A 26-year-old gravida 1, para 0 pregnant woman was referred to the maternal fetal care center (MFCC) at 19 weeks’ gestation because of concern for bladder exstrophy. Fetal ultrasonography followed by fetal magnetic resonance imaging (MRI) performed at 18 4/7 weeks’ gestation (Fig 1) showed a large cyst of the inferior anterior abdominal wall, an absent bladder, bilateral clubfoot, suspicion for pelvic left kidney, a small right kidney, and a 2-vessel umbilical cord. This was a naturally conceived and uneventful pregnancy. Amniocentesis before referral showed a normal female karyotype, 46,XX, elevated α-fetoprotein, and normal acetylcholinesterase level. The microarray was normal. The maternal medical history was noncontributory. The family history was notable for a maternal grandmother with cleft palate. The patient was a nonsmoker without a history of past or current drug use, and her only medication was prenatal vitamins. Her prenatal laboratory findings were remarkable for group B Streptococcus positivity.The first MFCC visit was at 21 5/7 weeks’ gestation and included fetal echocardiography, fetal MRI, and fetal ultrasonography. Fetal echocardiography showed normal cardiac structure and function. The fetal MRI (Fig 2) was significant for oligohydramnios, an empty left renal fossa, a left-sided low pelvic kidney crossed fused to the right kidney without hydroureter or hydronephrosis, no identified urinary bladder, a 2-vessel cord with a large cyst emanating from the lower pelvis, bilateral clubfoot, low termination of the spinal cord, and differential signal intensity of the left lung suggesting possible nutmeg lung or lymphangiectasia. The fetal brain was normal. Fetal ultrasonography confirmed the aforementioned findings with the addition of a small intrapelvic fluid collection that could be a small decompressed bladder. The differential diagnosis of the abdominal cystic midline mass included bladder exstrophy and an omphalocele versus urachal cyst.Repeat fetal MRI and fetal ultrasonography at 24 2/7 weeks’ gestation showed an increase in the amniotic fluid volume with a low normal amniotic fluid index (AFI), but also a new finding of an omphalocele (Figs 3 and 4) containing large bowel, small bowel, and a small amount of liver. In addition, there was continued presence of a right crossed fused ectopic kidney (Fig 5), absent urinary bladder, 2-vessel cord, low termination of the spinal cord to L5, and slightly less severe left clubfoot with a right foot that was nearly normal in appearance. The previously viewed abdominal cystic structure had disappeared. The combination of these findings raised concern for bladder exstrophy or the omphalocele, exstrophy of the cloaca, imperforate anus, and spinal defects (OEIS) complex, previously known as cloacal exstrophy.Ultrasonography performed at 29 5/7 weeks’ gestation was consistent with prior imaging, though the amount of amniotic fluid at this visit was below normal, with an AFI of 6.5 cm, compared to 9.5 cm at 24 2/7 weeks’ gestation. The omphalocele was redemonstrated and contained most of the large and small bowel, a small amount of the liver, and the gallbladder.Because of persistent oligohydramnios and intrauterine growth restriction (0 percentile), the patient was admitted for monitoring at 31 3/7 weeks’ gestation and given a full course of betamethasone to accelerate lung development. She was briefly readmitted at 32 4/7 weeks’ gestation to exclude preterm premature rupture of membranes in the setting of a low AFI. She had multiple reassuring nonstress tests and was discharged from the hospital. She later presented at 34 2/7 weeks’ gestation with preterm premature rupture of membranes; tocolysis was not performed because of oligohydramnios and growth restriction. The infant was delivered via a classic cesarean section for breech presentation and the known multiple anomalies.Patients seen in the MFCC at our institution receive multidisciplinary care by physicians from various specialties, who develop a comprehensive plan for the fetus, including timing and mode of delivery, delivery room management, and postnatal care. Neonatologists met with the family at 28 3/7 weeks’ gestation and provided counseling regarding the plan for immediate postnatal management, including resuscitation, protection of any exposed organs, peripheral intravenous catheter placement, and the presence of the pediatric surgery team at delivery in addition to neonatology. The multidisciplinary team included pediatric colorectal surgery, urology, orthopedic surgery, neurosurgery, and cardiology. Below is a summary of the evaluations, decision-making approach, and suggested management plans from the perspective of each subspecialty. Overall, both the family and providers remained optimistic about the prognosis, and per chart review, pregnancy termination was not discussed.Surgery was the primary consulting service throughout this patient’s MFCC course. Of utmost concern initially was oligohydramnios, given the importance of adequate amniotic fluid volume for lung development; however, the amniotic fluid volume increased by 24 weeks’ gestation. Despite extensive review of initial imaging, the etiology of the anterior cystic abdominal mass remained uncertain. Given the position of the cyst in the umbilical cord, the differential diagnosis included urinary bladder, omphalocele, and urachal cyst. A significant change in appearance of the anterior abdominal wall mass was noted on repeat fetal MRI at 24 2/7 weeks’ gestation, when the previous simple cyst resolved and an omphalocele became evident. This change then suggested that the most likely diagnosis was OEIS complex versus bladder exstrophy. The plan for postnatal repair was discussed with the family and would likely include separation of cecal and bladder plates, tubularization of the cecal plate, hindgut rescue, stoma creation with all of the cecum and hindgut in continuity, and need for eventual bladder reconstruction and closure. These initial surgeries would be done in collaboration with urology. The recommended mode of delivery was cesarean section given the significance of the anomalies present and to avoid damaging the externalized anatomy. Timing of delivery would depend on fetal growth and amniotic fluid levels in addition to the usual obstetric management.During the initial MFCC visit, urology also counseled the family. Due to the absence of pubic diastasis at that time, bladder or cloacal exstrophy seemed less likely, particularly because a possible bladder had been identified in the fetal pelvis. There was no sign of urinary tract dilation which was reassuring, and a plan was made to repeat imaging later in gestation. On a second MRI, the findings were more consistent with OEIS complex or cloacal exstrophy, and the family was counseled that a definitive diagnosis could only be made after delivery on examination of the infant. Postnatal repair was predicted to involve multiple reconstructive surgeries, the first of which would be separation of the bowel from the bladder, closure of the posterior bladder wall with the inner surface remaining exposed on the lower abdomen, and tubularization of the cecal plate with end colostomy and stoma creation by pediatric colorectal surgery. However, it was again stressed to the family that the exact type of repair would depend on the infant’s physical examination findings at birth. Postnatally, the infant would need nonurgent renal ultrasonography to evaluate for urinary tract dilation and radiography to assess for pubic diastasis. In the delivery room, the bladder and cecal plates would be kept moist to prevent trauma.Fetal MRI at 21 5/7 weeks’ gestation showed the spinal cord below its expected lowest normal position of L3, raising a concern for a tethered cord. There was no evidence of spina bifida or Chiari malformation, and the brain was normal. Neurosurgery recommended head ultrasonography after birth, followed by a spinal MRI at a later date with surgery as needed, in coordination with other involved subspecialists.Orthopedic surgery met with the family to discuss the treatment and outcome of bilateral clubfoot, which were initially graded as moderate in degree. The treatment recommended was the Ponseti method, which consists of nonemergent sequential casting (5–8 casts) in the first few weeks after birth followed by a heel cord tenotomy and repositioning. Once the feet are repositioned, children often require braces and exercises until about 4 years of age. The family was advised that functional outcomes are quite good and limitations minimal. On subsequent imaging, the right foot appeared normal, and the left foot appeared slightly improved. The plan was to consult orthopedics after delivery given the nonemergent nature of this finding.A complete fetal echocardiogram showed normal biventricular function and structure. Given the presence of multiple congenital anomalies and inherent limitations of fetal echocardiography, postnatal follow-up echocardiography was recommended during the first few weeks after birth. No further fetal follow-up was planned.The female infant was born via cesarean delivery at 34 2/7 weeks’ gestation and was small for gestational age, with a birthweight of 2,080 g (less than the 1st percentile), length of 44 cm (less than the 1st percentile), and head circumference of 31 cm (less than the 1st percentile). She emerged with some cry and fair tone. Resuscitation involved less than 1 minute of positive pressure ventilation for a heart rate of less than 100 beats/min followed by transition to continuous positive airway pressure (CPAP) for mild respiratory distress. The infant’s Apgar scores were 6 and 9 at 1 and 5 minutes, respectively. The lower half of the infant including exposed viscous was placed in a plastic bag similar to that used for gastroschisis protection and moistened with sterile saline. The postnatal examination findings were significant for a moderate-sized omphalocele, exstrophy with bladder and cecum exposed, and an imperforate anus (Figs 6 and 7). She was transferred on CPAP to the adjacent children’s hospital for further surgical management.The infant was taken to the operating room (OR) on the first day after birth for a stage I OEIS repair, which included omphalocele repair, stage 1 repair of the cloacal exstrophy with hindgut rescue and a colostomy, the Ladd procedure, and gastrostomy placement (Fig 8). Pediatric surgery and urology first examined the infant with the administration of general anesthesia to clarify the anatomy. The omphalocele contained small bowel covered by a thin sac with a breech in the integrity. The cecal plate and prolapsed ileum had a distinct border of what appeared to be the bladder halves laterally and inferiorly. A separate type of mucosa was noted, which appeared to be vaginal in nature with a small orifice that looked like a cervix at the inferior border. An additional orifice consistent with a prolapsed fallopian tube was also present. At the inferiormost aspect, a single perineal opening was seen, and a 5-French feeding tube was passed to the exstrophy plate and represented a urogenital sinus. The right ureteral orifice was also identified.The cecal plate was marked out and subsequently separated from the bladder plate. The omphalocele sac was removed in its entirety after ensuring that the abdomen would likely be able to be closed. The entire gut was then run and malrotation was noted; a Ladd procedure was performed without appendectomy given that the appendix might be needed in the future for urologic repair or antegrade enemas. The hindgut emerged from behind the cecal plate and was lifted off the sacrum to allow for an end colostomy. The blood supply from the hindgut emerged posteriorly and was preserved.Once the cecal plate was separated from the bladder halves, it was tubularized with a 2-layer anastomosis and was in continuity with the hindgut. The end of the hindgut was then brought up for the end colostomy. Of note, 2 hemiuteri were identified in the right abdomen during the exploration. A 14-French Malecot gastric tube was placed for nutritional support postoperatively.To close the abdomen, the superior aspect of the fascia was closed down to the level of the bladder plates and the 2 bladder halves were brought together at the inferior aspect of the abdomen. The bladder plate was covered with a large transparent film dressing and the superior skin was closed with reinforced wound closure strips. The infant remained intubated and was transferred from the OR to the NICU for further management.The infant was admitted from the delivery room on CPAP. The initial chest radiograph showed low lung volumes and evidence of mild respiratory distress syndrome with superimposed transient tachypnea of the newborn. After the staged OEIS and cloacal exstrophy repair, as detailed before, the infant remained stable with the exception of intraoperative hypotension that briefly required dopamine. On postoperative day 6, the infant was noted to have dehiscence of her lateral closure of the bladder plates with bowel herniation, for which she was taken back to the OR for revision with fascial closure, lateralization of the colostomy, and redo of the vesicocutaneous suture line.She underwent intubation for both her initial surgery and subsequent surgery for wound dehiscence. On both occasions, the first attempt at extubation was unsuccessful and she required reintubation. However, extubation was eventually successful and she was transitioned to CPAP after a short course of dexamethasone dosed for airway edema. She was weaned to room air at 16 days of age and was stable in room air for the remainder of her hospitalization.Her birth admission was also notable for exposure to fentanyl and intravenous dexmedetomidine hydrochloride, which were weaned gradually and then discontinued at 16 days of age; a postoperative mild acute kidney injury that resolved without intervention; iatrogenic anemia requiring a red blood cell transfusion; and indirect hyperbilirubinemia that resolved with phototherapy. In addition, she received cefoxitin for 48 hours on 2 occasions: after her first surgery until her ureteral stent was removed and postoperatively after repair of the wound dehiscence. Enteral trophic feedings were started at 15 days of age via the surgical gastrostomy tube and advanced to total feedings without difficulty. Parenteral nutrition was discontinued at 25 days of age and lipids were stopped 2 days later; she received most of her feedings via her surgically placed gastrostomy tube, but would take up to one-third of her feeds orally. After extensive parent education, the infant was discharged from the hospital with an end colostomy, gastrostomy tube, and a plan to use a transparent film dressing to protect her bladder plate.Preoperative echocardiography showed normal cardiac structure and function with a patent ductus arteriosus and patent foramen ovale. Postnatal renal ultrasonography confirmed the prenatal finding of crossed fused renal ectopia (Fig 9). Spinal ultrasonography confirmed the presence of a tethered cord (Fig 10), and neurosurgery follow-up with a spine MRI was scheduled for 3 months of age. Orthopedics was consulted with a plan to cast her left clubfoot a few weeks after discharge, and they also planned to follow her pelvic diastasis (Fig 11). Lastly, there was concern about developmental dysplasia of the hip versus immaturity, and repeat hip ultrasonography was scheduled for 2 months of age.At 3 months of age, the infant had a second-stage cloacal exstrophy repair, including bladder closure, omphalocele closure, epigastric hernia repair, urethroplasty, and advancement vaginoplasty. Following this surgery, she was able to void normally via her urethra. She had serial casting of her left clubfoot followed by heel cord tenotomy at 2 months of age and additional casting. She is currently in the bracing phase of the Ponseti protocol, wearing her braces 12 hours per day, and her foot has corrected well. She is ambulating with a normal toddler gait but with a slightly wider base likely because of her pelvic diastasis, which measures 4 cm. There are no plans to correct this surgically as her bladder is functioning normally at this time. Ultrasonography of her hip at 6 weeks of age had findings consistent with physiologic immaturity and no further intervention was required. Her spinal MRI at 6 weeks of age was consistent with prior imaging and showed a low-lying tethered spinal cord with posterior lipoma. At 17 months of age, she underwent a laminectomy with release of her tethered spinal cord and excision of the aforementioned lipoma.The child is now 23 months old and doing well overall; she is growing and developing appropriately, eats and drinks by mouth, and recently had her gastrostomy tube removed. The most pertinent issue at this time is a recurrent Clostridium difficile infection that has been treated with 5 courses of vancomycin unsuccessfully with fecal microbiota transplantation planned.OEIS complex is a rare syndrome consisting of omphalocele, exstrophy, imperforate anus and spinal defects. (1) Previously referred to as cloacal exstrophy, it is the severe end of the spectrum of genitourinary malformations referred to as bladder exstrophy epispadias complex (BEEC). (2) The less severe end of the spectrum is epispadias or nonclosure of the urethral plate. Classic bladder exstrophy, which is a lower abdominal wall defect with an evaginated bladder plate, represents the intermediate part of the spectrum. (2) Children with OEIS complex may also have limb defects, genital defects, and müllerian duct abnormalities. (1)(3) The incidence of OEIS syndrome is reported to be between 1 in 200,000 and 1 in 250,000 live births, but the true incidence has been hypothesized to be higher due to spontaneous abortions, stillbirths, and elective terminations. (1) A case series in 2007 reported a much higher incidence of 1 in 27,174 liveborn infants, (4) and others have reported estimates between 1 in 10,000 and 1 in 70,000 live births. (5) This syndrome includes a constellation of birth defects that pose both diagnostic and management challenges for clinicians and quality-of-life challenges for children and their families. Despite these difficulties, the prognosis for OEIS complex can be excellent at experienced centers, and thus pregnancy termination is not routinely recommended. (2) As a result, there has been a goal shift from survival to improving cosmetic and functional outcomes for these children.The etiology of OEIS complex is indeterminate. Most cases are sporadic, but recurrence in siblings and occurrence in monozygotic twins has been documented, suggesting a genetic component. (6) Individual cases have been associated with chromosomal abnormalities, but a single recurrent cause has not been identified. (5)(7) Indeed, most infants have a normal karyotype. (8) Microarray studies, including high-resolution single nucleotide polymorphism and oligonucleotides performed on a case series of 13 patients with confirmed OEIS complex have not revealed a recurrent aberration. (7) The same study assessed 14 proposed candidate genes implicated in caudal or urogenital malformations in humans or mouse models and did not find any disease-causing mutations. (7) Certain environmental associations have been identified, such as maternal diazepam use, (1) maternal clomiphene use, (9) any fertility treatment, and maternal obesity with a body mass index greater than 30 kg/m2. (5) Another study showed in vitro fertilization specifically to be a risk factor, with a 7.3-fold increase in incidence of exstrophy-epispadias in this population. (10)The pathogenesis is likely from a single localized defect in early mesoderm development at the time of ventral body wall closure. As a result, there is a persistent common cloaca, which leads to bladder exstrophy and incomplete spinal development. (11)(12) It has been detected on prenatal ultrasonography as early as the first trimester, but given embryologic considerations, specifically physiologic herniation of the hindgut between weeks 9 and 10 and return of herniated bowel to the abdomen by week 12, it is often diagnosed more definitively in the second trimester. Early discovery of simple cystic ventral wall masses that appear to be “empty omphaloceles” in the first trimester, as seen in this patient, warrant close monitoring for future development of OEIS complex, as suggested by a prior case report. (12)(13)OEIS syndrome is also associated with increased maternal α-fetoprotein and increased nuchal translucency in the first trimester, but both of these findings are nonspecific. (11)(12) Clinical suspicion for OEIS complex often arises during routine prenatal ultrasonography with the identification of a ventral body wall defect. (4)(14) Advancements in prenatal imaging have enabled earlier detection of OEIS complex, with most cases identified in the late first or early second trimester. Pregnant women with a concern for a fetus with OEIS complex should be referred to comprehensive quaternary care centers, where a multidisciplinary team can care for both the patient and fetus. During the prenatal period, the family receives extensive prenatal counseling, meeting with members of maternal-fetal medicine, neonatology, pediatric surgery, pediatric radiology, and pediatric urology to confirm the diagnosis and to develop a postnatal surgical plan. At our center, additional expert ultrasonography is performed with fetal MRI to further define the anomalies present.The ultrasonographic diagnosis of OEIS complex requires the following major criteria: nonvisualization of the bladder, a large midline infraumbilical anterior wall defect or cystic structure, abdominoschisis (ie, abdominal wall that is split or has a defect), and lumbosacral anomalies, all of which were present in this case. Minor criteria include lower extremity defects, renal anomalies, ascites, widened pubic symphysis, narrow thorax, hydrocephalus, and single umbilical artery (15) as well as the later added “elephant trunk sign,” which represents ultrasound visualization of the midline prolapsed ileum between the 2 hemibladders. (16) In a case series of 15 fetuses in which prenatal ultrasonography was performed between 12 and 25 weeks’ gestation, 11 of 15 had an omphalocele, 8 had nonvisualization of the bladder, 10 had a sacral mass, neural tube defect, or vertebral defect, 6 had renal defects, and 8 had lower extremity defects. (4) It is important to note that in this cohort, postnatal physical examination findings were not precisely correlated with prenatal ultrasound findings, and ultrasonography alone was associated with false-negative findings. (4) Therefore, the use of fetal MRI is important to further characterize the malformations present, especially the external and internal genital anomalies. (14) Thus, when ultrasonography is inconclusive, fetal MRI can be useful for prognosis and aid in family decision-making around continuation of pregnancy. (8)Despite expert imaging, counseling and prognosticating are particularly challenging in these infants, as the full extent of anomalies may not be definitively determined until the postnatal examination. (4) The differential diagnosis includes other ventral body wall defects such as limb-body wall complex and pentalogy of Cantrell, which carry a significantly poorer prognosis. Källén et al developed diagnostic criteria to determine the likelihood of OEIS syndrome prenatally: for infants with at least 2 OEIS malformations, the probability of having a third was 100% if the infant had an omphalocele and bladder exstrophy, 68% if the infant had an omphalocele and spine defects, and 69% if the infant had an omphalocele with anal atresia. (3) There was also a strong association with spina bifida. (3) Because of the number of anomalies present, children require extensive postnatal staged surgical repair and may have difficulties later in life, including issues of infertility, sexual identity, sexual satisfaction, and ambulation. (12)Surgical correction of OEIS syndrome is complex and requires coordination among multiple surgical subspecialities. The goals of surgical repair include omphalocele closure, bladder reapproximation, separation of the hindgut and end colostomy creation, and finally, closure of the abdominal wall. (17) Several initial considerations include single versus staged approach and immediate versus delayed repair, which depend on the severity of the underlying defects and medical stability, respectively, (17)(18) as well as consideration of the need for supplemental nutrition and upfront gastrostomy tube placement at the time of initial surgery.Neonates with a narrow pubic diastasis and mild to moderate omphalocele are candidates for a single-stage closure of the abdominal wall and bladder with a diverting colostomy and pelvic osteotomy. However, neonates with a large omphalocele and wide diastasis require a staged approach, with urology first joining the bladder halves. Closure of the bladder, placement of the bladder in the pelvis, and abdominal wall closure can be deferred until the infant is older. (17)(19) This was the approach in our case given the large omphalocele. For infants with significant pelvic diastasis greater than 4 cm, the staged approach increases the odds of successful primary closure by 4-fold and pelvic osteotomy increases the odds of success by 6-fold. (19) In a retrospective study of 112 primary closures, pelvic osteotomy was significantly associated with success and less likely to require reoperation. (20) Pelvic osteotomy facilitates closure by reducing tension on the abdominal wall and can be performed with bladder closure or separately as a staged procedure 2 to 3 weeks before bladder closure. (19)Typically, infants went to the OR in the first 72 hours after birth to optimize intestinal function and prevent contamination of the urinary tract. However, many infants with OEIS complex are premature and therefore, have comorbidities such as respiratory distress syndrome or a hemodynamically significant patent ductus arteriosus, which can substantially increase the risk of surgery in the first 72 hours of age. A recent retrospective review of 10 cases of OEIS complex at our institution demonstrated that delayed first-stage repair (n=6, range 6–87 days) was safe with no significant difference in median hindgut length. (18) All infants were discharged from the hospital on full enteral nutrition and without oxygen. (18) Delayed repair was associated with a longer NICU stay (corrected for medical comorbidities), longer duration of intubation, and longer duration of parenteral nutrition use. Prevention of urinary tract infections is often cited as a motivating factor for early surgery, but no urinary tract infections were observed in this cohort. It was concluded that delayed repair allows infants to recover from the sequalae of prematurity and grow, which reduces perioperative risk and is especially important in the setting of growth restriction and low birthweight. (18)Historically, OEIS complex was thought to be “almost always fatal,” with many case studies and series recommending that perinatal counseling emphasize to families the need for complex genitourinary surgery with unfavorable outcomes. (11) Indeed, it was the potential impact of significant urologic and sexual handicaps that led physicians to recommend pregnancy termination in the setting of cloacal exstrophy. (21) However, medical advancements have led to almost universal survival rates, and research now focuses on improving surgical management and outcomes. (20)(22) Adults born with OEIS complex or cloacal exstrophy cope with several quality-of-life issues, including urinary and fecal incontinence, sexual dysfunction, infertility, psychological disorders and impact of gender reassignment (no longer performed), chronic medical problems, and need for continued complex medical care throughout their lifespan. (21)(22) In a recent systematic review and meta-analysis, 21 articles were identified that addressed health-related quality of life (HRQOL) in patients with BEEC and 5 of those were included in a meta-analysis. (23) The pooled estimate effect indicated a worse HRQOL, though the effect sizes were small or less than small; this included overall quality of life, mental health in children and adolescents, and physical functioning in children and adolescents, as well as mental health, physical functioning, and social functioning in adults. (23)One of the few studies to assess quality-of-life issues in adults was a survey administered to a cohort of 23 female patients with BEEC aged 19 to 41 years with a median age of 26.5 years, of whom 17 patients completed the questionnaire. (21) Most women (65%) experienced varying degrees of urinary incontinence, and in the most severe cases, reported feeling seriously handicapped because of the resultant social, professional, and sexual limitations. Four women reported that their professional satisfaction was moderately or severely affected in part because of the need for frequent bathroom visits. (21) Regarding sexual and reproductive function, 88% of women had regular menses, and 3 women became pregnant and delivered 4 healthy children via classic cesarean section, which was performed to protect their reconstructions. Most of the women were sexually active (76%), but half experienced challenges such as incontinence during/after intercourse, reduced clitoral sensitivity, and dyspareunia from introital stenosis. Overall, the most important factors that had a negative impact on quality of life were urinary incontinence and poor body image from surgical scars and genital cosmesis. (21)In this same study, psychological distress was reported by 41% of women, 5 of whom felt their lives were severely affected by the need for psychological and psychiatric care, including prolonged medical treatment and hospitalization for depression, anxiety, and eating disorders. Most patients reported consistently feeling negative emotions such as loneliness, fear of talking about their medical problems, feeling of being handicapped, poor body image, and fear of the future, especially around parenting and sexuality. (21) In contrast, a cross-sectional study of quality-of-life issues, which surveyed children with BEEC ages 1 to 18 years (median age 8.41 years, n=80) and their parents, showed mean HRQOL total scores in the average range; however, the scores varied greatly, with lower scores associated with male sex, older age, and classic bladder exstrophy versus epispadias. Positive outcomes were hypothesized to be linked to resiliency, and the importance of psychological care was emphasized as a result. (24) A similar conclusion was drawn from the cohort study of women with BEEC; patients with cloacal exstrophy were found to benefit from extensive psychosocial support and mental health counseling for coping with issues of continence and sexuality. (22) In our multidisciplinary colorectal clinic, we have started addressing the psychosocial side of development with our patients from a very young age, continuing through adolescence and adulthood.OEIS syndrome is a rare, significant complex of congenital anomalies that requires extensive staged postnatal surgeries to achieve functioning urinary, bowel, and genital systems. During the initial family counseling, it is important to emphasize that prenatal findings can be incomplete despite the use of advanced imaging such as fetal MRI, and thus definitive diagnosis can be made on postnatal examination. As such, the final surgical plan is determined after delivery, including timing and staging of the repair based on the infant’s anatomy, gestational age at birth, and comorbidities. There are many quality-of-life issues for children, adolescents, and adults with OEIS complex, and extensive psychosocial support for both the individual and the family is paramount. Additional work is needed to improve both cosmesis and function for individuals with OEIS complex, and it is important to ensure comprehensive follow-up throughout the lifespan to maintain health, minimize complications, and inform further surgical advancements.