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Abstract
Fetal abdomino-pelvic cystic lesions are uncommon and can have varied etio-pathogenesis.
Most commonly they originate from the gastrointestinal or genitourinary tract. These
include choledochal cyst, hydronephrosis, renal cyst, mesenteric/omental cyst, ovarian
cyst, meconium pseudocyst, and hydrocolpos/hydrometrocolpos among others. Fetal hydrometrocolpos
is rare with a reported incidence of 0.006% and its diagnosis requires a high index
of suspicion. Antenatal ultrasound and magnetic resonance imaging (MRI) is invaluable
in diagnostic evaluation. This case report describes the imaging features of antenatally
detected congenital hydrometrocolpos with Mullerian duplication secondary to cloacal
malformation using antenatal ultrasound and MRI. Per-operative findings and other
possible differential diagnoses are discussed along with a brief review of literature.
The aim of this study was to describe lessons learned from the authors' series of patients with cloaca and convey the improved understanding and surgical treatment of the condition's wide spectrum of complexity. The medical records of 339 patients with cloaca operated on by the authors were retrospectively reviewed. A total of 265 patients underwent primary operations, and 74 were secondary. All patients were approached posterior sagittally; 111 of them also required a laparotomy. The average length of the common channel was 4.7 cm for patients that required a laparotomy and 2.3 cm for those that did not. Vaginal reconstruction involved a vaginal pull-through in 196 patients, a vaginal flap in 38, vaginal switch in 30, and vaginal replacement in 75 (36 with rectum, 31 with ileum, and 8 with colon). One hundred twenty-two patients underwent a total urogenital mobilization. Complications included vaginal stricture or atresia in 17, urethral strictures in 6, and urethro-vaginal fistula in 19, all of which occurred before the introduction of the total urogenital mobilization. A total of 54% of all evaluated patients were continent of urine and 24% remain dry with intermittent catheterization through their native urethra and 22% through a Mitrofanoff-type of conduit. Seventy-eight percent of the patients with a common channel longer than 3 cm require intermittent catheterization compared with 28% when their common channel was shorter than 3 cm. Sixty percent of all cases have voluntary bowel movements (28% of them never soiled, and 72% soiled occasionally). Forty percent are fecally incontinent but remain clean when subjected to a bowel management program. Forty-eight patients born at other institutions with hydrocolpos were not treated correctly during the neonatal period. The surgeons failed to drain the dilated vaginas, which interfered with the drainage of the ureters and provoked urinary tract infections, pyocolpos, and/or vaginal perforation. In 24 patients, the colostomy was created too distally, and it interfered with the pull-through. Twenty-three patients suffered from colostomy prolapse. All of these patients required a colostomy, revision before the main repair. Thirty-six patients underwent reoperation because they had a persistent urogenital sinus after an operation done at another institution, and 38 patients underwent reoperation because they suffered from atresia or stenosis of the vagina or urethra. The series was divided into 2 distinct groups of patients: group A were those with a common channel shorter than 3 cm (62%) and group B had a common channel longer than 3 cm (38%). The separation of these groups has important therapeutic and prognostic implications. Group A patients can be repaired posterior sagittally with a reproducible, relatively short operation. Because they represent the majority of patients, we believe that most well-trained pediatric surgeons can repair these type of malformations, and the prognosis is good. Group B patients (those with a common channel longer than 3 cm), usually require a laparotomy and have a much higher incidence of associated urologic problems. The surgeons who repair these malformations require special training in urology, and the operations are prolonged, technically demanding, and the functional results are not as good as in group A. It is extremely important to establish an accurate neonatal diagnosis, drain the hydrocolpos when present, and create an adequate, totally diverting colostomy, leaving enough distal colon available for the pull-through and fixing the colon to avoid prolapse. A correct diagnosis will allow the surgeon to repair the entire defect and avoid a persistent urogenital sinus. Cloacas comprise a spectrum of defects requiring a complex array of surgical decisions. The length of the common channel is an important determinant of the potential for urinary control, and predicts the extent of surgical repair.
Abstract Subdivision of cloaca into urogenital and anorectal passages has remained controversial because of disagreements about the identity and role of the septum developing between both passages. This study aimed to clarify the development of the cloaca using a quantitative 3D morphological approach in human embryos of 4–10 post‐fertilisation weeks. Embryos were visualised with Amira 3D‐reconstruction and Cinema 4D‐remodelling software. Distances between landmarks were computed with Amira3D software. Our main finding was a pronounced difference in growth between rapidly expanding central and ventral parts, and slowly or non‐growing cranial and dorsal parts. The entrance of the Wolffian duct into the cloaca proved a stable landmark that remained linked to the position of vertebra S3. Suppressed growth in the cranial cloaca resulted in an apparent craniodorsal migration of the entrance of the Wolffian duct, while suppressed growth in the dorsal cloaca changed the entrance of the hindgut from cranial to dorsal on the cloaca. Transformation of this ‘end‐to‐end’ into an ‘end‐to‐side’ junction produced temporary ‘lateral (Rathke's) folds’. The persistent difference in dorsoventral growth straightened the embryonic caudal body axis and concomitantly extended the frontally oriented ‘urorectal (Tourneux's) septum’ caudally between the ventral urogenital and dorsal anorectal parts of the cloaca. The dorsoventral growth difference also divided the cloacal membrane into a well‐developed ventral urethral plate and a thin dorsal cloacal membrane proper, which ruptured at 6.5 weeks. The expansion of the pericloacal mesenchyme followed the dorsoventral growth difference and produced the genital tubercle. Dysregulation of dorsal cloacal development is probably an important cause of anorectal malformations: too little regressive development may result in anorectal agenesis, and too much regression in stenosis or atresia of the remaining part of the dorsal cloaca.
Hydrocolpos is a condition rarely mentioned in the literature. The purpose of this report is to increase the index of suspicion for hydrocolpos in patients with cloaca and to describe our approach for its treatment with the hope that errors in the management of hydrocolpos can be avoided. We reviewed 411 medical records of patients diagnosed with cloaca and managed at our Center during the last 26 years. Emphasis was placed on evaluating for the presence of hydrocolpos, type of drainage, and complications related to the persistence of the hydrocolpos. One hundred seventeen cloaca patients had an associated hydrocolpos (28.4%). Forty-two cases (36%) were initially managed at other institutions at which the hydrocolpos was not drained. Complications experienced by this group included: multiple urinary tract infections (8), hydrocolpos infection (7), sepsis (7), failure to thrive (6), ruptured hydrocolpos (4), and development of hydronephrosis in previously normal kidneys (2). Forty-one patients (35%) had other modalities of treatment, aimed to drain the hydrocolpos, including vesicostomy (26), intermittent perineal catheterization (8), single aspiration (6), or plasty of the perineal orifice (1). In all of these cases, the hydrocolpos persisted or reaccumulated. Thirty-four patients (29%) underwent an effective drainage of the hydrocolpos at birth; 29 at other institutions, 15 with a tube vaginostomy, 13 with a tubeless vaginostomy, and 1 with a catheter placed and left in the vagina through cystoscopy. Five cases had a tube vaginostomy done by us. In all these cases, the vagina remained adequately drained as demonstrated radiologically. Proper drainage of the hydrocolpos alone, with no urologic intervention, dramatically improved the hydronephrosis in 13 cases. Hydrocolpos in patients with cloacas must be diagnosed and treated early in life. Our preferred approach is a transabdominal indwelling vaginostomy tube. The drainage of the hydrocolpos alone may dramatically improve the hydronephrosis, and therefore, we suggest that only after the hydrocolpos is drained should a urological intervention be contemplated. Failure to drain the hydrocolpos can result in serious complications. Copyright 2010 Elsevier Inc. All rights reserved.
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