|Year : 2018 | Volume
| Issue : 1 | Page : 41-42
Kidney disease in cloacal malformations
Department of Nephrology, Sevome Shaban Hospital, Tehran, Iran
|Date of Web Publication||28-Jun-2018|
Sevome Shaban Hospital, Tehran
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Malaki M. Kidney disease in cloacal malformations. Asian J Pediatr Nephrol 2018;1:41-2
Malformations with persistent cloaca, the common cavity at the end of gastrointestinal, urinary, and reproductive tracts, are rare anomalies that affect one per 50,000–70,000 live births with varying presentation and severity., We reviewed the records of 80 patients presenting with ambiguous genitalia, diagnosed as defined previously  at a single center over 4 years. Clinical, biochemical, and radiological findings in the ten (12.5%) cases that were secondary to cloacal malformations are presented in [Table 1].
Patients were evaluated chiefly for genital ambiguity (100%), renal dysfunction (70%), urinary tract infections (50%), imperforate anus (70%), or omphalocele (30%). Nine of the 10 patients were assigned a female gender at birth. The sole patient reared as boy had imperforate anus and rectourethral fistula, undescended bilateral testes (palpable in inguinal canals), microphallus, and seizures secondary to hypocalcemia and hypoglycemia. Klebsiella sp. was the most common cause of urinary tract infections, and renal failure is the chief cause of morbidity and death.
Disturbance of cloacal septation during caudal regression leads to abnormalities in organs in the proximity, such as ureteral bud, mullerian duct derivatives and vertebral column. We found vesicoureteral reflux (VUR) and/or unilateral renal agenesis, urinary tract fistulae, anteverted kidney malposition and bladder anomalies [Table 1]. Structural kidney diseases are identified in 33–83% of cloacal malformations, with VUR being most common., Genitourinary tract abnormalities included rectourethral and rectovaginal fistulae, vagina and urethra joined as a common duct, absence of cervix, duplicated uterus and/or bladder, and classic forms where urogenital sinus and rectum form a common sac. Malformations of the gastrointestinal tract and anorectal region included anal imperforation and omphalocele. Other abnormalities described are cardiac , and facial anomalies  and umbilical hernia  [Table 1].
Seven patients progressed to renal failure, two of whom had severe bilateral renal dysplasia with glomerular filtration rate irreversibly below 15 ml/min/1.73 m 2, while five were rescued partially by hydration and treatment of urinary tract infection. Similar to our findings, Fernando et al. reported chronic renal failure in 50% of patients with cloacal malformations. Metabolic acidosis, hyponatremia, and hyperkalemia were observed in 50% patients, potentially related to pseudohypoaldosteronism with acute pyelonephritis. Five patients succumbed to complications of renal failure, including two in infancy.
This report highlights the variety and severity of findings in patients with cloacal malformations and underscores the importance of evaluating for extrarenal as well as renal lesions in patients with ambiguous genitalia.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
I am grateful for the support of Dr. Mohammad Mosadegh.
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