Endocrinopathy in POEMS syndrome: the Mayo Clinic experience

OBJECTIVE: To determine the prevalence and characteristics of endocrinopathies at diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome. PATIENTS AND METHODS: From January 1, 1960, through June 30, 2006, we identified 170 patients with POEMS syndrome in the Mayo Clinic dysproteinemia database. We abstracted information about endocrine abnormalities from their medical records. RESULTS: Of the 170 patients with POEMS syndrome during the entire study period, the 64 patients seen after 2000 had more complete endocrine evaluations; of these 64 patients, 54 (84%) had a recognized endocrinopathy (38 men; median age, 50 years; interquartile range, 43-59 years). Hypogonadism was the most common endocrine abnormality; 26 (79%) of 33 men had subnormal total testosterone levels, and 10 men had gynecomastia. Among the 35 patients with measured prolactin levels, 7 men and 3 women had elevated levels. Hypothyroidism was noted in 17 men and 11 women. Abnormalities in glucose metabolism were present in 24 (48%) of 50 patients; 16 patients had impaired fasting glucose levels, and 8 were diagnosed as having diabetes. Adrenal insufficiency (defined by an abnormal response of cortisol to stimulation with standard high-dose [250 microg] synthetic adrenocorticotropic hormone) was noted in 6 of 9 patients tested. Fourteen (27%) of 51 patients tested had hypocalcemia. Twenty-nine (54%) of 54 patients had evidence of multiple endocrinopathies in the 4 major endocrine axes (gonadal, thyroid, glucose, and adrenal). CONCLUSION: The high prevalence of endocrinopathy in our study, to our knowledge the largest published series of POEMS cases, calls for a thorough endocrine investigation in patients presenting with this syndrome.     

Laparoscopic cholecystectomy: early Mayo Clinic experience.

Prospective data and follow-up information were collected on the initial 200 patients who underwent laparoscopic cholecystectomy at the Mayo Clinic. The operation was completed laparoscopically in all but five patients, who required conversion to laparotomy because of dense scarring or stones in the common bile duct. The median surgical time was 85 minutes. The major postoperative complications were retained stones in the common bile duct (in seven patients), intraperitoneal hemorrhage that necessitated transfusion (in two patients), and intra-abdominal abscess and pulmonary infection (in one patient each). The median hospital stay was 1 day (range, 0 to 8 days), and the median times to full activity and normal employment were 8 days and 12 days, respectively. Laparoscopic cholecystectomy is associated with a low frequency of complications in most patients with symptomatic gallstones and allows a rapid return to normal activity. Currently, laparoscopic cholecystectomy is the treatment of choice for most patients with symptomatic cholelithiasis.     

Mediastinal lymphangioma: Mayo Clinic experience of 25 cases

OBJECTIVE: To describe the clinical findings, treatment outcomes, disease recurrence rates, and survival of patients with pathologically confirmed mediastinal and cervicomediastinal lymphangioma. PATIENTS AND METHODS: There are 2 patient cohorts. Cohort A consisted of 12 Mayo Clinic patients with pathologically confirmed medilastinal or cervicomediastinal lymphangioma identified from 1986 to 1999. Cohort B consisted of 13 additional patients with mediastinal lymphangioma who had been previously reported from the Mayo Clinic (from 1976 to 1986). All patients were retrospectively identified, and follow-up was performed by either telephone or medical record review. RESULTS: The mean age at the time of diagnosis was 36.5 years, with a male-female ratio of 1:3. All but 3 patients were symptomatic at presentation, with dyspnea being the most common symptom. Computed tomographic scans commonly revealed a homogeneous, low-attenuation mass that often Involved vascular or airway structures. Although 3 patients were initially observed, all patients had surgical intervention because of symptoms or enlargement of the mass. Thoracotomy with resection was the most common surgical intervention. Five recurrences were noted. Recurrence was minimized by complete excision of the lymphangioma. On follow-up that spanned 23 years, 75% of patients were alive. These survival rates were not statistically different from the expected survival rates of the same age- and sex-matched controls. Only 1 death was attributed to complication from lymphangioma. CONCLUSION: Mediastinal and cervicomediastinal lymphangioma are rare lesions that can be treated successfully with surgical excision. Prognosis appears to be excellent because no difference in survival was found between patients and age- and sex-matched controls.     

Robot-assisted laparoscopic cholecystectomy: initial Mayo Clinic Scottsdale experience

OBJECTIVE: To review a single institution's experience with robot-assisted laparoscopic cholecystectomy. PATIENTS AND METHODS: We reviewed retrospectively all cholecystectomies performed with the assistance of a surgical robot at the Mayo Clinic in Scottsdale, Ariz, from October 2002 to July 2003. Cholanglograms were obtained intraoperatively for patients with elevated results on liver function tests. The gallbladder was removed by the surgeon with the use of the robot. A surgical assistant at the operating table exchanged instruments in the robot arms and applied clips when needed. Total operating time, assembly time of the robot, complications, and postoperative course were evaluated. RESULTS: Nineteen patients underwent robot-assisted laparoscopic cholecystectomy; 16 had symptomatic cholelithiasis, 2 had gallbladder polyps, and 1 had acute cholecystitis. Of the 19 surgeries, 16 were completed successfully with robotic assistance. In 3 consecutive patients, a mechanical problem occurred with the robot; however, all procedures were completed laparoscopically. The mean set-up time, including patient positioning and preparation and robotic installation, was 28.1 minutes. The mean +/- SD operating time was 82.3+/-17.9 minutes without a cholanglogram and 102+/-20.9 minutes with a cholanglogram. There were no complications and no conversions to an open procedure. CONCLUSION: Robotic surgery offers many potential advantages, including surgeon comfort, elimination of surgeon tremor, improved imaging, and increased degrees of freedom of the operative Instruments, compared with conventional laparoscopic surgery. However, patient outcomes and operative costs need to be evaluated further.     

Fulminant hepatitis: Mayo Clinic experience with 34 cases

From 1974 through 1982, fulminant hepatitis was diagnosed in 34 patients at our institution. Of these patients, only two survived (survival rate, 6%). This syndrome was caused by viruses (B and non-B hepatitis and herpes simplex) in 23 patients, hepatotoxic drug in 6, Wilson's disease (hepatolenticular degeneration) in 3, and industrial poisons in 2. Most of the patients died within 10 days after the onset of encephalopathy. The poor prognosis in our group of patients was probably related to the preponderance of older patients and cases caused by non-B hepatitis virus. In our patients, the clinical course was complicated by renal failure, ascites, bleeding, sepsis, pancreatitis, and seizures. The major cause of death was hepatic failure.     

Adrenal hemorrhage: a 25-year experience at the Mayo Clinic

OBJECTIVE: To characterize the clinical course of adrenal hemorrhage (AH) by using a systematic review of the presentation, associated conditions, and outcomes in patients with AH seen at our institution between 1972 and 1997 (a 25-year period). PATIENTS AND METHODS: A computer search of recorded dismissal diagnoses identified 204 patients with a diagnosis of AH, but only 141 fulfilled our study criteria. Their records were analyzed systematically by presentation, bilateral or unilateral hemorrhage, corticosteroid treatment, and survival. RESULTS: AH is a heterogeneous entity that occurs in the postoperative period, in the antiphospholipid-antibody syndrome, in heparin-associated thrombocytopenia, or in the setting of severe physical stress and multiorgan failure. Standard laboratory evaluation is not helpful in establishing the diagnosis. Of the 141 cases of AH, 78 were bilateral, and 63 were unilateral. Corticosteroid treatment in situations of severe stress or sepsis had little effect on outcome (9% vs. 6% survival with and without corticosteroid treatment, respectively). This is in sharp contrast to AH occurring postoperatively (100% vs. 17% survival with or without treatment, respectively) or in the antiphospholipid-antibody syndrome (73% vs. 0% survival, respectively). CONCLUSIONS: A high index of suspicion is required to make a timely diagnosis of AH. Fever and hypotension in the appropriate clinical setting necessitate further investigation. Although the diagnosis of AH is infrequently made while the patient is alive, appropriate imaging techniques are useful for establishing a timely diagnosis. In severe physical stress or sepsis, AH may be a marker of severe, preterminal physiologic stress and poor outcome.     

Carcinosarcoma of the lung: Mayo Clinic experience and response to chemotherapy

The clinical presentation, clinical course, and results of various treatment modalities of 17 patients with carcinosarcoma of the lung were reviewed. This group of patients was 0.2% of all Mayo Clinic patients with lung cancer who had been treated between 1971 and 1982. Most patients were men in the sixth decade of life who had a history of smoking. Ten of 17 neoplasms were located in the upper lobes. Noninvasive diagnostic tests had a low yield in detecting carcinosarcomas. Pulmonary resection with curative intent was performed in 15 of 17 patients; however, only 4 patients were alive at 6, 8, 28, and 39 months, respectively, postoperatively. The median survival was 1 year. Doxorubicin-based chemotherapeutic programs produced an objective response in two of four patients.     

Severe hypoxemia associated with liver disease: Mayo Clinic experience and the experimental use of almitrine bismesylate

Severe hypoxemia associated with chronic liver disease is an uncommon disorder most likely due to an intrapulmonary vascular abnormality that has characteristics of both ventilation-perfusion mismatching and diffusion limitation. Anatomically, the intrapulmonary vascular abnormalities can occasionally be detected by angiography. Physiologically, the gas exchange abnormalities can be substantiated by contrast-enhanced two-dimensional echocardiography. Although orthodeoxia and platypnea have frequently been found in these patients, echocardiographic data suggest that vascular abnormalities can exist in the absence of orthodeoxia. We describe 11 patients who had severe hypoxemia and chronic liver disease and review their pulmonary angiographic, contrast echocardiographic, and arterial blood gas findings. Among five of these patients who were given almitrine bismesylate, an experimental medication thought to alter ventilation-perfusion relationships in patients with chronic obstructive pulmonary disease, one had improved oxygenation. We recommend that patients with hypoxemia associated with chronic liver disease have detailed studies to rule out reversible forms of hypoxemia and that those with severe hypoxemia undergo testing to determine the existence of intrapulmonary vascular abnormalities, especially if liver transplantation is considered.     

Medulloblastoma--prognostic factors and outcome of treatment: review of the Mayo Clinic experience

From March 1965 through December 1984, 58 patients (35 male and 23 female patients; median age, 17 years) with posterior fossa (PF) medulloblastoma underwent surgical treatment and postoperative radiation therapy at our institution. Radiation fields were the craniospinal axis in 39 patients, PF plus spinal axis in 12, PF in 6, and whole brain in 1. Median radiation doses were 43 Gy (22 to 60 Gy) to the PF and 34 Gy (6.2 to 50 Gy) to the spinal axis. Overall 5- and 10-year survivals were 50% and 33%, respectively; 5- and 10-year relapse-free survivals were 46% and 32%. Treatment failed in 34 patients (59%): in 18 who had irradiation to the craniospinal axis (13 had received 50 Gy or less to the PF) and in 16 who had a radiation field of less than the craniospinal axis. A statistically significant (P less than 0.05) improvement in 10-year survival was associated with the following prognostic variables: PF dose of 50 Gy or more, whole-brain irradiation, and spinal axis irradiation. In comparison with subtotal resection, total resection was correlated with better 10-year relapse-free survival but not overall survival. All five patients with initial treatment failure only in the spine had received a radiation dose of 30 Gy or less to the spinal axis. The 2-year survival after relapse was 46% with salvage chemotherapy or irradiation in 23 patients and 0% in the 11 patients who received no further treatment (P less than 0.01).     

Pulmonary arteriovenous fistulas: Mayo Clinic experience, 1982-1997.

OBJECTIVE: To describe the results of analysis of clinical, physiologic, diagnostic, and therapeutic aspects and complications in patients with pulmonary arteriovenous fistulas (PAVFs). PATIENTS AND METHODS: Retrospective review of medical records of all patients with the diagnosis of PAVF evaluated at Mayo Clinic Rochester from 1982 through 1997. Demographic characteristics, presence or absence of hereditary hemorrhagic telangiectasia, clinical features, and results of imaging studies and blood gas analyses, treatments, and complications related to PAVFs were reviewed. RESULTS: Among the 93 patients, 44 were male and 49 female. The mean age at the time of evaluation was 40 years (range, 5-83 years). Fifteen patients (16%) were asymptomatic. History of hereditary hemorrhagic telangiectasia was present in 52 patients (56%). Notable clinical findings included epistaxis in 46 (49%), hemoptysis in 14 (15%), cyanosis in 27 (29%), clubbing in 18 (19%), dyspnea in 53 (57%), and pulmonary bruits/murmurs in 32 (34%). Chest x-ray films with or without tomograms showed abnormal findings in 87 (94%), of which 68 (73%) suggested PAVF. Polycythemia was detected in 12 (13%). Pretherapy arterial PO2 measured on room air averaged 56 mm Hg (range, 32-95 mm Hg), and the posttherapy PO2 averaged 77 mm Hg (range, 46-110 mm Hg). Echocardiography with indocyanine green dye was diagnostic of extracardiac right-to-left shunt in 26 (90%) of 29 patients tested. Diagnostic studies revealed single lesions in 32 patients (34%) and multiple lesions in 61 (66%). The most prominent complications of the disease were neurologic events in 34 patients (37%). These complications included transient ischemic attacks, hemiplegia, brain abscesses, and seizures. Surgical resection alone was carried out in 18 patients (19%), embolization therapy alone in 41 (44%), and both therapies in 7 (8%). The 48 patients treated with embolization required 78 embolization sessions with more than 200 lesions occluded. Complications of treatment included postembolization hemothorax in 1 patient and right-sided hemiparesis in another patient. Follow-up disclosed that 1 patient died from PAVF-related complications. CONCLUSIONS: Among our patients with PAVFs, hereditary hemorrhagic telangiectasia was observed in more than half and neurologic complications in more than one third. Because of the considerable risk of neurologic and other complications, definitive treatment should be considered in patients with PAVFs. Embolization is currently the preferred treatment in most patients. Frequent follow-up of treated patients is necessary because PAVFs tend to increase both in number and in size over time.     

Quest for quality: department of laboratory medicine and pathology, Mayo Clinic.

This article describes our journey for quality in the Department of Laboratory Medicine and Pathology (DLMP) at Mayo Clinic. It provides the background of the department and the process for the development and implementation of the quality program. In addition, a quality conference and the development of a quality school are outlined. Throughout the course of this process, valuable lessons were learned and are discussed. We are pleased with the success of the quality journey. However, we realize that the quest has just begun. We look forward to the future and the challenges that lie ahead.     

Effect of blood conservation efforts in cardiac operations at the Mayo Clinic

In a retrospective study of 388 patients who had undergone cardiac operations at our institution during two time periods-before (1982) and after (1984) introduction of autologous transfusion-we analyzed the effect of blood conservation efforts and autologous transfusion on blood usage, postoperative complications, and duration of hospitalization. Cell salvage techniques resulted in a significant reduction (P less than 0.0001) in use of not only homologous blood (from a mean of 9.6 units per patient in 1982 to 3.2 units in 1984) but also fresh-frozen plasma and platelet concentrates. We found no significant difference in morbidity or mortality for the two study periods. Although the mean duration of hospitalization decreased from 11.7 days in 1982 to 9.6 days in 1984, this change was probably related to factors other than the introduction of blood conservation efforts. Thus, techniques used to decrease the amount of blood replacement needed for cardiac surgical procedures are beneficial.     

Survival of home parenteral nutrition-treated patients: 20 years of experience at the Mayo Clinic

OBJECTIVE: To present the largest single institutional review of demographics, associated primary diseases, and survival of patients receiving home parenteral nutrition (HPN). MATERIAL AND METHODS: We conducted a retrospective review of medical records of all Mayo Clinic patients treated with HPN between 1975 and 1995. The probability of survival was calculated by using Kaplan-Meier analysis. RESULTS: In the 225 study patients requiring HPN (median age, 51 years), the main underlying primary diseases were as follows: inflammatory bowel disease (IBD) (N = 50), nonterminal active cancer (N = 39), ischemic bowel (N = 35), radiation enteritis (N = 32), motility disorder (chronic pseudo-obstruction) (N = 26), and adhesive intestinal obstruction (N = 18). Other conditions included intestinal and pancreatic fistula, refractory sprue, dumping syndrome, and protein-losing enteropathy. The overall probability of 5-year survival during HPN was 60%. The probability of survival at 5 years based on the primary disease was 92% for IBD, 60% for ischemic bowel, 54% for radiation enteritis, 48% for motility disorder, and 38% for cancer. The probability of 5-year survival stratified by age at initiation of HPN was as follows: younger than 40 years, 80%; 40 through 60 years, 62%; and older than 60 years, 30%. Most deaths during therapy with HPN were attributable to the primary disease. Among the 20 patients who died of an HPN-related cause, 11 deaths were from catheter sepsis, 4 from liver failure, 2 from venous thrombosis, and 2 from metabolic abnormalities. CONCLUSION: Survival of HPN-treated patients is best predicted on the basis of the primary disease and the age at initiation of HPN. Patients with IBD and age younger than 40 years have a better 5-year survival in comparison with other groups. Most deaths during treatment with HPN are a result of the primary disease; HPN-related deaths are uncommon.     

Early outcome after placement of a metallic intracoronary stent: initial Mayo Clinic experience

After percutaneous transluminal coronary angioplasty, 3 to 7% of patients have in-hospital coronary closure, and the risk of subsequent myocardial infarction is high, even with an urgent bypass operation. Disrupted coronary morphologic integrity, particularly with large dissections, may be associated with an increased risk of acute coronary closure. A percutaneously placed coronary vascular stent may rapidly alleviate acute or threatened coronary closure and could reduce morbidity. Between October 1989 and June 1990, placement of a balloon-expandable, flexible metallic coil (Gianturco-Roubin) coronary stent was attempted 16 times in 15 Mayo Clinic patients--for treatment of actual or threatened abrupt coronary closure in 10 patients, for primary treatment of a lesion judged to be at high risk for dissection with conventional balloon angioplasty in 1, and for elective treatment of restenotic lesions in 5. Placement of the stent was successful in 15 attempts (94%). Associated complications, which were uncommon, were similar to those noted after balloon angioplasty. No early deaths occurred, and no patient required coronary artery bypass grafting. Stents were successfully placed in both urgent and elective circumstances in native coronary arteries and saphenous vein grafts, and they were used in primary atheromatous and restenotic lesions. Our initial experience with this metallic coil stent indicates that it is efficacious for vascular disruption that is threatening or causing coronary closure after angioplasty. Furthermore, elective placement of a stent may be safely undertaken in patients with high-risk coronary lesions or recurrent restenotic lesions. The long-term outcome in all groups of patients who receive coronary stents is unknown.     

Biliary bypass for ductal adenocarcinoma of the pancreas: Mayo Clinic experience, 1970-1975

During a recent 6-year period at the Mayo Clinic, 151 biliary bypass procedures for ductal adenocarcinoma of the pancreas were done, with operative mortality of 6.0% and an acceptable operative morbidity. Relief of jaundice was universal among survivors. Median survival was 6.0 months, and 3-year survival was 2%. These figures are compared with those for resective procedures.