S. Bousnina

Abdominal Tuberculosis in Children

Background: Abdominal tuberculosis (TB) includes infection of the gastrointestinal tract, peritoneum, mesentery, abdominal lymph nodes, liver, spleen, and pancreas. The most common forms of abdominal TB in children are adhesive peritonitis and nodal disease. Patients and Methods: We report our experience with abdominal TB treated in our hospital from 1995 to 2008. Results: Thirteen patients (3 boys and 10 girls) of mean age 9.8 years were diagnosed as having abdominal TB. Eight patients presented with abdominal distension and abdominal pain. Fever was seen in 4 patients. One patient had surgical abdominal pain and 2 had abdominal mass. Two patients had coexisting pleural effusion and 1 of them had multifocal TB. Abdominal TB involved peritoneum in 9, abdominal lymph nodes in 7, gastrointestinal tract in 3, spleen in 2 patients, and liver in 1. Ascitic fluid analysis of 9 patients showed exudative fluid with predominately lymphocytes. Laparotomy was performed in 3 patients. The diagnosis of abdominal TB was confirmed histopathologically in 5 patients and microbiologically in 3. The remaining patients had been diagnosed by ascitic fluid diagnostic features, abdominal imaging, tuberculin skin test, history of exposure, and a positive response to antituberculous treatment. Twelve patients completed the antituberculous therapy without any complications. One patient with multifocal TB had neurological sequelae. Conclusions: In the areas with a high prevalence of tuberculosis and confirmatory investigations are inadequately available, treatment may be initiated, based on strong clinical diagnosis and supportive investigations. In such situations, it is the response to therapy that indirectly proves the diagnosis.

Le carcinome mucoépidermoïde du poumon: À propos de 10 cas

Resume Les carcinomes mucoepidermoides (CME) du poumon sont rares. Ils sont classes en carcinomes de bas grade qui relevent dans la mesure du possible d’un traitement chirurgical conservateur et en carcinomes de haut grade qui doivent beneficier d’une chirurgie large suivie parfois de radiotherapie. Nous rapportons une serie de 10 CME du poumon (5 de bas grade et 5 de haut grade). L’âge moyen etait de 43,9 ans avec des extremes allant de 10 a 67 ans et le sex ratio etait de 8 hommes pour 2 femmes. Seuls 5 patients etaient tabagiques. Les 10 patients ont ete operes ; 8 ont eu une lobectomie et 2 une pneumonectomie. Une radiotherapie complementaire a ete pratiquee dans 2 cas de CME de haut grade. Trois malades (2 CME de haut grade et 1 CME de bas grade) sont decedes dans un tableau de metastases diffuses alors que les 7 autres, bien suivis, sont en bon etat general.Mucoepidermoid carcinoma of the lung is rare. The microscopic findings distinguish low grade and high grade tumors. Conservative surgical resection is appropriate if possible for low grade tumors. For the high grade tumors, combined wide surgical resection and radiotherapy is recommended. We report 10 cases of mucoepidermoid carcinoma of the lung (5 low grade, 5 high grade) in 8 male and 2 female patients with a mean age of 43.9 years. Only 5 of 10 were smokers. All 10 patients underwent surgery. Operative procedures included 8 lobectomies and 2 pneumonectomies. Two patients received radiation therapy postoperatively. Three patients (2 high grade and 1 low grade) died. The other 7 patients were alive without evidence of recurrence.

Congenital salivary gland anlage tumor of the nasopharynx.

Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may be present with variable symptoms. Salivary gland anlage tumor, also referred as congenital pleomorphic adenoma, is a very rare benign congenital tumor of the nasopharynx, which may produce nasal obstruction and other associated, nonspecific symptoms. We report a case of congenital salivary gland anlage tumor causing a severe neonatal respiratory distress with pulmonary hypertension. The tumor was removed and the outcome was favourable without recurrence at five years of the follow up.

L’embolie pulmonaire hydatique: À propos de 7 observations

Resume L’embolie pulmonaire hydatique est une pathologie rare, de diagnostic particulierement difficile, dont le traitement est mal codifie et dont le pronostic est generalement pejoratif. Nous avons effectue une etude retrospective concernant les dossiers de patients hospitalises entre 1994 et 2002, chez qui le diagnostic d’embolie pulmonaire hydatique a ete retenu. Sept observations ont ete colligees. Chez tous nos patients, la rupture du kyste dans la circulation s’est produite spontanement. Tous nos patients etaient symptomatiques. La radiographie thoracique a revele des images d’echinococcose metastatique pulmonaire dans tous les cas. Le scanner et/ou l’angioscanner thoracique ont permis de poser le diagnostic dans 5 cas. Dans un cas, le diagnostic a ete fait par l’angiographie pulmonaire, et dans un autre par les donnees de la chirurgie et de l’examen anatomo-pathologique. La recherche de la localisation hydatique primitive a permis de retrouver la notion de kystes hepatiques chez tous les patients associes dans un cas a un kyste de l’oreillette droite. Le kyste cardiaque n’a pas ete opere et tous les kystes hepatiques ont ete reseques. Une embolectomie a ete effectuee chez un seul patient. Un traitement medical a base d’albendazole a ete entrepris chez 5 patients. L’evolution a ete marquee par le deces de 2 patients dans un tableau d’hemoptysie abondante. Les 5 autres patients sont regulierement suivis, avec un recul allant de 6 mois a 5 ans. Le diagnostic de l’embolie pulmonaire hydatique repose essentiellement sur l’imagerie. Le traitement repose sur l’extirpation chirurgicale du foyer emboligene, puis en second lieu une embolectomie peut etre proposee. La place du traitement medical n’est pas encore clairement etablie. Le pronostic est generalement redoutable a plus ou moins breve echeance.

Multiple Congenital Anomalies/Mental Retardation Syndrome With Multiple Circumferential Skin Creases: A New Syndrome?

We describe a combination of multiple congenital anomalies, a severe psychomotor retardation and seizures in a 9-year-old Tunisian boy with circumferential ringed skin creases. He had symmetrical circumferential skin creases on arms, legs, and penis. Craniofacial anomalies included: an elongated face, tight forehead, hypertelorism, bilateral epicanthic folds, upslanting palpebral fissures, microphthalmia, convergent strabismus, wide nasal bridge, aberrant teeth, dental caries, and low-set posteriorly rotated ears with overfolded thick helices. He had also ureterocele, hypospadias, and others anomalies. The magnetic resonance imaging of the brain showed hypoplastic vermis, hypoplastic corpus callosum, and dilatation of ventricles. Chromosomal analysis revealed a normal male karyotype with 46,XY. Skin biopsy was normal. To the best of our knowledge, this combination of anomalies has not been reported and this case may be a unique syndrome.

Decompressive hemicraniectomy in a space-occupying presentation of hemiconvulsion–hemiplegia–epilepsy syndrome

A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling right hemisphere with marked temporal herniation. The baby underwent a decompressive right hemicraniectomy with temporal cortical biopsies. The post-operative course was favourable. The histological findings were unspecific, showing a gliotic spongiosis with disseminated granular cells. The post-operative MRI depicted a right hemisphere atrophy. To our knowledge, a space-occupying presentation of HHE syndrome requiring surgical decompression has never been described before while only a few reports dealt with the neuropathological aspects of this syndrome.

Atypical Teratoid/Rhabdoid Tumor of the Spine in a 4-Year-Old Girl

Primary spinal atypical teratoid/rhabdoid tumor is extremely rare. The authors present a case of atypical teratoid/rhabdoid tumor occurring in a 4-year-old girl. Magnetic resonance imaging The authors showed an intramedullary mass extending from the bulbomedullary junction to T1 with leptomeningeal dissemination. The patient died 2 weeks after diagnosis.

Tuberculous Meningitis in Bacille Calmette-Guerin–Vaccinated Children : Clinical Spectrum and Outcome

The Bacille Calmette-Guérin vaccination (BCG) contributed widely to reduce tuberculosis incidence in developing countries. The aim of this report was to assess the clinical “spectrum” and outcome of tuberculous meningitis in 16 Bacille Calmette-Guérin–vaccinated Tunisian children. They were 9 boys and 7 girls aged 2 to 168 months (median 72 months ± 65.88). Patients presented mainly with nonspecific symptoms. Neurologic severity was classified as grade I (n = 6) and grade II or III (n = 10). At short-term course, the majority of patients developed serious complications: hydrocephalus (n = 12), seizures (n = 8), tuberculoma (n = 6), and acute respiratory failure (n = 2). Three patients died. Among survivors, 4 patients showed a complete recovery while 9 developed permanent sequelae which were mild (n = 6) to severe (n = 3). Despite the Bacille Calmette-Guérin vaccination, tuberculous meningitis remains a life-threatening condition; vaccinated children have shown common presentation of tuberculous meningitis in terms of severity and poor outcome.

An Unusual Homozygous Arylsulfatase: A Pseudodeficiency in a Metachromatic Leukodystrophy Tunisian Patient

Metachromatic leukodystrophy is an autosomal recessive neurodegenerative lysosomal disease characterized by a deficiency of the lysosomal enzyme arylsulfatase A and the subsequent accumulation of sulfatide in neuronal and visceral tissues. Clinical diagnosis is usually confirmed by in vitro analysis of arylsulfatase A activity but may be complicated in cases of arylsulfatase A pseudodeficiency and sphingolipid activators protein deficiency. We report the case of a 3-year-old boy who presented a severe form of late infantile metachromatic leukodystrophy. This patient was found to be homozygous for the arylsulfatase A pseudodeficiency. This condition is rare and can lead to a severe disease. Prenatal diagnosis was performed in this family, and the fetus was healthy.

Central system nervous tuberculosis in infants.

The lack of specific symptoms and signs in patients with tuberculous meningitis makes early diagnosis difficult. In this report, we reviewed the clinical features and laboratory findings of 6 infants with central system nervous tuberculosis during a 10-year period. One of the patients had multifocal tuberculosis. The mean time to the diagnosis was 32 ± 13.4 days. A contact source was identified in only 2 patients. All 6 patients had abnormal cerebrospinal fluid findings, less than 500 cells/μL with lymphocytic predominance. Computerized tomography (CT) and/or magnetic resonance imaging (MRI) revealed hydrocephalus with basal enhancement in 2 patients. One patient developed pontocerebellar and pituitary tuberculomas, which were responsible for compression and diabetes insipidus, 1 year after antituberculous treatment. These localizations are very rare. On the follow-up, 3 patients had hypoacousia and only 1 had severe sequelae, despite a diagnostic delay.