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.

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.

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.

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.

[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.

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.

[Epidemiological changes of paediatric urolithiasis in Tunisia]. / Evolution épidémiologique de la lithiase urinaire infantile en Tunisie.

BACKGROUND: The clinical and biological characteristics of urolithiasis in children are still evolving in our country. Since the eighties no study has been conducted to determine incidence of urolithiasis in Tunisia. AIM: To define the current status of urolithiasis in Tunisian children. METHODS: The records of 414 children with urolithiasis treated between 1983 and 2007 were found in a multi-centric study which took into consideration to age, sex, and history diagnosis and physical, laboratory, and radiologic findings. Clinical and biological date were performed in 344 cases. Stone analysis was performed by spectrophotometry. RESULTS: The incidence of paediatric urolithiasis has steadily decreased from 0.7/ 10 000 of the child population / year in 1991-1994 to 0.1 cases/10 000 children/ year in 2007. There was a continuous decrease in bladder stone over the past 25 years (47.1% in 1982-1986 Vs 10.2% in 2002-2007). Stones were homogeneous in 31.4% of cases. The main component was calcium oxalate stones in 36.4% of the cases. Since the eighties the increase of oxalates stones (15.6% in eighties Vs 51.5% actually) were with depends to purines and struvite stones. CONCLUSION: Epidemiologic characteristics of the urinary lithiasis (sex, age, localization and composition) have changed in Tunisia, due to the the improvements of standard of living. The occidentalization of the nutritional practices and the receding of the culinary traditions gave birth to new risks of urolithiasis characterized by the emergence of calcium oxalate stones.

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.

Urinary stone composition in pediatric patients: a retrospective study of 205 cases.

BACKGROUND: Stone composition has changed substantially over the past decades in developed countries. We studied urinary stone composition in Tunisian children. METHODS: We studied 205 children ranging from 3 months to 16 years old age and who were diagnosed as having urinary stones (122 boys and 83 girls). Stone analysis was performed respectively using a stereomicroscope and by infrared spectroscopy to determine, respectively, the morphological type and molecular composition of each. RESULTS: Of 205 stones analysed, 77 (37.5%) were composed of a single constituent. Calcium oxalate (CaOx) stone were predominant, being a constituent of 54.7% of stones, followed by calcium phosphate and purines; 14.6% of cases. Calcium oxalate monohydrate was more frequently seen in girls (p < 0.01), while struvite was more common in boys (p < 0.001). Teenagers were more affected by whewellite stones compared with infants (p < 0.05). Purine stones were predominant in 20% of cases, but its prevalence decreased with age. CONCLUSIONS: The increase in calcium oxalate stones in school age children and the decrease of struvite and purines stones confirm the change in the etiology of urolithiasis with respect to age.

Nutritional aspects of idiopathic nephrolithiasis in Tunisian children.

OBJECTIVE: We evaluated the metabolic and the nutritional aspects of 134 urolithiasic children, in order to outline the risk factors that contribute to idiopathic stone formation in children. MATERIAL AND METHODS: In this prospective study 134 children (56 females, 78 males) with renal calculi were evaluated. The age of the patients ranged 6 months to 16 years. A dietary survey was performed on every child. All patients were investigated with respect to stone localization, serum and urine risk factors. Statistical analysis of data was carried out using software SPSS 11.0 for Windows. Statistical significance was determined using chi-square test. RESULTS: Hypercalciuria was the commonest risk factor detected in this group (28.3%). A decrease of water intake was noted in all age group specially in the rural area (549.6 vs 1150.6 ml/day), and an increase in animal protein intake in 17 cases (mean 1.9 g/kg). In addition, increased intake of starchy foods and food with high oxalate content (sorgum) was observed in our 10-16 years group (51%). Calcium oxalate monohydrate represents the principal component of idiopathic stone (58.2%), which is more frequent in children (68%) than infants (51.7%) (P < 0.02). CONCLUSIONS: The high frequency of idiopathic urolithiasis highlights the influence of dietary habit in stone formers in our country. The increase in calcium oxalate stones in school-age children confirms the change in the etiology of urolithiasis according to age.

[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.

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.

Paediatric urolithiasis in central coast region of Tunisia: changes in stone composition according to age and gender.

OBJECTIVE: Studies evaluating the influence of age and gender on the distribution of the various types of paediatric urinary calculi are scarce. Aim of our study was to high-light the modification of epidemiological characteristics of this pathology depending on patients' age and gender. PATIENTS AND METHODS: We present the results of a study based on 205 calculi (from 122 boys and 83 girls) analyzed by infrared spectroscopy between 1993 and 2007. 54.6% of the patients were under 5 years. RESULTS: Calcium oxalate (CaOx) was the predominant constituent in 54.7% of stones, followed by calcium phosphate and ammonium urate (14.6% each). We found a male preponderance for struvite stones (12.3% vs 1.2%), and an increasing prevalence of calcium oxalate stones with age (42.9% in infants vs 59.3% in older children). Purines stones were observed in 20% of cases, but their prevalence decreases with age (28.6% in infants vs 18.5% in old children). CONCLUSION: The increase of calcium oxalate stone rate in school age children and the decrease of purines stones confirm the change on the etiology of urolithiasis according to age.

Paediatric urolithiasis in central coast region of Tunisia: clinical characteristics.

OBJECTIVE: To show an outline of the clinical and biological characteristics of paediatric urolithiasis among Tunisian children in the coast region. PATIENTS AND METHODS: This retrospective study included 168 children under the age of 16 years presented with urinary stones (100 boys and 68 girls). Patients were reviewed in a multi-centric study with regard to age at diagnosis, sex, history, and physical, laboratory, and radiologic findings. The physical and chemical analysis of stones was carried out respectively by a stereomicroscope and by infra-red spectroscopy. RESULTS: The sex ratio was 1.47. The clinical presentation of this pathology was dominated by abdominal pain (28%), hematuria (25.6%), dysuria (16.7%) and urinary tract infection (14.3%). Stones were located in the upper urinary tract in 75.6% of cases. Of the urine cultures, 14.3% were positive. Whewellite was more frequent in children stones than in infants (p < 0.05) and was the main component in 46.4% of stone section and 55.4% in stone surface. CONCLUSION: The male prevalence of paediatric urolithiasis is progressively decreasing in Tunisia. The epidemiological profile of renal stones in our country has changed towards a predominance of calcium oxalate stone and upper tract location.