Simplified access for division of the low cervical/high thoracic H-type tracheoesophageal fistula.

H-type tracheoesophageal fistulas (H-TEF) often are located in the low cervical/high thoracic region where determination of the most appropriate surgical approach is difficult. When it can provide adequate exposure, a cervical incision is preferred because of the likelihood of decreased morbidity. A child with VACTERL association presented with recurrent respiratory problems. Esophagogram showed an H-TEF below the level of the clavicle. A vascular guide wire was placed through the H-TEF with the ends brought out through the mouth. Under fluoroscopic guidance, gentle traction was placed on the wire to bring the fistula into the neck for an easily accessible cervical exposure, thus eliminating the need for a thoracotomy.

Treatment of pseudotumors with nonsteroidal antiinflammatory drugs.

Large inflammatory pseudotumors (IPT) traditionally are managed with extensive surgical resection. This approach, which often is associated with significant morbidity, has been deemed necessary because of the uncertainty of diagnosis, symptomatology, and involvement of vital structures. Also, there is a lack of other reliable therapy for this clinically aggressive yet histologically benign disease characterized by an overreactive inflammatory response. The authors treated 2 cases of abdominal IPT with nonsteroidal antiinflammatory drug (NSAID) with successful results. After a diagnosis of IPT on tumor biopsy, an NSAID trial can confirm the diagnosis and treat the disease by causing tumor shrinkage and eventual resolution. Excision remains indicated in easily resectable tumors and in nonresponders to NSAID therapy.

Renal artery stenosis and pheochromocytoma: coexistence and treatment.

PURPOSE: The aim of this study was to develop an optimal treatment plan for coexisting pheochromocytoma and renal artery stenosis. METHODS: A retrospective analysis of the evaluation and management of a hypertensive 14-year-old boy was conducted. RESULTS: Secondary causes of hypertension were excluded initially including intracranial, cardiac, and endocrine abnormalities. A renal scan showed right renal function at 39%. Angiography confirmed a 90% subsegmental stenosis, as well as noting a blush suggesting a tumor. A computerized tomography (CT) scan of the abdomen showed a right adrenal mass. Serum epinephrine was 3,006 pg/mL (normal, <1,009 pg/mL) and 24-hour urinary norepinephrine was 2,001 microg (normal, <105 microg/24 h), suggesting the presence of a pheochromocytoma. During the operation for resection, a right subsegmental renal artery was found to be draped around a renal hilar mass; the adrenal gland was normal. The tumor was resected, and pathology confirmed a pheochromocytoma. Postoperatively, the patient had some mild hypertension that gradually resolved. CONCLUSIONS: Extraadrenal pheochromocytomas (paragangliomas) occur more frequently in children and are most commonly located in the renal hilum. In this location, they may present initially as renal artery stenosis as a result of direct arterial compression, fibrous bands, or catecholamine-induced vasospasm. Our experience supports tumor resection of extraadrenal pheochromocytomas as the definitive treatment for both conditions.

Alternative method for repair of the difficult infant hernia.

BACKGROUND: Inguinal hernia repair in the small infant is often technically difficult. An alternative operative approach has been developed that can simplify troublesome repairs, while decreasing the potential risks of damage to cord structures and of recurrence. METHODS: Thirteen small infants (weight 1400-3000 grams) underwent inguinal hernia repair using a technique of direct closure of the internal inguinal ring via a trans-hernial sac approach. Dissection of cord structures from the sac was avoided. RESULTS: All hernia repairs remained intact on follow-up of 4-28 months. One patient early in the series developed a noncommunicating hydrocele, prompting the addition of sac eversion to the original technique. CONCLUSIONS: An alternative method for simplified repair of difficult infant hernias has been used with success. While it does not supplant traditional repair technique for most patients, it should be considered for use in selected situations.

A novel technique for correction of intestinal atresia at the ligament of Treitz.

PURPOSE: After reconstruction of jejunal atresias at the ligament of Treitz, many patients do not respond to simple tapering and anastomosis requiring repetitive operations because of dysfunction of dilated proximal bowel. A new operative approach using lateral duodenectomy and duodenojejunostomy (LDAD) is reported. METHODS: Three infants with atresias within 10 cm of the ligament of Treitz were treated with LDAD, and their records are reviewed retrospectively. The entire duodenum is visualized after creating a malrotation; this is followed by opening the dilated duodenum and resecting dilated proximal jejunum. The resection is extended proximally, incorporating the lateral duodenal incision, excising the lateral duodenal wall, and preserving the ampulla. The residual duodenum is fashioned into a tube and anastomosed to the spatulated distal jejunum. RESULTS: Three infants underwent this procedure over a 4-year period. Two had undergone tapering enteroplasties previously but were unable to tolerate oral feedings; 1 infant had LDAD primarily. All were ultimately successfully managed by LDAD and were feeding within 14 days. Follow-up is from 14 to 49 months. CONCLUSION: Although experience is limited to 3 patients, the prompt return of intestinal function with LDAD may justify primary use of this more radical procedure in difficult-to-treat proximal atresias.

Reoperation after esophageal replacement in childhood.

BACKGROUND: Esophageal replacement is associated with significant morbidity that may lead to operative interventions. This study reviews the management and outcome of children who underwent reoperation after esophageal replacement. METHODS: Eighteen patients who underwent esophageal replacement from 1985 to 1997 were reviewed retrospectively. Ten patients underwent reoperation. Patient management, perioperative morbidity, and the dietary intake at follow-up were recorded for each patient. RESULTS: Of the reoperated patients, 7 had esophageal atresia, 2 had caustic ingestion, and 1 had achalasia. Nine patients received a colon interposition, and 1 received a reverse gastric tube as the initial esophageal replacement. Seven patients required revision of the anastomoses. Three patients required complex esophageal reconstruction: 1 underwent gastric transposition, 1 underwent free jejunal graft, and 1 underwent gastric transposition combined with free jejunal graft. Seven patients were eating well at follow-up. Two patients still required partial gastrostomy tube feeding. One patient died 6 months postoperatively from aspiration pneumonia. CONCLUSIONS: Esophageal replacement continues to be a challenging operation associated with significant complications. Most reoperative procedures were directed toward strictures and persistent fistulae. Complete graft failure can be managed by gastric transposition or free jejunal graft. Despite the perioperative morbidity, most patients have good functional outcome.

Pulmonary sequestration presenting as mitral valve insufficiency.

Although pulmonary sequestrations commonly present with infectious complications, problems relating to high blood flow through the lesion are rarely apparent. A 4-year-old girl was referred for cardiac catheterization and evaluation for mitral valve surgery. An echocardiogram had demonstrated left atrial and ventricular enlargement and significant mitral regurgitation with an enlarged valve annulus. Angiography results showed a very large aorta to left atrial shunt through an unsuspected intralobar sequestration. Lobectomy with removal of the sequestration resulted in significant improvement in cardiac chamber size and function over a 2.5-year follow-up period, thus obviating the need for cardiac surgery and removing a potential source of infection. Careful evaluation of chest imaging studies will lead to the correct diagnosis and treatment in patients with pulmonary sequestration who are thought initially to have primary cardiac disease.

Long-term follow-up of patients treated with ileoendorectal pull-through and right colon onlay patch for total colonic aganglionosis.

BACKGROUND/PURPOSE: This study was performed to assess the long-term follow-up of five patients who underwent one-stage ileoendorectal pull-through with right colon onlay patch for total colonic aganglionosis (TCA) at Kaiser Permanente Medical Center. METHODS: A retrospective review of inpatient and outpatient charts and telephone follow-up of all patients were conducted to obtain current data regarding growth, development, bowel function, and postoperative and late complications. RESULTS: Follow-up has ranged from 2 to 11 years. All patients are at or above the 50th percentile for weight by age and are continent with 1 to 5 daily bowel movements. Only two patients required reoperation. A perirectal abscess developed in one patient 2 months postoperatively. In the second patient a functional obstruction was relieved by sphincterotomy. CONCLUSIONS: Ileoendorectal pull-through with right colon onlay patch is associated with few early and late postoperative complications; it appears to be superior to other procedures in the early postoperative period because of the more rapid return to acceptable stooling patterns. This method of reconstruction provides an excellent opportunity for normal growth, development, and long-term bowel function.

Simultaneous correction of malrotation and gastroesophageal reflux in infants.

Gastroesophageal reflux (GER) is often associated with gastrointestinal malrotation in infants. Primary correction of the malrotation, reserving a secondary antireflux procedure for those patients with persistent symptoms of GER, is most commonly practiced. This decision is based on the notion that an antireflux procedure may be unnecessary and is associated with added morbidity. We retrospectively reviewed 12 infants with GER and malrotation. All infants had symptoms attributed to GER and/or malrotation. A control group of seven infants with malrotation only was included for comparison of operative duration and postoperative recovery. Infants who received concurrent Ladd and Nissen procedures (Group 1, n=8) had immediate resolution of symptoms. Infants treated by Ladd procedure alone (Group 2, n=4), had persistent symptoms, despite postoperative medical therapy. A subsequent antireflux procedure was necessary. Comparison of operative times showed that a simultaneous procedure added, on average, 35 minutes to the Ladd procedure. Postoperative feeding and hospital stay were comparable between Group 1 and the control group. In contrast, Group 2 patients had longer hospitalizations due to ineffective medical therapy for persistent GER. No significant morbidity was noted. We recommend comprehensive surgical treatment with concurrent Ladd and Nissen procedures. This approach provides expedient and effective treatment of GER and malrotation, with minimal increase in operative time and no increase in morbidity .

Congenital bronchobiliary fistula: diagnosis and postoperative surveillance with HIDA scan.

Congenital bronchobiliary fistula (CBBF) is an extremely rare anomaly with myriad presentations that often include common bile duct abnormalities. Traditionally, bronchoscopy and bronchography have been used to establish the diagnosis. A full-term neonate with pneumonia and bilious secretions was diagnosed as having CBBF by means of a HIDA scan. Successful repair consisted of a right thoracotomy, high ligation of the fistula via an extrapleural approach, and cholecystography to confirm biliary drainage into the duodenum. The HIDA scan is a safe and efficient means to diagnose CBBF. When used in combination with surgical contrast studies, it provides clear delineation of all variations of this abnormality, enabling one-stage correction.

A comparison of the hemodynamic and ventilatory effects of abdominal insufflation with helium and carbon dioxide in young swine.

Abdominal CO2 insufflation has been shown to cause hypercarbia, acidemia, and decreased oxygenation in a pediatric animal model. Such metabolic derangements have prompted a search for alternative insufflation gases. This study compares the hemodynamic and ventilatory changes that occur during pneumoperitoneum with CO2 and helium. Four juvenile swine were intubated and given general anesthesia. Minute ventilation was adjusted to obtain a baseline Pco2 of between 32 and 36 mm Hg, and was kept constant for the duration of the experiment. The subjects initially were insufflated with CO2 or helium at a pressure of 10 mm Hg. Peak ventilatory pressure, end-tidal CO2 (ETCO2) arterial pH, Pco2, Po2, and right atrial and inferior vena caval pressures were measured before and during a 1-hour insufflation period. After desufflation, Pco2 and pH were restabilized. The same parameters were then measured during reinsufflation with the alternate gas. CO2 insufflation caused significant decreases in pH, from 7.51 +/- 0.03 to 7.32 +/- 0.06, and Po2 increased from 261 +/- 49 to 189 +/- 33 mm Hg. Pco2 increased from 35.0 +/- 1.4 to 57.9 +/- 6.3 mm Hg. ETCO2 also increased, from 29.0 +/- 2.2 to 47.2 +/- 5.0 mm Hg. Helium insufflation caused pH to decrease from 7.51 +/- 0.01 to 7.42 +/- 0.04. Pco2 increased from 32.8 +/- 0.8 to 43.5 +/- 3.9 mm Hg, and ETCO2 increased from 27.8 +/- 0.5 to 36.8 +/- 3.1 mm Hg. These alterations were significantly less than those with CO2 pneumoperitoneum. Po2 decreased as well-from 266 +/- 30 to 212 +/- 21 mm Hg. During insufflation with both gases, peak ventilatory pressure and right atrial pressure increased significantly. Abdominal insufflation with CO2 or helium causes hypercarbia, acidemia, and increased ETCO2 in this juvenile animal model. These derangements are significantly less with helium. This gas may prove to be the more suitable insufflation agent for pediatric patients.

Operative treatment of gastroesophageal reflux in low birth weight infants.

Operative correction of symptomatic gastroesophageal reflux (GER) has been used frequently and successfully in both children and larger infants for many years. In contrast, surgical repair of GER has been applied relatively sparingly in very small infants because of perceived technical and postoperative difficulties. We retrospectively reviewed our experience with Nissen fundoplications performed for symptomatic GER in low birth weight (LBW) infants ( < or = 2500 grams the time of surgery). Twenty-one consecutive cases from 1988 to 1993 were evaluated. At the time of surgery, the average age was 9.1 weeks and the average weight was 2100 grams (range, 1220 to 2500 grams). All infants had failed a trial of medical management. Follow-up from 1 month to 5 years showed no intraoperative complications and resolution of symptoms in 91 per cent of our patients. Technical factors found to enhance the success of the operative repairs included appropriately sized dilators, division of the short gastric vessels, and a loose 360-degree wrap. With suitable technique, the surgical management of symptomatic GER can be performed safely in small infants.

A comparison of laparoscopic versus open splenectomy in children.

Laparoscopic operative procedures are gaining wider acceptance in pediatric patients. Although laparoscopic splenectomy is being performed more frequently, no studies to date have compared this procedure with the standard open technique with respect to operative outcomes. We performed a case control study of seven laparoscopic splenectomies and 14 open splenectomies. Two-tailed t test was used to compare the two groups for mean operative time, mean hospital stay, mean interval before tolerating a regular diet, and total parenteral narcotic dose in morphine equivalents. Operative time was significantly longer in the laparoscopic group (221 minutes vs 59 minutes, P < 0.001). Hospital stay, interval before tolerating a regular diet, and postoperative narcotic dose did not differ significantly between the two groups. In the laparoscopic group, one operation was converted to an open procedure secondary to bleeding complications. Three patients required mini-laparotomies for removal of extremely large spleens after completion of the dissection. Pediatric laparoscopic splenectomy does not appear to have advantages over the traditional method with regard to operative time, hospital stay, postoperative ileus, or postoperative pain. Larger studies, including cost analysis, are needed before major proposed advantages of laparoscopic splenectomy can be accepted.

Ultrasonic resection of neuroblastomas. Long-term local tumor control.

OBJECTIVE: To evaluate the effectiveness of ultrasonic aspiration in achieving local tumor control of bulky neutroblastomas that are considered unresectable by conventional means. DESIGN: A retrospective review of 12 patients undergoing ultrasonic aspiration as part of multimodal treatment protocols. SETTING: A pediatric oncology referral center. PATIENTS: Twelve children with large neuroblastomas located in the abdomen (n = 5), chest (n = 5), and neck (n = 2). Follow-up was 1.5 to 7.5 years. INTERVENTIONS: Ultrasonic aspiration of the tumor was primary therapy (n = 7) or followed initial chemotherapy (n = 5). All patients underwent subsequent chemotherapy or autologous bone marrow transplantation. MAIN OUTCOME MEASURES: The incidences of residual disease and local recurrence were examined. RESULTS: Tumor-related symptoms were effectively relieved in all 12 patients. Recurrent local disease led to death in two. One patient died of distant metastases. CONCLUSIONS: Ultrasonic aspiration minimized blood loss and did not cause damage to adjacent organs. It provided nearly complete tumor resection, enhanced the effectiveness of chemotherapy protocols, and decreased the need for supportive care. Ultrasonic aspiration is a safe and effective method for obtaining local control of large neuroblastomas.

Minimally invasive surgery in children with cancer.

BACKGROUND: The safety and efficacy of minimally invasive oncologic procedures in children have not been well defined and only limited anecdotal experience has been published. METHODS: A retrospective review of all patients undergoing either a laparoscopic or thoracoscopic procedure at Childrens Cancer Group institutions between December 1, 1991, and October 1, 1993, was performed. RESULTS: Eighty-five children underwent 88 minimally invasive surgical procedures as part of the evaluation or treatment for cancer at 15 participating centers. In 25 patients, laparoscopy was performed and 60 patients underwent 63 thoracoscopic operations. Tissue biopsies were taken in 67 cases and diagnostic material was obtained in 99% of the biopsies. Seven complications occurred, all within the thoracoscopic group. These included conversion of six operations to an open procedure. One patient developed atelectasis postoperatively. CONCLUSIONS: In pediatric patients with suspected cancer, laparoscopy was highly accurate with minimal morbidity; thoracoscopy was nearly as efficient with slightly higher morbidity. Both modalities are useful for assessment of resectability, for staging purposes, and for evaluation of recurrent or metastatic disease.

Diagnosis, management, and outcome of cervicofacial teratomas in neonates: a Childrens Cancer Group study.

The management of cervicofacial teratomas in neonates is often complicated and may result in significant morbidity and death. A Childrens Cancer Group (CCG) retrospective study was conducted to evaluate a multiinstitutional experience with the treatment of these extremely rare neoplasms. Twenty neonates with cervicofacial teratomas, presenting from 1971 to 1994, were identified from nine CCG institutions. Fourteen neonates had cervical teratomas, and six had orofacial teratomas. There were 12 males and eight females. A diagnostic prenatal ultrasound examination was performed in six cases. Life-threatening airway obstruction occurred in seven infants (35%) in the early postnatal period. Two neonates died in the delivery room without ever having their airway secured. Two other infants with a prenatal diagnosis survived only because tracheostomies were performed by pediatric surgeons who were in the delivery room. Three other patients were orally intubated, one after sustaining hypoxic cardiac arrest. Eighteen infants had their primary tumor excised. Three patients required tracheostomy. After resection, two patients had evidence of unilateral recurrent laryngeal nerve injury, and two required prolonged thyroid hormone replacement. Histological examination showed eight mature and seven immature teratomas. Four infants (20%) clearly had malignant lesions. Pulmonary metastases occurred in two patients and contributed to one late death at 6 months of age. The overall survival rate was 85%, and the mean follow-up period was 5 years (range, 2 months to 16 years). Twelve of 17 surviving patients (70%) have had an excellent functional and cosmetic outcome. Four children have varying degrees of developmental delay and mental retardation. Hypoxia at birth was believed to have contributed to these problems in two cases.(ABSTRACT TRUNCATED AT 250 WORDS)

Effect of ophthalmic metipranolol and timolol on exerise-induced tachycardia.

PURPOSE: We compared the systemic beta-adrenoceptor blockade elicited by metipranolol with timolol in a randomized, double-masked, multiple crossover study in 24 healthy volunteers. METHODS: On each of 4 test days, subjects exercised on a treadmill for 10 min at 4 mi/h at a 5% gradient. After a 30-min rest, they received one drop OU of either 0.1 or 0.3% metipranolol HCI, 0.5% timolol maleate, or an artificial tear vehicle. RESULTS: With 10 min of exercise, mean heart rate increased from +/-80 to 140 beats/min preinstillation. During the postinstillation exercise, mean heart rate increased to a maximum of 141.5 +/- 19.6, 141.8 +/- 17.2, 139.7 +/- 19.7, and 131.5 +/- 16.9 beats/min in the vehicle, 0.1% metipranolol, 0.3% metipranolol, and 0.5% timolol groups, respectively. Pairwise comparisons at this time revealed a significant difference between timolol and each of the other treatments. CONCLUSIONS: Given the ocular hypotensive equivalency of various concentrations of metipranolol to timolol in other studies, it appears that metipranolol may have an improved therapeutic index relative to timolol.

Abdominal expansion as a bridging technique in stage IV-S neuroblastoma with massive hepatomegaly.

Stage IV-S neuroblastoma has a relatively favorable outcome. However, urgent surgical treatment may be necessary for management of life-threatening complications related to massive hepatomegaly caused by metastatic tumor infiltration. The enlarged liver often becomes of primary concern because diaphragmatic elevation results in life-threatening respiratory embarrassment. An external SILAS-TIC dome has been used as a temporizing procedure to decrease the intraabdominal pressure. The authors developed a modification of this approach using an internal polytetrafluoroethylene patch to create a ventral hernia. This technique decreases the potential complications of prosthetic material use, namely, risk of infection. The graft may be left in place for an extended period and removed in staged operations as the bulk of the metastatic tumor regresses.

Diagnosis and treatment of respiratory symptoms of initially unsuspected gastroesophageal reflux in infants.

Gastroesophageal reflux (GER) in infants is most commonly thought of as repeated excessive vomiting and failure to thrive, with most infants responding favorably to medical therapy. However, GER may also manifest exclusively with a variety of respiratory symptoms that, if not detected and treated early, may lead to life-threatening complications. During the period of 1987 to 1992, 39 neonates and infants underwent Nissen fundoplication for the treatment of respiratory symptoms attributed to GER. Symptoms included apnea and bradycardia (64%), pneumonia (31%), cyanosis (28%), cough (18%), and stridor (15%). Most patients were ascribed at least one incorrect diagnosis to explain respiratory symptoms. These include apnea of prematurity (38%), bronchopulmonary dysplasia (31%), asthma (8%), and subglottic stenosis (8%). All patients underwent a variety of investigations and medical treatments without noticeable clinical improvement. These included bronchoscopy, esophagoscopy, and polysomnograms. Treatment such as antibiotics, theophylline, bronchodilators, steroids, and oxygen were directed at presumed primary respiratory disease. On the other hand, H2 blockers, metoclopramide, positioning, and thickened feeds were prescribed to treat GER without objective evidence of disease. Ultimately, GER was demonstrated by upper gastrointestinal series in 64%, pH probe in 61%, and both studies in 38%. All patients underwent Nissen fundoplication after failed attempts at medical therapy. A total of 95% of patients had resolution or substantial improvement of respiratory symptoms postoperatively. Preoperative hospitalization averaged 37.0 days, and postoperative stay averaged only 14.2 days. We present a series of patients with GER, all of whom presented with respiratory symptoms.(ABSTRACT TRUNCATED AT 250 WORDS)

Hemodynamic and ventilatory effects of abdominal CO2 insufflation at various pressures in the young swine.

Laparoscopic procedures requiring abdominal CO2 insufflation are applied with increasing frequency to the pediatric age group. Whereas the physiological effects accompanying insufflation have been studied in laboratory and clinical adult subjects, little is known of these effects in the juvenile subject. Young swine (weight, 10 to 12 kg, approximate age, 6 weeks) were subjected to abdominal CO2 insufflation at 10 and 15-mm Hg insufflation pressures (IP) to evaluate potential metabolic and hemodynamic effects. After intubation, minute ventilation was adjusted to obtain a baseline PCO2 between 38 and 42 mm Hg, and was kept constant during the subsequent study period. Four subjects underwent a total of eight trials, with insufflation periods of 1 hour each. Serial measurements of peak ventilatory pressure, tidal volume, end tidal CO2, arterial pH, PCO2, PO2, right atrial (RA), and inferior vena caval (IVC) pressure were obtained. Mean pH decreased from 7.45 +/- 0.03 to 7.22 +/- 0.03 at 10 mm Hg IP, and from 7.44 +/- 0.01 to 7.19 +/- 0.03 at 15 mm Hg IP. Mean PCO2 increased from 39.9 +/- 0.4 to 70.0 +/- 1.0 mm Hg at 10 mm Hg IP, and from 39.9 +/- 0.5 to 76.8 +/- 1.6 mm Hg at 15 mm Hg IP. PO2 decreased by approximately 37% at both IPs. End-tidal CO2 increased by 53% at 10 mm Hg IP, and by 68% at 15 mm Hg IP. Right atrial pressure did not increase significantly, and IVC pressure increased in proportion to the IP. Abdominal CO2 insufflation in this model produced marked acidemia, hypercapnia, decreased oxygenation, and increased exhaled CO2.(ABSTRACT TRUNCATED AT 250 WORDS)