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11 Aplasia cutis congenita of the scalp: the success of conservative approach in treatment of a large defect
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  1. Marko Bašković,
  2. Zoran Barčot,
  3. Andrija Car,
  4. Marina Jurić
  1. Children’s Hospital Zagreb

Abstract

Aplasia cutis congenita (ACC) is a rare, heterogeneous group of congenital disorders characterized by the focal or widespread absence of the skin. ACC can occur anywhere on the body; however, the vast majority of cases occur on the scalp midline. At birth, the lesions may have already healed with scarring or may remain superficially eroded to deeply ulcerated while in approximately 15%–30% of cases, the scalp defect is associated with a defect in the underlying bone and dura mater, with exposure of the brain and sagittal sinus. There is no consensus for early management and treatment modalities for large ACC cases. We present a case of a female infant at the age of 2 days (birth 40 + 3 weeks, vaginal, birth weight 3530 g, birth length 52 cm, and Apgar score 10/10) which has been moved from a maternity hospital to our institution due to aplasia cutis congenita of the scalp. She was born of the first regular pregnancy of a 29–year–old mother. At birth, in the parietal skin area above the wide–open fontanel, a 6 cm × 8 cm defect was observed without associated malformations on the rest of the body. Ultrasound of the brain and heart was neat. On the craniogram, partially parietal bone mutually was undeveloped.

Neurological status of the newborn was neat. Magnetic resonance brain made using standard techniques and T1 HIRES, and Blackbone technique was neat with sustained continuity of dura without signs of cerebral herniation.

Initially, Staphylococcus aureus was isolated, and Garamycin therapy with vaseline gas was initiated twice a day. Daily tracking of local findings was improving. One month after receipt, the eschar was gradually demarcated while the smaller nonepithelialized granulation zone treated merbromin with the baths. A protective helmet was created for the child to temporarily protect the brain and the soft tissue. Successive treatments from week to week have seen the reduction of the eschar with the closure of the bony segments. After 6 months, on the skin, a well–developed subcutaneous with the presence of capillary bleeding was observed. Within 1 month, Suprasorb A + Ag® and Suprasorb H® (Lohmann and Rauscher) wound dressings were introduced in the therapy. The iodine cream and the Mepitel® (Mölnlycke Health Care) were introduced into the therapy by removing the Suprasorb® wound dressing. Ten months since the onset of conservative treatment, the aplastic area was almost completely cured.

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