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Clinical and Experimental Obstetrics & Gynecology  2020, Vol. 47 Issue (2): 154-158    DOI: 10.31083/j.ceog.2020.02.5141
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OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins: a case report and literature review
Y. Q. Xu1, X. L. Chen2, S. Zhao2, P. W. Chen2, X. M. Yin1, S. Y. Xiong1, Z. Ding3, F. Xiong4, X. H. Yang2, *()
1Department of Ultrasound, Baoan Central Hospital of Shenzhen/The 5th affiliated hospital of Shenzhen University Health Science Center, 60 Xixiang Leyuan Street, Baoan district, Shenzhen, 518102, P.R. China
2Department of Ultrasound, Hubei Women and Children’s Hospital, 745 Wuluo Road, Wuhan, Hubei 430070, P.R. China
3The Institute of Metabolic Diseases, Baoan Central Hospital of Shenzhen/The 5th affiliated hospital of Shenzhen University Health Science Center, 60 Xixiang Leyuan Street, Baoan district, Shenzhen, 518102, P.R. China
4Department of Medical Genetics, School of Basic Medical Sciences, Southern Medical University, Guangzhou 510515, Guangdong, P.R. China
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Abstract  

The omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex represents severe midline abdominal and pelvic defects of unknown etiology. This complex is rare, affecting 1 in 200,000 to 400,000 pregnancies and is extremely rare in twin or triplet gestations. Here, we report the OEIS complex in a spontaneous monozygotic twin pregnancy and previously reported OEIS complex in twin or triplet pregnancy are reviewed. Spontaneous pregnancy and unremarkable family history, but concordance of monozygotic twins for the defect may support the theory that early malformation complexes, e.g., OEIS, may be related to errors in monozygotic splitting in early blastogenesis.
Key words:  Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS)      Twin pregnancy     
Published:  15 April 2020     
Fund: WJ2018-H0148/Hubei Province health and family planning scientific research project
*Corresponding Author(s):  X. H. Yang     E-mail:  yangxiaohong123@163.com
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Y. Q. Xu   
X. L. Chen   
S. Zhao   
P. W. Chen   
X. M. Yin   
S. Y. Xiong   
Z. Ding   
F. Xiong   
X. H. Yang   

Cite this article: 

Y. Q. Xu, X. L. Chen, S. Zhao, P. W. Chen, X. M. Yin, S. Y. Xiong, Z. Ding, F. Xiong, X. H. Yang. OEIS complex (omphalocele-exstrophy-imperforate anus-spinal defects) in monozygotic twins: a case report and literature review. Clinical and Experimental Obstetrics & Gynecology, 2020, 47(2): 154-158.

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https://ceog.imrpress.com/EN/10.31083/j.ceog.2020.02.5141     OR     https://ceog.imrpress.com/EN/Y2020/V47/I2/154

Figure 1.  — Figures of twins. (a): Twin A and B with an estimated gestational age of 15 weeks on ultrasonographic image. (b): Post-mortem examination showing a large abdominal wall defect in twin A and B. (c): Kyphoscoliosis of twin A and B on X ray film.

Table 1  — The reported 30 cases of OEIS complex in twins or triplets
Case No. Study Year Zygozity Concordance
1 Koffler et al. [5] 1978 monozygotic discordant
2 Schinzel et al. [6] 1979 monozygotic discordant
3 Ornoy et al. [7] 1980 monozygotic discordant
4 Nance [8] 1981 monozygotic discordant
5 Redman et al. [9] 1981 monozygotic concordant
6 Lowry and Baird [10] 1982 Not reported concordant
7 monozygotic concordant
8 Hesser et al. [11] 1984 Not reported discordant
9 Langer et al. [12] 1992 monozygotic discordant
10 Chitrit et al. [13] 1993 monozygotic concordant
11 Kapur et al. [14] 1994 monozygotic concordant
12 Meizner et al. [15] 1995 monozygotic discordant
13 monozygotic discordant
14 Bruch et al. [16] 1996 dizygotic discordant
15 Pinette et al. [17] 1996 Not reported discordant
16 Goldfischer et al. [18] 1997 monozygotic concordant
17 Kramer et al. [19] 1997 monozygotic concordant
18 Lee et al. [20] 1999 monozygotic concordant
19 Shanske AL et al. [21] 2003 trizygotic discordant
20 Siebert et al. [22] 2005 monozygotic concordant
21 Noack et al. [23] 2005 dizygotic concordant
22 Ben-Neriah et al. [24] 2007 dizygotic discordant
23 monozygotic concordant
24 Keppler-Noreuiletal. [25] 2007 monozygotic discordant
25 Not reported discordant
26 Not reported discordant
27 Yokoyama E et al. [26] 2007 trizygotic discordant
28 Tiblad et al. [27] 2008 Monozygotic discordant
29 Mathew R et al. [28] 2009 monozygotic discordant
30 Kunapinun N et al. [29] 2017 monozygotic discordant
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