Large paraesophageal hernias in children. Early experience with laparoscopic repair.

BACKGROUND: Large paraesophageal hernias (LPEH) in children are unusual. The aim of this study is to report the management of LPEH and our initial experience with the laparoscopic approach. METHODS: Since September 2005, four children aged 4-17 years underwent laparoscopic repair of LPEH at the Children's Hospital of Eastern Ontario. Three children had previous fundoplications and two of these had a gastrostomy. Closure of the hiatal defect included crural sutures and prosthetic material, either polytetrafluoroethylene-polypropylene mesh or porcine small intestinal submucosal patch. The charts were retrospectively analyzed. RESULTS: The operative time was 300-540 minutes, with one conversion and two mediastinal pleural tears. Postoperative chest X-ray showed neither mediastinal nor intrapleural air. The median hospital stay was 3 days. Contrast X-ray showed no recurrent hernia and an intact fundoplication. CONCLUSION: LPEH in children is usually a complication of previous fundoplication. Laparoscopic repair is technically demanding but feasible. Adequate crural repair using mesh may reduce the incidence of recurrence. In view of the rarity of LPEH in children, a combined multicenter study is needed to evaluate the results of laparoscopic repair.

Beware of stapled side-to-side bowel anastomoses in small children.

Side-to-side, functional end-to-end stapled anastomosis (SS-EESA) is a frequently employed technique to re-establish continuity following bowel resection. We describe, for the first time in children, two cases of an important complication of this form of bowel anastomosis. Patient 1 had resection of a jejunal lymphangioma and formation of an SS-EESA at the age of 3 years. By the age of 7 years he was demonstrating symptoms consistent with malabsorption, which was confirmed by hydrogen breath testing. An upper GI contrast study indicated a segmental dilatation of the distal small bowel. Elective laparotomy revealed partial volvulus of a greatly dilated SS-EESA. Patient 2 had undergone bowel resection as a neonate for ileal atresia, with end-to-end anastomosis. An anastomotic stricture developed at two months of age that was resected with formation of an SS-EESA. Multiple ensuing episodes of partial small bowel obstruction were managed non-operatively until, at 5 years of age, she presented with complete bowel obstruction. At operation, volvulus of a hugely dilated SS-EESA was found. Intraoperative cultures of the succus entericus were consistent with bacterial overgrowth. Both patients were successfully treated with resection of the SS-EESA and primary anastomosis. SS-EESA can be complicated by bacterial overgrowth, massive dilatation and volvulus. In patients with SS-EESA who present with recurrent obstructive symptoms, this complication should be considered.

A fluorochromic stain for nucleic acids to demonstrate submucosal and myenteric neurons in Hirschsprung's disease.

Acridine orange, a rapid and technically simple fluorochromic stain of nucleic acids, was applied to sections of 18 specimens of resected distal colon in infants with Hirschsprung's disease. Neurons were readily identified in the submucosal and myenteric plexuses of normal ganglionic segments because of their intense orange cytoplasmic fluorescence of ribonucleic acid (RNA) that contrasted with the pale green of the smooth muscle and connective tissue. This technic, which may be applied to both frozen and paraffin-embedded sections, is a useful adjunct in the confirmation of aganglionic segments of bowel wall.

Ureterocele--a familial congenital anomaly.

Nonidentical twins with ectopic ureteroceles, and duplication of the urinary collecting system in two more members of the same family are described. This incidence may indicate that inborn anomalies of the urinary tract may have a genetic background.

Mitochondrial myopathy of cerebro-hepato-renal (Zellweger) syndrome.

The muscles of four infants with cerebro-hepato-renal (Zellweger) syndrome were studied during life and/or at necropsy. A mitochondrial myopathy was demonstrated, similar to mitochondrial alterations demonstrated in liver and brain in this disease. Muscle fibers with red-staining subsarcolemmal aggregates were identified with Gomori trichrome stain in two cases. Subsarcolemmal and intermyofibrillar zones of increased concentrations of NADH-TR, SDH, and cytochrome-c-oxidase activity were demonstrated histochemically in all four cases. Degenerative and cytoarchitectural changes in muscle fibers were not found. Ultrastructural studies showed large aggregates of mitochondria and increased lipid in the subsarcolemmal and intermyofibrillar spaces. Degenerative changes in mitochondria and lipid also were demonstrated, but paracrystalline inclusions were not seen. The distribution of these changes was not uniform between patients or between different muscles in the same patient. The diaphragm was affected more severely than proximal or distal muscles of the extremities. Direct involvement of muscle mitochondria in this disease may interfere with energy metabolism and contribute to the clinical findings of hypotonia, weakness, and respiratory insufficiency. The muscle biopsy with histochemistry and electron microscopy may be used as a diagnostic adjunct in suspected cases, but the variation encountered dictates dictates caution in the interpretation of negative findings.

Gastroschisis: primary closure or Silon pouch.

There has been a substantial increase in the incidence of gastroschisis in the last few years, and total parenteral nutrition has enabled many of these neonates to survive the long post-operative course. From 1969 to 1976 inclusive, 44 neonates with gastroschisis were treated with either primary closure or the application of a Silon pouch. Fifteen neonates were managed by primary closure. Three of these neonates developed clinical sepsis, but no other intraabdominal complications ensued. There were two deaths (13%) in the early postoperative period, due to CNS hemorrhage and aspiration. This gorup of 15 babies had 17 abdominal operations and the survivors averaged 32 days of parenteral intravenous nutrition before complete oral alimentation could be successfully started. Two ventral hernias required late repair. Twenty-nine neonates were managed primarily with a Silon pouch. Eighteen of these newborns developed sepsis, three Silon sacs had to be removed because of necrotic bowel within, and two other infants developed small bowel fistulae. There were 12 deaths (35%). These 12 infants had prolonged intestinal malfunction that lasted an average of 67 days. This group of 29 babies had 64 abdominal operations and the survivors averaged 46 days of parenteral intravenous nutrition prior to full oral feeds. Four ventral hernias required later repair. The comparison of results in these two groups of newborns reveals a striking difference in their post-operative course and survival. Although the Silon sac has until now become the accepted surgical procedure for gastroschisis, these results conclusively indicate the advantage of primary reduction and closure of the abdominal wall defect.

Pleomorphic adenoma arising in a salivary rest in childhood.

Two children with pleomorphic adenomas developing in heterotopic salivary tissue are presented. These are unusual lesions, rarely seen in adults and not previously described in the pediatric age group.

The unusual presentation of pancreatitis in infancy.

Two infants with acute pancreatitis are described. It appears that acute pancreatitis in infancy presents in one of two ways: part of a severe generalized illness, or as ascites. The outlook for the former depends on the prognosis of the generalized illness. If pancreatitis can be recognized as the cause of the ascites, then adequate treatment will result in survival. Although the cause in some infants is said to be idiopathic, exploration should be performed, the lesser sac investigated and if the child is in reasonable condition, appropriate study of the biliary tree and pancreatic ductile system should be considered. If a pseudocyst is present, adequate drainage is mandatory. Early diagnosis and appropriate treatment will be possible only if this rare disorder is suspected. The eventual prognosis is excellent and the chance of recurrent attacks is minimal.

Occurrence of contralateral inguinal hernia following unilateral repair in a pediatric hospital.

A review of 904 unilateral hernia repairs was conducted at the Children's Hospital of Eastern Ontario over the period of January 1985 to December 1987. Repairs were done in 758 boys and 89 girls. Repairs of 57 contralateral inguinal hernias were completed following prior unilateral repair (52 boys, 5 girls). Occurrence of contralateral hernias was 5.6% (5.7% for boys, 4.7% for girls). The relative risk in boys with right hernia following left hernia was 8.5%, whereas left hernia following right hernia was 4.3%. Neither sex nor laterality should be considered as an indicator for contralateral exploration in children with a clinical diagnosis of unilateral hernia repair.

Experience with 55 Silon pouches.

The application of a Silon pouch usually indicates a desperate if not impossible situation in which the abdominal contents cannot be returned to the abdominal cavity and therefore require some type of covering. Since 1968, we have had experience with 55 Silon pouches in infants and children. Almost all of these pouches were applied to newborns. There were 22 deaths in this series (40%), although 12 of these deaths were in the diaphragmatic hernia group and were almost all due to respiratory failure unrelated to the pouch itself. There were nine deaths in the gastroschisis and omphalocele group (28%) and in all these instances infection (especially Candida) was the underlying cause. While much debate exists as to how the Silon pouch should be handled after its application our series demonstrated that the longer it stayed in place the greater the chance of infection. Furthermore if broad spectrum antibiotics were used for a prolonged period of time, Candida overgrowth of the pouch followed by Candida sepsis and death were almost inevitable.

Ultrasonography in the management of possible appendicitis in childhood.

One hundred thirty-four children referred to the pediatric surgical service with the diagnosis of possible acute appendicitis underwent abdominal ultrasonography within 24 hours of admission. A final diagnosis of appendicitis was made in 45, and of gynecological disease in 11 children. One child had pancreatitis, another jejunal perforation, and a third gross mesenteric lymphadenopathy. In 75 children the clinical picture completely resolved without a definitive diagnosis being made. Clinical diagnosis of gynecological disease showed two false-negatives, and three false-positives, whereas the ultrasonographic diagnosis was accurate in all patients. The sensitivity of the pediatric surgical diagnosis at the time of admission for acute appendicitis was 49% (23 false-negatives) and the specificity was 95% (three false-positives). Ultrasonographic diagnosis of appendicitis had a sensitivity of 89% (five false-negatives) and a specificity of 92% (five false-positives). There was a negative laparotomy rate of 0.7% (one patient) using both clinical evaluation and ultrasonography. These data suggest that abdominal ultrasonography in the child with possible appendicitis is an important diagnostic adjunct.

The simultaneous occurrence of paraduodenal and paracecal herniae presenting with volvulus of the intervening bowel.

A very rare coincidence of paraduodenal and paracecal hernia with volvulus of the intervening small intestine is reported. The unusual anatomical set up predising to volvulus of a large loop of small bowel has been described. Restoration of normal anatomy was made possible by the application of principles of treatment of internal herniae.

Diffuse necrotizing tracheobronchitis: an acute and chronic disease.

Necrotizing tracheobronchitis (NTB) is characterized by acute episodes of airway obstruction, hypercarbia, and lack of chest movement in mechanically ventilated neonates. Emergency bronchoscopic removal of necrotic tissue is essential for survival. Although postmortem lesions extend into smaller bronchi, survivors have not demonstrated residual tracheobronchial abnormalities. Two infants were treated successfully for NTB but succumbed to diffuse tracheobronchial strictures with progressive pulmonary hyperinflation. A third neonate with esophageal atresia and left pulmonary agenesis developed NTB. Despite initial postbronchoscopic improvement, the infant died at age 6 weeks with diffuse obstructing NTB. All three infants required endotracheal intubation and mechanical ventilation. High-frequency jet ventilation was not used. Tracheal cultures for fungi, bacteria and viruses were negative. Successful treatment of NTB may be followed acutely by recurrence of NTB and chronically by diffuse tracheobronchial strictures and emphysema.

Routine use of the nitric oxide stain in the differential diagnosis of Hirschsprung's disease.

Hirschsprung's disease (HD) is defined as a congenital absence of ganglion cells in the distal bowel. Functionally, there is a loss of enteric neuromuscular inhibition. Inhibitory intestinal innervation includes extrinsic nonadrenergic, noncholinergic (NANC) nerves. Nitric oxide (NO) is proposed to be a NANC neurotransmitter. Sites of NO synthesis can be localized using a NO-dependent nicotinamide adenine dinucleotide phosphate (NADPH) diaphorase histochemical assay. We present a study of the distribution of NO neural elements in patients with HD. Routine hematoxylin-eosin (HE) histology as well as histochemical localization of NO synthase activity was carried out on fixed laminae and sectioned tissue of infant colon. NO synthase positive nerve cells and fibers were found throughout the wall of the proximal ganglionated colon. In the myenteric plexus disposition of these nerves parallels the known NANC innervation. "Aganglionic" distal colon displayed disrupted ganglia and increased nerve fibers. Selective preservation of NO synthesizing neurons was also seen. Punctate labeling of an apparent nonneuronal origin was also noted on the surface of arterioles. NO stain simplifies the pathological diagnosis of HD. The presence of NO positive nerve cells in HD suggests that aganglionosis is a misnomer. The lack of characteristic HE findings in other forms of neuronal intestinal dysplasia indicates the need for routine simple, more sensitive neural staining of colonic biopsies in selected infants with constipation.

Use of human dura in pediatric chest wall reconstruction after tumor resection.

The most difficult aspect of the surgical treatment of chest wall tumors is reconstruction of the large residual defect. Materials that have been used include Marlex, Goretex, Vicryl, bone, metal, and fascia. The authors' successful experience with dehydrated human dura (Tutoplast) for moderate-size defects is described. A large Askin's tumor in a 13-year-old boy required resection of the right posterior aspect of the 9th to 11th ribs and the transverse process of T-10, the 12- x 12-cm thoracic defect was closed with dura. Partial soft-tissue coverage was obtained with the latissimus dorsi muscle. Although a scoliosis secondary to paraspinal muscle resection has developed, the chest wall is stable, without evidence of a flail chest, at 18 months of follow-up. A 6-year-old girl underwent left anterior chest wall resection of three ribs for an epithelioid sarcoma. Human dura and a myocutaneous flap were used for reconstruction, with good functional and cosmetic results at 16 months of follow-up. Dura is simple to use, has low antigenicity, and in experimental studies appears to be incorporated into the tissues, acting as a collagen matrix. For moderate-size chest wall defects, it appears to be an excellent alternative to synthetic prosthesis.

Combined posterior sagittal and three-flap anoplasty in the repair of anorectal anomalies.

PURPOSE: The aim of this study was to describe a new technique for the repair of high and intermediate imperforate anus. METHODS: From 1989 to 1999, 22 children with high and intermediate imperforate anus (17 boys, 5 girls) were operated on with a combination of a posterior sagittal and 3-flap perineal anoplasty. Long-term clinical follow up (to a maximum of 10 years) was done in all patients as well as a recent phone interview with a questionnaire regarding bowel function and degree of satisfaction with the result of the surgical correction. A continence ratio (CR, patient score/maximum possible score) was obtained by a blinded interviewer. RESULTS: A continence survey was obtained in 19 patients. The average CR was 0.68. The CR for high anomalies was 0.62 and for intermediate anomalies was 0.78 (0.84 for girls and 0.64 for boys). Patients with sacral anomalies had a CR of 0.58. Two patients with Trisomy 21 had associated Hirschsprung's disease and were excluded from analysis. CONCLUSIONS: Advantages of this combined surgical approach are excellent anatomic exposure, the ability to limit rectal mobilization to a minimum, reduction of the incidence of mucosal prolapse, the new skin-lined anal canal may assist attainment of continence by providing a "sensory warning zone," and, finally, the cosmetic appearance is satisfactory.

Use of clindamycin and gentamicin in pediatric colonic surgery.

The combination of clindamycin and gentamicin was used to treat 25 pediatric surgical patients. In 9 of the 11 patients with perforated appendicitis, B. fragilis was isolated from the peritoneal cavity. There was a rapid clinical response to treatment in all cases. No side effects were observed. Clindamycin and gentamicin seem to be the antibiotics of choice for such infections.

Esophageal function following Livaditis repair of long gap esophageal atresia.

Proximal pouch esophagomyotomy (Livaditis) allows for repair of long gap esophageal atresia (EA). Postoperative esophageal functional studies in these patients are lacking. Six such infants were followed for up to 42 months. Esophageal function was assessed clinically and by barium swallow, manometry, 24 hr pH monitoring, esophagoscopy, and biopsy. Operative complications included two minor anastomotic leaks and two asymptomatic diverticula at the myotomy site. All patients had dysmotility on barium swallow. Gastroesophageal reflux (GER) was seen in four. Manometry showed a variable aperistaltic segment in each infant but lower esophageal sphincter pressures and relaxation were retained. Twenty-four hour pH monitoring showed an increase in frequency and duration of GER. All four patients biopsied had esophagitis. Five of the six patients showed normal growth velocity. Livaditis modified repair of EA was not associated with significant surgical complications. Esophageal motility showed abnormalities similar to those reported after the standard repair of EA. Myotomy did not adversely affect the esophageal function.

Fetal tumors associated with hydrops: the role of the pediatric surgeon.

Fetal tumors are being diagnosed with increasing frequency and great accuracy by antenatal ultrasound. High-output cardiac failure and hydrops indicate fetal distress. Management may be limited by the gestational age of the fetus. Our experience with three fetal tumors demonstrates the dilemma with respect to timing of delivery and prognosis. Following the diagnosis of a large sacrococcygeal tumor, a 22-week-gestation fetus became hydropic and died. Another fetus with a rapidly growing posterolateral chest wall mass required cesarean section delivery at 29 weeks gestation. Postnatal course was complicated by pulmonary hypoplasia, intratumoral hemorrhage, and death. The third fetus had an enlarging tumor in the right lobe of the liver. Poor biophysical profile and mild hydrops necessitated cesarean section delivery at 34 weeks. Right hepatic lobectomy was performed and the infant was subsequently discharged home at one month of age. The deleterious effects of the fetal tumor and the need for its removal have to be carefully weighed against the ability of the fetus to survive postnatally.