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Müllerian duct anomalies are rare in the general population, occurring in less than 3% of women, but much more prevalent in female patients with anorectal malformation, occurring in up to 30% of these patients. Unicornuate uterus with a rudimentary non-communicating horn is a congenital anomaly of Müllerian development which can be seen in isolation or in conjunction with other anomalies, with several case reports described in patients with VACTERL association. These anomalies may be asymptomatic until the patient develops dysmenorrhea or devastating obstetrical complications. We describe the successful surgical management of an obstructive Müllerian anomaly in a post-pubertal female patient with anorectal malformation.

Acquired urethrovaginal fistulae and urethral atresia are rare findings in pediatric patients, but have been described in adult patients related to trauma or iatrogenic injury. Little exists in the published literature to guide management of such conditions in children, but lessons learned from congenital causes can help. Herein we discuss the preoperative evaluation and management of a child with an acquired urethrovaginal fistula and urethral atresia likely related to in utero compression from an intrapelvic sacrococcygeal teratoma and provide several images detailing the complex anatomy.

Background: Approximately 7% of adolescent girls have reproductive tract anomalies. Anomalies associated with complete outflow track obstruction present with primary amenorrhea and cyclic abdominal pain. Some outflow tract obstructions may be associated with renal, anorectal, vertebral, cardiac, tracheoesophageal, and limb anomalies. Outflow tract obstructions may occur at varying anatomic locations – vaginal, cervical, or uterine, each requiring different surgical management. Methods: We present the preoperative evaluation and surgical management of three different gynecologic outflow tract obstructions. Results: Physical exam, ultrasound, and MRI were obtained for each patient. The first obstruction was a transverse vaginal septum, which was treated with transperineal resection to allow menstrual egress. The second obstruction was identified as distal vaginal atresia and managed with operative pull-through of her native vagina. The third obstruction was found to be cervico-vaginal atresia and required hysterectomy and salpingectomy with preservation of the ovaries. Conclusions: Preoperative examination of the perineum and imaging with accurate interpretation is crucial to identify subtle but important distinctions in diagnosis, which can result in markedly different interventions ranging from transperineal resection of a transverse vaginal septum, vaginal pull-through procedure, to hysterectomy. The location of the gynecologic outflow obstruction dictates the proper surgical treatment of these patients. Keywords: Mullerian obstruction, Adolescent, Anorectal malformation

A subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning.We retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014-2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement.50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement.Urethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement.Level IV.Case series with no comparison groups.

This chapter describes the surgical management of an ectopic pregnancy via laparoscopic procedure as performed by the following approaches: laparoscopic as against open abdominal surgery, whether it is a laparoscopic salpingectomy, where the entire fallopian tube is surgically removed, or laparoscopic salpingostomy, where the products of conception only are removed from the fallopian tube, merely by incising the fallopian tube and suctioning the material out, leaving the fallopian tube intact. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure(s) in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: abdominal access, anatomic assessment, salpingostomy or salpingectomy, removal of ectopic pregnancy.

This chapter describes the surgical procedure for incision and drainage of Bartholin gland duct cyst or abscess as performed by open approach. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: bimanual examination, vertical incision, placement of Word catheter, or marsupialization.

Aim of the Study The goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5 cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5 cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant. Methods We reviewed voiding cystourethrograms of healthy female patients between ages 6 and 36 months and measured the patient's urethral length. Results Ninety-one children were included. The mean urethral length for patients age 6–12 months was 2.50 cm, age 12–24 months was 2.31 cm, and age 24–36 months was 2.59 cm. There was no difference between the urethral length in the three groups (p = 0.38). Of 91 patients, 87 (96%) had a urethral length > 1.5 cm. Conclusion A urethra of at least 1.5 cm was present in the majority of normal control patients. We believe therefore that for urogenital sinus and cloacal repair, surgeons can extrapolate that patients need a 1.5 cm urethra at the end of the reconstruction. Additional follow-up is needed to determine if this urethral length as an independent factor maintains dryness in the long term after cloacal repair. Type of Study Case Series. Level of Evidence III.

Introduction Patients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients. Methods We developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses. Our weekly planning strategy includes a meeting with representatives of all specialties to review all patients prior to evaluation in our multidisciplinary clinic, followed by combined exams under anesthesia or surgical intervention as needed. Results There are 31 people working directly in the Center at present. From the Center's start in 2014 until 2017, 1258 patients were cared for from all 50 United States and 62 countries. 360 patients had an ARM (110 had a cloacal malformation, 11 had cloacal exstrophy), 223 presented with HD, 71 had a spinal malformation or injury causing neurogenic bowel, 321 had severe functional constipation or colonic dysmotility, and 162 had other diagnoses including familial polyposis, Crohn's disease, or ulcerative colitis. We have had 170 multidisciplinary meetings, 170 multispecialty outpatient, and 52 nurse practitioner clinics. In our bowel management program we have seen a total of 514 patients in 36 sessions. Conclusion This is the first report describing the design of a multidisciplinary team approach for patients with colorectal and complex pelvic malformations. We found that approaching these patients in a collaborative way allows for combined medical and surgical decisions with many providers simultaneously, facilitates therapy, and can potentially improve patient outcomes. We hope that this model will help establish new-devoted centers in other locations to encourage centralized care for these rare malformations. Level of evidence IV.

Introduction A significant number of internationally adopted children have congenital birth defects. As a specialist center for colorectal diagnoses, we evaluate such children with an anorectal malformation (ARM) and have found that a significant number need a reoperation. Knowledge of the common complications following ARM surgery has led us to develop treatment algorithms for patients with unknown past medical and surgical history, a situation typically encountered in the adopted population. Methods The results of investigations, indications, and rate of reoperation were assessed for adopted children with an ARM evaluated between 2014 and 2016. Results 56 patients (28 males) were identified. 76.8% required reoperative surgery. Mislocation of the anus outside the sphincter complex was seen in 50% of males and 39.3% of females. Anal stricture, rectal prolapse, retained vaginal septum, and a strictured vaginal introitus were also common. Conclusion The reoperative surgery rate in the internationally adopted child with an ARM is high. Complete, systematic evaluation of these children is required to identify complications following initial repair. Development of mechanisms to improve the primary surgical care these children receive is needed.