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Posted: Jan 02, 2008 at 0000 hrs IST

A baby born to a couple after 12 years of their marriage had severe developmental problems. The baby’s pelvis and other systems within were not fully developed and connected. The pelvic bones were spread out and far apart, the skin and the muscles covering the abdomen were not formed, there was a large cyst behind the bladder, 3 ureters instead of two with a balloon formation at the ends, a tiny opening of the anus and a closed and double vagina. The Neonatal Surgeon, Dr Dasmit Singh, who operated on her said, “This is an extremely rare case.” Adding he also disclosed that he comes across 5 to 6 cases of Exstrophy Bladder (developmental problem of the urinary bladder) per year, but not the entire pelvic systems malformed, like this.

The baby was stabilized in the NICU, investigated and then surgery was planned. The baby needed reconstruction of her Urinary Bladder, Ureters, Vagina, Abdominal wall and the Pelvic bones. In addition, there was a double Vagina and one of them was not open to the exterior, so secretions had collected within it, forming a large abdominal cyst (Duplication of Vagina with Hydrometrocolpos). The Ureters were also double on one side with the end of the ureter like a balloon instead of being like a pipe (Ureterocoel with Duplication). The abdominal wall was also not developed below the umbilicus.

Generally a complex congenital anomaly of this nature would need 4 to 5 surgeries to set it right and even then the result may not be very favourable. Keeping in mind all the options, it was decided to attempt all the surgeries in a single stage itself.

After 6 hours of operation, all the developmental defects were put in place by Dr Dasmit Singh, the Pediatric Surgeon of Pune who is especially known for routinely performing Laparoscopic and Thoracoscopic Surgeries on children and also for Single Stage Repairs of all anomalies, including Hypospadias (Birth defects of the Genitals). The baby needed highly specialized care in the NICU for 3 weeks and made an uneventful recovery.

“This is generally not a type of genetic disorder, it is usually a developmental defect. It is essential to have a good team that can combine expert care with dedication to obtain a good outcome in such tiny babies. The role of the Neonatal and Paediatric Surgeon cannot be effectively accomplished without the expert contribution of the Anaesthetists, Neonatologists, Junior Doctors, Nurses, Ayahs and the supportive team of the other departments of the hospital,” Singh stated.The baby is now a month and a half old and has a normal life. The bladder can already hold urine quite well.

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