Place of Laparoscopy in Peritoneal Tuberculosis.

Introduction: Peritoneal tuberculosis (PT) is a rare form of extrapulmonary tuberculosis in children. The diagnosis is difficult because of its clinical polymorphism. Laparoscopy is the gold standard to make the diagnosis by exploring and performing peritoneal biopsies. Our aim was to show the place of laparoscopy in the diagnosis of PT, to compare the anatomopathological results of peritoneal biopsies with a macroscopic appearance to quick start antituberculosis treatment. Case Reports: We reported 4 patients with PT, 3 girls and 1 boy. The middle age was 9 years old. The revealing symptomatology was ascites in all cases. Radiological exploration was not contributing. Laparoscopy was performed for all patients. The exploration revealed an agglutination of the intestinal loops with the presence of whitish micronodules scattered over the entire abdominal cavity. Peritoneal biopsies were done in all cases. Histological examination confirmed the diagnosis of PT in all patients and antituberculosis treatment was introduced. There was a good clinical evolution with a follow-up of 30 months. Conclusion: PT is a public health problem due to its clinical and biological polymorphism. Laparoscopy with peritoneal biopsies remains the essential means for the diagnosis of this pathology in children.

Primary hydatid cyst of the small intestine masquerading as intestinal duplication in a child.

Hydatid disease or hydatidosis is a worldwide zoonosis disease caused by the tapeworm of Echinococcus granulosus and still widely endemic in Tunisia especially in rural areas where the sheep-dog cycle is dominant. It is an important public health problem in the pediatric age group causing significant morbidity and mortality. We report a case of primary hydatid cyst of the small intestine in a child and we want to highlight the difficulty that we meet in the diagnosis despite the contribution of imaging.

[Clinical and biological study of the 31 cases of gallstones]. / Étude clinique et biologique de 31 cas de lithiase biliaire.

This study reports the clinical and biological signs, as well as the morphological aspect and the chemical composition of the calculus during the biliary stones. The study population consisted of 31 patients with an average age of 49 years (30 women and one man) with biliary lithiasis and who had cholecystectomy. Hepatic colic and epigastralgia were the most evocative clinical signs. The calculus were pigmentary (n=6), cholesterolic and mostly single (n=18), and mixed (n=6) and one infectious multiple lithiasis. Cholesterol was found in 22 calculi (70.96%). We have found a significant increase in liver enzymes and total bilirubin, which is more pronounced in pigmentary lithiasis. Our results showed that most gallstones were composed of cholesterol. These results indicate the influence of diet and chronic hemolysis in calculus formation. More investigation should allow knowing the nutritional and environmental factors influencing gallstones formation in Tunisia, in order to prevent this disease.

Alimentary tract duplications in children: report of 16 years' experience.

BACKGROUND: Alimentary tract duplications (ATDs) are a rare condition in children, characterised by a large pathogenic, clinical, and histological polymorphism. Surgical observation and pathologic evaluation of the resected specimens are the only way to confirm the diagnosis. In this study, we want to analyse the anatomical, clinical and therapeutic aspects of this entity. PATIENTS AND METHODS: A total of 12 cases of ATD were diagnosed over a 16-year period at paediatric surgery department. The diagnosis was evoked on clinical and radiological data. Histological study of the resected specimens confirmed the diagnosis in all cases. RESULTS: The mean age of patients at diagnosis was 41 months with a peak of incidence at the 1 st year of life (42%). Out of a total 12 cases, 10 were girls and 2 were boys. Abdominal pain and vomiting were the most frequent presenting features. Ultrasonography, tomodensitometry and magnetic resonance imaging were useful for diagnosis. ATDs were localised on the oesophagus in one case, the stomach in one case, the duodenum in four cases, the ileum in five cases, and the colon in one case. All these duplications were cystic, with three communicating duplications. All patients underwent surgery, and resection procedure was chosen according to duplication type and site. Histological study confirmed the diagnosis in all cases. CONCLUSION: ATDs are a rare condition in children. Diagnosis relies on histology, and treatment can only be by means of surgery. The outcome after surgery is generally favourable. Diagnosis and precocious surgery of ATDs can warn serious complications.

Peptic oesophageal stricture in children: management problems.

BACKGROUND: Peptic oesophageal stricture (PES) is a serious complication of gastroesophageal reflux disease (GERD) in childhood. The treatment of PES is still controversial, ranging from simple oesophageal dilations to resection/anastomosis of the stenotic portion of the oesophagus. In this study, we want to share our experience with 11 children with GERD and PES. PATIENTS AND METHODS: A retrospective review of clinical data obtained from children who underwent dilation and antireflux surgery for PES was performed. RESULTS: A total of 11 patients were diagnosed with PES. The clinical picture was dominated by dysphagia. Barium swallow showed hiatal hernia in nine cases (82%). Oesophageal strictures were located most commonly in the lower third of the oesophagus (91%). Three Children (27%) with PES had a neurologic impairment and patients had a mean duration of symptoms of 20 months (range, 3 month to 6.2 years) before intervention. Children received a median of four dilations (range, 1-21 dilations) for PES. Time to first dilation from age of diagnosis was a mean of 4.5 months (range, 2-14 months). Antireflux surgery was performed in all patients. Post-operatively, seven patients required repeat oesophageal dilation. Patients were followed with serial dilation for a median of 6 years (range, 1-9 years) and only one patient has a continued requirement of oesophageal dilation for PES. CONCLUSION: GERD complicated by PES is an important condition affecting a significant number of children. Early and effective treatment of both stricture and GERD is required to improve the prognosis of this serious condition.

Ureterocele containing a stone in a duplex system.

The formation of calcified stone in the ureterocele, a frequent event in adults, is very uncommon in a pediatric age. We present a case of a ureterocele containing a calcified stone in an 8-year-old girl. The diagnosis was made with radiological investigations (intravenous pyelogram, ultrasonography and, eventually, voiding cystourethrogram). Meatostomy and calculus extraction, or open surgery in more complicated cases, are the treatments of choice.

[Congenital pulmonary malformations: clinical, radiological and treatment features]. / Les malformations broncho-pulmonaires: aspects diagnostiques radiologiques et thérapeutiques.

BACKGROUND: Congenital broncho-pulmonary malformations (CBM) are rare, essentially presented by congenital lobar emphysema, bronchogenic cysts, pulmonary sequestrations and cystic adenomatoid malformations. The diagnosis can be in prenatal. In postnatal, symptoms are variable. Radiological investigations lead to diagnosis in all cases. AIM: To study the principal clinic, radiologic and therapeutic of the congenital broncho-pulmonary malformations through ten cases. METHODS: Retrospective study of 10 cases of congenital bronchopulmonary malformations diagnosed between 2003 and 2010 in our institution. RESULTS: The mean ages at the time of diagnosis is 2 months (4 days to 16 months). The sex ratio is 1. The symptoms consisted of recurrent pneumonia in 4 cases, respiratory distress in 2cases, bronchiolite in 2 cases and 2 cases of antenatal diagnosis. All patients have a chest X-ray, night patients have a chest computerized tomography and one patient has a bronchial endoscopy. Ten cases of BPM have been investigated: five congenitals lobar emphysema, tow pulmonary sequestrations, tow cystic adénomatoid malformation and one bronchogenic cyst. Eight patients required surgical treatment involving pneumonectomy (1 case), lobectomy (5 cases),segmentectomy (1 case) and in 1 case the pulmonary sequestration was treated by ligature of the anomalous artery with pulmonary resection. The histopathological examination confirmed the diagnosis in all cases. The postoperative period was uneventful in 8 cases with a mean of follow-up of 2 years (5 months to 5years). Tow patient died after surgical treatment. CONCLUSION: The diagnosis of BPM malformations can be clinical, confirmed by radiological investigations. The improvement in prenatal ultrasound diagnosis modified the management strategy. The treatment varies frome attitude conservatrice to pneumonectomy.

[Primary psoas abscesses of the psoas muscle in children]. / Les abcès primitifs du psoas chez l'enfant.

BACKGROUND: Primary psoas abscesses of the psoas muscle are relatively rare in childhood and can determine problems of diagnosis and therapeutic assumption. AIM: To demonstrate that ultrasonography is an excellent means in diagnosis and treatment of psoas abscess in children limiting the use of CT more costly and radiant and the surgery that is decaying. METHODS: Over a 15 years period (January 1995-december 2009), 16 children with psoas abscess were studied retrospectively. The diagnosis gait was based on questioning, clinical examination and imaging techniques and, in first sight, ultrasonography. RESULTS: Median age of our patients was 6.4 years (extremes 18 months-14 years) and mean delay of evolution was 12 days. Fever and lumbar pain were both constants, associated with another signs like painful boitery, psoitis, mictional burns and lumbar mass. The ultrasonography made in 16 patients allowed to the diagnosis in all cases. The tomodensitometry, made in 3 patients, confirmed the ultrasonography. Echo guided percutaneous drainage, made in 14 cases, was successful in 12. The examination of pus showed S Aureus in 11 cases and E coli in the other. CONCLUSION: Ultrasonography is a very important means in the primary psoas abscess in children. In addition to give diagnosis, this technique allows the drainage and the cure of the collection, limiting the open surgery to some particular cases.

Primary hyperoxaluria type 1 in Tunisian children.

BACKGROUND: Primary hyperoxaliuria type 1 is an autosomal recessive disorder characterized by increasing urinary excretion of calcium oxalate, recurrent urolithiasis, nephrocalcinosis, and accumulation of insoluble oxalate throughout the body. This inborn error of metabolism appears to be a common cause of end stage renal disease in Tunisia. AIMS: To review the clinical, biological and radiological futures of primary hyperoxaluria type 1 and to correlate these aspects with the development of end-stage renal disease. METHODS: we retrospectively reviewed 44 children with Primary hyperoxaliuria type I who were treated in our department during a period of 15 years between 1995 and 2009. The diagnosis was established by quantitative urinary oxalate excretion. In patient with renal impairment, the diagnosis was made by infrared spectroscopy of stone or by renal biopsy. RESULTS: Male to female ratio was 1.2. The median age at diagnosis was 5.75 years. About 43 % of those were diagnosed before the age of 5 years. Initial symptoms were dominated by uraemia. Four patients were asymptomatic and diagnosed by sibling screening of known patients. Nephrocalcinosis was present in all patients. It is cortical in 34%, medullary in 32% and global in 34%. At diagnosis, twelve children were in end-stage renal disease (27%). Pyridoxine response, which is defined by a reduction in urine oxalate excretion of 60% or more, was found in 27%. CONCLUSION: In the majority of patients, the clinical expression of Primary hyperoxaliuria type 1 is characterized by nephrocalcinosis, urolithiasis and renal failure. Pyridoxine sensitivity is associated with better outcome.

[Uropathies and nephropathies of Bardet-Biedl syndrome in children]. / Uropathies et Nephropathies Dans Le Syndrome De Bardet-Biedl Chez L'enfant.

BACKGROUND: The Bardet-Biedl syndrome is an autosomal recessive disease, characterised by obesity, retinal degeneration, hypogenitalism in men, polydactylism and an often moderate mental retardation. With these cardinal features, others clinical findings (secondary features) including diabetes, congenital heart defects, hypertension or syndactyly can be seen. Renal involvement is almost constant, but varies from a moderate impairment of the tubular functions to chronic renal failure caused by malformative uropathy or glomerulopathy. AIM: Report a new cases. METHODS: We report 6 patients with Bardet-Biedel syndrome who had renal involvement. RESULTS: Three patients had cystic dysplasia, one patient an increased fractional sodium excretion, one other a vesico-ureteral reflux and the last patient developed end-stage renal failure following acute post streptococcal glomerulonephritis. We insist on precocious diagnosis and multidisciplinary treatment of these renal lesions, to ovoid or, at least, to slow down the evolution to the terminal renal failure, essential prognosis factor. CONCLUSION: Renal involvement, is considered as a major criteria predicting high morbidity and mortality during Bardet-Biedl disease.

Reversible nephrotic syndrome secondary to pulmonary hydatid disease.

Most patients with pulmonary hydatidosis are children. The disease may be asymptomatic or revealed by unusual events such as a glomerulopathy. An 8-year-old boy from a rural part of Tunisia presented with generalised oedema and macroscopic haematuria. There was no familial history of renal disease. He had a normal blood pressure (100/60 mmHg), and a pleural effusion was detected. Urinalysis showed nephrotic range proteinuria (375 mg/kg/d) and microscopic haematuria. His serum total protein concentration was 40 g/l and his serum albumin was 10 g/l. Renal biopsy showed capillary wall thickening and duplication, and mesangial cell proliferation in the glomeruli, characteristic of mesangiocapillary glomerulonephritis. Renal and abdominal ultrasound images showed increased echogenicity of the kidneys and mild ascites. Radiology revealed three large pulmonary hydatid cysts. The largest cyst occupied the entire right upper lobe and compressed the superior vena cava. Hydatid disease was confirmed by a strongly positive serum enzyme-linked immunosorbent assay (ELISA) for echinococcus. The patient was treated with high protein intake, dipyramidol and captopril; both right lung cysts were resected, followed by the left pulmonary cyst 4 weeks later. Hydatid cyst was confirmed histopathologically. He recovered well, the serum ELISA for echinococcus became negative, and follow-up urine examination and thoracic computerised tomography were normal 6 months after surgery, confirming good renal recovery and absence of pulmonary hydatid disease.

[Urinary tract infections in children following renal transplantation: a single-center experience]. / Infections urinaires de l'enfant transplante rénal: expérience d'un centre de néphro-pédiatrie.

BACKGROUND: Urinary tract infection (UTI) is the most common complication after kidney transplantation and represents a potential life-threatening risk for the immunocompromised child. AIM: The aim of this report is to determinate incidence, risk factors, microbiologic features and evaluate the impact of this complication on graft outcome and patient mortality. METHODS: We performed a retrospective cohort study reviewing the medical records of 17 children from 38 who received a renal transplant in our center between January 1992 and june 2008 and who present an urinary tract infection. RESULTS: All patients received Lich-Gregoire implantation and insertion of double-J stunt. Antibioprophylaxis was not systematic. After a mean period of 6 years, 9 children (5+4) developed early UTI (during the first month after transplantation) and 5 (3+2) had late UTI. Three patients (2+1) with an indeterminate nephropathy developed early and late UTI. Causal agents are: E. Coli, Klebsiella Pneumoniae and Candida albicans. The further voiding cystourethrography showed a vesico-ureteral reflux on graft in 5 cases. Among the 17 patients, 4 lost their graft and are actually on haemodialysis. CONCLUSION: The urinary tract infection represents the major complication after renal transplantation. Diagnosis ant treatment must be made early to avoid the loss of the graft.

[Posterior uretral valves. About a series of 44 cases]. / Valves de l'urètre postérieur. A propos de 44 cas.

OBJECTIVE: To explore the physical symptoms and radiological findings for the diagnosis of posterior urethral valves (PUV), to point of the therapeutic snags depending on gravity and delay of lesions. METHODS: We reviewed the records of 44 patients with PUV. Mean age: 2 years 1/2 (range 1 day-13 years). More than 75% of patients were less than 2 years old. RESULTS: In utero diagnosis was made in 8 patients (14%). After birth, the diagnosis was based on urological signs (as frequent as diagnosis is made belatedly) and extra urological signs. Cystourethrography (CUG) showed posterior urethral dilatation in all cases. Ultrasonography (US) showed abnormalities in 30 cases and intravenous urography (IVU), made in 21 cases, was normal in appearance in 7 of them. At diagnosis, it was a renal failure in 36 patients and 8 of them had a terminal renal deficiency. Urethroscopy made in 40 patients, showed PUV in all cases. There were 29 type I valves, 3 type II valves, 5 type III and 3 unclassifiables cases. Treatment was endoscopic in 41 cases and by lamination in the 3 others. Mean follow up is 9 years (range 16 months-19 years) and 19 patients have terminal renal failure from which 3 are deceased. CONCLUSION: PUV are dangerous obstructive uropathies in boy whom end at terminal renal failure in more than 33% of cases. Precocious diagnosis and early ablation of PUV are able to limit the complications inherent to this pathology.

Compound heterozygosity of novel missense mutations in the gamma-glutamyl-carboxylase gene causes hereditary combined vitamin K-dependent coagulation factor deficiency.

Hereditary combined vitamin K-dependent (VKD) coagulation factor deficiency is an autosomal recessive bleeding disorder associated with defects in either the gamma-carboxylase, which carboxylates VKD proteins to render them active, or the vitamin K epoxide reductase (VKORC1), which supplies the reduced vitamin K cofactor required for carboxylation. Such deficiencies are rare, and we report the fourth case resulting from mutations in the carboxylase gene, identified in a Tunisian girl who exhibited impaired function in hemostatic VKD factors that was not restored by vitamin K administration. Sequence analysis of the proposita did not identify any mutations in the VKORC1 gene but, remarkably, revealed 3 heterozygous mutations in the carboxylase gene that caused the substitutions Asp31Asn, Trp157Arg, and Thr591Lys. None of these mutations have previously been reported. Family analysis showed that Asp31Asn and Thr591Lys were coallelic and maternally transmitted while Trp157Arg was transmitted by the father, and a genomic screen of 100 healthy individuals ruled out frequent polymorphisms. Mutational analysis indicated wild-type activity for the Asp31Asn carboxylase. In contrast, the respective Trp157Arg and Thr591Lys activities were 8% and 0% that of wild-type carboxylase, and their compound heterozygosity can therefore account for functional VKD factor deficiency. The implications for carboxylase mechanism are discussed.

[Angiomatoid fibrous histiocytoma: rare tumor of intermediate malignancy]. / Histiocytome fibreux angiomatoide: une tumeur rare a malignite intermediaire.

Angiomatoid fibrous histiocytoma is a rare tumour affecting young adults. Unlike conventional malignant fibrous histiocytoma, its extension is only local thus giving a good prognosis. We report the cases of a 9 year-old girl and a 16 year-old boy presenting respectively, with an axillary tumour 5 cm of diameter and a paravertebral subcutaneous tumour 1.5 cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection.