Aplasia Cutis Congenita

Bharti, Gaurav; Groves, Leslie; David, Lisa R.; Sanger, Claire; Argenta, Louis C.

doi:10.1097/scs.0b013e3181f73937

Cited by 56 publications

(31 citation statements)

References 27 publications

(98 reference statements)

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“…The scalp is the most frequent site of involvement, although trunk and extremities may also be involved [ 6 ]. ACC may be unilateral or less frequently bilateral.…”

Section: Discussionmentioning

confidence: 99%

Type VI Aplasia Cutis Congenita: Bart’s Syndrome

Kulalı

Baş

Kale

et al. 2015

Case Reports in Dermatological Medicine

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Bart's syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis. The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow-up examinations showed complete healing. We emphasized that it is important to use relatively simple methods for optimal healing without the need for complex surgical interventions.

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“…The scalp is the most frequent site of involvement, although trunk and extremities may also be involved [ 6 ]. ACC may be unilateral or less frequently bilateral.…”

Section: Discussionmentioning

confidence: 99%

Type VI Aplasia Cutis Congenita: Bart’s Syndrome

Kulalı

Baş

Kale

et al. 2015

Case Reports in Dermatological Medicine

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“…Due to often poor and anomalous circulation of the scalp surrounding the defect, wound breakdown or flap necrosis, with the potential for associated infection, is significant and can be life-threatening in a neonate [13,19,38]. Neonatal scalp flaps are fragile, and their perfusion may be impaired before surgery due to abnormal vasculature [46], which is common in patients with Adams-Oliver syndrome due to the microvascular abnormalities [19,46,47,48].…”

Section: Management Of Scalp Defectsmentioning

confidence: 99%

“…The safest plan depends on the patient's condition and associated diagnoses but usually includes surgical intervention, preferably within 24 h, unless the anesthetic or surgical risks are prohibitive. Nonsurgical management can be considered for small lesions with intact dura, particularly if the bony defect is small and does not overlie the superior sinus [38], but even these are best managed surgically.…”

Section: Risksmentioning

confidence: 99%

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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“…Friedan has classified 9 congenital cutaneous aplasia according to its location, its physiopathology, its association with other syndromes. The reported incidence of aplasia congenita varies from one in 10,000 live births to 2.8 in 10,000 newborns, with a pre-disposition for female patients [3]. The etiology of the ACC remains uncertain, but the most likely hypotheses are an anormaly either of closure of the neural tube or of the prenatal vascular development [4].…”

mentioning

confidence: 99%

“…The lesion is non-inflammatory, well dermarcated, and varying in size. The defects can rarely present as ulcers, which appear stamped out and have rotund stellate or elongated forms [3]. Histological features vary depending on the depth and duration of aplasia.…”

mentioning

confidence: 99%