Eitan Gross

Gastric Transposition for Esophageal Replacement in Children: Experience With 41 Consecutive Cases With Special Emphasis on Esophageal Atresia

ObjectiveTo evaluate the authors’ experience with gastric transposition as a method of esophageal replacement in children with congenital or acquired abnormalities of the esophagus. Summary Background DataEsophageal replacement in children is almost always done for benign disease and thus requires a conduit that will last more than 70 years. The organ most commonly used in the past has been colon; however, most series have been fraught with major complications and conduit loss. For these reasons, in 1985 the authors switched from using colon interpositions to gastric transpositions for esophageal replacement in infants and children. MethodsThe authors retrospectively reviewed the records of 41 patients with the diagnoses of esophageal atresia (n = 26), corrosive injury (n = 8), leiomyomatosis (n = 5), and refractory gastroesophageal reflux (n = 2) who underwent gastric transposition for esophageal replacement. ResultsMean ± SE age at the time of gastric transposition was 3.3 ± 0.6 years. All but two transpositions were performed through the posterior mediastinum without mortality or loss of the gastric conduit despite previous surgery on the gastric fundus in 8 (20%), previous esophageal operations in 15 (37%), and previous esophageal perforations in 6 (15%) patients. Complications included esophagogastric anastomotic leak (n = 15, 36%), which uniformly resolved without intervention; stricture formation (n = 20, 49%), all of which no longer require dilation; and feeding intolerance necessitating jejunal feeding (n = 8, 20%) due to delayed gastric emptying (n = 3), feeding aversion related to the underlying anomaly (n = 1), or severe neurological impairment (n = 4). No redo anastomoses were required. ConclusionsGastric transposition reestablishes effective gastrointestinal continuity with few complications. Oral feeding and appropriate weight gain are achieved in most children. Therefore, gastric transposition is an appropriate alternative for esophageal replacement in infants and children.

Analysis of the Costs of Surgery for Hirschsprung's Disease: One-Stage Laparoscopic Pull-Through Versus Two-Stage Duh.amel Procedure

The purpose of this report is to evaluate the cost-effectiveness of a single-stage laparoscopic pull-through for Hirschsprungs disease compared to the traditional two-stage Duhamel procedure. In this series of 33 children, the length of hospitalization (2.5 ± 3.5 vs 10.6 ± 3.9, p<0.0 1), cost (19,088 ± 13,075 vs 34,110 ± 19,443, p<0.05), and complications were all significantly less with the laparoscopic assisted pull-through compared to the more traditional open approach.

Complications of pelvic exenteration in children who have genitourinary rhabdomyosarcoma

BACKGROUNDnRhabdomyosarcoma is the most frequently encountered soft tissue sarcoma in children younger than 15 years of age.nnnMETHODSnThe authors reviewed the early and long-term morbidity rate and clinical outcome of pelvic exenteration for genitourinary rhabdomyosarcoma (GU-RMS) in children. Of 43 patients with this diagnosis who were seen at St Jude Childrens Research Hospital between 1963 and 1994, 17 underwent pelvic exenteration. These 13 boys and four girls ranged in age from 6 months to 15 years (median, 3.5 years). Their primary sites included prostate (n = 12), bladder (n = 4), and uterus (n = 1). All patients received multiagent chemotherapy and either preoperative or postoperative radiation therapy. Anterior exenteration was performed in 15 patients and total exenteration in two. The complications were classified as early or late (occurring within or after 30 days from the date of surgery) and as major or minor (depending on the risk of death or physical or functional disability).nnnRESULTSnThirteen of the 17 patients (76%) had 49 surgery-related complications. Wound infection (24%), fistula, abscess, and malnourishment (12% each) were the most common early major complications, with hydronephrosis (35%), bowel obstruction (24%), acute pyelonephritis, fistula, lymphedema, and ureter stenosis (12% each) making up the majority of late complications in this category. Relatively frequent minor complications included protracted paralytic ileus and hematuria (18%), cholelithiasis, chronic diarrhea and, peristoma skin irritation (12%). Disease-free survival was associated with the timing of surgery (P = .002). All but one of the 12 patients who underwent surgery within 6 months of diagnosis are alive, compared with only one of five whose surgery was performed after a longer interval. Pelvic exenteration for GU-RMS was associated with a high rate of serious complications in this series of patients treated over 31 years.nnnCONCLUSIONnWith recent advances in surgical techniques, as well as improvements in preoperative and postoperative care, this procedure should continue to be applied in cases resistant to conventional therapy.

Duodenal duplication cyst manifested by duodeno-jejunal intussusception and hyperbilirubinemia

A rare case of duodenal duplication cyst containing stones in a 17-year-old patient is presented. The cyst, acting as a leading point for duodeno-jejunal intussusception caused proximal small bowel obstruction and hyperbilirubinemia. Preoperative diagnosis was based on abdominal computerized tomography. At operation, the cyst wall was unroofed creating free drainage into the duodenal lumen without damaging the biliary and pancreatic ducts with resolution of symptoms.

Epithelioid sarcoma in children

Literature regarding the natural history and prognosis of epithelioid sarcoma in the pediatric population is sparse. During the past 11 years, eight patients with epithelioid sarcoma were treated at St Jude Childrens Research Hospital. Of the eight children, six of whom were male, seven presented with localized disease and one with metastatic disease. The primary tumor was located in the upper extremity in four patients; the other sites were the scalp, external ear, back, and mediastinum. The mean duration of symptoms before diagnosis was 8.3 months (range, 1 to 12 months). Surgical treatment comprising amputation or wide local excision was performed in six patients, three of whom had additional lymph node dissection. Three patients received multiagent chemotherapy, and four patients were treated with supplementary radiation. Two patients died of progressive local and distant disease within 9 months of diagnosis. The other six patients are alive and have no evidence of disease 12 months to 10 years 6 months after the completion of treatment.

Soft tissue sarcoma of the hand in children: Clinical outcome and management

Soft tissue sarcomas (STS) of the hand are rare in children and adolescents. From 1965 through 1995, 18 children with STS of the hand were treated at our institution. Rhabdomyosarcoma (RMS) was diagnosed in 11 patients; alveolar histological results predominated (7 of 11 cases). Seven patients presented with metastatic disease and died 4 to 23 months (median, 9 months) from diagnosis; their surgical treatment comprised above-elbow amputation (n = 1), local excision (n = 1), and biopsy (n = 5). For the four patients who presented with localized RMS, surgery consisted of wide local excision (n = 1), local excision (n = 2), or ray amputation (n = 1). With an average follow-up of 5.5 years (range, 4 months to 18 years), 3 of the 11 patients diagnosed with RMS still survive (27%). The remaining seven patients presented with nonrhabdomyosarcomatous soft tissue sarcoma (NRSTS); the most common histological variants were epithelioid and malignant fibrous histiocytoma (two cases each). Surgical treatment for these patients comprised ray amputation (n = 3), wide local excision (n = 3), excisional biopsy (n = 1), and regional lymph node dissection (n = 3). One patient received adjuvant multiagent chemotherapy; three patients received supplemental radiotherapy. Six of the seven (85%) patients are alive with no evidence of disease at an average follow-up of 4.7 years (range, 6 months to 12 years).

Successful treatment of progressive stage 4s hepatic neuroblastoma in a neonate with intra-arterial chemoembolization

Stage 4s neuroblastoma (NB) is a unique entity seen in infants less than 1 year of age, with metastatic disease confined to liver, skin, or bone marrow. Despite metastatic spread, stage 4s NB has a favorable outcome. An exception to this is seen in neonates who present with progressive enlargement of the liver with secondary respiratory compromise and liver failure. We describe a 4‐week‐old neonate who presented with 4s NB, with a rapidly enlarging liver, resulting in respiratory and hepatic failure, who had a rapid, sustained, and ongoing response to chemoembolization of the hepatic artery. This approach is feasible at this age, and may be effective in improving the outcome in this group of patients.

Outcome of Treatment for Pediatric Sarcoma of the Foot: A Retrospective Review Over a 20-Year Period

Soft tissue sarcomas (STS) arising in the foot are rare in children. From 1975 to 1994, 18 children (median age, 13.5 years) were treated at St Jude Childrens Research Hospital for STS of the foot. Five children had rhabdomyosarcoma (RMS), with alveolar histology in four cases. All of these patients presented with metastatic disease, which proved fatal within 9 to 24 months despite combined modality treatment with chemotherapy, radiation, and surgery. Thirteen patients had nonrhabdomyosarcoma soft tissue sarcoma (NRSTS). The most common histological variant was synovial sarcoma (7 patients). Of the 10 who presented with localized disease, seven survived 1 to 12 years (median, 7 years). Surgical treatment for these consisted of local excision (n = 2), wide local excision (n = 3), below knee amputation (n = 1 ), ray amputation (n = 1). Three received supplemental radiation, and one was also treated with chemotherapy. Local or distant recurrence proved fatal in the three other children who had localized disease. Three children who presented with metastatic NRSTS died 8 to 14 months after diagnosis. All surviving patients are without significant functional disability. In our experience, pediatric RMS of the foot has a poor outcome, attributable to both alveolar histology and presence of metastatic disease at diagnosis. In contrast, NRSTS of the foot is more often localized, and limited surgery with adjuvant radiation in the absence of clear margins should be the treatment of choice.

Nested (Ossifying) Stromal Epithelial Tumor of the Liver: Case Report

Nested stromal-epithelial tumor (NSET) of the liver is an extremely rare primary hepatic tumor with uncertain malignant potential. To date, only 11 cases have been described. We describe the case of a 2 1/2-year-old girl with an incidental liver mass. The mass was discovered on follow-up abdominal imaging for asymptomatic hydronephrosis diagnosed on antenatal ultrasound. Needle biopsy showed a mixed stromal and epithelial process in a nested pattern, with foci of ossification and no significant pleomorphism or necrosis. The nest cells stained with WT-1, cytokeratin 18, and CD56. Ossifying stromal epithelial tumor of the liver was strongly suspected. The findings were confirmed in the subsequent partial hepatectomy specimen. To our knowledge, this is the 12th case of NSET in the English-language literature and the 3rd case of NSET associated with genitourinary system abnormalities. Possible associations with dysregulated WT-1 expression are discussed.

Continuous 13C-methacetin breath test differentiates biliary atresia from other causes of neonatal cholestasis.

Background and Aim: Distinguishing biliary atresia (BA) from other causes of neonatal cholestasis (NC) is challenging. Continuous BreathID 13C-methacetin breath test (MBT) is a novel method that determines liver function. Methacetin is metabolized uniquely by the liver and 13CO2 is measured passively, through a nasal cannula in the exhaled breath. The aim of this study was to assess the ability of MBT to differentiate BA from other causes of NC. Methods: MBT was performed in infants with NC before any invasive procedure. Percent dose recovered (PDR) peak and time to peak (TTPP) of 13C recovered were correlated with blood test results and degree of fibrosis on liver biopsy. Results: Fifteen infants were enrolled in the study. Eight were eventually diagnosed as having BA. MBT showed that infants with NC from various causes reached the PDR peak after 44.5u200a±u200a6.7 minutes, whereas infants with BA reached the PDR peak value after 54.7u200a±u200a4.3 minutes (Pu200a<u200a0.005). This suggested low cytochrome P450 1A2 activity in the BA group. The area under the curve (AUC) was 0.95 (95% confidence interval [CI] 0.83–1), sensitivity of 88%, and specificity of 100%. Conclusions: This pilot study shows that MBT can differentiate between BA and other causes of NC by time to peak of methacetin metabolism. The results suggest that MBT may be used as part of the diagnostic algorithm in infants with liver disease. Larger-scale studies should be conducted to confirm these initial observations.