Gastrointestinal manifestations of multiple endocrine neoplasia type 2

OBJECTIVE: To determine the clinical features, natural history, and role of surgery for gastrointestinal manifestations of the multiple endocrine neoplasia type 2 (MEN 2) syndromes. SUMMARY BACKGROUND DATA: The MEN 2 syndromes are characterized by medullary thyroid carcinoma and other endocrinopathies. In addition, some patients with MEN 2A develop Hirschsprung's disease (HD), and all patients with MEN 2B have intestinal neuromas and megacolon that can cause significant gastrointestinal problems. METHODS: From 83 families with MEN 2A, eight patients with HD were identified (MEN 2A-HD). These and all patients with MEN 2B followed at the authors' institution (n = 53) were sent questionnaires to describe the onset and type of gastrointestinal symptoms and treatment they had before the diagnosis of MEN 2. Records of all patients responding were reviewed, including radiographic imaging, histology, surgical records, and genetic testing. RESULTS: Thirty-six of the 61 patients (59%) responded (MEN 2A = 8, MEN 2B = 28) to the questionnaires. All patients with MEN 2A-HD were operated on for HD 2 to 63 years before being diagnosed with MEN 2. All patients responding were underweight as infants and had symptoms of abdominal pain, distention, and constipation. Eighty-eight percent had hematochezia, 63% had emesis, and 33% had intermittent diarrhea before surgery. All patients with MEN 2A-HD had rectal biopsies with a diverting colostomy as the initial surgical procedure. This was followed by a colostomy takedown and pull-through procedure at a later interval. Ninety-three percent of patients with MEN 2B had gastrointestinal symptoms 1 to 24 years before the diagnosis of MEN 2. Symptoms included flatulence (86%), abdominal distention or being underweight as a child (64%), abdominal pain (54%), constipation or diarrhea (43%), difficulty swallowing (39%), and vomiting (14%). Seventy-one percent of patients with MEN-2B with gastrointestinal symptoms had radiographic imaging, 32% were admitted to the hospital, and 29% underwent surgery. CONCLUSIONS: Patients with MEN 2A-HD had a typical HD presentation and always required surgery. Patients with MEN 2B have significant gastrointestinal symptoms, but less than a third had surgical intervention. Understanding the clinical course and differences in these patients will improve clinical management.     

Preoperative prediction of the risk of pulmonary complications after esophagectomy for cancer

OBJECTIVES: Pulmonary complication is a frequent morbid event after esophagectomy for cancer. Its prediction may help select patients for preoperative rehabilitation. METHODS: We performed a retrospective review of 292 patients (231 men and 61 women; mean age, 60.1 years) who underwent esophagectomy for cancer between 1980 and 2000. Data were analyzed to identify factors associated with the development of pulmonary complications (reintubation for isolated respiratory failure and pneumonia). A scoring system was developed, and its ability to predict complications was assessed. RESULTS: Resection was performed for squamous cancer (n = 100), adenocarcinoma (n = 186), and other histologic types (n = 6) in patients with stages 0 or I (n = 53), II (n = 94), III (n = 114), and IV (n = 23) disease. Pulmonary complications, which developed in 78 (27%) patients, were associated with a 4.5-fold increase in operative mortality (7%-32%). Multivariable analysis identified independent predictors of pulmonary complications to be patient age (odds ratio [OR], 1.31; 95% confidence interval [CI], 0.99-1.74; P =.059), percentage forced expiratory volume in 1 second (OR, 1.21; 95% CI, 1.07-1.38; P =.003), and possibly performance status (OR, 1.48; 95% CI, 0.88-2.50; P =.14). A scoring system using these 3 covariates was developed, which predicted incremental risk of pulmonary complications (P =.013). The incremental risks of cardiovascular and overall cardiopulmonary complications were also predicted with this scoring system (P <.01 for each). CONCLUSIONS: A scoring system using patient age, spirometry, and performance status helps predict the likelihood of pulmonary and cardiovascular complications after esophagectomy and can help select patients who may benefit from preoperative cardiopulmonary rehabilitation.     

Surgical treatment of hemangiomas of soft tissue

Hemangioma is one of the most common soft tissue tumors comprising 7% of all benign tumors. The etiology is unclear. Many treatment modalities for the symptomatic deep subdermal or intramuscular hemangioma have been used, but surgical excision is the preferred treatment. During the past 20 years, 89 patients with soft tissue hemangiomas were treated by surgical excision at the authors' institution. This study was done to define the clinical characteristics of pathologically proven hemangiomas and to evaluate the outcome of the operative procedures. Intralesional or marginal excision for symptomatic hemangiomas yields satisfactory results for pain relief, functional recovery, and avoidance of recurrence. According to the data a hemangioma of the soft tissues is a benign lesion in which more aggressive surgery (wide or radical excision) or other modalities such as radiation usually are not warranted.     

Combined endovascular and surgical treatment of head and neck paragangliomas--a team approach

BACKGROUND: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas. METHODS: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization. RESULTS: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy. CONCLUSIONS: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen.     

Durability of benefits of endovascular versus conventional abdominal aortic aneurysm repair

PURPOSE: Endovascular abdominal aortic aneurysm (AAA) repair is reported to result in less initial patient morbidity and a shorter hospital length of stay (LOS) when compared with conventional AAA repair. We sought to examine the durability of this result during the intermediate follow-up interval. METHODS: The records of all admissions for all patients who underwent AAA repair during a 26-month interval were reviewed. RESULTS: Three hundred thirty-seven (337) patients underwent procedures to repair AAAs (163 open and 174 endovascular). Endovascular procedures were performed with a variety of devices (Talent, 108; Ancure, 36; AneuRx, 26; Zenith, 2; and Cordis, 2) and configurations (141 bifurcated and 33 aortomonoiliac). The mean follow-up period was 10.6 months (endovascular repair) and 12.3 months (open repair). LOS did not significantly vary by device (P =.24 to P =.92) or configuration (P =.24). The initial median LOS for procedures was significantly shorter (P =.009) for endovascular repairs (5 days) than for open procedures (8 days). However, the patients who underwent endovascular repair were more likely to be readmitted during the follow-up interval when compared with patients who underwent open procedure. The readmission-free survival rate after AAA repair at 12 months was 95% for patients for open AAA repair versus 71% for patients for endovascular repair (P <.001). If the total hospital days were compared, including the initial and all subsequent AAA-related admissions, there was no significant difference for mean LOS for patients who underwent endovascular versus open AAA procedures (11 days versus 13.6 days; P =.21). The patients for endovascular AAA repair most commonly needed readmission for treatment of endoleak (n = 31), wound infection (n = 12), and graft limb thrombosis (n = 9). Although women had similar LOS to men for endovascular repair (P =.44), they had longer initial LOS for open AAA repair (15 versus 10 days; P =.03). After endovascular repair, women were more likely than men to be readmitted by 12 months (51% versus 71% readmission-free survival rate; P =.03) and they had longer LOS on readmission (13.2 versus 5.2 days; P =.006). No gender differences were identified for patients after open AAA repair regarding readmission-free survival rate (P =.09) or LOS on readmission (P =.98). CONCLUSION: Although initial LOS was shorter for the patients who underwent endovascular as compared with conventional AAA repair, this advantage was lost during the follow-up interval because of frequent readmission for the treatment of procedure-related complications, chiefly endoleak. These readmissions frequently involved the performance of additional invasive procedures. Gender differences existed regarding LOS and the likelihood of complications after open and endovascular AAA repair.     

Tramadol infusion for postthoracotomy pain relief: a placebo-controlled comparison with epidural morphine

We compared continuous IV tramadol as an alternative to neuraxial or systemic opioids for the management of postthoracotomy pain in a prospective, randomized, double-blinded, controlled study. General anesthesia was supplemented by thoracic epidural analgesia with 0.25% bupivacaine. At rib approximation, patients received one of the following: IV tramadol (150-mg bolus followed by infusion, total 450 mg/24 h, n = 29), epidural morphine (2 mg, then 0.2 mg/h, n = 30), or patient-controlled analgesia (PCA) morphine only (n = 30). All patients received PCA morphine and rescue morphine as necessary postoperatively. For the first 24 h, pain and sedation scores and respiratory, cardiovascular, and side effect measures were monitored. There was no significant difference in pain scores and PCA morphine use between tramadol and epidural morphine. Pain scores at rest and on coughing were lower in the Tramadol and Epidural Morphine groups than in the PCA Morphine group at various time points over the first 12 h. The Tramadol and Epidural Morphine groups used significantly less hourly PCA morphine than the PCA Morphine group at specific time points in the first 10 h. Vital capacities in the Tramadol group were significantly closer to baseline values at the 20-h point than in the PCA Morphine group. We conclude that an intraoperative bolus of tramadol followed by an infusion was as effective as epidural morphine and avoided the necessity of placing a thoracic epidural catheter. IMPLICATIONS: A prospective, randomized, double-blinded, placebo-controlled study of postthoracotomy pain relief showed that IV tramadol in the form of a bolus followed by continuous infusion was as effective as epidural morphine. The use of tramadol avoids the necessity of placing a thoracic epidural catheter.     

Increased vascularisation enhances axonal regeneration within an acellular nerve conduit

Despite major advances in microsurgical techniques, the functional results of periphery nerve repair remain largely unsatisfactory. Furthermore, if a defect exists such that a nerve graft is required, the results are generally worse than those following a primary repair. The autologous nerve graft, the current 'gold standard', has inherent disadvantages and there has been a long quest to find a suitable alternative. The role of vascularisation in nerve regeneration is poorly defined and the aim of this work was to define and quantify the effects of increased vascularisation on nerve regeneration. Rat sciatic nerve defects (1 cm) were bridged with a silicone chamber containing vascular endothelial growth factor (VEGF; 500 or 700 ng/ml) in a laminin-based gel (Matrigel) known to support axonal regeneration. Chambers were harvested between 5 and 180 days to follow the progression of neural and vascular elements. Following immunohistochemical staining, computerised image analysis demonstrated that VEGF significantly increased vascular, Schwann cell and axonal regeneration within the chamber up to 30 days post-insertion, and stimulated regeneration of up to 78% more myelinated axons at 180 days, compared to plain Matrigel control. Furthermore, the non-linear vascular dose-response to VEGF was clearly reflected in the Schwann cell and axonal staining intensity, supporting the highly significant relationship between vascularisation and Schwann cell staining seen within the chamber (P <0.001). Target-organ re-innervation at 180 days was similarly enhanced by VEGF in an identical dose-dependant manner. VEGF at 500 ng/ml increased recovery of gastrocnemius muscle weight by 17% and footpad innervation by 51% (P <0.05) compared to control, indicating the long-term functional benefits of VEGF.     

Synchronous benign and malignant salivary gland tumors in ipsilateral glands: a report of two cases and a review of literature

BACKGROUND: Ipsilateral salivary gland tumors of different histologic types are rare and make up less than 0.3% of all salivary gland neoplasms. Only nine cases of synchronous benign and malignant ipsilateral parotid gland tumors have been described in the literature. METHODS: Two additional cases of synchronous benign and malignant neoplasms in the parotid gland are reported and discussed with a review of literature. RESULTS: Our first case describes a pleomorphic adenoma and a salivary duct carcinoma, an entity not previously reported in the literature. The second case documents the most common benign and malignant ipsilateral parotid gland neoplasm reported in this case series, a Warthin's tumor and a mucoepidermoid carcinoma. CONCLUSIONS: Synchronous salivary gland tumors exhibiting both benign and malignant components are uncommonly observed, with only nine cases published to date. We describe two additional cases of a synchronous benign and malignant ipsilateral parotid gland tumor.