Congenital extrahepatic portocaval malformation: Rare but potentially treatable cause of pulmonary hypertension.

OBJECTIVE: Abernethy malformation (congenital extrahepatic portosystemic shunt) is a rare anomaly of the splanchnic venous system. Though rare, it is an important cause of pulmonary artery hypertension (PAH) which is often missed. All patients with PAH should be carefully evaluated for presence of Abernethy malformation before labelling them as Idiopathic PAH. METHODS: This is a retrospective analysis of prospectively collected data. We reviewed the data of all patients referred to our center for evaluation of PAH. 10 patients were diagnosed to have an extrahepatic portocaval malformation. We reviewed their presentation, diagnosis, catheterization data, intervention and their outcome along with review of literature. RESULTS: 10/104 patients with pulmonary hypertension and no intra or extracardiac shunt were found to have extrahepatic portocaval shunt (EHPCS). 3 patients had EHPCS type 1 and 7 had type 2 EHPCS. 6/7 patient with EHPCS type 2 underwent closure of the shunt. There was no procedure related complication. There was one death 3 months post procedure and one patient who was advised surgical closure was lost to follow up. Closure of the shunt resulted in normalization of the pulmonary artery pressures in 4/5 patients. CONCLUSION: Congenital portosystemic malformations form an important and potentially treatable cause of pulmonary hypertension.

Guided endodontic therapy: Management of pulp canal obliteration in the maxillary central incisor.

This is a case report of the management of pulp canal obliteration of maxillary central incisor using guided endodontic therapy. Patient reported with discolored and proclined 11. Intraoral periapical (IOPA) Radiographs and cone-beam computed tomography (CBCT) showed periapical radiolucency. The canal was obliterated till the middle third and was patent in the apical 7 mm. Root canal treatment (RCT) was planned using guided endodontics. Information of CBCT and digital surface scan were integrated using Blue Sky Bio software. A transparent three-dimensional (3D) model of the tooth and stent, designed with a sleeve for insertion of bur, was 3D-printed using clear resin. After orientation was found satisfactory on the 3D model, access opening was initiated with the orientation of round bur through the stent. IOPAs were taken after preparation of every 3 mm. The canal was located in the apical third. Cleaning and shaping were performed, and RCT completed successfully. Guided endodontic therapy is useful in the management of calcifications in anterior teeth.