[Portal cavernoma at child about two observations]. / Cavernome portal chez l'enfant a propos de deux observations.

We report two observations of portal cavernoma diagnosed successively in Bamako and Dakar. The first is a 6-year-old male admitted to the service for ascites and abdominal pain. At admission the clinical parameters (weight, height, temperature, cranial perimeter and temperature) were within the norms for age. The clinical examination noted a moderate skin-mucosal pallor, asthenia. The biological assessment returned to moderate normochrome anemia with impaired pancreatic function while renal and hepatic functions were maintained. The abdominal scan performed after two low-contribution abdominal ultrasounds, objected signs in favor of a portal cavernoma with perisplenic and gastric varicose veins. The second is an 8-year-old male child born from an unborn marriage and from a followed pregnancy with premature delivery. His pathological history includes a notion of prematurity that required a stay in neonatology with umbilical catheterization and repeated abdominal pain. He had an acute abdominal episode in March 2015 justifying a surgical hospitalization for suspicion of appendicitis. At admission the clinical parameters (weight, height, temperature, cranial perimeter and temperature) were within the norms for age. The abdominal ultrasound prescribed for this was suggestive of portal cavernoma, later confirmed by abdominal computed tomography.

Nous rapportons deux observations de cavernome portal diagnostiqué successivement à Bamako et à Dakar. Le premier est un enfant de 6 ans de sexe masculin admis dans le service pour ascite et douleurs abdominales. L'examen clinique notait une pâleur cutanéo-muqueuse modérée, une asthénie. Le bilan biologique retrouvait une anémie modérée normochrome normocytaire avec une fonction pancréatique perturbée tandis que les fonctions rénales et hépatiques étaient conservées. Le scanner abdominal réalisé après deux échographies abdominales peu contributives, objectivait des signes en faveur d'un cavernome portal avec varice péri-splénique et gastrique. Le second est un enfant de 8 ans de sexe masculin né d'un mariage non consanguin et issu d'une grossesse suivie avec accouchement prématuré. Il est le 3e enfant de sa fratrie et scolarisé. On retrouve dans ses antécédents pathologiques une notion de prématurité ayant nécessitée un séjour en néonatologie avec cathétérisme ombilical et des douleurs abdominales à répétition. L'enfant a commencé à se plaindre de douleurs abdominales récurrentes vers l'âge de 6 ans. Douleurs de siège péri ombilical sans réveil nocturne dans un contexte de constipation chronique d'allure fonctionnelle. Il a fait un épisode abdominal aigu justifiant une hospitalisation en chirurgie pour suspicion d'appendicite. A l'admission les paramètres cliniques (poids, taille, température, périmètre crânien et température) étaient dans les normes pour l'âge. L'échographie abdominale prescrite à cet effet était évocatrice de cavernome porte, confirmé par la suite par la tomodensitométrie abdominale.

Manejo quirúrgico de los carcinomas diferenciados de tiroides Experiencia en un país de bajos ingresos / SURGICAL MANAGEMENT OF DIFFERENTIATED THYROID CARCINOMAS. EXPERIENCE IN A LOW INCOME COUNTRY

ience in the management of differentiated thyroid carcinomas in a low- income country. Materials and Methods: We performed a retrospective study in our department where 21 cases of differentiated thyroid carcinomas were recorded from February 2001 to December 2010. Results: We performed 334 thyroidectomies for 326 patients. Of this group, 21 differentiated thyroid carcinomas were diagnosed. Differentiated thyroid carcinomas represented 6.4% of all thyroid neoplasm managed during the same period (n=326). Median age was 44 years (range 13 - 75 years). Male to female ratio was 1:20. Six (6) patients underwent primary hemithyroidectomy in other institutions while the fifteen left were entirely managed in our clinic. Of them, one patient was referred with positive fine needle aspiration cytology for papillary thyroid carcinoma (incidental detection by fine needle aspiration biopsy) and another had history of sinus pyriform fistula. Pathology of surgical specimens showed 13 cases of papillary thyroid carcinomas and 8 cases of follicular thyroid carcinomas with association to Hashimoto thyroiditis and Grave's disease in respectively in 1 case. Twenty cases were incidentally discovered by thyroid surgery and undergone completion thyroidectomy with prophylactic central neck dissection, completion thyroidectomy alone, modified lateral neck dissection alone and surveillance respectively in 13, 1, 1 and 6 cases. Complications of thyroid surgery were bilateral recurrent laryngeal nerve paralysis and hematoma respectively in 1 case. Median hospital stay was 5 days ranged from 3 to 15 days. During the follow-up period, most of our patients were lost of follow-up. Conclusion: Management guidelines of differentiated thyroid carcinomas are well established but not applicable to low- income country for several reasons. National guidelines, based on further researches, must then be implemented to improve our practice

[Dermatofibrosarcoma protuberans of the face: Long term patient of a follow up].

We report the case of a patient 40 years old affected by a facial tumour diagnosed as a dermatofibrosarcoma protu­berans. He has been followed in our clinic for the last 22 years. He got a recurrency after 11 years and after an other surgery he his actually free of disease. The purpose of this article is to evaluate the tumoral recurrency and the long term follow up.

[Prevalence of congenital heart diseases in Koranic schools (daara) in Dakar: a cross-sectional study based on clinical and echocardiographic screening in 2019 school children]. / Prévalence des cardiopathies congénitales en milieu scolaire coranique (daara) à Dakar : étude transversale basée sur le dépistage clinique et échographique de 2019 élèves.

Congenital heart diseases are one of the major cardiovascular diseases in developing countries. Most prevalence studies were based on clinical examination of children with echocardiographic confirmation of suspected cases and underestimate its prevalence. The objective of this study was to investigate the prevalence of congenital heart disease in "daara" (Koranic schools) in the city of Dakar and its suburbs on the basis of clinical examination and Doppler echocardiography in school children. This cross-sectional survey was carried out from 9(th) August to 24(th) December 2011, and included a population of 2019 school children aged 5 to 18 years in 16 selected "daaras" under the Academic Inspectorate of Dakar and its suburbs. Anamnestic, clinical and echocardiographic data were recorded in a validated questionnaire. A p < 0.05 was considered to be statistically significant in bivariate analysis. 2 019 school children were included out of which 60.1% were male (sex-ratio: 0.66). The average age was 9.7 years (± 3.3 years). 18 cases of congenital heart diseases were detected being a prevalence of 8.9 per 1 000 (95 % CI: 1.8 to 7.9). This included 6 cases of inter-atrial septal aneurysm, 5 cases of peri-membranous ventricular septal defects, 4 cases of patent ductusarteriosus and 3 cases of tetralogy of Fallot. Factors correlated with the presence of congenital heart disease were ageless than 8 (p <0.001) and residence in the suburbs of Dakar (p <0.001). We also detected 10 cases of rheumatic valvular disease, a prevalence of 4.9 per 1 000 (95% CI: 2.4 to 9.1). Our study shows a high prevalence of congenital heart diseases, which is almost identical to the WHO estimates and that ultrasound screening is more sensitive than clinical screening. Reducing the prevalence of these diseases requires implementation of appropriate policies, focusing on awareness and early detection.

Long-term outcomes (>5-year follow-up) with porcine acellular dermal matrix (Permacol) in incisional hernias at risk for infection.

PURPOSE: We reviewed retrospectively all patients undergoing abdominal wall reconstruction using porcine acellular dermal matrix (PADM) from 2004 to 2008 with follow-up assessment in 2012. Technique, short-term (infection, seroma, wound dehiscence), and long-term (mesh infection, recurrence) complications, and hernia recurrences were evaluated by physician examination ≥5 years postoperatively. RESULTS: 56 patients at high risk for infection had elective operation; nine had non-elective operation for complications of prior incisional hernia/hernia repair. Operations were clean, clean-contaminated, contaminated, or grossly infected in 49, 32, 12, and 6%, respectively. Techniques of repair included 10 onlay (six reinforced primary closures, four bridging patches), 47 sublay (20 reinforced primary closures, 27 bridging patches), six inlay, and two sandwich (sublay and onlay). Early complications (≤30 days postoperatively) occurred in 19 of the 65 patients (29%), including two prosthetic dehiscences from fascial attachment, 13 wound infections, and 4 seromas. After a mean follow-up of ≥5 years in 59 of 65 patients, physician-reported incidences of infection requiring removal of mesh or hernia recurrence were 25 and 66%, respectively. Hernia recurrence occurred in 12 of 26 (46%) patients with a reinforced repair and 27 of 33 (82%) with patched repairs. Mesh infection occurred in 7 of 24 patients with sublay patch and in 4 of 19 sublay reinforcement. The greatest recurrence rate was in contaminated (71%) and grossly infected wounds (100%), while recurrence rate was 63% in clean and 63% in clean-contaminated wounds. CONCLUSIONS: At ≥5 years of follow-up, use of PADM as a bioprosthesis in ventral hernia in high-risk patients is unreliable as a definitive repair in the majority of patients, but may provide satisfactory outcomes in some patients.

Prevascular hernias of the abdominal wall: a difficult problem, a difficult repair.

INTRODUCTION: Secondary prevascular hernias occurring anterior to the iliofemoral vessels and involving the lower abdominal wall are rare. These hernias can be difficult to repair because of loss of the inguinal ligament and the musculofascial elements of the inguinal region. MATERIALS AND METHODS: We describe a new technique of repair using an intraperitoneal sling of prosthetic material. The sling is sewn posteriorly to the anterior surface of the sacrum, and is then draped caudally to be sewn to the pubis and Cooper's ligaments before being reflected anterior to be sewn to the posterior surface of the anterior abdominal wall. This sling "patches" the hernia defect as a form of intraperitoneal sublay. CONCLUSIONS: We describe three such patients with satisfactory results at follow-ups of 8-16 months. Potential problems and considerations are discussed.

[Post operative Caldwell-Luc procedure maxillary cyst: Report of a case]. / Syndrome post Caldwell Luc: à propos d'un cas.

Rare complication of radical surgery (Caldwell Luc procedure) of the maxillary sinus, maxillary cyst can occurred several years after. We describe the case of a patient 56 years old, who came to our consultationfor repeated pain in the right maxillary region with moderate facial asymmetry. In her history we noted a previous sinus surgery (Caldwell Luc) more than 10 years before. The imaging showed an expansive process filled with proteinic tissue of the posterior wall of the maxillary. With the history of the former surgery, we suspected the existence of a mucocele. She underwent an endonasal surgery and the pathological examination of the specimen revealed an intra osseous cyst lined with ciliated respiratory type mucosa post surgery of the maxillary sinus. The diagnosis of a maxillary cyst complicating a Caldwell Luc was established based on the results obtained with the clinico-radiological and pathological results.

Distribution of TMEM100 in the mouse and human gastrointestinal tract--a novel marker of enteric nerves.

Identification of markers of enteric neurons has contributed substantially to our understanding of the development, normal physiology, and pathology of the gut. Previously identified markers of the enteric nervous system can be used to label all or most neuronal structures or for examining individual cells by labeling just the nucleus or cell body. Most of these markers are excellent but have some limitations. Transmembrane protein 100 (TMEM100) is a gene at locus 17q32 encoding a 134-amino acid protein with two hypothetical transmembrane domains. TMEM100 expression has not been reported in adult mammalian tissues but does appear in the ventral neural tube of embryonic mice and plays a role in signaling pathways associated with development of the enteric nervous system. We showed that TMEM100 messenger RNA is expressed in the gastrointestinal tract and demonstrated that TMEM100 is a membrane-associated protein. Furthermore TMEM100 immunoreactivity was restricted to enteric neurons and vascular tissue in the muscularis propria of all regions of the mouse and human gastrointestinal tract. TMEM100 immunoreactivity colocalized with labeling for the pan-neuronal marker protein gene product 9.5 (PGP9.5) but not with the glial marker S100ß or Kit, a marker of interstitial cells of Cajal. The signaling molecule, bone morphogenetic protein (BMP) 4, was also expressed in enteric neurons of the human colon and co-localized with TMEM100. TMEM100 is also expressed in neuronal cell bodies and fibers in the mouse brain and dorsal root ganglia. We conclude that TMEM100 is a novel, membrane-associated marker for enteric nerves and is as effective as PGP9.5 for identifying neuronal structures in the gastrointestinal tract. The expression of TMEM100 in the enteric nervous system may reflect a role in the development and differentiation of cells through a transforming growth factor ß, BMP or related signaling pathway.

Interactions of allelic variance of PNPLA3 with nongenetic factors in predicting nonalcoholic steatohepatitis and nonhepatic complications of severe obesity.

OBJECTIVE: Allelic variation (rs738409C→G) in adiponutrin (patatin-like phospholipase domain-containing protein 3, PNPLA3) has been associated with hepatic steatosis and liver fibrosis. The physiologic impact of the PNPLA3 G allele may be exacerbated in patients with severe obesity. In this study, we investigated the interactions of PNPLA3 rs738409 with a broad panel of metabolic and histologic characteristics of nonalcoholic fatty liver disease and nonalcoholic steatohepatitis (NASH) in patients with medically complicated obesity. DESIGN AND METHODS: Consecutive patients undergoing bariatric surgery were selected for a prospective study. They underwent extensive laboratory and histologic (liver biopsy) assessment, as well as evaluation of rs738409 polymorphism by TaqMan assay. RESULTS: Only 12 (8.3%) of the 144 patients had normal liver histology, with 72 (50%) NASH, of whom 15 (10.4% of total patients) had fibrosis stage 2-3. PNPLA3 GG genotype correlated positively (P < 0.05) with serum levels of alanine aminotransferase (ALT), asparate aminotransferase (AST), glucose, fibrinogen, and insulin-dependent diabetes mellitus, homeostasis model assessment-insulin resistance, and presence of NASH. Multivariate analysis indicated that PNPLA3 rs738409 G versus C allele remained an (independent) risk factor for NASH, in addition to CK-18 >145 IU/l, glucose >100 mg/dl, and C-reactive protein (CRP) >0.8 mg/dl. The probability of NASH increased from 9% (no risk factor) to 82% if all four risk factors were present. CONCLUSIONS: In this cohort of patients with medically complicated obesity, PNPLA3 rs738409 G allelic expression is associated with hepatic (NASH) and nonhepatic complications of obesity, such as insulin resistance. These novel findings may be related to a greater impact of PNPLA3 variant in magnitude and scope in patients with severe obesity than in less obese populations. Further studies are needed to characterize the nature of these associations.

[Aneurysmal dilatation of pulmonary artery and its branches on mitral stenosis: a case report]. / Dilatation anévrismale de l'artère pulmonaire et de ses branches sur sténose mitrale à propos d'un cas.

Pulmonary arteries aneurysms are rare and the rarely described bilateral aneurysms. A 45-year-old patient carrier of mitral stenosis was admitted for dyspnoea class III of the NYHA, chest pain and a not infectious cough. The clinical examination found semiology of mitral stenosis, tricuspid incapacity and pulmonary arterial hypertension. The electrocardiogram showed atrial fibrillation and right ventricle hypertrophy. Chest X-ray found a cardiomegaly, an aspect of double outline of the inferior right bow, a prominent aspect of the left average bow reminding an aneurysm of the left pulmonary artery. The echocardiography Doppler found a pure tight mitral stenosis (mitral surface=0.6 cm(2)), a dilation of the trunk of the pulmonary artery (diameter=74 mm) and of its branches (diameter of the right pulmonary artery=28 mm, diameter of the left pulmonary artery=36 mm) seat of a spontaneous contrast. The left atrium and right cardiac cavities were also dilated. There was an important tricuspid incapacity with a major pulmonary hypertension (systolic pulmonary arterial=109 mmHg). The thoracic angioscan showed a pseudoaneurysm of the trunk of pulmonary artery and its branches to their distal parts. Under diuretic, anticoagulating and analgesic treatment the clinical signs improved however the spontaneous contrast persisted. The patient was rejected by the surgery for exceeded clinical board. She is at present followed in our service for 5 months.

Schistosoma hematobium-associated glomerulopathy.

Schistosomiasis is the second most devastating tropical parasitic disease worldwide and is responsible for many urological complications. However, glomerular injury is a rare complication mainly described with Schistosoma mansoni. We report a case of membranoproliferative glomerulonephritis (MPGN) associated with Schistosoma hematobium infection in a young Senegalese boy living in a rural area. Clinical presentation was with steroid-resistant with nephrotic syndrome. Renal biopsy showed type 1 MPGN with the presence of S. hematobium eggs surrounded by a gigantocellular granuloma. Despite therapy with antihelminthic and immunosuppressive drugs, evolution was characterized by progression to end-stage renal disease over 1 year. More efforts should be made on the prevention and early detection of schistosomiasis among at-risk populations.

The vascular endothelium masks the persistent inhibition of rat thoracic arterial tone induced by S-nitrosoglutathione.

AIM: In endothelium-denuded arteries, the nitric oxide (NO) donor S-nitrosoglutathione (GSNO) induced a persistent hypo-reactivity to vasoconstrictors, and low-molecular weight thiols such as N-acetyl cysteine (NAC) produced a relaxant effect. These effects were attributed to the formation of vascular NO stores. In arteries with a functional endothelium, such long-lasting effects on arterial tone have not been well characterised. In this study, we proposed to examine the possibility of storing exogenous NO when the vascular endothelium is still able to produce its own NO. METHODS: For this purpose, changes in isometric tension of isolated arteries were assessed in organ chambers, and nitrosothiol formation was characterised by confocal microscopy. RESULTS: In rat aortic rings with endothelium pre-exposed to GSNO, the contractile response to norepinephrine (NE) was not attenuated in comparison with control rings, but NAC induced a relaxant effect. However, an attenuation of the response to NE was observed in GSNO-exposed, intact aortic rings after inhibition of NO synthase by N(ω)-nitro-L-arginine methylester (L-AME) or in GSNO-denuded rings. The relaxing effects of NAC were due to the mobilisation of NO from nitrosothiols after nitrosylation of protein SH residues. Moreover, the hypo-reactivity to NE and the relaxant effect of NAC were abolished by 1H-[1,2,4] oxadiazolo(4,3-a)quinoxalin-1-one (ODQ), an inhibitor of soluble guanylyl cyclase, and partially by the K+-sensitive channel inhibitor tetra-ethyl-ammonium (TEA). CONCLUSION: These data show that endothelium-derived NO masked the persistent effect of GSNO in rat thoracic aorta. However, the ability of GSNO to form releasable NO stores without altering the vascular tone can be particularly useful in preventing endothelial dysfunction in which NO formation decreases.

Changes in interstitial cells of cajal with age in the human stomach and colon.

BACKGROUND: Aging produces inevitable changes in the function of most organs including the gastrointestinal tract. Together with enteric nerves and smooth muscle cells, interstitial cells of Cajal (ICC) play a key role in the control of gastrointestinal motility, yet little is known about the effect of aging on ICC. The aim of this study was to determine the effect of aging on ICC number and volume in the human stomach and colon. METHODS: Gastric and colonic tissues from patients aged 25-70 and 36-92 years old, respectively, and with no co-existent motility disorders were immunolabeled with an anti-Kit antibody and ICC were counted in the circular muscle and myenteric regions. Network volumes were measured using 3D reconstructions of confocal stacks. The effects of aging were determined by testing for linear trends using regression analysis. KEY RESULTS: In both stomach and colon, the number of ICC bodies and volume significantly decreased with age at a rate of 13% per decade. ICC size was only affected in the myenteric plexus in the colon. The changes associated with age were not differentially affected by sex or colonic region. CONCLUSIONS & INFERENCES: The number and volume of ICC networks in the normal human stomach and colon decline with age. This decrease in ICC likely reduces the functional capacity of the gastrointestinal motor apparatus, may contribute to changes in gastrointestinal motility with aging and may influence intestinal responses to insults such as disease, operative interventions and medications in older patients. Tissue specimens must be carefully age-matched when studying ICC in disease.

Obstructive sleep apnoea and perioperative complications in bariatric patients.

BACKGROUND: The objective of this study was to determine the relationship between perioperative complications and the severity of obstructive sleep apnoea (OSA) in patients undergoing bariatric surgery who had undergone preoperative polysomnography (PSG). METHODS: The records of 797 patients, age >18 yr, who underwent bariatric operations (442 open and 355 laparoscopic procedures) at Mayo Clinic and were assessed before operation by PSG, were reviewed retrospectively. OSA was quantified using the apnoea-hypopnoea index (AHI) as none (≤ 4), mild (5-15), moderate (16-30), and severe (≥ 31). Pulmonary, surgical, and 'other' complications within the first 30 postoperative days were analysed according to OSA severity. Logistic regression was used to assess the multivariable association of OSA, age, sex, BMI, and surgical approach with postoperative complications. RESULTS: Most patients with OSA (93%) received perioperative positive airway pressure therapy, and all patients were closely monitored after operation with pulse oximetry on either regular nursing floors or in intensive or intermediate care units. At least one postoperative complication occurred in 259 patients (33%). In a multivariable model, the overall complication rate was increased with open procedures compared with laparoscopic. In addition, increased BMI and age were associated with increased likelihood of pulmonary and other complications. Complication rates were not associated with OSA severity. CONCLUSIONS: In obese patients evaluated before operation by PSG before bariatric surgery and managed accordingly, the severity of OSA, as assessed by the AHI, was not associated with the rate of perioperative complications. These results cannot determine whether unrecognized and untreated OSA increases risk.