Jamila Chahed

Lipoblastoma in childhood: About 10 cases

Background: Lipoblastoma is a rare benign mesenchymal tumour of embryonal fat that occurs almost exclusively in infants and children. This determined the epidemiological, clinical and therapeutic aspect of this disease. Materials and Methods: A total of 10 cases of pathologically proven lipoblastoma from 2003 to 2012 were reviewed. Results: There were six boys and four girls ranging in age from 7 months to 9 years. A soft-tissue mass was the main complain in nine patients. The various locations of the mass were mediastinal, thigh, buttock, inguino-scrotal, the greater omentum and the Latissimus dorsi. Lesions measured 5-15 cm. complete excision was done. The median time of follow-up was 42 months (ranges between 18 and 84 months). There were no recurrences. Conclusion: It is important to consider lipoblastoma in the diagnosis of a rapidly enlarging fatty mass in children. Complete resection is the only definitive treatment and should not be delayed when impingement on surrounding structures is imminent. There is a tendency for these lesions to recur despite presumed complete excision. Therefore, follow-up for a minimum of 5 years is recommended.

Burns injury in children: is antibiotic prophylaxis recommended?

Background: Wound infection is the most frequent complication in burn patients. There is a lack of guidelines on the use of systemic antibiotics in children to prevent this complication. Patients and Methods: A prospective study is carried out on 80 patients to evaluate the role of antibiotic prophylaxis in the control of infections. Results: The mean age was 34 months (9 months to 8 years). There was a male predominance with sex ratio of 1.66. The mean burn surface size burn was 26.5% with total burn surface area ranging from 5% to 33%, respectively. According to American Burn Association 37% (30/80) were severe burns with second and third degree burns >10% of the total surface body area in children aged <10 years old. Scalds represented 76.2% (61/80) of the burns. Burns by hot oil were 11 cases (13.7%), while 8 cases (10%) were flame burns. The random distribution of the groups was as follow: Group A (amoxicilline + clavulanic acid) = 25 cases, Group B (oxacilline) = 20 cases and Group C (no antibiotics) = 35 cases. Total infection rate was 20% (16/80), distributed as follow: 8 cases (50%) in Group C, 5 cases (31.2%) in Group A and 3 cases in Group B (18.7%). Infection rate in each individual group was: 22.9% (8 cases/35) in Group C, 20% (5 cases/25) in Group A and 15% (3 cases/20) in Group B (P = 0.7). They were distributed as follow: Septicaemia 12 cases/16 (75%), wound infection 4 cases/16 (25%). Bacteria isolated were with a decreasing order: Staphylococcus aureus (36.3%), Pseudomonas (27.2%), Escherichia coli (18.1%), Klebsiella (9%) and Enterobacteria (9%). There is a tendency to a delayed cicatrisation (P = 0.07) in case of hot oil burns (65.18 ± 120 days) than by flame (54.33 ± 19.8 days) than by hot water (29.55 ± 26.2 days). Otherwise no toxic shock syndrome was recorded in this study. Conclusion: It is concluded that adequate and careful nursing of burn wounds seems to be sufficient to prevent complications and to obtain cicatrisation. Antibiotics are indicated only to treat confirmed infections.

Congenital pancreatic cyst with Ivemark II syndrome: a rare case

An infant with congenital pancreatic cyst with Ivemark II syndrome is reported because it is a rare association. The infant had associated situs inversus, asplenia, and complex congenital heart disease. The pancreatic cyst was successfully managed by cystoduodenostomy because of connection to the biliary tract. The infant succumbed as a result of heart failure at age 2 months. Prognosis depends on the presence of life-threatening malformations.

Urinary lithiasis and urinary tract malformations in children: A retrospective study of 34 cases

BACKGROUND Although the association of urinary lithiasis and urinary tract malformation is not rare, their management poses challenges. The aim of this study was to evaluate the relationship between urolithiasis and malformations of the urinary system. There were 34 patients (19 males and 15 females) with a mean age of 4.8 years (range, 2 months to 14 years). All patients had urinary lithiasis with a urinary tract malformation. Abdominal pain was the most frequent clinical symptom (38%). Urinary infection was found in 7 patients (21%) and macroscopic haematuria was present in 10 patients (29%). The most frequent urinary tract malformations were megaureter (8 cases), uretero-pelvic junction obstruction (7 cases) and vesico-ureteric reflux (8 cases), but its malformative origin could not be confirmed. Treatment consisted of lithiasis extraction in 32 cases associated with specific treatment of the uropathy in 27 cases. Postoperative outcome was uneventful in all cases. In fact, urinary lithiasis and urinary tract malformation association is not rare. Indeed, 9-34% of urinary lithiasis are noted to be associated with urinary tract malformation. Positive diagnosis relies specifically on kidney ultrasound, intravenous urography, and urethrocystography. Treatment depends on the type of urinary tract malformation, localisation and size of the urinary lithiasis. CONCLUSION In conclusion, urinary lithiasis and urinary tract malformation association is a frequent eventuality. Surgical intervention is the usual mode of treatment.

Video-assisted surgery in the management of hydatid renal cyst in children

Renal hydatid disease is rare in children. Open surgery is the traditional method of treatment, but minimally invasive techniques are being increasingly used. Herein, we report our experience with laparoscopic management of renal hydatid cyst in four children via a transperitoneal approach in three cases and a retroperitoneoscopy in one. We conclude that transperitoneal laparoscopy can be offered for the management of hydatid renal cyst associated with other intraperitoneal localizations, whereas the retroperitoneoscopy is limited for the treatment of isolated hydatid renal cysts.

Familial Near-Total Intestinal Aganglionosis

Near total aganglionosis represents the most extreme and rare form of Hirschsprungs disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.

Can Intestinal Intussusception Complicate Visceral Leishmaniasis

To the Editor: A 3-y-old boy was hospitalized for an occlusive syndrome. On physical examination, there was apyrexia, altered general health, pallor with severe dehydration, hypotension and edema of the lower limbs. Abdomen was distended with an enormous splenomegaly. There was pancytopenia, aregenerative anemia with macrocytosis and coagulation disorders (low prothrombin time, hypofibrinogenemia). A plain abdominal radiograph showed air fluid levels with absence of colonic intestine (Fig. 1). Abdominal exploration via laparotomy showed an ileoileal intussusception with a necrotic zone. The patient had intussusception reduction with resection of the necrotic zone. Furthermore, we noticed the presence of purpuric and petechial lesions on the intestinal wall, mesenteric lymph nodes and a massive splenomegaly. The post-operative course was characterised by persistence of the splenomegaly (Fig. 2), edema, pancytopenia, coagulation disorders in spite of a normal hemodynamic state and disappearance of digestive symptoms. Thus an associated pathology to acute intestinal intussusception (AII) such as disseminated intravascular coagulation (DIC), hemopathy or a hypersplenism was suspected. Further explorations showed a hypoalbuminemia with hypergammaglobulinemia and hypo alpha 2 globulinemia. The association of all these disorders in a child who lives in an endemic region of leishmaniasis, evocated visceral leishmaniasis (VL) diagnosis. The diagnosis was confirmed by positive leishmaniasis serology and myelogram. Treatment consisted of meglumine anti-moniate (Glucantime). The follow up was favorable. Acute intestinal intussusception is a common cause of mechanical bowel obstruction among children. The etiology is identified in 2–12 % of the cases and 20 % of the cases in children aged over 2 y [1]. There are two types of acute intestinal intussusception: AII idiopathic represents 90–95 % of intussusceptions of the children and the AII secondary to local injury of the

Management of digestive lesions associated to congenital epidermolysis bullosa

Background: Congenital epidermolysis bullosa (CEB) is a rare genodermatosis. The digestive system is very frequently associated with skin manifestations. Pyloric atresia (PA) and oesophageal stenosis (OS) are considered the most serious digestive lesions to occur.The aim of this work is to study the management and the outcome of digestive lesions associated to CEB in four children and to compare our results to the literature. Patients and Methods: A retrospective study of four observations: Two cases of PA and two cases of OS associated to CEB managed in the Paediatric Surgery Department of Fattouma Bourguiba Teaching Hospital in Monastir, Tunisia. Results: Four patients, two of them are 11 and 8 years old, diagnosed as having a dystrophic epidermolysis bullosa since the neonatal period. They were admitted for the investigation of progressive dysphagia. Oesophageal stenosis was confirmed by an upper contrast study. Pneumatic dilation was the advocated therapeutic method for both patients with afavourable outcome. The two other patients are newborns, diagnosed to have a CEB because of association of PA with bullous skin lesions with erosive scars. Both patients had a complete diaphragm excision with pyloroplasty. They died at the age of 4 and 3 months of severe diarrhoea resistant to medical treatment. Conclusion: Digestive lesions associated to CEB represent an aggravating factor of a serious disease. OS complicating CEB is severe with difficult management. Pneumatic dilatation is the gold standard treatment method. However, the mortality rate in PA with CEB is high. Prenatal diagnosis of PA is possible, and it can help avoiding lethal forms.

SFCP P-077 - Traitement laparoscopique du kyste hydatique du rein chez l’enfant

Objectifs le kyste hydatique renal est rare chez les enfants. La chirurgie a ciel ouvert etait la voie d’abord chirurgical traditionnelle. Actuellement, les techniques mini-invasives sont de plus en plus utilisees. Materiels et methodes les auteurs rapportent leur experience de traitement laparoscopique du kyste hydatique du rein chez cinq enfants, colliges entre 2007et 2013. Resultats L’approche etait transperitoneale dans quatre cas et retroperitoneale dans un cas. L’âge des patients a varie de 4 a 14ans. Le kyste etait gauche dans 4 cas. Il etait isole dans deux cas et associe a des localisations hepatique ou splenique dans 3 cas. Les suites eraient simples dans tous les cas. Conclusion le traitement laparoscopique du kyste hydatique du rein est une technique simple et acceptable pour l’enfant. La voie transperitoneale peut etre pratiquee en cas de kyste hydatique renal associe a d’autres localisations intra-peritoneales, alors que la voie retroperitoneale peut etre utilisee pour le traitement des kystes hydatiques renaux isoles.

SFCP P-014 - Problèmes diagnostique et thérapeutique des malformations adénomatoides kystiques des poumons. Expérience personnelle.

Objectif discuter certains problemes diagnostique et therapeutique des malformations adenomatoides kystiques des poumons (MAKP) et prevenir une exerese pulmonaire inutile. Le diagnostic differentiel se pose avec d’autres malformations broncho-pulmoaires ainsi que certaines affections aqcuises. Patients et Methode Etude retrospective de 49 enfants operes pour suspicion de MAKP dans notre service depuis 1996. Analyse d’une experience personnelle de 13 cas d’erreurs diagnostique et therapeutique. Resultats Le diagnostic pre-operatoire a ete etabli sur la radiologie. 43 enfants ont eu une lobectomie. Le diagnostic de MAKP a ete confirme par l’histologie dans 38 cas. Ailleurs, l’examen histologique a conclu a: emphseme interstitial (1cas), infarctus pulmonaire ante-natalet post-natal (2cas), hemangiomatose pulmoanire (1cas), lesion hybride (1cas), abces du poumon, lesions non classees (7cas) et dilatation des bronches (1cas). Conclusion L’attitude diagnostique et therapeutique des MAKP repose sur une collaboration multidisciplinaire. Le diagnostic evoque sur la clinique et la radiologie, necessite une confirmation histologique. Certains problemes diagnostiques necessite une confrontation avec des anatomopathologistes avertis pour eviter une exerse pulmonaire inutile.