Laparoscopic Bilateral Adrenalectomy in a Young Female Patient with Recurrent Cushing's Disease.

INTRODUCTION: Synchronous bilateral adrenalectomy is undertaken less often due to numerous perioperative challenges and rare circumstances of patients needing this procedure. Bilateral adrenalectomy is an important second-line option for patients with persistent or recurrent hypercortisolism following transsphenoidal surgery for Cushing's disease. Here, we present a challenging case of synchronous laparoscopic bilateral adrenalectomy for a young female patient with recurrent Cushing's disease and fertility wishes. Case Presentation. A 21-year-old recently married patient who was diagnosed with Cushing's disease with a pituitary microadenoma had undergone two attempts of transsphenoidal excision of the pituitary tumour. Follow-up evaluation showed an unresectable residual tumour with invasion of the intracavernous part of the left internal carotid artery. As the patient had the hypothalamic-pituitary-ovarian axis intact with strong fertility wishes, she was offered bilateral adrenalectomy instead of radiotherapy. She was prepared for the surgery with close perioperative support from the endocrinology and anaesthesia teams. Intravenous hydrocortisone infusion was started at the induction of anaesthesia. Transperitoneal approach was used with the patient positioned in left and right lateral positions for right and left glands, respectively. A meticulous surgical technique was used for the identification of adrenal veins to clip them before division followed by handling of the glands. The patient had minimal haemodynamic disturbances during surgery. Intraoperative blood loss was less than 100 ml, and operative time was 220 minutes. She had a gradual recovery following postoperative respiratory distress due to basal atelectasis and consolidation. Cortisol levels were less than 20 nmol/L postoperatively, suggesting successful surgical intervention. Two months after surgery, she continued on maintenance therapy of oral hydrocortisone and fludrocortisone and was encouraged to go ahead with pregnancy. CONCLUSION: Although bilateral adrenalectomy is considered a high-risk procedure, these risks can be mitigated and performed safely while maintaining close multidisciplinary perioperative support.

Seeking the unseen: Localization and surgery for an occult sporadic insulinoma.

Insulinomas are rare pancreatic neuroendocrine tumours and the commonest cause for endogenous hyperinsulinaemic hypoglycemia. Small tumours are not easily detected by conventional cross-sectional imaging making localization prior to surgical removal a challenge. Selective arterial calcium stimulation is an invaluable adjunct to localization in such circumstances. This is further supplemented by intraoperative ultrasonography. A 39-year-old male was referred with features of Whipple's triad of 10 months duration. Clinical and biochemical evaluation including C-peptide and serum insulin levels during supervised hypoglycemia concluded endogenous hyperinsulinaemia as the underlying aetiology. Contrast CT and MRI of the abdomen failed to localize the tumour. Selective arterial calcium stimulation localized the lesion in distal pancreas. During the surgery, tumour was further localized to the tail of the pancreas using intraoperative ultrasonography and enucleated. Histology confirmed an insulinoma and patient made an unremarkable recovery and was well more than a year after the surgery.

Recovery of the Hypothalamo-Pituitary-Adrenal Axis After Transsphenoidal Adenomectomy for Non-ACTH-Secreting Macroadenomas.

CONTEXT: Secondary adrenal insufficiency is a potential complication of transsphenoidal adenomectomy (TSA). Most centers test recovery of the hypothalamo-pituitary-adrenal (HPA) axis after TSA, but, to our knowledge, there are no data predicting likelihood of recovery or the frequency of later recovery of HPA function. OBJECTIVE: To assess timing and predictors of HPA axis recovery after TSA. DESIGN: Single-center, retrospective analysis of consecutive pituitary surgeries performed between February 2015 and September 2018. PATIENTS: Patients (N = 109) with short Synacthen test (SST) data before and at sequential time points after TSA. MAIN OUTCOME MEASURES: Recovery of HPA axis function at 6 weeks, and 3, 6, and 9 to12 months after TSA. RESULTS: Preoperative SST indicated adrenal insufficiency in 21.1% Among these patients, 34.8% recovered by 6 weeks after TSA. Among the 65.2% (n = 15) remaining, 13.3% and 20% recovered at 3 months and 9 to 12 months, respectively. Of the 29% of patients with adrenal insufficiency at the 6-week SST, 16%, 12%, and 6% subsequently recovered at 3, 6, and 9 to 12 months, respectively. Preoperative SST 30-minute cortisol, postoperative day 8 cortisol, and 6-week postoperative SST baseline cortisol levels above or below 430 nmol/L [15.5 µg/dL; AUC ROC, 0.86]; 160 nmol/L (5.8 µg/dL; AUC ROC, 0.75); and 180 nmol/L (6.5 µg/dL; AUC ROC, 0.88), were identified as cutoffs for predicting 6-week HPA recovery. No patients with all three cutoffs below the threshold recovered within 12 months after TSA, whereas 92% with all cutoffs above the threshold recovered HPA function within 6 weeks (OR, 12.200; 95% CI, 5.268 to 28.255). CONCLUSION: HPA axis recovery can occur as late as 9 to 12 months after TSA, demonstrating the need for periodic reassessment of patients who initially have SST-determined adrenal insufficiency after TSA. Pre- and postoperative SST values can guide which patients are likely to recover function and potentially avoid unnecessary lifelong glucocorticoid replacement.

Myxedema Crisis Presenting with Seizures: A Rare Life-Threatening Presentation-A Case Report and Review of the Literature.

Myxedema crisis is a life-threatening extreme form of hypothyroidism with a high mortality rate if left untreated. Myxedema crisis is commonly seen in older patients, especially in women, and is associated with signs of hypothyroidism, hypothermia, hyponatraemia, hypercarbia, and hypoxemia. Patients might present with different organ specific symptoms. Seizures are a recognized but rare manifestation of myxedema with a very high mortality rate. Prompt diagnosis and appropriate management may improve the prognosis. Many contributory factors may involve development of seizures in a patient with myxedema. Hyponatraemia is one such cause, which is seen in moderate-severe form in the background of myxedema. We report an elderly male who presented with generalized tonic clonic seizure preceded by memory impairment and drowsiness. He had moderate hyponatraemia and very high thyroid stimulatory hormone levels in association with low free thyroxin levels. Diagnosis of myxedema crisis was made and patient was successfully treated with sodium correction and thyroid hormone replacement.

Safe and pragmatic use of sodium-glucose co-transporter 2 inhibitors in type 2 diabetes mellitus: South Asian Federation of Endocrine Societies consensus statement.

Diabetes prevalence shows a continuous increasing trend in South Asia. Although well-established treatment modalities exist for type 2 diabetes mellitus (T2DM) management, they are limited by their side effect profile. Sodium-glucose co-transporter 2 inhibitors (SGLT2i) with their novel insulin-independent renal action provide improved glycemic control, supplemented by reduction in weight and blood pressure, and cardiovascular safety. Based on the clinical outcomes with SGLT2i in patients with T2DM, treatment strategies that make a "good clinical sense" are desirable. Considering the peculiar lifestyle, body types, dietary patterns (long duration religious fasts), and the hot climate of the South Asian population, a unanimous decision was taken to design specific, customized guidelines for T2DM treatment strategies in these regions. The panel met for a discussion three times so as to get a consensus for the guidelines, and only unanimous consensus was included. After careful consideration of the quality and strength of the available evidence, the executive summary of this consensus statement was developed based on the American Association of Clinical Endocrinologists/American College of Endocrinology protocol.

An unusual case of recurrent Guillain-Barré syndrome with normal cerebrospinal fluid protein levels: a case report.

BACKGROUND: Guillain-Barré syndrome is an acquired polyradiculo-neuropathy, often preceded by an antecedent event. It is a monophasic disease but a recurrence rate of 1-6 % is documented in a subset group of patients. Patients with Guillain-Barré syndrome show cerebrospinal fluid albuminocytologic dissociation. Normal cerebrospinal fluid protein levels during both initial and recurrent episodes of Guillain-Barré syndrome is a rare occurrence and has not been described earlier in the literature. CASE PRESENTATION: Twenty-five-year-old Sri Lankan female with past history of complete recovery following an acute inflammatory demyelinating polyneuropathy (AIDP) variant of Guillain-Barré syndrome 12 years back presented with acute, ascending symmetrical flaccid quadriparasis extending to bulbar muscles, bilateral VII cranial nerves and respiratory compromise needing mechanical ventilation. Nerve conduction study revealed AIDP variant of Guillain-Barré syndrome. Cerebrospinal fluid analysis done after 2 weeks were normal during both episodes without albuminocytologic dissociation. She was treated with intravenous immunoglobulin resulting in a remarkable recovery. Both episodes had a complete clinical recovery in three and four months' time respectively, rather a faster recovery than usually expected. CONCLUSION: Recurrence of Guillain-Barré syndrome can occur in a subset of patients with Guillain-Barré syndrome even after many years of asymptomatic period. Normal cerebrospinal fluid profile does not exclude Guillain-Barré syndrome and may occur in subsequent recurrences of Guillain-Barré syndrome arising the need for further studies to identify the pathophysiology and the possibility of a different subtype of Guillain-Barré syndrome.

Guillain-Barré syndrome presenting with Raynaud's phenomenon: a case report.

BACKGROUND: Guillain-Barré syndrome is an immune mediated acute inflammatory polyradiculo-neuropathy involving the peripheral nervous system. Commonest presentation is acute or subacute flaccid ascending paralysis of limbs. Rarely autonomic dysfunction can be the presenting feature of Guillain-Barré syndrome. Raynaud's phenomenon, although had been described in relation to many disease conditions, has not been described in association with Guillain-Barré syndrome up to date. CASE PRESENTATION: We report the first case of Guillain-Barré syndrome presenting with Raynaud's phenomenon in a 21-year-old previously well boy. New onset Raynaud's phenomenon was experienced followed by acute ascending flaccid paralysis of lower limbs and upper limbs together with palpitations and postural giddiness. Nerve conduction studies showed acute inflammatory demyelinating polyneuropathy with cerebrospinal fluid cyto-protein dissociation. He was treated with intravenous immunoglobulin and showed a satisfactory clinical recovery of muscle weakness, Raynaud's phenomenon and autonomic disturbances. CONCLUSION: Guillain-Barré syndrome presenting with Raynaud's phenomenon is not being reported in literature previously. Although the underlying mechanism is not fully understood, Raynaud's phenomenon should prompt the physician to consider Guillain-Barré syndrome with a complimentary clinical picture.

Angioedema as the first presentation of B-cell non-Hodgkin lymphoma--an unusual case with normal C1 esterase inhibitor level: a case report.

BACKGROUND: Acquired angioedema is a rare but recognized manifestation of lymphoproliferative disorders due to deficiency in C1 esterase inhibitor. Normal level of C1 esterase inhibitor proteins in association with angioedema due to lymphoproliferative disease is a rare and an uncommon finding caused by antibodies produced from the underlying disease. Antibodies cause inactivation of C1 esterase inhibitor, thus resulting in C1 esterase inhibitor dysfunction despite of normal quantity of C1 esterase inhibitor. CASE PRESENTATION: A 50-year-old Sri Lankan male presented with first episode of angioedema without any family history. Physical examination revealed mild pallor with swelling of tongue, lips and perioral region. On investigations, erythrocyte sedimentation rate was persistently high and bone marrow with immunohistochemistry revealed infiltration with B-cell type low grade non-Hodgkin lymphoma. Computed tomography scan of the chest and abdomen showed paratracheal and subcarinal lymphadenopathy and splenomegaly, with the findings being compatible with lymphoma. He had normal C1 esterase inhibitor protein level with reduced activity and low C1q, C4 levels indicating antibodies against C1 esterase inhibitor causing dysfunctional C1 esterase inhibitor. CONCLUSION: Adult onset angioedema should prompt physicians to suspect underlying lymphoproliferative disorder despite of C1 esterase inhibitor protein level being normal. Though uncommon, presence of antibodies against C1 esterase inhibitor secondary to lymphoproliferative disorder should be considered in the presence of normal C1 esterase inhibitor protein levels with low functional capacity in the background of acquired angioedema.

Lemierre's syndrome secondary to community-acquired methicillin-resistant Staphylococcus aureus infection presenting with cardiac tamponade, a rare disease with a life-threatening presentation: a case report.

BACKGROUND: Lemierre's syndrome is a rare condition characterized by thrombophlebitis of internal jugular vein, septicemia and septic metastatic infection of different organs. It is preceded by an oropharyngeal infection by anaerobic organisms. Community-acquired methicillin-resistant Staphylococcus aureus is now emerging as a causative organism in Lemierre's syndrome. Clinical manifestations vary depending on the organ system affected by the infection. Although rare, patients may present with life-threatening conditions such as cardiac tamponade. CASE PRESENTATION: We report the first case, to our knowledge, of Lemierre's syndrome presenting with cardiac tamponade secondary to community-acquired methicillin-resistant S. aureus in a previously well 45-year-old Sri Lankan lady. Fever, sore throat and left-sided neck pain complicated with facial and left upper limb swelling were followed by severe shortness of breath for 24 h. There was tachycardia with pulsus paradoxus, low blood pressure and soft heart sounds. Pericardial effusion with cardiac tamponade was detected on echocardiogram and methicillin-resistant S. aureus species were isolated in both blood and pericardial fluid cultures. Venous duplex of neck veins and computed tomography scan of the neck showed thrombosis of left-sided internal jugular, external jugular, subclavian and axillary veins. Diagnosis of Lemierre's syndrome was made, and patient had a satisfactory recovery following emergency pericardiocentesis and a prolonged course of antibiotics. CONCLUSIONS: Although uncommon, Lemierre's syndrome is a life-threatening condition. Patients may present with cardiac tamponade secondary to purulent pericarditis in Lemierre's syndrome, where emergency pericardiocentesis is lifesaving. Community-acquired methicillin-resistant S. aureus is emerging as a causative agent in Lemierre's syndrome, and awareness is required amongst physicians for prompt diagnosis and appropriate empirical treatment to prevent mortality and morbidity associated with the disease.