Abstract

Neonatal autopsy is a developing field in Sri Lanka. It needs human expertise as well as infra-structure with basic and advanced facilities in imaging, serology, genetics, biochemistry etc. Sir Lanka has relatively low neonatal mortality rates compared to other South Asian countries. Cloaca anomaly is a rare pathological condition almost always affecting female infants due to abnormal development of cloaca during the embryonic period. This is basically due to disordered development of endodermal – incorporation of allantois into the hindgut, formation of mesodermal urorectal septum and rupture of the cloacal membrane during the 4th and 7th week of gestation. Sometimes it is associated with disordered development of metanephros, mesonephric duct and paramesonephric duct from which the upper portion of the urinary tract including kidney, ureteric bud and female genital tract develop including fallopian tubes, uterus and upper two-thirds of the vagina. Isolated abnormal development of cloaca is compatible with life and it may be amenable to surgery, though such surgery may invariably involve multiple steps. On the other hand, the outcome is poor when it is associated with other anomalies elsewhere in the body. This case elaborates the death of a female neonate within two hours of birth with a cloacal anomaly complicated by multiple other anomalies of kidney, ureter and lung. It emphasizes the limitations of antenatal investigations in delineating all anatomical and pathological conditions and the need for a meticulous neonatal autopsy to compensate the limitations of antenatal diagnostic techniques.

Highlights

  • Cloaca anomaly is a rare pathological condition almost always affecting female infants due to abnormal development of cloaca during the embryonic period after five weeks of gestation.[1,2] Cloacal malformation is a condition in which there is a single opening for the genital, urinary and gastrointestinal tracts.[2]

  • No accurate data are available regarding the incidence of cloacal anomalies in Sri Lanka at present

  • Variations from this isolated anomaly are common due to associated disordered development of metanephros, mesonephric duct and paramesonephric duct from which the upper portion of the urinary tract including kidney, ureteric bud and female genital tract develop including fallopian tubes, uterus and the upper two-thirds of the vagina. This incidence is misdiagnosed as fistulae including ureto-vaginal fistula, rectovaginal fistula or recto-uterine fistula

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Summary

Introduction

Cloaca anomaly is a rare pathological condition almost always affecting female infants due to abnormal development of cloaca during the embryonic period after five weeks of gestation.[1,2] Cloacal malformation is a condition in which there is a single opening for the genital, urinary and gastrointestinal tracts.[2].

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