Neurofibromatosis Type 1 or Von Recklinghausen Disease: About Three Cases to the National Hospital of Niamey and Literature Review

We report three cases of neurofibromatosis type 1 disease with literature review, collected in the department of neurology and internal medicine from National Hospital of Niamey (HNN). Two of them were men and the first signs were noted by the mother at the birth in 2 cases. Only one case of consanguinity was observed. Clinically, light brown spots on the skin, neurofibromas, Lisch nodules were constantly observed. Histopathological’s exam confirmed neurofibromas. Moreover, cutaneous and ophthalmological manifestations lead to the diagnostic. Two cases of orthopedic complications were observed: one scoliosis and one Congenital dysplasia of the long bones. There was no specific treatment. Neurofibromatosis type 1 or von Recklinghausen’s disease is the most frequent phacomatosis and its diagnosis is usually composed of a set of clinical criteria of the National Institute Health (Bethesda, 1988).

Epidemio-Clinical, Therapeutic and Evolutive Aspects of Pulmonary Embolism in Young Subject in the Cardiology Department in Point “G” Hospital University Center Bamako

Objective: The purpose of this work was to determine the epidemiological, clinical and evolutionary aspects of the pulmonary embolism of the young person in the cardiology department of the University Hospital Center (CHU) Point G in Bamako-Mali. Methodology: This was an analytical study from January 01, 2018 to December 31, 2018 in the CHU Point G cardiology department, including all patients hospitalized during this period. Results: Of 1379 hospitalized patients, 19 patients were concerned by pulmonary embolism of the young person. The prevalence of pulmonary embolism of the young person was 1.37%. The most affected age group was 21 - 30 (47.4%) of patients. The predominance was female (89.47%) with a gender ratio of 0.11 in favour of women. The average age in the series was 29.79 years with extremes of 16 years and 40 years. Factors predisposing to pulmonary embolism were dominated by peri partum, cardiomyopathy and obesity with 47.3%, 31.57% and 21.1%, respectively. The dominant signs were chest pain and dyspnea in 94.7% and 89.5% of cases respectively. Pulmonary embolism was unlikely in 60.52% according to the Geneva and Wells score simplified. At the thoracic angioscanner, the embolism was bilateral in 52.6% of cases and distal in 36.8% of patients;in 10 patients who performed cardiac ultrasound, pulmonary arterial hypertension (70%), dilation of the right ventricle (20%) and left ventricular dilation (40%). Hyper leucocytosis (47.4%), anemia and low prothrombin rate (TP) (22.2%) were the most found biological abnormalities. More than 2/3 (68.5%) our patients had an intermediate mortality risk according to the PESI (Pulmonary Embolism Severity Index) score. The average hospital stay was 10 days. Hospital mortality was 10.5%. Conclusion: The pulmonary embolism of the young person is a frequent, serious and multifactorial pathology and the female sex is most affected especially during peri partum periods, hence the need for preventive measures. Clinical signs are not specific and based on the assessment of clinical probability. Pulmonary angioscanner remains the confirmation review in our context.

Splenectomy in the Surgery Department “A” at the University Hospital Point G Bamako

We conducted a retrospective study that included patients with erythrocyte and hemolytic pathology justifying a splenectomy and patients presenting splenic trauma for which hemostasis splenectomy is required. We performed 27 total splenectomy in which 26 are by laparotomy (92.86%) and 1 by laparoscopy (3.57%). A partial splenectomy was performed by laparoscopy. The morbidity was marked by 2 cases of infectious syndrome and 1 case severe anemia. The mortality was 7.14% (n = 2). Splenectomy is part of the therapeutic arsenal for benign or malignant hematological disorders that constitute the main indication for elective splenectomy. Splenectomy was one of the most common operations in abdominal surgery. During the past decade, an increased rate of late complications, specially septic and thromboembolic complications are well documented. The risk is related to the indication of splenectomy, and is less than 1% in adults without immunodeficiency. However, these overwhelming postsplenectomy infections are associated with a high mortality rate. The best treatment of these infections is preventive measures which are based on vaccination and education of asplenic patients.

The Lithiasis of Choledoque: Epidemiological Aspects, Clinics and Therapeutiques in the Surgery Department “A” at the University Hospital Point G

Considered as rare in Africa lithiasis of the main bile duct (MBD) is a potentially serious pathology, diagnosis is facilitated by imaging tests (ultra-sound, CT, MRI cholangiopancreatography). Its surgical management is improved by laparoscopy coupled with interventional endoscopy. However, laparotomy remains the only way in Mali. This was a 14-years retrospective and descriptive study (2010-2014). All patients with BPV lithiasis were included. We studied the field, antecedents, clinical, biological, radiological, therapeutic and outcome aspects. 40 cases of stones in the main bile duct were collected during the study period. The hospital frequency was 2.8 cases per year, the clinical signs dominated by the Charcot triad (pain, fever, jaundice) found in 40 cases (100%). The average age was 60 years, female represented 70% of cases. Ultrasonography was the most requested review in 40 cases (100%). The procedure performed was cholecystectomy associated with choledochotomy with calculation extraction in all patients (100% of cases). One case of biliary fistula and one case of wall abscess were observed. The mortality was 5% and the simple suited in 90% of the cases. The lithiasis of the main bile duct is an infrequent pathology in Mali and potentially serious. This diagnosis is assisted in our context by ultrasound and CT. Laparotomy remains the only route of entry in Mali.

Partial Ventricular Atrioventricular Canal Aged about a Case

Introduction: The partial ventricular atrioventricular canal is a congenital heart malformation of the ostium primum type of variable surface that can reach the single atrium where the interatrial septum is no more than a cephalic muscular vestige attached to the roof of the atrium. His diagnosis at the age of 73 is not a common situation. The interest of this clinical case that we report lies in the peculiarity of its mode of discovery and its evolution under medical treatment. Observation. He was a 73-year-old patient with no known cardiovascular risk factor and has since been at about 18 years of age with heart failure syndrome with progressive dyspnea, cough and edema of the lower limbs, but never explored for lack of financial means, and the evolution was enamelled by episodes of cardiac decompensation, the last of which dated back to 03/07/2018 justifying a hospitalization in our service. Cardiac ultrasound shows a partial atrioventricular canal. The care was medical. Conclusion: The atrioventricular canal is a congenital heart disease complex of poor prognosis.