Search

Volvulus of the transverse colon is very rare in children. Three cases that occurred in a short time span are described and the scarce literature is reviewed. All patients presented with colonic obstruction and in all three the condition was diagnosed at laparotomy, as CT-scanning is not an available option in either of the two institutions. The transverse colon had not become gangrenous but was resected after detorsion for redundancy. The postoperative course was complicated and relaparotomy had to be performed for reobstruction in all cases. Only if the 'reverse' bean sign is recognized on the preoperative plain abdominal radiograph this rare diagnosis can be suspected., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Besides laparoscopic pyloromyotomy, the operation for pyloric stenosis has been performed using 2 standard open surgical exposures: the right upper quadrant (RUQ) incision and the semi-circumumbilical (UMB) incision. The aim of this study was to compare the morbidity and cosmetic results of both open exposures., Methods: Between 1990 and 1995, we performed 104 pyloromyotomies through a RUQ incision. These operations were retrospectively compared with 133 UMB incisions performed between 1995 and 1999., Results: There were no significant differences between the 2 groups regarding age at presentation, sex, and preoperative status. Only a significantly higher percentage of patients with a metabolic alkalosis before surgery was found in the UMB group, but this did not affect morbidity rate. The groups did not differ significantly with respect to mucosal perforations (P =.95), wound infections (P =.53), inadequate pyloromyotomies (P =.42), or other complications. The mean operating time was slightly longer in the UMB group (P <.025). The UMB approach produced a better cosmetic result, with an almost invisible scar., Conclusions: This study has shown that the UMB approach has equal intra- and postoperative complication rates as compared with the RUQ approach. The main advantage of the UMB approach is that it produces an excellent long-term cosmetic result.

Purpose: The aim of this study was to compare presentation, complications, diagnosis, and treatment of choledochal cysts in pediatric and adult patients., Methods: Forty-two patients were analyzed after subdivision into 3 groups: group A, less than 2 years (n = 10); group B, 2 to 16 years (n = 11); group C, greater than 16 years (n = 21)., Results: The cysts were classified as extrahepatic (n = 33), intrahepatic (n = 5), and combined (n = 4). Seventy-six percent of patients presented with abdominal pain, (20 of 21 group C), and 57% with jaundice, (10 of 10 group A). Cholangiocarcinoma occurred in 6 patients, 4 of whom had previously undergone internal drainage procedures. Excision of the extrahepatic cyst was performed in 27 of 37 patients. Five patients, of whom, 4 had cholangiocarcinoma, were beyond curative treatment at the time of diagnosis. Six patients had died at the closure of this study, 5 of them had carcinoma., Conclusions: Presenting symptoms are age dependent with jaundice prevailing in children and abdominal pain in adults. In view of the high risk of cholangiocarcinoma, early resection and not internal drainage is the appropriate treatment of extrahepatic cysts. Patients who had undergone internal drainage in the past still should undergo resection of the cyst., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Background: Apple peel atresia is the rarest type of small bowel atresia. Because of its rare occurrence and high mortality rate, little is known about the long-term outcome of these children., Methods: The patient charts, operative reports, and office notes of 15 children with apple peel atresia from 6 pediatric surgical centers in the Netherlands were reviewed. Long-term follow-up was assessed through review of office notes and through questionnaires., Results: The median age at the time of operation was 1.5 days. Postoperatively, 53% suffered from cholestasis, and 40% were septic. Three patients died (20%). At follow-up at a median age of 24 months, 1 child showed growth retardation and 2 children suffered from short bowel syndrome. At the time of the questionnaire, all children showed normal growth and development., Conclusions: Even though children with apple peel atresia often suffer serious morbidity like short bowel syndrome and sepsis during the postoperative course, late morbidity turned out to be low. If the patients survive the operative and direct postoperative period, and survive the morbidity associated with malnutrition and the long-term use of total parenteral nutrition, they have a good chance of having normal bowel function with normal growth and development., (Copyright 2002 by W.B. Saunders Company.)

The authors report a case of a 38-year-old man with an esophageal squamous cell carcinoma after repair of esophageal atresia with tracheoesophageal fistula. This carcinoma occurred at a young age, near to the scar of the old anastomosis, in a patient with no other apparent risk factors. It is hypothesized that stasis caused by impaired esophageal motility may be the underlying cause. A single case is not enough to unequivocally prove a possible relationship between esophageal atresia and the development of esophageal cancer. Now that the first generation of survivors of esophageal atresia is reaching middle aged adulthood, one should, however, be aware of a possible increased incidence in these patients. J Pediatr Surg 36:629-630., (Copyright 2001 by W.B. Saunders Company.)

Purpose: The aim of this study was (1) To determine the value of fine-needle aspiration cytology (FNAC) in children with persistent or suspicious peripheral lymphadenopathy and (2) to analyze whether a history of previous malignancy influenced the accuracy of FNAC., Methods: A retrospective study in an Academic Children's Hospital of 73 FNACs of peripheral lymph nodes in 64 patients (35 boys and 29 girls; median age, 9 years; range, 15 months to 20 years) was performed between 1992 and 1997. Eleven patients were excluded because aspirated material appeared inadequate. Outcome was compared with results of subsequent surgical biopsies, clinical follow-up, or both. Patients were divided into group A "de novo" lymphadenopathy (n = 39) and group B lymphadenopathy and a history of previous malignancy (n = 23)., Results: Group A: FNAC showed a benign diagnosis in 25 cases, a malignancy in 13, and was inconclusive in 1. Outcome was false-negative in 2 and false-positive in 1. Sensitivity and specificity were 86% and 96%, respectively. FNAC helped avoid additional surgical procedures in 27 cases (61%). However, if FNAC showed malignant lymphoma, open biopsy was inevitable (8 of 13) to establish proper classification. Group B: FNAC showed a benign diagnosis in 10 cases and a malignancy in 13. Outcome was false-negative in 1, and false-positive in 1. Sensitivity and specificity were 92% and 90%, respectively. FNAC helped avoid additional surgical diagnostic procedures in 25 cases (86%)., Conclusions: (1) Fine-needle aspiration cytology is a rapid, simple and accurate diagnostic procedure to differentiate between benign and malignant peripheral lymphadenopathy in children. FNAC can avoid open biopsy in at least 60% of cases. (2) A history of previous malignant disease does not influence the accuracy of the test.

Background/purpose: Neuroblastoma is the most solid common extracranial malignancy in childhood. Despite multimodality treatment, high-risk disease continues to carry a poor prognosis. Glucocorticoids have been shown previously to induce differentiation in murine neuroblastoma cell lines, but no such effect has been documented in human neuroblastoma cells. Glucocorticoids are known to be active in the differentiation process of the neural crest. These studies describe the effects of dexamethasone on 6 human neuroblastoma cell lines., Methods: Dexamethasone was added to cultured neuroblastoma cell lines (LA1-5S, LA1-15N, BE[2]S, BE[2]N, SH-EP-1, SH-SY5Y) maintained in media supplemented with either normal serum or charcoal-depleted serum. Proliferation assays were performed, and flow cytometry was used to assess alterations in cell cycle. Cells were closely monitored for morphological changes with serial phase-contrast microscopy. Immunohistochemistry (3F8, NF-1, TRK-A) of cultured cells was used to evaluate differentiation. Glucocorticoid receptor levels was assessed using immunoblotting., Results: Dexamethasone decreased the rate of cellular proliferation in both standard and charcoal-depleted conditions. Flow cytometry showed a G1 accumulation. Increased expression of the differentiation-associated antigens was found in cells cultured in charcoal-depleted media, and a further augmentation was seen with the addition of dexamethasone. In standard media, dexamethasone had no detectable effect on the expression of these antigens. Glucocorticoid receptor expression was found to be comparable in all cell lines., Conclusions: Human neuroblastoma cells are sensitive to the differentiating effects of dexamethasone in an environment of charcoal-depleted serum. This phenomenon may be caused by the existence of growth and mitogenic factors in serum that are inhibiting differentiation.

Adult tibial shaft from a bone graft bank was used as a solid homologous bone graft for midsternal expansion thoracoplasty in an infant with Jeune's asphyxiating thoracic dystrophy. The technique appeared successful, but the child grew out of her chest in her second year of life. Expansion thoracoplasty for Jeune's disease probably should only be reserved for children who survive their first year of life without major surgery.

Purpose: After nephrectomy for unilateral Wilms' tumor (WT), two potential hazards threaten the remaining kidney. The development of a metachronous WT (2% to 3%), and decrease in renal function after chemotherapy. Our study aims included: (1) to clarify how many WT patients could benefit from partial nephrectomy (PN) without jeopardizing the high cure rate, (2) to establish the function of the kidney remnant, and (3) to test the radiological criteria for preoperative selection of PN candidates., Methods: A retrospective analysis of 90 consecutive cases of histologically verified WT (1982 to 1992) was carried out. Data were studied independently by the surgeon, the radiologist, and the pathologist. The preoperative selection criteria for PN were functioning kidney, tumor confined to upper or lower pole leaving two thirds of the kidney tumor free, and no hilar or vascular structures involved. After PN, renal function was estimated by a calculated creatinine clearance (CC) combined with the assessment of the relative function of each kidney by renal scintigraphy., Results: There were 46 boys and 44 girls with median age at diagnosis of 3 years 6 months (range, 3 months to 16 years 7 months). Stage I disease occurred in 40 patients, stage II in 23, stage III in 16, and stage IV occurred in 11 patients. Nearly all patients received preoperative chemotherapy. Radiological analysis of 85 available data sets (5 missing) suggested that PN was possible in 13 patients, and data were inconclusive in two. All 13 patients selected by the radiologist appeared to be in agreement with the pathological criteria for resectability. Of these cases, complete resection by PN was performed in five, could have been possible in retrospect in two, and appeared impossible in six. In two more patients, PN was performed; in both cases the radiologist had predicted PN to be impossible. Median follow-up after PN was 61 months, range, 45 to 167 months. Two patients died of distant metastasis, and no local recurrence occurred. Five are in remission with a median CC level of 110 mL/min, range, 85 to 124. Relative functions of the kidney remnants were 20% to 50%. Contralateral nephrectomy for progressive obstruction was performed in one, but CC level remained normal (85 mL/min). Comparing the preoperative radiological prediction with the combined surgical and pathological findings in a 2 x 2 frequency table, the sensitivity of the radiological findings could be calculated to be 80%, the specificity 97%, and the accuracy 87%., Conclusions: (1) Partial nephrectomy is safe in approximately 10% of Wilms' tumor patients. (2) The function of the kidney remnant remains well, and even permits contralateral nephrectomy. (3) Preoperative imaging shows an accuracy of 87% to predict the possibility for partial nephrectomy.

Purpose: The treatment of pericardial effusion resulting in cardiac tamponade has undergone an evolution in recent years, with the use of less invasive drainage methods in selected cases. To determine optimal therapy for pediatric oncology patients with pericardial effusion and tamponade, the authors reviewed their institutional experience with percutaneous catheter drainage., Methods: Patient records and operative reports were reviewed, and nine patients were identified who met clinical and echocardiographic criteria of cardiac tamponade and were treated with percutaneous pericardial catheter drainage., Results: The median age at time of diagnosis was 14 years (range, 5 months to 19 years), and the male:female ratio was 7:3. Underlying malignancies included acute myeloblastic leukemia in three, acute lymphoblastic leukemia in one, and Hodgkin's disease, B-cell lymphoma, medulloblastoma, desmoplastic small round cell tumor, and rhabdomyosarcoma in one each. EIght patients (89%) were receiving granulocyte colony-stimulating factor (GCSF) during the period when tamponade developed. All patients had a large or moderate-to-large pericardial effusion and right ventricular collapse with hemodynamic compromise on echocardiography, and two patients (22%) also had pericardial thickening. In nine patients, percutaneous catheter drainage was performed intraoperatively and under fluoroscopic or echocardiographic guidance. A median of 300 mL (range, 82 to 500 mL) of fluid was removed from the pericardial sac during the initial drainage, and cytology was positive in one (6%). Complete echocardiographic resolution was observed in eight patients (89%); a small posterior component persisted in one patient but was not significant hemodynamically. The catheters remained in place for a median of 5 days (range, 1 to 35 days) while repeat aspirations were performed. Tamponade resolved in all patients, and one died of overwhelming systemic sepsis. The survival period was 10 to 22 months, and tamponade or the drainage procedure did not contribute to death. Four patients remain alive after 4 month to 7 years of follow-up., Conclusion: Cardiac tamponade was effectively treated in all patients and did not recur with percutaneous catheter drainage alone. THere was no evidence of pericardial loculation or infection despite pancytopenia being prevalent with underlying illness and chemotherapy. Percutaneous catheter drainage is an effective treatment for pediatric oncology patients with pericardial tamponade. Because of its simplicity in comparison to move invasive techniques, initial treatment with percutaneous drainage should be considered in this patient population.

To identify prognostic factors for renal carcinoma in young patients, a retrospective analysis was performed of 22 patients (< or = 21 years of age) with histologically verified renal cell carcinoma. Demographic, staging, and treatment variables were collected in a database, and their effect on survival was determined using Kaplan-Meier probability distribution. The median age was 15.5 years (range, 3 to 21 years), and the male:female ratio was 13:9. Only three patients were black. Histopathologic examination showed 15 clear cell tumors, 4 mixed cell type, 2 papillary, and one well-differentiated adenocarcinoma. The median size of the primary tumor was 10 cm (range, 5 to 20). There were seven patients with stage I tumors, one with stage II, and 14 with stage IV. Complete resection of the primary tumor was accomplished in 12 patients. The overall 5-year survival rate was 30% (confidence interval, 20% to 40%). The 5-year survival rate was better for patients who had complete resection of the primary tumor (60% v 10%). Unresectability was associated with involvement of nodes and/or occurrence of metastases, thus an independent effect of complete resection on survival could not be demonstrated. The data showed that age, tumor size, location, and histology were not predictors of outcome; tumor stage and complete surgical resection were the only meaningful prognostic factors. The presentation of renal cell carcinoma as a localized or systemic disease may reflect a twofold biological behavior. In the first group, the disease is curable with resection, in the second, it is unaffected by surgery or adjuvant therapy. In light of the very low incidence of this renal malignancy in childhood, prospective multicenter studies will be required to improve the poor therapeutic results.

A tracheal left mainstem bronchus and gastric outlet obstruction owing to gastric mucosal intussusception occurred in a child who had esophageal atresia and tracheoesophageal fistula. Bronchography and bronchoscopy, indicated because of persisting atelectasis and ventilation dependency, showed a tracheal left mainstem bronchus. The atelectasis disappeared after reimplantation of the left mainstem bronchus into the carina. Feeding problems and recurrent pulmonary infections complicated the postoperative course. Radiographic imaging and esophagogastroscopy showed severe reflux esophagitis and a prepyloric mass that consisted of a gastric mucosal intussusception. Subsequent gastrotomy and resection of the intussuscepted mucosal fold relieved the gastric outlet obstruction. An antireflux procedure was performed simultaneously. The child recovered satisfactorily. It is suggested that gastric mucosal intussusception may be associated with esophageal atresia rather than with the presence of a gastrostomy tube, as has been proposed in the literature.