A New Operative Approach for Long-Gap Esophageal Atresia.

Surgical management of long-gap esophageal atresia (LGEA) remains challenging. Yet, there is a consensus among pediatric surgeons to preserve native esophagus. We used a new surgical technique to successfully manage three children diagnosed with LGEA. This technique consists of a combined thoracic and cervical approach to the EA repair using the patient's native esophagus. All patients initially had had gastrostomy and continuous upper pouch suction while awaiting surgery. This new technique has the potential to become the choice method in LGEA management.

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 a favourable 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.

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.

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.

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.

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.

Contribution of laparoscopy in the abdominal tuberculosis diagnosis: retrospective study of about 11 cases.

OBJECTIVE: Abdominal tuberculosis is one of the most frequent extra-pulmonary localizations. Its diagnosis is difficult and may lead to a delayed prescription of specific treatment. This study is aimed at stressing the role of laparoscopy associated with a biopsy in the diagnostic confirmation of abdominal tuberculosis particularly in doubtful cases. METHODS: The diagnostic features of 11 cases hospitalized for abdominal tuberculosis in the Paediatric Surgery Department of Fattouma Bourguiba Hospital in Monastir for a 6-year period (2001-2006), were evaluated retrospectively. The diagnosis of abdominal tuberculosis was substantiated histopathologically by laparoscopy in all cases. The epidemiological and clinical characteristics along with the laboratory, radiological and histological data were studied. RESULTS: Eleven cases of abdominal tuberculosis with a mean age of 5.6 years were diagnosed. It was peritoneal tuberculosis in all cases and associated with intestinal localization in one case. A conversion to laparotomy was practiced in three patients: appendicular plastron in one case, pseudo-tumor aspect of an intestinal loop in another case and because of their pathological aspect appendicectomy and caecum biopsy in the third. The diagnosis was confirmed histologically by biopsies in nine cases and on excision pieces in the other two cases. All patients had an uneventful course with an antituberculosis treatment. CONCLUSION: Abdominal tuberculosis is still frequent in Tunisia. Because of its non-specific clinical presentation and the limited means of investigation, a laparoscopy with biopsy should be practiced as first line diagnostic tool in case of doubtful abdominal tuberculosis. The earlier the diagnosis is established and an adapted antituberculosis treatment is started, the better the prognosis is.