Cloaca, Posterior Cloaca and Absent Penis Spectrum

Peña, Alberto; Bischoff, Andréa

doi:10.1007/978-3-319-14989-9_16

Cited by 3 publications

(7 citation statements)

References 120 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…reported a high association between PCM and hydrocolpos (65%), as compared to typical cloacae and hydrocolpos (25%). [ 3 ] We found a similar association in our series, with 3 (75%) patients presenting with hydrocolpos and requiring a tube vaginostomy procedure. The high incidence of hydrocolpos with cloacae, especially PCM, may be due to the anatomical configuration of this malformation which makes drainage of fluid from vagina more difficult.…”

Section: Discussionsupporting

confidence: 82%

“…A common concern raised previously by various authors[ 2 3 4 ] and as also seen in our experience is the delay in recognition of this complex and rare malformation. This is partly due to the lack of understanding of the complexity of the anatomy among the first contact physicians.…”

Section: Discussionsupporting

confidence: 64%

“…We describe a very rare and unusual series of patients with “posterior cloaca.” These patients usually have a single perineal orifice, as per the classical definition of “cloaca;” but what makes this defect unique is the common finding of a posteriorly displaced UGS which opens into the anterior wall of an “anatomically normal” anus. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…This is quite different from the anatomy seen in patients with persistent UGS anomaly, wherein the UGS opens at the site of a normal urethral opening with the presence of a normal anus. [ 3 ]…”

Section: Discussionmentioning

confidence: 99%

“…However, in some cases, instead of a single opening located posteriorly, the UGS is deviated posteriorly, to open just anterior to a normal anus. Although this anatomical variation should not be labeled a “cloaca” by the classical definition, due to the posterior mislocation of the UGS, it can be considered as a part of the spectrum of “posterior cloacal malformations (PCMs).”[ 3 ] Furthermore, such malformations are frequently associated with genitourinary anomalies and hence merit a thorough genital examination and a complete urological evaluation. This series outlines our experience of management of these rare and complex malformations.…”

Section: Introductionmentioning

confidence: 99%

See 4 more Smart Citations

Posterior Cloaca

Mandelia

Kapoor

Gupta

et al. 2022

Journal of Indian Association of Pediatric Surgeons

View full text Add to dashboard Cite

Aim: Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations. Methods: This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed. Results: During the study period, four girls with a median age of 2 (range: 0–5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of “urorectal septal defect.” On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos ( n = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra ( n = 3); (c) uterine didelphys ( n = 1); (d) bilateral grade 5 vesicoureteric reflux ( n = 1); and (e) vaginal calculus ( n = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus ( n = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained. Conclusions: PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome.

show abstract

Section: Discussionsupporting

confidence: 82%