Van Wyk-Grumbach syndrome: The importance of thyroid function tests in a child presenting with multicystic ovaries.

INTRODUCTION: Van Wyk-Grumbach syndrome (VWGS) is a rare presentation of long-standing hypothyroidism, characterized by delayed bone age, enlarged bilateral multicystic ovaries, and isosexual precocious puberty. CASE PRESENTATION: We report here the clinical details of the case of Van Wyk Grumbach Syndrome in an 11-year-old girl who was first presented to the pediatric surgeon for hemoperitoneum. The patient underwent an emergency exploratory laparotomy, which showed enlarged ovaries with multiple cysts that were bleeding. She also had severe hypothyroidism, hyperprolactinemia, hyperestrogenism, pituitary adenoma, and delayed bone age. Significant improvement in symptoms was noted after thyroid hormone replacement therapy. IMPORTANCE AND CONCLUSION: This case highlights that it is crucial to consider thyroid assessment in a patient with multicystic ovaries to avoid misdiagnosis, unnecessary investigations for malignancy and/or surgical intervention, and possible complications.

A rare cause of adult's epileptic crisis: Infratentorial multinodular and vacuolating neuronal tumor.

Multinodular and vacuolating neuronal tumor (MVNT) of the cerebrum is a rare and benign lesion which has been recently included in World Health Organization Classification of the central nervous system tumors. Another entity of lesions would present the same radiological characteristics; however, it would be in the posterior fossa, hence its name: infratentorial multinodular and vacuolating neuronal tumor. They are frequently characterized by the presence of cystic (or cyst-like) nodular lesions and may show cortical involvement. They mainly can be differentiated from other cystic entities, by their clustered and unchanging nature over time. We present the case of a 54-year-old patient who has consulted for epileptic seizures. Magnetic Resonance Imaging showed a multicystic-looking mass involving the vermis and the left cerebellar hemisphere that had no changes at follow-up in the last year suggestive of infratentorial multinodular and vacuolating neuronal tumor.

Bilateral Lipoadenoma of the Adrenal Glands and Humeral Brown Tumor: An Unusual Association.

Adrenal adenolipomas are rare lipomatous adrenal tumors that can be either functional or not. Only 7 cases have been reported in the English literature so far. However, brown tumors are benign, rare, historical lesions, with histological similarity to giant tumors that can be encountered in 1% of all primary hyperparathyroidism cases. We report the case of an unusual association of bilateral lipoadenoma of the adrenal glands and humeral brown tumor in a 35-year-old patient. He presented to the emergency department with a pathological fracture of the left humerus secondary to a brown tumor. The medical investigations have concluded to primary hyperparathyroidism. The screening for multiple endocrine neoplasia type 1 revealed the presence of bilateral nonsecreting adrenal masses whose anatomopathological study concluded adenolipomas. Adrenal tumors may constitute a part of multiple endocrine neoplasia type 1 in 20 to 40% of cases. In this view, it is necessary to check for the presence of other endocrine gland tumor locations such as primary hyperparathyroidism, neuroendocrine tumors of the duodenum and pancreas, or pituitary adenomas.

An Uncommon Presentation of Adrenal Cyst with Subclinical Cushing's Syndrome: A Diagnosis Dilemma.

Adrenal cysts are a rare entity that is usually nonfunctional and asymptomatic. Their association with adrenal neoplasms was rarely described in the literature. We report a unique case of a 40 -year-old woman who was referred for evaluation of a left adrenal incidentaloma with subclinical Cushing's syndrome. The tumor was suspicious for malignancy regarding computed tomography scan (CT scan) features. Therefore, a laparoscopic left partial adrenalectomy was performed. Pathology examination showed multilocular spaces lined by endothelial cells which are compatible with endothelial adrenal cyst, associated to an adrenocortical adenoma. We further discuss the management of adrenal cyst with review of the literature.

[Posterior mediastinal glomangiomatosis: Exceptional location of a rare tumour]. / La glomangiomatose médiastinale postérieure : localisation exceptionnelle d'une tumeur rare. À propos d'un cas.

INTRODUCTION: Glomangiomatosis is a benign tumour proliferation which develops from the glomus cells in the wall of a vessel, and which contains abnormal venous capillaries. Its usual location is dermal at the extremities, mediastinal presentation is exceptional. OBSERVATION: A 63-year-old patient, followed for scoliosis, was admitted for a spontaneous haemothorax. The CT scan found hypervascularized left paravertebral masses. Thoracoscopy with biopsy provided the diagnosis of a glomus tumour. Given that its diffuse nature makes surgical excision difficult and the risk of intraoperative bleeding very high, treatment with interleukin alpha 2 was proposed to the patient. After a 3-year course, we did not observe any evolutionary change in the lesions. CONCLUSION: Glomangiomatosis is an insidiously evolving vascular tumour which must be considered in the presence of vascular lesions. The reference treatment is surgical excision when possible. On the other hand, hasty surgery in diffuse forms remains dangerous given the haemorrhagic nature of this tumour.

Gastric duplication cyst in an adult with autoimmune hemolytic anemia: a case report and review of the literature.

BACKGROUND: Gastric duplication cysts are uncommon congenital anomalies found primarily in children and rarely seen in the adult population. Accurate diagnosis of cysts before resection is difficult even using the most advanced imaging techniques. CASE PRESENTATION: In this report, we describe a 28-year-old Moroccan patient with a history of autoimmune hemolytic anemia who presented with an asymptomatic abdominal cystic mass detected during abdominal computed tomography performed before splenectomy. Magnetic resonance imaging performed for accurate characterization showed a high-signal-intensity cystic mass on T2-weighted images, located between the patient's stomach and spleen. The patient underwent a complete cyst resection during exploratory laparotomy. The histological examination showed a cyst lined by three different epithelia with bundles of smooth muscle, which suggested a gastric duplication cyst. CONCLUSIONS: We report a case of gastric cyst duplication in an adult with autoimmune hemolytic anemia, and we discuss this rare association, radiological findings, and the unique histological findings of this case.

[Hydatic pulmonary embolism: a rare complication of hepatic hydatid cyst]. / Embolie pulmonaire d'origine hydatique : une complication rare du kyste hydatique du foie.

Hepato-pulmonary hydatidosis is a parasitic disease common in Mediterranean countries. Hydatid pulmonary embolism is extremely rare and is due to rupture of a cardiac hydatid cyst or, more rarely, rupture of a hepatic hydatid cyst. We report three cases of hydatid pulmonary embolism secondary to rupture of a hydatid cyst into the inferior vena cava. Thoracic imaging, mainly CT angiography and MRI, was important for both the diagnosis and decisions on treatment. The prognosis of intra-arterial pulmonary hydatid cyst is poor because of the risk of acute fatal complications such as anaphylactic shock and vascular rupture and also of chronic progression to cor pulmonale and respiratory failure. The therapeutic management is difficult and often only partially effective hence the importance of focusing on preventative treatment.

[Acute pulmonary edema, new complication of sclerosing mediastinitis]. / Œdème aigu du poumon, une nouvelle complication de la médiastinite sclérosante.

Sclerosing mediastinitis is a rare and benign disease, which can cause obstruction of mediastinal structures, this determines the clinical presentation and prognosis. The obstruction of pulmonary venous structures secondary to mediastinal fibrosis has been rarely reported, clinically it mimics mitral stenosis. We describe the clinical case of a young patient with idiopathic sclerosing mediastinitis. The radiologic exams found stenosis of the right pulmonary artery, the right bronchus and pulmonary veins. Through this observation, we discuss this rare entity that although benign, can also be deadly. We study the different etiologies, clinical and therapeutic possibilities.

[Acute lower gastrointestinal bleeding: role of imaging]. / Hémorragies digestives basses aiguës: intérêt de l'imagerie.

Acute lower gastrointestinal bleeding is a frequent cause of hospital admission. Their management is complex and typically requires a multidisciplinary approach. The imaging techniques are variable. Because of the rapid image acquisition, excellent spatial resolution and multiplanar reformatting capabilities, multidetector-row CT is the imaging modality of choice in these patients. The authors will review the different types of imaging work-up and management of patients with acute lower gastrointestinal bleeding.

Sheehan's Syndrome A Case Report and Literature Review.

Post-partum pituitary necrosis (Sheehan's syndrome) is a rare complication of post-partum hemorrhage. The diagnosis can be erratic and often delayed. In this case report of Sheehan's syndrome in the post-partum period, the signs were characterized by agalactia, severe hypoglycemia, and low serum levels of thyroid hormones, cortico-adrenal hormones, and gonadotrophin (FSH, LH). The hypophyseal magnetic resonance imaging confirmed the diagnosis of hypopituitarism secondary to pituitary necrosis.

[Pelvic trauma: impact of iodinated contrast material extravasation at MDCT on patient management]. / Traumatisme pelvien: impact de l'extravasation du produit de contraste iodé au scanner multidétecteur dans la prise en charge thérapeutique.

PURPOSE: To determine the impact of iodinated contrast material extravasation at MDCT in selecting patients with pelvic fractures that require vascular embolization. PATIENTS AND METHODS: All patients with severe pelvic fracture admitted to the trauma unit between 1998 and 2004 underwent contrast enhanced MDCT prior to additional specific treatment. All patients with extravasation underwent immediate vascular embolization for hemodynamic stabilization. Orthopedic management was proposed when no contrast extravasation was demonstrated. The presence or absence of contrast extravasation on MDCT and the hemodynamic status of the patients were recorded. RESULTS: Seventy-four patients with pelvic fracture (mean age: 34 years) underwent contrast enhanced MDCT prior to therapeutic management. Vascular embolization was performed in 42 patients: 38 showed contrast extravasation and 4 were in hemodynamic shock but showed no extravasation. Orthopedic management was performed in 27 patients after negative MDCT and 5 hemodynamically stable patients with positive MDCT. CONCLUSION: In our patient population, the presence of iodinated contrast material extravasation at MDCT was a determinant factor in the selection of patients for vascular embolization.

[Stab injury to the cervical cord: report of three cases]. / Traumatismes par coup de couteau de la moelle cervicale: à propos de trois cas.

Retrospective review of the value of MR in the evaluation of stab injuries to the cervical cord in three patients admitted to our hospital. The mean patient age was 25 years old. Clinical examination showed hemiparesis in the first 2 cases and a Brown-Sequard syndrome in the third patient. Cervical spine radiographs showed no bony lesion. MR showed cord contusions for the first two patients with associated epidural hematoma in the second case, and cord laceration for the third case. MR of the cervical spine should be obtained in all patients with suspicion of cord injury following stab injury to the neck in the absence of bony lesion on plain radiographs or CT, even in the absence of neurological symptoms at the time of admission.

Hereditary-hemorrhagic telangiectasia: one-step magnetic resonance examination in evaluation of liver involvement.

PURPOSE: To describe the magnetic resonance imaging (MRI) features of hepatic involvement in hereditary-hemorrhagic telangiectasia (HHT) and to determine the interobserver agreement for all of them. PATIENTS AND METHODS: Twenty-three consecutive patients (including 17 women, mean age: 55 years) with HHT, according to the Curaçao criteria, underwent prospective MRI of the liver, including parenchymal, angiographic and biliary sequences, in one step. The scans were analyzed to determine the presence of vascular, biliary and parenchymal abnormalities (Mann-Whitney U test, kappa). The diameters of the hepatic vessels in the 23 patients were compared with those of 23 subjects with no signs of HHT or vascular or liver disease. RESULTS: MRI of the liver was abnormal in 21 patients with suspected HHT (91%). Vascular abnormalities were found in 21 patients (91%), consisting of marked dilatation of the hepatic artery (N=14), intrahepatic telangiectases (N=21), arteriosystemic venous shunting (N=19), arterioportal shunting (N=11) and aneurysms of the hepatic artery (N=3). Regenerative nodular hyperplasia was identified in 17 patients (74%) and ischemic cholangitis in nine (39%). No such lesions were found in the controls. The diameter of the hepatic artery proper was greater in patients with HHT than in the controls: 8.69+/-1.63 mm versus 5.17+/-0.44 mm, respectively (P<0.05). Good interobserver agreement was found with parenchymal and vascular abnormalities (0.62) and moderate interobserver agreement (0.42) with biliary abnormalities. CONCLUSION: One-step MRI of the liver appears to be an excellent tool for the evaluation of liver involvement in HHT, revealing vascular abnormalities, telangiectases, arteriovenous shunting, focal-liver lesions and ischemic cholangitis.

[Splanchnic vein aneurysms: a report of 13 cases]. / Les anévrismes veineux du réseau splanchnique: à propos de 13 patients.

PURPOSE: Splanchnic vein aneurysms are a rare and poorly known entity. From a review of 13 cases and reports from the literature, we have defined the imaging features and modalities for their diagnosis and management. PATIENTS AND METHODS: Retrospective study of 13 patients (7 females, 6 males, mean age of 53.5 years) who presented between 1993 and 2005 with splanchnic vein aneurysms. All patient charts were reviewed to collect and analyze epidemiological data, underlying etiologies, imaging work-up for diagnosis and management. Aneurysms were defined as fusiform or saccular enlargement of the portal venous vein or its branches, superior mesenteric vein and splenic vein. RESULTS: A total of 16 splanchnic vein aneurysms were identified. Four patients had portal venous hypertension. The imaging work-up was variable and included mainly non-invasive modalities (US: 8 patients; CTA: 8 patients and MRA: 9 patients) and conventional angiography in 2 patients. Eight aneurysms involved the portal vein (50%), 5 the splenic vein (31.3%), and 3 the superior mesenteric vein (18.7%). None of the patients underwent surgical or endovascular management. Imaging follow-up was performed in 12 patients, with no interval change over time with up to 10 years follow-up. CONCLUSION: In our series, the portal vein was most frequently involved. Diagnosis was exclusively based on imaging and management was limited to follow-up.