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Posterior urethral valves in children: a review of 28 cases in Yaounde, Cameroon

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par Andreas TEHJI CHIABI
Université of Yaounde I - Specialist Diploma in Clinical Sciences, Option Paediatrics 0000
  

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III. TREATMENT (Tables 13 and 14)

Of our 28 patients, 2 were lost to follow-up immediately after diagnosis and so did not undergo surgery. 1 patient with end-stage renal failure had ureterostomy in France. So only 25 patients had at least one major surgical procedure mentioned in Table 13. A

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POSTERIOR URETHRAL VALVES IN CHILDREN: A review of 28 cases in Yaounde

total of 20 endoscopic resections were done, 1 in Britain and 1 in France.

BLOCKSOM vesicostomies were done in 6. 2 were closed by the end of the study after endoscopic ablation. Of the remaining 4, 2 were yet to be closed amongst which one, who had vesicostomy at the age of 3 weeks was lost to follow-up and was later seen at the age of 6 years in the prospective phase of the study ; and 2 had died at 2 days post -op. of septicaemia. BLOCKSOM vesicostomy is a tubeless bladder diversion. It was first performed by BLOCKSOM in 1956 in a 75 year old man with carcinoma of the urethra (42). The advantages are that it is tubeless, readily reversible, easy to perform and does not require any appliance. By decompressing the urinary tract it allows a very ill, often azotemic and septic child to recover from the long-term effects of obstruction or severe reflux (42). Definitive reconstructive surgery can be postponed until the patient's condition is optimal, renal function has improved or stabilized, infection is finished or until the patient's size is more appropriate for the particular procedure.

All the patients who underwent cystostomy later underwent endoscopic ablation. Two cases of catheter ablation were done before the advent of endoscopic surgery in Cameroon. 1 later underwent endoscopic resection and the other went into end-stage renal failure and died.

Secondary procedures were performed in only 11 patients. High diversions were performed in 6 cases - 3 ureterostomies and 3 nephrostomies for severe bilateral hydronephrosis. All the ureterostomies and nephrostomies were closed Primary ureteroplasties were done in 4 patients. One patient had urethrostomy for meatal stenosis following catheter ablation done elsewhere. Catheter ablation is abandoned in our institutions since the advent of endoscopic surgery.

Although controversy still exists as to the management of PUV, the current attitude is a primary valve ablation followed by observation and vesicostomy reserved for patients in whom valve ablation is not technically possible or in a child with severe renal failure .(43, 44,31,45). The long-term outcomes with primary diversion and primary valve ablation are the same, but performance and reversibility of diversion

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POSTERIOR URETHRAL VALVES IN CHILDREN: A review of 28 cases in Yaounde

requires more major surgical procedures (31).

IV. OUTCOME OF THE PATIENTS (Table 15)

The overall mortality was 21 %. COULIBALY et al (18) had 15%; FALL et al (20)14%, LOTTMAN (46) 4% and WARSHAW et al (38) 4.5%. 10 patients (36%) were lost to follow-up

Causes of deaths: 3 died of septicaemia, 2 post-obstructive diuresis and 1 of chronic renal failure

* Septicemia:1 patient (2 months old) died 2 days after an emergent vesicostomy for uraemia, from Pseudomonas septicaemia. l (3 months) died of klebsiella sepsis and cardiac decompensation and 1 (2 years old) died of klebsiella pneumonia and Enterobacter cloacae septicaemia one week after having undergone endoscopic resection and ureteroplasty for bilateral hydronephrosis.

* Chronic renal failure: The patient who died of chronic renal failure was 18 years old. He had undergone catheter ablation in the neonatal period, developed end-stage renal failure at 7 and had a kidney transplantation (the donor was the mother) in France. Before transplantation he had had a nephrostomy and several peritoneal dialysis. Back home in Cameroon, he was on immunosuppressors (Cyclosporine and Azathioprine) developed skin Kaposi sarcoma, severe lung infection graft rejection and died.

* Post -obstructive diuresis: One patient (9 days old) died 2 days after an emergency BLOCKSOM vesicostomy for uraemia. The other 4 months old died 2 months after endoscopic resection, in a hospital out of Yaounde. He had developed gastro-enteritis, and this added to the polyuria he has been having and inadequate dehydration caused severe dehydration and death.

Post-obstructive diuresis is persistent polyuria following valve ablation or relief of any obstruction of the urinary (47, 48). This can provoke a dramatic urinary loss of salt and water and hypotonic urine. Severe polyuria carries a risk of dehydration, particularly with diarrhoea and vomiting or high solute feeds (47). There are two major causes: (48)

1) Urea, through its osmotic effect and possibly natriuretic humoral substances 73

 
 
 

POSTERIOR URETHRAL VALVES IN CHILDREN: A review of 28 cases in Yaounde

which inhibit the reabsorption of NaCl and water in the proximal and distal tubules.

2) Obstruction per se, possibly through increased pressure within the renal pelvis

inhibits the reabsorption of fluid and loss of urine concentration ability. Appropriate but cautious fluid replacement should be administered to patients with post-obstructive diuresis depending in large, on what is excreted.

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