Cysto-Vaginoscopy of a 3D-Printed Cloaca Model: A Step toward Personalized Noninvasive Preoperative Assessment in Patients with Complex Anorectal Malformations

Introduction For the classification of the complexity of cloacal malformations and the decision on the operative approach, an exact anatomical assessment is mandatory. To benefit from using three-dimensional (3D)-printed models in preoperative planning and training, the practicability of these models should be guaranteed. The aim of this study was to evaluate the quality and feasibility of a real-size 3D-printed cloaca model for the purpose of cysto-vaginoscopic evaluation. Materials and Methods We performed a 3D reconstruction and printed a real-size, rubber-like 3D model of an infant pelvis with a cloacal malformation and asked invited pediatric surgeons and pediatric urologists to perform a cysto-vaginoscopy on the model and to complete a brief questionnaire to rate the quality and feasibility of the model and to indicate whether they would recommend the model for preoperative planning and training. Results Overall, 41 participants rated the model quality as good to very good (M = 3.28, standard deviation [SD] = 0.50, on a scale from 1 to 4). The model was rated as feasible for preoperative training (M = 4.10, SD = 0.75, on a scale from 1 to 5) and most participants (85.4%) would recommend the model for preoperative training. The cysto-vaginoscopy of the model was considered as a valid training tool for real-life cases and improved the confidence on the anatomy of a cloaca. Conclusion The results of our study indicate that patient-specific 3D-printed models might be a useful tool in the preoperative evaluation of complex anorectal malformations by simulation of cysto-vaginoscopy with an excellent view on anatomical structures to assess the whole spectrum of the individual cloacal malformation. Our model might be a valuable add-on tool for specialty training in pediatric colorectal surgery.

Prenatale echografische diagnose van een cloacale malformatie

Prenatal ultrasound diagnosis of a cloacal malformation Cloacal malformations are a spectrum of rare congenital disorders caused by the arrest of the cloacal division during different stages in early embryogenesis. They necessitate critical intervention at birth, posing important surgical challenges. The majority unfortunately remains undetected during prenatal screening. Knowledge about sonographic red flags, however, may improve antenatal detection and therefore postnatal outcome as a result of a timely referral to multidisciplinary tertiary centers. A 29-year-old patient presents at the hospital due to decreased fetal movements in the third trimester of pregnancy. The ultrasound reveals an anechoic bilobed cystic mass with a thick midline septum within the pelvis of a female fetus. The diagnosis of a cloacal anomaly with associated duplicated vaginas and uteri is made. Severe accumulation of urine lies at the base of bilateral hydrocolpos, peritoneal ascites and a urinary tract obstruction with an adverse influence on both pulmonary and renal development. The baby is prematurely delivered by an emergency caesarean section. Her condition is poor, requiring immediate respiratory resuscitation. An explorative laparotomy on the following day confirms the diagnosis. The first steps towards anatomical correction are taken. It may be concluded that the triad of bilateral hydronephrosis, a pelvic cystic mass and an absent normal bladder within a female fetus should always raise suspicion of a cloaca.

Preoperative evaluation of persistent cloaca using contrast-enhanced ultrasound in an infant

Persistent cloaca (PC) is the most intricate anorectal malformation. Contrast-enhanced ultrasound (CEUS) is safe for hepatic, renal, splenic, vascular, and intracavitary assessment in children, but it is little applied for cloacal malformation. Our results demonstrate that CEUS can not only display the uterine, vagina, bladder, urethra, rectum, and rectovaginal fistula, but also measure the length of the common channel, bladder neck to the common channel, and distal urethra to the perineum. To our knowledge this may be the first report of preoperative evaluation by CEUS in an infant with PC.

Cloacal Malformation in Female Children: Outcome of Initial Management

Objective: To document our experience of initial management of Cloacal malformation in female patients. Methods: A descriptive retrospective study was conducted in the Department of Pediatric Surgery of the National Institute of Child Health Karachi from January 2010 to September 2018. Female patients with diagnosis of Cloacal malformation were included in this study. Data regarding the age at presentation, mode of presentation, clinical features, presence of hydrocolpos, and associated anomalies were noted. Surgical procedures performed in these patients and the outcomes were also documented. Data was analyzed on SPSS Version 20. Results: Sixty females were included in the study. Age ranged from birth to three years with a median of four days. Patients admitted through emergency were 44 (73.33%) while 16 (26.66%) were admitted through outpatient clinic. Hydrocolpos was found in 15 (25.00%) patients. Five (8.33%) patients had massive abdominal distention and were presented with severe respiratory distress. Among them three had massive hydrocolpos, one patient had pneumoperitoneum secondary to Meckel’s perforation and one patient was having massive colonic dilatation. Hydronephrosis and hydroureter were found in 14 (23.33%) patients, while dilated bladder was found in three (5.00%) patients. After optimization of patients, bowel diversion was done as transverse colostomy in 39 (65.00%) patients, high sigmoid colostomy in 17(28.33%) patients while ileostomy was done in three (5.00%) patients. All patients with hydrocolpos had tube vaginostomy. None of the patients required bladder diversion and urinary tract dilatations were gradually subsided in nine patients in the post-operative period. Associated anomalies were found in 22(36.66%) of cases predominantly of sacral origin. Operative complications were found in 18(30.00%) patients, including stoma site in infection in 12(20.00%) patients, vaginal tube dislodgement in two patients, wrong placement of tube in one patient and vaginostomy stenosis in one case. While bowel stoma retraction occurred in four patients, prolapsed was found in three patients. Renal failure occurred in one patient. In total, 37(61.00%) patients had smooth recovery, 18(30.00%) patients had some complications, while Five (8.33%) patients were expired due to sepsis. Conclusions: Majority of cloacal malformations present in neonatal life. Initial management is an important step in dealing with these patients. Care must be taken during the abdominal exploration regarding drainage of hydrocolpos and appropriate placement of bowel stoma. doi: https://doi.org/10.12669/pjms.36.2.1095 How to cite this:Zamir N. Cloacal Malformation in Female Children: Outcome of Initial Management. Pak J Med Sci. 2020;36(2):---------. doi: https://doi.org/10.12669/pjms.36.2.1095 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.