How to treat hepatic pulmonary fusion: case report with review of literature.

Introduction: Hepatic pulmonary fusion (HPF) occurring with right diaphragmatic hernia is rare. Some sporadic reports exist in the literature. It may range from just fibrovascular communication to complete parenchymal fusion which may be complex to divide.Patients and methods: We report a case of a 1-day old newborn male presented with respiratory distress relevant to a right diaphragmatic hernia.Results: The patient was operated after initial stabilization. During surgery, a 10-cm wide posterolateral defect was found. The herniated liver was only partially reducible because of HPF. This rare condition was treated by the division of the fusion and diaphragm repair using a vicryl patch.Conclusion: Through our case and a review of the literature, we will discuss the different alternatives in the treatment of HPF.

Accessory Scrotum.

Introduction: An accessory scrotum is characterized by a characteristic skin appearance in addition to a normal scrotum in the anatomically normal position. Case report: We report an accessory inguinal left hemiscrotum in a 15-day-old male infant. There was a normal scrotum and penis. The testes were descended and normally located within the normal scrotum. Additional scrotal skin was located anterior and lateral to the normal scrotum. Conclusion: The accessory ectopic scrotum is readily observed by physical examination. Cosmetic excision is the treatment of choice.

Correction to: Full title: peripheral venous catheter complications in children: predisposing factors in a multicenter prospective cohort study.

Following publication of the original article [1], one of the authors flagged that the title of the article was submitted (incorrectly) with "Full title:" at the beginning.

Peripheral venous catheter complications in children: predisposing factors in a multicenter prospective cohort study.

BACKGROUND: Peripheral venous catheterization (PVC) is frequently used in children. This procedure is not free from potential complications. Our purpose was to identify the types and incidences of PVC complications in children and their predisposing factors in a developing country. METHODS: We conducted a prospective observational multicenter study in five pediatric and pediatric surgery departments over a period of 2 months. Two hundred fifteen PVC procedures were conducted in 98 children. The times of insertion and removal and the reasons for termination were noted, and the lifespan was calculated. Descriptive data were expressed as percentages, means, standard deviations, medians and interquartile ranges. The Chi2 test or the Fisher test, with hazard ratios and 95% confidence intervals (CI95%), as well as Student's t test or the Mann-Whitney U test were used to compare categorical and quantitative variables, respectively, in groups with and without complications. The Spearman test was used to determine correlations between the lifespan and the quantitative variables. The Kruskal Wallis test was used to test for differences in the median lifespan within 3 or more subgroups of a variable. Linear regression and logistic binary regression were used for multivariate analysis. A p-value <0.05 was considered significant. RESULTS: The mean lifespan was 68.82 ± 35.71 h. A local complication occurred in 111 PIVC (51.9%) cases. The risk factors identified were a small catheter gauge (24-gauge) (p = 0.023), the use of a volume-controlled burette (p = 0.036), a longer duration of intravenous therapy (p < 0.001), a medical diagnosis of respiratory or infectious disease (p = 0.047), the use of antibiotics (p = 0.005), including cefotaxime (p = 0.024) and vancomycin (p = 0.031), and the use of proton pump inhibitors (p = 0.004).The lifespan of the catheters was reduced with the occurrence of a complication (p < 0.001), including the use of 24-gauge catheters (p = 0.001), the use of an electronic pump or syringe(p = 0.036) and a higher rank of the intravenous device in each patient (p = 0.010). CONCLUSIONS: PVC complications were frequent in our pediatric departments and are often associated with misuse of the device. These results could engender awareness among both doctors and nurses regarding the need for rationalization of the use of PVC and better adherence to the recommendations for the use of each drug and each administration method.

Diaphragmatic eventration in children.

BACKGROUND: Diaphragmatic eventration (ED) is a rare anomaly defined by a permanent elevation of a hemidiaphragm without defects. Clinical manifestations are diverse, ranging from asymptomatic to life-threatening respiratory distress. The aim of this study is to report our experience of management of eight children with ED over the past 15 years. METHODS: A retrospective study was conducted involving 8 infants and children with ED managed at the department of pediatric surgery of Tunis Children's Hospital. RESULTS: Five patients (2 males, 3 females) whose ages ranged from 5 month to 7 years (mean, 13 months) were operated on using diaphragmatic plication (3 right-sided and 2 left-sided plications) for symptomatic ED with a dramatic improvement in their respiratory status. Another infant (a 3-month-old boy) was admitted for respiratory distress that required mechanical ventilatory support. He died before operation because of sepsis. Two other asymptomatic patients with incidentally diagnosed ED were followed up for 5 years on average. Chest x-ray suggested eventration in all cases. Ultrasound was found to be a useful modality for diagnosis in 6 cases. There were no cases of associated malformation. CONCLUSION: Diaphragmatic eventration in children is usually congenital but may be acquired. Chest ultrasound is an important imaging modality for diagnosis. Diaphragmatic plication appears to be safe and effective. Surgical intervention is not recommended in asymptomatic patients.

Presternal giant epidermal cyst: 2 cases.

BACKGROUND: Epidermal cysts are a common benign lesion, occurring often above the shoulder and within the face and the scalp. Presternal site and huge volume are quite rare, especially in children. AIM: The authors report on two paediatric cases, discuss etiopathogenesis and diagnostic difficulties. CASES: A 2, 5 and 3-year-old boys were admitted for voluminous subcutaneous tumour of the chest. The lesions had appeared since neonatal period with rapid growth after a minimal trauma for the last year. The physical examination found a great presternal cystic mass of 70 mm in diameter which is painless round and mobile. Ultrasonography and magnetic resonance imaging, showed a subcutaneous cystic mass with no mediastinal involvement or other localization in both. Complete surgical excision including the overlying skin with direct cutaneous closure was performed in both cases. The histological examinations confirmed the diagnosis of epidermal cyst. Postoperative courses were uneventful with no recurrence during respectively 18 months and 5 years follow-up periods. CONCLUSIONS: Giant presternal epidermal cyst is uncommon and can raise diagnostic problems. Imaging investigations facilitate accurate diagnosis. Because of malignant degeneration, surgical excision should be mandatory.

Xanthogranulomatous pyelonephritis in childhood: diagnosis difficulties and success of conservative treatment.

BACKGROUND: Xanthogranulomatous pyelonephritis is an uncommon form of chronic pyelonephritis rarely seen in children. Preoperative diagnosis is difficult and management is still obscure. AIM: The authors report on a focal pseudotumoral case and discuss therapeutic modalities. CASE: A 2-year-old boy was admitted for a 2-weeks history of fever and right flank pain. Physical examination revealed a right lumbar mass. White blood cell count was 12.800/mm3 and C reactive protein elevated (50 mg/l). The urine culture was negative. Ultrasonography and computerized tomography demonstrated a focal multilocular cystic mass of the lower polar of right kidney, measuring 80 millimeters, with peripheral enhancement and perirenal involvement. At surgical exploration, a huge abscess of the lower polar of the kidney with dense adhesions to adjacent structures were noted. Excision of the collection and local drainage were performed. Pathologic examination of biopsy specimen confirmed the diagnosis of xanthogranulomatous pyelonephritis. Intraoperative culture was positive for Escherichia coli. Successful treatment of the lesion was achieved with adjunctive antibiotic therapy. The postoperative course was uneventful over a follow-up period of 2 years. CONCLUSION: Xanthogranulomatous pyelonephritis should be considered in the differential diagnosis of a renal mass. A first-line conservative treatment must be strongly recommended in pediatric focal cases.

[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.

[Interventional endoscopy in the gastrointestinal tract in children]. / Activité endoscopique digestive interventionnelle chez l'enfant.

BACKGROUND: Interventional endoscopic procedures involving the gastrointestinal (GI) tract in children are very common. Over the last several years the number of procedures in this area has steadily increased. AIM: To study indications and results of GI interventional endoscopies performed in a Pediatric GI unit. METHODS: Retrospective study over a period of 10 years (1999-2008). Data were collected from endoscopic files. All procedures were performed endoscopically under general anesthesia. RESULTS: 185 interventional endoscopic procedures were done during this period in 96 patients (58 boys) median age: 4.9±4.7 years (1 month-13 years). Indications were as follows: esophageal stenosis (n=46; 47.9%), rectal polyp resection (n=21; 19.8%), esophageal varices ligation (n=19; 19.7%), foreign body extractions (n=8; 8.3%) and percutaneous endoscopic gastrostomy (n=2; 2.1%). One hundred and thirteen esophageal dilations (61.1% of procedures) were performed in 46 patients: caustic stenosis (n=22; 22.9%), peptic stenosis (n=13; 13.5%) and anastomotic stenosis (n=11; 11.4%). CONCLUSION: The main indication of interventional GI endoscopy in this series was oesophageal stenosis followed by rectal polyp resection, esophageal varices ligation and foreign body extractions.

Idiopathic congenital anomalous bands: About ten cases with systematic review of the literature.

BACKGROUND AND OBJECTIVES PURPOSE: There are various causes and associated conditions in anomalous bands. Idiopathic congenital anomalous bands are extremely rare. The use of the term "congenital bands" may, in reality, not describe the same situation. The study aims to report our series of idiopathic anomalous bands and proposes an accurate and consistent classification of anomalous bands in order to clarify the origin of each band. METHODS: This study, conducted from January 2005 to January 2018, included all patients admitted to the emergency departments with a clinical diagnosis of intestinal obstruction resulting from bands that have no identifiable embryological or acquired basis called "idiopathic". Recorded operative findings included the site of obstruction and the operative procedure to relieve it. RESULTS: The sample consists of seven boys and three girls with age range from one day to 9 years with symptoms and signs indicative of intestinal obstruction. Surgical intervention was performed, and intraoperative findings revealed a thick and vascularized idiopathic band, in different locations, which was responsible for intestinal obstruction in all patients. Clinical courses were uneventful in eight cases. CONCLUSION: Idiopathic anomalous congenital bands causing intestinal obstruction are not frequently encountered in surgical practice and these bands are often difficult to classify and define. We believe that our new classification is a practical communication tool for medical professionals to summarize and clarify the different types of anomalous bands.

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.

Fabricated or induced illness in twins associated with insertion of trocar needles into their bodies.

Fabricated and/or induced illness (previously known as Munchausen syndrome by proxy) is a form of child abuse in which the perpetrator induces, exaggerates or fabricates illness in his/her child. Two-month-old twins were referred to the paediatric surgery centre with trocar needles lodged in different organs. A radiograph undertaken in one of them because of acute respiratory distress demonstrated needles in the heart and diaphragm which were removed surgically. The second twin, referred one week after his brother, had a needle deep in his liver and it was decided not to operate owing to the possibility of haemorrhage but, sadly, he died unexpectedly and the autopsy concluded that it was owing to sudden infant death syndrome. Because of a number of admissions to different units in the same hospital, there was a delay in the eventual diagnosis. Recurrent admissions to different hospital units should raise the suspicion of fabricated and/or induced illness.

[Clinical characteristics and outcome of cystic fibrosis: report of 16 cases]. / Particularités cliniques et évolutives de la mucoviscidose. A propos de 16 observations.

AIM: The aim of this report is to determine clinical characteristics and outcome of Cystic Fibrosis (CF). METHODS: Cases of CF managed at Infantile Medicine A Department in Children's Hospital of Tunis during 13 years (1994-2006) were reviewed. RESULTS: 16 children had CF. They were 8 males and 8 females. 13 patients were consanguineous and four had similar familial cases. The mean age at diagnosis was 19 months (10 days, 13 years). 3/4 of patients were symptomatic within the first trimester of life. Revealing symptoms were: obstructive bronchopathy associated to chronic diarrhea (n=6), edema-anemia-hypotrophy-hypoproteinemia syndrome (n=3), meconium ileus (n=4), bronchiectasis (n=2) and chronic diarrhea (n=1). The diagnosis was confirmed by sweat test and genotypic data. The F508 del was the most frequent mutation (54%). Clinical outcome was characterized by the occurrence of respiratory and nutritional complications: acute respiratory failure (n=6), chronic respiratory failure (n=3), chronic pseudomonas aeruginosa infection (n=6) at a medium age of 3.8 years, recurrent haemoptysis (n=2), pleural effusion (n=2), a malnutrition (n =10) and diabetes associated to puberty delay in one patient. Seven patients died at mean age of 4.4 years (6 months, 17.3 years). Among surviving patients, six had no compromised nutritional status or lung function. Prenatal diagnosis was performed in three families. CONCLUSION: CF is characterized by earliest onset and severity of symptoms. Therapeutic insufficiency is the main cause of precocious complications and poor prognosis in our series.

[Cholestasis in infants: a study of the Children's Hospital of Tunisia]. / Les cholestases du nouveau né et du nourrisson: experience de l'Hôpital d'Enfants de Tunis.

BACKGROUND: Cholestasis in infant constitutes an heterogeneous group of disease; diagnosis and management are often difficult. THE AIM of the study is to describe clinical, paraclinical characteristics and outcome of infants hospitalized for cholestasis in children's Hospital of Tunis. METHODS: A retrospective study of 94 infants with cholestasis was conducted. Patients were hospitalized in four departments of paediatrics of our hospital between January 1995 and December 2005. Cholestasis complicating severe sepsis and visceral leishmaniasis were excluded. RESULTS: Incidence of cholestasis was 8.5 cases/year which represented 0.72% of the hospitalizations. Sex ratio was 1.08 and mean age at diagnosis was 105 days (extremes: 1 day- 24 months). Biliary atresia was the most common cause of extra hepatic cholestasis (13.8%). Normal A GT cholestasis (11.7 %), benign neonatal cholestasis (11.7%) and bile duct hypoplasia (9.5%) represented the most common aetiologies of intra hepatic cholestasis. Aetiology remained unknown in 12.7% of cases. Only three infants with biliary atresia had Kasaï operation. After a mean follow-up of 6 years, 18% of patients had portal hypertension, 14.8% had hepatic failure and mortality rate was 14.8%. CONCLUSION: Cholestasis of unknown aetiologies are frequent in our hospital. Poor prognosis, in our study, is due to delay to diagnosis and difficulties in medical and surgical management.

Intestinal duplication revealed by posterior reversible encephalopathy syndrome.

We report a unique case of intestinal duplication detected on posterior reversible encephalopathy syndrome (PRES) in a 13-year-old girl. She was admitted to the pediatric Emergency Department because of generalized seizures. Radiological assessment revealed a large, well-defined, thick-walled cystic lesion in the mid abdomen, suggestive of duplication cyst associated to a PRES. Exploration confirmed the diagnosis of ileal duplication cyst, and the mass was resected. The postoperative course was uneventful. Both hypertension and neurological dysfunction resolved after the mass resection. A followup brain magnetic resonance imaging was performed 9 months later and showed complete resolution of the cerebellar changes. Although extrinsic compression of the retroperitoneal structures has not been reported in the literature as a complication of duplication cyst, we strongly believe that this is the most logical and plausible hypothesis that would explain the pathogenesis of PRES in our patient.