Foregut duplication cysts: a diagnostic challenge.
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Cysts↗
Biomedical subjects
L Spitz
Publications and source records attributed to L Spitz.
Postoperative elective ventilation in babies with "marked anastomotic tension" after repair of esophageal atresia.
The repair of esophageal atresia, preserving the patient's own esophagus is the surgical procedure of choice. In "long-gap" type or in "tension anastomosis" cases, anastomotic complications were known to be higher than in usual cases. From this report, postoperative elective ventilation for 5 days together with neck flexion position reduced such complications with minimal subsequent complications related to the mechanical ventilation.
Anastomosis, Surgical↗
Delayed surgery for congenital diaphragmatic hernia.
Between January 1987 and December 1990, 67 neonates were treated for congenital diaphragmatic hernia, symptomatic within 6 h of birth. The mortality rate was 33 per cent. Preoperative stabilization was achieved in 47 patients, all of whom survived initial treatment, although two died later. Stabilization could not be achieved in 20 neonates, all of whom died within 3 days of birth, 18 without undergoing operation and two after early repair. Intensive resuscitation with controlled, delayed operation for congenital diaphragmatic hernia gives long-term results similar to those of urgent operative repair. This approach avoids operation in the majority of those who subsequently die.
Female↗
Abdominal inflammatory myofibroblastic tumours in children.
Inflammatory myofibroblastic tumours (inflammatory pseudotumours) occurring at intra-abdominal sites in children have rarely been described. This paper reports three patients with this tumour, two of whom presented with fever, anaemia and an abdominal mass, the third with chronic duodenal obstruction. All had experienced significant weight loss. At operation, each had a large fibrous tumour (7-18 cm in diameter) originating from the transverse mesocolon, small bowel mesentery and duodenum respectively. Intraoperative frozen section histological examination in one patient was misinterpreted as a sarcoma. All the lesions were judged to have been completely excised, but one was ruptured during operation and the patient subsequently developed recurrent tumour nodules. Abdominal inflammatory myofibroblastic tumours are rare. They may be suspected before operation but their clinical, radiological and pathological features may be confused with those of malignancy. Complete excision is necessary to avoid local recurrence.
Child↗
The ontogeny and distribution of neuropeptides in the human fetal and infant esophagus.
The innervation and neuropeptide expression of fetal and infant human esophagus were studied. Esophageal samples (n = 30) from 8 weeks' gestation to 28 months of age were immunostained using antisera to general and specific neuronal antigens, and the results were quantified using computer-assisted image analysis. Nerve protein (protein gene peptide 9.5 and synaptophysin) and glial cell protein (S100) immunoreactivities were present by 8 weeks' gestation in primitive cell bodies and fibers in the outer layers of the esophagus. Immunoreactivity for peptides was first detected in fibers at 11 weeks' gestation in myenteric plexus and at 13 weeks' gestation in muscle. Peptide-immunoreactive cell bodies were not seen until 13-15 weeks. A pattern of immunoreactivity for neuropeptides comparable with that seen in mature neonates and infants was present by 22 weeks of gestational age. The percentage area of protein gene peptide 9.5-immunoreactive and vasoactive intestinal peptide-immunoreactive nerve fibers increased from low levels to 3.68% and 0.27%, respectively, at 13 weeks and peaked at 18 weeks (10.50% and 4.74%). These findings provide a foundation for future research into the contribution of neuropeptides to pediatric esophageal dysmotility.
Aging↗
Anastomotic leakage following surgery for esophageal atresia.
Of 199 neonates undergoing primary or delayed primary repair of esophageal atresia, 34 (17%) developed anastomotic leakage, 7 of which (3.5%) were major anastomotic disruptions. Infants with major leaks developed signs within 5 days and all required early reoperation, necessitating abandonment of the esophagus in 6. The remaining 27 were minor leaks demonstrated by water-soluble contrast studies and were successfully treated nonoperatively. Gastroesophageal reflux was unassociated with this complication but the use of braided silk sutures was associated with a significantly increased risk of anastomotic leakage when compared with polyglycolic acid (relative risk, 3.2) or polypropylene (relative risk, 2.6) sutures. Following anastomotic leakage there was a significantly increased risk (relative risk, 2.04) of subsequent esophageal stricture formation.
Anastomosis, Surgical↗
Gastric transposition for esophageal substitution in children.
Fifty-four gastric transposition procedures have been carried out for esophageal substitution in the 10-year period 1981 through 1990. The indication for esophageal replacement was esophageal atresia in 36 (19 long-gap atresia with distal fistula and 17 isolated atresia), caustic stricture in 9, intractable peptic reflux stricture in 3, 2 achalasia and 1 each of prolonged foreign body impaction, diffuse leiomyoma, congenital esophageal stenosis, and congenital short esophagus. Eight patients had previously undergone an unsuccessful colonic replacement procedure. The age at gastric transposition ranged from 4 months to 16 years. The procedure of choice was posterior mediastinal transposition without thoracotomy in 37 cases. The esophagogastric anastomosis leaked in 7 patients (12.9%), all of which closed spontaneously, whereas 5 patients developed an anastomotic stricture that responded to bouginage. There were 5 deaths (9.2%). Major complications developed postoperatively in 12 patients: 4 required additional gastric drainage procedures, 2 required temporary tracheostomy, 2 developed adhesion obstruction, and 1 each developed paraesophageal hernia, leakage of the jejunal feeding tube, tracheomalacia, and major hemorrhage following resection of a colonic graft. Major but temporary feeding problems were encountered in 12 children. Medium-term results were assessed as excellent in 67%, good in 20%, fair in 6%, and poor in 6% of the 34 patients surviving longer than 1 year postoperatively (ie, excluding 7 patients lost to follow-up).
Adolescent↗
A study of mucosal gut immunity in infants who develop Hirschsprung's-associated enterocolitis.
The aim of this study was twofold. First, to establish quantitatively the distribution of the immunoglobulin-containing (plasma) cells, T and B lymphocytes in the lamina propria of the rectal mucosa of normal neonates and neonates with Hirschsprung's disease (HD). Second, to review the neonates with HD to determine any differences in these cell populations between those who subsequently developed Hirschsprung's enterocolitis (HEC) and those who did not. Two conclusions can be drawn from the results of our study of rectal mucosal immune defenses. First, neonates with HD have no deficiencies in these defenses when compared with normal neonates. Second, neonates with HD who subsequently develop HEC have no premorbid deficiency in these defenses. It was noted that the pan-T cell count in the infants who went on to develop HEC appeared to be increased, although this did not reach statistical significance. The use of fresh or frozen material would permit a more detailed analysis of the separate T cell subsets.
Child, Preschool↗
Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia.
Congenital esophageal stenosis caused by tracheobronchial remnants occurred in eight children, six of whom had associated esophageal atresia and/or tracheoesophageal fistula. Symptoms usually began in early infancy but delayed diagnosis was a common feature. The mean lag period between presentation and definitive operation was 4.6 years (range, 1 month to 16 years). Errors in diagnosis were common. Six were initially diagnosed as having inflammatory strictures secondary to reflux esophagitis. Seven children were subjected to repeated esophagoscopy and bouginage of the "stricture" (mean no. = 3.4), with invariable failure to ameliorate dysphagia. Antireflux procedures were performed in three patients. In all children, symptoms were dramatically relieved following resection of the stenotic segment or esophageal replacement. Although a rare entity, congenital esophageal stenosis due to tracheobronchial remnants should be considered a possibility in patients with esophageal stricture, presumed to be inflammatory in nature, which fails to respond to standard therapy.
Adolescent↗
CHARGE and esophageal atresia.
CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association. All had major cardiac anomalies, predominantly tetralogy of Fallot. The majority of patients had primary repair of the esophagus. The postoperative course was stormy in all patients, with a high incidence of complications and 70% died. The recognition of features of the CHARGE association is important because it has major prognostic and therapeutic implications.
Abnormalities, Multiple↗
Brown bowel syndrome: a late complication of intestinal atresia.
Two children, aged 11 years, who originally had jejunal atresia corrected in the neonatal period, developed massive dilatation of the proximal small intestine. This resulted in circular muscular hypertrophy with lipofuscin deposits giving the typical appearance of "brown bowel." The condition was associated with malnutrition and vitamin E deficiency. Because of relatively short bowel, the condition was treated by limited resection and extensive tapering of the dilated segment, end-to-end reanastomosis, vitamin E supplementation, and intensive nutritional support.
Child↗
The role of lymphatics in the pathogenesis of pneumatosis in experimental bowel ischemia.
Twelve spontaneously delivered, normally suckled, small-for-gestational-age (weighing 756 to 1,213 g) neonatal piglets were used to assess the role of the mesenteric vasculature in the pathogenesis of neonatal necrotizing enterocolitis (NEC) by producing intestinal ischemia. Component vessels (arteries, veins, lymphatics) of the mesenteric vascular arcades were variously occluded by ligation for 48 hours. Nine adjacent vessels of the same type or nine adjacent combinations of vessels were occluded in piglets 12 to 18 hours postpartum. Arterial plus lymphatic ligation induced lesions showing the complete histopathological spectrum of NEC (mucosal stripping, hemorrhage, submucosal disruption and destruction, full-thickness necrosis, inflammatory infiltration) including pneumatosis intestinalis. Two of the lowest birth weight animals produced complete NEC in response to lymphatic ligation alone. A condition consistent with "prepneumatosis" was found when lymphatics only were ligated. The distended lymphatic vessels in the submucosa resembled pneumatosis with reference to shape size and distribution, but contained milk-derived lipids, some proteins and lymph but no gas. Arterial ligation alone induced NEC-like lesions without pneumatosis. Venous ligation alone induced minor congestive/hemorrhagic lesions. Pneumatosis appears to originate in the lymphatic vessels of the submucosa in this experimental model of NEC. Lymphatic occlusion alone can cause complete NEC in very SGA neonatal piglets. Arterial plus lymphatic occlusion produces a unique combination of specific pathology resembling human NEC.
Animals↗
Surgical treatment of hyperinsulinaemic hypoglycaemia in infancy and childhood.
Despite a greater awareness of hyperinsulinaemic hypoglycaemia, one in three patients has some degree of mental retardation by the time the diagnosis is made. The diagnosis is established by demonstrating high plasma insulin concentrations during an episode of hypoglycaemia. Twenty one hyperinsulinaemic infants and children were referred for surgical treatment after failing to respond to medical management. The surgical procedure of choice is a 95% pancreatectomy. Recurrence of the hypoglycaemia may develop after less radical resections as occurred in one patient who then underwent an extended resection 72 hours postoperatively. Patients who fail to respond to optimal medical treatment should be referred for surgery early and not as a last resort if permanent neurological damage is to be avoided.
Child, Preschool↗
Prolapse of the stomach through a gastrostomy site.
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Female↗
Jejunal diverticulum in a child presenting with small bowel obstruction.
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Child, Preschool↗
Quantitative study of the development and maturation of human oesophageal innervation.
By 8 wk gestation, the human fetal oesophagus is identifiable as a hollow epithelium-lined tube with primitive nerve and muscle precursors present. From 8-16 wk gestation, the muscle layers and innervation mature until fetal swallowing commences at 16 wk. This study examines quantitatively the development and maturation of nerve fibres and cell bodies within the oesophagus using histochemistry. Oesophageal samples (n = 35) from 8 wk gestation to 28 months of age and adults (n = 3) were immunostained using antisera for the general nerve marker, protein gene product (PGP 9.5), the glial tissue marker S100, and the synaptic vesicle protein synaptophysin (p38). Histochemical staining for NADH diaphorase enzyme activity was also used to identify neurons. Computer-assisted image analysis of the muscularis externa permitted detailed quantification of cell size, nerve density and myenteric (plexus) fraction. At 8 wk gestation, PGP and synaptophysin were present in immature neurons throughout the cytoplasm, but from 10 wk synaptophysin was localised solely at nerve synapses. S100 immunoreactivity was also detected from 8 wk gestation onwards and was confined to glial tissue. Nerve cell size increased with maturation from 6 microns at 8 wk gestation to 20 microns at term and 21 microns at 28 months. The numbers of cells, nerve density (% area occupied by nerves throughout section) and myenteric fraction (% area occupied by ganglion cells and nerve fibres within the myenteric plexus) all peaked at 16-20 wk gestation and, whereas the number and density then fell towards adult levels, the myenteric fraction fell during the late second trimester and became constant from 30 wk gestation.(ABSTRACT TRUNCATED AT 250 WORDS)
Adult↗
Morbidity and mortality in 46 patients with the VACTERL association.
During a 10-year period, 313 infants were treated at the Hospital for Sick Children for esophageal atresia, of whom 46 had features of the VACTERL association. These 46 patients had a mortality rate of 24%, most deaths being caused by cardiovascular abnormalities. Infants weighing less than 2,050 g had a mortality rate of 26%, double that of heavier infants. During the first year of life there was considerable morbidity resulting from the tracheoesophageal abnormalities. Whenever possible primary repair of the esophagus should be performed; however, many patients with VACTERL association had an unusually long gap between the two ends of the esophagus, and repair under tension was associated with a high incidence of complications. Of the 35 survivors, 25 are in a satisfactory, stable condition, but 10 still require treatment necessitating repeated admission to hospital. Anorectal, renal or skeletal anomalies rarely caused death, but, when severe, were associated with a poor quality of life.
Abnormalities, Multiple↗
Primary anastomosis in apple-peel bowel syndrome.
During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine). In comparison, all four of the enterostomy group were hospitalised for between 70 and 175 days. All twelve patients survived.
Anastomosis, Surgical↗