Acute Myocardial Infarction Attributable to Adrenergic Crises in a Patient with Pheochromocytoma and Neurofibromatosis 1

ObjectiveTo describe a rare case of acute myocardial infarction in a patient with neurofibromatosis 1 and pheochromocytoma and to review the literature on the coexistence of these 2 diseases, the causes of myocardial injury in patients with pheochromocytoma, and the utility of genetic testing and pheochromocytoma screening for those patients and their families.MethodsWe present a case report, including the detailed clinical, laboratory, and radiographic data, results of adrenal mass pathology, and results of coronary angiography. We also survey other relevant reports available in the literature.ResultsA 43-year-old woman with a history of longstanding hypertension, neurofibromatosis 1, headaches, sweating, and palpitations presented to the hospital with chest pain and shortness of breath. She was found to have an acute myocardial infarction and pulmonary edema, as well as a right adrenal mass. A pheochromocytoma was suspected, and phenoxybenzamine was added to her treatment regimen. Cardiac catheterization showed nonobstructive coronary disease. The levels of plasma catecholamine metabolites were extremely high. The patient underwent uncomplicated laparoscopic right adrenalectomy 2 weeks after this admission. Surgical pathology confirmed the diagnosis of pheochromocytoma.ConclusionAdrenergic crisis attributable to pheochromocytoma can result in acute myocardial infarction even in the absence of obstructive coronary disease. Inclusion of pheochromocytoma in the differential diagnosis of hypertension in patients with neurofibromatosis is very important and helps avoid mistakes in the management of such patients. (Endocr Pract. 2007;13:269-273)     

Hemorrhagic Shock As the Initial Manifestation of Pheochromocytoma: Report of A Sequential Management Strategy

ObjectiveTo describe a patient presenting with hemorrhagic shock attributable to bleeding pheochromocytomas and the sequential management strategy used for treating this patient.MethodsWe summarize the clinical presentation,diagnostic work-up, surgical management, and pathologicfeatures of our patient and review the pertinent literature.ResultsA 38-year-old man with multiple endocrineneoplasia type 2A and bilateral pheochromocytomas presented initially with nearly fatal retroperitoneal and intraperitoneal hemorrhage rather than the characteristic hypertensive paroxysms. After lifesaving operative interventionand a 5-month period of rehabilitation and convalescence,the patient underwent bilateral retroperitoneoscopic adrenalectomy as definitive treatment. Thus, the abdomen thathad been operated on multiple times because of hemorrhage was left undisturbed, and the patient had a successfulrecovery.ConclusionNear-fatalintraperitonealhemorrhageis a very rare initial manifestation of pheochromocytoma.Our current patient with bilateral pheochromocytomaspresented in this dramatic manner. This case shows that a sequential management strategy of damage-control surgical treatment followed by future resection of the tumorsafter appropriate a-adrenergic blockade is a safe and effective therapeutic option. (Endocr Pract. 2012;18:e81-e84)     

Pheochromocytoma-Induced Aggression?

ObjectiveTo document a case of pheochromocytoma with an unusually high plasma ratio of norepinephrine to epinephrine concentrations (NE:E), and a history of violent and aggressive behavior (which has been reported to be associated with increased NE:E ratios).MethodsWe present the history of present illness, history of aggressive behavior, and the clinical course of a man who was found to have pheochromocytoma with a remarkable catecholamine profile. We also review the literature on the relationship of catecholamine ratios to behavior.ResultsA 33-year-old man presented to the emergency department with the chief complaint of palpitations and chest pain. A physical exam revealed markedly elevated blood pressure. On admission, a computed tomographic scan of the abdomen revealed a 10 by 10-cm heterogeneous mass of 20 Hounsfield units superior to the right kidney. His plasma NE:E ratio was 35, and his 24-hour urine ratio of normetanephrine to metanephrine concentrations was greater than 26. The tumor was successfully removed with laparoscopic adrenalectomy, and the histologic findings revealed benign pheochromocytoma. There was no immediate change in the patient’s behavior. He was incarcerated the week after surgery, and lost to follow-up.ConclusionPrimarily norepinephrine-producing pheochromocytoma may have contributed to this patient’s violent and aggressive behavior. Catecholamine levels may remain elevated for 1 week following surgery. Even if this patient’s norepinephrine level had dropped rapidly after removal of the pheochromocytoma, and was not elevated a week later when he was arrested, it is possible that his aggressive behavior may have been conditioned by long exposure to elevated levels of norepinephrine. (Endocr Pract. 2011;17:e126-e129)     

Pheochromocytoma: A Cause of St-Segment Elevation Myocardial Infarction,Transient Left Ventricular Dysfunction,and Takotsubo Cardiomyopathy

ObjectiveTo report the case of a patient with a pheochromocytoma and apical left ventricular dysfunction that resolved after surgical resection of the pheochromocytoma, to review the effects of catecholamines on myocyte function and the concept that takotsubo cardiomyopathy (TC) is caused by excess catecholamines, and to illustrate the difficulty in the management of an acute coronary syndrome (ACS) during a hypertensive crisis attributable to a pheochromocytoma.MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies in a 60-year-old man with an ACS, TC, and an incidentaloma later diagnosed to be a pheochromocytoma. The association with TC and the pertinent literature are reviewed.ResultsA 60-year-old man was suspected of having myocardial ischemia on the basis of symptoms of paroxysmal chest pain extending to the left shoulder, diaphoresis, ST-segment elevation on an electrocardiogram, and elevated serial levels of cardiac enzymes. Coronary angiography did not reveal substantial coronary artery obstruction but detected ballooning of the apical, anterior, and inferior cardiac walls, consistent with TC. He had a history of labile hypertension and palpitations of 3 months’ duration. An adrenal mass detected on a prior computed tomographic scan and increased 24-hour urine catecholamine levels were consistent with a pheochromocytoma. Treatment with phenoxybenzamine was initiated, and he underwent a right adrenalectomy, which confirmed that the tumor was a pheochromocytoma and dramatically improved the patient’s condition.ConclusionPheochromocytomas manifest with labile blood pressures and should be considered in the differential diagnosis of ACS. This case also supports the concept that TC is caused by excess catecholamines. (Endocr Pract. 2012;18:e77-e80)     

Pheochromocytoma and Tetralogy of Fallot: A Rare but Potentially Dangerous Combination

ObjectiveTo describe a case of pheochromocytoma (PHEO) with tetralogy of Fallot (TOF) and discuss the difficulties encountered during the management of this patient, with a review of the literature.MethodsWe report the clinical course, imaging, and management issues of our patient and review relevant literature.ResultsA 14-year-old female who was known to have TOF presented with classical paroxysmal symptoms and worsening dyspnea. She was diagnosed as having epinephrine-secreting PHEO based on biochemical, radiologic, and functional imaging. She was treated with an α-1 blocker for control of paroxysms but developed severe cyanotic spells. She required addition of a calcium-channel blocker for control of the paroxysms and underwent successful cardiac repair.ConclusionTreatment of the combination of cyanotic congenital heart disease (CCHD) and PHEO requires an individualized and multidisciplinary approach with judicious use of available medications. This is the first case of uncorrected TOF and epinephrine-secreting PHEO. Our case also reiterates the need for further studies to better understand the pathophysiologic link between PHEO/paraganglioma and CCHD. (Endocr Pract. 2014; 20:e80-e85)     

Synchronous Parathyroid Carcinoma,Parathyroid Adenoma,and Papillary Thyroid Carcinoma in a Patient with Severe and Long-Standing Hyperparathyroidism

ObjectiveTo describe a patient presenting with the rare constellation of synchronous parathyroid carcinoma, parathyroid adenoma, and papillary thyroid carcinoma.MethodsWe summarize the clinical presentation, diagnostic work-up, surgical management, and pathologic features of our patient and review the pertinent literature.ResultsThe patient was a 59-year-old man who presented with severe clinical manifestations of long-standing primary hyperparathyroidism, a serum calcium concentration of 14.4 mg/dL, and a parathyroid hormone level of 2,023 pg/mL. He was found to have a 3.4-cm parathyroid carcinoma on the left side and a 3.2-cm papillary carcinoma in the right thyroid lobe. In addition, a 917-mg parathyroid adenoma was found on the right side.ConclusionSynchronous parathyroid and thyroid carcinomas are extremely rare. To our knowledge, our patient is the first documented case with a parathyroid adenoma in addition to synchronous parathyroid and thyroid carcinomas. The presence of concurrent parathyroid carcinoma and parathyroid adenoma can cause diagnostic confusion and should be considered in patients presenting with severe hyperparathyroidism. Any concomitant thyroid nodules must be investigated to rule out thyroid carcinoma. (Endocr Pract. 2009;15:463-468)     

Hypertensive Crisis,Catecholamine Cardiomyopathy,and Death Associated with Pseudoephedrine use in a Patient with Pheochromocytoma

ObjectiveTo describe a woman with a previously unrecognized pheochromocytoma who died after ingesting over-the-counter pseudoephedrine-containing medications.MethodsWe present a case report including laboratory, radiographic, and pathologic findings in a patient with a previously unrecognized pheochromocytoma.ResultsA 31-year-old woman had symptoms consistent with intermittent, excessive release of catecholamines since childhood. She developed an upper-respiratory infection and used over-the-counter medications containing pseudoephedrine. Subsequently, she developed a hypertensive crisis with congestive heart failure and died of a cardiac arrhythmia and shock. Findings from postmortem examination included a right adrenal pheochromocytoma, congestive heart failure, and catecholamine cardiomyopathy.ConclusionThis patient death may be linked to the use of pseudoephedrine hydrochloride. Physicians and pharmacists should warn patients with known pheochromocytoma or those at risk for having pheochromocytoma on the basis of family history or genetic testing that pseudoephedrine use may be harmful. Over-the-counter packaging should include such warning. (Endocr Pract. 2008; 14:93-96)     

Rhabdomyolysis after Withdrawal of Thyroid Hormone in a Patient with Papillary Thyroid Cancer

ObjectiveTo report a case of rhabdomyolysis presenting with severe hyperkalemia after withdrawal of thyroid hormone in a patient with differentiated thyroid cancer.MethodsWe describe the clinical and laboratory findings of the study patient and review the relevant literature.ResultsA 54-year-old man with progressive generalized weakness and myalgias presented with acute renal failure and hyperkalemia. He had undergone total thyroidectomy for papillary thyroid cancer 6 weeks earlier and had discontinued thyroid hormone 2 weeks before his current presentation in preparation for thyroid remnant ablation. He had a history of multiple colon and small-bowel resections for familial adenomatous polyposis and desmoid tumor. He was severely dehydrated on examination. Laboratory tests results included the following values: creatine phosphokinase, 5265 U/L (reference range, 52-336 U/L); creatinine, 2.1 mg/dL; potassium, > 8.0 mEq/L; and thyrotropin, 92.2 mIU/L. His condition was diagnosed as rhabdomyolysis, and his fluid deficit and hyperkalemia were treated aggressively. Cardiac status remained stable, and both acute renal failure and hyperkalemia improved. He then received remnant ablation, and thyroid hormone was restarted. His muscle complaints resolved over the following 3 months.ConclusionsHypothyroidism-induced rhabdomyolysis can occur during thyroid hormone withdrawal and can present with life-threatening hyperkalemia. Patients undergoing thyroid hormone withdrawal should be assessed for risk of rhabdomyolysis, and preventive strategies should be implemented, including prevention of dehydration.The use of recombinant thyrotropin, rather than thyroid hormone withdrawal, should be considered in those who are at high risk for such complications. (Endocr Pract. 2008;14:1023-1026)     

Necessity for Long-Term Follow-Up of Patients With Head and Neck Paraganglioma and Mutation in the Succinate Dehydrogenase Genes: An Index Case Report and Literature Review

ObjectiveTo describe a patient with hereditary head and neck paraganglioma (HNPGL) and to review the literature on these rare tumors.MethodsWe review the English-language literature regarding SDH mutations, HNPGL, hereditary paraganglioma-pheochromocytoma syndrome, and the role of functional imaging in the follow-up of these tumors. We also describe the clinical findings, imaging results, and follow-up of a man who initially presented with HNPGL and subsequently developed metastatic pheochromocytoma 20 years later.ResultsA 66-year-old man presented with a history of hypertension, palpitations, sweating, and elevated urinary norepinephrine. Iodine-123-metaiodobenzylguanidine (¹²³I-MIBG) scan demonstrated a left suprarenal mass and multiple avid lesions in the abdomen, chest, and posterior cranial fossa. Histologic examination confirmed aSubmitted for publication February 25, 2012 Accepted for publication May 14, 2012To purchase reprints of this article, please visit: www.aace.com/reprints. Copyright © 2012 AACE.metastatic pheochromocytoma, and molecular genetic testing revealed a mutation in the SDHD gene. The patient had had surgery 20 years earlier for HNPGL. Although most HNPGLs arise sporadically, susceptibility genes have been identified in approximately one-third of cases. Optimal follow-up remains controversial. We reiterate a need for longterm follow-up of patients with a mutation in an SDH gene. ¹²³I-MIBG, highly specific for identifying ectopic neuroendocrine tissue, may have a role in long-term follow-up.ConclusionsAlthough HNPGLs rarely metastasize, their malignant potential is difficult to predict. Routine surveillance for at-risk patients is recommended. Patients with a mutation in an SDH gene should therefore undergo regular surveillance. (Endocr Pract. 2012;18:e130-e134)     

Iatrogenic Osteoporosis,Bilateral Hip Osteonecrosis,and Secondary Adrenal Suppression in an Hiv-Infected Man Receiving Inhaled Corticosteroids and Ritonavir-Boosted Highly Active Antiretroviral Therapy

ObjectiveTo report the first case of severe osteoporosis associated with a vertebral pathologic fracture and osteonecrosis of femoral heads in an HIV-infected man receiving inhaled corticosteroids and ritonavir-boosted antiretroviral therapy.MethodsWe describe an HIV-infected man with severe osteoporosis, bilateral hip osteonecrosis, and secondary adrenal suppression, including detailed clinical, laboratory, and radiographic data, and review the related literature.ResultsA 60-year-old man with a 15-year history of HIV infection and a medical history of long-standing bronchiectasis treated with inhaled corticosteroids and hypogonadism treated with testosterone was referred to the endocrinology clinic after experiencing an osteoporotic vertebral fracture. He was taking ritonavir-boosted antiretroviral therapy. Osteonecrosis of both hips was also diagnosed, which required total hip replacement therapy.Laboratory evaluation revealed adrenal insufficiency due to increased effect of exogenous inhaled steroids and no other secondary causes of osteoporosis. A bone densitometry study showed osteoporosis of both hips and the lumbar spine. He was treated with intravenous pamidronate. During treatment, he developed bilateral femoral fractures after minor trauma.ConclusionsGiven the potential for increased serum levels of inhaled corticosteroids in patients taking ritonavirboosted highly active antiretroviral therapy, attention must be paid to the risk of bone loss in HIV-infected patients taking inhaled corticosteroids. Prescribing calcium and vitamin D supplementation and considering early osteoporosis screening are reasonable measures for this patient population. Interaction between inhaled corticosteroids and ritonavir may increase risk of hypothalamus-pituitary-adrenal axis suppression. (Endocr Pract. 2011;17:74-78)     

Thymic Hyperplasia and Graves Disease: Management of Anterior Mediastinal Masses in Patients with Graves Disease

ObjectiveTo describe a case of an anterior mediastinal mass (AMM) in a patient with Graves disease.MethodsWe report the clinical presentation, diagnosis, management, and outcome of a 34-year-old man with dyspnea on exertion.ResultsInitial evaluation of the patient’s complaints revealed a large AMM on chest radiography and then chest computed tomography. After occurrence of additional symptoms, the patient was diagnosed as having Graves disease and treated with antithyroid medications. Despite an appropriate biochemical response, he continued to experience severe dyspnea on exertion. A repeated computed tomographic scan 8 weeks after initiation of therapy showed no appreciable decrease in size of the AMM. He elected to undergo thymectomy. An intraoperative phrenic nerve injury resulted in a paralyzed left hemidiaphragm, leaving the patient with considerable difficulties in his career and profoundly decreased exercise tolerance.ConclusionThe differential diagnosis of an AMM includes several malignant lesions with a risk often warranting early surgical excision. In light of the association of benign thymic hyperplasia with Graves disease, thymectomy may be delayed in expectation of thymic regression with medical therapy. The timing of regression is variable, and very few reports exist in the literature. In our current case, the patient opted for thymectomy relatively early and had an unfortunate complication. The lack of clinical evidence regarding management of an enlarged thymus in patients with Graves disease, however, makes management decisions more difficult. (Endocr Pract. 2009;15: 534-539)     

Intravascular Lymphoma: an Unusual Diagnostic Outcome of an Incidentally Detected Adrenal Mass

ObjectiveTo describe a rare diagnosis of intravascular lymphoma in a patient presenting with an incidentally discovered adrenal mass.MethodsWe describe the patient’s clinical history and the findings from biochemical evaluation, radiologic studies, and surgical pathology and review the relevant literature.ResultsA 43-year-old woman developed sudden onset of flank pain associated with a flushing sensation and presented to the emergency department where computed tomography showed a 5-cm left adrenal mass. She had normal electrolytes, and serum and urinary test results were negative for pheochromocytoma. A 24-hour urinary cortisol level was minimally elevated, and the midnight salivary cortisol value was within the reference range. Magnetic resonance imaging revealed a 6.5 × 5.8-cm left adrenal lesion that demonstrated moderate T2-weighted signal and gradual delayed enhancement with no drop in signal on out-of-phase images. Since the lesion lacked high intensity and the biochemical testing results did not suggest a pheochromocytoma, it was deemed likely that the mass was a malignant lesion of the left adrenal gland. A laparoscopic left adrenalectomy was performed. Morphologic and immunohistochemical findings were consistent with a large B-cell lymphoma, which by virtue of its near exclusive distribution in vascular spaces, was consistent with the diagnosis of intravascular large B-cell lymphoma.ConclusionIntravascular large B-cell lymphoma should be included in the differential diagnosis of an incidentally detected adrenal mass even though the diagnosis is rare. (Endocr Pract. 2008;14:884-888)     

Idiopathic Sporadic Tumoral Calcinosis of the Hip: Successful Oral Bisphosphonate Therapy

ObjectiveTo report the case of a woman with idiopathic sporadic tumoral calcinosis treated successfully with orally administered bisphosphonates.MethodsWe report the clinical, laboratory, and imaging findings and describe the clinical course of tumoral calcinosis. The literature was reviewed for the pathophysiologic features and strategies for treatment of tumoral calcinosis. In addition, we specifically reviewed the use of bisphosphonates in tumoral calcinosis and the possible pharmacologic basis for the therapeutic benefit.ResultsA 45-year-old woman presented with a 6-week history of left-sided pain in the hip in conjunction with stiffness after a trivial fall 4 weeks before the onset of symptoms. The findings on conventional radiology of the hip joint were consistent with tumoral calcinosis of the left hip. The biochemical profile of the patient was unremarkable. Oral treatment with alendronate, 70 mg once a week, yielded alleviation of symptoms within 12 weeks. Radiology of the left hip repeated after a period of 15 months revealed notable regression of the calcified lesion.ConclusionBisphosphonate therapy may be considered as an alternative to surgical treatment in patients with idiopathic sporadic tumoral calcinosis. (Endocr Pract. 2007;13:182-186)     

Bariatric Surgery and the Role of the Clinical Endocrinologist: 2011 Update

ObjectiveTo discuss the emerging roles of bariatric surgery and clinical endocrinology within the context of obesity and diabetes mellitus comprehensive care plans and cost-effective strategies.MethodsRelevant literature is reviewed and clinical cases are presented.ResultsThe global obesity epidemic poses many challenges to clinical endocrinologists and has fomented a coordinated effort among specialists to revolutionize management paradigms. Technologic innovation drives the need for accelerated learning and research efforts in bariatric surgery. The national shortage of physicians with expertise in nutritional medicine compounds the management problems for this expanding patient population. Certain issues merit continued attention and research, such as gastric banding for mild obesity, surgery for treatment of diabetes, sleeve gastrectomy, and nutritional and metabolic consequences.ConclusionClinical endocrinologists should have a central role in the perioperative decision-making for patients undergoing bariatric surgery. (Endocr Pract. 2011;17:788-797)     

Mixed Corticomedullary Carcinoma of the Adrenal Gland: A Case Report

ObjectiveTo report the case of a 78-year-old woman with mixed corticomedullary carcinoma of the adrenal gland, and to review other reported lesions that exhibit clinical and/or histopathologic features of both adrenal cortical and medullary differentiation.MethodsWe describe the patient’s clinical findings and laboratory test results, as well as the gross and histopathologic features of her tumor. We also review the literature pertaining to mixed corticomedullary adenomas and cortical tumors with clinical features of pheochromocytoma, and vice versa.ResultsA 78-year-old woman with a 10-cm left adrenal mass was hospitalized for management of hypertensiveurgency. Laboratory workup revealed elevated urinary metanephrine excretion and elevated serum dehydroepiandrosterone sulfate levels. She underwent left adrenalectomy. Pathologic examination of the lesion showed mixed cortical and medullary histologic characteristics, as well as gross and microscopic evidence of malignancy. Including the present case, we identified 17 cases of neoplasms that exhibit features of mixed corticomedullary differentiation.ConclusionsThis report represents the first documented case of mixed corticomedullary carcinoma. Several benign lesions combine clinical, biochemical, and/or histopathologic evidence of both adrenal cortical and medullary differentiation, including mixed corticomedullary adenomas and corticotropin-secreting pheochromocytomas. The differential diagnosis of a lesion with mixed cortical and medullary features should also include a malignant neoplasm. (Endocr Pract. 2012;18:e37-e42)     

Pheochromocytoma Presenting as Takotsubo-Like Cardiomyopathy Following Delivery

ObjectiveDiagnosis of pheochromocytoma during pregnancy can be difficult, and the tumor carries an unfavorable prognosis if not diagnosed and treated in a timely manner.MethodsTo present a case of Takotsubo-like cardiomyopathy characterized by transient left ventricular apical ballooning due to pheochromocytoma following delivery.ResultsA few hours after Caesarean section, a 32-year-old Caucasian female presented with pulmonary edema followed by cardiac arrest with echocardiographic and ventriculographic evidence of reversible acute myocardial failure characteristic of Takotsubo-like cardiomyopathy. A previously unrecognized adrenal pheochromocytoma was found during her clinical work-up. Left ventricle (LV) function normalized after surgical removal of the tumor, which was carried out after implementing an alpha-adrenoreceptor blockade. Hemorrhagic necrosis of the pheochromocytoma was seen on histopathologic analysis; this may have triggered the sequence of events leading to the development of Takotsubo-like cardiomyopathy and hemodynamic collapse.ConclusionTo the best of our knowledge, this is the first reported case of Takotsubo-like cardiomyopathy related to pheochromocytoma following delivery. This emphasizes the increased cardiovascular risk if pheochromocytoma is not diagnosed and treated in a timely manner, especially during pregnancy. (Endocr Pract. 2014;20:e233-e236)     

Active Crohn Disease and Hypercalcemia Treated With Infliximab: Case Report and Literature Review

ObjectiveTo report a case of 1,25-dihydroxyvitamin D–mediated hypercalcemia caused by active Crohn disease that improved with infliximab therapy.MethodsWe present the clinical and laboratory findings and describe the clinical course of a patient who had hypercalcemia during Crohn disease exacerbations. The literature is reviewed regarding 1,25-dihydroxyvitamin D production in Crohn disease, and the 3 cases of hypercalcemia in individuals with Crohn disease reported in the literature are described.ResultsA 50-year-old man with long-standing Crohn disease treated with multiple bowel resections presented for take-down ileostomy. He was hypercalcemic and had suppressed parathyroid hormone and parathyroid hormone–related peptide levels. Histopathology of the resected ileostomy site and adjacent small bowel indicated active Crohn disease. Hypercalcemia promptly resolved after a few days of treatment with intravenous glucocorticoids. One month later, hypercalcemia recurred in the presence of an inappropriately high 1,25-dihydroxyvitamin D level and increased urinary calcium and serum angiotensin-converting enzyme levels. The serum and urinary calcium levels became normal with infliximab therapy. Three previous reports of hypercalcemia caused by active Crohn disease describe effective treatment with glucocorticoids. This is the first report of successful response to infliximab in this setting.ConclusionHypercalcemia mediated by 1,25-dihydroxyvitamin D in the setting of Crohn disease may respond to glucocorticoid-sparing immunomodulators. (Endocr Pract. 2008;14:87-92)     

Failure of Multiple Therapies in the Treatment of a Type 1 Diabetic Patient with Insulin Allergy: A Case Report

ObjectiveTo describe the clinical manifestations of insulin allergy and explain a systematic management approach.MethodsWe present the clinical, laboratory, and pathologic findings of a type 1 diabetic patient with allergy to subcutaneous insulin and briefly review the related literature.ResultsAn 18-year old woman with type 1 diabetes mellitus had an insulin allergy and developed subcutaneous nodules after insulin administration. Human and analogue insulins were used, but painful nodule formation persisted. Treatment with antihistamines, steroids, and omalizumab and insulin desensitization were ineffective. The patient required pancreatic transplant because glycemic control could not be achieved due to the insulin allergy.ConclusionsInsulin allergy is not a common condition and can be challenging in patients with type 1 diabetes. Therefore, identifying patients with true insulin allergy and applying a stepwise approach to their treatment is important. (Endocr Pract. 2011;17:91-94)     

Late Manifestation of Tracheal Rupture After Thyroidectomy: Case Report and Literature Review

ObjectiveTo report an extremely rare case of delayed tracheal rupture after thyroidectomy and to review the existing related literature.MethodsWe present the history, clinical findings, radiographic evaluation, management, and intraoperative findings in a patient who presented with subcutaneous emphysema 9 days after total thyroidectomy. In addition, we review the literature and discuss the diagnostic challenges as well as management options.ResultsA 17-year-old female patient underwent a total thyroidectomy for Graves disease. On postoperative day 9, the patient presented with face and neck swelling attributable to subcutaneous emphysema. After conservative management failed, the patient underwent surgical exploration of the neck, which revealed a 2.5-cm linear vertical tear in the anterior aspect of the trachea, with no evidence of necrosis. The tear had viable edges and was primarily repaired with use of muscle flap reinforcement. The patient recovered with no other complications.ConclusionDelayed tracheal rupture should be suspected in all patients who present with subcutaneous emphysema after a thyroid surgical procedure. Review of the pertinent literature suggests that conservative management is suitable in patients with a stable condition. Surgical repair is indicated in those patients who fail to demonstrate clinical improvement. (Endocr Pract. 2012;18:e73-e76)     

Recurrent Takotsubo Cardiomyopathy Associated with Pheochromocytoma

ObjectiveTo describe a case of recurrent takotsubo cardiomyopathy in a patient with pheochromocytoma.MethodsWe present a case report, including clinical and laboratory data. In addition, the current relevant literature pertaining to pheochromocytoma and takotsubo syndrome is reviewed and summarized.ResultsIn 2004, an 81-year-old woman with no history of cardiac disease presented with chest discomfort, and takotsubo syndrome was diagnosed. No emotional or physical stressors were identified at that time. Her left ventricular systolic function normalized during that hospitalization. In 2007, the patient was readmitted to the hospital with chest discomfort and ST-segment elevation. Cardiac catheterization demonstrated only minor nonobstructive coronary artery disease. She was again found to have takotsubo syndrome with a classic apical hypokinetic segment. Treatment with a heart failure regimen was initiated, and she was screened for pheochromocytoma as the precipitant for her recurrent takotsubo cardiomyopathy. A 24-hour urine collection showed minimally elevated normetanephrine excretion of 719 μg (reference range, 148 to 560) and vanillylmandelic acid of 8.3 mg (reference range, < 8.0). The plasma normetanephrine level was 1.57pg/mL (reference range, < 0.9). Subsequent magnetic resonance imaging revealed a left adrenal mass (2 cm by 1 cm). Ultimately, the patient underwent left adrenalectomy, and the pathology report was consistent with pheochromocytoma. She has been asymptomatic since then, and a repeated echocardiogram demonstrated normal left ventricular systolic function.ConclusionIn patients presenting with takotsubo cardiomyopathy, a precipitating factor, such as emotional or physical stress, can often be identified. In some patients (such as our current case), however, pheochromocytoma may be the underlying disease and should be considered. (Endocr Pract. 2009;15:560-562)