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We reviewed the neurologic and developmental courses in 10 children with opsoclonus-myoclonus ("dancing eyes syndrome") and neuroblastoma. All patients are alive without evidence of neoplastic disease after 8+ to 111+ months of follow-up. All had localized disease and 50% had extraabdominal tumors. Neuroblastomas of nine children had favorable Shimada histologic characteristics, and all tumors had single copies of the N-myc oncogene. After neuroblastoma resection, all patients had persistent opsoclonus-myoclonus or ataxia that responded to therapy with adrenocorticotropic hormone. Nine children had relapses of neurologic symptoms. Three years after resection, six of seven patients with sufficient follow-up were free of symptoms and had discontinued therapy. However, nine children had chronic neurologic deficits, including cognitive and motor delays, language deficits, and behavioral abnormalities. All six patients in educational programs required special assistance. Five children required physical, occupational, or speech therapy. Long-term developmental and cognitive problems should be anticipated in patients with neuroblastoma who have opsoclonus-myoclonus or ataxia or both, and early intervention should be instituted to try to minimize these deficits.

A retrospective analysis of the medical records of 234 children with renal tumors managed over a 25-year period at the Children's Memorial Medical Center was undertaken to evaluate long-term morbidity and mortality. There was a significant increase in survival over the years of the study. The 5-year survival for patients treated during the period 1985 to 1989 was 94% versus 68% for the period 1965 to 1969. Thirty-three children have died, 15 with known disease progression. Long-term morbidity included scoliosis (39), cardiorespiratory insufficiency (13), hypertension (7), renal insufficiency (7), small bowel obstruction (10), chest wall deformity (3), amenorrhea (1), leg length discrepancy (1), and 1 patient with an esophageal stricture. One patient with cardiomyopathy secondary to adriamycin has recently undergone cardiac transplantation. Five patients with renal insufficiency have required dialysis. Of these five, one patient has had two renal transplants. The presence of distant metastases and positive hilar or regional lymph nodes were the only findings at operation that were associated with an increased mortality (P = .005). There was a significantly increased mortality in those children operated on by general surgeons or urologists at other hospitals (11/43) versus those operated upon at our hospital (22/191) (P = .033). There was no statistical difference in the staging or histology among these children. We feel that the careful and systematic approach of a radical nephrectomy assures accurate staging of the tumor removing gross and microscopic disease in the abdomen.

Aims The optimal surgical treatment for extremely-low-birth-weight (ELBW) neonates with pneumoperitoneum is controversial. This study aimed to identify clinical factors associated with two known causes of pneumoperitoneum-necrotizing enterocolitis (NEC) and spontaneous intestinal perforation (SIP), and assesses the treatment outcome with primary peritoneal drainage (PPD) vs. laparotomy. Methods We reviewed and analyzed clinical characteristics and outcome from records of neonates with pneumoperitoneum treated at our institution from January 1999 to January 2003. Results Forty-six neonates (31 NEC, 15 SIP) were treated with either PPD (20 with NEC, 13 with SIP) or laparotomy (11 with NEC, 2 with SIP). In neonates who underwent PPD, those with NEC (vs. SIP) were less likely to have a patent ductus arteriosus, but were more likely to have been fed, have drains placed later in life, have a subsequent laparotomy, a longer total parental nutrition course, a higher 30-day mortality, and to take more days to begin enteral feeds. Conclusion The etiology of pneumoperitoneum (NEC vs. SIP) in ELBW neonates can usually be determined preoperatively. Neonates with SIP should have a drain placed while those with NEC should undergo laparotomy.

Nonoperative management of splenic and hepatic injuries in children is safe, and the majority of those with isolated injuries do not require blood transfusion. Thirty-seven children were treated for blunt splenic or hepatic trauma from November 1983 to September 1989. There was one death in a patient with a lethal head injury. No operations were performed on those with isolated splenic or hepatic injuries. Three of those with multiple injuries underwent delayed laparotomy. Two had perirenal and retroperitoneal hematomas without active bleeding, and one had a bowel obstruction secondary to an intramural jejunal hematoma. There were no late complications related to the splenic or hepatic injuries. Eight children (22%) required surgery for other injuries. Twelve children were not transfused, including the majority (811) of those with isolated splenic or hepatic injury. The hematocrit of four of these children fell to below 28% and this anemia was well-tolerated. Two children with bleeding disorders (factor VIII [antihemophilic factor] and factor XII [Hageman factor] deficiency) did not require packed red blood cells transfusion. Two clinically distinct groups of children received blood transfusions: (1) eight patients with multiple injuries were transfused during initial resuscitation when unstable or during early operation for other system trauma (mean, 62.0 mL blood/kg body weight); and (2) three hemodynamically stable patients with isolated injuries and 14 stable patients with multiple injuries were transfused empirically after initial resuscitation solely because of decreasing blood counts. They received an average of 16.5 and 21.1 mL blood/kg body weight, respectively. Transfusions should be limited or withheld in those who are hemodynamically stable after initial resuscitation. The hematocrit can decrease to 24% to 25% in these children without complications.

Each case of conjoint twins is unique. Preoperative imaging is helpful to determine the feasibility of separation. Shared and separate organs can be delineated; and operative technique and problems anticipated. We performed ultrasonography, CT, magnetic resonance imaging, and arteriography preoperatively on ischiopagus conjoint twins. The single most helpful study was CT performed during a bolus intravenous contrast medium injection into one twin. Arteriography was also very helpful. The other studies were complementary but added little additional structural details. Sedation of conjoint twins is complicated yet crucial for optimal imaging studies. A combination of oral and intramuscular sedation was used and worked well for all of the studies.

The recent pattern of immigration from Indochina and Latin America to the United States suggests that tuberculosis will remain a significant public health problem. Two infants recently seen with probable congenital tuberculosis prompted critical evaluation of the 24 cases of congenital tuberculosis reported in the English literature since the introduction of isoniazid in 1952. Failure to thrive, jaundice, and central nervous system involvement, all reported in previous reviews and textbooks to be very common, were unusual presenting manifestations. In contrast, hepatomegaly, a finding not mentioned in the recent literature, was common. Diagnostic procedures previously underutilized but found in this review to be useful included liver biopsy, biopsy of skin lesions when present, and cultures of gastric aspirates. Factors which enable differentiation of congenital from early postnatally acquired tuberculosis include (1) the presence of known maternal tuberculosis at delivery, (2) whether infant and mother were separated from birth until the onset of illness, and (3) whether other tuberculous exposure of the infant can be determined. Insufficient data prevent recommendation of a preferred regimen of drugs in addition to isoniazid for the treatment of congenital tuberculosis. However, the responses of our patients suggest that streptomycin can be omitted without hazard. Information regarding the long-term prognosis of survivors is lacking, but early diagnosis and institution of appropriate therapy has markedly decreased the mortality of this previously fatal disorder.

We have seen a modest improvement in the survival of a homogeneous group of critically ill newborns with congenital diaphragmatic hernia since 1979. Twenty-seven "critical" infants have been treated who developed respiratory distress shortly after birth, required urgent resuscitation, and could not be stabilized before operation. Two died with other anomalies that appeared incompatible with prolonged survival. Ten of the 27 lived. This survival contrasts with that of only two of 17 similarly affected babies treated from 1962 to 1978. In addition, there has been no operative mortality outside of this "critical" group since 1979; whereas six noncritical babies died between 1967 to 1978. Our current therapeutic plan includes the early establishment of a respiratory alkalosis and vasodilator therapy before or during transport. Postoperatively we have attempted to maintain the baby's arterial pH>7.5, Pco 2 2 ∼150 torr. The most effective ventilatory parameters have been a rate of 130, PEEP of 5 and an inspiratory:expiratory ratio of 1:1. Peak airway pressures are kept as low as possible. Pharmacologic and ventilator therapy are weaned slowly, and intensive support has been required for at least 48 hours in each baby. Retained secretions and atelectasis of the hypoplastic lung persisted for two to several weeks postoperatively. Two babies that are one year of older still appear to have severely hypoplastic lungs on chest x-ray. M-mode echocardiography has been used to measure ventricular ejection periods. The right ventricular systolic time interval correlates with the degree of pulmonary hypertension. This noninvasive modality is a promising means of following pulmonary vascular resistance. Evidence of decreasing resistance can be taken as an indication to wean aggressive therapy.

INTRAPERITONEAL ANTIBIOTICS have been used for over 30 Deyr. spite numerous experimental and clinical investigations, their role in clinical peritonitis has not been clarified. A number of retrospective studies have included patients of varying ages with peritonitis of diverse origins. Appendicitis, with generalized peritonitis in children is still a major clinical problem. The benefits of systemic antibiotics in these cases remains controversial and the use of intraperitoneal antibiotics has not been widely reported. At the Children's Memorial Hospital we have carried out a double-blind controlled study of the effects of intraperitoneal and wound irrigation with kanamycin in cases of perforated appendicitis. METHODS Children with perforated appendicitis and established peritonitis undergoing operation from April 1969 to July 1974 were included in this study. Cultures of peritoneal fluid or pus were obtained on each patient. The use of systemic antibiotics and ~.eritoneal or wound drainage was left to the discretion of the surgeon. Kanamycin and placebo solutions were supplied in numerically coded vials, previously randomized by Bristol Laboratories. These were added to identically coded 500 ml bottles of normal saline by the hospital pharmacy prior to use. Before closure of the peritoneum the peritoneal cavity and the wound were irrigated with either a 0.25% solution of kanamycin or with normal saline. No attempt was made to suction all of the irrigant. A volume of 20 ml/kg of body weight was used up to a maximum of 500 ml. Irrigation with the kanamycin solution delivered 50 mg of drug/kg body weight. Each patient was specifically observed during the intraoperative and postoperative periods for signs of respiratory depression. None exhibited any sign of depression or other evidence of drug toxicity. The postoperative courses of 79 patients with perforated appendicitis were analyzed. This patient population included 37 boys and 42 girls. Nineteen were black, 59 white, and one oriental. Forty-eight per cent were between six and 10 yr of age; 24% were 13 mo to 5 yr; and 28%, 11-16 yr. Wound infections that necessitated the drainage of a primarily closed wound or prevented a planned delayed primary closure were distinguished from wounds showing only prolonged or purulent drainage at sites of peritoneal or wound drains. Localized intra-abdominal abscesses requiring operative drainage have been listed separately from presumed pelvic phlegmons that resolved spontaneously.

Complete excision of Wilms' tumor may require resection of adjacent organs and removal of intracaval tumor propagation. Extension of tumor to the right atrium can be determined preoperatively guiding a direct and safe approach to intracardiac tumor at the time of nephrectomy. Preoperative ultrasonography of two children with Wilms' tumor demonstrated caval and right atrial tumor. Laparotomy for nephrectomy and abdominal caval exposure was combined with cardiopulmonary bypass and atriotomy. In both patients, tumor contiguous with the renal pelvis extended from the iliac bifurcation into the right atrium with a large atrial mass. In one patient nephrectomy was performed first, and she was then placed on cardiopulmonary bypass. Caval tumor was easily removed through the atriotomy and open renal vein. In the second patient, bypass was instituted first because of cardiac instability. The large right atrial mass extended through an atrial septal defect into the left atrium. The cardiac tumor and a large amount of caval tumor were removed. Bypass was discontinued after repair of the ASD. Tumor remained in the IVC below the renal veins necessitating a separate venotomy. Combined abdominal and cardiac exploration allows safe and complete excision of all gross tumor. Ultrasonography is a sensitive and noninvasive method of diagnosing retrohepatic and atrial tumor extension and can be obtained easily even on very sick patients.

Twelve of forty patients (30%) treated for esophageal atresia at the Children's Memorial Hospital in Chicago between June 1976 and May 1980 required a circular esophagomyotomy with or without upper pouch bougienage to achieve an anatomical repair. Ten patients had a distal tracheoesophageal fistula; 1 had a proximal fistula as well; and 2 had no fistula. Over 40% of the patients were small for gestational age. One half had severe associated anomalies including congenital heart disease in 1/3 and the VATER association in 1/4. Three patients died, all from congenital heart disease. Immediate complications in 6 patients included anastomotic leaks (3), tracheal injuries (2), and mucosal entry at the myotomy site (1). Late complications included symptomatic gastroesophageal reflux (60%), anastomotic strictures (40%), and severe tracheomalacia requiring long-term tracheotomy (20%). Altered esophageal motility (77%) contributed to poor weight gain (less than 3rd percentile) in most patients. In spite of these complications, satisfactory results were achieved in 8 of the 9 long-term survivors. The ninth patient had a turbulent neonatal period and has persistent swallowing difficulties at 36 mo follow-up. Although an anatomical repair can now be achieved in all patients with esophageal atresia utilizing bougienage and myotomy, some ill neonates may be better served by traditional staging techniques.

Endodermal sinus tumors occur in the gonads and in a variety of extragonadal locations, particularly as the malignant component of sacral teratomas. Light and electron microscopic studies as well as the presence of alpha fetoprotein support the concept that these lesions arise from germ cells that migrate from the extraembryonic yolk sac. These tumors in all locations are highly malignant and metastasize to lymph nodes, lung, liver and bone. Peritoneal implants are seen from tumors in the ovary, testis, and following ventriculoperitoneal shunts for yolk sac tumors of the pineal gland. There is no satisfactory therapy for the endodermal sinus tumor, regardless of location, when there are distant metastases at the time of diagnosis; however, we have documented complete regression of metastases in one child following therapy with vinblastine, bleomycin, and cis-platinum. Nine of 13 boys with endodermal sinus tumors of the testis survived in this series of patients. All survivors were treated with orchiectomy and lymph node excision. Surgical excision combined with chemotherapy and radiation led to survivors in children with large extragonadal pelvic and retroperitoneal tumors. We recommend vigorous multimodal therapy with surgery and chemotherapy. Radiation is given to the primary lesion when it is too extensive for initial resection or to eradicate residual disease following chemotherapy. Alpha fetoprotein is a valuable tumor marker.

In contrast to the two prior decades, empyema in children has become a rare disease during the past 10 years. Thirty-two children with empyema from all causes were treated at The Children's Memorial Hospital in Chicago in the 11 years between 1970 and 1982. Twenty cases followed postinfectious pneumonia, most commonly due to Staphylococcus aureus. The remaining patients had either an esophageal leak or a pleural infection following an intrathoracic operation. Fourteen of our patients were managed with a "mini-thoracotomy," which allowed accurate drainage of purulent material, debridement of fibrinous exudate, and rapid expansion of the lung with prompt relief from fever and toxicity. In our hands, this procedure provided results which were superior to those obtained with simple closed chest tube drainage. In addition, during this period of time, ultrasound examination of the chest has provided a rapid, accurate technique for the early detection and localization of fluid accumulations within the pleural cavity. Pre-drainage thoracentesis can be accurately guided by ultrasonic imaging.

Twenty cases of pneumonectomy in infants and children performed over a 35-yr period have been reviewed. Pneumonectomy may be necessary for inflammatory, congenital, neoplastic, and traumatic conditions. The postoperative complications of progressive mediastinal shift can be minimized by the use of a pleural prosthesis.

Bronchoplastic reconstruction can salvage normal Bronchial and pulmonary tissue distal to diverse obstructive bronchial lesions. Five children underwent proximal bronchial and limited pulmonary resections allowing preservation of normal distal parenchyma. Three patients were infants between 2 1/2 and 12 months of age. All five survived without immediate or late complications. Follow-up included interval bronchoscopy and chest radiography, and ranged from 8 months to 12 years. Cardiopulmonary bypass was unnecessary even in the case of a large carinal lesion. Techniques used to insure adequate ventilation during bronchotomy included positioning the endotracheal tube above the carina with clamp occlusion of the open mainstem bronchus, positioning the tube within the opposite mainstem bronchus, and inserting a sterile tube into an open mainstem bronchus (with connections to sterile ventilator tubing passed across the operative field). Preoperative bronchoscopy and radiographic studies localized the extent and base of the lesion, allowing precise placement of the bronchotomy. Conservative excision was accomplished by frozen section examination of all margins of resection. Ingenious usage of available tissue was essential to a successful reconstruction.

An acute pneumonic process in an immunosuppressed child poses a diagnostic and therapeutic challenge. These patients tolerate infection poorly. An open lung biopsy may provide prompt diagnosis. Nevertheless, a beneficial change in therapy that results in survival does not necessarily follow. Fifty-six immunosuppressed children with acute respiratory symptoms and interstitial pulmonary infiltrates underwent lung biopsy from 1974 to 1985. The most common underlying diagnosis was acute lymphocytic leukemia (60%). A specific etiology was determined in 46 (82%). Operative morbidity in 52% included prolonged intubation, recurrent pneumothorax, and hemorrhage. Overall, mortality was 34%. Those patients with solid tumor and those who required postoperative ventilation had a statistically significant higher mortality than all others. We defined biopsy "patient benefit" as follows: (1) the biopsy yielded an etiology for which a change of treatment was required; and (2) the child survived this acute illness. Despite the successful diagnostic results of this procedure, only 13 (23%) of the patients derived clinical benefit. Even though a specific infectious etiology was diagnosed in 39 (69%) patients only ten (18%) of these improved and survived after an appropriate change in therapy. Eight of these had Pneumocystis carinii. One survivor benefited from the treatment of documented radiation pneumonitis. Another was successfully treated for graft v host reaction but this diagnosis also was made by skin biopsy. One half of the biopsies were performed very early in the course of the illness, specifically to exclude Pneumocystis carinii of which we saw a peak incidence in 1978 to 1979.(ABSTRACT TRUNCATED AT 250 WORDS)

Children with foregut cysts of the mediastinum can present at any age with nonspecific respiratory symptoms or dysphagia. Chest radiograph and barium esophagram are recommended for initial evaluation, but they fail to identify some lesions. In other cases, an obvious mass may be confused with a solid neoplasm. We have operated upon 34 infants and children with mediastinal bronchogenic cysts 23 and esophageal duplications 11 from 1968 through 1985. This review of their clinical course and radiographic imaging studies emphasizes some of the diagnostic pitfalls that lead to operative delay. Twelve (35%) of these patients were asymptomatic. The correct diagnosis was delayed longer than 3 months from the onset of symptoms in 14 of the 22 symptomatic children. Fifteen of these presented with pneumonia or symptoms or airway obstruction. In 11, the cyst was in a perihilar or subcarinal location, areas in which a lesion can be "hidden" behind the cardiac silhouette. Five children with esophageal duplication had severe neonatal respiratory failure, chest pain, hematemesis or dysphagia. The immediate preop chest radiograph revealed a mass in 29 of 34 cases. However, the lesion was initially missed or never seen in eight of those who were symptomatic. Esophagram, performed in 23, was diagnostic in only six and was normal in four. Prior to 1979 when CT scanning became available at this institution, 11 of 19 children (57%) underwent extensive workup, but the preop diagnosis was correct in only 50%. Since 1979, only 4 of 15 (26%) have required similar evaluation, and the preop diagnosis has been correct in all. Computed tomography with contrast is indicated in those patients presenting as diagnostic dilemmas and should be the next study performed after chest radiograph, esophagram, and ultrasound when applicable: (1) Recurring symptoms without diagnosis, (2) Acute symptoms, but confusing radiograph. Three children had a "hidden" cyst obscured by lobar pneumonia in 2 and congenital cystic disease in the other. In a fourth infant, a giant cystic esophageal duplication filled the entire hemithorax. (3) question of a solid tumor mass (neurogenic neoplasm). (4) Planning a complex operation. Multiple operations to completely excise extensive duplications may be avoided by preoperative delineation of the full extent of the lesion.

We provided parenteral nutrition to 40 very low birth-weight premature infants (birth weight 815 +/- 17 grams, gestational age 27 +/- 2 weeks) with a superior vena cava catheter. To avoid the risk of transport, catheterization was performed under sterile conditions in the intensive care nursery. The central venous catheter facilitated administration of calories to sustain growth, especially in infants whose catheters remained in place for 3 weeks or longer. The overall incidence of catheter related sepsis was high (30%) but the majority of cases were due to Staphylococcus epidermidis and resolved without incident. Other complications of parenteral nutrition were minimal. This approach is a safe and effective means of providing nutrition to the very low birth-weight infant.

This case report describes the antenatal diagnosis of a fetal pulmonary anomaly. Early detection afforded detailed parental counseling and prompt neonatal diagnosis and treatment.

A 5-year-old boy presented with dysphagia, and barium swallow revealed a narrowing in the upper esophagus. Biopsies of the circumferential lesion showed gastric mucosa. The heterotopic gastric mucosa was resected via a cervical approach. After resolution of a minor anastomotic leak, he remains asymptomatic 10 years following resection with no evidence of recurrence by esophagrams and endoscopy.

Reflux of bacteria-laden intestinal contents into the biliary tree can be prevented by an intussuscepted valve in an isolated segment of jejunum interposed between the porta hepatis and the duodenum. This method of bile duct reconstruction was adopted in 1979 and since then has been used in 17 children with biliary atresia and 9 with choledochal duct cysts. Those with choledochal duct cysts are well. Follow-up HIDA scans demonstrate normal bile flow, and ultrasound examinations have not revealed dilated bile ducts. Bile flow was established in 13 infants with biliary atresia and was persistent in 8. Two expired because of unrelated problems between 2 to 6 months postoperatively. One of these children developed cholangitis, but at autopsy the nipple valve was incompetent. Six children currently have normal serum bilirubin levels and are clinically well. The average postoperative stay for these patients was 7.5 days. Three were rehospitalized for brief periods for suspected cholangitis which was not proven. Bile flow was never established in 4 babies and was present only briefly in 5. Five of these children died of progressive liver failure and one from unrelated causes. Two have had liver transplants, and a third is awaiting transplantation.

Ascending cholangitis remains among the most serious complications following operations for biliary disorders. The bacterial count of refluxing intestinal contents can be reduced by using an enteric conduit from the biliary tract to the relatively sterile duodenum. A valvular conduit prohibits reflux of intestinal contents and permits unobstructed antegrade flow of bile. This can be created by intussuscepting approximately 1 cm of intestine in the midportion of the conduit. During the last 3 yr, valvular conduits were created in 11 patients. Seven of these children were treated for biliary atresia, and 4 had operations for choledochal duct cysts. There have been no deaths or morbidity resulting from the use of the valvular conduits. The postoperative courses in these patients indicate that the use of an intussusception valve may be beneficial in the prevention of ascending cholangitis.

Right middle lobe syndrome (RMLS) is characterized by a spectrum of diseases from recurrent atelectasis and pneumonitis to bronchiectasis of the middle lobe. It has been described among all age groups, although the diagnosis in pediatrics may be delayed or missed because of non-specific symptoms or findings. Twenty-one children with RMLS were evaluated during the past 10 years with particular attention to the history, bronchoscopic and pathologic findings. Most of these patients had asthma or a family history of atopic disorders; 3 patients had a family history of RMLS. Only two of the 21 patients had sufficient obstruction on bronchoscopy to account for their disease. Four had evidence of concomitant laryngeal pathology. The various theories of pathogenesis are discussed. In this series, the non-obstructive (impaired collateral ventilation) theory appeared to be most plausible. Bronchoscopy was performed in all instances to rule out obstruction due to foreign body or tumor. It was therapeutic in two-thirds of the cases. Resolution occurred promptly in one-third, and eventually in another third. Of the remaining patients, 4 required lobectomy and were cured; two have decreased but persistent symptoms. An aggressive medical management following bronchoscopy is warranted in all cases, especially when there is a possibility of asthma.

The spectrum of diseases which results in gastrointestinal hemorrhage in children is quite unlike that seen in adults. Carcinoma is rare, while medical diseases and coagulation disorders are sufficiently common that the surgeon must be wary of operating on a child for bleeding without an excellent indication

The removal of one lung from a beagle puppy results in minimal interference with lung function or the arterial gases. The removal of air from the empty pleural cavity results in a shift of the mediastinum and overdistention of the contralateral lung. An immediate decrease in the PO 2 and increase in the PCO 2 is seen. Significant increase in the alveolar-arterial CO 2 gradient reflected marked increase in dead space ventilation. Biopsies of the overdistended lung demonstrated emphysema and disruption of alveoli. These changes may explain some of the deterioration of lung function and the complication of contralateral pneumothorax following repair of a Bochdalek diaphragmatic hernia. Our study suggests that the mediastinum should be stabilized in the midline after repair of a diaphragmatic hernia or after a pneumonectomy in an infant or small child.

Meconium diseases in infancy cannot be neatly separated into discrete categories of meconium plug syndrome, meconium ileus, and meconium peritonitis; nor can the therapy of each condition. A patient with any of the three may or may not have cystic fibrosis. All babies with any form of meconium obstruction or atresia must have a sweat chloride test to confirm or rule out this diagnosis. Repeated gastrografin enemas can decrease the need for operative intervention in all babies with meconium plug syndrome and in selected babies with meconium ileus. Babies with emconium diseases can now be managed with a low perinatal mortality.