Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.

Improving Outcomes in Catheter-Directed Thrombolysis for the Management of Acute Budd-Chiari Syndrome: A Case Report.

Traditionally catheter-directed thrombolysis is performed for recanalization of hepatic vein thrombosis in acute Budd-Chiari syndrome. Successful recanalization of the hepatic veins requires a continuous infusion of the thrombolytic agent for an adequate duration due to increased resistance to blood flow in the setting of luminal thrombosis. Here, we describe a case of acute Budd-Chiari syndrome in a young female in whom prolonged catheter-directed thrombolysis of the right hepatic vein was performed for a duration of 84 hours using alteplase as the thrombolytic agent. This was followed by angioplasty and stent placement. We observed that prolonged catheter-directed thrombolysis was associated with a progressive reduction in clot burden with improved luminal patency of the hepatic vein and improved outcome of subsequent angioplasty and stenting. There was a rapid improvement in liver function tests after the procedure and liver enzymes returned to baseline within a week. A follow-up ultrasound scan showed normal blood flow and a patent lumen of the right hepatic vein. In the absence of complications, prolonged catheter-directed thrombolysis in acute Budd-Chiari syndrome can achieve adequate recanalization of the hepatic veins and improved long-term clinical outcomes. This may obviate the need for other invasive procedures like TIPS (transjugular intrahepatic portosystemic shunt)/DIPS (direct intrahepatic portosystemic shunt) and liver transplantation.

Doppler hemodynamic liver index and hepatic elastic modulus: Advanced ultrasonographic techniques for non-invasive prediction of esophageal varices in cirrhosis.

PURPOSE: Currently esophagoscopy is the gold standard for assessment of esophageal varices in cirrhosis. Predicting the presence of esophageal varices, varices needing treatment (VNT) and variceal grade by advanced ultrasonographic techniques using a combination of Doppler hemodynamic liver index (HDLI) (quantifying portal hypertension) and hepatic elastic modulus (quantifying hepatic fibrosis) would be a cost-effective and non-invasive alternative to routine endoscopy. METHODS: Our cross-sectional study consisted of cirrhotic patients diagnosed using clinical features and laboratory parameters. Portal venous Doppler and liver sonoelastography were performed in selected subjects for obtaining measurements of HDLI (portal vein diameter/mean velocity) and hepatic elastic modulus respectively. Within 3 days of ultrasound, the subjects underwent upper GI endoscopy for assessment of presence, VNT and grade (F1, F2, F3) of varices. Subjects were divided into two groups (without and with varices) and data analyzed using XLSTAT. RESULTS: A total of 60 subjects (26 without and 34 with varices) were evaluated. Mean Doppler HDLI of subjects with varices was significantly higher (0.72 vs. 0.59, p < 0.0001) with progressive increase in values with variceal grade, being highest in grade-F3 (mean 0.77). Likewise, mean hepatic elastic modulus was also higher in subjects with varices (28.9 vs. 12.6 kPa, p < 0.0001) and showed progressive increase with grade (51.1 kPa for F3). For predicting presence of varices, maximum accuracy of elastic modulus was at cut-off of 14.5 kPa (sensitivity, specificity 83% and 84.6%) and Doppler HDLI was at 0.66 (66% and 92.3%). Good inter-rater agreement was present (κ 0.66). CONCLUSION: Combination of Doppler HDLI and hepatic elastic modulus is an excellent non-invasive method for predicting the presence, VNT, and variceal grade and may obviate need for routine endoscopic screening in cirrhosis.

Ultrasound diagnosis of syndromic cases with vascular malformations and soft tissue overgrowth: A rare case of CLOVES syndrome.

Introduction: Syndromes associated with vascular malformation and soft tissue overgrowth in the paediatric population present with multiple soft tissue swellings. Ultrasound is the initial investigation of choice for paediatric soft tissue swellings. Ultrasound evaluation can accurately assess the nature of vascular malformations and pattern of lipomatous hypertrophy in areas of soft tissue overgrowth to facilitate early diagnosis of such syndromes. Case Report: Here, we report a case of CLOVES (congenital lipomatous overgrowth (CLO), vascular malformations (V), epidermal nevi (E), and spinal/skeletal anomalies/scoliosis (S)) syndrome in a 6-year-old girl referred for evaluation of soft tissue swellings. CLOVES syndrome is a rare overgrowth syndrome in the paediatric population which presents with multiple soft tissue swellings. The ultrasound and clinical features of the syndrome have been illustrated to help radiologists accurately diagnose this rare syndrome based on detailed ultrasound and clinical evaluation. Discussion: Radiological features of CLOVES syndrome and differentiating ultrasound features of other such syndromes have been described in detail. A systematic stepwise approach to diagnosing complex syndromic associations of vascular malformations with lipomatous overgrowth has been proposed. Role of ultrasound in the management, Wilms tumour screening and follow-up of CLOVES syndrome have also been discussed. Conclusion: Ultrasound plays a crucial role in the early diagnosis and management of complex syndromes presenting with soft tissue swelling in the paediatric population. It also aids in the differentiation of such syndromes, tumour screening, guided sclerotherapy and follow-up of vascular lesions encountered in such syndromes.