Search

Background: Absent pulmonary valve with ventricular septal defect is a rare cardiac malformation. The aim of our study is to specify the anatomic characteristics and the clinical and echocardiographic features of this cardiac malformation and to discuss its management., Methods: We report 8 cases of absent pulmonary valve with ventricular septal defected collected over a period of 24 years. The diagnosis was made during the first year of life in all cases in the presence of respiratory symptoms and/or cyanosis. It was confirmed by cardiac catheterization in 4 cases and echocardiography in 4 cases., Results: Pulmonary vascular obstructive disease, related to aortopulmonary collateral vessels, was noted in one patient who died at the age of 20 years. One patient was lost to follow up and 6 patients were operated. Closure of the ventricular septal defect, widening of the pulmonary tract and insertion of a pulmonary valve were performed in the 6 cases. Reduction procedure of the pulmonary arteries was performed in 3 cases. One death related to early postoperative infective endocarditis was noted. At a mean follow up of 2 years, the 5 survivors are going well with no significant pulmonary stenosis., Conclusion: Although named "tetralogy of Fallot with absent pulmonary valve", absent pulmonary valve with ventricular septal defect is different from tetralogy of Fallot by aneurysmal dilatation of the pulmonary arteries which may compress the bronchial tree and lead to respiratory symptoms that can be severe with respiratory distress. Pulmonary arterioplasty eliminate airways obstruction and its results are satisfactory. The need for insertion of a pulmonary valve is debatable.

Introduction: Langerhan's cell histiocytosis is defined as an abnormal proliferation of Langerhans cells in various organs and tissues (bone, skin, lymph nodes...). Among facial locations, mandibular involvement is the most frequent and occurs in young people less than 20 years. The aim of this report is to review the characteristic features of this disease, the current means of diagnosis and treatment through two case reports., Cases: Two male patients aged 10 and 55 years respectively presented with single mandibular location of Langerhans cell histiocytosis. The diagnosis was confirmed by histological examination of the biopsy specimen. The first patient underwent surgical resection of the tumor. No recurrence was observed. In the second patient, the postoperative course was marked by the appearance of an inflammatory phenomenon in the adjacent skin and mucosa, successfully treated by intralesional corticosteroid therapy., Discussion: Langerhan's cell histiocytosis is a rare disease. The mandibular location has variable clinical expressions which can vary from dental mobility to tumor development in the gingiva. The radiological appearance is also variable; histological proof is required for diagnosis. Treatment depends on the number and locations of the lesions. In case of a single location, the treatment options may vary from a "wait and see" attitude to a non aggressive surgery, with a possible local corticosteroid therapy or radiothe-rapy. For multiples locations, chemotherapy is indicated.

Hydatid cysts in the heart are rare, with an unpredictable outcome and numerous complications, requiring rapid surgical management. We report the case of a 19 year old female patient, who underwent surgery with extra corporeal circulation for a hydatid cyst of the interventricular septum of the heart, complicated by secondary pulmonary echinococcus infection, confirmed on CT and MRI. Resection of the cyst was performed via a right auriculotomy. The post operative period was favourable; the other pulmonary sites were treated medically. We emphasise the methods of diagnosis and management.