Obstetrical Outcomes in Adult Patients Born with Complex Anorectal Malformations and Cloacal Anomalies: A Literature Review

Abstract

a b s t r a c tPatients born with complex anorectal malformations often have associated M€ ullerian anomalies, which might affect fertility and obstetrical outcomes. Other vertebral-anorectal-tracheoesophageal-renal-limb associations, such as renal or cardiac anomalies, could also affect pregnancy intention, fertility rates, and recommendations about mode of delivery or obstetrical outcomes. Associated conditions present at birth, like hydrocolpos, could also potentially affect fertility. Depending on the complexity of the anomaly, primary reconstruction might include vaginoplasty, vaginal interposition, perineal body reconstruction, and extensive pelvic dissection. After the initial reconstruction, patients might have multiple additional surgeries for stoma reversal, bladder augmentation, and creation of conduits, all with potential for pelvic adhesions. Pregnancy intention, fertility rates, mode of delivery, and obstetrical outcomes data are limited in this patient population, making it challenging to counsel patients and their families. We sought to evaluate all available literature in an attempt to better counsel families. A PubMed literature search was undertaken to review this topic. Search terms of "cloaca," "anorectal malformation," "pregnancy," "cloacal exstrophy," "vaginal delivery," and "cesarean section" were used and citation lists from all identified articles were checked to ensure that all possible articles were included in the review. We also outline comorbidities from the fetal period to adulthood that might affect reproductive health. Of the articles on anorectal and cloacal anomalies, 13 reports were identified that covered obstetrical outcomes. They were in patients with previous anorectal malformation, cloaca, and cloacal exstrophy repair. Twenty-four pregnancies were reported in 16 patients. Two ectopic pregnancies, 5 spontaneous miscarriages, 1 triplet pregnancy, and 16 singleton pregnancies were reported with a total of 19 live births. Regarding the method of conception, 15/18 pregnancies occurred spontaneously and 3/18 were via assisted reproductive technology with in vitro fertilization. There were 19 live births, of which at least 8 were preterm. M€ ullerian anatomy was reported in 8 of 13 articles. Only 2 patients underwent vaginal delivery (1 patient with repaired cloaca malformation had an operative vaginal delivery and 1 patient with repaired imperforate anus with rectovaginal fistula had a normal spontaneous vaginal delivery). The remaining patients all underwent a cesarean section. There were no reported cases of maternal mortality, and maternal morbidity was limited to recurrent urinary tract infections and worsening chronic kidney disease. There is a paucity of information regarding obstetrical outcomes in adult anorectal and cloaca patients. However, patients with previous cloacal repairs have achieved pregnancy spontaneously, as well as with in vitro fertilization. Patients with repaired cloacal malformations are at increased risk of preterm birth and cesarean delivery. Most p...

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation

“…More than half of patients born with a cloacal malformation have an associated Müllerian anomaly, most commonly uterine didelphys, but other anomalies including unicornuate uterus and uterine agenesis have been reported as well [4,8]. We reviewed different Müllerian anomalies such as number of cervices, presence of vaginal septum, uterine malformations and somewhat surprisingly did not find any significance in these variables to predict the need of vaginal replacement in our population.…”

Müllerian duct anomalies with term pregnancy: a case report

“…Other congenital abnormalities commonly associated with MDAs include vertebral body anomalies (29%) (that is, fused or wedged vertebral bodies, spina bifida; 22–23%), heart abnormalities (14.5%), and syndromes such as the Klippel-Feil syndrome (7%) [ 6 ]. Other conditions involving vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies, and limb abnormalities (VACTERL association), such as renal or cardiac anomalies in patients with MDAs, could also affect their conception, fertility, modes of delivery, and obstetrical outcomes [ 7 ].…”

Reconsidering Diagnosis, Treatment, and Postoperative Care in Children with Cloacal Malformations

“…57 A recent review article reported on all available obstetrical outcomes in the literature in patients with CM. 58 There were no maternal mortalities and the most common complications were recurrent urinary tract infections and premature deliveries secondary to spontaneous preterm labor. Patients with CM had varying modes of delivery including vaginal delivery, operative vaginal delivery, and cesarean section.…”