RESUMEN
Rectal prolapse is a common disease in childhood and observed mainly at the age of 1-4 years old (95% of cases). If conservative treatment is ineffective, surgical correction of rectal prolapse in children without previous anorectal surgery is performed at the age of over a year. There is a single report on examination of patients aged 4-16 years after surgical correction of anorectal malformations with postoperative rectal prolapse. We present diagnosis and successful surgical treatment of rectal prolapse in an infant who underwent previous perineal proctoplasty for fistulous form of anorectal malformation.
Asunto(s)
Prolapso Rectal , Recto , Humanos , Prolapso Rectal/cirugía , Prolapso Rectal/etiología , Prolapso Rectal/diagnóstico , Recto/cirugía , Recto/anomalías , Resultado del Tratamiento , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Complicaciones Posoperatorias/diagnóstico , Masculino , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/diagnóstico , Femenino , Preescolar , Reoperación/métodosRESUMEN
OBJECTIVE: Treatment of neonates with anorectal malformations (ARMs) can be challenging due to variability in anatomic definitions, multiple approaches to surgical management, and heterogeneity of reported outcomes. The purpose of this systematic review is to summarize existing evidence, identify treatment controversies, and provide guidelines for perioperative care. METHODS: The American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee (OEBP) drafted five consensus-based questions regarding management of children with ARMs. These questions were related to categorization of ARMs and optimal methods and timing of surgical management. A comprehensive search strategy was performed, and the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines were used to perform the systematic review to attempt to answer five questions related to surgical care of ARM. RESULTS: A total of 10,843 publications were reviewed, of which 90 were included in final recommendations, and some publications addressed more than one question (question: 1 n = 6, 2 n = 63, n = 15, 4 n = 44). Studies contained largely heterogenous groups of ARMs, making direct comparison for each subtype challenging and therefore, no specific recommendation for optimal surgical approach based on outcomes can be made. Both loop and divided colostomy may be acceptable methods of fecal diversion for patients with a diagnosis of anorectal malformation, however, loop colostomies have higher rates of prolapse in the literature reviewed. In terms of timing of repair, there did not appear to be significant differences in outcomes between early and late repair groups. Clear and uniform definitions are needed in order to ensure similar populations of patients are compared moving forward. Recommendations are provided based primarily on A-D levels of evidence. CONCLUSIONS: Evidence-based best practices for ARMs are lacking for many aspects of care. Multi-institutional registries have made progress to address some of these gaps. Further prospective and comparative studies are needed to improve care and provide consensus guidelines for this complex patient population.
Asunto(s)
Malformaciones Anorrectales , Humanos , Malformaciones Anorrectales/cirugía , Recién Nacido , Medicina Basada en la Evidencia , Recto/anomalías , Recto/cirugía , Canal Anal/anomalías , Canal Anal/cirugía , Ano Imperforado/cirugía , Procedimientos Quirúrgicos del Sistema Digestivo/métodosRESUMEN
ABSTRACT: Currarino syndrome (CS) is a rare congenital syndrome characterized by a triad of anorectal malformation, sacral deformity, and presacral mass. In about 50% of cases, it is caused by HLXB9 gene mutation in chromosome 7q36. A 13-month-male child presented with presacral discharging sinus with a history of surgery for anorectal malformation and perineal fistula at the time of birth. On detailed investigation, the child revealed to have anal atresia, hemisacrum, and presacral mass. Histopathology of presacral mass showed features of immature teratoma. The presacral mass in CS is mostly an anterior myelomeningocele or presacral teratoma. The development of immature teratoma in presacral mass is very rare. The histopathological identification of immature component of teratoma in the presacral mass of CS is important for risk stratification and further management. Suspicion of CS should be raised in any child presenting with partial phenotype of the triad.
Asunto(s)
Canal Anal , Anomalías del Sistema Digestivo , Recto , Sacro , Siringomielia , Teratoma , Humanos , Teratoma/patología , Teratoma/cirugía , Teratoma/diagnóstico , Masculino , Canal Anal/anomalías , Canal Anal/cirugía , Canal Anal/patología , Sacro/anomalías , Sacro/cirugía , Sacro/patología , Anomalías del Sistema Digestivo/cirugía , Anomalías del Sistema Digestivo/diagnóstico , Anomalías del Sistema Digestivo/patología , Anomalías del Sistema Digestivo/genética , Siringomielia/cirugía , Siringomielia/genética , Siringomielia/patología , Siringomielia/diagnóstico , Siringomielia/diagnóstico por imagen , Lactante , Recto/anomalías , Recto/cirugía , Recto/patología , Ano Imperforado/cirugía , Ano Imperforado/diagnóstico , Ano Imperforado/genética , Ano Imperforado/patologíaRESUMEN
AIM: To study the published literature on robotic-assisted pull-through procedures for anorectal malformation. METHOD: A PubMed search was done on 10th April 2024, with the words "robotic AND Anorectal malformation". The articles were screened for relevance and the data were compiled on the safety, feasibility, technical details, and limitations of robotic-assisted procedures in children for anorectal malformation. RESULTS: The search robotic and anorectal malformation gave ten articles. Two were excluded as they were not relevant. Two articles were added from cross-reference. Ten publications on robotic-assisted procedures for anorectal malformation were studied, describing procedures in thirty-three cases. The youngest child operated was 3 months old. All except one case were done in males. Most articles were from the US and the Kingdom of Saudi Arabia (KSA). The principles involved in robotic-assisted anorectoplasty (RAARP) were the same as that of laparoscopic procedures. Complications reported included pelvic abscess, epididymo-orchitis, and stricture of pelvic tunnel or conversion to open. The magnification and endo-wrist technology of robotics facilitated the sharp dissection and ligation at origin of fistula. The mean operating time was 228.7 min (docking and console time), shortest being 86 min and mean hospital stay was 7 days. The number of ports used varied from 3 to 4 with 8.5 mm being the most commonly used umbilical port and 8 mm as working ports, although in one article, a 12 mm port was used for telescope. The prolonged operating time and cost are the two factors to be addressed in RAARP. CONCLUSION: Robotic surgery is feasible in infants with ARM and safe in expert hands. Robotics is a very effective tool with its better ergonomics, tremor filtration, 3D magnification, and dexterity. Increasing awareness and referral to high-volume centers can tide over the cost factor, and good training of the surgeons can reduce the operative time.
Asunto(s)
Malformaciones Anorrectales , Procedimientos Quirúrgicos Robotizados , Humanos , Procedimientos Quirúrgicos Robotizados/métodos , Malformaciones Anorrectales/cirugía , Masculino , Lactante , Femenino , Tempo Operativo , Recto/cirugía , Recto/anomalíasRESUMEN
Familial episodic pain syndrome (FEPS) is an early childhood onset disorder of severe episodic limb pain caused mainly by pathogenic variants of SCN11A, SCN10A, and SCN9A, which encode three voltage-gated sodium channels (VGSCs) expressed as key determinants of nociceptor excitability in primary sensory neurons. There may still be many undiagnosed patients with FEPS. A better understanding of the associated pathogenesis, epidemiology, and clinical characteristics is needed to provide appropriate diagnosis and care. For this study, nationwide recruitment of Japanese patients was conducted using provisional clinical diagnostic criteria, followed by genetic testing for SCN11A, SCN10A, and SCN9A. In the cohort of 212 recruited patients, genetic testing revealed that 64 patients (30.2%) harbored pathogenic or likely pathogenic variants of these genes, consisting of 42 (19.8%), 14 (6.60%), and 8 (3.77%) patients with variants of SCN11A, SCN10A, and SCN9A, respectively. Meanwhile, the proportions of patients meeting the tentative clinical criteria were 89.1%, 52.0%, and 54.5% among patients with pathogenic or likely pathogenic variants of each of the three genes, suggesting the validity of these clinical criteria, especially for patients with SCN11A variants. These clinical diagnostic criteria of FEPS will accelerate the recruitment of patients with underlying pathogenic variants who are unexpectedly prevalent in Japan.
Asunto(s)
Pruebas Genéticas , Canal de Sodio Activado por Voltaje NAV1.7 , Canal de Sodio Activado por Voltaje NAV1.8 , Canal de Sodio Activado por Voltaje NAV1.9 , Humanos , Canal de Sodio Activado por Voltaje NAV1.7/genética , Canal de Sodio Activado por Voltaje NAV1.9/genética , Japón/epidemiología , Canal de Sodio Activado por Voltaje NAV1.8/genética , Masculino , Femenino , Pruebas Genéticas/métodos , Adulto , Adolescente , Niño , Predisposición Genética a la Enfermedad , Adulto Joven , Preescolar , Mutación , Dolor , Recto/anomalíasRESUMEN
PURPOSE: To describe the long-term bowel function of anorectal malformation (ARM) patients and explore the potential influence factors. METHODS: ARM patients with follow-up data > 10 years were included. Cases of cloaca, Currarino syndrome, and VACTERL syndrome were excluded. Rintala score and PedsQL 4.0 were used to assess bowel function score (BFS) and quality of life (QoL). Based on the results, patients were divided into satisfactory group with BFS ≥ 17 and unsatisfactory group with it < 17. Comparisons between the groups were made. RESULTS: Among the 81 patients were 44 males and 37 females. Follow-up time was 138 (126,151) months. 16 (19.75%) patients had associated anomalies. 23 (28.40%) patients had reoperations, and fistula recurrence was the most common reason. BFS of the patients was 20 (18,20). QoL score was 100 (100,100), which correlated positively with BFS (r = 0.648, P < 0.001). The satisfactory and the unsatisfactory groups had 69 and 12 cases, and their BFS were 20 (20,20) and 11 (8,15) respectively, which had statistical difference (P < 0.001). Total QoL score and psycho-social health score of the unsatisfactory group were lower (P < 0.001). Only reoperations were statistically different between the groups (P < 0.001). CONCLUSIONS: Long-term (> 10 years) bowel function of ARM patients is good in this study. Defecation problems have negative impacts on QoL and mainly affects their psycho-social health. Primary anorectoplasty is extremely important. Reoperations, which are most commonly seen in recto-urethral fistula recurrence, adversely affect the outcome.
Asunto(s)
Malformaciones Anorrectales , Calidad de Vida , Humanos , Masculino , Femenino , Estudios Retrospectivos , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Estudios de Seguimiento , Niño , Preescolar , Canal Anal/anomalías , Canal Anal/cirugía , Adolescente , Lactante , Recto/anomalías , Recto/cirugíaRESUMEN
PURPOSE: Congenital anorectal stenosis is managed by dilations or operative repair. Recent studies now propose use of dilations as the primary treatment modality to potentially defer or eliminate the need for surgical repair. We aim to characterize the management and outcomes of these patients via a multi-institutional review using the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. METHODS: A retrospective database review was performed using the PCPLC registry. The patients were evaluated for demographics, co-morbidities, diagnostic work-up, surgical intervention, current bowel management, and complications. RESULTS: 64 patients with anal or rectal stenosis were identified (57 anal, 7 rectal) from a total of 14 hospital centers. 59.6% (anal) and 42.9% (rectal) were male. The median age was 3.2 (anal) and 1.9 years (rectal). 11 patients with anal stenosis also had Currarino Syndrome with 10 of the 11 patients diagnosed with a presacral mass compared to only one rectal stenosis with Currarino Syndrome and a presacral mass. 13 patients (22.8%, anal) and one (14.3%, rectal) underwent surgical correction. Nine patients (8 anal, 1 rectal) underwent PSARP. Other procedures performed were cutback anoplasty and anterior anorectoplasty. The median age at repair was 8.4 months (anal) and 10 days old (rectal). One patient had a wound complication in the anal stenosis group. Bowel management at last visit showed little differences between groups or treatment approach. CONCLUSION: The PCPLC registry demonstrated that these patients can often be managed successfully with dilations alone. PSARP is the most common surgical repair chosen for those who undergo surgical repair. LEVEL OF EVIDENCE: III.
Asunto(s)
Dilatación , Humanos , Masculino , Estudios Retrospectivos , Femenino , Lactante , Preescolar , Constricción Patológica/cirugía , Dilatación/métodos , Sistema de Registros , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/complicaciones , Niño , Resultado del Tratamiento , Recto/anomalías , Recto/cirugía , Enfermedades del Recto/terapia , Enfermedades del Recto/cirugía , Enfermedades del Recto/congénito , Enfermedades del Recto/diagnóstico , Canal Anal/anomalías , Canal Anal/cirugía , Ano Imperforado/cirugía , Ano Imperforado/complicaciones , Ano Imperforado/diagnósticoAsunto(s)
Recto , Humanos , Recto/anomalías , Recto/cirugía , Recto/diagnóstico por imagen , Femenino , Recién Nacido , MasculinoRESUMEN
BACKGROUND: This study describes the presentation and initial management of anorectal malformation (ARM); evaluating the frequency, causes and consequences of late diagnosis. METHODS: A prospective, population cohort study was undertaken for newly diagnosed ARMs in the UK and Ireland from 01/10/2015 and 30/09/2016. Follow-up was completed at one year. Data are presented as n (%), appropriate statistical methods used. Factors associated with late diagnosis; defined as: detection of ARM either following discharge or more than 72 h after birth were assessed with univariable logistic regression. RESULTS: Twenty six centres reported on 174 cases, 158 of which were classified according to the type of malformation and 154 had completed surgical data. Overall, perineal fistula was the most commonly detected anomaly 43/158 (27%); of the 41 of these children undergoing surgery, 15 (37%) had a stoma formed. 21/154 (14%, CI95{9-20}) patients undergoing surgery experienced post-operative complications. Thirty-nine (22%) were diagnosed late and 12 (7%) were detected >30 days after birth. Factors associated with late diagnosis included female sex (OR 2.06; 1.0-4.26), having a visible perineal opening (OR 2.63; 1.21-5.67) and anomalies leading to visible meconium on the perineum (OR 18.74; 2.47-141.73). 56/174 (32%) had a diagnosis of VACTERL association (vertebral, anorectal, cardiac, tracheal, oesophageal, renal and limb); however, not all infants were investigated for commonly associated anomalies. 51/140 (36%) had a cardiac anomaly detected on echocardiogram. CONCLUSION: There is room for improvement within the care for infants born with ARM in the UK and Ireland. Upskilling those performing neonatal examination to allow timely diagnosis, instruction of universal screening for associated anomalies and further analysis of the factors leading to clinically unnecessary stoma formation are warranted. LEVEL OF EVIDENCE: II (Prospective Cohort Study <80% follow-up).
Asunto(s)
Malformaciones Anorrectales , Diagnóstico Tardío , Humanos , Irlanda/epidemiología , Femenino , Recién Nacido , Masculino , Reino Unido/epidemiología , Malformaciones Anorrectales/diagnóstico , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/epidemiología , Estudios Prospectivos , Diagnóstico Tardío/estadística & datos numéricos , Canal Anal/anomalías , Canal Anal/cirugía , Recto/anomalías , Recto/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/diagnóstico , Estudios de Seguimiento , Tráquea/anomalías , Tráquea/cirugía , Ano Imperforado/cirugía , Ano Imperforado/diagnóstico , Lactante , Esófago/anomalías , Esófago/cirugía , Deformidades Congénitas de las Extremidades/diagnóstico , Deformidades Congénitas de las Extremidades/cirugía , Deformidades Congénitas de las Extremidades/epidemiología , Cardiopatías Congénitas/diagnóstico , Cardiopatías Congénitas/cirugía , Riñón/anomalías , Columna Vertebral/anomalíasRESUMEN
BACKGROUND: Long-term urinary outcomes after anorectal malformation (ARM) repair are affected by surgical approach and sacral anomalies. This study aimed to compare laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) in terms of urinary complications. METHODS: Between 2001 and 2022, 45 patients were treated with LAARP or PSARP. The rectourethral fistula and inflow angle between the fistula and rectum was confirmed by preoperative colonography. The incidence of urinary complications and treatment were compared between the two groups. RESULTS: Four patients (14%) had remnant fistula and five patients (17%) had neurogenic bladder dysfunction in LAARP group, while three patients (18%) had urethral injury in PSARP group. All patients with remnant fistula were asymptomatic and followed without treatment. The incidence of remnant fistula improved between earlier decade and later decade. In all cases with urethral injury, suture repair was performed and no postoperative leakage was noted. All five patients with neurogenic bladder dysfunction had spine abnormalities that required clean intermittent catheterization (CIC) and two were free from CIC finally. CONCLUSIONS: It is important to check inflow angle preoperatively to prevent remnant fistula. For PSARP, meticulous dissection is required when separating fistula from urethra because they create common wall. The most contributing factor to neurogenic bladder is sacral anomalies. Preoperative evaluation and postoperative urinary drainage are important.
Asunto(s)
Malformaciones Anorrectales , Laparoscopía , Fístula Rectal , Enfermedades Uretrales , Vejiga Urinaria Neurogénica , Fístula Urinaria , Humanos , Lactante , Recto/cirugía , Recto/anomalías , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/cirugía , Malformaciones Anorrectales/epidemiología , Vejiga Urinaria Neurogénica/etiología , Laparoscopía/efectos adversos , Resultado del Tratamiento , Fístula Rectal/cirugía , Fístula Rectal/complicaciones , Fístula Urinaria/etiología , Fístula Urinaria/cirugía , Enfermedades Uretrales/etiología , Enfermedades Uretrales/cirugía , Complicaciones Posoperatorias/etiología , Uretra/cirugía , Estudios Retrospectivos , Canal Anal/anomalíasRESUMEN
OBJECTIVE: Congenital heart diseases (CHD) are the most frequently associated anomalies with anorectal malformations (ARM). Nevertheless, many specific aspects of CHD in ARM patients have yet to be studied. The aims of this study were to evaluate the prevalence and distribution of CHD in neonates-infants with ARM, and to explore whether the severity of ARM, and the presence of VACTERL association, had an impact on CHD rate, severity, and timing at first cardiac surgery. STUDY DESIGN: All consecutive newborn-infants with ARM managed in our tertiary center (January-1999; December-2021) were collected from a prospective database and retrospectively analyzed. Prevalence and distribution of CHD in ARM patients were assessed. Patients were divided into groups depending on ARM severity and presence of VACTERL association. Pairwise comparison for CHD prevalence, severity, and timing at first cardiac surgery was performed between groups. RESULTS: Of 396 ARM patients identified, those with severe ARM showed a higher number of overall CHD compared to patients with non-severe ARM (36.7 % vs. 25.2 %, p = 0.032). VACTERL + patients had a significantly higher prevalence of CHD (73.4 % vs. 16.4 %; p <0.001) and major CHD (51.7 % vs. 26.9 %; p = 0.008) when compared with VACTERL-patients. Furthermore, VACTERL + patients underwent first cardiac surgery at a significantly younger age than VACTERL-patients (5.2 ± 15.2 months vs. 11.9 ± 6.3, p = 0.039). CONCLUSIONS: Patients with severe ARM had a higher number of CHD compared to patients with non-severe ARM. VATERL + patients had significantly more CHD and more severe CHD than VACTERL-patients. Early screening for CHD is strongly recommended in all newborns diagnosed with ARM before surgery. LEVEL OF EVIDENCE: III retrospective comparative study.
Asunto(s)
Canal Anal , Malformaciones Anorrectales , Cardiopatías Congénitas , Deformidades Congénitas de las Extremidades , Recto , Centros de Atención Terciaria , Tráquea , Humanos , Cardiopatías Congénitas/epidemiología , Canal Anal/anomalías , Recién Nacido , Estudios Retrospectivos , Masculino , Femenino , Malformaciones Anorrectales/epidemiología , Deformidades Congénitas de las Extremidades/epidemiología , Tráquea/anomalías , Recto/anomalías , Recto/cirugía , Prevalencia , Esófago/anomalías , Esófago/cirugía , Columna Vertebral/anomalías , Índice de Severidad de la Enfermedad , Anomalías Múltiples/epidemiología , Riñón/anomalíasRESUMEN
BACKGROUND: Despite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. METHODS: A retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. RESULTS: Twenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1-9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). CONCLUSIONS: Using MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. LEVEL OF EVIDENCE: Level III.
Asunto(s)
Malformaciones Anorrectales , Incontinencia Fecal , Imagen por Resonancia Magnética , Humanos , Estudios Retrospectivos , Masculino , Femenino , Imagen por Resonancia Magnética/métodos , Malformaciones Anorrectales/cirugía , Incontinencia Fecal/etiología , Lactante , Preescolar , Resultado del Tratamiento , Laparoscopía/métodos , Estudios de Seguimiento , Estreñimiento/etiología , Canal Anal/anomalías , Canal Anal/cirugía , Niño , Cirugía Asistida por Computador/métodos , Recto/cirugía , Recto/anomalías , Complicaciones Posoperatorias/etiología , Enema/métodos , Ano Imperforado/cirugía , Ano Imperforado/diagnóstico por imagen , Laxativos/uso terapéutico , Laxativos/administración & dosificaciónRESUMEN
In children with anorectal malformations (ARMs), it is essential to have a diagnostic imaging method that helps with the evaluation of the internal anatomy. In patients with a persistent cloaca, an ARM variant, in which the measurement of the urethral channel and common channel determines surgical management, there are multiple options for imaging. Magnetic resonance imaging (MRI) is an excellent method for this purpose, from which accurate measurements of channel length can be obtained. Additionally, the use of volumetric/isotropic sequences allows multiplanar reformatting. We present our experience with pelvic MRI and intracavitary non-paramagnetic contrast (MR genitography). This method uses volumetric T2-weighted images and the instillation of saline solution as a contrast agent to distinguish the common channel, length of the urethra, anatomy of the vagina, and presence and location of the rectal fistula. We believe this technique to be particularly useful for those working in settings with limited MRI resources.
Asunto(s)
Malformaciones Anorrectales , Femenino , Humanos , Niño , Animales , Malformaciones Anorrectales/diagnóstico por imagen , Malformaciones Anorrectales/cirugía , América Latina , Hospitales Pediátricos , Imagen por Resonancia Magnética/métodos , Espectroscopía de Resonancia Magnética , Recto/diagnóstico por imagen , Recto/anomalías , Cloaca/diagnóstico por imagen , Cloaca/anomalíasRESUMEN
The objective of this scoping review is to provide a summary of the current literature regarding adolescents and young adults with histories of cloacal anomalies. Preferred Reporting Items for Systematic Reviews and Meta-analysis Extension for Scoping Reviews were used. Data were categorized into four domains-urologic, colorectal, gynecologic/obstetric, and sexual/psychosocial. The current literature has poor study quality and mostly consists of retrospective studies of small cohorts with varying definitions of outcomes. Women with cloacal anomalies are at high risk for urologic dysfunction but can maintain kidney health and achieve social continence with medical and surgical management. Sexual function and adult healthcare transition are areas ripe for improved future research.
Asunto(s)
Colon , Sistemas de Apoyo Psicosocial , Recto , Transición a la Atención de Adultos , Anomalías Urogenitales , Adolescente , Femenino , Humanos , Adulto Joven , Colon/anomalías , Riñón/anomalías , Recto/anomalías , Estudios Retrospectivos , Anomalías Urogenitales/psicologíaRESUMEN
AIM: Compare the laparoscopic treatment (LT) and the posterior sagittal anorectoplasty treatment (ST) of the rectovaginal fistulas (RvaF) in a single center. We have previously reported feasibility and results of LT in this rare variety of anorectal malformations (ARM) [1-3]. MATERIAL AND METHODS: 19 patients were treated between February 2000 and November 2020. Nine underwent a LT and 10 a ST. Both surgical techniques were previously described. [2][4][5] The distal posterior wall of the vagina was kept intact in the LT. A technical change was introduced in the ST for that purpose. The fistula was treated from the inside of the rectum, avoiding the opening of the distal vagina as described for the treatment of a urethra rectal bulbar fistula in males. Age at operation, associated anomalies, sacral ratio index (SR), complications, urinary continence, presence of spontaneous intestinal movements, constipation, soiling and requirements of bowel management program (BMP) were analyzed. RESULTS: Associated anomalies occurred in 17 patients (89.5 %), 63 % of which were urological. Five (26 %) had a SR below 0.4; 4 in the LT group and 1 in the ST group. The mean age at the time of operation was 23.2 (8-59) in ST and 17.6 months (4-32) in LT. Average operative time was 190.4 min for ST (120-334) and 195.8 min (90-270) for LT (p 0.13). One patient in the LT group presented a mild rectal prolapse and 2 a partial wound dehiscence after the ST. Only 15 patients were evaluable for functional results (8 in ST and 7 in LT). Mean follow up was 83 months (12-197). All patients are clean with a bowel management program. Five of the 7 patients undergoing a LT had a bad prognosis (SR < 0,4). Three (43 %) are clean with diet or any treatment, 3 (43 %) using laxatives or enemas and 1 (14 %) with a trans anal irrigation system. Only 1 of the 8 patients in the STgroup had a bad prognosis. Six (40 %) needed a diet; 4 (50 %) laxatives or enemas and 1 (10 %) a cecostomy button for antegrade enemas. CONCLUSIONS: Patients with RvaF had a high index of associated anomalies. The difference of operative time was not statistically significative. No differences in functional results between both groups were observed. LT is a valid option to treat RvaF.
Asunto(s)
Malformaciones Anorrectales , Laparoscopía , Fístula Rectal , Masculino , Femenino , Humanos , Lactante , Recto/cirugía , Recto/anomalías , Fístula Rectovaginal/etiología , Fístula Rectovaginal/cirugía , Laxativos , Resultado del Tratamiento , Fístula Rectal/cirugía , Laparoscopía/métodos , Malformaciones Anorrectales/cirugía , Canal Anal/cirugía , Estudios RetrospectivosRESUMEN
OBJECTIVE: To present a unique series of children with previously repaired anorectal malformations (ARM) with subsequent urethral pathology repaired via a posterior sagittal exposure and highlight the associated technical advantages. METHODS: Using a retrospective review of all procedures performed in our pediatric colorectal and pelvic reconstruction program from January 2020 through December 2022, we compiled a case series of patients with a history of ARM and prior posterior sagittal anorectoplasty (PSARP) who had urethral pathology and concurrent indication for redo-PSARP. Clinical features, operative details, and postoperative outcomes were collected. RESULTS: Six male patients presented at a median age of 4.3 years, all born with an ARM of recto-urinary fistula type, of which 3 were recto-prostatic, 1 recto-bladder-neck, and 2 unknown type. In addition to redo-PSARP, 2 underwent remnant of the original fistula excision and 4 had urethral stricture repair. One required post-operative Heineke-Mikulicz anoplasty. Patients underwent cystoscopy 4-6 weeks post-reconstruction, and none showed urethral stricture requiring treatment. Post-procedurally, 5 patients were able to void urethrally and 1 required additional bladder augmentation/Mitrofanoff. CONCLUSION: Redo-PSARP completely mobilizes the rectum, thereby providing excellent exposure to the posterior urethra for repair. This approach also allows the option of a rectal flap for augmented urethroplasty as well as harvest of an ischiorectal fat pad for interposition.
Asunto(s)
Malformaciones Anorrectales , Fístula Rectal , Estrechez Uretral , Humanos , Masculino , Niño , Preescolar , Malformaciones Anorrectales/complicaciones , Malformaciones Anorrectales/cirugía , Uretra/cirugía , Estrechez Uretral/patología , Canal Anal/anomalías , Resultado del Tratamiento , Recto/cirugía , Recto/anomalías , Estudios Retrospectivos , Fístula Rectal/cirugíaRESUMEN
BACKGROUND: In recent decades, magnetic resonance imaging (MRI) has gained prominence as a standard diagnostic method for preoperative assessment in patients with anorectal malformations and a colostomy, with the potential to replace the classic fluoroscopic distal pressure colostogram (FDPC). Three MRI techniques are available: MRI-distal pressure colostogram with gadolinium (MRI-DPCG) or saline (MRI-DPCS) instillation into the colostomy and native MRI without colostomy instillation. OBJECTIVE: To evaluate and compare the diagnostic accuracy of MRI (native MRI, MRI-DPCG and MRI-DPCS) in the preoperative workup of boys with an anorectal malformation and a colostomy and to compare it to FDPC. MATERIALS AND METHODS: Sixty-two boys with preoperative MRI using one of the three approaches and 43 with FDPC met the inclusion criteria for this retrospective study. The presence and localization of rectal fistulas according to the Krickenbeck classification were evaluated and compared with intraoperative findings. RESULTS: The accuracy of fistula detection for MRI in general (regardless of the technique), MRI-DPCS, MRI-DPCG, native MRI and FDPC was 95% (59/62, P<0.001), 100% (12/12, P=0.03), 100% (30/30, P<0.001), 85% (17/20, P=0.41) and 72% (31/43, P=0.82), respectively. The accuracy of describing fistula type in patients with a correctly detected fistula using these methods was 96% (45/47, P<0.001), 100% (9/9, P<0.001), 100% (23/23, P<0.001), 87% (13/15, P<0.001) and 67% (13/21, P=0.002), respectively. CONCLUSION: MRI is a reliable method for detecting and classifying fistulas in boys with an anorectal malformation and a colostomy and can be considered the modality of first choice for preoperative workup.
Asunto(s)
Malformaciones Anorrectales , Fístula Rectal , Masculino , Humanos , Malformaciones Anorrectales/diagnóstico por imagen , Malformaciones Anorrectales/cirugía , Recto/diagnóstico por imagen , Recto/cirugía , Recto/anomalías , Colostomía , Estudios Retrospectivos , Imagen por Resonancia Magnética/métodos , Fístula Rectal/cirugía , Espectroscopía de Resonancia MagnéticaRESUMEN
BACKGROUND: Assessment of postoperative bowel function in anorectal malformation (ARM) patients is crucial for benchmarking outcomes. We compared existing bowel function scoring systems in various aspects in patients with ARM. METHODS: With ethical approval, this was a cross-sectional study involving 5 paediatric surgery referral centres in Malaysia, comparing the Kelly, Japanese Study Group of Anorectal Anomalies (JSGA), Holschneider and Krickenbeck bowel function questionnaires. We recruited patients aged 4-17 years, who had completed definitive surgery & stoma closure (where relevant) > 12 months prior to participation. We standardised outcomes of each scoring system into categories ('good', 'fair', 'poor' and 'very poor') to facilitate comparison. Parents & patients were surveyed and asked to rate the ease of understanding of each questionnaire. The difference in protocol scores rated between parents and patients were compared. Association of each bowel function scoring protocol with type of anomaly was assessed. Statistical significance was p < 0.05. RESULTS: Thirty-nine parents (21 mothers, 18 fathers) and 23 patients were included in this study. Fair agreement was found between Kelly and Krickenbeck protocols (κ = 0.343; p < 0.001), between JSGA constipation and Holschneider protocols (κ = 0.276; p = 0.002); JSGA constipation and Krickenbeck protocols (κ = 0.256; p = 0.004); and between Holschneider and Krickenbeck protocols (κ = 0.273; p = 0.003). Only the Kelly protocol showed significant correlation between parents and patients' answers (ρ = 0.459, p = 0.028). Krickenbeck demonstrated the best negative correlation of patients' scores with ARM types (ρ = -0.401, p = 0.001). The Kelly protocol ranked highest when comparing ease of understanding. CONCLUSION: All the questionnaires appeared comparable in assessing postoperative faecal continence in ARM patients. The Kelly questionnaire performed best in 3 key areas of assessment. LEVEL OF EVIDENCE: Level III Cross-Sectional Study.
Asunto(s)
Malformaciones Anorrectales , Incontinencia Fecal , Niño , Humanos , Malformaciones Anorrectales/cirugía , Recto/cirugía , Recto/anomalías , Defecación , Canal Anal/cirugía , Estudios Transversales , Incontinencia Fecal/etiología , Estreñimiento/etiologíaRESUMEN
Due to the controversy on the feasibility of laparoscopic-assisted anorectoplasty (LAARP) for the treatment of the anorectal malformation (ARM) with rectobulbar fistula (RBF), this study aimed to compare the outcomes of LAARP and posterior sagittal anorectoplasty (PSARP) for ARM with RBF. Demographic data, postoperative complications, and bowel function of RBF patients who underwent LAARP and PSARP at 2 medical centers from 2016-2018 were retrospectively reviewed. Eighty-eight children with RBF were enrolled, including 43 in the LAARP group and 45 in the PSARP group. There were no significant differences in the sacral ratio (P = .222) or sacral agenesis (P = .374). Thirty-seven and 38 patients in the LAARP and PSARP groups were followed up for a median of 4.14 years. The postoperative complications were comparable between the groups (P = .624), with no cases of urethral diverticulum. The urination of all cases was normal and no evidence of cyst formation was found on MCU or MRI during the follow-up period. The incidence of rectal prolapse was similar between the 2 groups (9.3% vs 17.8%, P = .247). The groups had equivalent Bowel Function Score (15.29 ± 2.36 vs 15.58 ± 2.88, P = .645), but the LAARP group had better voluntary bowel movement (94.6% vs 84.2%, P = .148) by Krickenbeck classification. The intermediate-term outcomes of LAARP show that the urethral diverticulum was rare by the intraluminal incision of the fistular and the bowel function was comparable to that of PSARP in ARM with rectobulbar fistula. However, LAARP was associated with smaller perineal wounds.