Torsion of a wandering spleen: a case report.

BACKGROUND: Wandering spleen is a rare entity in child. It is generated by laxity or failure of development of spleen ligaments causing the migration of the spleen from its normal anatomical site to another abdominal or pelvic location. It can be congenital or acquired. The most dangerous complication is the occurrence of torsion of the spleen around its pedicle. aim: Report of a new observation and present the diagnostic, treatment and outcome aspects of torsion of ectopic spleen. CASE: We report the case of a 17 years old girl who was admitted for an acute abdomen pain and fever. Clinical examination revealed generalized abdominal defence and an under umbilical mass which was very painful on palpation. Ultrasound and CT scans have visualized the pelvic mass, which measured four inches long axis. The spleen wasn't on its normal seat. The patient was operated urgently. Surgical exploration showed that the mass corresponded to a wandering spleen in pelvic position, necrotic and twisted around its pedicle. A splenectomy was performed with simple sequences. CONCLUSION: torsion of the wandering spleen can progress to total necrosis of the spleen mass. This complication is feared in any ectopic and painful spleen.

[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.

Inguinoscrotal hernia revealing a testicular hemangioma: Report of a neonatal case.

Testicular hemangioma (TH) is a congenital, uncommon and non-malignant testicular tumor. In the following report, we present a child who was brought to our emergency department with swelling of the right scrotum. Investigations were consistent with a TH and an associated hernia. We conducted an inguinal exploration given the hernia accompanying the TH. The pathological tissue findings were suggestive of a TH. In our further research, we found that this was the first neonatal hemangioma in the literature. It's important to know that an inguinal hernia can lead to the non-recognition of certain tumors.

A rare case of giant mesenteric lipoblastoma causing an abdominal mass in an 8-month-old child.

INTRODUCTION AND IMPORTANCE: Giant mesenteric lipoblastoma (LB) is a rare and benign tumor derived from adipocytes. It may imitate malignant tumors, and its diagnosis is challenging before surgery. The diagnosis can be guided by imaging studies but cannot be confirmed. Just a few cases of lipoblastoma originating from the mesentery are reported in the literature. CASE PRESENTATION: We present a case of a rare giant lipoblastoma arising from the mesentery of an 8-month-old boy who consulted our emergency department for an incidentally discovered abdominal mass. CLINICAL DISCUSSION: LB is most common in the first decade of life, with a high incidence in boys. LBs are generally found in the trunk and extremities. Intra-abdominal locations are rare; however, intraperitoneal tumors generally reach larger dimensions. CONCLUSIONS: Tumors that arise in the abdomen are usually larger and may be discovered by physical exam as an abdominal mass and may cause compression symptoms.

Recurrent urinary tract infections led to the diagnosis of cross-renal ectopia: A case report.

INTRODUCTION: Crossed renal ectopy (CRE) is a very rare congenital kidney anomaly. CRE is usually asymptomatic and is most often discovered incidentally, but the patient may sometimes develop various renal manifestations. CASE PRESENTATION: We report a case of a girl who was symptomatic of several episodes of urinary tract infections and whose investigations including ultrasound, uroscan, renal scan and voiding cystourethrogram concluded that she had a crossed left renal ectopy without vesico-ureteral reflux. A regular Follow-up revealed no further symptoms or complications, with partial resolution of the hydronephrosis, and no recurrence of urinary tract infections after hygienic rules. This case didn't require surgical intervention. CLINICAL DISCUSSION: CRE is an infrequently occurring congenital malformation. Uroscan is an excellent tool to describe the full anatomical details of this pathology; and the information provided is crucial for surgeons, nephrologists, and radiologists to aid in the proper handling of this pathology. CONCLUSIONS: CRE is a rare disease that can be diagnosed incidentally. Treatment is only indicated if complications occur or if there is other associated renal disease. Patients require continuous follow-up and need to be examined for potential complications.

Mechanical complications of peritoneal dialysis in Tunisian children.

INTRODUCTION: Peritoneal dialysis (PD) is the method of choice for extra-renal replacement therapy (ERT) for children with end-stage renal disease (ESRD), because of its various advantages. However, it presents different infectious and non-infectious complications, causes of important morbidity and mortality. AIM: To determine the mechanical complications of PD in our center and to identify risk factors of their occurrence. METHODS: We retrospectively collected the records of 99 patients who were treated with PD within the past eleven years in the department of pediatrics of the University Hospital Charles Nicolle of Tunis. Analysis examining possible risque factors were performed using parametric and non-parametric tests. RESULTS: A total of 63 patients had mechanical complications with an incidence of peritoneal dialysis catheter revision of 1 procedure every 38 months. The causes were dominated by catheter migration (27.5%) and obstruction by fibrin or blood clotting (19.8%). A history of peritonitis (p=0.046) and the presence of comorbidity (p=0.008) were the two independent risk factors for catheter revision. Inguinal hernia was noted in six patients. No patient presented with a hydrothorax. Seven patients presented an episode of hemoperitoneum. CONCLUSION: Our results lead us to focus our efforts on preventing peritonitis and controlling morbidity. Prospective studies will enable us to confirm our results.

Congenital giant megaureter prenatally diagnosed: A case report.

Congenital giant megaureter (CGM) is uncommon in pediatic population, defined as congenital localized or total dilatation of the ureter to over 10 times the normal diameter with a normal bladder. Herein, we reported an entirely dilated CGM presented as neonatal bowel obstruction in a newborn baby. Our experience has suggested that CGM should be considered as a differential diagnosis of abdominal distension and occlusive syndrome.

Chest Wall Monophasic Synovial Sarcoma in a 12-Year-Old Boy: A Case Report.

We report a rare case of synovial sarcoma of the chest wall in a child. A 12-year-old male presented to our pediatric surgery department with chief complaint of swelling accompanied by slight pain of the upper right side of the anterior chest wall since approximately 5 months. Firstly, we performed an ultrasound (US)-mass biopsy. Histopathological examination showed fibromatosis. After that, we underwent surgical resection. Pathological examination revealed a monophasic type synovial sarcoma. Immediately post-operation, the patient manifested a loss of the extension of the forearm, hand, and fingers on the right side. Physical therapy was performed with good results. On the basis of our diagnosis, we performed 6 cycles of multi-drug adjuvant chemotherapy and then radiotherapy. After 3 years of surgery, there was no evidence of recurrence. However, careful observation may be required.

[Duodenal duplication revealed by acute pancreatitis. A case report]. / Duplication duodenale revelee par une Pancreatite aigue. A propos d'un cas.

BACKGROUND: Duodenum duplications are uncommon congenital anomalies. Most symptomatic cases are diagnosed in childhood and usually present with obstructive or bleeding symptoms. Acute pancreatitis is rarely attributed to these cysts. AIM: To report a new case of duodenum duplication revealed by acute pancreatitis. CASE REPORT: This 3 year old child presented with an acute pancreatitis. Abdominal ultrasonography and Computer tomography were performed showing a cystic mass depending of the 2nd duodenum. Diagnosis of duodenal duplication is made in laparotomy. A surgical resection of the duplication was performed respecting the papilla. Microscopic examination of the specimen confirmed the duodenal duplication. The patient was asymptomatic after the intervention. CONCLUSION: Duodenum duplications are uncommon congenital anomalies. Acute pancreatitis might be revealing presentation.

Congenital hyperinsulinism: review of 12 Tunisian cases.

BACKGROUND: Congenital hyperinsulinism in infancy (CHI) is a heterogeneous disorder with respect to genetics and response to therapy. Data on CHI are sporadic in North African population. AIM: To characterize the clinical features and outcome of 12 Tunisian patients with CHI. METHODS: data of patients diagnosed with CHI during the period 1989-2007 were retrospectively analyzed. Diagnosis was considered whenever hyperinsulinemia ≥ 10µ UI/ml was concomitant to hypoglycemia < 3mmol/l and/or high insulin to glucose ratio > 0.3 and/or positif glucagon test. Transient causes of hypoglycemia, adrenal and growth hormone deficiency were excluded. RESULTS: There were nine infants diagnosed at a median age of 17 months and three newborns. Permanent hyperammoniemia, found in one patient, guided to leucine-sensitive hyperinsulinism. Seven patients presented with seizures, two with psychomotor delay and one with recurrent malaises. Among 42 assays of plasmatic insulin, when in hypoglycemia, 40% only were ≥ 10µU/ml. Three patients resisted to diazoxide and underwent subtotal pancreatectomy complicated by diabetes mellitus in two cases and persistent hypoglycemia in one patient. Histological examination concluded to diffuse hyperplasia of pancreatic cells. Diazoxide was discontinued in four out the eight responders' patients. Four patients died, seven patients developed variable degrees of mental retardation and five suffered from epilepsy. CONCLUSION: Early onset forms were, as reported in the literature, mostly resistant to medical therapy. The high proportion of neurological sequelae is related to diagnosis delay or to a late surgery. We focus on the importance of a precocious diagnosis and aggressive treatment of hypoglycemia.

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.

[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.

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.

Sternal cleft, a rare congenital anomaly. Report of the first Tunisian case.

BACKGROUND: Sternal cleft is an uncommon visually dramatic congenital anomaly of the chest wall. It is resulting of failure of the two lateral mesodermal sternal bars fusion by the eight weeks of gestation. Superior defects are the commonest forms, usually isolated. AIM: Clinical and surgical aspects of sternal cleft are presented. The advantages of early surgery in the neonatal period are developed. CASE REPORT: We report the first Tunisian case of a superior sternal cleft associated to haemangioma in a newborn boy. Scanning shows non-appearance of manubrium at the upper part of sternum. Sternal bars showing a U-shaped incomplete sternal cleft. Surgical repair consisted of reconstructing a new sternum from sternal bars and resection of haemangioma. The patient had good aesthetic and functional results. CONCLUSION: The appearance of a child with its heart bulging through its chest wall is very disturbing to parents. Early surgery is most easy and most comforting.

[Pancreatic-pleural fistula in children: a rare cause of great abundant pleurisy]. / La fistule pancréatico-pleurale chez l'enfant: étiologie rare d'une pleurésie de grande abondance.

Pancreatic-pleural fistula is a very rare complication of false cysts of the pancreas. Our study aimed to describe this rare pathology affecting children. We emphasized the importance of evoking this diagnosis in the presence of great abundant pleurisy even in the absence of the digestive signs. We here report the case of a 2-year old child with false cyst of the pancreas complicated by pleural fistula detected after great abundant pleurisy with no digestive signs associated.

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.

Sacrococcygeal yolk sac tumor: an uncommon site.

A 30-month-old male infant presented with sacrococcygeal and pre-sacral mass. Ultrasound (US) abdomen revealed a huge pre-sacral mass with irregular margins extending into the pelvis, pushing the rectum antero-laterally. CT scan and MRI confirmed the US findings. Serum alpha fetoprotein level was abnormally elevated. Histopathological examination of surgical-specimen suggested sacrococcygeal yolk sac tumor (YST).