Tuan H. Pham

Pheochromocytoma and Paraganglioma in Children: A Review of Medical and Surgical Management at a Tertiary Care Center

OBJECTIVE. The aim of this study was to review our institutional experience managing pheochromocytomas and paragangliomas in children. METHODS. A retrospective chart review of the Mayo Clinic database from 1975 to 2005 identified 30 patients <18 years of age with histologically confirmed pheochromocytoma or paraganglioma. RESULTS. There were 12 patients with pheochromocytomas and 18 with paragangliomas. The most common presenting symptoms were hypertension (64%), palpitation (53%), headache (47%), and mass-related effects (30%). Nine patients (30%) had a genetic mutation or documented family history of pheochromocytoma or paraganglioma. Fourteen patients (47%) had malignant disease, whereas 16 (53%) had benign disease. Logistic analysis showed that statistically significant risk factors for malignancy were (1) paraganglioma, (2) apparently sporadic, as opposed to familial, pheochromocytoma or paraganglioma, and (3) tumor size of >6 cm. Surgical resection was performed for 28 patients (93%), with perioperative mortality and major morbidity rates of 0% and 10%, respectively. Resection achieved symptomatic relief for 25 patients (83%). All patients with benign disease appeared cured after resection. For patients with malignant disease, the 5- and 10-year disease-specific survival rates were 78% and 31%, respectively, and the mean survival time was 157 ± 32 months. CONCLUSIONS. The incidence of malignant pheochromocytoma/paraganglioma was high in children (47%), particularly those with apparently sporadic disease, paraganglioma, and tumor diameters of >6 cm. Patients with a known genetic mutation or familial pheochromocytoma/paraganglioma were more likely to achieve resection with negative microscopic margins and had improved disease-specific mortality rates. Surgical resection remains the treatment of choice for pheochromocytoma and paraganglioma.

Outcome of adrenocortical tumors in children

PURPOSE This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome. METHODS An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and adenomas. RESULTS The mean age of the 23 children was 9.0 +/- 1.6 years; girls predominated (female-to-male ratio = 1.9:1) as did cancers (ACC 16, adenoma 7); tumor hormone production (74%); and advanced stage for disease (66%). All malignancies were more than 2.5 cm. Adrenalectomy, including en bloc resection of adjacent structures (35%) achieved grossly negative margins in 70% of patients. Three patients received chemotherapy or chemoradiation as primary treatment without surgery. There was no perioperative mortality; morbidity was 10% (pneumothorax, acute renal failure, chylous ascites, and thrombocytosis). Surgical cure without adjuvant therapy was achieved for all adenomas and ACC stages I and II. For ACC stage III and IV, median survival was 21 months, 5-year survival was 0%. All advanced-staged ACC received adjuvant therapy. Surgically negative margins conferred a survival advantage. CONCLUSIONS Children, especially females with ACC present with large advanced-staged tumors. Surgically negative margins with or without en bloc resection improves survival. The high percentage of children with functioning tumors suggests earlier detection is possible.

Minimally Invasive Repair for Pectus Excavatum in Adults

BACKGROUND The purpose of this study is to review the minimally invasive pectus excavatum repair in adults to determine the safety and effectiveness. METHODS An Institutional Review Board approved chart review identified patients 17 years or older who underwent minimally invasive pectus excavatum repair (MIPER) between January 1999 and January 2004. RESULTS Nineteen patients underwent MIPER. Indications for surgery were reduced exercise tolerance (13), dyspnea on exertion (17), improve self-perception (10), and chest pain (6). There were no intraoperative complications or conversions to open repair. Twelve patients (63%) required one strut and seven patients (37%) required two struts. Postoperative complications included self-resolving asymptomatic pneumothorax in six patients and pneumonia in one. Pain at six weeks postoperatively was mild to none in most patients and all had no pain at three months postoperatively except one patient with strut displacement. Two patients required removal of one of two struts due to displacement. The mean postoperative pectus index was significantly lower than preoperative value: 2.5 versus 4.6, p = 0.002. Among six patients with strut removal at two years postoperatively, two patients had mild recurrence of their deformity. CONCLUSIONS Minimally invasive pectus excavatum repair can be performed safely in adults. This approach is technically more challenging in adults with one-third of the patients requiring two struts for optimal repair. The risk of strut displacement is higher than in the pediatric population. The long-term effectiveness and durability of this procedure in adults is still unknown.

Liver transplantation for pulmonary vascular complications of pediatric end-stage liver disease

PURPOSE Hepatopulmonary syndrome (HPS) and portopulmonary hypertension (PPH) are poorly understood pulmonary complications of end-stage liver disease (ESLD). We present a case series of children with HPS and PPH. METHODS After institutional review board approval, query of our medical database identified children 0 to 18 years of age with ESLD diagnosed with HPS or PPH. Data were collected via chart review. RESULTS We identified 7 children with either HPS (n = 5) or PPH (n = 2). Patients with HPS presented with progressive dyspnea over a mean of 7 months (range, 4-12 months) at a mean of 13 years (range, 5-17 years) of age. Pulmonary shunting by albumin perfusion scan averaged 41% (range, 20%-66%) with an initial mean resting SpO(2) of 88% (range, 84%-94%) and mean SpO(2) during exertion of 79% (range, 60%-89%). Four patients required supplemental O(2) and, upon United Network for Organ Sharing (UNOS) appeal, received pediatric model for ESLD (or Child-Pugh) score exceptions, enabling them to undergo orthotopic liver transplant (OLT) within 1-2 months. The fifth patient was initially rejected by the UNOS regional review board, but 6 months of worsening hypoxemia led to OLT 2 months after successful UNOS appeal. All patients with HPS undergoing OLT experienced complete resolution of hypoxemia within 8 months. Both children with PPH were treated with intravenous epoprostenol, which lowered or stabilized mean pulmonary artery pressure and bridged them to OLT within 7 months of listing. Overall, there were no pulmonary complications; however, 1 patient with PPH expired shortly after OLT. The remaining patients are alive at a median follow-up of 27 months (range, 6-96 months). CONCLUSION Hepatopulmonary syndrome and PPH are uncommon complications of ESLD in children. Epoprostenol can bridge PPH patients to OLT. OLT leads to rapid resolution of HPS and PPH and currently represents the only successful treatment for these children.

Pediatric endoscopic injuries: incidence, management, and outcomes.

BACKGROUND Endoscopy has assumed a significant role in the management of gastrointestinal disorders. However, endoscopic-related injuries are poorly reported. METHODS Review of our surgical database (1980-2006) identified all patients age 18 years or younger with an endoscopic-related injury managed at our institution. RESULTS A total of 3269 colonoscopies were performed; there were 3 iatrogenic perforations (incidence, 0.09%). All were managed operatively: 2 underwent fecal diversion, and 1 was repaired primarily. There was 1 postoperative complication (internal hernia) and no deaths related to colonoscopic perforation. Nine thousand three hundred eight esophagogastroduodenoscopy (EGD) procedures were performed, resulting in 6 iatrogenic injuries (0.06%): bleeding (2), perforation (2), and mucosal tears (2) (0.02% each). All EGD-related injuries occurred in the esophagus except for 1 duodenal perforation which was managed operatively by primary repair. Both patients with bleeding complications underwent repeat endoscopy and cautery with satisfactory results. The other 3 patients were managed conservatively without complication. Three hundred eighty-nine endoscopic retrograde cholangiopancreatogram procedures were performed with 2 iatrogenic injuries (0.5%): 1 bleed and 1 perforation (0.25% each). Both were managed conservatively without complication. CONCLUSION Endoscopic injuries are uncommon. Colonoscopic perforations require prompt surgical intervention. Esophagogastroduodenoscopy- and endoscopic retrograde cholangiopancreatogram-related injuries are amenable to conservative therapy in clinically stable patients devoid of peritonitis.

Sensitivity and Utility of Parathyroid Scintigraphy in Patients with Primary Versus Secondary and Tertiary Hyperparathyroidism

IntroductionParathyroid scintigraphy (PS) may be used to localize hyperactive parathyroid glands preoperatively. Performance of PS in the setting of secondary and tertiary hyperparathyroidism (HPT) is not well quantified. The performance of PS in secondary/tertiary HPT versus primary HPT may reflect physiologic as well as radiopharmaceutical kinetic differences between multigland hyperplasia versus adenoma. The aim of this study was to review the performance of PS in secondary/tertiary HPT with a comparison to that for primary HPT. Moreover, we evaluated (1) the sensitivity of PS in detecting enlarged glands, and (2) PS detectability as a function of gland weight.MethodsWe performed a retrospective review of the Mayo Clinic database from 2000 to 2004. We identified 40 patients with secondary or tertiary HPT as well as a matched control group of 40 patients with primary HPT who had had preoperative PS and underwent parathyroid surgery.ResultsParathyroid scintigraphy correctly localized all enlarged glands in 88% of patients in the primary HPT group. PS correctly identified both the number and locations of all hyperplastic glands in only 28% of the secondary/tertiary HPT patients. PS failed to identify one enlarged gland in 23% of the patients and two or more enlarged glands in 40% of the patients. PS correctly detects the largest gland in 88% of the patients with secondary and tertiary HPT. The mean gland weight detectable by PS was 612 ± 120 mg for primary HPT. In secondary/tertiary HPT, glands detected by PS had a mean weight of 950 ± 109 mg, whereas the mean weight was 276 ± 34 mg for undetected glands (P < 0.002).ConclusionsParathyroid scintigraphy is a sensitive study for localizing parathyroid glands preoperatively in primary HPT patients. Its sensitivity is low in secondary and tertiary HPT patients. Thus PS has limited value as a preoperative localization study in secondary/tertiary HPT patients.