Management of trichobezoar: About 6 cases.

BACKGROUND: Trichobezoar is an uncommon clinical entity in which ingested hair mass accumulates within the digestive tract. It is generally observed in children and young females with psychological disorders. It can either be found as an isolated mass in the stomach or may extend into the intestine. Untreated cases may lead to grave complications. MATERIAL AND METHODS: We retrospectively analyzed the clinical data of six patients treated for trichobezoar in Monastir pediatric surgery department during 16-year-period between 2004 and 2019. Imaging (abdominal computed tomography and upper gastroduodenal opacification) and gastroduodenal endoscopy were tools of diagnosis. RESULTS: Our study involved 6 girls aged 4 to 12. Symptoms were epigastric pain associated with vomiting of recently ingested food in 3 cases and weight loss in one case. Physical examination found a hard epigastric mass in all cases. The trichobezoar was confined to the stomach in 4 cases. An extension into the jejunum was observed in 2 cases. Surgery was indicated in all patients. In two cases, the attempt of endoscopic extraction failed and patients were then operated on. All patients had gastrotomy to extract the whole bezoar even those with jejunal extension. Psychiatric follow-up was indicated in all cases. The six girls have evolved well and did not present any recurrence. CONCLUSION: open surgery still plays a crucial role in Trichobezoard management . After successful treatment, psychiatric consultation is imperative to prevent reccurrence and improve long term prognosis.

Intussusception Caused by Heterotopic Pancreas: A Tunisian Case Series of 5 Pediatric Patients.

Heterotopic pancreas (HP) is a rare congenital developmental anomaly of the gastro-intestinal tract, defined as the presence of pancreatic tissue found in ectopic sites. Intussusception caused by isolated HP is extremely rare. Pediatric reports concerning this pathology are case reports. Here, we report cases of secondary intussusception, in which conservative treatment failed and surgery was performed. The aim of this review is to study the epidemiologic and clinical aspects of HP in pediatric patients from our institution. We retrospectively collected patients who were treated in the pediatric surgery department for intussusception caused by HP, from January 1986 to November 2018. We investigated five patients, three boys and two girls, aged 5 months to 2 years. The diagnosis was made incidentally during the operation. HP was found in the jejunum in three cases and in the ileum in two cases. HP was removed. The postoperative course was uneventful. Although rare, HP should be included in the differential diagnosis of gastrointestinal diseases, causing secondary bowel intussusception.

[Ureterocele associated with simplex ureter in children: clinical and therapeutic features]. / Urétérocèle sur uretère simplex chez l'enfant: aspects cliniques et thérapeutiques.

Ureterocele is a pseudo-cyst dilation of the terminal submucosal ureter. It is a rare malformative uropathy, in particular associated with simplex ureter. We conducted a retrospective study over a period of 10 years. Twelve medical records of patients whose data were collected at the Department of Paediatric Surgery of Monastir between 2006 and 2016 were examined. The average age of patients was 2.7 years (ranging from 7 days to 11 years) with a sex ratio of 1. Patients´ clinical status was dominated by fever due to upper urinary tract infection. Diagnosis was mainly based on renal bladder ultrasound, intravenous urography (IVU) and retrograde urethrography and cystography. Ureterocele was unilateral in 10 cases and bilateral in 2 cases (on a total of 14 cases). It was associated with simplex ureter in all cases and all patients underwent endoscopic surgery. No perioperative adverse event was reported. The postoperative course was uneventful. Clinical and radiological improvements were reported in all cases. Ureterocele associated with simplex ureter is a very rare urinary abnormality. Early diagnosis is essential to avoid upper urinary tract involvement. Endoscopic treatment is a good alternative leading to satisfactory results.

Femoral hernia in children: How to avoid misdiagnosis?

BACKGROUND: Femoral hernias are an uncommon groin pathology among pediatric patients. Therefore, they are frequently misdiagnosed. In the present study, we review our experience with this rare surgical entity during the past 25 years. METHODS: The medical records of 19 patients who underwent 22 femoral hernia repairs between January 1994 and December 2019 were retrospectively analysed. RESULTS: Patients' age ranged from 2 to 12 years (mean age was 5. 5 years) with an approximately equal sex ratio (10 girls/9 boys). There were three bilateral cases identified separately. They were discovered and managed at different times. All the children were referred with a groin lump, but the correct pre-operative diagnosis was made in only 13 cases (59%). In the remaining cases, four were identified intraoperatively following negative exploration for a supposed inguinal hernia. The other five were found to have a femoral hernia 1 month to 12 months after ipsilateral inguinal hernia repair. All patients underwent elective surgery. The femoral canal was closed using either Lytle or McVay procedure. Recurrence occurred in only one patient 2 months after initial repair. CONCLUSION: Femoral hernias are often misdiagnosed. Pre-operative diagnosis can be obtained through careful clinical assessment. In equivocal cases, ultrasonography and laparoscopy could be useful. A correct pre-operative diagnosis will lead to suitable treatment, thus avoiding unnecessary reoperations and their related complications.

Promising proteins detected by Western blot from Echinococcus granulosus protoscoleces for predicting early post-surgical outcomes in CE-affected Tunisian children.

BACKGROUND: Cystic echinococcosis (CE) affects predominantly young patients in highly endemic areas. Improved serological methods are needed for the follow-up of CE cases, especially given the high rates of post-surgical relapse that require detection as soon as possible. METHODS: We designed a study to investigate the value of antigenic proteins extracted from Echinococcus granulosus (E. granulosus) protoscoleces, and of recombinant B2t and 2B2t proteins, for assessing the efficacy of surgical treatment carried out on CE-affected children. This study was performed on 278 plasma samples collected from 59 Tunisian children surgically treated for CE and monitored for 3 years post-surgery. The patients were classified according to post-surgical outcomes into a "non-relapsed" (NRCE) and a "relapsed" (RCE) group. We performed in-house ELISAs to measure anti-B2t and anti-2B2t IgG and immunoblotting for the detection of IgG against SDS-PAGE-resolved E. granulosus protoscoleces-specific antigens. The Wilcoxon test was applied to assess anti-B2t and anti-2B2t IgG levels. We applied the Cochran Q test to compare the distribution of immunoblotting antigenic bands between 1-month and 1-year post-surgery. RESULTS: The probability of being "relapse-free" when a decrease in antibody titers occurred between 1 month and 1 year post-surgery was 81% and 75%, respectively, for anti-B2t and anti-2B2t IgG. We identified five protoscolex protein bands of 20, 26/27, 30, 40 and 46 kDa as highly immunoreactive by immunoblot for both RCE and NRCE patients at 1 month post-surgery, and significantly lower immunoreactivity after 1 year (p < 10-4) for NRCE compared to RCE patients. The proteins at 26/27 and 40 kDa displayed the best performance in predicting the outcome, with an 84% probability of being relapse-free when the reactivity against the 40 kDa antigen, the doublet at 26/27 kDa, or both was absent or disappeared between 1 month and 1 year post-surgery, and a 93% probability of being relapsed when both bands remained reactive or increased in intensity between the two time points. CONCLUSIONS: The B2t protein could be useful for the prediction of CE early post-surgical outcomes. The proteins of E. granulosus protoscoleces, especially the doublet P26/27 and P40, could be promising predictive biomarkers for the post-surgical follow-up of CE cases as well.

Isolated central sterna clefts: A rare congenital malformation.

Sternal cleft is a rare congenital anomaly which is generally observed at birth. The aetiology remains obscure. Superior clefts are more frequent than inferior ones, and isolated central clefts are extremely rare. Surgery is recommended to protect the heart and other mediastinal contents from trauma and also to improve respiratory dynamics. We present the case of a newborn with isolated central clefts and we will give a review of the literature.

Peritoneal Hydatid Cysts in Children: A Case Series of Rare Echinococcosis Localization.

Peritoneal hydatid cysts are rare in children even in endemic areas. The primary or secondary origin of this site remains controversial, especially in children. Secondary peritoneal hydatid cysts are mainly the result of spontaneous or traumatic rupture of concomitant liver cysts or the leakage of cystic content during surgery. The purpose of our study is to present the largest case series of peritoneal hydatidosis in children. In addition, we aimed to assess the clinical and paraclinical findings as well as the management of echinococcosis at this location in children. The present case series is a study of ten children with peritoneal hydatid cysts, who underwent surgical intervention between 2013 and 2018 in the Pediatric Surgery Department, University of Monastir (Monastir, Tunisia). The mean age of the children was six years. All children presented abdominal pain, and underwent ultrasonography and contrast-enhanced computed tomography of the abdomen. Two patients had been operated on for lung hydatid cysts six months prior to the study. In two cases, radiologic investigations revealed the presence of an uncomplicated hepatic hydatid cyst located in segments II and IV. All patients underwent surgery, of which four underwent laparoscopy. Post-surgery, all patients received albendazole for three months, and the follow-up period was uneventful. Currently, all patients are in good health. Peritoneal hydatid disease is frequently secondary to the rupture of a primary hepatic cyst. Diagnosis is performed by abdominal ultrasound, computed tomography, and a positive serology result. Open or laparoscopic excision can be combined with medical treatment.

Soluble programmed death-1 (sPD-1) as predictor of early surgical outcomes of paediatric cystic echinococcosis.

AIMS: Following treatment, cystic echinococcosis (CE) exhibits a relatively high relapse rate. Here, we evaluated the value of soluble programmed death-1 (sPD-1), sPD-1 ligand (sPD-L1) and anti-recP29 antibody concentrations, as predictors of early surgical treatment outcomes in young CE-affected patients. METHODS AND RESULTS: This prospective study included 59 Tunisian children (177 plasmas), where CE was surgically treated and monitored for 3 post-operative years. Based on CE post-surgical development, patients were clustered into a 'No relapsed' CE (NRCE; n = 39) and a 'Relapsed' CE (RCE; n = 20) group. Plasma levels of sPD-1, sPD-L1 and anti-recP29 IgG were measured using ELISA. In the NRCE group, sPD-1, sPD-L1 and anti-recP29 IgG concentrations were significantly lower at D365 than at D30. By contrast, in the RCE group, no significant difference was observed between D0, D30 and D365. When considering individual variations, the probability to be 'relapse-free' was 67% and 73% when anti-recP29 IgG and sPD-L1 level, respectively, decreased between D30 and D365. The probability to be 'relapse-free' was 86% when the sPD-1 level decreased between D30 and D365 (P = .003; chi-square test). CONCLUSION: sPD-1 may be a useful biomaker for the early evaluation of surgical procedure efficacy in paediatric CE cases.

Capitonnage seems better in childhood pulmonary hydatid cyst surgery.

BACKGROUND: Pulmonary hydatid disease remains an important healthcare problem. Conservative operative interventions including cystotomy or cystotomy with capitonnage are the two commonly used techniques. However, there is no scientific consensus over selection of these operative interventions. AIM: The aim of this study is to compare these two methods: capitonnage and uncapitonnage in the surgery of childhood pulmonary hydatid cyst in regard to the postoperative period. METHODS: This is a retrospective analysis of 136 patients operated for pulmonary hydatid disease between January 2010 and July 2017 according to two techniques. Group A was cystotomy with capitonnage (n = 76), and group B was cystotomy alone (n = 60). We compared the postoperative outcomes. RESULTS: Our data showed pneumothorax(PNO) and emphysema were seen in 30% of Group B and only in 13.2% in Group A, and the persistence of residual cavity in 23.3% in Group B and 7.9% in Group A (p = 0.014). We have not seen any case of recurrence with capitonnage. CONCLUSION: We conclude that capitonnage appears to prevent PNO and emphysema formation and a remaining residual cavity in the long term with a significant difference. And it prevents prolonged postoperative air leak and hospitalization with a slightly nonsignificant difference. It is difficult to say with absolute certainty that the noncapitonnage group is inferior to the capitonnage group, since several factors can influence the evolution. TYPE OF STUDY: Clinical research article Level of evidence III.

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.

Male Gender and Prematurity are Risk Factors for Incarceration in Pediatric Inguinal Hernia: A Study of 922 Children.

OBJECTIVES: The purpose of this study was to document clinical features of inguinal hernia (IH) in the pediatric population. It provides data to evaluate associated risk factors of incarcerated hernia, its recurrence as well as the occurrence of contralateral metachronous hernia. MATERIALS AND METHODS: We report a retrospective analytic study including 922 children presenting with IH and operated from 2010 to 2013 in our pediatric surgery department. RESULTS: We managed 143 girls (16%) and 779 boys (84%). The mean age was 2 years; the right side was predominantly affected (66.8%, n = 616). Incarcerated hernia was documented in 16% of cases with an incidence of 33% in neonates. The incarceration occurrence was 15.5% in males versus 2.09% in females. The surgical repair was done according to Forgue technique. Postoperatively, four cases of hernia recurrence were documented, and contralateral metachronous hernia was reported in 33 children with 7.7% females versus 2.8% males. Forty-five percent of them were infants. The mean follow-up period was 4 years. We think that incarceration can be related to several risk factors such as feminine gender, prematurity, and the initial left side surgical repair of the hernia. CONCLUSION: IH occurs mainly in male infants. Prematurity and male gender were identified as risk factors of incarceration. Contralateral metachronous hernia was reported, especially in female infants and after a left side surgical repair of the hernia.

Priapism in the Newborn: Shall We Intervene?

Idiopathic neonatal priapism is rarely published. We report the case of a newborn presenting with priapism on the first day of life and reviewed the published data on the management and the follow up of this condition.

Genetic relationship between the Echinococcus granulosus sensu stricto cysts located in lung and liver of hosts.

G1 genotype of Echinococcus granulosus sensu stricto is the major cause of hydatidosis in Northern Africa, Tunisia included. The genetic relationship between lung and liver localization were studied in ovine, bovine and human hydatid cysts in Tunisia. Allozyme variation and single strand conformation polymorphism were used for genetic differentiation. The first cause of genetic differentiation was the host species and the second was the localization (lung or liver). The reticulated genetic relationship between the liver or the lung human isolates and isolates from bovine lung, is indicative of recombination (sexual reproduction) or lateral genetic transfer. The idea of two specialized populations (one for the lung one for the liver) that are more or less successful according to host susceptibility is thus proposed.

Right Congenital Diaphragmatic Hernia Associated With Hepatic Pulmonary Fusion: A Case Report.

We present a case of male newborn presented with respiratory distress at 21 hours of life. The patient was operated for right congenital diaphragmatic hernia (CDH). Hepatic pulmonary fusion (HPF) was found at surgery.

First molecular evidence of the simultaneous human infection with two species of Echinococcus granulosus sensu lato: Echinococcus granulosus sensu stricto and Echinococcus canadensis.

Cystic echinococcosis is a widespread zoonotic parasitic disease especially in Tunisia which is one of the most endemic countries in the Mediterranean area. The etiological agent, Echinococcus granulosus sensu lato, implies dogs and other canids as definitive hosts and different herbivore species as intermediate hosts. Human contamination occurs during the consumption of parasite eggs passed in the environment through canid feces. Hydatid cysts coming from a child operated for multiple echinococcosis were collected and analyzed in order to genotype and to obtain some epidemiological molecular information. Three targets, ribosomal DNA ITS1 fragment, NADH dehydrogenase subunit 1 (nad1), and mitochondrial cytochrome c oxydase subunit 1 (CO1) genes, were amplified and analyzed by RFLP and sequencing approach. This study presents the first worldwide report in human of a simultaneous infection with Echinococcus granulosus sensu stricto (genotype G1) and Echinococcus canadensis (genotype G6) species. This is also the first report of the presence of E. canadensis in the Tunisian population which argues in favor of a greater importance of this species in human infestation in Tunisia than previously believed.

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.

Assessment of MYCN amplification status in Tunisian neuroblastoma: CISH and MLPA combining approach.

BACKGROUND: Neuroblastoma (NB) shows a complex combination of genetic aberrations. Some of them represent poor genetic prognosis factors that require specific and intensive chemotherapy. MYCN amplification consists of the major bad outcome prognostic factor, it is indeed frequently observed in aggressive neuroblastomas. To date different methods are used for MYCN status detection. OBJECTIVES: The primary aim of our study was to provide a critical assessment of MYCN status using 2 molecular techniques CISH and MLPA. We also focused on the correlation between neuroblastoma genetic markers and patient's clinical course among 15 Tunisian patients. METHODS: we developed a descriptive study that includes 15 pediatric Tunisian patients referred to our laboratory from 2004 to 2011. We reported the analysis of fresh and FFPE NB tumors tissues. RESULTS: No significant correlation was found between COG grade and patients overall survival. Assessment of NMYC gene copy number by kappa statistic test revealed high concordance between CISH and MLPA tests (kappa coefficient = 0.02). CONCLUSION: Despite misdiagnosing of MYCN status fewer than 5 copies, MLPA remains an effective molecular technique that enables a large panel of genomic aberrations screening. Thus combining CISH and MLPA is an effective molecular approach adopted in our laboratory. Our results allow pediatric oncologists to set up the first Neuroblastoma therapeutic strategy based on molecular markers in Tunisia.