Walter M. Morgan

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.

Laparoscopic evaluation for a contralateral patent processus vaginalis

Between May 1, 1992 and March 1, 1993, 221 consecutively treated children under 10 years of age evaluated by the authors for a known inguinal hernia were involved in a prospective protocol to determine whether diagnostic laparoscopy has a place for evaluation of the contralateral inguinal region. Twenty-six had known bilateral inguinal hernias and did not require diagnostic laparoscopy. Of the other 195 children who underwent laparoscopy before hernia repair, 86 had a unilateral hernia with a contralateral patent processus vaginalis (CPPV), and 109 had only a unilateral hernia. After anesthesia, it was suspected on prelaparoscopic clinical examination that 55 patients had a CPPV. During laparoscopy, it was noted that 31 (56%) had patent processus vaginalis and 24 (44%) did not. Of the 140 patients believed to have a CPPV on prelaparoscopic clinical examination, 60 (43%) did and 80 (57%) did not. Insufflation alone was not diagnostic of CPPV; of the 195 patients undergoing laparoscopy, insufflation resulted in a positive finding on the known side in only 129 (66%) and on the contralateral side in 23 of the 86 patients (27%) found to have a CPPV. There were no complications related to the laparoscopy or the hernia repair.

Laparoscopic evaluation for a contralateral patent processus vaginalis: part III

OBJECTIVES Between May 1, 1992 and August 1, 1996, 759 consecutive children younger than 10 years of age were evaluated and treated for known inguinal hernia. These children were participating in a prospective investigation of the potential role of diagnostic laparoscopy in the evaluation of the contralateral inguinal anatomy. The initial two series of data (parts I and II of this three-part series) were previously presented at the 1993 and 1995 American Academy of Pediatrics meetings. METHODS Of 759 patients, 100 children were diagnosed with bilateral inguinal hernias and therefore did not undergo laparoscopy. Thirty-two patients did not undergo laparoscopic evaluation due to technical difficulties or complicated clinical situations. The patients contralateral inguinal region was carefully examined under anesthesia, and predictions were made regarding the likelihood of contralateral patent processus vaginalis (CPPV). Six hundred twenty-seven children underwent diagnostic laparoscopy to confirm the presence or absence of CPPV. Laparoscopy was initially exclusively performed through the umbilicus prior to repair of the known hernia, but over the last 26 months, 250 children successfully underwent laparoscopy through the ipsilateral hernia sac. RESULTS Of patients younger than 1 year of age, 114 were diagnosed with both a known unilateral hernia and CPPV, whereas 132 had a unilateral hernia only (46% versus 54%). Among children older than 1 year of age, 148 (39%) were diagnosed with unilateral hernia and CPPV, and 233 (61%) were diagnosed with a unilateral hernia alone. After examination under anesthesia, 233 of the 627 patients were suspected of having a CPPV, and 107 were confirmed at laparoscopy (46%). The remaining 394 patients were not believed to have a CPPV. Normal inguinal anatomy was confirmed in 234 patients (59%), but 160 patients were found at laparoscopy to have a CPPV (41%). CONCLUSIONS A contralateral patent processus vaginalis may be present in a surprising number of young patients being evaluated for a known inguinal hernia. Laparoscopy can be performed without a separate incision when the ipsilateral hernia sac is of sufficient width to allow passage of the scope. Laparoscopy is the best method for evaluating the contralateral inguinal region, particularly in younger children, as it prevents unnecessary inguinal exploration and it decreases the risk that the child will later present with a clinical contralateral hernia.

Laparoscopic evaluation for contralateral patent processus vaginalis: Part II

Between May 1,1992 and August 1, 1995, 599 consecutively treated children under 10 years of age evaluated by the authors for a known inguinal hernia were involved in a prospective protocol to determine whether diagnostic laparoscopy has a place for evaluation of the contralateral inguinal region. The experience with the first 221 patients was reported at the 1993 AAP meeting. In this total experience, 81 patients had known bilateral inguinal hernias and did not require diagnostic laparoscopy. Five hundred eighteen patients had a unilateral inguinal hernia with the status of the contralateral region being unknown. Between May 1, 1992 and May 1, 1994, 368 children underwent evaluation using an umbilical approach. However, for the past 14 months, 150 patients have undergone the diagnostic laparoscopy through the ipsilateral hernia sac. Among the children under 1 year of age, 98 were found to have a unilateral hernia and also a contralateral patent processus vaginalis (CPPV) and 110 had a unilateral hernia. Of the children older than age 1,116 had a unilateral hernia and CPPV and 194 had only a single hernia. After induction of anesthesia, it was suspected on clinical examination that 195 of the 518 patients had a CPPV. However, laparoscopy showed that only 94 (48%) had a CPPV. In the remaining 323 patients, the surgeon believed that a CPPV was not present based on the examination. This negative finding was verified in only 198 patients (81%), but a surprising 125 (39%) did have CPPV documented at the time of endoscopy. Insufflation alone was very unrellable for documenting the presence of CPPV. Of the 214 patients for whom CPPV on the contralateral side was documented during laparoscopy, only 41 (19%) had a positive finding on insufflation. This experience has convinced the authors that diagnostic laparoscopy is the most accurate means to ascertain whether a patient should undergo contralateral inguinal exploration. In addition, laparoscopy through the ipsilateral inguinal sac is now the preferred approach.

Laparoscopic cholecystectomy in infants and children: modifications and cost analysis.

Between June 1990 and February 1993, 26 children underwent laparoscopic cholecystectomy. Their ages ranged from 25 months to 19 years (mean, 12.3 years; median, 13 years). Only six of them had hemolytic diseases associated with gallstones. Five presented with acute cholecystitis. Laparoscopic cholecystectomy was performed on these five, within 5 days of admission; the mean postoperative hospital stay was 2.5 days. The other 21 patients underwent elective cholecystectomy; their mean postoperative stay was 1 day. Several modifications have been made in our technique. Three 5-mm ports and one 10-mm umbilical port are used. In addition, direct incision of the umbilical fascia is performed with insertion of a blunt trocar and cannula rather than using the Veress needle for insufflation. The importance of positioning the epigastric cannula in the left upper quadrant in small children cannot be overemphasized. Cholangiography is now attempted in all patients and is easier with the Kumar cholangioclamp and sclerotherapy needle, under fluoroscopy. The total hospital charges for the patients who underwent elective laparoscopic cholecystectomy are compared retrospectively with those of seven children who had elective open cholecystectomy during the same period. In addition, a comparison is made between the two groups with respect to the costs of operating room equipment and postoperative pain control.

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.

Cardiorespiratory changes in children during laparoscopy

The authors prospectively examined the cardiorespiratory changes during brief laparoscopy (less than 15 minutes) in children. Intraoperative ventilatory management included a tidal volume of 12 mL/kg, with the rate adjusted to achieve an end-tidal CO2 (PETCO2) of 30 to 35 mm Hg. The initial rate and tidal volume were not changed during the procedure. Baseline measurements of heart rate, blood pressure, peak inflating pressure (PIP), PETCO2, and oxygen saturation were recorded every minute for 5 minutes before the start of the laparoscopic procedure, and every minute during the laparoscopic procedure. Fifty-five patients were enrolled in the study (age range, 1 month to 7 years; weight range, 5.2 to 31 kg). PIP increased from the baseline value of 20 +/- 2.5 to 23 +/- 3.2 cm H2O (P < .01) during laparoscopy. The increase in PIP was 5 or more in six patients, with a maximum of 7. PETCO2 increased from the baseline value of 32 +/- 3.1 to 35 +/- 4.8 mm Hg (P < .01). The PETCO2 returned to baseline within 10 minutes after completion of the laparoscopy. No increase in ventilatory parameters was required during the brief laparoscopic procedure.

Total parenteral nutrition and intestinal development: A neonatal model

Total parenteral nutrition (TPN) is widely used in premature and/or surgical neonates, but there is little information available about its effects on intestinal growth and development. Adult TPN models have demonstrated mucosal atrophy, and a young piglet model showed similar but increased intestinal hypotrophy. We have investigated these effects in the neonatal piglet model. Five three-day old piglets received a glucose (40 g/kg/d), amino acid (8 g/kg/d), and fat (4 g/kg/d) solution intravenously for 3 weeks. Matched littermates were fed an artificial sow-milk formula enterally at an equivalent caloric rate (215 kcal/kg/d). A third littermate was sow breast fed for the same study period. No differences were seen in the TPN or formula-fed piglets in weight gain (31 to 34 g/kg/d), hematocrit (25% to 27%), BUN (12 to 13 mg/dL), total serum protein (4.1 to 4.4 g/dL), or total bilirubin (0.4 to 0.6 mg/dL); however, the TPN animals were mildly hyperglycemic (167 mg/dL). The sow-fed control group had greater weight gain (51 g/kg/d) but were without caloric restriction. There were significant decreases in weight and length of the gastrointestinal tract, particularly in the proximal small bowel of the TPN piglets. Compared with formula piglets or sow-fed controls, the TPN proximal small bowel weight was reduced by 67% and 72%, respectively. Similar but less marked differences were seen in the TPN distal small bowel. There were no significant differences in the proximal or distal small bowel measurements between the formula and sow-fed piglets, despite their differences in overall weight gain.(ABSTRACT TRUNCATED AT 250 WORDS)

In utero Decompression of a Cystic Grade IV Sacrococcygeal Teratoma

Sacrococcygeal teratomas (SCT) are the most common congenital tumors in the newborn. The prevalence rate is approximately 1 per 40,000 births, with 80% occurring in females. The majority of these tumors are external, protruding from the perineal region. Intrapelvic SCTs, by contrast, are extremely rare and difficult to diagnose in utero. Only 15% of the SCTs are entirely cystic, the majority being mixed or solid tumors. We describe a case of a fetal cystic presacral (grade IV) SCT, discovered at 22 weeks of gestation, which resulted in bilateral ureteral obstruction and hydronephrosis. This is the first known reported case of prenatally decompressing a cystic SCT via an amniotic catheter to alleviate a mass effect in the fetus.

Congenital Hemangiopericytoma of the Clitoris

Hemangiopericytoma is a rare vascular tumor that is believed to arise from the pericyte of Zimmermann. Approximately 10% of cases occur in children and a third are congenital. To our knowledge we report the first case of a congenital hemangiopericytoma arising from the clitoris, describe its management and review the literature.