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DOI: 10.1055/s-0042-103155
Aplasia cutis congenita
Aplasia cutis congenitaPublication History
eingereicht 21 August 2015
akzeptiert 11 February 2016
Publication Date:
22 August 2016 (online)
![](https://www.thieme-connect.de/media/hamipla/201604/lookinside/thumbnails/2015-08-0802_10-1055-s-0042-103155-1.jpg)
Zusammenfassung
Hintergrund: Beim Erkrankungsbild der Aplasia cutis congenita handelt es sich um eine seltene kongenital bedingte Anlagestörung der Haut. Diese manifestiert sich zumeist an der Kopfhaut, jedoch können auch tiefer liegende Gewebsschichten wie Knochen oder Dura mater betroffen sein. Blutungen aus dem Sinus sagittalis sowie Infektionen stellen die häufigsten Komplikationen dar. Es existieren zahlreiche Fallberichte, jedoch besteht bisher kein eindeutiger Konsens über das therapeutische Management, welches sowohl konservative als auch chirurgische Maßnahmen beinhaltet.
Methoden: Wir berichten über 3 Neugeborene mit Aplasia cutis congenita mit isolierter Hautmanifestation ohne Beteiligung tieferliegender Gewebsschichten. In allen 3 Fällen wurden die Patienten konservativ mit Fettgaze als Wundauflage behandelt und engmaschig kontrolliert.
Ergebnisse: Bei allen 3 Neugeborenen konnte innerhalb weniger Monate eine komplikationslose Abheilung sämtlicher Hautdefekte erzielt werden.
Schlussfolgerungen: Die konservative Therapie mit Fettgaze stellt eine verlässliche therapeutische Option bei isolierter Hautmanifestation dar.
Abstract
Background: Aplasia cutis congenita is a rare congenital disorder characterised by the absence of skin. It mostly affects the scalp, but may also involve deeper tissue layers such as bone or dura mater. Bleeding from the sagittal sinus and infections are the most common complications. Numerous case reports have been published, but there is no consensus on therapeutic management, which includes both conservative and surgical treatment.
Methods: We report on 3 newborns with aplasia cutis congenita solely affecting the skin without involvement of deeper tissue layers. All 3 patients were treated conservatively with fatty gauze being used as a wound dressing. The patients were monitored in tight intervals.
Results: In all 3 newborns, the skin defects healed within a few months without any complications.
Conclusion: Conservative treatment with fatty gauze represents a reliable therapeutic option in case the disease solely affects the skin.
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Literatur
- 1 Browning JC. Aplasia cutis congenita: approach to evaluation and management. Dermatologic therapy 2013; 26: 439-444
- 2 Frojd V, Maltese G, Kolby L et al. Conservative Healing of an 11×9-cm Aplasia Cutis Congenita of the Scalp with Bone Defect. Journal of neurological surgery reports 2014; 75: e220-223
- 3 Ribuffo D, Costantini M, Gullo P et al. Aplasia cutis congenita of the scalp, the skull, and the dura. Scandinavian journal of plastic and reconstructive surgery and hand surgery/Nordisk plastikkirurgisk forening [and] Nordisk klubb for handkirurgi 2003; 37: 176-180
- 4 Bharti G, Groves L, David LR et al. Aplasia cutis congenita: clinical management of a rare congenital anomaly. The Journal of craniofacial surgery 2011; 22: 159-165
- 5 Demmel U. Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. European journal of pediatrics 1975; 121: 21-50
- 6 Benjamin LT, Trowers AB, Schachner LA. Giant aplasia cutis congenita without associated anomalies. Pediatric dermatology 2004; 21: 150-153
- 7 Frieden. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986; 14: 646-660
- 8 Narang. Adams-Oliver syndrome: a sporadic occurence with minimal disease expression. Pediatric dermatology 2008; 25: 115-116
- 9 Kim CS, Tatum SA, Rodziewicz G. Scalp aplasia cutis congenita presenting with sagittal sinus hemorrhage. Archives of otolaryngology–head & neck surgery 2001; 127: 71-74
- 10 Starcevic M, Sepec MP, Zah V. A case of extensive aplasia cutis congenita: a conservative approach. Pediatric dermatology 2010; 27: 540-542
- 11 Fullana F, Gonzalez M, No M et al. Aplasia cutis congenita of the scalp in five successive generations of one family. Plastic and reconstructive surgery 1995; 95: 214-215
- 12 Aknin J, Seguin P, Brunon J et al. Skin aplasia of the vertex. Report of a familial form. Revue de stomatologie et de chirurgie maxillo-faciale 1992; 93: 267-272
- 13 Santos de Oliveira R, Barros Juca CE, Lopes Lins-Neto A et al. Aplasia cutis congenita of the scalp: is there a better treatment strategy?. Child’s nervous system: ChNS: official journal of the International Society for Pediatric Neurosurgery 2006; 22: 1072-1079
- 14 Rodriguez-Garcia C, Gonzalez-Hernandez S, Hernandez-Martin A et al. Aplasia cutis congenita and other anomalies associated with methimazole exposure during pregnancy. Pediatric dermatology 2011; 28: 743-745
- 15 Pieretti ML, Alcala R, Boggio P et al. Aplasia Cutis Congenita Associated with Fetus Papyraceus. Pediatric dermatology 2015; 32: 858-861
- 16 Ustuner P, Dilek N, Saral Y et al. Coexistence of aplasia cutis congenita, faun tail nevus and fetus papyraceus. Journal of dermatological case reports 2013; 7: 93-96
- 17 Mazza JM, Klein JF, Christopher K et al. Aplasia cutis congenita in a setting of fetus papyraceus associated with small fetal abdominal circumference and high alpha-fetoprotein and amniotic acetylcholinesterase. Pediatric dermatology 2015 32: 138-140
- 18 Louise L, Annabel M, Hubert L et al. Fetus papyraceus: congenital pulmonary anomalies associated with congenital aplasia cutis on the surviving twin. Pediatric dermatology 2013; 30: e143-e145
- 19 Seo JK, Kang JH, Lee HJ et al. A case of adams-oliver syndrome. Annals of dermatology 2010; 22: 96-98
- 20 Canter HI, Vargel I, Nasir S et al. Use of a water-vapour permeable polyurethane film (omiderm) in the non-surgical treatment of aplasia cutis congenita. Scandinavian journal of plastic and reconstructive surgery and hand surgery/Nordisk plastikkirurgisk forening [and] Nordisk klubb for handkirurgi 2004; 38: 232-235
- 21 Snape KM, Ruddy D, Zenker M et al. The spectra of clinical phenotypes in aplasia cutis congenita and terminal transverse limb defects. American journal of medical genetics Part A 2009; 149A: 1860-1881
- 22 Chitnis MR, Carachi R, Galea P. Familial aplasia cutis congenita. European journal of pediatric surgery: official journal of Austrian Association of Pediatric Surgery [et al.]=Zeitschrift fur Kinderchirurgie 1996; 6: 100-101
- 23 Glasson DW, Duncan GM. Aplasia cutis congenita of the scalp: delayed closure complicated by massive hemorrhage. Plastic and reconstructive surgery 1985; 75: 423-425
- 24 Mannino FL, Jones KL, Benirschke K. Congenital skin defects and fetus papyraceus. The Journal of pediatrics 1977; 91: 559-564
- 25 Benvenuto C, Kraemer CK, Kruse RL et al. Familial epidermolysis bullosa with aplasia cutis congenita: Bart's syndrome?. Skinmed 2003; 2: 319-321
- 26 Yang JY, Yang WG. Large scalp and skull defect in aplasia cutis congenita. Br J Plast Surg 2000; 53: 619-622
- 27 Koshy CE, Waterhouse N, Peterson D. Large scalp and skull defects in aplasia cutis congenita. Br J Plast Surg 2001; 54: 276-277
- 28 Mossaz CF, Kokich VG. Redevelopment of the calvaria after partial craniectomy in growing rabbits: the effect of altering dural continuity. Acta anatomica 1981; 109: 321-331
- 29 Ross DA, Laurie SW, Coombs CJ et al. Aplasia cutis congenita: failed conservative treatment. Plastic and reconstructive surgery 1995; 95: 124-129
- 30 Trobs RB, Barenberg K, Hemminghaus M et al. Herniation of the brain after conservative treatment of a large congenital skull defect in an infant with Adams-Oliver syndrome. Journal of pediatric surgery 2010; 45: 2064-2067
- 31 Azad. An adherent dressing for aplasia cutis congenita. Br J Plast Surg 2005; 58: 1159-1161
- 32 Kantor J, Yan AC, Hivnor CM et al. Extensive aplasia cutis congenita and the risk of sagittal sinus thrombosis. Archives of dermatology 2005; 141: 554-556
- 33 Smartt Jr JM, Kim EM, Tobias AM et al. Aplasia cutis congenita with calvarial defects: a simplified management strategy using acellular dermal matrix. Plastic and reconstructive surgery 2008; 121: 1224-1229
- 34 Schnabl SM, Horch RE, Ganslandt O et al. Aplasia cutis congenita--plastic reconstruction of three scalp and skull defects with two opposed scalp rotation flaps and split thickness skin grafting. Neuropediatrics 2009; 40: 134-136
- 35 Ploplys EA, Muzaffar AR, Gruss JS et al. Early composite cranioplasty in infants with severe aplasia cutis congenita: a report of two cases. The Cleft palate-craniofacial journal: official publication of the American Cleft Palate-Craniofacial Association 2005; 42: 442-447
- 36 Ng PC, Lee CH, To KF et al. Severe congenital absence of skin in a preterm infant. Journal of paediatrics and child health 1999; 35: 306-308
- 37 Theile RJ, Lanigan MW, McDermant GR. Reconstruction of aplasia cutis congenita of the scalp by split rib cranioplasty and a free latissimus dorsi muscle flap in a nine month old infant. Br J Plast Surg 1995; 48: 507-510
- 38 Beekmans SJ, Don Griot JP, Mulder JW. Split rib cranioplasty for aplasia cutis congenita and traumatic skull defects: more than 30 years of follow-up. The Journal of craniofacial surgery 2007; 18: 594-597
- 39 Ghazi BH, Williams JK.. Use of Integra in complex pediatric wounds. Annals of plastic surgery 2011; 66: 493-496
- 40 Loreti. Aplasia cutis congenita: report of four cases and literature review. Eur J Plast Surg 2004; 27: 114-119
- 41 Basterzi Y, Bagdatoglu C, Sari A et al. Aplasia cutis congenita of the scalp and calvarium: conservative wound management with novel wound dressing materials. The Journal of craniofacial surgery 2007; 18: 427-429
- 42 Shivakumar SK, Dwarakanath S, Swaroop G et al. Aplasia cutis congenita of the scalp: therapeutic modalities. Neurology India 2006; 54: 312-313
- 43 Lahiri A, Nishikawa H. A nonadherent dressing for aplasia cutis congenita. Journal of plastic, reconstructive & aesthetic surgery: JPRAS 2006; 59: 781-782
- 44 Canter HI. Comment on „An adherent dressing for aplasia cutis congenita“. Journal of plastic, reconstructive & aesthetic surgery: JPRAS 2006; 59: 1469-1470
- 45 Bleasdale B, Finnegan S, Murray K et al. The Use of Silicone Adhesives for Scar Reduction. Advances in wound care 2015; 4: 422-430