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.

Fast evaluation of intraoperative biopsies for ganglia in Hirschsprung's disease.

PURPOSE: The most important diagnostic features of Hirschsprung's disease (HD) are the combination of aganglionosis and hypertrophic nerve bundles. Acetylcholinesterase staining is widely used for diagnosis of HD as it identifies hypertrophic nerves in both diagnostic and intraoperative biopsies. The main drawback of this method is in the identification of ganglia. It has been suggested that the combination of this method together with another histochemical marker would be a superior diagnostic tool. Hematoxylin and eosin is still the diagnostic measure of choice for identifying ganglia in many centers, although it presents a persistent diagnostic challenge for pathologists trying to rapidly and accurately interpret the frozen biopsies that guide intraoperative decision making. METHODS: Therefore, we sought to develop a fast, intraoperative immunohistochemical protocol for visualization of ganglia and nerves in HD specimens that can be used in conjunction with these other tools. RESULTS: With the use of acetone fixation and immunofluorescence staining with antibodies to neurofilament 68 and tubulin, ganglia in sections of full thickness and suction biopsies could be visualized in only 10 minutes. This protocol facilitated the identification of ganglia in hematoxylin and eosin-stained adjacent sections and also identified hypertrophic nerve trunks. CONCLUSION: This method should significantly enable the identification of ganglia in suction and full thickness biopsies.

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.

Metastatic renal cell carcinoma in a child: 11-year disease-free survival following surgery.

A child with metastatic renal cell carcinoma (RCC) is presented. This case is unusual in that the patient has remained disease free for 11 years following surgery and only one course of chemotherapy prior to thoracotomy. The management of metastatic RCC is reviewed and the genetic mechanisms leading to its development briefly discussed.

Does laparoscopy adversely affect gas exchange and pulmonary mechanics in the newborn? An experimental study.

Notwithstanding the clinical acceptability of pediatric laparoscopy, the physiological consequences of carbon dioxide (CO(2)) peritoneal insufflation on lung mechanics and gas exchange are not established. This study seeks to establish the affects of increased intraabdominal pressure (P ABD) from CO(2) insufflation on lung mechanics, the compliance of the respiratory system (CRS stat), and gas exchange. Ten anesthetized tracheostomized, and paralyzed rabbits (2.5-3.1 kg) were examined. Mechanical ventilation was established to produce normal ventilation (PaCO(2) 35-45 Torr). Continuous pulse, blood pressure (BP), and serial arterial blood gas measurements were obtained by femoral arteriotomy. The animals were examined at baseline conditions (P ABD = 0 mm Hg), at 2 levels of external compression (P ABD = 6 and 12 mm Hg, respectively), and at two levels of intraperitoneal CO(2) insufflation (P ABD=6 and 12 mm Hg, respectively). Increased P ABD, whether applied externally or by intraperitoneal insufflation, did not alter the CRS stat (ANOVA p = 0.557), nor systematically change the pH, PaCO(2) or PaO(2) (ANOVA p = 0.541, p = 0.545, p = 0.446, respectively). Neither BP nor pulse rate showed any change. Under conditions encountered during laparoscopy, P ABD of 0-12 mm Hg did not have a deleterious effect on ventilation and gas exchange in the ventilated and paralyzed small animal.

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.

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.

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.

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.

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.

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.

Complications of the Grice-Green operation.

A case presentation involving complications resulting from errors in surgical technique with the Grice-Green procedure is discussed in this report. The patient was treated 18 years after bilateral tendo achillis lengthenings and Grice-Green extra-articular subtalar arthrodeses with autogenous tibial bone grafts. The complications encountered were severe adductovarus deformity of the feet with multiple painful plantar callosities, in-toed gait with weightbearing on the lateral aspect of the feet, inversion ankle instability, and tripping over the feet. Deformity and symptoms were worse on the right foot. The patient required further surgery, consisting of bilateral calcaneal osteotomy, metatarsal neck osteotomies, excision of callosities and ostectomies for painful plantar metatarsal base lesions, tenotomy of the abductor hallucis and digital fusions. The original surgery was performed for idiopathic flatfeet. Despite the many different causes of flatfoot for which the Grice-Green procedure has been employed, most authors believe it should not be used for idiopathic flatfeet, and restrict its use to the original indication, that of paralytic pes valgus secondary to poliomyelitis. The reader should also realize that there are very long-term consequences to surgery on the pediatric patient.

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.

Acute scrotal abnormalities in children: diagnosis by combined sonography and scintigraphy.

Both scintigraphy and real-time sonography have been used to assess acute symptoms involving the scrotum. However, because of its high sensitivity and ability to document physiologic abnormalities, scintigraphy has been the procedure of choice. Scintigraphy, however, lacks specificity; its value lies mainly in serving to distinguish torsion from nontorsion. The purpose of this study was to supplement scrotal scintigraphy with sonography to determine if the combination improves diagnosis and management compared with scintigraphy alone. Forty-three scrotal scintigrams and sonograms were performed on 40 consecutive patients with acute scrotal symptoms. The interpretation of the scintigram was altered by sonography in six (14%) of the combined scans, directly affecting clinical management. In three patients with acute hydroceles diagnosed by sonography, exploratory surgery was avoided despite scintigraphic findings suggesting testicular torsion. Scintigraphy was normal in two patients with spontaneous testicular detorsion, whereas sonography showed recent spermatic-cord torsion that required subsequent orchiopexy. In a patient with epididymitis and orchitis, sonography showed a complicating scrotal abscess, which was not apparent on scintigraphy and which required antibiotic treatment. The addition of sonography to the scintigraphic evaluations of children with acute scrotal abnormalities changed the diagnosis and clinical management in 14% of the patients studied.

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.

Removal of esophageal foreign bodies with a Foley balloon catheter under fluoroscopic control.

Between 1982 and 1985 removal of a nonorganic, smooth, radiopaque foreign body in the esophagus with a Foley balloon catheter under fluoroscopic control without sedation was attempted in 38 children. An ultra-low-dose fluoroscopic unit was used. In 35 children the foreign body (a coin) was either easily removed (in 29 cases) or advanced into the stomach (in 6). No complications of the procedure were observed. In three children the foreign body could not be removed by this means; it was subsequently removed by endoscopy (in two cases, both of coins) or esophagotomy (in two cases, both of coins) or esophagotomy (in one, of a stone). When carefully performed, removal of blunt, recently ingested esophageal foreign bodies with a Foley catheter under fluoroscopic control is a safe mode of treatment.

Neuroblastoma: a specific sonographic tissue pattern.

Previous attempts to determine a sonographic appearance characteristic of neuroblastoma have had diverse results. Sonograms of 53 abdominal tumors, including 10 neuroblastomas, imaged during 1982-1986 were reviewed. Four of the patients with neuroblastoma had a distinctive sonographic "lobule" of increased echogenicity in a part of the larger tumor mass. This sonographic appearance was secondary to the growth pattern of the tumor and was not cell specific. Correlative CT scans in two of the four patients did not differentiate this lobule. Histologically, the lobule was an aggregate of uniform neuroblastoma cells (marginated by reticulin and collagen) without hemorrhage, necrosis, or calcification. This tissue pattern was not seen in any of the other 43 neoplasms, including 12 Wilms' tumors. When identified sonographically, the lobule identified in this study seems specific for neuroblastoma and is a valuable diagnostic sign in children with an abdominal mass.

Ultrasonography and the impalpable testis.

The accuracy of inguinal ultrasonography in the pre-operative localization of the maldescended testis was assessed in 51 patients with 57 maldescended testes. Pre-operative sonographic results were compared with the clinical assessment and the operative findings. Testes located in the inguinal canal or at the internal ring, which were occasionally impalpable, were readily demonstrated by sonography. The management of children with an impalpable maldescended testis should include a sonographic examination as the investigative procedure of choice.