Congenital Absence of Skin

Farmer, A. W.; Maxmen, M D

doi:10.1097/00006534-196004000-00001

Cited by 73 publications

(27 citation statements)

References 0 publications

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“…The dura in patients with composite type aplasia cutis congenita can appear completely normal but is often significantly attenuated, often appearing to be only arachnoid, and the exposed dura often contains large tortuous veins [40]. The exposed dura must be kept continuously moist until securely covered with viable tissue.…”

Section: Management Of the Duramentioning

confidence: 99%

“…Large defects almost always straddle the superior sagittal sinus [40]. Surgical techniques for scalp closure include rotational scalp flaps, split-thickness skin grafts [24], and latissimus dorsi muscle flaps [25].…”

Section: Management Of Scalp Defectsmentioning

confidence: 99%

“…Rotation or transposition of full-thickness scalp flaps provides immediate secure closure, avoids exsanguination and subsequent eschar formation, and greatly reduces the risk of secondary infection [15,20,40]. Two flaps are usually required to achieve complete coverage, but occasionally only one is needed [11].…”

Section: Management Of Scalp Defectsmentioning

confidence: 99%

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Aplasia Cutis Congenita of the Scalp, Composite Type: The Criticality and Inseparability of Neurosurgical and Plastic Surgical Management

Winston

Ketch²

2016

Pediatr Neurosurg

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Background/Aims: The object of this report is to review the management of patients having the composite type of aplasia cutis congenita in the context of the relevant literature on this condition. Methods: Clinical records, neuroimaging and photographic documentation of identified cases of composite type aplasia cutis congenita, with a comprehensive review of the literature, are the material basis of this report. Results: Two neonates with composite type aplasia cutis congenita are described as examples of this disorder, and their management, including complications, is discussed. Both had satisfactory outcomes. Conclusions: Early aggressive surgical management with scalp rotation flaps has a low rate of complications, provides satisfactory functional and esthetic outcome, minimizes hospital stays, and should provide cost-effective care by reduction of the time to secure wound closure. Most bony defects, even large ones, commonly ossify completely. Optimal case management requires a synchronized neurosurgical and plastic surgical team. Intuitive nonsurgical and surgical approaches by the inexperienced can be hazardous.

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Section: Management Of the Duramentioning

confidence: 99%

Section: Management Of Scalp Defectsmentioning

confidence: 99%

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Aplasia Cutis Congenita of the Scalp, Composite Type: The Criticality and Inseparability of Neurosurgical and Plastic Surgical Management

Winston

Ketch²

2016

Pediatr Neurosurg

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“…Among the intrauterine factors, amniotic adhesions and bands have been implicated as the most likely cause. 2,3 The scalp variety of ectopia cutis congenita is thought to be an autosomal dominant gene defect, [4][5][6] but recent evidence suggests primary failure of differentiation. At birth, a history of obstetrical trauma or forceps delivery must be ruled out before labelling this condition.…”

mentioning

confidence: 99%

Aplasia Cutis Congenita: A Report of Five Cases in Riyadh

et al. 1998

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“…The first patient was reported by C ampbell [2] in 1826. Most of the reported cases showed lesions which were small and generally limited to the scalp [1][2][3][4][5][6][7][8]. Bart [9,10] reported instance with extensive lesions in association with Epidermolysis bullosa dystrophica, and suggested a relationship between these two diseases.…”

mentioning

confidence: 99%