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At least 127 records · Page 7Linked to original sources
PubMed2004

Tracheal agenesis: management of the first 10 months of life.

Tracheal agenesis is a potentially lethal congenital anomaly, appearing only at birth. We describe a newborn preterm infant who presented with immediate respiratory distress and no audible cry. There was almost complete tracheal agenesis with a very short segment of distal trachea (only two tracheal rings) arising from the anterior wall of the esophagus, before dividing into the mainstem bronchi. The anomaly was unsuspected prenatally, as the scan showed pyloric atresia and complex congenital cardiac disease. Despite the patient's difficult course, with correction of the rare-associated malformations (cardiac and gastrointestinal tract anomalies), the fact that the child is lively and neurologically normal for her age, requires that we now consider the patency of the airway and the possibility of surgical correction, in accordance with a good quality of life.

Abnormalities, Multiple↗
PubMed2006

Anorectal malformation with tubular hindgut duplication.

The association of hindgut duplication and anorectal malformation is rare. Published classifications of this association are confusing in respect of terminology. We report a case of blind-ending, Y-shaped tubular duplication of the distal hindgut, associated with an anorectal malformation (rectourethral fistula) affecting the colon proper. Surgical options at time of presentation and of reconstructive surgery are discussed. A review and suggested modification of the classifications is presented.

Abnormalities, Multiple↗
PubMed1989

Orthotopic transplantation of the liver in children with biliary atresia and polysplenia syndrome: report of two cases.

Biliary atresia is the most common indication for liver transplantation in infants and children, despite the advent of the Kasai operation. Coexisting anomalies, which have been noted in up to 27% of patients with biliary atresia, may form an association known as the "polysplenia syndrome," which includes (1) polysplenia, (2) midgut malrotation, (3) preduodenal portal vein, (4) absent prerenal inferior vena cava with azygos continuation, (5) situs inversus, (6) symmetric liver, (7) hepatic arterial anomalies, and (8) bilobed right lung with hyparterial bronchus. Two of 31 patients undergoing orthotopic liver transplantation for biliary atresia following failed portoenterostomy over the past 11 years manifested the polysplenia syndrome with absent prerenal inferior vena cava. The clinical course of these patients, constellation of anomalies, and technical adjustments required to perform liver transplantation are described. We do not believe that these complex congenital anomalies preclude liver transplantation.

Abnormalities, Multiple↗
PubMed1981

Duplications of the alimentary tract.

A review of 53 patients with 60 duplications of the alimentary tract treated at the Royal Hospital for Sick Children, Glasgow, from 1947 to 1978 has been made. This consisted of 19 foregut, 32 midgut and 9 hindgut duplications. The experience with these patients has been recorded and discussed with reference to the literature.

Abnormalities, Multiple↗
PubMed2006

Foregut abnormalities.

Explore the source record for details and available documents.

Diagnostic Techniques, Digestive System↗
PubMed1990

Cloacal exstrophy: individualized management through a staged surgical approach.

Cloacal exstrophy, centered on the maldevelopment of the primitive streak mesoderm and cloacal membrane, results in bladder and intestinal exstrophy, omphalocele, gender confusion, and hindgut deformity. The surgical management and outcome of 10 of 14 survivors (1965 to 1988) are described. Genotypic males (6) were assigned male (2) or female (4) phenotype. Genotypic females (4) were unchanged. All had omphalocele closure in the newborn period. Two had loop stomas. Eight had end stomas (ileostomy [6], ileocolostomy [2]). Toddler and adolescent reconstruction differed in each. Early in the study, abdominoperineal pull-through failed in four patients, necessitating permanent stoma. Four patients had a stoma from the outset. Augmentation using colon remnant improved water loss and nutrition in two infants. Exstrophy turn-in for urinary reservoir was considered in all, but was impossible in three who required urinary diversion. Six patients had exstrophy turn-in and now void by clean intermittent catheterization (4), continent vesicostomy (1), and incontinent (1). Hindgut augmentation improved urinary capacity in two. Two genotypic-phenotypic males had penile lengthening. Four genotypic male-phenotypic females had early orchiectomy with subsequent clitoroplasty or vaginoplasty. Four genotypic-phenotypic females had clitoroplasty or vaginoplasty. Cloacal exstrophy is compatible with a useful life and sound psychologic development, but requires staged reconstruction with long-term support and follow-up.

Abnormalities, Multiple↗
PubMed2003

Intussusception and intestinal malrotation in an infant: a case report.

An abnormal cecum position is usually found in patients with intestinal malrotation. We report one case with intussusception and intestinal malrotation in a 10-month-old infant. An unusual radiologic imaging feature and also abnormal intussusception mass location are discussed.

Digestive System Abnormalities↗
PubMed2003

[Anesthetic management for a radical operation in an infant with cloacal exstrophy].

We report the anesthetic management for a radical operation in an infant with cloacal exstrophy. Diagnosis of cloacal exstrophy with meningocele was made at the 26th week of gestation. Cesarian section was performed under general anesthesia in order to keep the infant in a state of apnea to prevent aerophagia, the so-called "sleeping baby", at the 38th week of gestation. As soon as the infant was born, she was intubated orotracheally and a radical operation (abdominal closure, removal of meningocele, and vesico-intestinal-pelvioplasty) was performed. Some complications occurred during the operation including hypotension, oliguria, hypothermia, hypoproteinemia, hyponatremia and hyperpotassemia. The operation time was 10 hours and 30 minutes. Mechanical ventilation was continued for 4 days in the NICU and the infant was discharged 37 days after the operation. Early prenatal diagnosis and simulation of the operation enabled an effective surgical procedure allowing us to minimize these intraoperative complications.

Abnormalities, Multiple↗
PubMed2005

Rectal prolapse following posterior sagittal anorectoplasty for anorectal malformations.

PURPOSE: Rectal prolapse is a known postoperative problem in children with anorectal malformations. The aims of this study were to determine the incidence of significant rectal prolapse (>5 mm), to objectively quantify its predisposing factors, and to offer recommendations as to its prevention and surgical treatment. METHODS: The authors reviewed their series of 1619 patients with anorectal malformations; 1169 underwent primary posterior sagittal anorectoplasty (PSARP) at their institution between 1980 and 2002, and complete records were available for 833. The series was analyzed for incidence of prolapse, type of anorectal malformation, status of the sacrum, muscle quality, associated vertebral and spinal anomalies, and postoperative constipation. A specific technique for prolapse repair was used. RESULTS: Of 833 patients, 45 developed significant rectal prolapse (3.8%). The mean age at the time of PSARP was 0.73 years (range, 0.19-5 years). The average time to recognition of prolapse following PSARP was 13.1 months. Of these 45 patients, 32 required surgical repair and of those, 3 required a second surgical repair. The incidence of prolapse varied by complexity of anorectal defect: cloaca (6.2%), rectobladder neck fistula (6.8%), rectourethral fistula (5.4%), rectovestibular fistula (1.2%), rectal atresia (0%), and rectoperineal fistula (0%). There was a significantly increased incidence of prolapse in patients with a low muscle quality score and in patients with vertebral anomalies (20% vs 3.2%). The presence of a tethered cord and an abnormal sacral ratio did not correlate with an increased incidence of prolapse. Twenty-two patients developed prolapse following colostomy closure, and of these, 12 (55%) suffered from constipation. CONCLUSIONS: The overall incidence of significant rectal prolapse following PSARP is low. Prevention of prolapse with the PSARP technique may be because of key technical steps. Patients with higher anorectal malformations, poorer muscle quality, and vertebral anomalies had a greater risk of developing postoperative rectal prolapse. The presence of tethered cord and quality of the sacrum were not predictive of postoperative prolapse. Constipation seems to be a factor in the development of prolapse.

Anal Canal↗
PubMed1997

[Intestinal emergencies in newborn infants].

Imaging plays a major role in most neonatal gastrointestinal emergencies. The role may vary from helping to establish a diagnosis, to the evaluation of associated abnormalities, to surgical planning, or to therapy for some conditions like meconium ileus or meconium plug syndrome. Plain radiographs and ultrasound serve a primary imaging modalities with bowel contrast examinations, CT scan, and MR imaging playing roles in more complex cases.

Abdomen, Acute↗
PubMed2004

Hindgut duplication--case report and literature review.

Colonic duplication is a rare congenital abnormality that can present diagnostic difficulties. A case of duplication of the entire large bowel and terminal ileum is presented, and a framework for diagnostic work-up and treatment options in these patients is discussed.

Colon↗
PubMed1982

Follow-up studies of children treated with a long-term intravenous nutrition (IVN) during the neonatal period.

The outcome of the 28 children treated with intravenous nutrition (IVN) for more than 4 wk during the neonatal period was investigated. Ten of the 28 children had a normal length of small bowel (group 1) and the other 18 children had a short bowel (group 2). Eight of these 28 children (4 in group 1 and 4 in group 2) died during or after cessation of IVN; three died from complication of IVN and 5 died from their initial disease. On physical examination, delay in catch-up growth was observed in the early postoperative period, particularly in children in group 2, but this diminished with time. Mental and emotional development showed both groups to be within the normal range, except for two children. Intellectual function was also normal in five children over the age of 4 yr who were examined. Hepatic dysfunction was noticed during IVN in 30.0% in group 1 and 66.7% in group 2, indicating that the longer the duration of IVN, the higher the incidence of liver damage. Furthermore, histologic abnormalities in these neonates lasted for a longer time. These results imply that long-term IVN during the neonatal period in surgical patients does not interfere with physical and developmental growth. However, careful and long-term follow-up of liver function after IVN is necessary.

Child↗
PubMed2006

Anal canal duplication in children: a new technique.

Anal canal duplication (ACD) is a very rare abnormality. Because of the high rate of malignant changes in anal canal duplications complete removal of the ACD is recommended. In the current study, simple mucosectomy for management of cases of ACD has been discussed. There was no complication and patients had good cosmetic results with a normal sphincter control. This technique is simple, safe, takes less time and achieves good anatomic appearance and functional results.

Anal Canal↗
PubMed2006

Anorectal anomaly: a review of 82 cases seen at JUTH, Nigeria.

BACKGROUND: The management of anorectal anomaly remains a challenge to Surgeons. This study was carried out to determine the pattern and outcome of management of anorectal malformations (ARM) in a Nigerian tertiary hospital. PATIENTS AND METHODS: The clinical and operative records of consecutive children with anorectal anomaly managed between October 1990 and September 2000 at the Jos University Teaching Hospital, Jos, were reviewed. RESULTS: There were 82 patients, (57.3%) males and 35 (42.3%) females (m:f=1.3:1). There were 20 (24.4%) cases each of the high and intermediate types, 40 (48.8%) low variety and 2 (2.4%) cases of persistent cloaca. The high type was proportionately commoner in the males. Less than one-third presented within 24 hours. Eighty three percent of patients (mainly males) presented in acute intestinal obstruction. Passage of stools from abnormal sites and "imperforate" anus were complaints in 60 (73.1%) patients each. A total of 63 patients had definitive corrective procedure. Four patients with stenotic anus were treated by serial anal dilatations while 29 with low anomaly had anoplasty during the neonatal period. Twenty eight patients with intermediate or high anomalies or persistent cloaca had definitive repair or pull-through operations carried out 6-12 months after an initial colostomy. The definitive pull-through operations included abdominoperineal pull-through in 11 patients, PSARP in 15, while 2 girls with persistent cloaca had posterior sagittal anorectovaginoure-throplasty (PSARVUP). Twenty nine children were fully continent of stools after surgery; three patients developed occasional faecal soiling; while six patients had faecal incontinence. CONCLUSION: Anorectal anomaly is common, but presentation is late in our environment. Although mortality rate was high (26%), early results of definitive operation among survivors were generally good after a mean follow-up period of 19 months.

Adolescent↗
PubMed2006

Surgical intervention of intestinal malrotations in paediatric patients without other congenital anatomical abnormalities: overview from a single center.

The subject of malrotation in infants and children without other congenital anatomical abnormalities is reviewed from the perspective of experience with 97 patients operated in 11 years. Fifty-five patients were younger than 6 weeks at operation. They often presented with bilious vomiting, in contrast to older children who presented with non-bilious vomiting or feeding problems. Patients younger than 6 weeks were operated more often acutely than older patients. Volvulus was more common in infants younger than 6 weeks. Two patients with a resulting short bowel syndrome died. In 73 of the surviving 95 (76.8%) children their symptoms disappeared. In the children younger than 6 weeks persisting abdominal problems were significantly less frequent than in older children. In the children presenting with proven gastro-esophageal reflux disease before the malrotation operation, abdominal problems persisted significantly more often. Although there remains considerable controversy over how older children without signs of vascular problems should be managed, failure to respond to radiographic evidence of malrotation could be considered malpractice if volvulus was to occur subsequently. For this reason, every patient with a radiological proven malrotation merits diagnostic laparoscopy.

Adolescent↗
PubMed1982

The bacterial flora of neonates with congenital abnormalities of the gastro-intestinal tract.

The development of the bacterial flora of neonates with congenital abnormalities of the gastro-intestinal tract was studied in 31 infants during the first 10 days of life. Specimens were collected from the umbilicus, mouth and gastro-intestinal tract on the pre-operative day, at operation and on post-operative days 1, 2, 3, 5, 7 and 10. Bacteria were isolated semi-quantitatively on a variety of plain and selective media and identified by conventional methods.Staphylococcus albus was the predominant species isolated from the umbilicus; it was recovered from 24 of the 31 babies. The viridans group of streptococci and Streptococcus salivarius were the commonest species isolated from the mouth; there were no differences between the babies with different abnormalities and treatment with antibiotics had no effect on the bacterial flora. Ten babies were colonized by each species on the pre-operative day, and 25 and 19 respectively by the tenth post-operative day. Anaerobic gram-positive cocci were the predominant oral anaerobes. Bacteria were not isolated from the rectal swabs of babies with tracheo-oesophageal fistula (TOF) or small bowel atresia on the pre-operative days. Post-operatively the predominant faecal isolates from babies with TOF were Str. faecalis, Escherichia coli and Clostridium perfringens. About 80% of the babies with small bowel atresia were colonized by Str. faecalis and Bacteroides vulgatus, 60% each by E. coli, Klebsiella aerogenes and Str. faecium. The five babies with necrotizing enterocolitis were colonized by Str. faecalis, E. coli, Cl. perfringens and Cl. difficile; Bacteroides spp. were not recovered from any of them. The commonest facultative species recovered from babies with large bowel obstruction were Str. faecalis and E. coli. B. vulgatus, Cl. perfringens and Bifidobacterium spp. were the commonest anaerobes and anaerobes outnumbered aerobes. No significant isolates were recovered from the wound swabs and none of the babies developed post-surgical sepsis.

Bacteria↗