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International Journal of Surgery Case... May 2021Intestinal malformations, such as intestinal atresia, malrotation, and anorectal malformation, are rare events, but concurrent intestinal atresia and anorectal...
INTRODUCTION
Intestinal malformations, such as intestinal atresia, malrotation, and anorectal malformation, are rare events, but concurrent intestinal atresia and anorectal malformation are rare in combination. These anomalies have similar in utero and perinatal symptoms, which can make the diagnosis of both defects prenatally challenging.
PRESENTATION OF CASE
We present a case of a male infant with a prenatal diagnosis of truncus arteriosus who on a 32-week routine prenatal ultrasound was suspected to have an intestinal malformation. On day of life one, the patient was taken to the operating room and found to have both type IIIa ileal atresia and anorectal malformation with normal bowel in between. A complete work up for vertebral defects, anorectal malformation, cardiac septal defects, esophageal atresia, renal anomalies, and radial limb defects (VACTERL) anomalies did not reveal additional anomalies.
DISCUSSION
In this case, prenatal information supported intestinal obstruction, and the rare combination of both intestinal atresia and anorectal malformation proved surprising and interesting.
CONCLUSION
We suggest providers be aware of the potential of multiple alimentary tract malformations to improve operative preparation and reduce the morbidity or mortality risk from repeat procedures when possible.
PubMed: 33964718
DOI: 10.1016/j.ijscr.2021.105945 -
Journal of Pediatric Surgery Nov 2021Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations...
BACKGROUND
Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses.
METHODS
A retrospective review was performed of patients with ARM treated at our pediatric colorectal center. All patients underwent protocolized urologic screening. ARM subtypes were ordered with increasing severity as follows in males: perineal, bulbar, prostatic and bladder neck fistulae. Females were similarly categorized as perineal, vestibular and vaginal fistulae followed by cloaca with <3 cm common channel and cloaca with >3 cm common channel. The following urologic diagnoses were assessed to determine whether a correlation existed with the severity of the ARM subtype: hydronephrosis, vesicoureteral reflux (VUR), solitary kidney, renal ascent anomalies (ectopic or pelvic), renal fusion anomalies (horseshoe or cross fused kidney), duplex kidney, hypospadias and undescended testicle. ARM subtypes were defined by distal rectal anatomy.
RESULTS
A total of 712 patients were included in our study with a mean age of 4 years and of whom 45% were male. The overall rate of urologic anomalies was greater in males than females (65% vs 56% p < 0.026). In both sexes, the rate of urologic anomalies increased with increasing severity of ARM subtype (p<0.00010) finding that males with bladder neck fistula and females with cloacal malformations, particularly with long common channels, being the highest incidence. In males and females, the rate of hydronephrosis increased as the complexity of ARM increased and this correlated significantly (p < 0.0001 vs p < 0.0003 respectively). Similarly, the incidence of VUR also increased as complexity of ARM increased in both males and females (p = 0.01 and p<0.0001 respectively). The remaining urologic diagnoses were not significantly correlated with severity of ARM.
CONCLUSIONS
Urologic anomalies occur at a high rate in children with ARM and appear to increase in frequency with increasing complexity of ARM subtype. These findings stress the importance of proper ARM screening and proactive collaboration with a clinician with expertise in pediatric urology early in the management of such children to improve early recognition of urologic diagnoses.
LEVEL OF EVIDENCE
Level III.
Topics: Animals; Anorectal Malformations; Child; Child, Preschool; Cloaca; Female; Humans; Male; Rectum; Retrospective Studies; Urology
PubMed: 33752911
DOI: 10.1016/j.jpedsurg.2021.02.051 -
Seminars in Pediatric Surgery Feb 2023Robotic colorectal surgery allows adult and pediatric surgeons to overcome the technical limitations of laparoscopic surgery. It also provides improved ergonomics in the...
Robotic colorectal surgery allows adult and pediatric surgeons to overcome the technical limitations of laparoscopic surgery. It also provides improved ergonomics in the field of surgery. Robotic surgery has several advantages in colorectal operations that require complex minimally invasive skills including anorectal malformations, Hirschsprung disease, and inflammatory bowel disease. In this section, we discuss the key aspects of colorectal surgery where robotic instrumentation seems ideal.
Topics: Adult; Humans; Child; Robotic Surgical Procedures; Colorectal Surgery; Digestive System Surgical Procedures; Inflammatory Bowel Diseases; Laparoscopy; Colorectal Neoplasms
PubMed: 36739693
DOI: 10.1016/j.sempedsurg.2023.151259 -
Journal of Clinical Medicine Mar 2023Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of... (Review)
Review
Despite surgical correction, children with anorectal malformations may experience long-term bowel dysfunction, including fecal incontinence and/or disorders of evacuation. Anorectal manometry is the most widely used test of anorectal function. Although considerable attention has been devoted to its application in the anorectal malformation cohort, there have been few attempts to consolidate the findings obtained. This systematic review aimed to (1) synthesize and evaluate the existing data regarding anorectal manometry results in children following anorectal malformation repair, and (2) evaluate the manometry protocols utilized, including equipment, assessment approach, and interpretation. We reviewed four databases (Embase, MEDLINE, the Cochrane Library, and PubMed) for relevant articles published between 1 January 1985 and 10 March 2022. Studies reporting post-operative anorectal manometry in children (<18 years) following anorectal malformation repair were evaluated for eligibility. Sixty-three studies were eligible for inclusion. Of the combined total cohort of 2155 patients, anorectal manometry results were reported for 1755 children following repair of anorectal malformations. Reduced resting pressure was consistently identified in children with anorectal malformations, particularly in those with more complex malformation types and/or fecal incontinence. Significant variability was identified in relation to manometry equipment, protocols, and interpretation. Few studies provided adequate cohort medical characteristics to facilitate interpretation of anorectal manometry findings within the context of the broader continence mechanism. This review highlights a widespread lack of standardization in the anorectal manometry procedure used to assess anorectal function in children following anorectal malformation repair. Consequently, interpretation and comparison of findings, both within and between institutions, is exceedingly challenging, if not impossible. Standardized manometry protocols, accompanied by a consistent approach to analysis, including definitions of normality and abnormality, are essential to enhance the comparability and clinical relevance of results.
PubMed: 37048627
DOI: 10.3390/jcm12072543 -
Journal of Pediatric Urology Apr 2022Urological problems are a recognised feature of anorectal malformation (ARM). Previous assumptions of favourable long-term urinary outcomes are being challenged.
INTRODUCTION
Urological problems are a recognised feature of anorectal malformation (ARM). Previous assumptions of favourable long-term urinary outcomes are being challenged.
OBJECTIVE
We hypothesised that urinary tract problems are common in ARM and frequently persist into adulthood. We retrospectively reviewed long-term renal and bladder outcomes in ARM patients.
STUDY DESIGN
Patients with ARM born between 1984-2005 were identified from electronic hospital databases. Their case notes were reviewed. Renal outcomes included serum creatinine and the need for renal replacement therapy. Bladder outcomes included symptom review, bladder medication, need for intermittent catheterisation, videourodynamics and whether the patient had undergone augmentation cystoplasty. RESULT (TABLE 1): The case notes of 50 patients were reviewed. The median age at last follow up was 18 years (range 12-34 years). The level of fistula was noted to be high in 17 patients, intermediate in eight, and low in 10. Four had cloaca. Congenital urological abnormalities were present in 25 (50%). An abnormal spinal cord was present in 22 (44%) patients. VACTERL association occurred in 27 (54%). Chronic kidney disease stage II or above was found in 14 (28%) patients, of whom four required a renal transplant. Abnormal bladder outcomes were found in 39 (78%) patients. Augmentation cystoplasty with Mitrofanoff had been performed in 12. Of those who had not undergone cystoplasty, 17 had urinary symptoms, including urinary incontinence in 12. Of the 39 patients with abnormal bladder outcome, 19 (49%) did not have a spinal cord abnormality. There was no significant statistical association between level of ARM and abnormal renal outcome or presence of bladder abnormality.
DISCUSSION
Adverse renal and bladder outcomes are common in our cohort of young people with ARM with a significantly higher incidence compared with current literature. We did not demonstrate an association between level of ARM or presence of spinal cord anomaly with persistent bladder problems. Congenital urological anomalies are more common in those who later have an abnormal renal outcome. Although this difference is statistically significant, one fifth of patients born with anatomically normal upper tracts develop reduced renal function, implying an important acquired component.
CONCLUSION
Bladder problems and reduced renal function affect a significant proportion of young adults with ARM. Neither adverse outcome is reliably predicted from ARM level, congenital urological anomaly or spinal cord anomaly. We advise continued long-term bladder and kidney follow-up for all patients with ARM.
Topics: Adolescent; Adult; Animals; Anorectal Malformations; Child; Cloaca; Humans; Retrospective Studies; Urinary Incontinence; Urology; Young Adult
PubMed: 35283020
DOI: 10.1016/j.jpurol.2022.01.019 -
Pediatric Surgery International Apr 2020The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal... (Review)
Review
The introduction of posterior sagittal anorectoplasty (PSARP) by deVries and Peña in the early 80s has impacted to the treatment of patients with anorectal malformations (ARM). It gained great recognition worldwide in a very short time, and since then, surgeons dealing with the treatment of this complex malformation could achieve tremendous progress in contemporary management of this anomaly. Despite the growing experience and body of information globally, the treatment of ARMs continues to be a challenge to the pediatric surgeons due to the nature and the variability of the anomaly, and short- and long-term problems continue to exist even after nearly 40 years of the PSARP era. Today, knowing more about it, pediatric surgeons are committed to do more for their ARM patients to have them as physically and socially healthy individuals.
Topics: Anal Canal; Anorectal Malformations; Child; Digestive System Surgical Procedures; Humans; Postoperative Complications; Plastic Surgery Procedures; Rectum
PubMed: 32086570
DOI: 10.1007/s00383-020-04629-9 -
World Journal of Pediatric Surgery 2020Congenital esophageal and anorectal malformation are common in neonates. Refractory esophageal anastomotic stricture and abnormal defecation after surgical correction in... (Review)
Review
BACKGROUND
Congenital esophageal and anorectal malformation are common in neonates. Refractory esophageal anastomotic stricture and abnormal defecation after surgical correction in infants are challenging surgical problems. Magnetic compression anastomosis (MCA) using mated magnets with their interposed compressed tissue may result in serosa-to-serosa apposition.
DATA SOURCES
A literature search was performed to establish an algorithm for these accidents by the authors to identify relevant articles published from 1977 to 2019 in Google, Medline, ISI Web of Knowledge Ovid, CNKI and library document delivery, using search terms "magnetics", "esophageal malformation", "anorectal" and "perforation". A total of 24 literatures were collected.
RESULTS
Magnamosis is technically feasible for alimentary tract anastomoses in pediatric patients. The magnets are most commonly made of neodymium-iron-boron and samarium-cobalt alloys, which have been employed to create solid anastomosis for long-gap esophageal atresia and refractory esophageal stricture without thoracotomy in children in recent years. Furthermore, magnamosis can be used for the functional undiversion of ileostomy. In anorectal malformations with favorable anatomy, this procedure may avoid an operative repair such as posterior sagittal reconstruction.
CONCLUSION
Translumenal anastomosis of digestive tract using the MCA is a reliable, minimally invasive and feasible method to treat congenital esophageal and anorectal malformation.
PubMed: 36474917
DOI: 10.1136/wjps-2020-000130 -
Sultan Qaboos University Medical Journal Aug 2022
Topics: Anorectal Malformations; Humans; Male; Penis
PubMed: 36072063
DOI: 10.18295/squmj.9.2021.139 -
Pediatric Surgery International Jan 2020Hirschsprung's disease (HSCR) and anorectal malformation (ARM) are often associated with other congenital malformations, but the association of each other is rare. Some...
BACKGROUND/PURPOSE
Hirschsprung's disease (HSCR) and anorectal malformation (ARM) are often associated with other congenital malformations, but the association of each other is rare. Some studies have reported the incidence of HSCR associated with ARM ranging from 2.0 to 3.4%. The purpose of this study was to update the current epidemiological and therapeutic features of this rare congenital association.
METHODS
A systematic literature search for relevant articles was performed in four databases using a combination of the following terms "association of Hirschsprung's disease and anorectal malformation", "aganglionosis and anorectal malformation" and "congenital megacolon and anorectal malformation" for studies published between 1952 and 2019. Reference lists were screened for additional cases.
RESULTS
Forty-three studies met the defined inclusion criteria, reporting a total of 126 patients who were diagnosed with HSCR with ARM. Thirty articles reported 42 single case reports of this association. Twelve articles reported 66 cases of HSCR in case series of 3309 ARM patients, resulting in an incidence of 2% of this association. Associated syndrome was found in 25 cases (20%): Currarino syndrome in 11, Down syndrome in 8, Cat eye syndrome in 4 and Pallister-Hall syndrome in 2 patients. Extent of aganglionosis was reported in 62 cases: short or rectosigmoid aganglionosis was reported in 44, long segment aganglionosis in 8, total colonic aganglionosis in 9 and total intestinal aganglionosis in 1 case.
CONCLUSION
Although the association of ARM and HSCR is rare, the incidence of HSCR among ARM cases seems to be higher than in the general pediatric population. There was a high incidence of coexistence of ARM and HSCR with severe associated syndromes.
Topics: Anorectal Malformations; Child; Hirschsprung Disease; Humans
PubMed: 31552492
DOI: 10.1007/s00383-019-04580-4 -
Journal of Indian Association of... 2023Gastrointestinal (GI) duplications are rare congenital malformations with diverse presentations. They usually present in the pediatric age, especially in the first 2...
CONTEXT
Gastrointestinal (GI) duplications are rare congenital malformations with diverse presentations. They usually present in the pediatric age, especially in the first 2 years of life.
AIMS
To present our experience with GI duplication (cysts) at a pediatric surgery tertiary care teaching institute.
SETTINGS AND DESIGN
It is a retrospective observational study undertaken in the department of pediatric surgery at our center between 2012 and 2022 for GI duplications.
MATERIALS AND METHODS
All children were analyzed for their age, sex, presentation, radiological evaluation, operative management, and outcomes.
RESULTS
Thirty-two patients were diagnosed with GI duplication. Slight male predominance was present in the series (M: F ≈ 4:3). Fifteen (46.88%) patients presented in the neonatal age group; 26 (81.25%) patients were under 2 years. In the majority of cases ( = 23, 71.88%), the presentation was acute onset. Double duplication cysts on opposite sides of the diaphragm were present in one case. The most common location was ileum ( = 17), followed by gallbladder ( = 6), appendix ( = 3), gastric ( = 1), jejunum ( = 1), esophagus ( = 1), ileocecal junction ( = 1), duodenum ( = 1), sigmoid ( = 1), and anal canal ( = 1). Multiple associations (malformations/surgical pathologies) were present. Intussusception ( = 6) was the most common, followed by intestinal atresia ( = 5), anorectal malformation ( = 3), abdominal wall defect ( = 3), hemorrhagic cyst ( = 1), Meckel's diverticulum ( = 1), and sacrococcygeal teratoma ( = 1). Four cases were associated with intestinal volvulus, three cases with intestinal adhesions, and two with intestinal perforation. Favorable outcomes were present in 75% of cases.
CONCLUSION
GI duplications have varied presentations depending on site, size, type, local mass effect, mucosal pattern, and associated complications. The importance of clinical suspicion and radiology cannot be underrated. Early diagnosis is required to prevent postoperative complications. Management is individualized as per the type of duplication anomaly and its relation with the involved GI tract.
PubMed: 37197242
DOI: 10.4103/jiaps.jiaps_108_22