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Surgical Management of Aplasia Cutis Congenita

Surgical Management of Aplasia Cutis Congenita Abstract • A child born without scalp, skull, or dura to cover the brain (aplasia cutis congenita) was successfully treated by a multidiscipline team. A coexisting ruptured omphalocele forced a change in treatment from the currently recommended regimen of mandatory early scalp closure. Homograft skin was used to protect the brain during the time the omphalocele was treated and skin flaps were delayed. Fluoroscein dye was utilized to determine flap viability and predicted ischemia until after a third delaying procedure was performed. The successful outcome suggests that the present philosophy of early surgical closure being essential for survival in infants with large cranial defects can be altered and, in fact, permanent full-thickness flaps may be designed, tested for viability, and delayed while homograft skin protects the infant's brain from infection and thrombosis. (Arch Surg 111:1160-1164, 1976) References 1. Campbell W: Case of congenital ulcer on the cranium of a fetus . Edinb J Med Sci 2:82, 1826. 2. Matson DD: Congenital defects of the scalp and skull , in Neurosurgery of Infancy and Childhood , ed 2. Springfield, Ill, Charles C Thomas Publisher, 1957. 3. Croce EJ, Purohit RC, Janovski NA: Congenital absence of skin (aplasia cutis congenita) . Arch Surg 106:732-734, 1973.Crossref 4. Farmer AW, Maxmen M: Congenital absence of skin . Plast Reconstr Surg 25:291-297, 1960.Crossref 5. Greig DM: Localized congenital defects of the scalp . Edinb Med J 38:341, 1931. 6. Hodgman JE, Mathres AW, Levan NE: Congenital scalp defects in twin sisters . Am J Dis Child 110:293, 1965. 7. Johnsonbaugh RE, Light IJ, Sutherland JM: Congenital scalp defects in father and son . Am J Dis Child 110:297-298, 1965. 8. Kahn EA, Olmedo L: Congenital defects of the scalp . Plast Reconstr Surg 6:435-440, 1950.Crossref 9. Rauschkolb RR, Enriques SI: Aplasia cutis congenita . Arch Dermatol 86:54-57, 1962.Crossref 10. Savage D: Localized congenital defects of the scalp . J Obstet Gynaecol Br Commonw 63:351, 1956.Crossref 11. Abt IA: Congenital skin defects . Am J Dis Child 14:113, 1917. 12. Anderson NP, Nou FG: Congenital defect of the scalp . Arch Dermatol Syph 46:257-263, 1947.Crossref 13. Ingalls NW: Congenital defects of the scalp: Studies in the pathology and development, III . Am J Obstet Gynecol 25:861-873, 1933. 14. Peer LA, Van Duyn J: Congenital defects of the scalp . Plast Reconstr Surg 3:722-726, 1948.Crossref 15. Moller KO: Congenital defects of the cranium caused by amniotic adhesions . Acta Gynecol Scand 2:144-157, 1923.Crossref 16. Kosnik EJ, Sayers MP: Congenital scalp defects: Aplasia cutis congenita . J Neurosurg 42:32, 1975.Crossref 17. O'Brien BM, Drake JE: Congenital defects of the skull and scalp . Br J Plast Surg 13:102, 1960.Crossref 18. Pap GS: Congenital defect of scalp and skull in three generations of one family: Case report . Plast Reconstr Surg 46:194-196, 1970.Crossref 19. Dowler VB: Congenital defect of the skin in a newborn infant . Am J Dis Child 44:1279, 1932. 20. Lynch PJ, Kahn EA: Congenital defects of the scalp: A surgical approach to aplasia cutis congenita . J Neurosurg 33:198-202, 1970.Crossref 21. Callaway JL, Noojin RO, Riley KA, et al: Congenital ectodermal defect: Report of an unusual case involving scalp and leg . J Pediatr 28:214-216, 1946.Crossref 22. Hunn J, Huygens MCL, Hugo H, et al: Hereditary scalp and cranial defects . Med J Aust 1:219-223, 1969. 23. Walker JC, Koemig JA, Irvin L, et al: Congenital absence of skin . Plast Reconstr Surg 26:209-217, 1960.Crossref 24. Montgomery H: Congenital aplasia , in Dermatopathology . New York, Harper & Row Publishers Inc, 1967, vol 1, p 75. 25. Freud P, Rhodes AW, Weisz A: A hereditary skin defect in the newborn infant . J Pediatr 27:591, 1945.Crossref 26. Conway H, Johnson G Jr: Congenital absence of the scalp and skull . Ann Surg 144:1035-1044, 1956.Crossref 27. Converse JM: Surgical closure of scalp defects , in Kahn EA, Crosley EC, Schneider RC, et al (eds): Correlative Neurosurgery , ed 2. Springfield, Ill, Charles C Thomas Publisher, 1969, p 599. 28. Thorvaldsson SE, Grabb WC: The intravenous fluorescein test as a measure of skin flap viability . Plast Reconstr Surg 53:576-578, 1974.Crossref 29. Myers MB: Prediction of skin sloughs at the time of operation with the use of fluorescein dye. Surgery 51:158-162, 1962. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Surgery American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 1976 American Medical Association. All Rights Reserved.
ISSN
0004-0010
eISSN
1538-3644
DOI
10.1001/archsurg.1976.01360280118019
Publisher site
See Article on Publisher Site

Abstract

Abstract • A child born without scalp, skull, or dura to cover the brain (aplasia cutis congenita) was successfully treated by a multidiscipline team. A coexisting ruptured omphalocele forced a change in treatment from the currently recommended regimen of mandatory early scalp closure. Homograft skin was used to protect the brain during the time the omphalocele was treated and skin flaps were delayed. Fluoroscein dye was utilized to determine flap viability and predicted ischemia until after a third delaying procedure was performed. The successful outcome suggests that the present philosophy of early surgical closure being essential for survival in infants with large cranial defects can be altered and, in fact, permanent full-thickness flaps may be designed, tested for viability, and delayed while homograft skin protects the infant's brain from infection and thrombosis. (Arch Surg 111:1160-1164, 1976) References 1. Campbell W: Case of congenital ulcer on the cranium of a fetus . Edinb J Med Sci 2:82, 1826. 2. Matson DD: Congenital defects of the scalp and skull , in Neurosurgery of Infancy and Childhood , ed 2. Springfield, Ill, Charles C Thomas Publisher, 1957. 3. Croce EJ, Purohit RC, Janovski NA: Congenital absence of skin (aplasia cutis congenita) . Arch Surg 106:732-734, 1973.Crossref 4. Farmer AW, Maxmen M: Congenital absence of skin . Plast Reconstr Surg 25:291-297, 1960.Crossref 5. Greig DM: Localized congenital defects of the scalp . Edinb Med J 38:341, 1931. 6. Hodgman JE, Mathres AW, Levan NE: Congenital scalp defects in twin sisters . Am J Dis Child 110:293, 1965. 7. Johnsonbaugh RE, Light IJ, Sutherland JM: Congenital scalp defects in father and son . Am J Dis Child 110:297-298, 1965. 8. Kahn EA, Olmedo L: Congenital defects of the scalp . Plast Reconstr Surg 6:435-440, 1950.Crossref 9. Rauschkolb RR, Enriques SI: Aplasia cutis congenita . Arch Dermatol 86:54-57, 1962.Crossref 10. Savage D: Localized congenital defects of the scalp . J Obstet Gynaecol Br Commonw 63:351, 1956.Crossref 11. Abt IA: Congenital skin defects . Am J Dis Child 14:113, 1917. 12. Anderson NP, Nou FG: Congenital defect of the scalp . Arch Dermatol Syph 46:257-263, 1947.Crossref 13. Ingalls NW: Congenital defects of the scalp: Studies in the pathology and development, III . Am J Obstet Gynecol 25:861-873, 1933. 14. Peer LA, Van Duyn J: Congenital defects of the scalp . Plast Reconstr Surg 3:722-726, 1948.Crossref 15. Moller KO: Congenital defects of the cranium caused by amniotic adhesions . Acta Gynecol Scand 2:144-157, 1923.Crossref 16. Kosnik EJ, Sayers MP: Congenital scalp defects: Aplasia cutis congenita . J Neurosurg 42:32, 1975.Crossref 17. O'Brien BM, Drake JE: Congenital defects of the skull and scalp . Br J Plast Surg 13:102, 1960.Crossref 18. Pap GS: Congenital defect of scalp and skull in three generations of one family: Case report . Plast Reconstr Surg 46:194-196, 1970.Crossref 19. Dowler VB: Congenital defect of the skin in a newborn infant . Am J Dis Child 44:1279, 1932. 20. Lynch PJ, Kahn EA: Congenital defects of the scalp: A surgical approach to aplasia cutis congenita . J Neurosurg 33:198-202, 1970.Crossref 21. Callaway JL, Noojin RO, Riley KA, et al: Congenital ectodermal defect: Report of an unusual case involving scalp and leg . J Pediatr 28:214-216, 1946.Crossref 22. Hunn J, Huygens MCL, Hugo H, et al: Hereditary scalp and cranial defects . Med J Aust 1:219-223, 1969. 23. Walker JC, Koemig JA, Irvin L, et al: Congenital absence of skin . Plast Reconstr Surg 26:209-217, 1960.Crossref 24. Montgomery H: Congenital aplasia , in Dermatopathology . New York, Harper & Row Publishers Inc, 1967, vol 1, p 75. 25. Freud P, Rhodes AW, Weisz A: A hereditary skin defect in the newborn infant . J Pediatr 27:591, 1945.Crossref 26. Conway H, Johnson G Jr: Congenital absence of the scalp and skull . Ann Surg 144:1035-1044, 1956.Crossref 27. Converse JM: Surgical closure of scalp defects , in Kahn EA, Crosley EC, Schneider RC, et al (eds): Correlative Neurosurgery , ed 2. Springfield, Ill, Charles C Thomas Publisher, 1969, p 599. 28. Thorvaldsson SE, Grabb WC: The intravenous fluorescein test as a measure of skin flap viability . Plast Reconstr Surg 53:576-578, 1974.Crossref 29. Myers MB: Prediction of skin sloughs at the time of operation with the use of fluorescein dye. Surgery 51:158-162, 1962.

Journal

Archives of SurgeryAmerican Medical Association

Published: Oct 1, 1976

References