Reddy K Ramesh, Srinivas S, Kumar Shiva, Reddy Surweshwar, Prasad Hari, Irfan G M
Niloufer Hospital, Institute of Child Health, Osmania Medical College, Hyderabad, A.P, India.
J Neonatal Surg. 2012 Jan 1;1(1):7. eCollection 2012 Jan-Mar.
We are presenting two cases of Sirenomelia (Mermaid Syndrome), which is an extreme example of the caudal regression syndrome. It invariably presents with lower limb fusion, sacral and pelvic bony anomalies, absent external genitalia, imperforate anus, and renal agenesis or dysgenesis. There are approximately 300 cases reported in the literature, 15% of which are associated with twinning, most often monozygotic. The syndrome of caudal regression is thought to be the result of injury to the caudal mesoderm early in gestation. One of our cases survived for 12 days after birth. This new born had an unusually high anorectal anomaly in which the colon was ending at the level of mid transverse colon, fused lower limbs and genital anomalies. Ultrasound of the abdomen revealed horseshoe kidney. Colostomy was performed on day 2 of life. The second case encountered was a stillborn baby on whom an autopsy was performed.
我们现报告两例美人鱼综合征病例,该综合征是尾椎退化综合征的极端表现形式。其总是表现为下肢融合、骶骨和骨盆骨骼异常、外生殖器缺如、肛门闭锁以及肾缺如或发育不全。文献报道约有300例病例,其中15%与双胎妊娠有关,最常见的是单卵双胎。尾椎退化综合征被认为是妊娠早期尾侧中胚层受损的结果。我们的其中一例病例出生后存活了12天。这名新生儿存在异常高位的肛门直肠畸形,结肠终止于横结肠中部水平,下肢融合以及生殖器畸形。腹部超声显示马蹄肾。出生第2天进行了结肠造口术。遇到的第二例病例是一名死产儿,并对其进行了尸检。