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.

[Biliary atresia and polysplenia syndrome]. / Atrésie des voise biliaires et syndrome de polysplénie.

Polysplenia syndrome is a rare malformation characterized by the association of multiple rates and other congenital anomalies dominated by cardiac, vascular, intestinal and bile malformations. We report the observation of a patient operated in the neonatal period (3 days) for an upper intestinal obstruction with situs inversus. Surgical exploration noted the presence of multiple rates, a preduodenal vein, a biliary atresia and a duodenal atresia. The surgical procedures performed were a latero-lateral duodeno-duodenostomy and hepatoportoenterostomy of KASAI with simple immediate and delayed outcomes. The follow up was of 23 years. We recall the epidemiological characteristics of this malformative association and we discuss the role played by the prognosis of polysplenia syndrome in the evolution of biliary atresia. The diagnosis and treatment of biliary atresia are always urgent to increase the chances of success of the Kasai, and the chances of prolonged survival with native liver. However, almost all long-term survivors (even anicteric) have biliary cirrhosis, which requires lifelong follow up.

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.

Gastric perforation in the newborn.

BACKGROUND: Gastric perforation in neonates is an uncommon condition. It could be spontaneous but a contributing cause should be sought. AIM: To review our experience of treating 8 neonates with gastric perforation over the past 20 years. METHODS: We reviewed the records of all newborns admitted to our hospital between 1990 and 2010 with regard to gender, age at admission, contributing factors, associated anomalies, site of perforation, type of operation, and outcome. RESULTS: Of the eight neonates, five were female and three male. The average weight was 2130 g. Four babies were premature. Three infants were ventilated for respiratory difficulty. Five patients had associated anomalies. Perforation occurred in the lesser curvature in 4, at the greater curvature in 3, and at the anterior antrum surface in 1. All patients were treated with gastrorrhaphy. Four neonates required additional gastrostomy. Mortality was 75% (6 infants). CONCLUSION: Active perinatal management, early treatment of primary pathologies, and protection of the stomach against distension in neonates at risk are essential in the management of neonatal gastric perforation.

Pyloric atresia: A report of ten patients.

Pyloric atresia (PA) is uncommon. It occurs in 1:100000 live births. Neonates usually present soon after birth with copious non-bilious vomiting. The treatment is surgical and its prognosis is poor, especially, when it is associated with epidermolysis bullosa (EB). The aim of this study was to evaluate the clinical presentation, diagnosis, operative management, post-operative courses, and outcome in infant with PA, based in our cases and literature review. Charts of 10 patients who underwent surgery for PA in the department of paediatric surgery in a Teaching Hospital in Tunisia (Monastir) between 1990 and 2012 were reviewed. Data were analysed for demographic, clinical, therapeutic, and prognostic characteristics. The average of age at presentation was 2 days and there were six males and four females. The main presenting symptoms were non-bilious vomiting in 90% of cases. Abdominal X-ray showed gastric dilatation with an absence of gas in the rest of the intestinal tract in 90%, and a pneumoperitoneum in one. The surgical approach was laparotomy in all cases. Gastric perforation was observed in one patient and was completely repaired. The distribution of the anatomic variations was type A in nine cases and type B in one. Five patients underwent excision of the diaphragm and Heineke-Mikulicz pyloroplasty and gastroduodenostomy in the other five cases. Identified associated anomalies were Down's syndrome in one and EB in 2 (20%), one family has three affected sibling. Post-operative mortality rate was 70%. No standard surgical approach can be adopted a better management of PA or the associated anomalies depends on an early diagnosis and the availability of neonatal intensive care unit.

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.

Recurrent intussusception in children and infants.

BACKGROUND: Recurrent intussusceptions in child and infants are problematic and there are controversies about its management. The aim of this study is to determine the details of the clinical diagnosis of recurrent intussusception and to determine the aetiology of recurrent intussusceptions. PATIENTS AND METHODS: It's a retrospective study of 28 cases of recurrent intussusception treated in the paediatric surgery department of Monastir (Tunisia) between January 1998 and December 2011. RESULTS: During the study period, 505 patients were treated for 544 episodes of intussusception; there were 39 episodes of recurrent intussusceptions in 28 patients; the rate of patients with recurrence was 5.5%. With comparison to the initial episode, clinical features were similar to the recurrent episode, except bloody stool that was absent in the recurrent group (P = 0,016). Only one patient had a pathologic local point. CONCLUSION: In recurrent intussusception, patients are less symptomatic and consult quickly. Systematic surgical exploration is not needed as recurrent intussusceptions are easily reduced by air or hydrostatic enema and are not associated with a high rate of pathologic leading points.

Soave transanal one-stage endorectal pull-through in the treatment of Hirschsprung's disease of the child above two-year-old: a report of 20 cases.

BACKGROUND: The definitive treatment of Hirschsprung's disease is the removal of the aganglionic bowel by a pull-through surgery. In most cases, this surgery is performed in infancy or in the neonatal period as presentation in older children and adulthood is uncommon. MATERIALS AND METHODS: It is a retrospective study of 20 patients above two-year-old who underwent a transanal Soave one-stage endorectal pull-through procedure for Hirschsprung's disease between January 2002 and December 2010. RESULTS: Twenty patients were recruited in this study. Fourteen were males and six were females. Patient ages ranged from 2 to 14 years (median age: five years and three months). All patients presented with persistent constipation and abdominal distension. Two of them had an intestinal obstruction that required colostomy. Ten patients (50%) had a recto-sigmoid Hirschsprung's disease. All patients were operated on using a Soave one-stage endorectal pull-through procedure. The laparoscopy was necessary during the pull-through in three cases. The average duration of the intervention was 240 minutes. That represents almost the double of the duration of the same procedure in newborns and infants in our department (130 minutes). Early postoperative complications included one case of anastomosis leakage and one case of intussusception. Late postoperative complications were perineum irritation in five cases (25%), anal stenosis in four cases (20%) and enterocolitis in one case (5%). None of our patients developed fecal incontinence. Soiling was reported in four cases (20%). There was no death. CONCLUSION: Soave transanal one-stage endorectal pull-through is safely feasible in children of more than two years of age. Laparoscopy may be necessary whenever there are difficulties in the pull-through.

Perinatally discovered complete tubular colonic duplication associated with anal atresia.

Complete tubular colonic duplication (CTCD) is exceedingly rare. The association of CTCD with an anorectal malformation is unusual. This malformation may be found unexpectedly at laparotomy. We present 3 cases of surgically proven neonate CTCD discovered at laparotomy for anal atresia. We reviewed the mode of clinical presentation, the imaging, and laparotomy findings. Our series illustrates that this rare disease presents perinatally in association with anal atresia, with or without other associated anomalies.

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.

Management of multiple echinococcosis in childhood with albendazole and surgery.

BACKGROUND/PURPOSE: Multiple echinococcosis (ME) is a severe disease in childhood inaccessible to an initial radical surgical treatment. The aim of this study was to evaluate the efficacy of Albendazole in ME and to discuss the role of surgery in this pathology. METHODS: Eleven patients were included in a prospective study between 1996 and 2004. ME was defined by the presence of 10 or more cysts in the same organ. Albendazole was given as 10 mg/kg daily continuously. Treatment outcome was defined as cure, improvement, stabilization or deterioration. Surgery was discussed after 1 year of treatment. RESULTS: Our patients totalized 296 cysts located essentially on the liver (178 cysts) and the lungs (78 cysts). With exclusive Albendazole therapy, 57.7% pulmonary cysts and 96% peritoneal cysts were considered as cured. This rate was only 31.5% in hepatic localization. After surgical therapy, 67.4% of hepatic cysts were cured. No productive biliary fistula was observed. Two patients were operated laparoscpically. The total treatment duration ranged between 1 and 5 years. Parasitologic examination of operated cysts showed that 30% of them were viable even after 3 years of treatment. CONCLUSION: Albendazole has proven a strong efficacy in pulmonary and peritoneal localizations. A complementary surgical treatment is often necessary in hepatic localizations and it is facilitated with previous Albenazole therapy. Combination of ABZ and surgery seems to have encouraging results and must be applied for those patients. The high rate of viable cysts after medical therapy is problematic and must incite to develop new antihelminthic agents.

Congenital bilobar emphysema.

Congenital bilobar emphysema is reported to be extremely rare. We describe 3 cases, and we review the diagnosis problems and treatment methods of this childhood respiratory tract pathologic condition.

Transverse testicular ectopia: a three-case report and review of the literature.

OBJECTIVES: To describe the embryologic, clinical, and therapeutic features of transverse testicular ectopia (TTE) and to review the literature on similar cases. METHODS: Three cases of TTE were discovered accidentally during surgery for inguinal hernia and undescended testes; a persistent Müllerian duct syndrome (PMDS) was associated in 1 case. We also performed a literature search for other reports of TTE. RESULTS: A conservative surgical approach was chosen: transseptal orchiopexy was preferred in 2 cases and the Müllerian remnant was preserved in the first case. CONCLUSIONS: TTE is a rare anomaly that usually presents as a unilateral cryptorchidism and a contralateral descendent testis with an associated hernia. In suspected cases, laparoscopy and ultrasonographic evaluation may be helpful in diagnosing this condition before surgery. Surgery, transseptal orchiopexy, is highly recommended to manage TTE especially when vasa deferentia are fused. In case of PMDS, the authors preferred preserving Müllerian remnants because of the risk of vas deferens and testicular blood supply damage. Recently, laparoscopy is useful for both diagnosing and management of TTE and associated anomalies.

Ovarian inflammatory myofibroblastic tumor in children.

Inflammatory myofibroblastic tumors or inflammatory pseudotumors are uncommon solid tumors that are highly vascularized, have an intermediate prognosis, and are associated with local recurrence, and rare metastasis. Inflammatory myofibroblastic tumors (IMT) most often are seen in the lung of young adults, but there have been reports of these tumors occurring in children, in various sites. The authors report a case of an ovarian IMT arising in a six-year-old girl (third case reported in the literature), treated by a right adnexectomy with uneventful postoperative course, and discuss recent reports.

Laparoscopic treatment of hydatid cyst of the liver in children. A report on 34 cases.

PURPOSE: This study evaluated the safety and efficiency of laparoscopically treated liver cysts in children. METHODS: From September 2001 to July 2004, 34 patients underwent laparoscopic treatment of hydatid cysts of the liver. All patients had chest x-ray, abdominal sonography, and hydatid serology. The different stages of the procedure were the same as in open surgery: puncture, aspiration, injection of scolicidal agent, reaspiration, removal of proligerous membrane, and resection of the dome. RESULTS: The patients' mean average age was 7 years and 7 months (range, 3-14 years). The number of cysts ranged from 1 to 10 with a diameter of 40 to 150 mm (mean diameter, 65.5 mm). One case had a mesenteric associated hydatid cyst, another splenic hydatid cyst. The average length of hospital stay was 5 days (range, 4-14 days). No per- or postoperative complications were reported. At 12 to 45 months follow-up, no recurrence has been reported. CONCLUSION: Laparoscopy represents an excellent approach for the treatment of hydatid cyst of the liver in children.