• Journal of Yeungnam Medical Science
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• v.9 no.2
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• pp.422-426
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• 1992

One case of Congenital Cutis Aplasia is presented. The defect involved includes full-thickness skin defect of scalp and cranium. The patient was treated with debridement of dirty necrosed crust which covered exposed dura mater and with double opposing rotation flap including pericranium for bone regneration. The donor site was covered with skin graft from right thigh. During operation, the superficial temporal arterty was found to be short and weak. And after operation, the margin of flap were congested and finally necrotized. The necrotic wound was treated with conservative management. The vascular impairment is thought to be main course of Congenital Cutis Aplasia. So we conclude that the treatment of choice is conservative management or careful flap surgery for coverage of defect area.

• Journal of Yeungnam Medical Science
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• v.10 no.2
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• pp.493-505
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• 1993

The congenital cutis aplasia mainly occurs in head, sometimes involving the skull and dura mater. It's cause and the rate of falling ill are not known yet, it is the disease that rate of death is high by the infection, such as, the injured vascular hemorrhange of meningitis. Craniosynostosis is the disease the appears the skull as well as the facial deformity with growing, has from the developmental difficiency, visual distibance, motor disturbance, convulsion to the neurologic impairment of mental retardation, and accompanies the each characteristic deformity following the suture fused. Satisfactory results was achieved by local flap surgery and conservative treatment on the infant, diagnosed as the congenital cutis aplasia, case 1 Also successful treatment experiences of craniosynostosis(oxycephaly, brachycephaly, trigonocephaly, cloverleaf deformity) through the frontal bone advancement and the barrel stave asteotomy, were reviewed & pursues and investigates the intracranial volume of before and after of surgery, and then reports with the literature investigation.

• Archives of Plastic Surgery
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• v.33 no.5
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• pp.672-675
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• 2006

Purpose: The purpose of this study is to document the surgical methods used in infants with aplasia cutis congenita treated with allogenic dermal matrix and cultured epithelial autografts. Methods: The large defects in both lower legs were replaced with allogenic dermal matrix to avoid the postoperative hypertrophic scar contracture and a full-thickness skin biopsy was taken from right groin area simultaneously. We sent the specimen to a commercial laboratory for culture and obtained cultured epithelial autografts($Holoderm^{(R)}$) after 2 weeks, placed it over the allogenic dermal matrix. Results: The skin-defected area were nearly epithelialized after 2 weeks and there were no significant problem on during 6 months follow-up. Conclusion: The surgical method using allogenic dermal matrix and cultured epithelial autograft provided an excellent coverage of large skin defects of infant with aplasia cutis congenita.

• Neonatal Medicine
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• v.16 no.1
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• pp.85-88
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• 2009

The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.

• Neonatal Medicine
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• v.28 no.1
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• pp.48-52
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• 2021

Aplasia cutis congenita (ACC) is a rare congenital disorder characterized by skin defects involving the epidermis, dermis, subcutaneous tissue, bone, and sometimes dura. It commonly affects the scalp in approximately 70% of cases, but the neck, trunk, and the extremities can also be affected. ACC can occur either as an isolated condition or associated with other anomalies and congenital syndromes, and it can be acquired either genetically or sporadically. Morbidity and mortality are associated with the defects of skull bone, dura, and other multiple anomalies. We herein report the case of a female infant, with a large scalp defect accompanied by a skull defect noted at birth, who developed mental retardation in the preschool years.

• Neonatal Medicine
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• v.18 no.2
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• pp.359-364
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• 2011

Purpose: Aplasia cutis congenita (ACC) is a rare condition characterized by a localized absence of skin, and it can be associated with other congenital anomalies. This study was done to evaluate clinical course and outcome of ACC in neonates. Methods: Based on the medical records, we retrospectively reviewed 8 neonates diagnosed with ACC at Cheil General Hospital and Women's Health Care Center, Kwandong university College of Medicine from January 2004 to December 2010. We classified ACC by Frieden's classification and analyzed the patient's demographic data, clinical course and outcome. Results: Among 8 patients with ACC, 5 patients were classified to group 1; scalp ACC without anomalies and 3 patients to group 7; ACC localized to extremities without blistering. Defect size was from 0.3 cm to 1.5 cm, limited in the superficial skin or subcutaneous tissue without associated anomalies. Defects were healed under conservative treatment with mild scar formation within four months. Conclusion: ACC could be diagnosed easily through physical examination in neonates. All cases showed good clinical outcome without surgical treatment. However because of small numbers and small sized defects of cases, further study including lesions of large size is needed.

• Archives of Craniofacial Surgery
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• v.20 no.6
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• pp.408-411
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• 2019

Hairless scalp areas can occur due to trauma, tumors, or congenital disease. This aesthetically unpleasing condition can lead to psychosocial distress, and thin skin flaps may be prone to scarring. Treating the hairless scalp by simple excision is challenging because of skin tension. Tissue expanders are a good option for hairless scalp resurfacing. However, a single expansion may be inadequate to cover the entire defect. This report describes good results obtained using a serial resurfacing method involving re-expansion of the flap with a tissue expander to treat two patients with large lesions: one due to aplasia cutis congenital and another who underwent dermatofibrosarcoma protuberance resection. The results suggest that scalp resurfacing by serial tissue expansion using a tissue expander can be used for extensive lesions.

• Archives of Plastic Surgery
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• v.37 no.4
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• pp.469-472
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• 2010

Purpose: Aplasia Cutis Congenita (ACC) is a rare disease characterized by the focal defect of the skin at birth, frequently involving scalp, but it may affect any region of the body. There are no etiology known but some conditions such as intrauterine vascular ischemia, amniotic adherences and viral infections are associated. The ideal treatment for the ACC is not known. Superficial and relatively small sized defects (< $3{\times}5\;cm$) may heal spontaneously and large defects related with risks of infection and bleeding may require aggressive surgical treatment. Hyalomatrix$^{(R)}$ is a bilayer of an esterified hyaluronan scaffold beneath a silicone membrane. It has been used as a temporary dermal substitute to cover deep thickness skin defect and has physiological functions derive from the structural role in extracellular matrix and interaction with cell surface receptor. This material has been used for the wound bed pre-treatment for skin graft to follow and especially in uncooperative patient, like a newborn, this could be a efficient and aseptic way of promoting granulation without daily irritative wound care. For this reason, using Hyalomatrix$^{(R)}$ for the treatment of ACC was preferred in this paper. Methods: We report a case of a newborn with ACC of the vertex scalp and non-ossified partial skull defect. The large sized skin and skull defect ($6{\times}6\;cm$) was found with intact dura mater. No other complications such as bleeding or abnormal neurologic sign were accompanied. Escharectomy was performed and Hyalomatrix$^{(R)}$ was applied for the protection and the induction of acute wound healing for 3 months before the split-thickness skin graft. During the 3 months period, the dressing was renewed in aseptic technique for every 3 weeks. The skin graft was achieved on the healthy granulation bed. Results: The operative procedure was uneventful without necessity of blood transfusion. Postoperative physical examination revealed no additional abnormalities. Regular wound management was performed in out-patient clinic and the grafted skin was taken completely. No other problems developed during follow-up. Conclusion: Hyalomatrix$^{(R)}$ provides protective and favorable environment for wound healing. The combination of the use of Hyalomatrix$^{(R)}$ and the skin graft will be a good alternative for the ACC patients with relatively large defect on vertex.