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Year : 2014  |  Volume : 59  |  Issue : 5  |  Page : 527-528
Absence of skin over scalp and short digits in a newborn


University College of Medical Sciences and Guru Teg Bahadur Hospital, Delhi, India

Date of Web Publication1-Sep-2014

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.139927

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How to cite this article:
Chhabra N, Kaur R. Absence of skin over scalp and short digits in a newborn. Indian J Dermatol 2014;59:527-8

How to cite this URL:
Chhabra N, Kaur R. Absence of skin over scalp and short digits in a newborn. Indian J Dermatol [serial online] 2014 [cited 2019 Jun 27];59:527-8. Available from: http://www.e-ijd.org/text.asp?2014/59/5/527/139927


A 1-day old female child, delivered at thirty six weeks by normal vaginal delivery at home, was admitted in intensive care unit with respiratory insufficiency and crusted lesion over parieto-occipital region of scalp. She was the third child of non-consanguineous parents. On examination, a 4 cm by 3 cm area of scalp defect with overlying scurfing crusts and perilesional atrophic scarring overlying the posterior fontanel was noted [Figure 1]. The underlying skull beneath the scalp defect was normal. Fingers 2-5 on both hands and toes 1-3 of left foot were small with absent nails [Figure 2] and [Figure 3]. Cardiac and neurological examination was normal. There was no family history of scalp or limb defects. The mother denied any history of maternal drug intake, infection or radiation exposure during pregnancy. Hands and feet X-ray of the child revealed hypoplasia of terminal phalanges of fingers and toes. Skull X-ray, chest X-ray, echocardiography, abdominopelvic ultrasonography, brain computed tomography were all normal.
Figure 1: Scalp defect with overlying scurfing crusts and perilesional atrophic scarring overlying the posterior fontanel

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Figure 2: Short fi ngers (2-5) with absent nails involving right hand

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Figure 3: Short toes (1-3) with absent nails involving left foot

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2.McGoey RR, Lacassie Y. Adams-Oliver syndrome in siblings with central nervous system findings, epilepsy, and developmental delay: Refining the features of a severe autosomal recessive variant. Am J Med Genet 2008;146:488-91.  Back to cited text no. 2
    
3.Koiffmann CP, Wajntal A, Huyke BJ, Castro RM. Congenital scalp skull defects with distal limb anomalies (Adams-Oliver syndrome-Mc Kusick 10030): Further suggestion of autosomal recessive inheritance. Am J Med Genet 1988;29:263-8.  Back to cited text no. 3
    
4.Jaeggi E, Kind C, Morger R. Congenital scalp and skull defects with terminal transverse limb anomalies (Adams-Oliver syndrome): Report of three additional cases. Eur J Pediatr 1990;149:565-6.  Back to cited text no. 4
    
5.Borg K, Pfeifle J. Multifocal aplasia cutis congenita, distal limb hemimelia, and cutis marmorata telangiectatica in a patient with Adams-Oliver syndrome. Br J Dermatol 1992;127:160-63.  Back to cited text no. 5
    
6.Bajpai M, Pal K. Aplasia cutis cerebri with partial acrania-Total reconstruction in a severe case and review of the literature.J Pediatr Surg 2003;38:e4.  Back to cited text no. 6
    
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8.Dyall-Smith D, Ramsden A, Laurie S. Adams-Oliver syndrome: Aplasia cutis congenita, terminal transverse limb defects and cutis marmorata telangiectatica congenita. Australas J Dermatol 1994;35:19-22.  Back to cited text no. 8
    
9.Frank RA, Frosch PJ. Adams-Oliver syndrome: Cutis marmorata telangiectatica congenita with multiple anomalies. Dermatology 1993;187:205-8.  Back to cited text no. 9
    
10.Fryns JP, Legius E, Demaerel P, van den Berghe H. Congenital scalp defect, distal limb reduction anomalies, right spastic hemiplegia and hypoplasia of the left arteria cerebri media.-Further evidence that interruption of the early embryonic blood supply may result in Adams-Oliver (plus) syndrome. Clin Genet 1996;50:505-9.  Back to cited text no. 10
    
11.Davis PM, Buss PW, Simpson BA, Sykes PJ. Near fatal haemorrhage from the superior sagittal sinus in Adams-Oliver syndrome. Arch Dis Child 1993;68:433.  Back to cited text no. 11
    
12.Romaný× J, Puig L, Aznar G, Demestre X, Altirriba O, Alomar A. Adams-Oliver syndrome with unusual central nervous system alterations. Pediatr Dermatol 1998;15:48-50.  Back to cited text no. 12
    
13.Zapata HH, Sletten LJ, Pierpont ME. Congenital cardiac malformations in Adams-Oliver syndrome. Clin Genet 1995;47:80-4.  Back to cited text no. 13
    
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15.Seo JK, Kang JH, Lee HJ, Lee D, Sung HS, Hwang SW. A case of Adams-Oliver syndrome. Ann Dermatol 2010;22:96-8.  Back to cited text no. 15
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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