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Conservative Healing of an 11 × 9-cm Aplasia Cutis Congenita of the Scalp with Bone Defect.

ABSTRACT: Objectives Aplasia cutis congenita is a rare congenital condition, and it is difficult to find scientific support for optimal treatment strategies. In addition, these may vary due to defect size, tissue layers involved, contemporary malformations, and the physiologic status of the affected child. Clinical Presentation This case report describes complete skin coverage in 20 weeks and uneventful healing of a large 11 × 9-cm defect of the vertex, involving both skin and skull bone, using conservative treatment. To prevent infection and promote healing, the defect was kept moist and covered at all times, and it was treated with surgical debridement when necessary. For infection control, ionized silver-coated dressings were used in addition to prophylactic antibiotics over the first 3.5 weeks. Follow-up was 2 years. Conclusion Surgical treatment is usually preferred for larger aplasia cutis congenita defects, but it is accompanied with potential risks and will exacerbate secondary reconstruction of alopecia or skull bone defects. This case shows that even very complex defects may be treated conservatively.

SUBMITTER: Frojd V

PROVIDER: S-EPMC4242817 | biostudies-other | 2014 Dec

REPOSITORIES: biostudies-other

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Project description:BackgroundAplasia cutis congenita (ACC), classified in nine groups, is likely to be underreported, since milder isolated lesions in wellbeing newborns could often be undetected, and solitary lesions in the context of polymalformative syndromes could not always be reported. Regardless of form and cause, therapeutic options have in common the aim to restore the deficient mechanical and immunological cutaneous protection and to limit the risk of fluid leakage or rupture of the exposed organs. We aimed to review our institutional prevalence, comorbidities, treatment and outcome of newborns with ACC.MethodsWe conducted a retrospective study including all newborns affected by ACC and admitted at the University Mother-Child Department from October 2010 to October 2019. Anthropometric and clinical characteristics of ACC1 versus a non-isolated ACC group were analyzed.ResultsWe encountered 37 newborns, 16 with ACC1 versus 21 with non-isolated ACC. The incidence rate of 0.1% in ACC1 was higher than expected, while 19% of cases showed intrafamilial autosomal dominant transmission. Higher birth weight centile, though lower than reference population, being adequate for gestational age, normal Apgar score and euglycemia characterizing ACC1 resulted associated to a rapid tissue regeneration. Non-isolated ACC, in relation to concomitant congenital anomalies and higher prematurity rate, showed more surgical and medical complications along with the risk of neonatal death. Specifically, newborns with ACC4 were characterized by the frequent necessity of abdominal wall defect repair, responsible for the occurrence of an abdominal compartment syndrome.ConclusionPrompt carefully assessment of the newborn with ACC in order to exclude concomitant other congenital malformations, provides clues to the underlying pathophysiology, and to the short-term prognosis. Family should be oriented toward identification of other family members affected by similar pathology, while obstetric history should exclude initial multiple pregnancy with death of a co-twin, placental anomalies and drug assumption. Molecular-genetic diagnosis and genetic counseling are integrative in individualized disease approach.

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Conservative Healing of an 11 × 9-cm Aplasia Cutis Congenita of the Scalp with Bone Defect.

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