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先天性皮肤发育不全

Aplasia cutis congenita.

作者信息

Irons G B, Olson R M

出版信息

Plast Reconstr Surg. 1980 Aug;66(2):199-203. doi: 10.1097/00006534-198008000-00003.

DOI:10.1097/00006534-198008000-00003
PMID:6996008
Abstract

Aplasia cutis congenita is an uncommon condition; fewer than 300 cases have been reported in the literature. Usually, the condition occurs as a focal scalp ulcer, but it may involve the full thickness of the skull or other areas of the body. Most lesions require coverage with a scalp flap, though only observation or split-thickness skin grafts may be adequate for smaller lesions. Four cases have been presented, representing a spectrum of therapeutic requirements from simple observation to emergency intervention to control life-threatening hemorrhage. The case of aplasia cutis congenita of the upper arm may represent a persistence of prenatal focal ischemia that has proved to be resistant to numerous attempts of split-thickness skin grafting.

摘要

先天性皮肤发育不全是一种罕见病症;文献报道的病例少于300例。通常,该病表现为头皮局灶性溃疡,但也可能累及颅骨全层或身体其他部位。多数皮损需要用头皮瓣覆盖,不过较小的皮损仅观察或采用刃厚皮片移植可能就足够了。本文介绍了4例病例,代表了从单纯观察到紧急干预以控制危及生命的出血等一系列治疗需求。上臂先天性皮肤发育不全的病例可能代表产前局灶性缺血持续存在,事实证明,多次刃厚皮片移植尝试均对其无效。

相似文献

1
Aplasia cutis congenita.先天性皮肤发育不全
Plast Reconstr Surg. 1980 Aug;66(2):199-203. doi: 10.1097/00006534-198008000-00003.
2
Aplasia cutis congenita: clinical management and a new classification system.先天性皮肤发育不全:临床管理和新的分类系统。
Plast Reconstr Surg. 2014 Nov;134(5):766e-774e. doi: 10.1097/PRS.0000000000000638.
3
[Aplasia cutis congenita in 4 infants].
Ned Tijdschr Geneeskd. 2002 Sep 28;146(39):1842-5.
4
Total reconstruction of aplasia cutis congenita involving scalp, skull, and dura.先天性皮肤发育不全累及头皮、颅骨和硬脑膜的全层重建。
Plast Reconstr Surg. 1986 Apr;77(4):650-3. doi: 10.1097/00006534-198604000-00026.
5
Aplasia cutis congenita of the scalp: issues in its management.头皮先天性皮肤发育不全:治疗中的问题
Pediatr Neurosurg. 1991;17(4):182-4. doi: 10.1159/000120592.
6
Aplasia cutis congenita of the scalp: delayed closure complicated by massive hemorrhage.头皮先天性皮肤发育不全:延迟闭合并伴有大量出血。
Plast Reconstr Surg. 1985 Mar;75(3):423-5. doi: 10.1097/00006534-198503000-00024.
7
Large scalp and skull defect in aplasia cutis congenita.先天性皮肤发育不全中的大面积头皮和颅骨缺损。
Br J Plast Surg. 2000 Oct;53(7):619-22. doi: 10.1054/bjps.2000.3413.
8
Aplasia cutis congenita: clinical management of a rare congenital anomaly.先天性皮肤发育不全:一种罕见先天性异常的临床管理
J Craniofac Surg. 2011 Jan;22(1):159-65. doi: 10.1097/SCS.0b013e3181f73937.
9
Scalp aplasia cutis congenita: closure by the L-shaped flap.
Childs Nerv Syst. 1992 Aug;8(5):287-8. doi: 10.1007/BF00300799.
10
Aplasia cutis congenita: management of a large skull defect with acrania.先天性皮肤发育不全:颅骨缺失伴巨大颅骨缺损的处理
J Craniofac Surg. 2009 Jul;20(4):1288-92. doi: 10.1097/SCS.0b013e3181ae2108.

引用本文的文献

1
Naturally occurring wounds and wound healing in chick embryo wings.鸡胚翅膀中的自然伤口与伤口愈合
Rouxs Arch Dev Biol. 1986 Aug;195(6):345-354. doi: 10.1007/BF00402868.
2
Failed conservative management of a case of aplasia cutis congenita in a low-income country.低收入国家一例先天性皮肤发育不全保守治疗失败的病例。
Clin Case Rep. 2016 Jun 30;4(8):756-8. doi: 10.1002/ccr3.611. eCollection 2016 Aug.
3
Aplasia cutis congenita of the scalp with large underlying skull defect: a case report.头皮先天性皮肤发育不全伴巨大颅骨缺损:一例报告
Neuroradiology. 1994 Aug;36(6):480-2. doi: 10.1007/BF00593689.
4
Congenital scalp and skull defects with terminal transverse limb anomalies (Adams-Oliver syndrome): report of three additional cases.伴有末端横断肢体畸形的先天性头皮和颅骨缺损(亚当斯-奥利弗综合征):另外三例报告
Eur J Pediatr. 1990 May;149(8):565-6. doi: 10.1007/BF01957693.