Wallace W. Neblett

Clinical Experience Over 48 Years With Pheochromocytoma

OBJECTIVE To analyze the presentation, localization, surgical management, pathology, and long-term outcome of a large series of patients with pheochromocytomas. SUMMARY BACKGROUND DATA There are several areas of controversy pertaining to pheochromocytomas. Although many studies report a higher rate of malignancy for extraadrenal pheochromocytomas than for adrenal pheochromocytomas, the number of patients with the former tumor are small and statistical analysis is lacking. There has also been recent debate as to whether microscopic features of the tumor may be predictive of future behavior. METHODS From 1950 to 1998, the authors observed 108 pheochromocytomas in 104 patients. The outcome of these patients has been followed prospectively. The medical records of these patients were reviewed for data on the presentation, localization, surgical management, pathology, and outcome. Patient survival was analyzed using Kaplan-Meier survival distributions. RESULTS This study included 66 female patients and 38 male patients. The average age at surgery was 42.3 years. Sporadic cases accounted for 84% of the patients; the other 16% had multiple endocrine neoplasia type 2, von Recklinghausens disease, von Hippel-Lindau disease, or Carneys syndrome. Of 64 adrenal tumors, 55 were initially considered benign, 6 had microscopic malignant features, and 3 had malignant disease. Mean patient follow-up was 12.6 years. To date, in five additional patients (none with microscopic disease) malignant disease developed (13% overall rate of malignancy). Recurrence occurred as late as 15 years after resection. Of 26 extraadrenal pheochromocytomas, 14 were initially considered benign, 8 had microscopic malignant features, and 4 had malignant disease. Thus, 46% of patients had either malignant disease or tumors with malignant features. Mean patient follow-up was 11.5 years. In one patient with benign disease and in one patient with malignant features, malignant disease developed (23% overall rate of malignancy). The difference in the rate of malignancy was not statistically significant between adrenal and extraadrenal pheochromocytomas. Patients with adrenal and extraadrenal pheochromocytomas also had similar rates of survival (p = NS). CONCLUSIONS The data suggest that patients with extraadrenal pheochromocytomas have the same risk of malignancy and the same overall survival as patients with adrenal pheochromocytomas. Lifelong follow-up of these patients is mandatory.

Implications of Early Sonographic Evaluation of Parapneumonic Effusions in Children With Pneumonia

Objective. To devise a clinically relevant grading system for the sonographic evaluation of parapneumonic effusions, and to evaluate length of hospital stay as a function of treatment approach and sonographic grades. Methods. Chest sonograms of 46 pediatric patients diagnosed with empyemas and admitted to two medical centers in the last 8 years were retrospectively evaluated using a grading system based on the degree of fibrinous organization within the parapneumonic effusions. Hospital charts were reviewed to determine the method of treatment and length of hospital stay. Patients were divided into two treatment groups: nonoperative (n = 26) (antibiotics alone, or combined with thoracentesis, or tube thoracostomy) and operative (n = 20) (open decortication, or video thoracoscopy and pleural debridement). Patients in the nonoperative group were further subdivided into two groups: those who received antibiotics alone (n = 11) and those who received antibiotics plus nonoperative drainage thoracentesis and/or tube thoracostomy (n = 15). Within each treatment group, patients were subdivided into two ultrasound grades: low (no evidence of organization) and high (evidence of organization such as fronds, septations, or loculations). Studentst test was performed to compare the lengths of hospital stay for each of the treatment groups and ultrasound grades. Results. The length of hospitalization was no different for patients with low-grade ultrasounds in the nonoperative (9.8 days) and operative groups (8.0 days). In contrast, length of hospitalization was significantly shorter for patients with high-grade sonograms in the operative group (8.6 days), when compared with the nonoperative group (16.4 days). Length of hospitalization for patients in the nonoperative group with high-grade sonograms was significantly longer (16.4 days) than for those with low-grade ultrasounds (9.8 days). Furthermore, when the nonoperative patients were divided into an antibiotics alone group and a nonoperative drainage group, the patients with low-grade sonograms had no difference in the length of hospitalization (9.0 days vs. 10.4 days), whereas those patients with high-grade sonograms in the nonoperative drainage group had a significantly longer hospitalization (19.9 days) than the antibiotics alone (high-grade) group (11.4 days). Conclusions. Patients with a low-grade sonogram had similar length of hospitalization if treated with either nonoperative or operative measures. Patients with high-grade sonograms had significantly shorter length of hospitalization when treated with decortication. Our retrospective study suggests that patients with high-grade ultrasound studies treated nonoperatively do not benefit from pleural drainage procedures or chest tube placement. This study demonstrates the usefulness of early sonographic evaluation of parapneumonic effusions. A prospective study evaluating the usefulness of sonographic assessment of severity of disease in the treatment of children with parapneumonic effusions is warranted on the basis of our retrospective data.

Management of major thromboembolic complications of umbilical artery catheters

Over the last 9 yr approximately 4000 infants have had physiologic monitoring with UAC at Vanderbilt University Medical Center. A larger number of them had minor ischemic complications prompting removal of the catheter, but 41 patients had major thromboembolic problems requiring varying degrees of surgical management. The place of surgery is clear in patients with catheter emboli or bleeding due to vascular avulsion related to catheter removal. Patients with arterial occlusion distal to the femoral artery may be treated expectantly although significant skin loss may occur. Occlusions at the femoral level allow limb survival, but the long-term outlook is not known; perhaps these patients may better be subjected to femoral thrombectomy in the future. Peripheral pulses are not normal early and Doppler studies suggest that collateral circulation is responsible for limb survival. Whether this will be sufficient to support adequate limb growth and function remains to be seen. Patients with signs of aortoiliac or mesenteric occlusion should be rapidly investigated and operated upon if survival is to be obtained. This study suggests that an aggressive surgical approach is in order. Computer-assisted radionuclide flow studies are helpful in diagnosing major aortic occlusion, as well as for followup.

Postpneumonic empyema in childhood: Selecting appropriate therapy

In order to identify appropriate treatment options for postpneumonic empyema, we reviewed the medical records and, when possible, obtained long-term follow-up chest radiographs and pulmonary function tests on children treated for empyema during the past 11 years. Fifty-one patients were treated in various ways, with antibiotics alone (N = 10), or in combination with tube thoracostomy (N = 23) or decortication (N = 18). Despite administration of appropriate antibiotics and establishment of pleural drainage, many children required prolonged hospitalization and eventual decortication. Based on this review, a scoring system was developed allowing early classification by severity of pleural disease. Factors found to be predictors of severe pleural disease include (1) low pleural fluid pH or (2) glucose; (3) presence of moderate or severe scoliosis or (4) pleural peel or parenchymal entrapment by chest radiography; and (5) infection due to anaerobes, gram-negative organisms, or mycoplasma. Complete opacification of a hemithorax on chest radiography and a pleural peel to thoracic ratio greater than 40% were also associated with severe pleural disease. In patients with mild disease (N = 7), response to antibiotics alone, rapid resolution of fever, and shorter hospital stays were observed. In patients with more severe infections (moderate = 22, severe = 22), decortication accomplished earlier defervescence, radiographic improvement, and hospital discharge than simple tube thoracostomy. No deaths or morbidity were associated with decortication, which could often be accomplished through a minithoracotomy. Follow-up chest radiographs and pulmonary fuction tests showed a prompt return to normal after decortication. This experience indicates utility of a pleural disease severity scoring system in selection of treatment options for children with postpneumonic empyema.(ABSTRACT TRUNCATED AT 250 WORDS)

Laparoscopic cholecystectomy in children: lessons learned from the first 100 patients.

BACKGROUND/PURPOSE Laparoscopic cholecystectomy is a very common operation in adults but is relatively infrequently required in children. A retrospective review of 100 consecutive infants and children undergoing laparoscopic cholecystectomies from 1990 to 1998 was performed to see what lessons have been learned from this relatively large population of pediatric patients. RESULTS The patients ranged in age from 25 to 230 months, with a mean of 105 months. Only 19 patients had hemolytic disease as the etiology for their cholelithiasis. Two patients had biliary dyskinesia. Seventy-eight patients underwent an elective operation. Twenty-two children required urgent hospitalization because of complications from their cholelithiasis: acute cholecystitis (n = 7), jaundice and pain (n = 6), gallstone pancreatitis (n = 5), acute biliary colic (n = 4). All 6 patients who presented with jaundice underwent endoscopic retrograde cholangiopancreatography (ERCP) before their laparoscopic cholecystectomy. Two patients required laparoscopic choledochal exploration. The operating time and postoperative hospitalization were significantly longer (P = .0005) in the complicated group when compared with the elective patients. No significant complications such as the need for reoperation, injury to the choledocuhus or to other viscera, bile leak, or retained choledocholithiasis occurred. CONCLUSIONS Laparoscopic cholecystectomy is a safe, effective procedure in children for removal of the gallbladder. The exact role of routine cholangiography and ERCP remains unclear.

Management of tracheobronchial and esophageal foreign bodies in childhood

During the last 9 years 72 patients with esophageal (EFB) and 68 with tracheobronchial foreign bodies (TBFB) have been managed by a variety of methods. Cotton, et al previously suggested that postural drainage is a safe alternative to endoscopy for TBFB and this was evaluated in a portion of this series. A wide variety of objects obstructed the esophagus of 72 patients. In 62 patients with previously normal esophagi, coins, food, and other smooth objects were all extracted with Foley balloon catheters under fluoroscopic control or were pushed into the stomach and later passed. Endoscopic extraction was only necessary in four patients who had sharp EFB, two of which had previously perforated. Neck drainage was not required. Three of six patients with poor esophageal motility or partial strictures who had EFB required endoscopic removal. Nuts, shells, small metallic or plastic objects as TBFB which were present three hours to 13 months were treated in 68 patients. In only three instances was endoscopy considered emergent because of respiratory distress. Patients who had eaten were endoscoped electively and 30 treated with postural drainage in the interim; only 1 of 30 patients avoided endoscopy. Fifty-five patients endoscoped had successful removal using 3–0 and 4–0 Fogarty balloon catheters including several patients refered following unsuccessful conventional bronchoscopy without magnification. In seven instances foreign-body forceps were required to remove sharp objects lodged distally and granulation tissue. In five instances endoscopy was unsuccessful for TBFB present at 48 hours to 6 months. Lobectomy was required in two cases but successful broncotomy was performed in the others and is considered preferable although later endoscopic follow-up is indicated to anticipate bronchostenosis.

Management of aortic thrombosis secondary to umbilical artery catheters in neonates

During the past ten years, we have surgically managed seven neonates who developed total occlusion of the distal aorta due to umbilical artery catheters. All patients experienced symptoms of congestive heart failure. Five patients presented with severe hypertension, and all of these had aortorenal involvement: three infants had aortorenal thrombosis and two infants had infrarenal aortoiliac thrombosis with suprarenal extension of thrombus. Two infants had aortoiliac thrombosis with clot confined to the infrarenal aorta. Aortic thrombosis imposes an additional severe hemodynamic insult in these already seriously ill infants. Survival in this group of patients depends upon prompt recognition of this problem, effective surgical correction, and careful perioperative management. Our experience suggests that this diagnosis should be entertained in the infant presenting suddenly with congestive heart failure, hypertension, or lower limb ischemia after umbilical artery catheterization. The diagnosis is preferably confirmed by real-time ultrasound and/or radionuclide flow scan, although aortography may sometimes be necessary. Surgical management includes early transabdominal aortotomy with thrombectomy. Prompt thrombectomy resulted in the survival of six patients. One infant died in acute renal failure. Renal function and leg perfusion is satisfactory in the remaining patients, although one child required later operative correction of renovascular hypertension. Two additional patients needed prolonged postoperative antihypertensive therapy for 14 to 34 months before this problem resolved. Long-term follow-up is necessary for managing renovascular hypertension and monitoring lower extremity perfusion.

Alcohol and the adolescent trauma population

Trauma is the leading killer of children and adolescents between 1 and 21 years of age. Alcohol-impaired driving represents the single greatest cause of mortality and morbidity of children over the age of 6. We retrospectively reviewed 878 consecutive adolescent (age range, 16 to 20 years) trauma admissions for blood alcohol concentration (BAC). Four hundred sixty-seven patients had BAC drawn, 258 were BAC-negative (group I), 209 (48%) were BAC-positive (group II). The adolescent drinkers were then compared with a group of 748 adult drinkers (group III). Groups I and II differ in sex, age, time of day of the accident, Injury Severity Score, Glasgow Coma Score, and Revised Trauma Score, whereas group II and III differ by type of accident, type of injury, socioeconomic factors (bad debt), time of day of the injury, and BAC. There were no significant differences in TRISS predicted survival, actual survival, nor mean length of stay. We conclude that (1) alcohol is a significant contributor to injury during adolescence, and (2) adolescent drinkers differ from adult drinkers in their habits, demographics, and socioeconomic status. These socioeconomic differences have implications for the access to and cost-effectiveness of interventions.

Early operation with intestinal diversion for necrotizing enterocolitis

Necrotizing enterocolitis (NEC) remains a highly lethal disorder despite significant advances in management during the past decade. Increased mortality with perforation and the frequent finding of massive intestinal necrosis have hampered efforts to improve survival. A recent experience with early operation and intestinal diversion in selected patients with clinical features suggesting impending intestinal gangrene suggests a place in the management of infants with rapidly progressive NEC.

Hirschsprung's disease with skip area (Segmental aganglionosis)

Hirschsprungs disease is characterized by a single aganglionic segment of colon extending distally to the anal margin. Well documented reports of segmental aganglionosis have been rare. We report a case of segmental aganglionosis in which there were two distinct aganglionic segments resected. The entire transverse colon between the two aganglionic segments was normally ganglionated, preserved, and utilized and functions in a normal fashion.