David A. Stringer

The late-presenting pediatric bochdalek hernia: A 20-year review

A 20-year retrospective study was made of children with congenital posterolateral (Bochdalek) hernias presenting more than 8 weeks after birth. The records of 26 patients (16 boys and 10 girls) were evaluated. Sixteen infants and children (62%) were originally misdiagnosed clinically and radiologically as having either infective lung changes, congenital lung cysts, or pneumothoraces; inappropriate thoracentesis occurred in four patients misdiagnosed as having a pneumothorax. Five patients had previously normal chest radiographs. The most useful investigation was a plain radiograph following passage of a nasogastric tube. Coexisting abnormalities (in particular, gut malfixation and malrotation) were common. All patients except one were operated on within days of presentation, and as emergencies if symptoms were acute. More than one third of our patients were left with a smaller than normal ipsilateral lung after their diaphragmatic hernia repair, and these lungs must be considered hypoplastic to some degree. Chest tubes made no difference in the lungs eventual expansion. Two deaths occurred as a result of acute cardiorespiratory arrest in previously well children. Therefore, the symptoms, signs, and radiologic findings of patients with diaphragmatic hernias presenting after the neonatal period may be difficult to interpret, and may result in diagnostic delay, misguided therapy, and a potentially fatal outcome.

The late-presenting pediatric morgagni hernia: A benign condition*

Data concerning 15 infants and children with late-presenting (more than 8 weeks) Morgagni hernias over the last 20 years (1966 to 1986) have been reviewed. Ten of the cases were clinically normal on presentation, and the most common symptoms and signs were gastrointestinal and respiratory. Only one child presented with acute symptoms. Five had previously normal chest x-rays, and two others had an incorrect initial radiologic assessment. Chest x-ray was the most common diagnostic test; preoperative barium studies were performed in three patients. Twelve patients had other major congenital abnormalities. Fourteen of the 15 had surgery, usually within days of presentation. At operation, 10 of the 14 hernias contained a hollow viscus, nine had a sac, and four had abnormal bowel fixation. Postoperatively, two children had radiologic evidence of impaired diaphragmatic motility. There was no mortality in this series. Overall, late-presenting Morgagni hernias are relatively benign.

Recurrent tracheoesophageal fistulas seventeen-year review

Twenty-three pediatric patients had recurrent tracheoesophageal fistulas during the 17-year period from 1965 to 1982 after 250 repairs of esophageal atresia and TEF. There were 13 males and 10 females. Four babies had their newborn repair at other hospitals. Sixteen atresias had end-to-end repairs (none under tension) while there were seven Duhamel repairs (six under tension). There were 9 postoperative leaks and 10 strictures; 8 babies had neither. The recurrent fistulas were diagnosed from 7 days to 9 years after newborn operation and more than 50% within two months. All but three had some coughing, choking, gagging, cyanosis, apnes, dying spells, wheezing, and recurrent chest infection. Each fistula was diagnosed by barium swallow, although in four patients as many as six x-rays were needed before it was identified. Not one bronchoscopy in seven infants was diagnostic. All fistulas but one were repaired. No fistula closed spontaneously. They were repaired between 1 day and 14 months after diagnosis, most within 7 months. Recovery in the majority of cases was uneventful. However, 5 of the 22 repairs had a second recurrent TEF presenting in similar fashion between 2 weeks and 2 years; all but one within 11 months. All were repaired within 9 months of discovery. The DuHamel anastomosis was more prone to recurrent fistulization than the more common end-to-end type. More of our own patients had their original anastomosis done without any tension. Under these circumstances the esophageal anastomosis is in close proximity to the tracheal closure and a leak or local abscess could easily result in a reconnection between the esophagus and trachea. Any TEF baby who has signs and symptoms related to feeding and/or chest infections must be considered to have a recurrent TEF and a persistent and diligent search should be made for it.

Abnormal carotid arteries in the velocardiofacial syndrome: a report of three cases.

Internal carotid arteries of unusual size and tortuosity were found before or at the time of pharyngeal flap surgery in three children who had the velocardiofacial syndrome with velopharyngeal insufficiency. In two cases, medial displacement of the arteries prevented surgery, and in the other, hypernasality persisted because only a narrow, asymmetrical flap could be raised. Medial displacement of the internal carotid arteries inhibits surgical treatment of velopharyngeal insufficiency, necessitating treatment with a prosthetic speech device in such children. Since displacement and tortuosity may be associated Findings in the velocardiofacial syndrome, the exact location of the internal carotids should be ascertained when pharyngeal flap surgery is planned.

Intussusception: Barium or air?

At The Hospital For Sick Children, the use of air has recently replaced the use of barium in the reduction of intussusceptions. The purpose of this study was to review the results from 200 consecutive patients with intussusceptions, 100 patients treated with barium enema and 100 patients treated with air enema. The groups were similar with regard to sex, average and median ages, and presenting symptoms and signs. Successful reduction was achieved in 75% of episodes of intussusception treated with barium enema and 76% treated with air enema. Failure of either modality showed a high association with the presence of either a lead point or an ileoileal or ileoileocolic intussusception. Among those cases of unsuccessful reduction, operation was performed in all 59 cases; resection in 30 cases, manual reduction in 19, and spontaneous reduction was found in 10. There were three perforations during attempted reduction with barium and two with air. All perforations were treated by resection and primary anastomosis. There were 18 recurrent intussusceptions following barium enema reduction and nine following air enema reduction. Therefore, with the lower absorption of x-rays by air and the relatively inert nature of air (compared with barium in the event of a perforation), we feel that air enema is the treatment of choice in the initial management of intussusception.

TREATMENT OF DISTAL INTESTINAL OBSTRUCTION SYNDROME IN CYSTIC FIBROSIS WITH A BALANCED INTESTINAL LAVAGE SOLUTION

Conventional treatment of distal intestinal obstruction syndrome (DIOS) with high doses of pancreatic enzymes, mucolytic agents, and enemas is neither predictably effective nor rapid in action. In 6 cystic fibrosis patients with DIOS a balanced, non-absorbable intestinal lavage solution produced clinical and radiological improvement and striking improvement in DIOS scores. It is suggested that a balanced intestinal lavage solution should be considered as an alternative treatment for DIOS in patients with cystic fibrosis.

Effects of cisapride in patients with cystic fibrosis and distal intestinal obstruction syndrome

In a double-blind, placebo-controlled, crossover trial, we investigated the effects of the prokinetic drug cisapride in patients with cystic fibrosis and chronic recurrent distal intestinal obstruction syndrome (DIOS). After a baseline period, 17 patients (12.9 to 34.9 years; 12 boys) received, in random order, cisapride (7.5 to 10 mg) and placebo three times daily by mouth, each for 6 months. Gastrointestinal symptoms (flatulence, abdominal pain, fullness, abdominal distension, nausea, anorexia, heartburn, diarrhea, vomiting and regurgitation) were scored three times monthly and physical examinations assessed. At baseline and at each 6-month period, assessment included food intake for 7 days, 3-day stool collection, pulmonary function tests, and abdominal radiographs. During cisapride therapy compared with placebo, there were significant reductions in flatulence (p less than 0.005), fullness, and nausea (p less than 0.05). Patients with the worst symptom scores benefited most from cisapride. With cisapride, 12 patients felt better and three worse (p less than 0.05); physicians judged 11 patients improved and two worse (p less than 0.05). No side effects were noted. There were no significant differences between cisapride and placebo periods in nutritional status, x-ray scores, pulmonary function, food intake (fat, protein, calories), stool size and consistency, and fecal losses of fat, bile acids, chymotrypsin, and calories. For acute episodes of DIOS, intestinal lavage was needed 6 times in 4 patients during treatment with cisapride, and 11 times in 6 patients receiving placebo. In comparison with unselected patients with cystic fibrosis and pancreatic insufficiency who were receiving enzyme supplements and who had no distal intestinal obstruction, fecal fat losses (percentage of intake) were almost twice as high in the study group with DIOS (31.2 +/- 20.6% vs 16.2 +/- 17.6%; p less than 0.01). We conclude that in the dosage used, long-term treatment with cisapride appears to improve chronic abdominal symptoms in patients with cystic fibrosis and DIOS, but fails to abolish the need for intestinal lavage. Cisapride treatment had no effect on digestion and nutritional status of cystic fibrosis patients with pancreatic insufficiency.

Velopharyngeal insufficiency due to hypertrophic tonsils: a report of two cases

Two children in whom idiopathic hypernasality was diagnosed were referred for investigation of velopharyngeal function. Multiview videofluoroscopic assessments showed the tonsils prolapsing posteriorly during speech, preventing the palate from fully approximating the posterior pharyngeal wall. Tonsillectomy was recommended. Postoperative evaluations found that the hypernasal resonance was eliminated in both children.

Hydrostatic reduction of intussusceptions caused by lead points

It is generally accepted that an intussusception caused by a lead point will not be reduced by hydrostatic barium enema. This was reported several years ago, and has continued to be a consistent finding, prompting us to attempt hydrostatic barium enema reductions of recurrent intussusceptions in infants and children and also in older children with a first intussusception. However, in the last 9 years we have treated five children whose ileocolic intussusceptions were caused by lead points but which were reduced by hydrostatic barium enema. The histories and physical examinations were not any different than those of the average pediatric patient with an intussusception. The ileocolic intussusceptions diagnosed by barium enema were reduced with adequate reflux of barium into the terminal ileum. However, a residual and persistent filling defect in the colon or ileocecal area made laparotomy mandatory. In all five cases, a lead point was found and resected. This experience has suggested to us that a residual intraluminal filling defect in the barium column following what appears to be adequate flooding of the terminal ileum should be interpreted as a lead point, and an indication for a laparotomy.

Neonatal intestinal pseudoobstruction

In the past 10 years, the diagnosis of pseudoobstruction lasting more than two months was confirmed in 10 infants after sepsis, meconium ileus, and Hirschsprungs disease were excluded, and surgery or autopsy failed to demonstrate a site of mechanical intestinal obstruction. Four infants had undergone prior operation for another anomaly: gastroschisis (2) and ileal atresia (2). Five of the remaining infants also had megacystis. The lack of coordinated intestinal motility was best appreciated by radiocontrast small bowel studies, which showed degrees of aperistalsis or segmentation. Rectal manometric studies were not helpful. Histology of the intestine was normal in seven, while a gross deficiency of nerve fibres was noted in one patient and a myopathy of smooth muscle in another. A variety of drugs used to stimulate peristalsis were ineffective. Seven patients had 25 operations, often to exclude mechanical causes of obstruction. The mainstay of treatment was TPN and intestinal decompression. Six children survived; their ages ranged between 8 months and 9 years (median age, 16 months). There has been improvement in intestinal peristalsis in five children, three of whom now tolerate a regular diet and two of whom are on TPN and are currently increasing oral intake. Four infants died, two from sepsis, two from TPN-related hepatic failure. In contrast to previous reports, we conclude that intestinal pseudoobstruction may be self-limited in some neonates, including those with megacystis. Therapy should consist of long-term nutritional support and treatment of other anomalies that may be present.