RESUMEN
In the past, an anteriorly located anus was often misdiagnosed and treated as an anorectal malformation (ARM) with a perineal fistula (PF). The paper aims to define the criteria for a normal anus, an anterior anus (AA) as an anatomic variant, and milder types of ARM such as congenital anal stenosis (CAS) and PF. An extensive literature search was performed by a working group of the ARM-Net Consortium concerning the subject "Normal Anus, AA, and mild ARM". A consensus on definitions, clinical characteristics, diagnostic management, and treatment modalities was established, and a diagnostic algorithm was proposed. The algorithm enables pediatricians, midwives, gynecologists, and surgeons to make a timely correct diagnosis of any abnormally looking anus and initiate further management if needed. Thus, the routine physical inspection of a newborn should include the inspection of the anus and define its position, relation to the external sphincter, and caliber. A correct diagnosis and use of the presented terminology will avoid misclassifications and allow the initiation of correct management. This will provide a reliable comparison of different therapeutic management and outcomes of these patient cohorts in the future.
RESUMEN
BACKGROUND: Outcome of patients operated for anorectal malformation (ARM) type rectovestibular fistula (RVF) is generally considered to be good. However, large multi-center studies are scarce, mostly describing pooled outcome of different ARM-types, in adult patients. Therefore, counseling parents concerning the bowel function at early age is challenging. Aim of this study was to evaluate bowel function of RVF-patients at preschool/early childhood age and determine risk factors for poor functional outcome. METHODS: A multi-center cohort study was performed. Patient characteristics, associated anomalies, sacral ratio, surgical procedures, post-reconstructive complications, one-year constipation, and Bowel Function Score (BFS) at 4-7 years of follow-up were registered. Groups with below normal (BFS < 17; subgroups 'poor' ≤ 11, and 'fair' 11 < BFS < 17) and good outcome (BFS ≥ 17) were formed. Univariable analyses were performed to detect risk factors for outcome. RESULTS: The study included 111 RVF-patients. Median BFS was 16 (range 6-20). The 'below normal' group consisted of 61 patients (55.0%). Overall, we reported soiling, fecal accidents, and constipation in 64.9%, 35.1% and 70.3%, respectively. Bowel management was performed in 23.4% of patients. Risk factors for poor outcome were tethered cord and low sacral ratio, while sacral anomalies, low sacral ratio, prior enterostomy, post-reconstructive complications, and one-year constipation were for being on bowel management. CONCLUSIONS: Although median BFS at 4-7 year follow-up is nearly normal, the majority of patients suffers from some degree of soiling and constipation, and almost 25% needs bowel management. Several factors were associated with poor bowel function outcome and bowel management. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Malformaciones Anorrectales , Fístula Rectal , Adulto , Canal Anal/anomalías , Canal Anal/cirugía , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/epidemiología , Malformaciones Anorrectales/cirugía , Niño , Preescolar , Estudios de Cohortes , Estreñimiento/complicaciones , Estudios de Seguimiento , Humanos , Fístula Rectal/epidemiología , Fístula Rectal/etiología , Fístula Rectal/cirugía , Recto/cirugía , Estudios Retrospectivos , Factores de Riesgo , Resultado del TratamientoRESUMEN
OBJECTIVE: To review the Anorectal Malformation Network experience with perineal groove (PG) focusing on its clinical characteristics and management. STUDY DESIGN: Data on patients with PG managed at 10 participating Anorectal Malformation Network centers in 1999-2019 were collected retrospectively by questionnaire. RESULTS: The cohort included 66 patients (65 females) of median age 1.4 months at diagnosis. The leading referral diagnosis was anal fissure (n = 20 [30.3%]): 23 patients (34.8%) had anorectal malformations. Expectant management was practiced in 47 patients (71.2%). Eight (17%) were eventually operated for local complications. The median time to surgery was 14 months (range, 3.0-48.6 months), and the median age at surgery was 18.3 months (range, 4.8-58.0 months). In the 35 patients available for follow-up of the remaining 39 managed expectantly, 23 (65.7%) showed complete or near-complete self-epithelization by a mean age 15.3 months (range, 1-72 months) and 4 (11.4%) showed partial self-epithelization by a mean age 21 months (range, 3-48 months). Eight patients showed no resolution (5 were followed for ≤3 months). Nineteen patients (28.7%) were primarily treated with surgery. In total, 27 patients were operated. Dehiscence occurred in 3 of 27 operated patients (11.1%). CONCLUSIONS: PG seems to be an underestimated anomaly, frequently associated with anorectal malformations. Most cases heal spontaneously; therefore, expectant management is recommended. When associated with anorectal malformations requiring reconstruction, PG should be excised in conjunction with the anorectoplasty.
Asunto(s)
Canal Anal/anomalías , Malformaciones Anorrectales/diagnóstico , Manejo de la Enfermedad , Perineo/anomalías , Procedimientos de Cirugía Plástica/métodos , Canal Anal/cirugía , Malformaciones Anorrectales/cirugía , Preescolar , Femenino , Humanos , Lactante , Masculino , Perineo/cirugía , Estudios RetrospectivosRESUMEN
INTRODUCTION: Intractable full-thickness rectal prolapse (IRP) unresponsive to conservative treatment remains a major problem after anorectoplasty for high or intermediate anorectal malformation (ARM). Surgical management must aim for a permanent fixation of the rectum to the presacral fascia. While in children with IRP following ARM repair the optimal procedure has not been established yet, laparoscopic posterior mesh-rectopexy (Well's procedure) has demonstrated efficacy in adults. PRESENTATION OF CASE: A male infant with intermediate ARM received laparoscopic-assisted anorectal pull-through at the age of 4 months. Three months later he developed mucosal prolapse and received multimodal conservative treatment. Because of progression into a full-thickness RP with ulcerations, the parents opted for surgical management. Well's procedure was performed at the age of 4 years. Using four ports, the rectum was circumferentially mobilized down to the pelvic floor and pulled inside. A 5â¯×â¯5â¯cm prolene mesh was tacked to the sacrum, enveloped posteriorly 270° around the rectum, fixed with interrupted prolene sutures on both edges and carefully covered with peritoneum. Any redundant external mucosa was excised from a perineal approach. There were no intra- and postoperative complications. Within 1.5 years of follow-up the boy had voluntary bowel movements and was toilet trained. No prolapse recurrence could be observed nor provoked. DISCUSSION: We present the first pediatric case of IRP secondary to laparoscopic ARM repair which has been successfully treated by combined Well's procedure and perineal mucosal resection. CONCLUSION: Well's procedure is a successful technique and should be further explored in children with ARM and IRP.
RESUMEN
PURPOSE: In 1989, Sauer introduced a technique to treat patients with total colonic aganglionosis (TCA) where he preserved the ileo-cecal valve and the right hemicolon to retain water and electrolyte absorption. This report examines the long-term outcome of patients who underwent this technique. METHODS: All TCA patients treated between 1981 and 2005 according to Sauer were invited in 2013 to participate in a questionnaire survey to assess their long-term outcome focusing on bowel function and quality of life (QoL), using standardized scores. RESULTS: Of eight TCA patients (2 females, 6 males), seven participated in the follow-up survey (median follow-up time 13.5 years (range 8-31.6). Early postoperative complications involving bouts of enterocolitis in 3 and anal strictures in 5 patients ceased with age. Bowel movements ranged from median 3-4 times a day (range 1-8). Bowel-function score (BFS) was reduced in 6 patients (median 16 points, range 8-19, max 20), who also reported soiling incidences with the need for night-time protective aids in two. QoL assessment, however, documented a good outcome with a median score of 10 points (range 7-13, max 13). CONCLUSION: This long-term investigation of TCA patients treated according to Sauer's technique documented a good QoL despite a reduced BFS.