Persistent Cloaca: APSA Prenatal Counseling Series

American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca TM from the Fetal Diagnosis and Treatment Committee of the American Pediatric Surgical Association Editor-in-Chief: Ahmed I. Marwan, MD Special thanks to Alberto Peña, MD, Andrea Bischoff, MD, Mariana Meyers, MD, and Carolina Guimaraes, MD ©2018, American Pediatric Surgical Association American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca Persistent Cloaca • Persistence of the early embryological stage of a common opening for rectum, vagina and urinary tract • Rare anomaly: 1:50,000 female births • Differential diagnosis: Fetal Abdominal Cysts (Please refer to Fetal Abdominal Cysts Brochure) Embryological facts of cloaca: o Development starts at 3 weeks o U-shaped endodermal cavity where hind gut, allantois, tail gut and later on the mesonephric ducts open o Carried anteriorly upon folding of the embryo o Cloaca is at first closed by the cloacal membrane which reaches up to the umbilical ring o At 6th week: Urorectal septum descends down to fuse with the cloacal membrane and the lateral folds -> Urogenital cavity anteriorly and anorectum posteriorly o Rapid growth of the genital tubercle displaces the cloacal membrane posteriorly o At 7th week: cloacal membrane breaks down creating 2 openings o Muscles surrounding the rectum develop at the same time (6th and 7th weeks) o No sexual differentiation until 9th weeks’ GA American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca Prenatal Considerations • Most common prenatal presentation: Fetal abdominal cystic mass • Cystic mass may be the hydrocolpos (50% of cases) or associated bladder outlet obstruction (BOO) • Duplicated vagina and uterus didelphys commonly seen • Prenatal US findings are 2ry to underlying anomaly o Septate or bilateral cystic pelvic mass is the most common Mullerian tube anomally associated with persistent cloaca o Hydrocolpos posterior to the bladder o Vesicomegaly and hydroureteronephrosis • Associated with increased incidence of other anomalies: ascites, urogenital, cardiac, GI, and skeletal Images courtesy of Mariana Meyers, MD Colorado Fetal Care Center - Children’s Hospital Colorado American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca Accurate Diagnosis: T argeted US, fetal echocardiography, fetal MRI and amniocentesis • It is important to make the distinction between hydrocolpos and distended urinary bladder • Urinary ascites may be seen if there is drainage via the fallopian tubes • MRI may be extremely helpful to delineate the anatomy and evaluate for associated anomalies Fetal Intervention Indications for prenatal decompression o Significant urinary obstruction leading to pulmonary hypoplasia – (This is a rare event) o Drainage of the hydrocolpos or peritoneal cavity may relieve obstruction Very few reports of antenatal fetal intervention for persistent cloaca with hydrocolpos exist (Shimada et al., 2001) American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca Postnatal Management • Newborn screening for patients with anorectal malformation Source: Colorado Fetal Care Center and The International Center for Colorectal and Urological Care – Children’s Hospital Colorado Source: Colorado Fetal Care Center and The International Center for Colorectal and Urological Care – Children’s Hospital Colorado American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca • After ruling out important associated anomalies during the first 24 hours of life, the patient should be taken to the operating room for a diverting descending colostomy and hydrocolpos drainage, if indicated. • The location of the proximal stoma should be in the center of a triangle formed by the umbilicus, the last rib and the top of the iliac crest (figure 1). The mucous fistula should be tapered to avoid prolapse and should be placed medially, and with enough distance from the proximal stoma, to allow for the stoma bag to only cover the proximal stoma. • An important and time-consuming portion of the operation is the cleaning of the distal bowel. This can be performed by inserting a catheter into the center of a purse string suture (figure 2) and irrigate it with saline solution until all the meconium is removed, the bowel is collapsed and well perfused. • When the hydrocolpos is small it can be drained through the same oblique incision done for the colostomy (figure 3). When there is a large bilateral hydrocolpos a midline infra-umbilical incision is preferred (figure 4). • During hydrocolpos drainage the surgeon should palpate the cervix (cervices) to assure that the opening for the tube insertion is done in the vagina. A pigtail catheter or a Foley catheter can be used, a purse string suture is recommended and, when the anatomy allows for it, the vagina should be tacked to the abdominal wall. The tube should remain in place until the main reconstruction happens. Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage [please arrange the above 4 images so they’re spaced nicely etc. caption = ©Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado] - High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage [please arrange the above 4 images so they’re spaced nicely etc. caption = ©Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado] - High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage [please arrange the above 4 images so they’re spaced nicely etc. caption = ©Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado] - High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage [please arrange the above 4 images so they’re spaced nicely etc. caption = ©Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado] - High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage © Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado American Pediatric Surgical Association Prenatal Counseling Series Persistent Cloaca • High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common channel; for surgical planning. • Panendoscopy to accurately measure the common channel • Multidisciplinary approach • Length of common channel varies from 1-12cm (Pena et al., 2004 – Rich et al., 1988) Figure 1: Descending colostomy and mucous fistula Figure 2: Cleaning of the distal bowel during colostomy opening Figure 3: Oblique incision for colostomy opening and hydrocolpos drainage Figure 4: Midline incision for colostomy opening and hydrocolpos drainage [please arrange the above 4 images so they’re spaced nicely etc. caption = ©Images courtesy of Alberto Pena and Andrea Bischoff, Children’s Hospital Colorado] - High pressure distal colostogram is indicated, once the baby is growing well, with simultaneous injection through the vaginostomy tube, if present, and/or common ©Images courtesy of Children’s Hospital Colorado