Randomized study of intensive MOPP-ABVD with or without low-dose total-nodal radiation therapy in the treatment of stages IIB, IIIA2, IIIB, and IV Hodgkin's disease in pediatric patients: a Pediatric Oncology Group study.

PURPOSE: To determine whether the addition of low-dose total-nodal irradiation (TNI) in pediatric patients with advanced-stage Hodgkin's disease who have received eight cycles of alternating mechlorethamine, vincristine, procarbazine, and prednisone (MOPP) and doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) will improve the event-free survival (EFS) and overall survival (OS) when compared with patients who have received chemotherapy only. PATIENTS AND METHODS: At diagnosis, 183 children and adolescents with stages IIB, IIIA2, IIIB, and IV Hodgkin's disease were randomized to receive eight cycles of alternating MOPP-ABVD with or without low-dose TNI. RESULTS: Of 183 patients, four were rendered ineligible before treatment was initiated. One hundred sixty-one of 179 patients (90%) were in complete remission (CR) at the completion of eight cycles of alternating MOPP-ABVD; 81 were in the chemotherapy-only group and proceeded to observation off therapy, whereas 80 of 161 were to receive combined modality therapy (CMT). Nine of 80 patients randomized at the time of diagnosis to receive CMT did not receive radiation (RT) because of a protocol violation, but were monitored for EFS and OS and included in all analyses. The estimated EFS and OS rates at 5 years for the 179 eligible patients are 79% and 92%, respectively. The actuarial EFS at 5 years was 80% for patients who received CMT and 79% for patients who received MOPP-ABVD only. The OS for the former group is estimated to be 87% and for the latter patients 96%. Age < or = 13 years of age at diagnosis and the attainment of a clinical CR after three cycles of chemotherapy were associated with a statistically significant improved EFS. CONCLUSION: Our results indicate that after the delivery of eight cycles of MOPP-ABVD, the addition of low-dose RT does not improve the estimated EFS or OS in pediatric patients with advanced-stage Hodgkin's disease.

Continuous communicating esophageal and gastric duplication.

Duplications of the esophagus or stomach alone are infrequent, and complete foregut duplication has only rarely been described. Most combined esophagogastric duplications present within the first year of life, and if communication with the normal alimentary tract does occur, it does so only either above or below the diaphragm. This report illustrates a case of continuous duplication of the esophagus and stomach with communication to the normal alimentary tract at both proximal and distal ends. Operative management and a review of the literature and embryology are described.

Congenital pancreatic pseudocyst: report of two cases.

Pancreatic pseudocysts have not been reported to occur in the fetus or newborn. The authors report two cases of histologically proven pancreatic pseudocysts in neonates, which were detected using prenatal ultrasonography. Surgical management included external marsupialization followed by internal drainage in one case, and excision in the other. Both patients ultimately did well. The etiology of these lesions remains unclear.

One-stage versus two-stage Soave pull-through for Hirschsprung's disease in the first year of life.

Several investigators have reported good results after a one-stage Soave procedure without a stoma for infants with Hirschsprung's disease. The authors reviewed their concurrent experience with the one- and two-stage approaches, comparing the two groups with respect to rate of complications and clinical outcome. Over a 3-year period, 36 infants with colonic Hirschsprung's disease presenting in the first year of life were treated with a Soave pull-through. Thirteen had a one-stage pull-through, and 23 had a two-stage procedure using an initial stoma. There was no difference with respect to median age at time of diagnosis, median follow-up period, length of aganglionosis, or male:female ratio between the groups. The incidences of major complications such as small bowel obstruction, segmental or acquired aganglionosis, anastomotic leak, and malabsorption were equal between the two groups. However, 13% of the two-stage patients required revision of the stoma. All major complications in the one-stage group were in those who weighed less than 4 kg at the time of surgery. Minor complications such as wound infection, perianal excoriation, and need for repeated dilatation were similar between the groups, but minor stoma-related complications (prolapse or retraction) occurred in 26% of the two-stage infants. When complications were stratified using a more sophisticated scale of severity, no significant difference was found between the groups. The overall complication rate was 1.5 events per patient in the one-stage group and 2.0 events per patient in the two-stage group. This small difference was related to the presence of a stoma in the two-stage group. Overall, 10 of 12 survivors in the one-stage group and 22 of 23 in the two-stage group were doing well, with normal bowel function noted on long-term follow-up (mean period, of 14 and 19 months, respectively). Both one- and two-stage approaches were associated with a significant complication rate, although long-term outcome was excellent in both groups. The higher complication rate in the two-stage group was attributable to the presence of a stoma. For small infants, it may be beneficial to delay the one-stage pull-through until weight exceeds 4 kg.

Mesenteric cysts in children.

BACKGROUND: Mesenteric cysts are uncommon benign abdominal masses. Approximately one third of patients with these lesions are children. METHODS: We reviewed our clinical records for the past 14 years (corresponding to the period of time in which ultrasonography and computed tomography became reliable methods for imaging pediatric patients) and found 10 patients with mesenteric cysts. RESULTS: We were surprised to discover that abdominal pain was a presenting complaint in all but one patient. Five of the patients had the findings of an acute surgical abdomen and were thought to have appendicitis. Two patients operated on for appendicitis were transferred to our hospital with the diagnosis of an abdominal mass. In each case the mass was a mesenteric cyst. Cyst distribution included the small-bowel mesentery in seven patients, the transverse mesocolon in two patients, and the right mesocolon in one patient. Six cases required concomitant bowel resection for the cyst removal, and all were cystic lymphangiomas. The resected specimens were described as cystic lymphangiomas in eight of the 10 cases. CONCLUSIONS: Mesenteric cysts should be considered as an origin for abdominal pain in children, particularly after exclusion of more common diagnoses. We have found ultrasonographic imaging to be a reliable method for the diagnosis of appendicitis in children and advocate its use as an initial imaging study in patients with an acute surgical abdomen and presumed appendicitis. If appendicitis is indicated unlikely by ultrasonogram, the examination can be extended to the remainder of the abdomen, which can reveal mesenteric cysts or other pathologic conditions.

Inflammatory myofibroblastic tumor of the spleen: report of a case in an adolescent.

Inflammatory myofibroblastic tumor (IMT) is an uncommon quasineoplastic process occurring in young patients. Splenic involvement is uncommon and occurs predominantly in older, adult patients. We present the youngest patient reported to date with splenic inflammatory myofibroblastic tumor and discuss the clinical, pathological and imaging features of this lesion.

Changing role of surgery in childhood lymphomas.

The use of combinations of chemo- and radiotherapy have decreased the need for surgical staging of Hodgkin's disease. The goal of decreasing therapy while maintaining cure rates, however, recreates a need for staging. Attempts are being made to devise criteria to decrease the need for surgical staging or to decrease the extent of the procedure. Non-Hodgkin's lymphoma requires adequate tissue samples for analysis, for example, for immunophenotyping to determine treatment. Surgical resection in non-Hodgkin's lymphoma is inadequate for treatment but may be helpful. Debulking, however, is no longer recommended.

With modern imaging techniques, is staging laparotomy necessary in pediatric Hodgkin's disease? A Pediatric Oncology Group study.

PURPOSE: To determine whether the information gained from staging laparotomy can be predicted by imaging and/or clinical factors in children with Hodgkin's disease. PATIENTS AND METHODS: Between 1986 and 1991, 216 consecutive pediatric patients with Hodgkin's disease underwent laparotomy and were treated on two concurrent protocols in a multiinstitutional cooperative group. All patients had computed tomography (CT) of the chest, abdomen, and pelvis. Clinical factors studied included sedimentation rate, B symptoms, histology, number and location of involved sites, sex, mediastinal involvement, and age. Pretreatment CTs were centrally reviewed in 88 cases for the presence and size of both supradiaphragmatic and infradiaphragmatic lymph nodes, intrinsic spleen lesions, and splenic size. Models were generated that were predictive of any abdominal disease, splenic involvement, extensive splenic involvement, and upstaging at the laparotomy. False-positive and false-negative rates were calculated. RESULTS: For the end point of any abdominal disease, a model based on B symptoms, histology, sedimentation rate, and number and location of involved sites was highly significant (P < .0001). However, the success in predicting abdominal disease in an individual patient was limited: false-negative rate, 26%; false-positive rate, 32%. Highly significant models based on clinical factors and/or radiographic findings were also generated to predict splenic involvement, extensive splenic involvement, and upstaging with laparotomy, but they also had high false-positive and false-negative rates. CONCLUSION: Laparotomy findings cannot be predicted accurately in the majority of patients based on knowledge of CT findings and clinical factors.

Optimum concentration of bupivacaine for combined caudal--general anesthesia in children.

Caudal epidural anesthesia has become widely accepted as a means of providing postoperative pain relief and intraoperative supplementation to general anesthesia for children. To determine the best concentration of bupivacaine for combined general-caudal anesthesia in children, 122 children aged 1-8 yr scheduled for outpatient inguinal herniorrhaphy were randomized to receive, in a double-blind fashion, caudal anesthesia with bupivacaine in one of six concentrations (0.125, 0.15, 0.175, 0.2, 0.225, or 0.25%). After incision, a programmed reduction in inspired halothane resulted, if tolerated by the subject, in an inspired halothane concentration of 0.5% 10 min after incision. End-tidal halothane concentration at hernia sac ligation for subjects receiving 0.175% bupivacaine (0.55 +/- 0.03%) was less than that for subjects receiving 0.15% bupivacaine (0.75 +/- 0.05%; P less than 0.05). Subjects receiving 0.175% bupivacaine also were discharged earlier from the postanesthesia care unit (PACU) (27 +/- 1 min) than were subjects receiving 0.15% bupivacaine (38 +/- 5 min; P = 0.05). Children receiving greater than or equal to 0.2% bupivacaine tended to complain more of leg weakness after surgery; however, the difference did not reach statistical significance (39 of 67 vs. 16 of 47; P = 0.057). The incidence of complaints of leg weakness and paresthesia was positively correlated with bupivacaine concentration (r = 0.706; P = 0.05). Subjects receiving 0.125% bupivacaine had higher pain scores on arrival to the PACU than did those receiving 0.2% bupivacaine (P = 0.05); there were no other differences in pain scores.(ABSTRACT TRUNCATED AT 250 WORDS)

Ischiopagus tetrapus twins: urological aspects of separation and 10-year followup.

Conjoined twins occur once in 50,000 births. Only 6% of conjoined twins are of the ischiopagus type in which the twins are joined symmetrically at the pelvis and fusion begins at the level of the common umbilicus. The longitudinal axis extends in a straight line in opposite directions and the genitourinary and gastrointestinal tracts are shared. Tetrapus is a subtype in which all 4 lower extremities are present and oriented at right angles to the axis of the common trunk. Two sets of female ischiopagus tetrapus twins were born in 1977 and successfully separated at the St. Louis Children's Hospital in the following year. We describe the genitourinary and associated anomalies, surgical separation and long-term urological followup of these 2 sets of ischiopagus tetrapus twins.

Caudal epidural anesthesia in conscious premature and high-risk infants.

Twenty premature or high-risk infants received caudal epidural anesthesia for inguinal herniorrhaphy, orchiopexy, and circumcision. Mean gestational age at surgery was 48 +/- 12 weeks; mean weight at surgery was 4,100 +/- 1,400 g. Caudal anesthesia, performed with 1 mL/kg of 0.375% bupivacaine, was successful in 19 of 20 infants. Onset of anesthesia occurred in 14 +/- 1 minutes; duration of surgical anesthesia was 89 +/- 8 minutes. Surgical conditions were generally excellent and the infants tolerated anesthesia and surgery well. No postoperative complications were observed. Caudal epidural anesthesia is an acceptable alternative to general or spinal anesthesia in premature and high-risk infants.

Cimetidine and neonatal small bowel adaptation: an experimental study.

Histamine2-blockers are being used more extensively in the pediatric short gut patient as an agent to accelerate small bowel adaptation. Reversal of lipid malabsorption and a direct trophic effect on the intestinal crypt cells have been postulated as the mechanism for the salutary influence of cimetidine. Weanling Sprague-Dawley rats underwent 85% small bowel resection. Controls had repair of a simple ileal transection. Rats received either a fat-defined rat chow only, or chow with high- or low-dose cimetidine, 2% cholestyramine, or cholestyramine/low-dose cimetidine. All animals were killed 2 weeks postresection, and ileal and jejunal sections were examined for changes in villous and crypt morphology. The animals receiving cimetidine showed earlier and more consistent weight gain than resected animals who received no adjunctive treatment. High- and low-dose regimens were equally efficacious. Cimetidine administered alone decreased fecal fat losses, but not when given with cholestyramine. The cimetidine/cholestyramine group showed increased weight gain when referenced to the resection controls despite continuing lipid malabsorption. Villous and crypt lengthening did not correlate with clinical evidence of adaptation. An augmented lymphocytic activity (plasma cell hyperplasia, enlargement Peyer's patches) was present in the hyperplastic ileal segments of the cimetidine-treated rats. Overall immunoreactivity was similar in all study groups. No significant differences in villous morphology or immunologic activity were seen in jejunal segments. The effects of H2-blockers on lipid absorption and intestinal hyperplasia are inadequate to explain the benefits of cimetidine in the short gut patient. Examination of the immunology of the short bowel complex merits further attention in elucidating cimetidine's action in this setting.

Cytoprotective agents in experimental small bowel volvulus.

An accepted experimental model for midgut volvulus was used to produce small bowel strangulation obstruction of 48 hours duration in Sprague-Dawley rats. A 93% perioperative mortality rate resulted after release of the volvulus. Treatment with three cytoprotective agents at the time of volvulus release resulted in the following mortality rates: superoxide dismutase, 89%; ibuprofen, 50%; prostaglandin E1 (PGE1, 11%. The predominant cause of death in all treatment groups was bowel infarction, with a smaller number succumbing to either sepsis or circulatory collapse. Concomitant administration of ephedrine or indomethacin to suppress prostaglandin E1's splanchnic vasodilatory activity did not cause any increase in mortality. A trial of aspirin, to simulate PGE's antiplatelet actions, showed no reduction in mortality when compared with detorsion alone. Prostaglandin E1 and, to a lesser extent, ibuprofen, appear to have cytoprotective effects during reperfusion of bowel compromised by volvulus, independent of their influence on the mesenteric vasculature and thrombogenesis.

Multiple granular cell tumors. A familial occurrence in children.

A 23-year-old black woman and her 6-year-old son, both with multiple granular cell tumors, are described herein. The mother and son both presented as children with multiple granular cell tumors. This is the first reported case of multiple lesions arising in childhood in successive generations. Only two other case studies of familial granular cell tumors have been reported, but in neither of these cases did multiple tumors present initially in both family members during childhood. A preponderance of multicentric lesions is reported in blacks. The tumors recurred locally in some of the sites where there were inadequate surgical margins, emphasizing the need for complete excision.

Postsplenectomy sepsis and other complications following staging laparotomy for Hodgkin's disease in childhood.

This is a survey of 234 pediatric patients in whom staging laparotomy/splenectomy was carried out (1975 to 1981) in the course of the Intergroup Hodgkin's Disease in Childhood Study (IHDCS). Relapse has occurred in 44 of these patients, and 12 have died, 7 secondary to extension of lymphoma, 2 with herpes or pneumocystis infections, 2 with leukemia, and 1 from an unrelated accident. During the period of surveillance (mean 5.5 yr), five episodes of bacterial sepsis (positive blood cultures) have occurred, including two due to Streptococcus pneumoniae; and three, to Hemophilus influenzae. The former occurred in the small group of patients in this series who had not received the prescribed pneumococcal vaccination. No fatalities were associated with these septic episodes. Intestinal obstruction secondary to adhesions (benign) occurred in eight patients and was managed without intestinal resection or mortality. One patient required operative release of an obstructed ureter following laparotomy, and one, oophorectomy for an infarcted (transposed) ovary.

Sodium homeostasis in infants with biliary drainage procedures.

We studied biliary excretion of sodium and chloride in 17 infants with external bile drainage through a "biliostomy" and describe four additional children who became ill from sodium depletion following external biliary drainage procedures for biliary tract anomalies. In the 17 infants, the mean +/- SD bile sodium concentration was 122 +/- 15 mEq/L. The mean +/- SD serum sodium concentration was low (132 +/- 7 mEq/L) (normal, 138 to 145 mEq/L). The mean +/- SD bile volume was 388 +/- 317 mL/day at one year following surgery (range, 40 to 1,000 mL/day). In the four children, clinical manifestations of sodium depletion (lethargy, anorexia, dehydration, and malnutrition) necessitated hospital admission. At that time, the serum sodium concentration ranged from 109 to 129 mEq/L, and the simultaneous urinary sodium concentration ranged from 0 to 5 mEq/L. Although dietary sodium was normal, biliary losses exceeded dietary intake, resulting in salt and water depletion despite renal conservation. Children with biliary drainage procedures are at risk for sodium depletion and should be monitored closely and supplemented accordingly until biliostomy closure is performed.

Management of experimental pneumothorax in weanling rabbits with the use of fibrin glue sclerosant.

Fibrin glue pleurodesis successfully sealed surgically created pneumothoraxes in 12 (92.3%) of 13 New Zealand white rabbits, an animal model chosen for its similarity to the thoracic configuration of the human neonate. All chest tubes were removed at 24 hours; there were no recurrences. Two rabbits, in whom human cryoprecipitate was used, died of an immunologically mediated pneumonitis. This reaction would not be expected in the human setting. Four months' follow-up revealed nearly total fibrin glue resorption. This "biodegradability" is well suited to the neonate, since alveolar barotrauma, not congenital emphysematous blebs, is the usual initiator of pneumothorax. Time-limited adhesions created by fibrin glue pleurodesis should be adequate for treatment of the acute event, while avoiding persistent pleural adhesions that could interfere with subsequent thoracic surgery or cause long-term deleterious effects on pulmonary function.

Traumatic liver laceration in the newborn: repair with fibrin glue.

A traumatic liver laceration in a 1,500 g premature infant was successfully treated with temporary hemostatic packing and subsequent fibrin glue repair. Literature review indicates this case to be the smallest survivor.

Use of low intensity roentgen ray imaging for determination of tube and catheter placement in the young primate: implication for use of the Lixiscope in the surgical neonate.

The new technology of low intensity roentgen ray imaging offers promise in rapid bedside location of tube and catheter placement in the surgical neonate. Using the Lixiscope we have been able to accurately detect in an animal model the exact location of various tubes and catheters used routinely in pre and postoperative neonatal care. Minimal training is required to be able to use the device. We think the Lixiscope offers detection of the positions of standard tubes and catheters with increased speed, as well as a reduction in the radiation exposure for patients and staff in the neonatal unit.