БИНОМ, 2013. Heger P.G. Pediatric dermatology. Differential diagnosis and treatment in children and adolescents [Detskaya dermatologiya. Differencial'naya diagnostika i lechenie u detej i podrostkov]. Moscow: Panfilov Publishing House: BINOM, 2013. (in Russian)

[2] Balsa R.E., Retruccelli M.C., Nichilo M.A. Aplasia cutis congenital. Dermatologia (Mexico). 1974; (18): 5.

[3] Черных О.О., Титова Н.Д. Врождённая аплазия кожи: практический подход к оценке и лечению редкой врождённой аномалии у новорождённого. В кн: Актуальные проблемы медицины: сборник материалов итоговой научно-практической конференции. Гродно; 2020: 769–73. Chernykh O.O., Titova N.D. Congenital aplasia of the skin: a practical approach to the assessment and treatment of a rare congenital anomaly in a newborn. In: Actual problems of medicine: a collection of materials of the final scientific and practical conference [Aktual'nye problemy mediciny: sbornik materialov itogovoj nauchno-prakticheskoj konferencii]. Grodno; 2020: 769–73. (in Russian)

[4] Скрипкина Ю.К., Бутова Ю.С. Клиническая дерматовенерология. В 2 т. Т. 2. М.: ГЭОТАР-Медиа; 2009. Skripkina Yu.K., Butov Yu.S. Clinical dermatovenerology. In 2 vols. 2 [Klinicheskaya dermatovenerologiya. V 2 t. T. 2]. Moscow: GEOTAR-Media; 2009. (in Russian)

[5] Козин В.М., Клишко С.С., Юнес И.В. и др. Очаговая аплазия кожи волосистой части головы у новорождённых монозиготных близнецов. Вестник ВГМУ. 2013; 12(2): 87–90. Kozin V.M., Klishko S.S., Yunis I.V., et al. Focal aplasia of the scalp skin in newborn monozygotic twins. Vestnik VGMU. 2013; 12(2): 87–90. (in Russian)

[6] Bharti G., Groves L., David L.R., et al. Aplasia Cutis Congenita. J. Craniofac. Surg. 2011; 22(1): 159–65.

[7] Сиротина З.В., Хомякова В.Н., Каргин В.П. Врождённая очаговая аплазия кожи у новорождённых (случай из практики). Здравоохранение Дальнего Востока. 2017; 2: 65–6. Sirotina Z.V., Khomyakova V.N., Kargin V.P. Congenital focal aplasia of the skin in newborns (a case from practice). Zdravoohranenie Dal'nego Vostoka. 2017; 2: 65–6. (in Russian)

[8] Pers M. Congenital absence of skin: pathogenesis and relation to ring-constriction. ActaChir. Scand. 1963; 126: 388–96.

[9] Студницина А.А. Дифференциальная диагностика кожных болезней (руководство для дерматологов). М.: Медицина; 1983. Studnitsina A.A. Differential diagnosis of skin diseases (a guide for dermatologists) [Differencial'naya diagnostika kozhnyh boleznej (rukovodstvo dlya dermatologov)]. Moscow: Medicine; 1983. (in Russian)

[10] Рук А., Даубер Р. Болезни волос и волосистой части головы. М.: Медицина; 1985. Ruk A., Dauber R. Diseases of the hair and scalp [Bolezni volos i volosistoj chasti golovy]. Moscow: Medicine; 1985. (in Russian)

[11] Silberstein E., Pagkalos V., Landau D. Aplasia cutis congenita: clinical management and a new classification system. Plast. Reconstr. Surg. 2014; 134(5): 766–74.

[12] Яп П., Мохамад Шах Н.С., Мат Саад А.З. Комплексная регенерация кожи головы при врожденной аплазии с использованием заменителя дермы INTEGRA. Ортопедия, травматология и восстановительная хирургия детского возраста. 2021; 9(2): 229–34. Yap P., Mohammad Shah N.S., Mat Saad A.Z. Complex regeneration of the scalp in congenital aplasia using INTEGRA derma substitute. Ortopediya, travmatologiya i vosstanovitel'naya hirurgiya detskogo vozrasta. 2021; 9(2): 229–34. (in Russian)

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Bardeeva K.A., Mishkin V.V., Pisklakov A.V., Ponomarev V.I., Pavlenko N.I.