Bengaluru: Fortis Hospitals Bangalore recently performed a complex and rare surgery on a 13-year-old boy from Bangalore who was suffering from an unusual birth defect. The patient, Balaji, was born with a condition known as Exstrophy-Epispadias Complex where the patient had an inability to contol his urination, due to an absent continence mechanism (which normally helps to hold back urine) and incompletely formed male sexual organs.

After many check-ups and one failed surgery at different medical institutions, Balaji’s parents finally approached Fortis hospitals Rajajinagar as their last ray of hope. Dr. Mohan Keshava Murthy and Dr. Z. Shakir Tabrez, Consultant Urologists, along with Dr. J V Srinivas, Consultant Orthopedic and Joint Replacement Surgeon at Fortis Hospitals Rajajinagar performed 2 sequential complex surgeries and came up with a permanent solution to the boy’s problems.

When Dr. Mohan undertook the case, he advised the need for a staged repair. This birth defect caused a constant leakage of urine without control. When he was 2 years old, he was operated for the defect. But the surgery didn’t help and though his parents consulted many other doctors, no solution was forthcoming. He continued to leak urine and was also ostracized by his classmates because of the foul smell that this condition caused.

Before the surgery, the doctors at Fortis prepared an operational plan which was – a) to give good abdominal cover over the bladder b) to try and restore the God given continence mechanism (the ability to retain urine) by a procedure called bladder neck reconstruction (Young Dees Leadbetter technique) c) to take the help of our orthopaedic specialist for fusion of the pubic bone and d) to reconstruct the urethral tube with a good tissue and skin cover (Cantwell Ransley repair).

The surgeons performed the steps a, b and c together in the first sitting and after the wound healed and the continence mechanism was restored, urethral reconstruction surgery was done. The specialists were also ready to augment or expand the capacity of the bladder by using a segment of bowel to reconstruct the urinary bladder if its capacity was found to be less. The entire procedure was performed under general plus regional anesthesia (for better post operative pain relief) which took about 4 hours and was absolutely uneventful.

The boy was discharged after 4 days in hospital. 4 weeks later when his catheter was removed, the boy could control his urine with ease.

The second part of the procedure was then planned – 6 weeks after the first. Again under similar anesthesia, the urethra was reconstructed (Cantwell Ransley repair). Some adhesions and tissues were freed along with the suspensory ligament, in order to give the boy better penile length. The urethra was then reconstructed over a catheter. He was discharged after 2 days in hospital. A week later his catheters were removed and Balaji was able to hold urine and pass it freely through his reconstructed urine passage.

He was also started on a testosterone supplement that would aid in growth of the supporting tissue, add length to his penis and help in his attaining puberty at the appropriate time.

The entire family was ecstatic as this would mean he would be able to mix and mingle with the rest of his school and society without being an outcast. Major part of the procedure took place during his summer holidays and hence he did not miss school either. Balaji’s treatment gives a ray of hope to all the other patients who suffer from such defects and don’t find the right medical treatment.

Exstrophy-Epispadias complex and other similar birth defects including hypospadias can and should be corrected at the appropriate time and with proper surgical technique and care.

“Treating such conditions requires a high level of expertise, keeping in mind the fragile nature of tissues and the tender young age of these patients. Procedures rectifying such rare defects require lot of expertise and medical support,” added Dr. Mohan.

“My son is finally normal and can live life like any other boy his age. All thanks to the medical team at Fortis Hospitals because of whom happiness is back in our lives,” said an elated mother.

Article Source: https://www.google.com/amp/s/www.apnnews.com/13-year-old-boy-suffering-from-rare-birth-defect-gets-new-lease-of-life-at-fortis-hospitals-bangalore/amp/

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