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PURPOSE: To highlight the utility of Colorectal Nurse Specialist (CNS) supervised parental administration of rectal washouts in the management of Hirschsprung's disease (HD). METHODS: Retrospective case note review of HD patients treated at a tertiary children's hospital in United Kingdom from January 2011 to December 2022. Data collected included demographics, complications, enterocolitis, obstructive symptoms and stomas. Primary pull-through (PT) is done 8-12 weeks after birth. Parental expertise in performing rectal washouts at home is ensured by our CNS team before and after PT. RESULTS: PT was completed in 69 of 74 HD patients. Rectal washouts were attempted on 63 patients before PT. Failure of rectal washout efficacy necessitated a stoma in four patients (6.4%). Of the 65 patients who had PT and stoma closed, three (4.5%) required a further stoma over a mean follow-up period of 57 months (Range 7-144 months). Two of these had intractable diarrhoea due to Total Colonic Aganglionosis (TCA). One patient (1.5%) had unmanageable obstructive symptoms requiring re-diversion. Hirschsprung-associated enterocolitis (HAEC) requiring hospital admission occurred in 14 patients (21%). CONCLUSION: Our stoma rates are lower compared to recent UK data. This could potentially be due to emphasis on parental ability to perform effective rectal washouts at home under CNS supervision.
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Neoplasias Colorrectales , Enterocolitis , Enfermedad de Hirschsprung , Enfermeras Especialistas , Niño , Humanos , Enfermedad de Hirschsprung/cirugía , Estudios Retrospectivos , PadresRESUMEN
Background: Conventionally, oral feeds after distal bowel anastomosis surgery (ileostomy/colostomy closure) are delayed until after bowel peristalsis is established. The safety of an early feeding regimen is not established in children. This study compared early feeding regimens with delayed feeding in children undergoing elective intestinal anastomosis surgeries. Materials and Methods: In this retrospective multicentric cohort study, children undergoing elective distal bowel anastomosis surgery were divided into Group A (oral feeds allowed within 6 h) and Group B (delayed feeds). The two groups were compared for the incidence of abdomen distension, vomiting, surgical site infection, duration of analgesia, length of hospital stay, and readmission rate. Results: During the study, 58 patients were included: Group A (n = 26) and Group B (n = 32). The duration of analgesia (1.9 vs. 4.01 days) and length of hospital stay (3.38 vs. 5.0 days) were significantly less in Group A. Abdominal distension (7.7% vs. 15.6%), vomiting (11.5% vs. 15.6%), surgical site infection rate (3.8% vs. 12.5%), and readmissions (0% vs. 3.1%) were less in Group A, but statistically not significant. Conclusion: Early feeding after the elective restoration of distal bowel continuity can be safely practiced in the pediatric population. It is associated with a reduced need for analgesia and shorter hospital stay.
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Posterior urethral valves are a common cause of congenital bladder outlet obstruction. Known associations include cardiac malformations and gastrointestinal abnormalities. In this case series, we report on two cases of PUV associated with anorectal malformations along with a case of PUV in monochorionic diamniotic twins. We explore the difficulty in achieving a diagnosis and the final management. The association of posterior urethral valves in a patient with anorectal malformation should be suspected in case of associated oligohydramnios or oliguria postnatally. There should be a high index of suspicion in twin pregnancy even if only one of the twins is suspected of bladder outlet obstruction.
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PURPOSE: Screening investigations for the vertebral, anorectal, cardiac, tracheo-oesophageal, renal and limb (VACTERL) association form an important part of the management of neonates with anorectal malformations (ARMs). We developed a proforma to define investigations and indications for referral. The aim of the current study was to determine if the proforma could improve screening rigour. METHODS: Four centres performed a 3-year retrospective audit of neonates with ARM. Following introduction of a proforma, the same data were collected prospectively for consecutive neonates over a further 2 years. The appropriate investigation of each component of the VACTERL association and the corresponding referral required for each abnormal result were defined. The proportion of patients undergoing appropriate investigation and referral was compared against these standards. An audit standard of 90% was set for each criteria. RESULTS: Prior to implementation of the proforma, 86 patients were audited, with a further 69 patients after. The audit standard was met in 7 criteria before introduction of the proforma in comparison to 10 criteria afterwards. CONCLUSION: The completeness of VACTERL screening and its documentation improved following introduction of the proforma. Performance remains imperfect. Review of specific criteria (such as definition of vertebral body screening) will help address this.
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Canal Anal/anomalías , Malformaciones Anorrectales/epidemiología , Auditoría Clínica , Esófago/anomalías , Cardiopatías Congénitas/diagnóstico , Riñón/anomalías , Deformidades Congénitas de las Extremidades/diagnóstico , Tamizaje Neonatal , Derivación y Consulta , Columna Vertebral/anomalías , Tráquea/anomalías , Humanos , Recién Nacido , Estudios Prospectivos , Estudios RetrospectivosRESUMEN
BACKGROUND: The presence of 13 pairs of ribs on pre-operative chest x-ray has been postulated to be an indicator for long gap esophageal atresia (EA). This study sought to determine the validity of this theory and identify associated pathological conditions in patients with EA and abnormal rib count. METHODS: Babies with EA from January 2005 - December 2012 were retrospectively analyzed. Information was gathered from neonatal health records and operation notes. Chest x-rays were reviewed to determine rib count. Long gap EA was defined as failure to achieve primary esophageal anastomosis. Statistical analysis performed with Fisher's exact test. RESULTS: Seventy-six patients were identified. Eight patients had long gap EA, with none of these patients having 13 pairs of ribs. Paradoxically, 10 patients with esophageal atresia +/- trachea-esophageal atresia (EA +/- TEF) and supernumerary ribs underwent primary repair. Nine patients had 11 pairs of ribs, of which 2 had pure EA and a long gap. Using Fisher's exact test to compare the groups of supernumary ribs and non-supernumary ribs there is a p value of 0.587. VACTERL association was identified in 40% of those with supernumerary ribs. Various associated syndromes and concomitant abnormalities were identified. CONCLUSION: We found no association between 13 pairs of ribs and long gap in esophageal atresia. Those with 13 pairs of ribs were more likely to have associated anomalies, although this was not statistically significant. Our cohort of patients was found to have a range of pathology related to genetic syndromes, further atresias, and malformations, which is well known to be associated with children born with EA +/- TEF. LEVEL OF EVIDENCE: Prognosis study - level IV.
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Anomalías Múltiples , Atresia Esofágica/patología , Costillas/anomalías , Fístula Traqueoesofágica , Anastomosis Quirúrgica , Atresia Esofágica/cirugía , Femenino , Humanos , Recién Nacido , Masculino , Pronóstico , Radiografía , Estudios Retrospectivos , Costillas/diagnóstico por imagen , Síndrome , Fístula Traqueoesofágica/cirugíaRESUMEN
BACKGROUND: Benign cystic peritoneal mesothelioma is a rare tumor comprising multiple cysts typically found within the abdominal cavity. Although the literature has numerous accounts of this entity in adults, only a handful of cases have been published regarding its presence in the pediatric population. CASE: We present the case of a prepubertal 10-year-old girl with the finding of benign cystic peritoneal mesothelioma at laparoscopy for suspected appendicitis, the management, and outcome. SUMMARY AND CONCLUSION: Although benign cystic peritoneal mesothelioma can be treated with primary excision, the high recurrence rate in adult cases of this disease necessitates the need for long-term follow-up of these patients and provides a challenge to surgeons in providing continuing care.
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Mesotelioma Quístico/cirugía , Neoplasias Peritoneales/cirugía , Apendicitis/diagnóstico , Niño , Diagnóstico Diferencial , Femenino , Humanos , Laparoscopía/métodos , Mesotelioma Quístico/patología , Recurrencia Local de Neoplasia , Neoplasias Peritoneales/patología , Resultado del TratamientoRESUMEN
INTRODUCTION: Rectal atresia/stenosis is a rare disorder in the spectrum of anorectal malformations and is particularly associated with a presacral mass. These patients are born with a normal anal canal but have a stricture or complete atresia located a few centimeters proximal to the dentate line. We present a surgical technique for the management of these patients, as well as their unique clinical concerns and outcomes. METHODS: We reviewed the records of 14 patients with rectal atresia and 3 with rectal stenosis. We describe a novel technique that we have developed for the preservation of the anterior dentate line that was performed in the last 13 patients. RESULTS: Rectal atresia/stenosis was associated with a presacral mass in 5 patients (29%). Definitive repair was completed using a circular rectorectal anastomosis in the first 4 patients and an anterior dentate line sparing procedure in the last 13. All patients older than 3 years have demonstrated the ability to have voluntary bowel movements. CONCLUSION: With the largest reported series of rectal atresia/stenosis, we have demonstrated a safe and effective technique for repair. Preoperative evaluation must be thorough because a significant number of these patients will have an associated presacral mass.