68 Ga-DOTATATE PET/CT imaging in endogenous hyperinsulinemic hypoglycemia: A tertiary endocrine centre experience.

OBJECTIVE: Literature regarding utility of 68 Ga-DOTATATE PET/CT in insulinoma localization across various subgroups [benign/malignant/multiple endocrine neoplasia-1 (MEN-1) syndrome associated] remains scarce. In this study, the performance of 68 Ga-DOTATATE PET/CT was compared with contrast-enhanced computed tomography (CECT) and 68 Ga-NODAGA-Exendin-4 PET/CT (whenever available) in an endogenous hyperinsulinemic hypoglycemia (EHH) cohort. DESIGN: Retrospective audit. PATIENTS: EHH patients [N = 36, lesions (n) = 49, final diagnosis: benign sporadic insulinoma (BSI) (N = 20), malignant insulinoma (N = 4, n = 14), MEN-1 syndrome associated insulinoma (N = 9, n = 15), Munchausen syndrome (N = 2) and drug-induced hypoglycemia (N = 1)] having both preoperative imaging modalities (CECT and 68 Ga-DOTATATE PET/CT). MEASUREMENTS: Per-lesion sensitivity (Sn) and positive predictive value (PPV) for histopathological diagnosis of insulinoma. RESULTS: Sn and PPV of 68 Ga-DOTATATE PET/CT were 67.3% and 89.2%; 55% and 100%; 85.7% and 100%; and 66.7% and 77% for overall EHH, BSI, malignant, and MEN-1 syndrome associated insulinoma cohorts respectively. Despite having comparatively lower sensitivity in BSI cohort, 68 Ga-DOTATATE PET/CT localized a pancreatic tail lesion missed by other modalities. 68 Ga-DOTATATE PET/CT had comparatively higher sensitivity in malignant insulinoma than BSI cohort. 68 Ga-DOTATATE PET/CT also paved the way for successful response to 177 Lu-based peptide receptor radionuclide therapy (PRRT). In MEN-1 cases, lower PPV as compared with BSI was due to uptake in non-insulinoma pancreatic neuroendocrine tumours (Pan-NET). CONCLUSIONS: 68 Ga-DOTATATE PET/CT has supplemental role in selected cases of BSI with negative and/or discordant results with CECT and 68 Ga-NODAGA-Exendin-4 PET/CT. In malignant insulinoma, 68 Ga-DOTATATE-PET/CT has an additional theranostic potential. Interference due to uptake in non-insulinoma Pan-NET in MEN-1 syndrome may hinder insulinoma localization with 68 Ga-DOTATATE-PET/CT.

Genotype-Phenotype Correlations in Asian Indian Children and Adolescents with Primary Hyperparathyroidism.

Childhood and adolescent primary hyperparathyroidism (PHPT) is a very rare disease. Data on its molecular genetics are scarce. We performed a retrospective analysis (January 2000-January 2021) to determine the deleterious germline variants and genotype-phenotype correlations in children and adolescents < 20 years diagnosed with PHPT from a single referral center. Clinical features, biochemistry, imaging, management, and genetics (clinical exome analyzed for 11 PHPT and 7 pancreatitis-associated genes, MLPA for CDC73) were recorded. Thirty-six patients (20 males; median age 17 years) were classified into those with familial and/or syndromic (F/S) or apparently sporadic (AS) presentation. Sixteen (44.4%) harbored pathogenic/likely pathogenic germline variants in PHPT-associated genes. The genetic yield in F/S group was 90% (MEN1:8/10; CDC73:1/10), and AS group was 26.9% (CDC73:4/26; CASR:3/26). F/S group had frequent asymptomatic presentation (60% vs none; P < 0.001), lower serum PTH (237.5 vs 1369.1 pg/mL; P = 0.001), and maximum parathyroid dimension (0.9 vs 2.2 cm; P = 0.01) than AS group. Among the AS group, renal involvement was higher in those with molecular diagnoses (71.4% vs 10.5%; P = 0.01). All those with novel CASR variants (including one homozygous) had hypercalciuria and histology-proven parathyroid adenoma/carcinoma. A missense CTRC VUS occurred in one patient with chronic pancreatitis. In summary, Asian Indian children and adolescents with PHPT have high genetic yield, even with apparently sporadic presentation. The phenotypic spectrum of CASR variants is expanded to include childhood/adolescent PHPT with hypercalciuria and single gland neoplasia. The proposed roles for renal involvement to predict molecular diagnosis among those with apparently sporadic presentation require further elucidation.

Large Solitary Pulmonary Cryptococcoma Mimicking Lung Carcinoma in an Immunocompetent Patient.

Cryptococcosis is a life-threatening mycosis typically seen in immunocompromised patients. Pulmonary cryptococcosis generally presents as multiple or solitary nodular opacities. Cryptococcal infection presenting as a destructing cavernoma (cryptococcoma) without diffuse infiltration of the lung is an extremely rare presentation, even in immunocompromised patients. This report presents a healthy, HIV negative, immunocompetent patient who presented with a large solitary lung mass provisionally diagnosed as a lung malignancy on radiological imaging that proved to be a large cryptococcoma after biopsy. The patient was treated with liposomal Amphotericin B and fluconazole, and the lesion showed regression on serial imaging. This case report thus highlights an unconventional presentation of pulmonary cryptococcosis in an immunocompetent individual.

Branchial cleft cysts: a pictorial review.

Cystic masses of the neck may represent a wide variety of pathological conditions. Among these, branchial anomalies are the commonest paediatric congenital lesions of the head and neck. This article intends to review this condition under the same headings, as well as facilitate their diagnosis with the help of appropriate radiological findings of patients diagnosed with these anomalies. In order to make an accurate diagnosis, it is imperative to have an idea about the possible differentials, and the normal embryological development of the branchial arches, as well as their anomalies, in the form of sinuses, fistulae, and cysts, with their features and subtypes. The medical history of the patient and clinical manifestations helps in suspecting branchial cleft cysts; confirmatory imaging modalities include computed tomography, magnetic resonance imaging, ultrasonography, and fine-needle aspiration. The mainstay of management is usually by surgical excision. The location, clinical picture, and radiological correlation, along with a strong degree of suspicion for the condition, facilitates the diagnosis of this relatively common embryological anomaly.

Percentage arterial enhancement: An objective index for accurate identification of parathyroid adenoma/hyperplasia in primary hyperparathyroidism.

BACKGROUND: Radiation exposure to neck by four-dimensional computerized tomography (4DCT) is relatively high and limits its use as a first-line investigation in evaluation of primary hyperparathyroidism (PHPT). Radiation exposure can be reduced by restricting the number of CT phases. Our aim was to study the performance of 4DCT in cohort of surgery-naïve PHPT patients, and to evaluate percentage enhancement as an objective radiological index to discriminate parathyroid lesions (adenoma/hyperplasia) from thyroid tissue and lymph nodes. MATERIALS AND METHOD: Retrospective study of 49 PHPT patients {(44 single-gland diseases (SGD) and five multiple-gland disease (MGD)} who underwent 4DCT (unenhanced, early arterial, early venous and delayed venous phase) pre-operatively. Two radiologists who were blinded to surgical location of parathyroid lesions examined the scans. Attenuation values were recorded for parathyroid lesions (n=50), thyroid gland (n=50) and lymph nodes (n=12) in different phases. Percentage enhancement for different phases was calculated as "(HU in a specific enhanced phase-HU in unenhanced phase)/HU in unenhanced phase" ×100. RESULTS: Inter-rater reliability between the two radiologists was 0.83 (Cohen's kappa). In SGD, sensitivity and PPV were 93.18% and 98.8% for lateralization, and 89.77% and 95.18% for quadrant localization, respectively. In MGD, 4DCT showed 50% sensitivity and 100% PPV. Percentage arterial enhancement showed highest area under curve (AUC=0.992) for differentiation of parathyroid lesions from thyroid tissue and lymph nodes. A cut-off value of 128.9% showed 95.8% sensitivity and 100% specificity for the identification of parathyroid lesions. CONCLUSIONS: We propose that percentage arterial enhancement can be used as an objective radiological index for accurate identification of parathyroid adenoma/hyperplasia.

Pulmonary mycotic pseudo-aneurysm with a prior history of ventricular septal defect. Case report with review of literature.

In general aneurysms of the pulmonary arteries are less frequent than intracranial, aortic or other vascular locations. Infectious causes include bacteria such as Staphylococcus sp and Streptococcus sp, mycobacteria, Treponema pallidium (syphilis) and rarely fungi. We report a 7 year old female with two right-sided parahilar pseudo-aneurysm of fungal origin with a prior history of ventricular septal defect. Pulmonary mycotic pseudo-aneurysms are very rare and require a high suspicion to diagnose. If a patient is still symptomatic for fever and cough for a long time, and consolidation on x-ray is not improving on antibiotics, contrast-enhanced computed tomography is indicated. It can be suspected that the "friable mass attached to ventricular septal defect patch" was a source of fungeal emboli to pulmonary arteries thus giving weight to the infective endocarditis etiology. A prior history of ventricular septal defect repair could favour fungal endocarditis.

Skeletal Metastasis From Carcinoma of the Gall Bladder: Need for Bone Scintigraphy Justified?

BACKGROUND: Carcinoma of the gall bladder has a guarded prognosis with predominant sites of involvement being liver and regional nodes. Osseous metastasis in carcinoma of the gall bladder is rare and hence bone scintigraphy does not form a part of the routine work-up for such patients. CASE REPORTS: We describe two patients with carcinoma of the gall bladder with osteolytic metastasis (stage 4). Conservative treatment was planned but both of them succumbed to the illness. CONCLUSIONS: We thus highlight the importance of performing a bone scan or PET CT in cases of carcinoma of the gall bladder. Besides, our cases challenge Paget's seed - soil theory for sites of metastasis.

Juvenile Granulosa Cell Tumour of the Ovary with Unilocular Pure Cystic Presentation: A Case Report and Review of Literature.

BACKGROUND: Granulosa cell tumours of the ovary are rare, hormonally active, oestrogen-secreting tumours of the ovary existing in two forms: the adult form and the even rarer juvenile form. These tumours present as predominantly solid lesions while the cystic, unilocular presentation is uncommon. CASE REPORT: We present an 18-year-old unmarried girl who presented with complaints of chronic pain, abdominal distension and presence of facial hair. Radiological examination revealed a large, purely cystic, unilocular lesion without any solid components, debris or septations. Histopathological diagnosis was of a juvenile granulosa cell tumour. CONCLUSIONS: Radiological criteria suggestive of malignant ovarian masses include thick, irregular walls and septae; papillary projections and solid, echogenic foci. Nonetheless, we propose that a malignant ovarian lesion should be included in the differential diagnosis of a unilocular, purely cystic ovarian lesion.

Venous complications of pancreatitis: a review.

Pancreatitis is notorious to cause vascular complications. While arterial complications include pseudoaneurysm formation with a propensity to bleed, venous complications can be quite myriad. Venous involvement in pancreatitis often presents with thrombosis. From time to time case reports and series of unusual venous complications associated with pancreatitis have, however, been described. In this article, we review multitudinous venous complications in the setting of pancreatitis and propose a system to classify pancreatitis associated venous complications.

Pancreaticopleural fistula: a review.

Pancreaticopleural fistula is a rare complication of chronic pancreatitis consequent to posterior disruption of the pancreatic duct. The fistulous track ascends into the pleural cavity and gives rise to large volumes of pleural fluid. Pancreaticopleural fistula thus poses a diagnostic problem since the source of pleural fluid is extrathoracic. To further complicate the matter, abdominal pain is seldom the presenting or significant feature. The pleural effusion is typically rapidly accumulating, recurrent and exudative in nature. Pleural fluid amylase in the correct clinical setting virtually clinches the diagnosis. Magnetic resonance cholangiopancreatography, endoscopic retrograde cholangiopancreatography and computed tomography may delineate the fistula and thus aid in diagnosis. Endoscopic retrograde cholangiopancreatography has emerged both as a diagnostic as well as therapeutic modality in select patients of pancreaticopleural fistula while magnetic resonance cholangiopancreatography is the radiological investigation of choice. Besides delineating the ductal anatomy, magnetic resonance cholangiopancreatography can help stratify patients for appropriate management. A near normal or mildly dilated pancreatic duct responds well to chest drainage with octreotide while endoscopic stent placement benefits patients with duct disruption located in head or body of pancreas. Failure of medical or endoscopic therapy calls in for surgical intervention. Besides, a primary surgical management may be tried in patients with complete ductal obstruction, ductal disruption in tail or ductal obstruction proximal to fistula site.

Wandering spleen with torsion causing pancreatic volvulus and associated intrathoracic gastric volvulus. An unusual triad and cause of acute abdominal pain.

CONTEXT: Wandering spleen is a rare medical entity in which the spleen is orphaned of its usual peritoneal attachments and thus assumes an ever wandering and hypermobile state. This laxity of attachments may even cause torsion of the splenic pedicle. Both gastric volvulus and wandering spleen share a common embryology owing to maldevelopment of the dorsal mesentery. Gastric volvulus complicating a wandering spleen is, however, an extremely unusual association, with a few cases described in literature. CASE REPORT: We present a case of a young female who presented with acute abdominal pain and vomiting. Radiological imaging revealed a intrathoracic gastric volvulus, torsion in an ectopic spleen, and additionally demonstrated a pancreatic volvulus - an unusual triad, reported only once, causing an acute abdomen. CONCLUSION: The patient subsequently underwent an emergency surgical laparotomy with splenopexy and gastropexy.

Spinal Hydatid as a Rare Cause of Posterior Mediastinal Lesion: Understanding Cervicothoracic Sign on Chest Radiography.

BACKGROUND: Location of an intrathoracic lesion on chest radiograph is facilitated by application of 'silhouette sign'. This helps narrow down the differential diagnoses. The list of probable diagnoses reduces further on determination of the density of the lesion. A spinal hydatid presents as a fluid-density posterior mediastinal lesion on chest radiograph with destruction of the vertebral body and preservation of the disc space. Spinal hydatid is, however, rare. CASE REPORT: We describe a case of a 30-year-old female with gradual-onset paraperesis since six months. Chest radiograph was suggestive of a posterior mediastinal lesion with fluid density and destruction of D4 vertebra. MRI findings were consistent with spinal hydatid. The patient was started on perioperative benzimidazole therapy with resection of the hydatid cyst. The drug therapy was continued for six months post-operatively. CONCLUSIONS: A chest radiograph helps localise the site and possible contents of the lesion. It also guides further investigations. MRI is the imaging modality of choice for spinal pathologies causing cord compression including spinal hydatid. Echinococcal involvement of the spine is a rarity but needs to be considered in the differential diagnoses for spinal causes of gradual-onset paraperesis.

Renal Cysts and Nephrocalcinosis in a Patient Deficient in 11 beta-Hydroxylase Enzyme.

BACKGROUND: Chronic hypokalemia is known to induce renal structural and functional abnormality. The former includes induction of renal cyst formation and interstitial fibrosis while the latter entails urine-concentrating defect. However, these hypokalemia-mediated changes occur in a handful of conditions including primary aldosteronism, distal renal tubular acidosis, Liddle's disease, apparent mineralocorticoid excess syndrome and Bartter's type 3 syndrome. Such a finding has never been described in an 11 beta-hydroxylase deficient individual. CASE REPORT: We describe a case of a 15-year-old male, deficient in 11 beta-hydroxylase enzyme, presenting with hypertensive haemorrhage in basal ganglia and chronic hypokalemia-mediated nephrocalcinosis and renal cysts. To add to the uniqueness, our patient was discovered to harbour bilateral testicular adrenal rests as well. CONCLUSIONS: An early diagnosis could help prevent these sequelae and preserve long-term renal function and safeguard against ill-effects of hypertension. Besides, aetiology of nephrocalcinosis should be sought for and corrected.

Left Aberrant Gastric Vein Causing Isolated Left Hepatic Portal Venous Gas Secondary to an Incarcerated Diaphragmatic Hernia.

BACKGROUND: Hepatic portal venous gas (HPVG) is an ominous radiological sign suggestive of underlying intestinal sepsis, infection or trauma. Portal pneumatosis secondary to gastric pathologies is rare. CASE REPORT: We report a rare case of a 34-year-old man who presented with acute epigastric pain and vomiting, diagnosed to have an incarcerated diaphragmatic hernia causing gastric pneumatosis and resultant portal venous gas. CONCLUSIONS: Our case highlights an unusual presentation of gastric pneumatosis secondary to an incarcerated hiatal hernia with resultant portal venous gas involving only the left lobe of the liver. An aberrant left gastric vein was responsible for this phenomenon in our case. A sound understanding of anatomical variants is thus crucial to radiological diagnosis.

Pseudoaneurysm Arising from Mitral Aortic Intervalvular Fibrosa (P-MAIVF) Communicating with Left Atrium (LA): Multiple Detector Computed Tomography (MDCT) Evaluation.

BACKGROUND: The entity pseudoaneurysm arising from the mitral aortic intervalvular fibrosa (P-MAIVF) is a rare cardiac finding caused by multiple factors. This entity is usually diagnosed with echocardiography and confirmed with cardiac computed tomography (CT). CASE REPORT: We presented a case of congenital P-MAIVF communicating with the left atrium (LA) and an aberrant right subclavian artery, misdiagnosed as primary mitral regurgitation (MR) in transthoracic echocardiogram (TTE) due to relative contraindications to transesophageal echocardiogram (TEE), revealed in a hemophilic patient, and diagnosed with cardiac CT. CONCLUSIONS: In conclusion, cardiac CT plays a definitive role not only in anatomical assessment and confirmation of the lesion but also in primary diagnostics in patients suspected of MAIVF - especially those with relative and absolute contraindications to TEE.

An Association of Chronic Hyperaldosteronism with Medullary Nephrocalcinosis.

BACKGROUND: An association between chronic hyperaldosteronism and medullary nephrocalcinosis has rarely been made, with only a handful of cases described in literature. CASE REPORT: We describe five cases of hyperaldosteronism with a long- standing history in whom associated medullary nephrocalcinosis was established. CONCLUSIONS: We infer that a chronic hyperaldosteronic status, whether primary or secondary, is a causal factor in the etiopathogenesis of medullary nephrocalcinosis. This article illustrates and summarizes various postulated theories that support our proposed association between hyperaldosteronism and nephrocalcinosis. We conclude that chronic hyperaldosteronism should be included as one of the causes of nephrocalcinosis and that our case series emphasizes the need of a well-organized retrospective study to prove it further.

Hemangioma of rib: a different perspective.

BACKGROUND: Hemangiomas are one of the common primary benign tumors of the intraosseous and soft tissue compartments in humans. Vertebral hemangiomas being the most common of all are seen in daily radiological practice. Hemangioma of the rib is rarely seen. CASE REPORT: We reported on a case of a rib lesion which had a classic imaging pattern of hemangioma. We highlighted the use of pre-operative embolization of such a vascular rib lesion before surgically removing the lesion by thoracotomy to reduce the risk of bleeding. CONCLUSIONS: We also emphasized overt complications of overzealous needling of such a vascular lesion for histopathological diagnosis as in our case.

Secondary varicocele caused by pancreatic pseudocyst obstructing testicular venous drainage.

A pseudocyst is a fluid/debris collection that occurs as a complication of pancreatitis. It can be symptomatic and cause compression of the surrounding structures. Our case report highlights a 29-year-old male who presented with secondary varicocele on left side caused by a huge pseudocyst seen to compress the left renal and testicular veins.

Dual-phase computed tomography for localization of parathyroid lesions in children and adolescents with primary hyperparathyroidism.

BACKGROUND: Childhood and adolescent primary hyperparathyroidism (PHPT) is a rare disease caused by single adenomas in 65-94% of patients. In this patient group, there is no data on computed tomography (CT) for pre-operative parathyroid localization that may facilitate focused parathyroidectomy. METHODS: Two radiologists reviewed dual-phase (nonenhanced and arterial) CT images of twenty-three operated children and adolescents [20:single-gland disease(SGD), 3:multi-glandular disease(MGD)] with proven histopathological PHPT. Percentage arterial enhancement (PAE) was calculated as [100*{arterial-phase Hounsfield unit (HU)-nonenhanced phase HU}/nonenhanced HU] of the parathyroid lesion(s), thyroid, and lymph node. RESULTS: Dual-phase CT lateralized 100%, localized to the correct quadrant/site 85% SGD (including 3/3 ectopic), and identified 1/3 MGD. PAE (cutoff ≥ 112.3%) was sensitive (91.3%) and specific (99.5%) in distinguishing parathyroid lesions from local mimics (P<0.001). The average effective dose was 3.16±1.01mSv, comparable to the planar/single photon emission CT (SPECT) Technetium 99m(Tc)-sestamibi and choline positron emission tomography (PET)/CT scans. Solid-cystic morphology identified in 4 patients harboring pathogenic germline variants (3:CDC73, 1:CASR) may serve as a radiological clue to molecular diagnosis. Nineteen out of 20 (95%) patients with SGD who had undergone single gland resection based on pre-operative CT findings were in remission over a median follow-up of 18 months. CONCLUSION: As most children/adolescents with PHPT have SGD, dual-phase CT protocols which reduce the effective radiation dose with high localization sensitivity for single parathyroid lesions may be a sustainable pre-operative imaging modality in this patient group.