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Journal of Pediatric Surgery Feb 2024Controlled outcomes into adulthood for females with anorectal malformation (ARM) are still scantily studied. The primary aim was to investigate bowel function, bladder...
BACKGROUND
Controlled outcomes into adulthood for females with anorectal malformation (ARM) are still scantily studied. The primary aim was to investigate bowel function, bladder function and health-related quality of life (HRQoL) in females operated for ARM.
METHODS
A cross-sectional questionnaire-based observational study was performed including females treated for ARM at our institution between 1994 and 2017. The bowel function was assessed with bowel function score (BFS) and urinary tract function with lower urinary tract symptoms (LUTS) questionnaires. HRQoL was investigated with validated age-dependent questionnaires. Patient characteristics were retrospectively retrieved from the medical records and descriptive statistics were used for analysis. HRQoL outcomes were compared with normative data whilst bowel and bladder function outcomes were compared to age-matched female controls.
RESULTS
Forty-four (41.5 %) of 106 females responded to the questionnaires. Ten of 29 patients (34.5 %) aged 4-17 years and 4 of 14 patients (28.6%) aged ≥18 years, reported a well-preserved bowel function (BFS≥17). Constipation issues decreased with age. BFS was similar in patients with perineal and vestibular fistulas. Thirty-six (83.7%) of the patients had at least one LUTS. No adult patients had issues with involuntary urinary leakage. Adults scored significantly (p = 0.004) lower than normative data regarding HRQoL, while children and adolescents scored comparably to norm data.
CONCLUSIONS
Only 28.6 % of the adult patients reported a well-preserved bowel function, similar to the proportion reported by children 4-17 years of age. Adult patients appear to have a diminished HRQoL, however the correlation with BFS was weak.
LEVEL OF EVIDENCE
III.
PubMed: 38443293
DOI: 10.1016/j.jpedsurg.2024.02.011 -
Indian Journal of Pathology &... Apr 2024Multicystic dysplastic kidney (MCDK) is defined as the presence of multiple noncommunicating cysts of various sizes, detected sonographically, without evidence of...
OBJECTIVES
Multicystic dysplastic kidney (MCDK) is defined as the presence of multiple noncommunicating cysts of various sizes, detected sonographically, without evidence of functioning renal parenchyma on dimercaptosuccinic acid renal scan. It has an incidence of 1:4000 live births. They are more commonly diagnosed in boys, usually on the left side, but may also be bilateral. There is the presence of primitive ducts surrounded by fibromuscular connective tissue. These are because of the disturbed connection of the ureteric bud with renal blastema and abnormal division at the stage of metanephros, resulting in an abnormal metanephros differentiation.
MATERIALS AND METHODS
Thirty cases of MCDK were included to study their histomorphology along with their clinical features. Cases were retrieved from the last seven years (2015-2021) from the Department of Pathology, Maulana Azad Medical College.
RESULT
Age ranged from 10 days to 18 years. The cases were between 1 years and 5 years of age. Six out of 30 cases (20%) were infants with three of them being neonates. Twenty-one cases were males. All the cases had unilateral kidney involvement with the left kidney being involved in 20 out of 30 cases. Twenty-eight cases underwent nephrectomy in view of small contracted nonfunctional kidneys with one of them being horseshoe shaped. Five cases had associated hydronephrosis (two ipsilateral and three bilateral). One case had Hirshprung's disease, four had anorectal malformation, two had posterior urethral valves with vesicourethral reflux, one had duplex moiety, and one had undescended testes. On histopathological examination, all of them showed the presence of immature disorganized tubules surrounded by a collarette of immature mesenchymal stroma. One of the cases showed osteoid formation and four had areas of immature cartilage. Normal kidney parenchyma was seen at the periphery in four cases.
CONCLUSION
This series has been presented to highlight the various histomorphological features of MCDK. MCDK can be managed conservatively in most of cases due to autoinvolution and, hence, needs to be differentiated from other close differentials like polycystic kidney disease, cystic nephroma, and cystic partially differentiated nephroblastoma in order to avoid unnecessary surgical intervention.
Topics: Humans; Multicystic Dysplastic Kidney; Male; Female; Tertiary Care Centers; Child; Infant; Adolescent; Child, Preschool; Infant, Newborn; Kidney; Nephrectomy
PubMed: 38427756
DOI: 10.4103/ijpm.ijpm_202_23 -
Journal of Indian Association of... 2024Complications following posterior sagittal anorectoplasty (PSARP) for ARM are well known. In this article, we present our experience of managing five patients who...
AIMS AND OBJECTIVES
Complications following posterior sagittal anorectoplasty (PSARP) for ARM are well known. In this article, we present our experience of managing five patients who required major redo surgeries for complications resulting from previous attempts to correct ARM.
MATERIALS AND METHODS
We reviewed all patients who underwent major redo surgeries in our hospital for complications from previous repairs for ARM, from June 2013 to June 2019. Data was obtained from hospital records and analysed.
RESULTS
Five patients whose ages ranged from 5 months to 14 years were included in the study. Four were boys and 1 was a girl. All patients had undergone PSARP in other hospitals. The presentations were retained distal bowel causing urinary retention and constipation (n=1), pulled through proximal urethra and bladder neck presenting as passage of urine from neo-anus (n=1), retained common channel (of cloaca) causing a 'H' type configuration (n=1), mispositioned neo-anus (n=1) following a primary PSARP and lastly undivided recto-urethral fistula causing fecaluria (n=1). All of them underwent redo repairs by posterior sagittal approach with documented improvement in their symptoms. Two of them required total bowel management to remain clean.
CONCLUSION
All the complications reported here have been described in literature nevertheless, this report will add to the body of experience. Posterior sagittal approach (PSA) has proved to be very successful technique in correcting these complications.
PubMed: 38405235
DOI: 10.4103/jiaps.jiaps_101_23 -
The British Journal of Surgery Jan 2024
Review
Topics: Humans; Anal Canal; Anorectal Malformations; Registries; Retrospective Studies
PubMed: 38364059
DOI: 10.1093/bjs/znae019 -
Annals of African Medicine 2024Colostomy is one of the common surgical procedures performed in pediatric surgical practice. The aim of this study was to retrospectively review our experience with...
BACKGROUND
Colostomy is one of the common surgical procedures performed in pediatric surgical practice. The aim of this study was to retrospectively review our experience with colostomy and closure (reversal) in children.
PATIENTS AND METHODS
A retrospective review of the data of all children aged 15 years and below who had colostomy and colostomy closure in the past 5 years.
RESULTS
Of the 67 children who had colostomy 42 (62.7%) boys and 25 (37.3%) girls, with an age range between 13 months and 8 years. Fifty-six (83.6%) of the children were <2 years. Anorectal malformation 53 (79.1%) was the common indication. Divided colostomy was performed in 62 (92.5%) patients and loop colostomy was performed in 5 (7.5%) patients. All the patients had intraperitoneal colostomy closure. A complication rate of 26.4% was seen. Duration of hospital stay ranged between 4 and 10 days. No mortality was recorded.
CONCLUSION
Colostomy reversal is a safe procedure but morbidity may ensure and can easily manage.
Topics: Male; Child; Female; Humans; Infant; Colostomy; Retrospective Studies; Intestine, Large; Anorectal Malformations; Morbidity; Postoperative Complications
PubMed: 38358167
DOI: 10.4103/aam.aam_95_23 -
Journal of Pediatric Surgery Jan 2024Vesico-ureteral reflux (VUR) is a common associated urological anomaly in anorectal malformation (ARM)-patients. High-grade VUR requires antibiotic prophylaxis to...
BACKGROUND
Vesico-ureteral reflux (VUR) is a common associated urological anomaly in anorectal malformation (ARM)-patients. High-grade VUR requires antibiotic prophylaxis to prevent urinary tract infections (UTI's), renal scarring and -failure. The exact prevalence of high-grade VUR in ARM patients is unknown. Hence, the aim of this study was determining the incidence of high-grade VUR in ARM-patients, and its associated risk factors.
METHODS
A multicenter retrospective cohort study was performed using the ARM-Net registry, including data from 34 centers. Patient characteristics, screening for and presence of renal anomalies and VUR, sacral and spinal anomalies, and sacral ratio were registered. Phenotypes of ARM were grouped according to their complexity in complex and less complex. Multivariable analyses were performed to detect independent risk factors for high-grade (grade III-V) VUR.
RESULTS
This study included 2502 patients (50 % female). Renal screening was performed in 2250 patients (90 %), of whom 648 (29 %) had a renal anomaly documented. VUR-screening was performed in 789 patients (32 %), establishing high-grade VUR in 150 (19 %). In patients with a normal renal screening, high-grade VUR was still present in 10 % of patients. Independent risk factors for presence of high-grade VUR were a complex ARM (OR 2.6, 95 %CI 1.6-4.3), and any renal anomaly (OR 3.3, 95 %CI 2.1-5.3).
CONCLUSIONS
Although renal screening is performed in the vast majority of patients, only 32 % underwent VUR-screening. Complex ARM and any renal anomaly were independent risk factors for high-grade VUR. Remarkably, 10 % had high-grade VUR despite normal renal screening. Therefore, VUR-screening seems indicated in all ARM patients regardless of renal screening results, to prevent sequelae such as UTI's, renal scarring and ultimately renal failure.
TYPE OF STUDY
Observational Cohort-Study.
LEVEL OF EVIDENCE
III.
PubMed: 38355337
DOI: 10.1016/j.jpedsurg.2024.01.008 -
BJR Case Reports Jan 2024Congenital pouch colon (CPC) is highly uncommon congenital anorectal malformation where a distended pouch-like structure replaces either some part of the colon or the...
Congenital pouch colon (CPC) is highly uncommon congenital anorectal malformation where a distended pouch-like structure replaces either some part of the colon or the entire colon and communicates to the genitourinary tract through a fistula. Diagnosis of CPC is usually made after birth when neonate/infant presents with abdominal distension and absence of anal opening. Making antenatal diagnosis of CPC is difficult because of the lack of specific and verifiable signs on sonography. Hence, only a few cases of antenatal diagnosis of CPC have been reported. In our case, CPC was suspected on a routine antenatal growth scan ultrasound in the late third trimester, showing a hypoechoic tubular-shaped lesion in the pre-sacral region. With this suspicion, we suggested an institutional delivery at a tertiary level centre, and diagnosis of type III CPC was confirmed on post-delivery imaging and emergency primary surgery, done on the day 3 of life (pouch resection, division of fistula, and protective colostomy). The child also underwent further corrective surgeries in a staged manner in second year of life and recovered completely. Beforehand diagnosis prevented any unnecessary delay in operative care, reduced postoperative complications, and improved the overall outcome of this otherwise complex condition.
PubMed: 38352258
DOI: 10.1093/bjrcr/uaad005 -
European Journal of Pediatric Surgery... Jan 2024We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The...
We present a simple surgical technique aiming to improve urine outflow through the common urogenital sinus in cloaca and facilitate drainage of existing hydrocolpos. The study included three cases of cloaca with associated hydrocolpos that were operated during the period 2022 through 2023. The patient is placed in the prone position for a standard posterior sagittal anorectoplasty. The distal rectal fistula is severed flush with the vagina/sinus leaving an open defect in the posterior wall of the vagina/sinus. The defect is then widened distally via a vertical incision (∼1 cm) through the posterior wall of the common urogenital sinus toward but not reaching the perineum. This vertical defect is then closed horizontally displacing the posterior vaginal wall downwards toward the perineum (posterior sinuplasty). The postoperative recovery was uneventful in the three cases. Adequate drainage of hydrocolpos was confirmed by imaging at follow-up, as well as improvement of upper urinary tract dilatation. In selected cases of cloaca, posterior sinuplasty is a simple procedure that can be applied during anorectoplasty to provide effective drainage of associated hydrocolpos.
PubMed: 38351952
DOI: 10.1055/a-2204-8629 -
Pediatric Surgery International Feb 2024Hirschsprung's disease (HD) may result in an impaired quality of life (QoL) due to bowel problems, postoperative complications and other health-related issues. The...
BACKGROUND
Hirschsprung's disease (HD) may result in an impaired quality of life (QoL) due to bowel problems, postoperative complications and other health-related issues. The Hirschsprung and Anorectal Malformation Quality of Life (HAQL) questionnaire is a disease-specific instrument developed in the Netherlands to measure the QoL in patients with HD and anorectal malformations. The aim of this study was to translate, culturally adapt and validate HAQL in a Danish Hirschsprung population.
MATERIAL AND METHODS
Translation and cultural adaptation were performed according to international guidelines. Invitations to participate in the validation were sent to 401 patients operated for HD during the period from 1985 to 2012. A total of 156 patients completed the translated and culturally adapted Danish versions of HAQL and 35 parents of children and adolescents completed the corresponding parent questionnaire. Reliability was evaluated in terms of internal consistency using Cronbach's α and test-retest reliability using Intraclass Correlation Coefficient for the retest step. Known groups comparison was performed with comparison of mild HD (defined as recto-sigmoidal HD) and serious HD (defined as more proximal disease).
RESULTS
The internal consistency of the dimensions was overall satisfactory for adults and adolescents but more problematic for children, where Cronbach's α was less than 0.7 in 60% of the dimensions. For both children and adolescents, the α-value was unsatisfactory for social functioning, emotional functioning, and body image. The test-retest reliability was overall good. The known groups comparison was only able to demonstrate a significant difference between mild and severe HD within one dimension.
CONCLUSIONS
The translated version of the HAQL questionnaires provides an overall reliable instrument for evaluating disease-specific QoL in a Danish HD population, but it is important to acknowledge the limitations of the questionnaire, especially in children and adolescents.
Topics: Adult; Child; Adolescent; Humans; Quality of Life; Anorectal Malformations; Reproducibility of Results; Surveys and Questionnaires; Hirschsprung Disease; Denmark
PubMed: 38334791
DOI: 10.1007/s00383-024-05634-y -
International Journal of Surgery Case... Mar 2024Oesophageal atresia, duodenal atresia, and anorectal malformations are rare. This report describes a case of an infant with these three conditions treated using a...
INTRODUCTION
Oesophageal atresia, duodenal atresia, and anorectal malformations are rare. This report describes a case of an infant with these three conditions treated using a multi-stage surgical procedure.
PRESENTATION OF CASE
A male infant was delivered via caesarean section at 34 weeks and 4 days of gestation, weighing 1709 g. Radiography at birth showed a coil-up of the gastrointestinal tube in the oesophagus, a double bubble sign; the patient was subsequently diagnosed with gross type C oesophageal atresia with duodenal atresia. A gastrostomy was performed at day 0. Oesophago-oesophageal anastomosis was performed after tracheoesophageal fistula and blind-end dissection. A duodeno-duodenal diamond-shaped anastomosis was performed, and a tube enterostomy was created from the gastric area near gastrostomy as a trans-anastomotic feeding tube. A colostomy was performed in the descending colon owing to a non-rotation-type anomaly of intestinal malrotation. After other multi-stage surgeries and weight gain, posterior sagittal anorectoplasty was performed at age 1 year 2 months.
DISCUSSION
Triple atresia (TA), characterized by triumvirate oesophageal atresia, duodenal atresia, and anorectal malformations, remains a clinical puzzle. Notably, standardized therapeutic guidelines for managing TA are lacking. The complexity of this constellation of anomalies necessitates astute diagnostic acumen and strategic treatment planning.
CONCLUSION
Our patient showed a favourable clinical course with an accurate and timely diagnosis, serving as an experience for an innovative multi-stage therapeutic strategy. Our case showed the appropriate challenges of TA while illuminating the potential for successful outcomes through meticulous clinical management.
PubMed: 38310785
DOI: 10.1016/j.ijscr.2024.109340