The many faces of pheochromocytoma.

OBJECTIVE: To recognize and manage pheochromocytomas in unusual settings. METHODS: Three case reports are presented with clinical, biochemical, imaging, and operative findings. The pitfalls in diagnosis of pheochromocytomas and management are addressed. RESULTS: We begin with a 27-yr-old gravida 2, para 1 Caucasian woman with unexplained tachycardia and hypertension during a routine pre-natal visit at 30 weeks estimated gestational age. Urinary studies revealed elevated catecholamines. Magnetic resonance imaging localized a 6.6-cm right adrenal mass with features consistent with a pheochromocytoma. She was medically managed with phenoxybenzamine and propranolol until 35 weeks, after which she underwent a combined Cesarean section, and open right adrenalectomy. Another patient, a 36-yr-old African-American woman presented to a hospital in cardiac arrest, with elevated serum troponins, and underwent cardiac catheterization, which revealed normal coronary arteries. A computed tomography (CT) scan revealed a left adrenal mass and CT-guided biopsy was consistent with a pheochromocytoma, although prior studies were negative. Finally, we present a 49-yr-old Caucasian woman who had a right adrenalectomy 10 yr prior and presented to the clinic with fluctuating blood pressures, headaches, and palpitations. Further testing revealed she had a recurrent metastatic pheochromocytoma. The challenges behind treating these patients are further explored. CONCLUSION: Antenatal diagnosis of pheochromocytoma, though challenging, is associated with lower maternal and fetal morbidity and mortality. The differential diagnosis for cardiac arrest in the presence of normal coronary arteries should include a pheochromocytoma. Finally, treatment with iodinated metaiodobenzylguanidine may be a therapeutic option for those patients with metastatic pheochromocytomas.

Radiologic characterization of adrenal masses: the role of computed tomography--derived attenuation values.

BACKGROUND: Recent studies suggest that low computed tomography (CT) attenuation values can be used to differentiate benign adrenal adenomas from non-adenomas. We examined the utility of non-enhanced CT attenuation values of

The validity of quick intraoperative parathyroid hormone assay: an evaluation in seventy-two patients based on gross morphologic criteria.

BACKGROUND: Parathyroidectomy for primary hyperparathyroidism has conventionally required identification of all parathyroid glands with excision of grossly abnormal glands. Using this approach, cure rates exceed 95%. Directed cervical exploration has been advocated using quick intraoperative parathyroid hormone (QPTH) assay with preoperative localization. Adoption of this approach requires validation of the accuracy of QPTH assay. METHODS: Patients with primary hyperparathyroidism undergoing bilateral neck exploration during a 31-month period were reviewed. Uniglandular (UGD) or multiglandular (MGD) disease was determined by gross morphologic criteria. QPTH assays were performed before skin incision and at 5, 10, and 20 minutes after excision of each abnormal gland. A 10-minute QPTH decrease of 50% from baseline levels indicated curative excision. These data were not used to guide extent of exploration or tissue resection. RESULTS: Of 72 patients, 55 (76%) had UGD and 17 (24%) had MGD. QPTH assay accurately predicted the disease state in 89%. Four (7%) UGD patients did not have an appropriate QPTH decline at 10 minutes. Four (24%) MGD patients had an inappropriate QPTH decline at 10 minutes. CONCLUSIONS: Using QPTH guided exploration, 6% (4 of 72) of patients would undergo unnecessary extended exploration and 6% (4 of 72) (95% CI, 1% to 13%) may require reoperation for unidentified MGD. These results validate the accuracy of QPTH assay.

Parathyroid carcinoma in a child.

Parathyroid carcinoma is a rare cause of hypercalcemia in children but should be considered in a child presenting with an extremely elevated serum calcium level. The authors report the fifth case of parathyroid carcinoma in a child less than 16 years of age.

Macrofollicular variant of papillary thyroid carcinoma with minor insular component.

BACKGROUND: The macrofollicular variant of papillary thyroid carcinoma that is the subject of this study has only recently been characterized. Information about its morphologic spectrum and biologic behavior is limited. METHODS: The authors reviewed 29 examples, including 17 previously reported cases. The clinical and pathologic features of five patients who had the macrofollicular variant of papillary thyroid carcinoma with a minor insular component were analyzed in detail. The insular component in thyroid carcinomas has been associated with aggressive clinical behavior. RESULTS: The ages of the 5 patients ranged from 31 to 70 years; the mean age was 40 years. Three patients presented with a palpable thyroid nodule and two with a large thyroid mass of long duration. The latter two tumors, which metastasized, were the largest (8 and 11 cm) and showed extrathyroidal and blood vessel invasion. All five tumors were composed predominantly of macrofollicles (>50%) and had a minor insular component that comprised less than 5% of the tumor mass. In most tumors, the macrofollicles were lined by cells with large, clear, grooved nuclei, and all five contained areas of conventional follicular variant of papillary thyroid carcinoma. In the two that metastasized, however, the lining of many macrofollicles consisted of cuboidal cells with small, hyperchromatic, follicular-type nuclei. Only the macrofollicular component was identified in the metastatic deposits in these two patients. All five patients were alive at last follow-up, two with metastases; but follow-up for this study is limited. CONCLUSIONS: A minor insular component is an additional feature of the macrofollicular variant of papillary thyroid carcinoma that may aid in diagnosis and does not appear to have an adverse effect on the excellent prognosis of patients with these tumors.

The anterior operative approach to the cervical vertebral artery.

BACKGROUND: Vertebral arterial trauma continues to be a perplexing diagnostic and therapeutic challenge. Operative management is often required despite improved radiologic interventions for these injuries. Accounts of the operative approaches to anterior cervical vertebral artery injuries have been limited. STUDY DESIGN: We reviewed our experience with anterior cervical vertebral arterial trauma in 53 consecutive patients requiring operative management during a 14-year period. In seven patients, the vertebral arterial injury was identified at urgent surgical intervention either for an expanded cervical hematoma or active bleeding. The remaining injuries were identified by arteriographic investigation of penetrating cervical trauma. The injuries were equally distributed between the three anatomic zones of the anterior cervical vertebral artery. The general features of the operative approaches that were used to manage these injuries were the emphasis of the study. RESULTS: The anterior approaches to patients with vertical arterial trauma were effective in controlling injuries in all cases. Proximal and distal ligation of the artery adjacent to the injury site was accomplished in 95 percent of the patients. Associated major cervical injuries in 43 percent of the patients (carotid artery, eight patients; pharyngoesophageal, six patients; and neurologic, nine patients) contributed to the postoperative morbidity rate and the overall mortality rate of 10 percent. CONCLUSIONS: The surgeon approaching vertebral arterial trauma should have a clear appreciation of the deep anterior cervical anatomy to expedite the operative management and avoid unnecessary complications related to a misdirected surgical dissection. The descriptions of the operative techniques used in this clinical experience can aid the surgeon in managing patients with vertebral vascular trauma.

Angiographic ablation of mediastinal parathyroid adenomas: local experience and review of the literature.

PURPOSE: To evaluate local experience with a modified technique for angiographic ablation of mediastinal parathyroid adenomas. PATIENTS AND METHODS: Three patients with likely mediastinal parathyroid adenomas that had single feeding arteries underwent attempted arteriographic ablation with a slow continuous infusion of contrast medium. Patients were closely monitored for symptoms and calcium dynamics immediately postprocedure and then on a long-term outpatient basis. RESULTS: All three patients were cured (follow-up 22 to 68 months) with no long-term complications. CONCLUSION: Percutaneous angiographic ablation with contrast medium is a reasonable alternative for patients with hyperparathyroidism due to a mediastinal adenoma who can be treated in centers with well-trained interventional radiologists.

Complex iliac arterial trauma: autologous or prosthetic vascular repair?

The methods for vascular reconstruction of iliac arterial trauma can be a challenging problem. Primary arterial repair is usually possible, but an extensive injury may require interposition grafting or extra-anatomic bypass. Some investigators have advocated prosthetic grafting as a reasonable method of repair for complex iliac arterial injuries. We challenge this concept and describe our experience with the exclusive use of autogenous vascular repair during involvement with 87 consecutive patients surviving iliac arterial trauma.

Penetrating abdominal aortic trauma: a report of 129 cases.

The clinical presentation, resuscitation, and operative management of 129 patients with penetrating injuries to the abdominal aorta treated between 1960 and 1989 were reviewed. This is the largest reported civilian or military experience with this specific injury; our review of the literature was limited to reports focusing on the detailed analysis of the management and outcome of aortic trauma. Eighty-two percent of patients were in shock; 18 patients (14%) underwent emergency room thoracotomies, with no survivors. Of 46 patients requiring operating room thoracotomies, only 20% survived. All patients underwent exploration. Thirty percent had three or more visceral injuries; only 2% had no concurrent visceral injury. There were associated vascular injuries in 58% of patients, the inferior vena cava being the most frequent (37%). The most significant predictor of death was continued bleeding at operation. Ninety percent of patients with free intraperitoneal bleeding died, in contrast to 35% with a contained retroperitoneal hematoma (p less than 0.001). Aortic injuries were supraceliac in 25% of patients, between the celiac and renal arteries in 25%, and infrarenal in 50%. The respective mortality rates were 70%, 80%, and 47% (p less than 0.05). Repair of the aortic defect was possible in 103 patients (80%). The most common repair was lateral arteriorrhaphy in 53% of patients, followed by end-to-end anastomosis in 15% and prosthetic repairs in 8%. The overall mortality rate was 62%, which is similar to that of previous reports. Despite advances in trauma care, a functioning paramedic system, and the use of aggressive means to obtain aortic control, penetrating aortic trauma remains highly lethal.

Pheochromocytoma with extension into the inferior vena cava: a case report.

Eleven cases of pheochromocytoma with extension into the inferior vena cava have been reported previously. This report describes a case of a right adrenal pheochromocytoma with extension into the inferior vena cava that was removed with the aid of cardiopulmonary bypass and circulatory arrest.

Pelvic abscess or pseudoaneurysm: diagnostic and therapeutic dilemma following iliac arterial trauma.

Intra-abdominal abscesses often complicate operations for abdominal trauma and are particularly dangerous in patients whose injuries involve major vessels. We report our experience with 10 patients who developed pelvic abscesses among 75 survivors of iliac arterial injuries. Pseudoaneurysms of primarily repaired iliac arteries occurred in 8 of these 10 patients. Emergency operations were required for acute arterial thrombosis or hemorrhage in four patients; massive hemorrhage that complicated the drainage of pelvic abscesses led to the recognition of the pseudoaneurysms in the other four patients. Three of the eight patients with pseudoaneurysm died of postoperative complications; ischemic extremity sequelae occurred in all five survivors. The association of pelvic abscesses with the complications iliac arterial repairs has not been previously emphasized. The integrity of an arterial repair should be arteriographically confirmed before proceeding with drainage of a pelvic abscess that developed after iliac arterial trauma.

Mineral metabolic effects of thyroidectomy and long-term outcomes in a family with MEN 2A.

We have followed a family with multiple endocrine neoplasia type 2A for 18 years. Four members have undergone total thyroidectomy for medullary thyroid carcinoma or C-cell hyperplasia, and one has required bilateral adrenalectomy for pheochromocytoma. None has developed hypercalcemic hyperparathyroidism, although parathyroid hormone levels were relatively high prethyroidectomy and fell postoperatively in the patients with high calcitonin levels. In three of the four cases, intestinal calcium absorption decreased following thyroidectomy.

Multiple nonatherosclerotic aneurysms unrelated to a clinical syndrome.

Two cases of multiple inflammatory aneurysms in young patients without evidence of autoimmune disease or an aneurysmal syndrome are presented. Neither case showed any clinical or histologic characteristics typical of Ehlers-Danlos syndrome, Marfan's syndrome, Takayasu's arteritis, or other aneurysmal syndromes. Pathologic findings showed adventitial and medical destruction in both patients; although the first case displayed more adventitial involvement, the second showed greater destruction of the medial elastic laminae. The decision to repair an aneurysm should be tempered by its location, the amount of involvement of the artery with multiple lesions, and the risk to the end-organ if repair is unsuccessful.

Subxiphoid pericardial window and penetrating cardiac trauma.

Between 1982 and 1986, 108 patients with penetrating wounds in proximity to the heart and no obvious signs of cardiac injury underwent a diagnostic subxiphoid pericardial window procedure. Eighty-four patients had stab wounds, 22 had gunshot wounds, and two had shotgun wounds. The subxiphoid pericardial window procedure produced positive results in 30 patients (28%) and negative results in 78 patients (72%). There were two false-positive subxiphoid pericardial window procedures. Eleven patients (39%) with cardiac injuries were not hypotensive and did not have signs or symptoms of cardiac injuries prior to the subxiphoid pericardial window procedure. When they were obtained (n = 80), electrocardiograms were abnormal in 57% and 21% of patients with and without cardiac injuries, respectively. At thoracotomy, 28 patients had injuries (ventricle, 20; atrium, three; and pericardium, five) and 21 required suture repair. Thirty-nine patients underwent a concomitant laparotomy. Forty-six abdominal visceral injuries were identified in 28 patients, including 21 hollow viscus injuries. There was no significant short-term or long-term morbidity (eg, pericarditis, tamponade, or post-pericardiotomy syndrome) attributable to the subxiphoid pericardial window procedure. There were no known missed cardiac injuries, and there were no deaths in the series. Some patients with penetrating wounds to the heart have no overt signs or symptoms of cardiac injury. A subxiphoid pericardial window procedure quickly identifies or excludes cardiac injuries and can be performed with minimal morbidity, even when there is contamination from the gastrointestinal tract.

Popliteal and shank arterial injury.

Arterial injuries below the adductor hiatus in the lower extremity result in amputation more often than do injuries in any other site. The major deterrents to limb salvage are delay in diagnosis and revascularization and extensive adjacent bone and soft-tissue trauma. Rapid diagnosis and repair are needed for all popliteal artery injuries and for infrapopliteal injuries that compromise distal flow. The optimal management of asymptomatic injuries of single shank arteries with normal flow in uninjured parallel vessels is not defined.

A decade of lower extremity venous trauma: patency and outcome.

Eighty-two patients with infrailiac venous injuries were reviewed. Veins were repaired in 75 patients and ligated in 7 patients. Clinical outcome could be correlated with anatomic patency for 41 patients who had postoperative venograms. Sixty-three percent of the repairs proved to be patent. Simple repairs were successful more often than complex ones. Site of injury and perioperative therapeutic adjuncts did not affect results. Eleven limbs were amputated, none solely because of venous injury. Edema was present at last follow-up in fewer patients with patent repairs than occluded repairs or ligations. Repair of major lower extremity venous injuries should be attempted routinely in stable patients. Improved patency of complex repairs may require increased use of balloon catheter thrombectomy and other adjunctive procedures. Postoperative venography is very useful for the evaluation of results.

Diagnosis of penetrating cervical esophageal injuries.

Arterial injuries pose the greatest early threat to the patient with penetrating neck trauma and esophageal injuries, the greatest late threat. Clinical evaluation reliably identifies 80 percent of esophageal injuries, which, in our opinion, is not adequate. In 118 minimally symptomatic or asymptomatic patients with penetrating neck trauma, the combination of esophagography with esophagoscopy identified all 10 esophageal injuries in 118 patients with penetrating neck trauma. These data suggest that patients with penetrating neck trauma and minimal clinical findings should be initially evaluated with arteriography and esophagography. If the results of arteriography or esophagography are positive, then neck exploration should be performed. If the results of esophagography are equivocal, then rigid esophagoscopy should be performed. If all test results are negative, then observation is justified.

Early steroid therapy for respiratory failure.

We performed a randomized double-blind trial to determine the usefulness of early methylprednisolone therapy for patients with pulmonary failure. We selected 81 acutely ill, mechanically ventilated patients at high risk for adult respiratory distress syndrome (ARDS). Thirty-nine patients received methylprednisolone, 30 mg/kg, every six hours for 48 hours; 42 patients received mannitol placebo. All patients were given a positive end-expiratory pressure of 5 cm H2O, monitored with pulmonary artery catheters, and treated for their primary disease processes. Twenty-five steroid-treated patients (64%) and 14 placebo-treated patients (33%) developed ARDS. Early infectious complications occurred in 30 steroid-treated patients (77%) and 18 placebo-treated patients (43%). There were no significant differences in factors predisposing to ARDS, ventilatory requirements, or days of intensive care. These results do not support the use of methylprednisolone for ARDS. Steroids failed to improve pulmonary function and were associated with an increased infection rate. Intensive pulmonary and general supportive care remain the preferred therapy for ARDS.

Civilian arterial trauma of the upper extremity. An 11 year experience in 267 patients.

Two hundred ninety-eight arterial injuries in 269 upper extremities were reviewed. Penetrating agents accounted for 250 injuries (93 percent) and blunt trauma for 19 (7 percent). Fifty-nine axillary, 126 brachial, 65 radial, and 48 ulnar arteries were damaged. Twenty-six extremities had more than one artery injured. The initial vascular examination revealed no abnormalities or was equivocal in 16 percent of all patients and in 32 percent of those with axillary artery injuries. Adjacent upper extremity structures were injured in 195 limbs (73 percent). Resection and primary anastomosis (54 percent) or vein interposition grafting (26 percent) were the most frequent methods of repair. Two deaths (0.7 percent) occurred and four amputations (1.5 percent) were required. Distal pulses were present at discharge in 93 percent of the evaluable extremities. Despite excellent success with arterial reconstruction, functional results were limited by associated nerve injuries. One hundred fifty patients (49 percent) had nerve deficits at discharge, and 71 (27 percent) had serious functional limitations.

Mortality and renal salvage after renovascular trauma. A review of 94 patients treated in a 20 year period.

Ninety-four patients with 96 renovascular injuries underwent operations over a 20 year period. Forty-nine patients had renal artery injury, 45 had isolated venous injury, and 33 had both vessels injured. Arterial revascularization succeeded in four patients, failed in five, and the results were not documented in three. Revascularization of acute renal artery thromboses was unsuccessful. Isolated renal vein injuries were repaired in 28 patients. The mortality rate was 37 percent for renal artery injuries and 28 percent for isolated renal vein injuries, despite the frequent choice of nephrectomy instead of reconstruction in unstable patients. Renal salvage was accomplished in 10 percent of patients with renal artery injury and in 51 percent of patients with isolated renal vein injury. Renal salvage is not often feasible in patients with renal arterial injuries because of associated renal vein injuries. The success of revascularization of traumatically occluded renal arteries is low and should probably be attempted only in unusual circumstances, such as bilateral injuries. Most isolated renal vein injuries are repairable, and reconstruction should be attempted in stable patients.