The expression of vascular endothelial growth factor and the type 1 vascular endothelial growth factor receptor correlate with the size of papillary thyroid carcinoma in children and young adults.

Vascular endothelial growth factor (VEGF) is essential for the growth of many solid tumors, but there are little data regarding VEGH in childhood thyroid cancers. We examined the relationships between VEGF, the type 1 VEGF receptor (FLT-1) and clinical outcome for a group of thyroid cancers in children and young adults. The expression of VEGF and FLT-1 were determined by immunohistochemistry using archival, paraffin-embedded thyroid tissue blocks and compared with the retrospective clinical outcome for each patient. The study included 67 children and young adults with papillary thyroid carcinoma (PTC, n = 42), follicular thyroid carcinoma (FTC, n = 8), benign lesions (n = 15), or controls (n = 2). VEGF expression was greater in PTC (mean intensity 2.23 +/- 0.25, p = 0.002) and FTC (2.8 +/- 0.73, p = 0.01) than benign lesions (1.0 +/- 0.27), and correlated with PTC size (r = 0.42, p = 0.008). FLT-1 expression was greater in PTC (mean intensity 2.8 +/- 0.17) than FTC (1.9 +/- 0.25, p = 0.015) and benign lesions (1.7 +/- 0.32, p = 0.002); and correlated with PTC size (r = 0.41, p = 0.01) as well as VEGF expression (r = 0.52, p = 0.002). Recurrent disease developed exclusively in patients with PTC which expressed VEGF (7/28, 95% CI 10.6%-44.2%). PTC that did not express VEGF (0/8, 95% CI = 0%-31.2%) did not recur; however, the difference was not statistically significant (p = 0.15). We conclude that the expression of VEGF and FLT-1 are directly correlated with the size of PTC in children and young adults.

Extensive surgery improves recurrence-free survival for children and young patients with class I papillary thyroid carcinoma.

BACKGROUND: Children with papillary thyroid cancer (PTC) rarely die of their disease, but are at high risk for recurrence, particularly with multifocal tumors (which occur in 42% of children with PTC). It is not clear if more extensive surgery, with an increased risk of complications, lessens the risk for recurrence. The authors hypothesized that patients with disease presumed to be confined to the thyroid gland (class I PTC) could have multifocal disease, involving the contralateral lobe, of which the surgeon is unaware. Treatment with less than subtotal thyroidectomy might be associated with a higher risk of recurrence. METHODS: The charts of 37 patients with Class I PTC diagnosed at < or =21 years of age between 1953 and 1996 were reviewed. The incidence of surgical complications and the risk of recurrence based on the extent of initial surgery ([1] lobectomy with or without isthmusectomy, [2] subtotal, or [3] total thyroidectomy) and adjunctive therapy with thyroid hormone or radioactive iodine (RAI) were examined. RESULTS: Eight patients had recurrent PTC. Patients treated with lobectomy with or without isthmusectomy were more likely to have recurrence than patients treated with subtotal or total thyroidectomy (Odds ratio, 8.7; 95% CI 1.4 to 54). Although the incidence of complications was statistically similar among the 3 surgical groups, 3 patients, all treated with more extensive surgery, had permanent hypoparathyroidism. There were too few patients to determine whether treatment with thyroid hormone or RAI offered additional benefit. CONCLUSIONS: In children with Class I PTC, more extensive surgery is associated with a lower risk of recurrence. This finding must be weighed against the risk of complications when determining the optimal treatment for individual patients.

The impact of initial surgical management on outcome in young patients with differentiated thyroid cancer.

BACKGROUND/PURPOSE: It is generally believed that differentiated thyroid cancer (DTC) in young patients has an excellent prognosis. This calls into question the need for more extensive surgical ablation of the thyroid gland with attendant risks of surgical complications. The purpose of this report was to investigate both the incidence of surgical morbidity and the impact of surgery on locoregional recurrence of disease. METHODS: The authors reviewed the clinical course of patients under 22 years of age treated for DTC within Department of Defense hospitals since 1950. Data were available for determination of surgical morbidity in 126 and for outcome in 105. RESULTS: The incidence of postoperative hypocalcemia was 17% and of recurrent laryngeal nerve injury 3%. Factors predictive of morbidity were (1) more extensive thyroid surgery (P = .023), and (2) the presence of gross tumor invasion (P = .022). The incidence of neck recurrence was analyzed among a cohort of 90 patients. A total of 19 (21%) patients had a local recurrence. The median time to recurrence was 24 months. The factor predictive of recurrence was the presence of gross invasion (P = .0001). A strong trend toward locoregional recurrence was found among patients with metastatic disease to more than five cervical nodes (P < .08). The primary operations on the thyroid and regional nodes were not significant predictors of neck recurrence. Among these 19 patients there have been no deaths, but 25% had persistent disease at a mean follow-up of 12.6 years. CONCLUSIONS: The incidence of surgical morbidity does increase with more extensive surgery. Outcome is predicted primarily by the initial extent of disease.

Early experience using telemedicine for neonatal surgical consultations.

BACKGROUND/PURPOSE: Telemedicine affords the opportunity to extend the presence of surgical evaluation to centers without an on-site pediatric surgeon. However, concern for cost, accuracy of diagnosis, and physician acceptance have limited its use. METHODS: Using a low-cost, desktop computer-based system, this study was designed to test the effectiveness of telemedicine in neonatal surgical consultation. RESULTS: Early experience with six video-teleconference (VTC) and six store-and-forward consultations are presented. Diagnosis was established accurately in all cases. With the three intestinal cases (jejunal atresia, duplication cyst, and r/o malrotation), diagnostic studies were guided by the images transmitted with the consult. Earlier care could be implemented in other cases. Technical problems were encountered primarily with the VTC modality, which also proved more consuming of physician time. CONCLUSIONS: Telemedicine was used successfully in each case and proved accurate in diagnosis and guiding further evaluation. This is the first report of the use of telemedicine for surgical consultation in the intensive care nursery.

Clinical features associated with metastasis and recurrence of differentiated thyroid cancer in children, adolescents and young adults.

OBJECTIVE: Differentiated thyroid cancer (DTC), including papillary (PTC) and follicular (FTC) variants, is unusual in children and accounts for only 10% of all cases. For that reason, knowledge of the clinical features which predict recurrence is limited. We reviewed 170 cases of childhood DTC to determine if specific clinical or pathological findings were associated with increased risk of recurrence. DESIGN: This was a retrospective study of children and adolescents with DTC registered in the Department of Defense Automated Centralized Tumor Registry. PATIENTS: We reviewed 137 cases of PTC and 33 cases of FTC diagnosed between 1953 and 1996 at < or = 21 years of age. RESULTS: In the PTC group (median follow-up 6.6 years, range 2 month-39.5 years), only one patient died, but 21 developed local and 6 developed distant recurrence. By univariate analysis, recurrence was more common in patients with multifocal (odds ratio 7.5) or large tumours (odds ratio 4.1), and in those with palpable cervical lymphadenopathy (odds ratio 3.0) or metastasis at diagnosis (odds ratio 2.8). By multivariate analysis focality was the best predictor of recurrence (P = 0.0019). In the FTC group (median follow-up 5 years, range 6 month-38.1 years), no patient died of disease, but 5 developed recurrence. As with PTC, recurrence was more likely in patients with multifocal tumours (odds ratio 22.0). CONCLUSIONS: Differentiated thyroid cancer in children and adolescents has low mortality, but a high risk of recurrence. Young patients with large, multifocal tumours that are already metastatic at diagnosis have the greatest risk of recurrence.

Is surgical ligation of a patent ductus arteriosus the preferred initial approach for the neonate with extremely low birth weight?

The optimal approach to a patent ductus arteriosus (PDA) in an extremely low birth weight (ELBW) neonate, whether initial surgical ligation or a trial of indomethacin, has not been established. The authors reviewed the records of 82 ELBW premature infants who had surgical ligation of a PDA during a 2-year period. Thirty-one received indomethacin before ligation. Bronchopulmonary dysplasia (BPD) occurred in 33% of the infants. Predictors of BPD were prolonged positive pressure ventilation, severe intraventricular hemorrhage (IVH) and lower birth weight (BW). Seventy-seven percent of the infants survived. Predictors of mortality were severe IVH, lower BW, and the occurrence of necrotizing enterocolitis (NEC). The indomethacin-treated infants had a lower incidence of NEC and IVH. Overall, 16% of the patients had perioperative morbidity, and 10% of the patients died. The study shows that a trial of indomethacin therapy is not associated with increased complications in ELBW infants with PDA.

Survival after aberrant right subclavian artery-esophageal fistula: case report and literature review.

Development of a fistula between an aberrant right subclavian artery and the esophagus is a rare cause of heretofore fatal hematemesis. We report the first known survivor of this devastating complication of the most common aortic arch anomaly. Intraoperative esophagogastroduodenoscopy, intraesophageal balloon tamponade, and arteriography were the keys to successful management. This lesion should be suspected in the setting of bright red, "arterial" hematemesis. Prolonged nasogastric and/or endotracheal intubation should be avoided in patients with a known aberrant right subclavian artery or other aortic arch anomaly.

Mediastinal tumors--airway obstruction and management.

Large mediastinal massess can cause compression of surrounding mediastinal structures. Patients may have symptoms of airway obstruction or cardiovascular compromise. The additive effects of anesthetics, paralysis, and positioning during biopsy can lead to acute airway obstruction and death. In some cases, tissue diagnosis can be achieved and treatment initiated without general anesthesia. When general anesthesia is necessary, specific measures should be taken to avoid disaster or immediately alleviate obstruction should it occur. Some patients at greatest risk will require pretreatment of the mass before tissue diagnosis. This article reviews these issues and provides a useful algorithm for managing patients with mediastinal masses.

Modified Nissen fundoplication: improved results in high-risk children.

A modified Nissen fundoplication was performed in 29 children (26 neurologically impaired and 3 neurologically normal). The modifications to the Nissen 360 degrees wrap were: (1) deliberate crural repair with pledgets; (2) recreation of the angle of His; and (3) anchoring wrap to the diaphragm. Presenting complaints were respiratory in 66%, nutritional in 28%, and for prophylaxis in 2 patients requiring gastrostomy tubes. Prospective follow-up was obtained in 26 of 29 patients for an average of 19 months. Early postoperative complications occurred in 3 patients, none due to recurrent reflux. Six late deaths occurred due to neurological deterioration, pulmonary disease, and medication overdose. Barium swallows were obtained in 20 of 22 available patients postoperatively and all wraps were intraabdominal. We conclude that the modified Nissen prevents wrap herniation and improves postoperative results in the high-risk neurologically impaired child.

Operative strategy for recurrent laryngeal cleft: a case report and review of the literature.

A case is reported of recurrent laryngotracheoesophageal cleft following two attempted repairs. The anterior approach to the larynx and trachea provided excellent exposure for defining the extent of the recurrent cleft, and allowed precise anatomic repair. Neurovascular structures were easily avoided by this operative method. Of 170 clefts reported in the literature, 19 recurred necessitating reoperation. The anterior approach to the larynx and trachea is appropriate in recurrent clefts and may be preferable at the initial repair.

Complications of gastroesophageal antireflux surgery in neurologically impaired versus neurologically normal children.

Antireflux surgery was performed in 234 children over a 5-year period; 153 were neurologically impaired (NI) and 81 were neurologically normal (NN). Initial presentation, demographic data, and type of antireflux operation were similar in the two groups. Eighty-six percent of the NI group versus 30% of the NN group had gastrostomy tubes placed. The incidence of late postoperative complications was 26% in the NI group and 12% in the NN group (P less than .01). During the late postoperative period, NI children underwent reoperation four times as frequently as NN children (19% v 5%, respectively; P less than .01). Wrap herniation accounted for 38% of complications and 59% of reoperations in the late postoperative period. Mortality due to aspiration occurred in 9% of the NI group versus 1% of the NN group. Combined failure rate (reoperation plus aspiration-induced deaths) was 28% in NI and 6% in NN (P less than .01). We conclude that neurological status is the major predictor of operative success and that wrap herniation due to crural disruption is the most common cause of operative failure.