Classic divided sigmoidostomy vs loop sigmoidostomy in anorectal malformations: time for change?

Purpose: Divided sigmoidostomy (DS) is the classic stoma for patients with anorectal malformations (ARM). Loop sigmoidostomies (LS) in ARM are associated with a higher risk of stoma prolapse and urinary tract infections (UTI). This is not clearly supported by literature.

Bladder duplication in a patient with a persistent urogenital sinus: Case report and systematic review of the literature.

Bladder duplication (BD) is a rare malformation that is often associated to other anomalies. We report a newborn diagnosed with BD in the sagittal plane, associated to persistent urogenital sinus (UGS), given the opening of the vagina immediately below the bladder neck. It is the fourth time this association is reported.

Anatomical Variations of the External Genitalia in Posterior Cloaca: Clinical Consequences of Misdiagnosis-A Systematic Review of the Literature and the ARM-Net Consortium Experience.

Purpose:  All types of cloacal malformations may be associated with anatomic variations of the external genitalia, including hypoplasia of the labia minora and enlarged clitoris; these variations could be even higher in posterior cloacas (PCs). If a careful physical examination is not performed, patients may be misdiagnosed with ambiguous genitalia (AG), leading to subsequent unnecessary testing, surgeries, or even wrong gender assignment. The aim was to analyze data of patients with PC within the ARM-Net registry, focusing on the description of the genitalia, gender assignment, and its consequences.

Laparoscopic Approach in Anorectal Malformations: How We Do It.

Laparoscopy has a major role in the treatment of anorectal malformations (ARM) since Georgeson et al. introduced the laparoscopic assisted anorectoplasty in 2000. The majority of ARM can be repaired through a posterior sagittal incision without the need to enter the abdomen.

One-year impact of a bowel management program in treating fecal incontinence in patients with anorectal malformations.

Background: Many patients with anorectal malformations (ARM) need a bowel management program (BMP) to manage lifelong problems of fecal incontinence or severe constipation. We aimed to evaluate the sustainability of the results in such a program.

Methods: A single-institution retrospective review was performed in children with ARM who attended our BMP (2015-2019).

Skip Segment Hirschsprung Disease Managed by Pull-Through of the Right Colon.

Hirschsprung disease is the most common neurocristopathy in children, resulting in the congenital loss of enteric ganglia. Rare reports of skip lesions have previously been reported in the literature. We present a case of skip lesions known prior to surgery and managed by pull-through of the right colon that allowed the preservation of the colon.

Correlation of anorectal malformation complexity and associated urologic abnormalities.

Background: Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses.

A pediatric colorectal and pelvic reconstruction course improves content exposure for pediatric surgery fellows: A three-year consecutive study.

Purpose: Interactive courses play an important role in meeting the educational needs of pediatric surgical trainees. We investigated the impact of a multimodal pediatric colorectal and pelvic reconstruction course on pediatric surgery trainees.

Methods: A retrospective evaluation was performed of pre- and post-course surveys for an annual colorectal and pelvic reconstruction course over 3 consecutive years (2017-2019).

Creating a collaborative program for the care of children with colorectal and pelvic problems.

The treatment of patients with colorectal disorders and their associated urologic, gynecologic, gastrointestinal, spinal, and orthopedic anomalies requires care from various medical and surgical specialties over the course of their lifetime. This is ideally handled by a collaborative center which facilitates the assessment and development of a long-term patient care plan among multiple specialties which can enhance the quality of care, improve communication among different specialties, and improve patient satisfaction and outcomes. We describe the process, as well as lessons learned in developing such a center.

Surgical Interventions for Functional Constipation: An Update.

Chronic idiopathic constipation, also known as functional constipation, is defined as difficult and infrequent defecation without an identifiable organic cause. Medical management with laxatives is effective for the majority of constipated children. However there is a subset of patients who may need evaluation by a surgeon.

Assessing the benefit of reoperations in patients who suffer from fecal incontinence after repair of their anorectal malformation.

Background And Aim: Patients with a previously repaired anorectal malformation (ARM) can suffer from complications which lead to incontinence. Reoperation can improve the anatomic result, but its impact on functional outcomes is unclear.

Methods: We performed a retrospective cohort study of patients with a previously repaired ARM who underwent redo PSARP at our Center and compared results at initial assessment and 12 months after redo.

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

Background: 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.

Methods: 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.

Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol.

Introduction:  Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained.

Materials And Methods:  We retrospectively reviewed our TCHD patients' charts from 2014 to 2017.

A call to ARMs: Accurate identification of the anatomy of the rectourethral fistula in anorectal malformations.

Recto-urethral fistulae are the most common finding in males born with an anorectal malformation (ARM). A high pressure distal colostogram is an important tool in visualizing the fistula, although the precise level at which the fistula communicates with the urethra may be difficult to interpret and is not reported in a uniform manner. This anatomy affects the surgical plan; determining the likelihood that the rectum is reachable via a posterior sagittal incision or better approached through the abdomen via laparoscopy or laparotomy helps counsel families and stratifies diagnoses for outcomes work.

Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: A single institution comparative study.

Background: Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups.

Methods: A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017.

Reliability of the Hirschsprung-Associated Enterocolitis Score in Clinical Practice.

Introduction:  There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity.

Urethral length in female infants and its relevance in the repair of cloaca.

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.

Impact on Patient Care of a Multidisciplinary Center Specializing in Colorectal and Pelvic Reconstruction.

Many patients with an anorectal malformation (ARM) or pelvic anomaly have associated urologic or gynecologic problems. We hypothesized that our multidisciplinary center, which integrates pediatric colorectal, urologic, gynecologic and GI motility services, could impact a patient's anesthetic exposures and hospital visits. We tabulated during 2015 anesthetic/surgical events, endotracheal intubations, and clinic/hospital visits for all patients having a combined procedure.

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

Patients born with complex anorectal malformations often have associated Müllerian 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.