Disparities in Healthcare Utilization: An Analysis of Disease Specific and Patient Level Factors in a Congenital Diaphragmatic Hernia Clinic.

Objectives: Our study examines if the disease severity profile of our Congenital Diaphragmatic Hernia (CDH) patient cohort adherent to long-term follow-up differs from patients lost to follow-up after discharge and examines factors associated with health care utilization.

Methods: Retrospective review identified CDH survivors born 2005-2019 with index repair at our institution. Primary outcome was long-term follow-up status: "active" or "inactive" according to clinic guidelines.

Updates on the Care of Cloacal Exstrophy.

Cloacal exstrophy is the most severe congenital anomaly of the exstrophy-epispadias complex and is characterized by gastrointestinal, genitourinary, neurospinal, and musculoskeletal malformations. Individualized surgical reconstruction by a multidisciplinary team is required for these complex patients. Not infrequently, patients need staged surgical procedures throughout childhood and adolescence.

Robotic-assisted laparoscopic Malone appendicostomy: a 6-year perspective.

Purpose: A robotic-assisted laparoscopic approach to appendicostomy offers the benefits of a minimally invasive approach to patients who would typically necessitate an open procedure, those with a larger body habitus, and those requiring combined complex colorectal and urologic reconstructive procedures. We present our experience performing robotic-assisted appendicostomies with a focus on patient selection, perioperative factors, and functional outcomes.

Methods: A retrospective review of patients who underwent a robotic-assisted appendicostomy/neoappendicostomy at our institution was performed.

Late Diagnosis of Hirschsprung Disease: Clinical Presentation and Long-Term Functional Outcomes.

Purpose: Hirschsprung Disease (HD) is typically diagnosed in the neonatal period. A small subset of patients have a prolonged course of abdominal distention and constipation prior to diagnosis. Late HD is defined as having been diagnosed at greater than or equal to one year of age.

Outcomes From Colonic Pull-Through for Cloacal Exstrophy Differ by Colon Length: A Multi-Institutional Study.

Background: Cloacal exstrophy (CE) represents a rare sub-group of anorectal malformations. Traditionally managed with a permanent colostomy, colonic pull-through (PT) has emerged to allow cleanliness without a life-long stoma. We sought to understand outcomes of PT in a large multi-center CE population.

Rectal Prolapse Following Repair of Anorectal Malformation: Incidence, Risk Factors, and Management.

Background: The incidence and optimal management of rectal prolapse following repair of an anorectal malformation (ARM) has not been well-defined.

Methods: A retrospective cohort study was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium registry. All children with a history of ARM repair were included.

Surgical Resection of Vulvar Labial Venous Malformations: A Single Center Experience.

Background: Venous malformations (VMs) involving the vulva are rare but often cause debilitating pain, aesthetic concern, and functional impairment. Treatment with medical therapy, sclerotherapy, operative resection, or a combination thereof may be considered. The optimal therapeutic approach remains unclear.

Saving the perineal body-A modification of the posterior sagittal anorectoplasty.

Background: Wound dehiscence of the anocutaneous anastomosis and/or reconstructed perineal body is a feared perioperative complication after posterior sagittal anorectoplasty (PSARP). Dehiscence of the perineal body can have long-term negative implications for fecal continence, sexual intimacy, obstetrical outcomes, and the need for reoperative surgery. We describe a modification to the traditional PSARP for repair of an imperforate anus with a rectovestibular, perineal, or absent fistula by sparing the perineal body, eliminating postoperative perineal body dehiscence and potentially improving long-term functional outcomes.

Does presence of a VACTERL anomaly predict an associated gynecologic anomaly in females with anorectal malformations?: A Pediatric Colorectal and Pelvic Learning Consortium Study.

Background: VACTERL association is defined by the presence of 3 or more anomalies in any of the following systems: vertebral, anorectal, cardiac, trachea-esophageal, renal, or limb. This study hypothesized that the presence of VACTERL association would correlate with an increased risk of gynecologic anomalies in patients with anorectal malformation (ARM).

Methods: This study is a cross-sectional, retrospective analysis from the prospectively collected, multicenter registry of the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC).

Primary and secondary vaginal reconstruction with autologous buccal mucosa and intravaginal wound vacuum therapy.

Background: Vaginal reconstruction with autologous buccal mucosa graft offers a promising alternative to the use of skin grafts and vascularized intestinal segments. Given the novelty of this procedure, the optimal approach to postoperative wound management remains unclear with current practices often requiring many months of vaginal stents/molds. This study aims to evaluate a newly developed negative pressure intravaginal wound vacuum placed at the conclusion of the vaginoplasty with the goals of facilitating graft take and healing.

Hindgut Duplication in an Infant with Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects (OEIS) Complex.

 The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts.

Technical Considerations in Primary Repair of a Congenital Prostatic Rectourethral Fistula in an Adult-Sized Patient.

Congenital anorectal malformations are generally diagnosed and repaired as a neonate or infant, but repair is sometimes delayed. Considerations for operative repair change as the patient approaches full stature. We recently encountered a 17-year-old male with an unrepaired congenital rectourethral fistula and detail our experience with his repair.

Urinary continence disparities in patients with anorectal malformations.

Purpose: While fecal incontinence is a primary concern for many children with anorectal malformations (ARM), urinary incontinence is also prevalent in this population. Racial, ethnic, and socioeconomic disparities in urinary continence have been observed in other conditions, but have not been previously evaluated in ARM. We aimed to evaluate urinary continence and associated demographic and socioeconomic characteristics in individuals with ARM.

Reoperative surgery in anorectal malformation patients.

Currently accepted primary repair of congenital anorectal malformations (ARMs) includes a posterior sagittal incision, which allows for optimal visualization and identification of important pelvic structures and anatomical features. Reconstructive surgery involves meticulous dissection and separation of pelvic structures, and careful reconstruction can result in good functional outcomes for many patients, who live without ongoing sequelae from their malformation. However, some patients may require reoperative procedures for anatomic or functional reasons.

Reoperative techniques and management in Hirschsprung disease: a narrative review.

The majority of children who undergo operative management for Hirschsprung disease have favorable results. A subset of patients, however, have long-term dysfunctional stooling, characterized by either frequent soiling or obstructive symptoms. The evaluation and management of a child with poor function after pull-through for Hirschsprung disease should be conducted by an experienced multidisciplinary team.

Can complex surgical interventions be standardized? Reaching international consensus on posterior sagittal anorectoplasty using a modified-Delphi method.

Background/purpose: In an effort to standardize educational experience, address future physician shortages, and improve quality of care to patients, many surgical specialties are discussing how to maximize exposure to index cases. One solution being explored is telementoring, which requires a well-developed educational curriculum with intraoperative objectives. The American College of Surgery Telementoring Task Force selected anorectal malformation and posterior sagittal anorectoplasty (PSARP) for the repair of imperforate anus as the initial educational focus for this pilot.

Safety of delayed surgical repair of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in infants with significant comorbidities.

Purpose: Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk.

A New Approach to Cloaca: Laparoscopic Separation of the Urogenital Sinus.

Cloaca malformation repair strategy is strongly dictated by common channel and urethral lengths. Mid to long common channel cloacas are challenging and often require laparotomy for dissection of pelvic structures. The balance of common channel and urethral lengths often dictates the approach for reconstruction.

Contrast enhanced colostography: New applications in preoperative evaluation of anorectal malformations.

Introduction: Understanding details of anatomic relationships between the colon and surrounding structures is a critical piece of preoperative planning prior to surgical repair of anorectal malformations (ARMs). Traditional imaging techniques involve ionizing radiation, distention of the rectum with supraphysiologic intraluminal pressures, and sometimes require sedation. Recent developments in the field of contrast agents have allowed the emergence of an ultrasound-based technique that can avoid these requirements while continuing to provide high resolution structural information in three dimensions.

Pediatric hepatic vascular tumors.

Vascular liver tumors in the pediatric population can present a diagnostic dilemma. The most common hepatic vascular tumors are hepatic hemangiomas; however the differential diagnosis can also include other benign lesions and malignant masses. Management is unique to the type and nature of the specific lesion.

Colorectal Vascular Anomalies.

Vascular anomalies represent a diverse group of tumors and malformations. Those involving the colon and rectum can greatly impact patients' quality of life. Proper workup to ensure the correct diagnosis can vary and may include endoscopic and radiographic studies.

Case series: Comparison of contrast-enhanced genitosonography (ceGS) to fluoroscopy and cone-beam computed tomography in patients with urogenital sinus and the cloacal malformation.

In this case series, contrast enhanced genitosonography is compared to genitography performed using fluoroscopy and cone-beam computed tomography in patients with urogenital sinus and the cloacal malformation. The method of contrast enhanced genitosonography is described, including contrast preparation, contrast administration, ultrasound imaging approaches, as well as the benefits and potential pitfalls of this technique compared to fluoroscopy and computed tomography.