Risk factors for acute chest syndrome in children with sickle cell disease undergoing abdominal surgery.

BACKGROUND/PURPOSE: The reported incidence of acute chest syndrome (ACS) in children with sickle cell disease (SCD) is 15% to 20%. Our current objective was to assess risk factors and morbidity associated with ACS. METHODS: The authors reviewed the outcome of children with SCD undergoing abdominal surgery over a 10-year period. RESULTS: From 1991 to 2003, 60 children underwent laparoscopic cholecystectomy (LC; n = 29), laparoscopic splenectomy (LS; n = 28), or both (LB; n = 3). Mean age was 8.6 (0.7 to 20) years, and 35 (58%) were boys. Fifty-four (90%) had a preoperative hemoglobin greater than 10 g/dL, but only 22 (37%) received routine oxygen after surgery. No surgery was converted to an open procedure. Four children (6.6%), all of whom underwent either LS or LB, had ACS associated with an increased length of stay (7.4 +/- 2.4 days) but no mortality. Factors associated with the development of ACS were age (3.0 +/- 1.7 v 9.4 +/- 5.7 years; P =.03), weight (12.1 +/- 3.0 v 32.6 +/- 18.2 kg; P =.04), operative blood loss (3.2 +/- 0.5 v 1.4 +/- 1.2 mL/kg; P =.03), and final temperature in the operating room (OR; 36.2 +/- 0.4 v 37.6 +/- 0.4 degrees C; P =.01). ACS was not significantly related to duration of surgery, OR fluids, or oxygen usage. CONCLUSIONS: Younger children with greater blood and heat loss during surgery appear more prone to ACS. Splenectomy also seems to increase the risk of ACS. The authors' current incidence (6.6%) of ACS in children with SCD undergoing abdominal surgery is much lower than previously reported. This may be explained by the aggressive use of preoperative blood transfusion or more routine use of laparoscopy.

Outcome and staging evaluation in malignant germ cell tumors of the ovary in children and adolescents: an intergroup study.

PURPOSE: The aim of this study was to perform an evaluation of outcome and the role of surgical staging components in malignant germ cell tumors (GCT) of the ovary in children and adolescents. METHODS: From 1990 to 1996, 2 intergroup trials for malignant GCT were undertaken by Pediatric Oncology Group (POG) and Children's Cancer Study Group (CCG). Stage I-II patients were treated with surgical resection and 4 cycles of standard dose cisplatin (100 mg/m2/cycle), etoposide, and bleomycin (PEB) chemotherapy. Stage III-IV patients were treated with surgical resection and randomly assigned to chemotherapy with PEB or high-dose cisplatin (200 mg/m2/cycle) with etoposide and bleomycin (HDPEB). Patients unresectable at diagnosis had second-look operation after 4 cycles of chemotherapy if residual tumor was seen on imaging studies. IRB approval of the protocols was obtained at each participating institution. An analysis of outcome data, operative notes, and pathology reports in girls with ovarian primary site was done for this report. RESULTS: There were 131 patients with ovarian primary tumors of 515 entered on these studies. Mean age was 11.9 years (range, 1.4 to 20 years). Six-year survival rate was stage, I 95.1%; stage II, 93.8%; stage III, 98.3%; stage IV, 93.3%. In only 3 of 131 patients were surgical guidelines followed completely. Surgical omissions resulting in protocol noncompliance resulted from failure to biopsy bilateral nodes (97%), no omentectomy (36%), no peritoneal cytology (21%), no contralateral ovary biopsy (59%). More aggressive procedure than recommended by guidelines included total hysterectomy and bilateral salpingo-oophorectomy in 6 patients and retroperitoneal node dissection in 10 patients. Correlation of gross operative findings with pathology results was carried out for ascites, lymph nodes, implants, omentum, and contralateral ovary. CONCLUSIONS: Pediatric ovarian malignant GCT (stages I-IV) have excellent survival with conservative surgical resection and platinum-based chemotherapy. Survival appears to have been unaffected by deviations from surgical guidelines. New surgical guidelines are proposed based on correlation of gross findings, histology, and outcome in these intergroup trials.

Long-term analysis of children with esophageal atresia and tracheoesophageal fistula.

BACKGROUND/PURPOSE: For children with esophageal atresia (EA) or tracheoesophageal fistula (TEF), the first years of life can be associated with many problems. Little is known about the long-term function of children who underwent repair as neonates. This study evaluates outcome and late sequelae of children with EA/TEF. METHODS: Medical records of infants with esophageal anomalies (May 1972 through December 1990) were reviewed. Study parameters included demographics, dysphagia, frequent respiratory infections (> 3/yr), gastroesophageal reflux disease (GERD), frequent choking, leak, stricture, and developmental delays (weight, height < 25%, < 5%, respectively). RESULTS: Over 224 months, 69 infants (37 boys, 32 girls) were identified: type A, 10 infants; type B, 1; type C, 53; type D, 4; type E, 1. Mean follow-up was 125 months. During the first 5 years of follow-up, dysphagia (45%), respiratory infections (29%), and GERD (48%) were common as were growth delays. These problems improved as the children matured. CONCLUSIONS: Children with esophageal anomalies face many difficulties during initial repair and frequently encounter problems years later. Support groups can foster child development and alleviate parent isolationism. Despite growth retardation, esophageal motility disorders, and frequent respiratory infections, children with EA/TEF continue to have a favorable long-term outcome.

Malignant retroperitoneal and abdominal germ cell tumors: an intergroup study.

BACKGROUND/PURPOSE: This randomized study examined survival (S) and event-free survival (EFS) rates using high-or standard-dose cisplatin-based combination chemotherapy and surgical resection for this subset of germ cell tumors. METHODS: Twenty-six of 317 patients enrolled on the POG 9049/COG 8882 intergroup study for malignant germ cell tumors had abdomen or retroperitoneum as the primary site. Twenty-five of 26 were eligible for inclusion (n = 25). Patients had biopsy or resection at diagnosis and randomization to chemotherapy including etoposide, bleomycin, and either standard-dose (PEB) or high-dose cisplatin (HDPEB). In patients with initial biopsy, delayed resection was planned. RESULTS: Median age was 26 months. There were 14 girls and 11 boys. There were 3 stage I to II, 5 stage III, and 17 stage IV patients. Surgical management included primary resection in 5, resection after chemotherapy in 13, and biopsy or partial resection in 7 patients. Overall 6-year EFS rate was 82.8% +/- 10.9%, and 6-year survival rate was 87.6% +/- 9.3%. By group, 6-year survival rate was 90.0% +/- 11.6% for PEB and 85.7 +/- 14.5% for HDPEB. Deaths include one from sepsis, one from malignant tumor progression, and one from bulky disease caused by benign components despite response of the malignant elements to chemotherapy. CONCLUSIONS: Malignant germ cell tumors arising in the abdomen and retroperitoneum have an excellent prognosis despite advanced stage in most children. Aggressive resection need not be undertaken at diagnosis, but a concerted attempt at complete surgical removal after chemotherapy is important to distinguish viable tumor from necrotic tumor or benign elements that will not benefit from further chemotherapy.

Portal venous air: the poor prognosis persists.

BACKGROUND/PURPOSE: The prognostic importance of portal vein air (PVA) in babies with necrotizing enterocolitis (NEC) has been controversial. This study compares the outcome in babies with NEC and PVA treated surgically versus those with medical management. METHODS: Forty neonates in the neonatal intensive care unit (NICU; 1995 through 1999) had (PVA) during their hospitalization. Babies were analyzed for gestational age (GA), birth weight (BW), and survival after operative versus medical management. RESULTS: The average GA was 26 weeks, average BW was 1,173 g. Twenty-three patients (57.5%) tolerated full feedings and 8 (20%) partial feedings at diagnosis. All 40 babies required intubation at birth with 23 (57.5%) requiring reintubation with onset of PVA. In all cases, PVA was present within 24 hours of onset of abdominal distension, feeding intolerance, or heme-positive stools. Two cases of PVA "resolved" only to recur later in the patients' courses. Thirty-two patients (80%) manifested pneumatosis intestinalis on abdominal radiographs, and 8 (20%) had perforations. Acidosis was present in 25 (63%) patients, and vasopressor support (dopamine) was required in 15 (38%), with 2 patients requiring support only preoperatively. Initial management consisted of bowel rest, fluid resuscitation, orogastric decompression, and broad-spectrum antibiotics. Operation was performed in 31 (78%). Seventeen underwent resection with ostomy formation with 6 deaths and 11 survivors. Four underwent resection using the clip and drop back method, with one death and 3 requiring an ostomy at second look laparotomy. Ten had NEC totalis and closure of the abdomen only. Overall operative mortality rate was 17 of 31 (54%). Nine seemingly stable patients were treated nonoperatively. Six had progressed disease and died before salvage laparotomy could be performed, whereas 3 (33%) survived without further therapy. CONCLUSIONS: PVA has been a relative indication for operation. This view has been challenged by the survival of some patients without laparotomy. Although nonoperative therapy seems appealing in hemodynamically stable patients without acidosis, our data confirm the poor prognosis of infants with PVA and NEC.

The utility of lung biopsy in recipients of stem cell transplantation.

BACKGROUND/PURPOSE: Pulmonary infiltrates in recipients of stem cell transplantation often present as diagnostic dilemmas. Although lung biopsy may establish the diagnosis of parenchymal disease, it remains unclear whether such a procedure results in a significant change in the patient's treatment and outcome. This study evaluates the efficacy of lung biopsy in recipients of stem cell transplantation. METHODS: The medical records of 15 stem cell transplant recipients who underwent 18 lung biopsies were reviewed. The indications for stem cell transplantation were leukemia in 10 patients, lymphoma in 2, histiocytosis in 1, neuroblastoma in 1, and Ewing's sarcoma in 1. The results of the lung biopsies were correlated to the clinical management and outcomes. RESULTS: The overall mortality rate was 67% (10 patients). Eight of the 9 patients who required mechanical ventilatory support at the time of lung biopsy died. The pathologic diagnoses were pneumonitis in 6 biopsies, fibrosis in 6, brochiolitis obliterans organizing pneumonia in 3, hemorrhage in 2, and infarction in 1. Therapy was changed in 1 patient who improved after a course of steroids for bronchiolitis obliterans organizing pneumonia. Lung biopsy cultures were positive in 6 patients but rarely resulted in changes in antibiotic therapy. CONCLUSIONS: Results of very few lung biopsies performed in stem cell transplant recipients redirected therapy. Furthermore, the ultimate outcome of these patients were not improved by the results of lung biopsies.

Pectus excavatum repair: experience with standard and minimal invasive techniques.

BACKGROUND/PURPOSE: The Nuss procedure is a minimally invasive pectus repair that helps avoid cartilage resection and osteotomy. This report compares outcomes in patients undergoing a standard pectus repair to patients with the Nuss procedure. METHODS: One hundred three children (ages 5 to 20 years) with severe pectus excavatum underwent repair. Patients were evaluated for type of repair performed, associated anomalies, cardiopulmonary function, operating time, analgesia requirements, complications, length of hospital stay, hospital and operative charges, and cosmetic result. Statistical analysis was performed using the Mann-Whitney rank sum test. RESULTS: There were 68 patients (average age, 12.6 years) in the standard group and 35, (average age, 9.5 years) in the Nuss group. Associated anomalies were found in 6 standard group and 2 Nuss group patients. Average operating time in Nuss was 3.3 hours and in open procedures, 4.7 hours. Postoperative complications occurred in 13 (20%) standard repair patients and 15 (43%) after the Nuss. In the standard group, 14 patients received intrathecal and 3 received epidural analgesia, while 35 (52%) required an intravenous patient-controlled anesthetic device (PCA; average, 1.8 days). In the Nuss group, 25 patients (71%) received epidural anesthesia (average, 3 days), and 31 (89%) utilized PCA (average 3.8 days). Four (6%) standard patients and 8 Nuss patients (29%) required reoperation. Length of stay averaged 4.0 days (range 2 to 30) in the standard group and 4.8 days (range, 2 to 11) in the Nuss group. Average operating room charge was $8,325 in the standard group and $9,480 in the Nuss group. Average hospital charge was $4,137 for the standard patient and $4,044 for the Nuss group. Analgesic requirements and length of hospital stay were increased (P <.05). The complication rate and operative and hospital charges were similar between groups. CONCLUSIONS: Although the Nuss repair is associated with shorter operating time, smaller incisions, and less dissection, early results indicate few other advantages. Drawbacks of the Nuss procedure include high complication and reoperation rates and lack of efficacy in older teenagers and those with connective tissue disorders. Long-term follow-up will be necessary to determine final cosmetic and functional outcomes and define the overall risks and benefits of this procedure as compared with the standard technique.

The effect of cisplatin dose and surgical resection in children with malignant germ cell tumors at the sacrococcygeal region: a pediatric intergroup trial (POG 9049/CCG 8882).

PURPOSE: This study was designed to evaluate (1) the efficacy of standard or high-dose cisplatin with etoposide and bleomycin and (2) the role of surgical resection in infants and children with malignant germ cell tumors of the sacrococcygeal region (SCT). METHODS: Seventy-four of 317 children presenting to Pediatric Oncology Group (POG)/Children's Cancer Group (CCG) institutions from 1990 through 1996 with malignant germ cell tumors had malignant SCT. There were 62 girls and 12 boys with a median age of 21 months (range, 3 days to 37 months) and median serum alpha-fetoprotein of 35,500 ng/mL. Twelve had undergone resection of a benign SCT as a newborn. Forty-four (59%) had evidence of metastatic disease at time of diagnosis. Presentation by type (Altman classification) was I, 0; II, 2; III, 30; and IV, 42. The initial procedure was biopsy in 45 and resection in 29. Patients were assigned randomly to receive 4 cycles of chemotherapy with etoposide (E) and bleomycin (B) and either high-dose cisplatin (200 mg/m(2) per cycle; HDP) or standard dose cisplatin (100 mg/m(2) per cycle; P). After completion of chemotherapy, 42 of 45 initially treated with biopsy underwent resection. RESULTS: Overall 4-year survival rate is 90% (SE = 4%) and 4-year event-free survival (EFS) is 84% (SE = 6%). Event-free survival data for subgroups of interest are as follows: 4-yr EFS% (SE) P Values Mets (44) 88 (6).48 No Mets (30) 80 (8) HDP EB (37) 89 (6).21 P EB (37) 78 (7) Initial Resection (29) 90 (7).50 Delayed Resection (42) 83 (7) Complete Resection (49) 90 (5).19 CR/PR Partial Resection (22) 77 (10) Biopsy Only (3) 33 (27).005 (3 way) CONCLUSIONS: (1) The current survival rate of malignant sacrococcygeal tumors is excellent even with metastases. (2) Delayed surgical resection is not associated with an adverse outcome. (3) In this subset the treatment comparison was inconclusive however, followed the trend in the overall study of more than 300 children in which the high-dose cisplatin group had superior EFS (P<.05).

Malignant mediastinal germ cell tumors: an intergroup study.

PURPOSE: This review was conducted to determine clinical characteristics and response to therapy in this rare pediatric neoplasm. METHODS: An intergroup Pediatric Oncology Group (POG) 9049/Children's Cancer Study Group (CCG) 8882 randomized trial was conducted to evaluate response rate and survival with chemotherapy using etoposide, bleomycin, and high or standard dose cisplatin for high-risk malignant germ cell tumors at extragonadal sites. For this review, a secondary analysis of clinical and operative findings in patients with primary site in the mediastinum was carried out. RESULTS: Of the 38 children with malignant mediastinal germ cell tumors (MGCT), 36 had sufficient data to be included in this review. Thirty-four tumors were anterior mediastinal, 2 were intrapericardial. Younger patients had respiratory complaints; older patients had chest pain, precocious puberty, or facial fullness. Yolk sac tumor was the only malignant element in girls. Boys had yolk sac tumor in 7, germinoma in 3, choriocarcinoma in 2, and mixed malignant elements in 15. Benign teratoma elements coexisted in 22 patients. Four patients had biopsy and chemotherapy without tumor resection, and only 1 survived. Fourteen patients had resection at diagnosis followed by chemotherapy with 12 survivors. Eighteen patients had biopsy followed by chemotherapy and postchemotherapy tumor resection with 13 survivors. Tumor size in response to chemotherapy for these 18 patients was stable or increased in 6, and decreased in 12 (mean decrease of 57% in greatest dimension). Overall, 26 of 36 patients survived, with a 4-year patient survival rate of 71%+/-10%, and a 4-year event-free survival rate of 69%+/-10%. Ten patients died: 5 of tumor (all boys > or =15 yr), 2 of sepsis, and 3 of second malignancy. CONCLUSIONS: Malignant MGCT is a complex tumor of varied histology with frequent coexistence of benign elements. Lesions often have incomplete regression with chemotherapy alone. Tumor resection may be undertaken at diagnosis or after attempted shrinkage with chemotherapy. Aggressive attempt at complete tumor resection should be offered to all patients even if bulky tumor persists after induction chemotherapy with expectation of a significant salvage rate. Boys > or =15 years may be a high-risk subgroup for mortality from tumor progression.

Gastroschisis: a plea for risk categorization.

BACKGROUND: The incidence of gastroschisis has increased in the past decade. A differing clinical course between "complex" (those with atresias, perforation, or stenosis) and "simple" cases has prompted a review of risk assessment factors. METHODS: A retrospective chart review was conducted of 103 infants with gastroschisis over 5 years (1992 to 1997). RESULTS: Of 103 infants, 52 were girls and 51 were boys. Seventy-one infants (69%) had a simple defect, and 32 (31%) were complex. The simple group had an average estimated gestational age of 37.5 weeks (range, 26 to 40), and a birth weight of 3.0 kg (range, 1.7 to 3.8). A total of 71% underwent primary repair, whereas 29% required a silo. Mechanical ventilation averaged 6.8 days (range, 1 to 19). Enteral feedings were initiated at 15 days (range, 3 to 27) with full enteral intake achieved by 22.4 days (range, 5 to 40). Three infants required home parenteral nutrition. The average length of stay (LOS) was 26.4 days (range, 10 to 57). Complications occurred in 26 infants (36%), including intravenous catheter sepsis (n = 15), pneumatosis (n = 2), pneumonia (n = 1), bowel obstruction (n = 7), wound infection (n = 5), and SVC thrombosis (n = 1). Survival rate was 100%. Thirty-two infants had complex defects; 27 patients had atresias, stenosis, or perforations; and 3 had volvulus. The average estimated gestational age was 34 weeks (range, 26 to 38), and birth weight was 2.0 kg (range, 0.9 to 4.0). Primary repair was performed in 65% and silo placement in 35%. Mechanical ventilation was required for 22.3 days (range, 2 to 14). Enteral feedings were initiated at 22.5 days (range, 6 to 56) with full feedings achieved at 50 days (range, 21 to 113). Fourteen infants required home total parenteral nutrition (TPN). The LOS was 85.4 days (range, 24 to 270). A total of 47 complications occurred in the complex group including catheter sepsis (n = 15), short bowel syndrome (n = 7), pneumatosis (n = 3), bowel obstruction (n = 4), pneumonia (n = 2), superior vena cava thrombosis (n = 1), enterocutaneous fistula (n = 1), and 9 deaths (28% mortality rate). CONCLUSIONS: These data indicate gastroschisis can be divided into low-risk (simple) and high-risk (complex) categories. These 2 groups have significant differences in clinical behavior, postsurgical complications, LOS, and mortality rate (0 v 28%). Although the overall survival rate was 91% (94 of 103), parents, referring physicians, and insurers must be made aware of the impact of risk categorization on the estimated cost, LOS, and outcomes.

Is the grass greener? Early results of the Nuss procedure.

BACKGROUND/PURPOSE: Minimal access surgery (MIS, Nuss Procedure) is gaining acceptance rapidly as the preferred method for pectus excavatum repair. This shift in operative management has followed a single institution's evaluation of the procedure. This report describes an additional experience with the Nuss procedure. METHODS: Twenty-one patients with pectus excavatum underwent repair by the Nuss Procedure. The patients ranged in age from 5 to 15 years (average, 8.2 years). There were 19 boys and 2 girls. RESULTS: In 1 patient (age 5 years) the MIS procedure was aborted because of persistence of chest wall asymmetry. The other 20 patients had completion of their procedure without intraoperative complication. The operating times ranged from 45 to 90 minutes; however, there was an additional anesthetic set-up time (average, 45 minutes). All cases utilized a single support bar (11 to 17 inches). Patients underwent extubation in the operating room and were admitted to a ward bed with an epidural catheter in place for pain control and received intravenous analgesia. The hospital stay ranged from 4 to 11 days and averaged 4.9 days. Early postoperative complications included ileus (n = 1), bilateral pleural effusion (n = 2), atelectasis (n = 1), fungal dermatitis (n = 1), pneumothorax (n = 1), and flipped pectus bar (n = 2). Delayed complications included flipped pectus bar (n = 2), marked pectus carinatum requiring bar removal (n = 1), mild carinatum (n = 1), mild bar deviation (n = 1), progressive chest wall asymmetry (n = 3) with 1 requiring bar removal and open pectus repair, pleural effusion (n = 1), and chronic persistent pain requiring bar removal (n = 1). The length of follow-up is 3 to 20 months with an average of 12.3 months. CONCLUSIONS: The Nuss Procedure is quick, minimally invasive, and a technically easy method to learn; however, our data indicate there is a significant learning curve. Although previous reports suggest that few complications occur, we believe further assessment of patient selection regarding age, presence of connective tissue disorder, and severe chest wall asymmetry are still needed. Long-term follow-up also will be required to assure both health professionals and the public that this is the procedure of choice for patients with pectus excavatum.

Efficacy of primary and secondary video-assisted thoracic surgery in children.

BACKGROUND/PURPOSE: Video-assisted thoracic surgery (VATS) is used commonly for diagnostic and therapeutic procedures in children. The purpose of this study was to determine the accuracy, efficacy, and complications associated with primary and secondary VATS in children. METHODS: Eighty-seven infants, children, and adolescents underwent 104 VATS procedures between March 1993 and April 1999. There were 47 boys and 40 girls with an age range of 6 months to 19 years. VATS was performed for excision of pulmonary nodule (n = 51), biopsy of infiltrate (n = 14), excision or biopsy mediastinal mass (n = 12), decortication of empyema (n = 16), pleurodesis and bleb excision for pneumothorax (n = 5), pleurolysis for P32 administration (n = 3), esophageal myotomy (n = 2), and thymectomy (n = 1). In 6 children a contralateral thoracic procedure was performed along with VATS (3 VATS, 3 thoracotomies). Secondary VATS was performed in 20 after prior thoracic procedures. RESULTS: VATS was efficacious for diagnostic or therapeutic purposes in 93 cases. Overall, 11 (11%) VATS required conversion to open thoracotomy. Average length of thoracostomy tube drainage (CTD) was 2.2 days, and average length of stay (LOS) was 3.7 days. Complications included prolonged air leak (> 7 days) in 3 (2 empyema, 1 nodule). Two children with malignancy and pulmonary infiltrates died within 30 days of progressive respiratory failure. There were no bleeding complications or deaths related to VATS. CONCLUSIONS: VATS is a safe and effective primary and secondary procedure in children resulting in a short length of CTD and LOS. Duration of CTD and LOS are prolonged if empyema is associated with a bronchopleural fistula, and VATS may not be of value in this setting.

Pediatric germ cell tumors.

Malignant germ cell tumors are relatively uncommon, accounting for approximately 3% of all childhood malignancies. Occurring with an incidence of approximately 4 per million among children less than 15 years of age, they account for approximately 225 new cases per year in the United States. Germ cell tumors occur in both gonadal and extragonadal sites, with extragonadal and testicular tumors predominating in children less than 3 years of age and with the gonads being the main location of tumors during and after puberty. They occur more frequently in girls than boys. Germ cell tumors are interesting for several reasons: (1) abnormal migration of primordial germ cells account for many of the childhood germ cell tumors; (2) markers exist to allow evaluation of the extent of resection and the development of recurrence for many of the tumors; and (3) the introduction of platinum-based chemotherapy has markedly improved the survival rate for germ cell tumors, as well as the salvage rate for recurrent or metastatic disease.

Complete surgical excision is effective treatment for children with immature teratomas with or without malignant elements: A Pediatric Oncology Group/Children's Cancer Group Intergroup Study.

PURPOSE: To determine whether the 3-year event-free survival (EFS) of children with completely resected immature teratomas is greater than 85%. PATIENTS AND METHODS: Patients with immature teratomas treated at Pediatric Oncology Group or Children's Cancer Group institutions were eligible. Pathology was centrally reviewed to confirm diagnosis and tumor grading. Follow-up included physical examination, measurement of tumor markers (alpha fetoprotein and human chorionic gonadotropin), and imaging. All patients were monitored for events, defined as tumor recurrence, second malignancy, or death. RESULTS: Seventy-three children (median age, 7.8 years) with extracranial immature teratomas were enrolled on study. Primary tumor sites included ovarian (n = 44), testicular (n = 7), and extragonadal (n = 22). However, on review, 23 patients had foci of yolk sac tumor (n = 21) or primitive neuroectodermal tumor (n = 2), whereas 50 had pure immature teratomas. Twenty-five patients had increased alpha fetoprotein (n = 18), human chorionic gonadotropin (n = 5), or both (n = 2); nine had foci of yolk sac tumor on review. Pathology review identified 23 patients with grade 1, 29 with grade 2, and 21 with grade 3 immature teratomas. With a median follow-up of 35 months, the overall 3-year EFS was 93% (95% confidence interval, 86% to 98%), with 3-year EFS of 97.8%, 100%, and 80% for patients with ovarian, testicular, and extragonadal tumors, respectively. Only four of 23 patients with immature teratoma and malignant foci developed recurrence, suggesting that surgical resection followed by close observation are effective treatment. Overall, five patients had disease recurrence 4 to 7 months from diagnosis, and four (80%) are disease free after platinum-based therapy. The fifth patient has residual tumor after cisplatin, etoposide, and bleomycin treatment requiring further therapy. CONCLUSION: Surgical excision is safe and effective treatment for 80% to 100% of children with immature teratoma.

Surgical resection alone is effective treatment for ovarian immature teratoma in children and adolescents: a report of the pediatric oncology group and the children's cancer group.

OBJECTIVE: In both adult women and children the potential for malignant recurrence from ovarian immature teratoma has prompted the standard use of chemotherapy after complete resection of the primary tumor. The efficacy of postoperative chemotherapy in children and adolescents with ovarian immature teratoma, however, has not been established. A pediatric intergroup trial (INT 0106) was designed to determine the need for postoperative chemotherapy in patients with ovarian immature teratoma after management with surgical resection only. STUDY DESIGN: Between 1990 and 1995, 44 patients with completely resected ovarian immature tumor and without postoperative chemotherapy, who were able to undergo assessment, were accrued. Tumor tissue was evaluated by central pathology review to confirm diagnosis and determine tumor grading of immature neural elements. Patients were followed carefully for recurrence of disease with appropriate diagnostic imaging and serum marker studies. RESULTS: Thirty-one patients had pure ovarian immature teratoma with a tumor grade of 1 (n = 17), 2 (n = 12), or 3 (n = 2). Age at diagnosis ranged between 1.5 and 15 years (median, 10). Of the 29 patients studied, the serum alpha-fetoprotein level was elevated in 10 (34%); the median level was 25 ng/ml. Thirteen patients had ovarian immature teratoma plus microscopic foci of yolk sac tumor. Tumor grade was 1, 2, or 3 in 1, 6, and 6 patients, respectively. Age ranged between 6 and 20 years (median, 12). In the 12 patients evaluated for serum alpha-fetoprotein, 10 (83%) had elevated levels; the median level was 262 ng/ml. The 4-year event-free and overall survival for the ovarian immature teratoma group and for the ovarian immature teratoma plus yolk sac tumor group was 97.7% (95% confidence interval, 84.9%-99.7%) and 100%, respectively. The only yolk sac tumor relapse occurred in a child with ovarian immature teratoma and yolk sac tumor who was then treated with chemotherapy and is alive and free of disease 57 months after recurrence. CONCLUSION: The results of this study suggest that surgery alone is curative for most children and adolescents with resected ovarian immature teratoma of any grade, even when elevated levels of serum alpha-fetoprotein or microscopic foci of yolk sac tumor are present. This experience strongly supports avoiding the long-term effects of chemotherapy in most children with ovarian immature teratoma by reserving postoperative therapy for cases with relapse.

Cryosurgical effects on growing vessels.

Cryosurgical treatment of unresectable hepatic malignancies has proven beneficial in adults. Concerns regarding its use in children include the effect on growth and the risk of injury to adjacent structures. To test the effect of cryoablation on adjacent vascular structures in a growing animal, liquid nitrogen cryoablation was performed on a juvenile murine model. Sprague Dawley rats underwent double freeze-thaw cryoablation of the abdominal aorta with interposed liver tissue. Serial sacrifices were performed over 120 days. Comparisons were made with sham-operated controls. Overall, animal growth paralleled that of sham controls through all time points. Gross examination of aortic diameter also showed similar growth in vessel size between the groups. Histologic analysis demonstrated injury after cryoablation with smooth muscle cell vacuolization, followed by cell death. Aortic media layer collapse resulted from cellular loss, however, elastin fiber composition was maintained. Aortic patency was preserved despite evidence of cellular injury and aortic wall remodeling. An associated thermal sink effect on the opposing wall was identified. After cryoablation adjacent to the abdominal aorta in adolescent rats, vascular patency is maintained and animal growth and structural function is preserved, despite cellular injury and wall compression. These observations suggest that cryoablation may be a useful treatment adjunct in young subjects.

The use of pleuroperitoneal shunts in the management of persistent chylothorax in infants.

BACKGROUND/PURPOSE: The development of chylothorax is a serious and often life-threatening clinical entity that may cause profound respiratory, nutritional, and immunologic complications and has become increasingly common in recent years. Optimal management of this problem has not been well defined because medical therapy has a significant failure rate. Surgical treatment of complicated chylothorax has become a mainstay of care. METHODS: Over the last 36 months, seven infants had a pleuroperitoneal shunt placed for the management of refractory chylothorax. Ages ranged from 10 to 66 days with a weight between 1,000 to 4,850 g. Five of the seven infants were ventilator dependent. The etiologies were congenital in four and acquired in three with one related to a cardiothoracic procedure, one related to superior vena caval thrombosis, and one postoperative diaphragmatic hernia repair with superior vena caval thrombosis. Associated conditions included a left congenital diaphragmatic hernia, asplenia, isolated renal agenesis, bronchopulmonary dysplasia, and a patent ductus arteriosus. Each patient was unresponsive to thoracentesis, tube thoracostomy, and dietary manipulation with preoperative volume of chest tube output ranging from 50 to 162 cc/kg/d. The duration of preoperative therapy in congenital occurrences ranged from 10 to 46 days (average, 22 days). A Denver double-valved shunt system was used and catheters were implanted under general anesthesia. Manual pumping was required postoperatively on an hourly basis. RESULTS: All seven patients had excellent results with the elimination of the chylothorax and resolution of symptoms. There were two complications. Shunt survival rate was six of seven (86%). Shunt removal ranged from 24 to 79 days (average, 44 days). Patient survival rate was five of seven (71%) with one infant dying of progressive pulmonary disease and one infant dying from viral sepsis; both had functioning shunts. One patient remains ventilator dependent secondary to chronic lung disease from prematurity. CONCLUSIONS: Pleuroperitoneal shunting is safe, simple, and an effective treatment of chylothorax in infants despite their size, age, or degree of prematurity.

Pediatric germ cell tumors.

Germ cell tumors are relatively rare tumors in childhood which often present with very large tumors in both gonadal and extragonadal locations. Extragonadal tumors are more common in neonates and infants, whereas gonadal sites predominate in childhood and adolescence. Management consists of surgical resection for localized disease, chemotherapy for residual or metastatic disease, and neoadjuvant chemotherapy and delayed surgical excision for unresectable lesions. The survival for children with germ cell tumors has improved significantly over the past 2 decades with the development of platinum-based chemotherapy. Mature and immature teratomas at any site, and completely resected (Stage I) malignant gonadal and extragonadal tumors, are treated with surgical excision and observation. Malignant lesions with microscopic residual, lymph node disease, or metastatic disease receive platinum-based chemotherapy. Current survival for low-stage (Stages I and II) gonadal sites approaches 100% and survival for higher stage (Stages III and IV) gonadal sites is approximately 95%. Survival for extragonadal lesions is approximately 90% for Stages I and II and 75% for Stages III and IV.

Laparoscopic splenectomy.

Splenectomy in childhood usually is required for hematologic disorders such as hereditary spherocytosis, immune thrombocytopenic purpura, or sickle cell disease. The laparoscopic approach for splenectomy in children has been developed and advanced over the past 5 years. New techniques and equipment allow the procedure to be performed safely and effectively. This article details the technique and reports the results of 63 infants, children, and adolescents who underwent laparoscopic splenectomy for hematologic disorders. Although operating times were longer than for open splenectomy, the narcotic requirement was less and length of stay was shorter.