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Case Studies | Pediatrics | India | Volume 11 Issue 12, December 2022 | Popularity: 4.8 / 10

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Aplasia Cutis Congenita: A Case Report

Pawan Nimbhorkar, Nikhilesh Kopparapu, Jayvardhan Lade, Rakesh Thamke, Vijay Kamale

Abstract: Aplasia Cuties Congenita (ACC) is a rare, heterogeneous group of congenital disorders, characterized by localized or widespread, complete or partial, absence of skin at birth mostly involving scalp. Incidence is about 3 in 10, 000 births and 500 cases have been reported. Causes include Intra-uterine infections, teratogenic drugs (e.g. methimazole), intra-uterine trauma, vascular compromise, genetic mutations. ACC is clinical diagnosis. USG (skull), MRI (Brain) and bone survey helps to rule out associated malformations. Case: We report a case of full-term newborn female baby, born by non-consanguineous marriage, delivered by Lower-segment Caesarean section (done in view of non-progression of labor and severe pre-eclampsia) with skin defect of 4?5 cms in size on occipital region (Vertex) since birth. No history of intra-uterine or birth trauma, skin infection/ fever/ teratogenic drugs during ANC period. No history of scalp defects/ ACC in family. On head-to-toe examination, 4?5 cms sized, Single, Oval shaped skin defect was present only over vertex of scalp, involving superficial skin layers, with no signs of local infection/ external hemorrhage atsite of defect. USG (Skull) and bone survey were normal. Diagnosis of ACC-I (ACC without associated anomalies) was made. Baby was managed Conservatively with topical antibiotics, skin care and follow up was taken. Conclusion: Aplasia Cutes Congenita: Type I (ACC-I) is a rare condition involving scalp without multiple anomalies. It is Clinical Diagnosis. Prognosis and Management of ACC depends on size, location and severity of defect, underlying cause and associated anomalies. Superficial lesions are treated conservatively, while large defects or deeper lesions involving large bone area require surgical intervention.

Keywords: Aplasia Cuties Congenita, Congenital disorder, Teratogenic drugs, Intra-uterine infections, Intra-uterine trauma

Edition: Volume 11 Issue 12, December 2022

Pages: 897 - 900

DOI: https://www.doi.org/10.21275/MR221220201211

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