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

Victoria Fröjd; Giovanni Maltese; Lars Kölby; Peter Tarnow

doi:10.1055/s-0034-1387195

Journal of Neurological Surgery Reports, Table of Contents

J Neurol Surg Rep 2014; 75(02): e220-e223
DOI: 10.1055/s-0034-1387195

Case Report

Georg Thieme Verlag KG Stuttgart · New York

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

Victoria Fröjd

¹The Craniofacial Unit, Department of Plastic and Reconstructive Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg, Sweden

,

Giovanni Maltese

¹The Craniofacial Unit, Department of Plastic and Reconstructive Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg, Sweden

,

Lars Kölby

¹The Craniofacial Unit, Department of Plastic and Reconstructive Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg, Sweden

,

Peter Tarnow

¹The Craniofacial Unit, Department of Plastic and Reconstructive Surgery, Institute of Clinical Sciences, Sahlgrenska Academy at the University of Gothenburg, Sahlgrenska University Hospital, Gothenburg, Sweden

› Author Affiliations

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.

Keywords

scalp defect - cranial defect - aplasia cutis congenita

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References

References
1 Rodríguez-García C, González-Hernández S, Hernández-Martín A, Pérez-Robayna N, Sánchez R, Torrelo A. Aplasia cutis congenita and other anomalies associated with methimazole exposure during pregnancy. Pediatr Dermatol 2011; 28 (6) 743-745
2 Snape KM, Ruddy D, Zenker M , et al. The spectra of clinical phenotypes in aplasia cutis congenita and terminal transverse limb defects. Am J Med Genet A 2009; 149A (8) 1860-1881
3 Hassed SJ, Wiley GB, Wang S , et al. RBPJ mutations identified in two families affected by Adams-Oliver syndrome. Am J Hum Genet 2012; 91 (2) 391-395
4 Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986; 14 (4) 646-660
5 Sybert VP. Aplasia cutis congenita: a report of 12 new families and review of the literature. Pediatr Dermatol 1985; 3 (1) 1-14
6 Demmel U. Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. Eur J Pediatr 1975; 121 (1) 21-50
7 Lahiri A, Nishikawa H. A nonadherent dressing for aplasia cutis congenita. J Plast Reconstr Aesthet Surg 2006; 59 (7) 781-782
8 Canter HI. Comment on “An adherent dressing for aplasia cutis congenita”. J Plast Reconstr Aesthet Surg 2006; 59 (12) 1469-1470
9 Azad S, Falder S, Harrison J, Graham K. An adherent dressing for aplasia cutis congenita. Br J Plast Surg 2005; 58 (8) 1159-1161
10 Başterzi Y, Bağdatoğlu C, Sari A, Demirkan F. Aplasia cutis congenita of the scalp and calvarium: conservative wound management with novel wound dressing materials. J Craniofac Surg 2007; 18 (2) 427-429
11 Saraiya HA. Management of aplasia cutis congenita of the scalp: a continuing enigma. Br J Plast Surg 2002; 55 (8) 707-708
12 Wexler A, Harris M, Lesavoy M. Conservative treatment of cutis aplasia. Plast Reconstr Surg 1990; 86 (6) 1066-1071
13 Bharti G, Groves L, David LR, Sanger C, Argenta LC. Aplasia cutis congenita: clinical management of a rare congenital anomaly. J Craniofac Surg 2011; 22 (1) 159-165
14 South DA, Jacobs AH. Cutis marmorata telangiectatica congenita (congenital generalized phlebectasia). J Pediatr 1978; 93 (6) 944-949
15 Ross DA, Laurie SW, Coombs CJ, Mutimer KL. Aplasia cutis congenita: failed conservative treatment. Plast Reconstr Surg 1995; 95 (1) 124-129
16 Tröbs RB, Barenberg K, Hemminghaus M, Günther M, Neid M. Herniation of the brain after conservative treatment of a large congenital skull defect in an infant with Adams-Oliver syndrome. J Pediatr Surg 2010; 45 (10) 2064-2067
17 Yang JY, Yang WG. Large scalp and skull defect in aplasia cutis congenita. Br J Plast Surg 2000; 53 (7) 619-622
18 Maillet-Declerck M, Vinchon M, Guerreschi P , et al. Aplasia cutis congenita: review of 29 cases and proposal of a therapeutic strategy. Eur J Pediatr Surg 2012;
19 Koshy CE, Waterhouse N, Peterson D. Large scalp and skull defects in aplasia cutis congenita. Br J Plast Surg 2001; 54 (3) 276-277
20 Harvey G, Solanki NS, Anderson PJ, Carney B, Snell BJ. Management of aplasia cutis congenita of the scalp. J Craniofac Surg 2012; 23 (6) 1662-1664
21 Canter HI, Vargel I, Nasir S, Kayikcioglu A. Use of a water-vapour permeable polyurethane film (omiderm) in the non-surgical treatment of aplasia cutis congenita. Scand J Plast Reconstr Surg Hand Surg 2004; 38 (4) 232-235
22 Santos de Oliveira R, Barros Jucá CE, Lopes Lins-Neto A, Aparecida do Carmo Rego M, Farina J, Machado HR. Aplasia cutis congenita of the scalp: is there a better treatment strategy?. Childs Nerv Syst 2006; 22 (9) 1072-1079
23 Bernbeck B, Schwabe J, Groninger A , et al. Aplasia cutis congenita of the scalp: how much therapy is necessary in large defects?. Acta Paediatr 2005; 94 (6) 758-760
24 Croce EJ, Purohit RC, Janovski NA. Congenital absence of skin (aplasia cutis congenita). Arch Surg 1973; 106 (5) 732-734
25 Rhee ST, Colville C, Buchman SR, Muraszko K. Complete osseous regeneration of a large skull defect in a patient with cutis aplasia: a conservative approach. J Craniofac Surg 2002; 13 (4) 497-500
26 Raposo-Amaral CE, Raposo-Amaral CA. Aplasia cutis congenita: impact of early treatment on calvarial osteogenesis. J Plast Reconstr Aesthet Surg 2011; 64 (9) e237-e240
27 Chu CY, Peng FC, Chiu YF , et al. Nanohybrids of silver particles immobilized on silicate platelet for infected wound healing. PLoS One 2012; 7 (6) e38360
28 Alimov V, Lovecchio F, Sinha M, Foster KN, Drachman D. Use of a silver-containing hydrofiber dressing for filling abscess cavity following incision and drainage in the emergency department: a randomized controlled trial. Adv Skin Wound Care 2013; 26 (1) 20-25