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Year : 2018  |  Volume : 1  |  Issue : 1  |  Page : 41-42

Kidney disease in cloacal malformations

Department of Nephrology, Sevome Shaban Hospital, Tehran, Iran

Date of Web Publication28-Jun-2018

Correspondence Address:
Majid Malaki
Sevome Shaban Hospital, Tehran
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/AJPN.AJPN_5_18

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How to cite this article:
Malaki M. Kidney disease in cloacal malformations. Asian J Pediatr Nephrol 2018;1:41-2

How to cite this URL:
Malaki M. Kidney disease in cloacal malformations. Asian J Pediatr Nephrol [serial online] 2018 [cited 2022 Jul 7];1:41-2. Available from: https://www.ajpn-online.org/text.asp?2018/1/1/41/235479


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.[1],[2] We reviewed the records of 80 patients presenting with ambiguous genitalia, diagnosed as defined previously [3] 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].
Table 1: Features at presentation

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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.[4],[5] 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 [2],[6] and facial anomalies [6] and umbilical hernia [6] [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.[5] 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.

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Conflicts of interest

There are no conflicts of interest.


I am grateful for the support of Dr. Mohammad Mosadegh.

  References Top

Brock WA, Pena A. Cloacal abnormalities and imperforate anus. In: Kelais PP, King LR, Belman AB, editors. Clinical Pediatric Urology. 3rd ed. Philadelphia: W. B. Saunders; 1992. p. 920.  Back to cited text no. 1
Abu-Rustum RS, Chabban M. Is 3-dimensoinal sonography is useful in prenatal diagnosis? J Ultrasound Med 2009:28:95-103.  Back to cited text no. 2
Zdravković D, Milenković T, Sedlecki K, Guć-Sćekić M, Rajić V, Banićević M, et al. Causes of ambiguous external genitalia in neonates. Srp Arh Celok Lek 2001;129:57-60.  Back to cited text no. 3
Warne SA, Wilcox DT, Ledermann SE, Ransley PG. Renal outcome in patients with cloaca. J Urol 2002;167:2548-51.  Back to cited text no. 4
Fernando MA, Creighton SM, Wood D. The long-term management and outcomes of cloacal anomalies. Pediatr Nephrol 2015;30:759-65.  Back to cited text no. 5
Lingaiah K, Parshwanath BA, Mysore SR, Krishnamurthy B, Ramachandra NB. A rare case of congenital heart disease with ambiguous genitalia. Indian J Hum Genet 2010;16:166-8.  Back to cited text no. 6
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