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Crucial role of rectoanal inhibitory reflex in emptying function after anoplasty in infants with anorectal malformations.

Constipation is a common problem after reconstructive surgery for anorectal malformations. The underlying pathophysiology of the constipation in these patients is unclear. The objective of this study was to compare manometric disturbance in infants with and without post-anoplasty constipation. Anorectal manometry studies were performed within 12 months of anoplasty, as a part of the follow-up protocol, in 24 infants aged less than 3 years who had anorectal malformations. The manometric profiles studied were mean resting anal pressure (ArP), mean resting rectal pressure (RrP), mean resting rectoanal pressure gradient (RRPG), peak squeeze pressure (PSP), and the presence of the rectoanal inhibitory reflex (RAIR). Eight of 24 infants (33%) experienced constipation during the examination period. There was no difference in pressure profiles between low and non-low anomalies. In the non-constipation group, RrP was 5.1 mmHg, ArP was 21.0 mmHg, RRPG was 16.0 mmHg, and PSP was 88.4 mmHg. In the constipation group, RrP was 7.3 mmHg (p = 0.37), ArP was 37.5 mmHg (p = 0.03), RRPG was 3.05 mmHg (p = 0.05), and PSP was 81.7 mmHg (p = 0.77). RAIR was present in 93.75% of cases without constipation and 12.5% of cases with constipation (p < 0.01). One patient who had clinical conversion from constipation to a good result also showed positive conversion of the RAIR. RAIR and anal resting tone play important roles in emptying function. As far as possible, these functions should be preserved during reconstruction.

Child, Preschool↗

Intestinal malrotation in neonates with nonbilious emesis.

Intestinal malrotation is a relatively uncommon condition with diverse outcomes. Familiarity with variations in the presentation of malrotation is imperative as early diagnosis and prompt subsequent surgical intervention are essential to optimizing outcome. The most frequent clinical sign in the neonate is bile-stained emesis. We report three cases of unsuspected malrotation that were diagnosed in neonates with a history of nonbilious emesis who were assessed for presumed gastroesophageal reflux or aspiration. Gastroesophageal reflux is a common condition among newborns, and can be a subtle presentation of malrotation. Clinicians should consider malrotation as a possible cause of reflux, particularly in infants with unusually pathologic or persistent symptoms necessitating ongoing treatment for reflux.

Appendectomy↗

Gastrointestinal duplications.

Gastrointestinal duplications are rare congenital lesions that can develop anywhere along the alimentary tract and may present in the newborn period as an abdominal mass. They are differentiated from other intraabdominal cystic lesions by the presence of a normal gastrointestinal mucosal lining. Multiple theories have been proposed to account for these lesions; however, no single theory adequately explains all the known duplications. They are most frequently single, tubular, or cystic and located on the mesenteric side of the native alimentary tract structure. Symptoms often are related to the location of the duplication; oral and esophageal lesions can create respiratory difficulties, whereas lower gastrointestinal lesions may cause nausea, vomiting, bleeding, perforation, or obstruction. Treatment is resection with care taken to protect the common blood supply of the native structures. Occasionally, a partial resection with mucosal excision is required to preserve intestinal mucosa.

Colon↗

Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.

Anal Canal↗

Anorectal atresia. An experimental model in the rat.

Aiming to assess the development of the anorectal sphincteric apparatus and whether it is influenced by the presence of an intestinal conduit through the muscular complex, we seek to develop an experimental model of anorectal atresia in foetuses of female pregnant rats, given orally the herbicide ethylenethiourea (ETU). Six 3-months-old, 300 g female Wistar rats were included in the study group (SG). The foetuses of another unexposed rat formed the control group (CG). In the SG 54, foetuses were alive, all had hypoplasia of the tail and 47 (87%) had no visible anal orifice. The 18 foetuses of the CG were normal. The histological study was performed at the level of the pubic symphysis. Type I anorectal atresia was defined when this section demonstrated an intestinal conduit and Type II when only a muscular cord was found, without any intestinal tube. This study was completed in 15 (32%) of the SG, 9 (60%) with anorectal atresia Type II and 6 (40%) with atresia Type I, and in 11 animals (61%) of the CG. The thickness of the sphincter muscle was larger in the CG (260.47+/-35.32 micro) than in the SG (208.98+/-55.01 micro), p<0.05, but this difference was due to the animals showing atresia Type II. To conclude: ETU had a teratogenic effect. The foetuses with atresia, particularly Type II, had a decreased development of the muscular complex. The presence of an intestinal conduit does not significantly influence the development of the muscular complex.

Anal Canal↗

Unusual association of upper gastro-intestinal anomalies in a newborn.

We are describing one case of persistent nonbilious post-prandial vomiting in a newborn due to hypertrophic pyloric stenosis, intestinal malrotation and gastric antral diaphragm. Those three anomalies were successively diagnosed as causes of persistent upper gastrointestinal subobstruction.

Digestive System Abnormalities↗

Alimentary tract duplications in infants and children.

This is a review of 30 duplications of the alimentary tract in 28 patients treated at the Surgical Unit of the Children's Department of the Medical University of Pécs, Hungary, and at the Department of Pediatric Surgery of the Medical Academy of Dresden, Germany, from 1964 to 1989. The ages of patients ranged from 1 day to 13 years, 80 percent were less than 2 years of age at initial presentation. There were 6 thoracic, 20 abdominal and 2 thoraco-abdominal duplications. Distended abdomen, vomiting, bowel obstruction and palpable abdominal mass were most frequently encountered. Plain thoracic and abdominal x-rays, ultrasonography, barium esophagogram, barium meal and enema were the most common diagnostic procedures. Emergency operative intervention was required in 18 patients. One infant died of an unrelated disease. Twenty-three duplications were cystic and 3 tubular. One patient had an appendiceal duplication, and another patient a flat lumenless duplication located on the perineum close to the anal opening. The surgical procedure--removal of the duplication--should not be more radical than necessary to eliminate the potential complaints and prevent recurrence. During surgery the common blood supply shared between the duplication and the native bowel must be carefully protected to avoid undue sacrifice of normal bowel.

Adolescent↗

The metabolic response to intravenous medium-chain triglycerides in infants after surgery.

OBJECTIVE: The aim of this study was to determine if administration of mixed medium-chain triglycerides (MCT)/long chain triglycerides (LCT) fat emulsion would increase net fat oxidation and if carbohydrate intake would influence net fat oxidation. STUDY DESIGN: Stable infants receiving total parenteral nutrition were studied after surgery. Respiratory gas exchange was measured by indirect calorimetry and urinary nitrogen excretion by the micro-Kjeldahl method. Intravenous fat (4 g/kg/day) was given as either pure LCT fat emulsion or 50/50 MCT/LCT fat emulsion. Carbohydrate intake was either "high" (15 g/kg/day) or "low" (10 g/kg/day). Four groups of patients were studied: group 1 = LCT and high-carbohydrate; group 2 = LCT and low-carbohydrate; group 3 = MCT/LCT and high-carbohydrate; group 4 = MCT/LCT and low-carbohydrate. RESULTS: At a carbohydrate intake of 15 g/kg/day, the calories available from glucose exceeded the measured resting energy expenditure (REE), and no differences were seen in either energy expenditure or net fat oxidation between patients receiving LCT and MCT/LCT fat emulsions. However, at a carbohydrate intake of 10 g/kg/day, when glucose calories were less than REE, net fat oxidation was significantly higher in patients receiving MCT/LCT (median, 1.94; range, 1.05-2.24 g/kg/day) compared with patients receiving LCT (median, 0.60; range, -0.09 to 1.35; P =.03). CONCLUSION: Providing that carbohydrate calories do not exceed REE, partial replacement of LCT by MCT in intravenous fat emulsions can increase net fat oxidation in infants after surgery.

Dietary Carbohydrates↗

Surgical management of alimentary tract duplications.

Alimentary tract duplications are unusual anomalies that may require surgical intervention in the neonate, infant, and occasionally in the older child. The clinical presentation of patients with alimentary tract duplications includes bleeding, abdominal pain, intussusception, and respiratory distress, or it may be an incidental finding on either abdominal examination or chest x-ray. A review of 96 patients with 101 duplications seen over the last 37 years is reported herein. Twenty-one duplications were confined to the thorax; three were thoracoabdominal, and 77 were abdominal. Seventy-four patients presented as infants less than 2 years of age, and 22 patients were older. Ectopic gastric mucosa was found in 21 duplications, and pancreatic tissue was found in five. Seventy-five duplications were cystic and 26 were tubular. Ultrasonography, computed tomography (CT), and myelography are helpful diagnostic tools. Ninety-four of the 96 patients underwent surgical management for their duplications. One duplication was found at necropsy, and one patient was asymptomatic and did not undergo operation. A single death occurred in a 2-day-old infant who had intrauterine volvulus and meconium peritonitis. Management was based on the age and condition of the patient, the location of the lesion, whether it was cystic or tubular and communicating with the true intestinal lumen, and whether it involved one or more anatomic locations. Generally, total excision was preferred, but staged approaches were sometimes necessary.

Adolescent↗

Results of biofeedback therapy for fecal incontinence in children with encopresis and following surgery for anorectal malformations.

INTRODUCTION: Some children with fecal incontinence respond to biofeedback therapy. However, whether they can achieve fecal continence posttherapeutically has not been clarified. We studied the serial results of biofeedback therapy and discuss the necessity of providing repeated biofeedback therapy at home. METHODS: Nineteen children with encopresis underwent one session of biofeedback therapy. Seven of 15 children with fecal incontinence that developed after surgery for anorectal malformations underwent three to eight sessions of biofeedback therapy; the remaining 8 underwent one (mean, 2.9) session only. The patients were hospitalized for one session of biofeedback therapy. To monitor the clinical outcome of intervention, we used serial score assessments from three months to two years posttherapeutically. RESULTS: Seventeen of 19 (90 percent) patients with encopresis showed clinical improvement after one session of therapy (P < 0.0001). Six months after treatment, however, six of ten (60 percent) patients with encopresis reported recurrent fecal incontinence after one therapeutic session. Clinical improvement was noted in 5 of 15 (33 percent) patients who had fecal incontinence after surgery for anorectal malformations. All five patients showed clinical improvement from six months to two years after several sessions of biofeedback therapy (P < 0.05). CONCLUSIONS: Biofeedback therapy is effective in most children with encopresis and in some children with anorectal malformations. However, some patients need repeated sessions of biofeedback therapy to achieve fecal continence. Therefore, a new portable biofeedback apparatus for the treatment of fecal incontinence at home may be helpful.

Adolescent↗

A case of duodenal web occurring in Prader-Willi syndrome.

Vomiting and abdominal symptoms are uncommonly seen in children with Prader-Willi syndrome (PWS). A case is described of a child with PWS who presented with vomiting, abdominal distension and abdominal pain due to a duodenal web causing gross gastric distension. Prompt attention to the onset of such clinical features in children with PWS may prevent excess morbidity and complications.

Abdominal Pain↗

The current practice of paediatric surgery in Malaysia.

This 10-year review of surgical conditions in infants at the University Hospital, Kuala Lumpur, highlights some of the more common problems encountered and outlines their management. Anorectal agenesis and Hirschsprung's disease were seen relatively more frequently than other anomalies of the gastrointestinal tract. The management of these two conditions and the operation of colostomy and its complications are singled out and presented in some detail.

Anal Canal↗

Enteric and rectal duplications and duplication cysts in the adult.

Alimentary tract duplication and duplication cysts are rare congenital malformations. The ileum is the most frequently affected site. However, alimentary tract duplication and duplication cysts can occur at any point along the gastrointestinal tract. Early diagnosis and prompt surgical treatment is the best way to prevent associated morbidity. This article presents the cases of three patients admitted to Gulhane Military Medical Academy with signs of acute abdomen, intra-abdominal mass and chronic abdominal pain. These patients were found to have enteric duplication, duplication cyst and/or retro-rectal cyst. The literature on alimentary tract duplications is reviewed.

Adult↗

No organ left behind: tales of gut development and evolution.

The function of an organ is dependent on its cellular constituents as well as on their assembly into a cohesive unit. The developing gut faces unique challenges as one of the longest and largest organs in the body and also because it is constantly interfacing with external factors through the diet. Its location deep within the body has until recently hampered investigation into its formation. The patterning of the gut along its longitudinal, dorsoventral, left-right, and radial axes is one of the fascinating issues that pertain to the development, function, and homeostasis of this understudied organ.

Animals↗

Endoscopic laser treatment of vascular anomalies of the upper gastrointestinal tract.

Vascular anomalies of the gastrointestinal tract are an important source of both acute and chronic blood loss. They present difficult management problems as they are often multiple, involving more than one part of the gastrointestinal tract, and may give rise to bleeds of increasing frequency and severity over a period of many years. We present the results of endoscopic argon and Nd YAG laser treatment of 18 patients with documented recurrent, severe haemorrhage from vascular lesions of the upper gastrointestinal tract with follow up of up to five years. Four patients with hereditary haemorrhagic telangiectasia, five with single angiodysplasias and three with multiple angiodysplasias have had their transfusion requirements reduced to minimal levels after one or more courses of laser treatment. Two of these have required no blood for over two years despite previous total requirements of 52 and 129 units of blood. Four patients were submitted to surgery (two in whom the laser failed to control haemorrhage and two who did not have immediate access to laser therapy at the time of severe bleed). Two of these, however, bled again soon afterwards. Two patients are still undergoing courses of laser therapy although have already shown reductions in transfusion requirements. Both lasers were effective. The Nd YAG laser appears to produce better long term results as its greater penetration makes it possible to ablate the main areas of vascular ectasia in the submucosa, although it does have a slightly higher risk of causing haemorrhage in the first few days after treatment.

Adult↗