Long-term survival with cloacal dysgenesis sequence

被引:1
作者
Yanai, Toshihiro [1 ]
Urita, Yasuhisa [2 ]
Amagai, Teruyoshi [3 ]
Kawakami, Hajime [1 ]
Watayo, Hiroko [1 ]
Masuko, Takayuki [1 ]
Matsuda, Satoshi [1 ]
Hirai, Misako [1 ]
Muraji, Toshihiro [1 ]
Hamada, Hiromi [4 ]
机构
[1] Ibaraki Childrens Hosp, Dept Pediat Surg, Mito, Ibaraki 3114145, Japan
[2] Univ Tsukuba, Dept Pediat Surg, Tsukuba, Ibaraki, Japan
[3] Mukogawa Womens Univ, Dept Food Sci & Nutr, Kobe, Hyogo, Japan
[4] Univ Tsukuba, Dept Obstet & Gynecol, Tsukuba, Ibaraki, Japan
关键词
Cloacal dysgenesis; Urorectal septum malformation; Urethral obstruction; Ambiguous genitalia; Anal atresia; SEPTUM MALFORMATION SEQUENCE; PRENATAL-DIAGNOSIS;
D O I
10.1007/s00383-011-3020-3
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
Cloacal dysgenesis sequence (CDS) is a rare and lethal malformation. We report such a case of long-term survival, currently to 12 years of age. In the fetal period, she received a timely placement of vesico-amniotic shunt for a megabladder due to a severe urethral obstruction. Postnatally, cystostomy and colostomy were created because of no perineal opening of urethra, vagina, and anus. Anorectoplasty, construction of efferent conduit, and colostomy closure were performed at 4 years of age. Ileovaginoplasty and ileovesicostomy which was a Mitrofanoff-type of conduit, and labioplasty were performed at the age of 11 years. To the best of our knowledge, only five survivors with CDS over 1 year of age have been reported.
引用
收藏
页码:107 / 110
页数:4
相关论文
共 11 条
[1]  
BLOTT M, 1987, LANCET, V2, P129
[2]   URORECTAL SEPTUM MALFORMATION SEQUENCE - REPORT OF 6 CASES AND EMBRYOLOGICAL ANALYSIS [J].
ESCOBAR, LF ;
WEAVER, DD ;
BIXLER, D ;
HODES, ME ;
MITCHELL, M .
AMERICAN JOURNAL OF DISEASES OF CHILDREN, 1987, 141 (09) :1021-1024
[3]   Cloacal Dysgenesis Sequence in a Set of Twins: New Insights Into Embryology and a Review of the Literature [J].
Gupta, R. ;
Parelkar, S. V. ;
Oak, S. ;
Sanghvi, B. ;
Kaltari, D. K. ;
Prakash, A. ;
Patil, R. ;
Bachani, M. .
EUROPEAN JOURNAL OF PEDIATRIC SURGERY, 2010, 20 (05) :355-357
[4]  
Honda S, 2010, JPN J PEDIAT SURG, V46, P591
[5]   Survival in a Neonate with Complete Urorectal Septum Malformation Sequence after Fetal Vesico-Amniotic Shunting for a Prominently Dilated Cloaca [J].
Kanamori, Yutaka ;
Iwanaka, Tadashi ;
Nakahara, Saori ;
Kawashima, Hiroshi ;
Komura, Makoto ;
Sugiyama, Masahiko ;
Kamei, Yoshimasa ;
Hyodo, Hironobu ;
Yamaguchi, Shun-ichi ;
Kohzuma, Shiro ;
Taketani, Yuji ;
Goishi, Keiji ;
Ishiguro, Akio ;
Tsuchida, Shinya ;
Takamizawa, Masaru ;
Igarashi, Takashi .
FETAL DIAGNOSIS AND THERAPY, 2008, 24 (04) :458-461
[6]   Fetal enterolithiasis: prenatal sonographic and MRI diagnosis in two cases of urorectal septum malformation (URSM) sequence [J].
Lubusky, M ;
Prochazka, M ;
Dhaifalah, I ;
Horak, D ;
Geierova, M ;
Santavy, J .
PRENATAL DIAGNOSIS, 2006, 26 (04) :345-349
[7]   Long-term survival and renal transplantation in a monozygotic twin with cloacal dysgenesis sequence [J].
Mukhtar, Rita A. ;
Baskin, Laurence S. ;
Stock, Peter G. ;
Lee, Hanmin .
JOURNAL OF PEDIATRIC SURGERY, 2009, 44 (12) :E31-E33
[8]   Urorectal septum malformation sequence: Ultrasound correlation with fetal examination [J].
Patil S.J. ;
Phadke S.R. .
The Indian Journal of Pediatrics, 2006, 73 (4) :287-293
[9]   Prenatal diagnosis of cloacal dysgenesis sequence: Differential diagnosis from other forms of fetal obstructive uropathy [J].
Qureshi, F ;
Jacques, SM ;
Yaron, Y ;
Kramer, RL ;
Evans, MI ;
Johnson, MP .
FETAL DIAGNOSIS AND THERAPY, 1998, 13 (02) :69-74
[10]   The prenatal diagnosis of cloacal dysgenesis sequence in six cases: can the termination of pregnancy always be the first choice? [J].
Sahinoglu, Z ;
Mulayim, B ;
Ozden, S ;
Etker, S ;
Celayir, A ;
Ozkan, F ;
Bilgic, R .
PRENATAL DIAGNOSIS, 2004, 24 (01) :10-16