We are reporting the case of a 32-year-old female who had suffered from fecal incontinence (FI). She was born with an imperforate anus and a recto-vaginal fistula; she underwent repair at 6 mo of age. At 29 years of age, she was still fecally incontinent despite extensive pelvic floor reeducation. A magnetic resonance imaging and an anal electromyography were performed. Because her symptoms were considered to be probably due to extra-sphincteric implantation of the neo-anus, a redo was performed of the recto-neo-anal intra-sphincteric anastomosis. A neurostimulator device was subsequently implanted for persistent incontinence. Solid and liquid FI resolved, and her quality of life improved markedly. Combining surgery to correct the position of the neo-anus within the anal sphincter complex and neurostimulation could thus become a new approach in cases of refractory FI for patients with imperforate anus as a newborn. Follow-up into adulthood after pediatric imperforate anus surgery should be recommended for adult patients with persistent FI.
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http://dx.doi.org/10.12998/wjcc.v5.i3.124 | DOI Listing |
Andes Pediatr
August 2023
Programa Madre Canguro /Alto riesgo neonatal Subred Sur-UMHES-MEISSEN, Bogotá, Colombia.
Unlabelled: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare entity that presents abdominal wall defects, entails high morbidity and mortality, and requires multidisciplinary management.
Objective: To describe a case with an unusual association between OEIS complex and diaphragmatic hernia and to discuss its pathogenesis and possible association with other midline malformations.
Clinical Case: A preterm female newborn of 33 weeks of gestational age, with prenatal diagnosis of giant omphalocele that, at birth, presented intact amnion coverture containing the entire liver and some bowel loops, open bladder exstrophy and exposed urethral orifices; uterus didelphys, no palpable gonads, and concurrent imaging findings of pelvic soft tissue extrusion, left diaphragmatic hernia (Bochdalek), multiple bone defects, myelomeningocele, and myelocystocele.
Objective: While the association of a syrinx with a tethered spinal cord in the context of VACTERL (vertebral defects [V], imperforate anus or anal atresia [A], cardiac malformations [C], tracheoesophageal defects [T] with or without esophageal atresia [E], renal anomalies [R], and limb defects [L]) association is known, the incidence of idiopathic syrinxes among these patients has not previously been reported. The authors aimed to characterize the incidence of syrinxes and the pattern of congenital anomalies in pediatric patients with VACTERL association, with a specific focus on the presence of idiopathic syrinxes in this population.
Methods: An institutional database was retrospectively queried for all pediatric patients with VACTERL association.
Surg Neurol Int
December 2024
Department of Neurosurgery, Graduate School of Medical Sciences, Kyushu University, Iizuka, Japan.
Background: Omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex is a rare, life-threatening congenital malformation primarily treated with abdominogenital repair. The optimal indication and timing of neurosurgical interventions for the associated spinal cord lesions remains insufficiently studied. We reviewed spinal dysraphism in OEIS to evaluate the best timing for neurosurgical intervention.
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December 2024
Department of Pediatric Surgery, Hokkaido Medical Center for Child Health and Rehabilitation, 1-1-240-6, Kanayama, Teine-ku, Sapporo, Hokkaido, 006-0041, Japan.
Purpose: This study aimed to identify surgical site infection (SSI) risk factors after anal reconstruction surgery in patients with anorectal malformations (ARMs).
Methods: This retrospective analysis from January 2013 to December 2022, including all pediatric surgical facilities in Hokkaido, Japan, examined consecutive patients with ARMs, excluding cloacal cases, regarding perioperative and SSI factors during their initial anal reconstruction surgeries.
Results: This study involved 157 cases of major clinical groups and 7 cases of rare/regional variants, among whom 4% developed SSIs.
Afr Urol
June 2024
Division of Urology, Department of Surgery, University of Utah School of Medicine, United States of America.
Bladder exstrophy-epispadias-cloacal exstrophy complex (BEEC) is a spectrum of congenital urologic anomalies that involve the bladder, urethra, genitalia, and pelvic musculoskeletal system, and can affect urinary continence, sexual health, and fertility. BEEC includes a wide spectrum of anatomical abnormalities with different levels of severity: epispadias represents the mildest phenotype, classic bladder exstrophy (CBE) is the most common defect, and cloacal exstrophy (CE) - often referred to as omphalocele, exstrophy, imperforate anus, and spinal defects (OEIS) complex - is the most severe form. BEEC disorders cause significant health problems and affect the health-related quality of life (QoL) of affected individuals.
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