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.

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.

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.

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.

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.

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.

A case controlled comparison of open and laparoscopic splenectomy in children.

BACKGROUND: This case controlled study compares the efficacy, safety, and cost of laparoscopic splenectomy (LS) and open splenectomy (OS) for hematologic disorders in children. METHODS: The records of 82 consecutive children and adolescents undergoing splenectomy for hematologic disorders between August 1994 and September 1997 were reviewed retrospectively. RESULTS: Fifty patients underwent LS by a lateral approach and 32 underwent OS through a left subcostal incision. Mean age was 7.76 years for LS and 6.9 years for OS. Patient weights were similar: (LS, mean 30.5 kg; OS, mean 27.6 kg). Hematologic indications included hereditary spherocytosis in 43 children (LS 26, OS 17), sickle cell anemia with sequestration in 13 (LS 7, OS 6), immune thrombocytopenic purpura in 14 (LS 8, OS 6), and 12 with other disorders (LS 9, OS 3). Concomitant cholecystectomy was performed in 10 of 50 LS and 6 of 32 OS cases. Accessory spleens were identified in 8 of 32 (25%) OS and 9 of 50 (18%) LS cases (P = .578). No LS procedures required conversion to OS. The mean estimated blood loss was 54.4 mL for LS and 49.0 mL for OS (P = .233). LS required a longer operative time (115 vs 83 minutes, P = .002), less need for postoperative intravenous narcotic (51% vs 100%, P < .0001), lower total narcotic doses (0.239 vs 0.480 mg/kg morphine, P = .006), shorter length of hospital stay (1.4 +/- 0.97 vs 2.5 +/- 1.43 days, P = .0001), and lower average total hospital charges ($5713 vs $6564) than OS. There were no deaths or major complications in either group. CONCLUSIONS: Laparoscopic splenectomy is a safe and effective procedure in children with hematologic disorders resulting in longer operative times, less narcotic administration, shorter length of stay, and lower total hospital charge.

Juvenile polyp in esophageal colon interposition.

Esophageal polyps are rare in children, whereas colorectal juvenile polyps are common. An 11-year-old boy with an esophageal colonic interposition for an esophageal stricture secondary to caustic injury was found to have a polypoid mass in the interposed colon. Fiberoptic endoscopic removal with diathermy was performed. Histological examination confirmed it was a juvenile polyp. The occurrence of a polyp in a colon removed from its natural location and serving a different function suggests the possibility that an unknown factor produced by colonic cells play a pivotal role in its development.

Long-term follow-up after bowel resection for necrotizing enterocolitis: factors affecting outcome.

BACKGROUND: Necrotizing enterocolitis (NEC) is the most common surgical emergency among newborns and is associated with a high morbidity and mortality. This study evaluates the long-term survival of infants requiring surgical intervention for NEC and factors affecting outcome. METHODS: A retrospective review of infants requiring surgery for complications of NEC at a tertiary care, pediatric hospital over a 16-year period was performed. Patients were evaluated for early and late morbidity and mortality, length of intestinal resection, presence of the ileocecal valve (ICV), days of parenteral nutrition (PN), and growth. RESULTS: Two hundred forty-nine patients were included, with an average gestational age of 30 +/- 5 (+/- SD) weeks and birth weight of 1.50 +/- 0.89 kg. The surgical mortality rate was 45%, with survivors (137) being larger (P < .001) and older (P < .001) at time of birth than nonsurvivors. Mortality rates varied inversely with gestational age and birth weight. Surgical survivors had an average of 21 +/- 26 cm of intestinal length resected. The ileocecal valve was preserved in 45% of infants. Growth was similar between infants with or without an ICV. Stratification of length of intestine resected showed that infants with larger resections had greater requirements for parenteral nutrition, but this had no influence on long-term growth at follow-up. CONCLUSIONS: Survivors of NEC are characterized by greater gestational age, greater birth weight, and older postgestational age at surgery. Infants who underwent greater intestinal resections required longer periods of PN. The length of intestine resected or presence of the ileocecal valve had no overall bearing on long-term outcome.

Intestinal atresia and stenosis: a 25-year experience with 277 cases.

OBJECTIVE: To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. DESIGN: Retrospective case series. SETTING: Pediatric tertiary care teaching hospital. PATIENTS: A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). INTERVENTIONS: Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. MAIN OUTCOME MEASURES: Morbidity and early and late mortality. RESULTS: Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. CONCLUSIONS: Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.

Long-term outcome for infants and children with sacrococcygeal teratoma: a report from the Childrens Cancer Group.

BACKGROUND/PURPOSE: Sacrococcygeal teratomas (SCT) are a relatively uncommon tumor affecting neonates, infants, and children. This study was designed to determine the effect of therapy on the long-term outcome of neonates and children with sacrococcygeal teratomas (SCT). METHODS: The authors conducted a retrospective review of children with SCT treated at 15 Childrens Cancer Group institutions from 1972 to 1994. RESULTS: One hundred twenty-six children presented with SCT diagnosed prenatally (n = 32), at birth (n = 79), or later in infancy (n = 15). For neonates, complete resection was performed except in two babies with lethal associated defects. All others (n = 15) underwent resection at the age of diagnosis. Six had a sacral mass identified at birth but had delayed surgery (1.5 to 11 months) and of these, two were malignant. Resection was via sacral (n = 96) or abdominosacral (n = 28) approach. Histology showed mature teratoma (MT, 69%), immature teratoma (IT, 20%), or endodermal sinus tumor (EST, 11%) at presentation. Seven neonates (5.6%) died of perioperative complications, whereas the remaining 117 were available for long-term follow-up. Between 6 and 34 months postresection, recurrent disease developed in 9 of 80 MT patients (11%) followed-up for a mean of 5 years. Recurrent disease was MT (n = 2) and EST (n = 7). The recurrent EST patients were treated with adjuvant chemotherapy. Six are alive with mean follow-up of 114 months, whereas one with metastatic disease was lost to follow-up. Recurrence (MT) developed in only 1 of 24 IT patients, and all are alive and well at mean follow-up of 39 months. Patients presenting with EST (n = 13) underwent excision, with two dying from non-EST causes. Six EST patients received no chemotherapy, with two of the six (33%) experiencing recurrence within 11 months and both disease free after salvage chemotherapy. The remaining five EST patients received adjuvant chemotherapy; four are alive and one died of metastatic disease. Of the 18 EST patients followed-up after resection (presentation, 11, recurrent teratoma, 7), 16 (89%) are free of disease with a mean follow-up of 91 months. CONCLUSIONS: (1) Benign teratomas have a significant recurrence rate mandating close follow-up for more than 3 years. (2) Surgical resection alone is adequate therapy for nonmetastatic malignant tumors. (3) Survival for malignant lesions with metastases is excellent with modern chemotherapy.

Reoperation after Nissen fundoplication in children with gastroesophageal reflux: experience with 130 patients.

OBJECTIVE: The authors evaluate reoperation for recurrent gastroesophageal reflux (GER) after a failed Nissen fundoplication. SUMMARY BACKGROUND DATA: Nissen fundoplication is an accepted treatment for GER refractory to medical therapy. Wrap failure and recurrence of GER are noted in 8% to 12%. METHODS: Medical records of 130 children undergoing a second antireflux operation for recurrent GER from January 1985 to June 1996 retrospectively were reviewed. RESULTS: One hundred one patients (78%) were neurologically impaired (NI), 74 (57%) had chronic pulmonary disease, and 8 had esophageal atresia. Recurrent symptoms included vomiting (78%), growth failure (62%), choking-coughing-gagging (38%), and pneumonia (25%). Gastroesophageal reflux was confirmed by barium swallow, gastric scintigraphy, and endoscopy. Operative findings showed wrap breakdown (42%), wrap-hiatal hernia (30%), or both (21%). A second Nissen fundoplication was performed in 128 children. Complications included bowel obstruction (18), wound infection (10), pneumonia (6) and tight wrap (9). There were two postoperative (<30 days) deaths (1.5%). Of 124 patients observed long term, 89 (72%) remain symptom free. Eight were converted to tube feedings. Twenty-seven required a third fundoplication, and 19 (70%) were successful outcome. Two with repetitive wrap failure due to gastric atony underwent gastric resection and esophagojejunostomy. CONCLUSION: Nissen fundoplication was successful in 91% of patients. In 9% with wrap failure, a second Nissen fundoplication was successful in 72%. Reoperation is justified in properly selectedpatients. Conversion to jejunostomy feedings is suggested for neurologically impaired after two wrap failures and a partial wrap in those with esophageal atresia and severe esophageal dysmotility. Repeated wrap failure due to gastric atony requires gastric resection and esophagojejunostomy.

The "other side" of pediatric hernias: the role of laparoscopy.

The role of contralateral inguinal exploration in neonates, infants, and children presenting with unilateral hernias is controversial. Factors considered by surgeons include the patient's age, sex, and side of the clinically apparent hernia. The purpose of this study was to evaluate the role of diagnostic laparoscopy performed through the clinically apparent hernia sac to identify a contralateral patent processus vaginalis (CPPV) in children and limit contralateral exploration to CPPV-positive patients. One hundred neonates, infants, and children underwent laparoscopic evaluation for a CPPV through the ipsilateral hernia sac. There were 79 boys and 21 girls. Forty-eight of 100 (48%) had a CPPV identified, which was confirmed operatively. Thirty-one of 68 patients (46%) with a right-sided and 18 of 32 (56%) with a left-sided hernias had a CPPV (P = 0.39). Thirty-six of 56 (64%) patients younger than 6 months of age had a CPPV compared to 13 of 44 (30%) older than six months (P = 0.001). Fourteen of 21 (67%) girls had a CPPV compared to 35 of 79 (44%) boys (P = 0.087). Laparoscopy through the hernia sac is a safe and effective means of identifying the presence of a CPPV and avoiding unnecessary contralateral inguinal exploration. Infants (< 6 months) are much more likely to have a CPPV.