Aplasia cutis congenita: clinical management and a new classification system.

Abstract

BACKGROUND:

Aplasia cutis congenita is a rare, congenital disorder. In its severe phenotype, it is potentially life threatening. Its management and the timing of surgery remain controversial because of the risks involved with both conservative and surgical approaches. Most literature is based on case reports and very small case series because of the rarity of the disorder. The authors present their experience treating newborns with aplasia cutis congenita and its progressive development.

METHODS:

Using a hospital registry, the authors found all cases of newborns diagnosed with aplasia cutis congenita during the years 2000 to 2013. Clinical data were gathered from hospital and clinic records, and photographs were obtained by the plastic surgery team.

RESULTS:

Twenty-two cases of aplasia cutis congenita were included in this study: 21 on the scalp and one on the foot heel. Eleven patients were male and 11 were female. Defect size ranged from 1 to 150 cm (average, 29 cm). Three patients died as a result of uncontrollable hemorrhage. Six patients underwent emergency coverage, one with allografts later replaced by split-thickness skin grafts and five by immediate split-thickness skin grafting. All of the patients who underwent immediate skin grafting survived and thrived.

CONCLUSIONS:

The authors emphasize the role of emergency split-thickness skin grafting in the treatment of large aplasia cutis congenita or ones with large veins or sagittal sinus exposure. The authors also present a practical, treatment-oriented classification that could assist physicians in estimating the severity and therefore prognosis of the disease and offer a treatment guideline.

CLINICAL QUESTION/LEVEL OF EVIDENCE:

Therapeutic, IV.

• Authors: Eldad Silberstein, Vasileios A Pagkalos, Daniella Landau, Alexander Bogdanov Berezovsky, Yuval Krieger, Yaron Shoham, Avraham Levy, Lior Rosenberg, Tali Silberstein
• Journal: Plastic and reconstructive surgery (Plast Reconstr Surg) Vol. 134 Issue 5 Pg. 766e-774e (Nov 2014) ISSN: 1529-4242 [Electronic] United States
• PMID: 25347652 (Publication Type: Journal Article)

Topics

• Ectodermal Dysplasia (classification, epidemiology, surgery)
• Female
• Follow-Up Studies
• Graft Rejection
• Graft Survival
• Heel (physiopathology, surgery)
• Humans
• Infant, Newborn
• Intensive Care Units, Neonatal
• Israel
• Male
• Occlusive Dressings
• Postoperative Complications (epidemiology, physiopathology)
• Rare Diseases
• Registries
• Retrospective Studies
• Risk Assessment
• Scalp (physiopathology, surgery)
• Severity of Illness Index
• Skin Transplantation (adverse effects, methods)
• Survival Rate
• Treatment Outcome
• Wound Healing (physiology)