Periosteal preservation: a new technique in resection of bone high-grade malignant tumors in children-about eleven cases.

OBJECTIVE: The purpose of this study was to describe a surgical technique of bone resection with periosteal preservation and reconstruction in patients with high-grade bone malignant tumors and to determine its effect on local recurrences, and time and quality of bone union in bone autografting reconstruction. PATIENTS AND METHODS: We retrospectively reviewed 11 cases of high-grade malignant bone tumors in children aged 4 to 16 years, who were treated with chemotherapy and tumor resection while preserving partially the adjacent periosteum. Tumors were located in the lower limb in eight cases; three tumors were in the humerus. The mean length of the bone defect after resection was 15.8 cm (range, 6-34.5 cm). Reconstruction was provided by non-vascularized autograft in eight cases (lower limb) and polymethyl methacrylate spacer in three cases (upper limb). Patients were followed up for a mean of 71 months. RESULTS: At the last follow-up, no patients had local recurrence. Three patients were dead because of metastasis. Bone union was good in time and quality in all children who had bone autografting. In cases of PMMA reconstruction, there was periosteal bone formation around the spacer. According to the MSTS functional score, patients with lower limb localizations had a mean score of 27.75 points and patients with upper limb localizations had a score of 24/30. CONCLUSION: Preservation of the periosteum in bone resection for malignant tumors could be a good adjuvant alternative for bone reconstruction, without increasing the risk of local recurrence. However, patients must be carefully selected.

A rare case report of a neonatal idiopathic intussusception in a full-term newborn.

INTRODUCTION: In contrast with the usual idiopathic intussusception appearing in infants, neonatal intussusception in full-term newborns is a rare entity and usually due to an organic lead point lesion. It has a misleading and variable presentation. This manuscript reports a very rare case of neonatal idiopathic intussusception in a full-term male newborn in order to highlight the difficulties in establishing an early diagnosis. PRESENTATION OF CASE: We present a full-term male newborn who was referred to our department at day 7 of life with fecaloid vomiting, distended abdomen and absence of intestinal transit. No bloody stool was identified. The newborn was in poor condition, dehydrated and hypothermic. He was promptly resuscitated and an abdominal ultrasound was quickly performed, it showed an ileocecal intussusception located in the right hypochondriac region. An emergency laparotomy was performed confirming the ultrasound findings. The pathological exam of the resected bowel confirmed the presence of an intussusception with areas of hemorrhagic rearrangements but no lead point was detected. The patient died from septicemia. CONCLUSION: Intussusception occurring in the neonate is still difficult to assess, because of its rarity and its uncommon mode of presentation. This case report underlines the importance to make early diagnosis, because once a critical condition develops, the mortality rate is likely to rise.

Pediatric Noncerebral Thromboembolism: Etiological Assessment and Outcome.

OBJECTIVE: To report clinical presentation and etiologic investigation findings during pediatric noncerebral thromboembolism. METHODS: Retrospective study of cases of vascular non cerebral thromboses admitted in Medicine infantile A Department of the Children's Hospital of Tunis over 08 years. RESULTS: We confirmed 14 cases of non cerebral vascular thromboses. So that these accidents constitute 0,26 ‰ of the overall etiologies of hospitalizations in the Department. The mean age of our patients was 56±41 months [25 days-12 1/2 years]. The sex ratio was 1.8. The vascular incident was venous in 2/3 of cases. The clinical presentation was mainly painful swelling in four cases, abrupt dyspnea and hematemesis in three cases each and the incident was locally asymptomatic in four cases. Thromboses locations included deep vein thrombosis of limbs (n=6), vena cava thrombosis (n=1), portal thrombosis (n=4) and pulmonary embolism (n=3). The promoting factors identified were: tumors in seven cases, thrombophilias and catheterization in four cases each, trauma, surgery and Behçet disease in one case each. Eleven patients received anticoagulant treatment including unfractioned heparin in three cases and low molecular weight heparin in the other cases. No one died while four patients developed sequelae. CONCLUSION: Vascular thromboses are rare in children. They are mostly venous and diagnosed in ill children especially those having central venous catheters. Outcome of pediatric thromboembolism depends on efficient anticoagulation therapy which is well tolerated by children.

Rare retro peritoneal tumor in infants.

Lipoblastoma is a rare mesenchymal tumor, occuring in less than three-year-old infants in 90% of the cases and in 40% of the cases in chidren aged less than one year. It can be present at birth. It is a benign tumor, with defined margins, that can be locally aggressive, without metastases. We report the case of retroperitoneal lipoblastomatosis, with a neonatal diagnosis made by ultrasonographic and CT features.

Prognostic factors in children with extracranial malignant germ cell tumors: a monocentric pediatric Tunisian study.

Background Extracranial Germ cell tumors (GCT) are a rare and a heterogeneous group of pediatric cancers but highly curable. Aim We aimed to review management, outcome and prognostic factors that influence overall survival (OS) in a pediatric Tunisian oncologic unit. Methods We retrospectively evaluated between January 1998 and December 2012, 33 patients affected by extracranial germ cell tumors and treated according to TGM95 protocol established by the SFOPin a pediatric Tunisian oncologic unit. Results Patients had a mean age of 57 months (ranges: 1 day-13 years). There were 19 girls and 14 boys. Primary sites included 12 sacrococcygeal, 11 ovarian, 6 testicular, 3retro peritoneal and 1 mediastinal site. After a mean follow up of 26.1 months (ranges: 0-96 months), OSat 2 years and 5 years were respectively 82% and 75%. Event-free survival were respectively 79% at 2 years and 74% at 5 years. Various prognostic factors have been studied according to Kaplan-Meier. Univariate analyses identified significant factors which influence strongly OS: the stage (p=0.04), the completeness of surgery (p<0.001) and the relapse (p = 0, 0001). A multivariate study showed that only the quality of resection and the clinical stage remained strong significant prognostic factors (p=0,021) for 5-year OS. Conclusion Disease stage, completeness of surgery and relapse have been established as the most powerful prognostic parameter in our analysis. The improvement of survival of patients affected by extracranial germ cell tumors in Tunisia is a real achievement mainly due to the success of salvage treatments.

Co-occurrence of Takayasu's arteritis and tuberculosis: Report of a Tunisian pediatric case.

Few reports on co-occurrence of Takayasu's arteritis (TA) and tuberculosis (Tb) have been published in childhood. A 12-year-old girl presented with 4-month's history of a dry cough, persistent fever, marked weakness, and weight loss. Physical examination revealed impalpable peripheral pulses and unrecordable blood pressure (BP) on upper limbs. In lower limbs, peripheral pulses were normal and BP reached respectively 160/90 and 140/87 mmHg. Laboratory investigations showed an erythrocyte sedimentation rate at 140 mm in the 1(st) h and microcytic anemia (8.6 g/dl). Doppler ultrasound and computed tomography angiography revealed significant thickening of the aortic-arch and both common carotid arteries wall, with luminal narrowing of the right common carotid and its branches and severe stenosis of the left subclavian artery. Simultaneously, the diagnosis of active pulmonary Tb was achieved based on radiological data, positive Mantoux test and successful response to antitubercular drugs. During follow-up, corticostetroids and methotrexate were required to control TA relapses.

Management of congenital bladder diverticulum in children: A report of seven cases.

BACKGROUND: The purpose of the study is to present the author's experience with congenital bladder diverticula in seven pediatric patients at a developing world tertiary care center. MATERIALS AND METHODS: Records of seven patients diagnosed and treated as congenital bladder diverticulum, from January 1998 to December 2009 were retrospectively reviewed for age, sex, clinical symptoms, investigative work-up, operative notes, and postoperative follow-up. RESULTS: All patients were males. Age at presentation ranged from six months to six years (mean three years and six months). All were manifested postnatally by urinary tract infection in four cases, bladder retention in three cases and abdominal pain in two cases. Diagnosis was suggested by ultrasound and confirmed by voiding cystourethrography (VCUG) in all cases and urethrocystoscopy in three cases. Open surgical excision of diverticulum was done in all the patients associated with ureteral reimplantation in four patients with VCUG-documented high-grade vesicoureteral reflux (VUR). Average follow-up was four years; there is a resolution of symptoms and no diverticulum recurrence at the defined mean follow-up. CONCLUSION: Recurrent urinary tract infections and voiding dysfunction in pediatric population should always be evaluated for congenital bladder diverticulum. Investigations such as abdominal ultrasound, VCUG and nuclear renal scanning, form an important part of preoperative diagnostic work-up and postoperative follow up. Diverticulectomy with ureteral reimplantation in case of high-grade reflux, provides good results without recurrence.

Neurological variant of Lemierre's Syndrome with purulent meningitis: a case report and literature review.

We report an unusual variant of Lemierre's Syndrome (LS) in a 10-year-old-girl admitted to the intensive care unit for septic shock with meningitis. The primary infection was otitis media. A gram negative bacillus was identified in the direct exam of the purulent ear discharge and the cerebrospinal fluid but cultures were negative. Computerized tomography of the neck revealed a thrombus in the internal jugular vein. Septic shock improved rapidly under supportive treatment. The patient recovered without sequellae after a prolonged duration of parenteral antibiotherapy and hospital stay. Neurologic variants of LS with meningitis, previously reported in the literature, are reviewed.

[Granulocytic sarcoma of the maxillary sinus: a pediatric case report]. / Sarcome granulocytaire du sinus maxillaire. À propos d'un cas pédiatrique.

BACKGROUND: Granulocytic sarcoma is a rare malignant extramedullary neoplasm of myeloid precursor cells, occuring before or after onset of leukaemia. Involvement of the head and neck region is rare, generally concerning the orbit. AIM: To illustrate imaging findings of granulocytic sarcoma in an unusual location; maxillary sinus. CASE: We report a case of maxillary sinus granulocytic sarcoma in a 13-month old boy revealed by facial nerve palsy, ptosis and jugal swelling, without any evidence of haematological disorders. The patient underwent computed tomography and magnetic resonance imaging exam which demonstrated a non specific maxillary sinus mass with destruction of the orbit floor and the alveolar bone. Diagnosis was obtained after bone biopsy allowing histological and immunohistochemical studies. CONCLUSION: Granulocytic sarcoma is a serious condition because of the associated hematologic disorders. In a sinus or orbit location, imaging features are non specific. Unless hematologic history is present, diagnosis is difficult and an immuno histo chemical study is required.

[Management of blunt duodenal and pancreatic injuries in children (about a series of 8 cases)]. / Prise en charge des traumatismes fermés du bloc duodéno- pancréatique chez l'enfant (a propos d'une série de 8 cas).

AIM: The aim of this retrospective study is to report eight cases of blunt duodeno-pancreatic trauma in infants, emphasizing on the role of imaging in acute assessment of the lesions and in further management. METHODS: We reported eight cases of duodeno-pancreatic injuries between 2006 and 2008, 5 boys and 3 girls with an age ranging from 3 to 12 years (median age: 7 years). Trauma circumstances were: car accident (n=2), domestic injury (n=5) and bicycle's fall injury (n=1). All patients underwent abdominal ultrasonography and CT scan in the initial evaluation and during the follow-up. RESULTS: Imaging showed the following pancreatic lesions: 3 corporeal fractures, 2 caudal fractures and one between the corporeal and the caudal portions. Four pancreatic haematomas were found. The associated lesions were duodenal, splenic, hepatic and renal. Two isolated duodenal haematomas were found. Two patients improved spontaneously, the six others developed complications: 4 acute pancreatitis, two infections, 3 pseudocysts and one retroperitoneal collection. Management was chirurgical in one case, medical in two cases, endoscopic in 2 cases and three percutaneaous drainages were performed. CONCLUSION: Blunt duodeno-pancreatic injuries in children have to be evaluated by an early imaging modality, in order to perform acute assessment of the lesions. Primary conservative treatment is advocated while clinic, biologic and imaging follow-up is required to detect complications, which management can be endoscopic, percutaneous or surgical.

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.

Megalencephalic leukoencephalopathy with subcortical cysts in a Tunisian boy.

Megalencephalic leukoencephalopathy with subcortical cysts is an autosomal recessive cerebral white-matter disorder in children. Megalencephalic leukoencephalopathy with subcortical cysts is caused by mutation in the megalencephalic leukoencephalopathy with subcortical cysts gene 1. The authors report a case of a 2-year-old Tunisian boy who had presented seizure following minor head trauma. He had megalencephaly, cerebellar ataxia, and mild pyramidal signs at physical examination. Magnetic resonance imaging showed extensive white-matter signal changes involving the corpus callosum and temporal subcortical cysts consistent with megalencephalic leukoencephalopathy with subcortical cysts. The molecular genetic study of the megalencephalic leukoencephalopathy with subcortical cysts gene 1 identified a homozygous mutation C46fsX57 in the patient. The mutation was found to be heterozygous in the parents. To the best of the authors' knowledge, this mutation has never been reported.

Pyelonephritis xanthogranulomatous in childhood: case report and literature review.

BACKGROUND: Xanthogranulomatous pyelonephritis (XGP) is a specific form of chronic inflammatory kidney disease rarely seen in children. The Symptoms are often vague and non-specific AIM: the aim of this paper is to return the particularities of imaging features in xanthogranulomatous pyelonephritis, insisting on differential diagnosis with renal tumors, especially in case of no renal stone or tract obstruction evidence. CASE: We report a case of xanthogranulomatous pyelonephritis in a 2-year-old boy involving the lower renal pole which demonstrates the diagnostic difficulties encountered in this disease CONCLUSION: Xanthogranulomatous pyelonephritis is a rare condition in children and should be included in the differential diagnosis of a child presenting a renal mass.