In a rare surgery, doctors from a city hospital successfully operated on a twoyear–old girl from Yemen who was born with a unique birth defect wherein a large portion of the abdominal wall was absent. The girl also had no urinary bladder andhadonly one kidney.

Doctors created an altogether new bladder inside by making a pouch using a portion of intestines.The girlwas operated three weeks ago and has now recovered and is all settofly backtoher country.

"Due to the rare birth defect, the baby had no control over her stool or urine, which kept dripping continuously causing pain and damaging the surrounding skin and emanating a bad odour," said the girl’s parents.

After an unsuccessful operation by doctorsin Yemen to reconstruct the urinary bladder, the child developed added complication of repeated urinary tractinfectionswhich,in turn, further compromised the one kidney that she had. Her relatives approached doctors at Jehangir hospital and her parents flew down to Pune in November.

"First, a magnetic resonanceimaging (MRI)scan was done to see why the child was not able to control passing of her stool. This, however, revealed yet another defect. The child’s spinal cord and nerves were adhering to the surrounding tissues and causing an undue stretching of the cord(tetheredcordsyndrome) causing damage to the spinal nerves," said paediatric surgeon Dasmit Singh who successfully operated on the child on November 24.

Moreover, the nerves were encased in a fatty tumour (intra–spinallipoma)which,ifleft untreated, would have led to a complete loss of lower limb movement,lossof sensation in the lower limbs and complete loss of urinary and bowel control. This had to be treated urgently.

"The surgery for this correction entailed operating from theback,cutting open the bones of the spine (laminectomy) and releasing all the attachments of the spinal cord. Thiswas madeeven moredifficultby thefatty tumour which also neededtobeexcised atthe same time," Singh explained. This first surgery was carried out by Singh where he was assistedby neurosurgeon PrashantKhandelwal.

"Within 15 days, the child began to experience the urge to passstool andwas abletodo so like any normal child," Singhsaid.

Attention was then turned to the problem of the urinary incontinence. The final decision taken by the parents was basedon thefactthattherewas no way that the child would passurinelike a normalchild.

"It was decided that a pouch had to be made within the body to collect the urine (neobladder) anddrain theurine from the single kidney into it. This pouch would be made from a portion of the intestines. A pipe would then have to be constructed from this pouch within the abdomen to the exterior of the abdominal wall to enable the child to pass a catheter into the neobladder every fewhours andempty the urine. This would ensure that nourineleaked, rendering the child ‘dry’," Singhsaid.

The second surgery – lasting for a total of 10 hours – was also performed by Singh where he was assisted by paediatric surgeon Kshama Kulkarni and a team of anaesthetistsincluding Sudhir Phadke, andMohan Swami.

Thechild,with nolessthan six tubes coming out of her frail body, each for a different purpose,wasshiftedtothe paediatricintensive careun it into thecareof a team of paediatriciansledby SanjayBafna.

"The child is completely fine now. Her mother currently performs the task of removing theurinethroughthecatheter, which she will be able to do herself as she grows older," said Singh.

• Doctors created an altogether new bladder inside by making a pouch using a portion of intestines to collect urine drained from her single kidney. For this, a part of the small intestines was isolated along with its blood supply and then stitched into a very complex–shaped pouch – the process of stitching itself taking almost five hours to complete
• The loose ends of the remaining intestines were stitched back together again to establish the continuity of the intestines
• The pouch was attached to the remains of the urinary bladder below. The tiny bladder was rolled up tight to form a safety pressure–relieving valve, in case the child forgot to empty the bladder with the catheter

(Source: Paediatric surgery department, Jehangir hospital, Pune)