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   2016| April-June  | Volume 13 | Issue 2  
    Online since May 17, 2016

 
 
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ORIGINAL ARTICLES
How to manage a late diagnosed Hirschsprung's disease
Mohamed Ouladsaiad
April-June 2016, 13(2):82-87
DOI:10.4103/0189-6725.182562  PMID:27251658
Background: How to manage a late diagnosed Hirschsprung's disease (HD) and how to avoid calibre discrepancy? Subjects and Methods: A retrospective study of all patients diagnosed with HD over 2 years in our hospital from January 2009 to December 2012. Data were analysed for clinical presentations, investigations, surgical procedures and post-operative outcome. Results: Fifteen patients, operated by one single surgeon, were included in this study. The mean age was 6 years (2-16 years). Patients had an ultra-short segment type in 4 cases, rectosigmoid type in 9 cases and descending colonic aganglionosis in 2 cases. Rectal wash out was effective in 12 patients. A blowhole transverse colostomy was performed in 2 patients. Twelve patients underwent one single stage endorectal pull-through. Anastomosis incongruence was avoided by a plication procedure never described before. The assessment of post-operative outcomes by the paediatric incontinence and constipation scoring system revealed a normal continence function in all our patients, but 3 patients suffered from soiling secondary to constipation. Conclusion: One single stage pull-through can be safe and effective in children with late diagnosed HD. Routine rectal washout is a good way to prepare the colon. In some cases, blowhole colostomy can be an option. Anastomosis incongruence is a challenge; we describe a plication procedure to avoid it.
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CASE REPORTS
Aneurysmal femoral neck cyst: Report of a paediatric case and review of literature
Oumar Ndour, Rodia Boseba, Jacque Barre Damipi, Juvenal Nibagora, Aimee Lakh Faye Fall, Gabriel Ngom, Mamadou Ndoye
April-June 2016, 13(2):103-106
DOI:10.4103/0189-6725.182568  PMID:27251662
The aneurysmal bone cyst (ABC) is a benign tumour of children and young adults. It represents approximately 1-2% of all bone tumours. The ABC may develop on all skeletal bones, but the proximal end of the femur is the most common location. The authors report a ABC femoral neck in a child of 13 years. This location is pretty special. Indeed, the fragility of the femoral neck due partly to the pathology itself and secondarily curettage requires a judicious attitude surgical (excisional curettage + bone graft + screw) to prevent the risk of high local recurrence and pathological fracture.
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ORIGINAL ARTICLES
Congenital retrosternal hernias of Morgagni: Manifestation and treatment in children
Oleksii Slepov, Sergii Kurinnyi, Oleksii Ponomarenko, Mikhailo Migur
April-June 2016, 13(2):57-62
DOI:10.4103/0189-6725.182557  PMID:27251653
Background: Due to scarcity of congenital diaphragmatic hearnias of Morgagni (CDHM), non-specific clinical presentation in the pediatric age group, we aimed to investigate the incidence, clinical manifestations, anatomical characteristics, and develop diagnostic algorithm and treatment of CDHM in children. Materials and Methods: The patients' records of children with CDHM treated in our hospital during past 20 years were retrospectively reviewed for the age at diagnosis, gender, clinical findings, anatomical features, operative details and outcome. Results: Since 1995 to 2014 we observed 6 (3 boys, 3 girls) patients with CDHM, that comprise 3.2% of all congenital diaphragmatic hernia cases (n = 185). Age at diagnosis varied from 3 mo. to 10y.o. Failure to thrive was main symptom in 4 patients, followed by recurrent respiratory infections (n = 3), dyspnea (n = 3), and gastrointestinal manifestations: constipation (n = 2), abdominal pain (n = 1). Work-up consisted of plain X-ray for all (n = 6), upper GI (n = 3), barium enema (n = 2), sonography (n = 6) and CT (n = 2). Abdominal approach used in 5 patients, and thoracotomy in one. Herniated contents were: liver lobes (n = 4), transverse colon (n = 3) and greater omentum (n = 1). 5 had right-sided lesion, 1- left-sided. Defect repaired using local tissues. Post-operative course was uneventful; all patients appeared well during follow-up. Conclusion: CDHM is very uncommon anomaly, very occasionally diagnosed at the early age. Failure to thrive and recurrent respiratory infections are most frequent clinical manifestations. In suspected CDHM we advocate the following work-up: plain chest and abdominal X-ray, contrast study (upper GI series or barium enema), ultrasonographic screen and CT scan. Surgical repair via abdominal approach, using local tissues and hernia sac removal is preferred.
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Diagnostic value of anti-smooth muscle antibodies and liver enzymes in differentiation of extrahepatic biliary atresia and idiopathic neonatal hepatitis
Mandana Rafeey, Lida Saboktakin, Jamshid Shoa Hasani, Shahnaz Naghashi
April-June 2016, 13(2):63-68
DOI:10.4103/0189-6725.182558  PMID:27251654
Background: We aimed to evaluate the diagnostic value of anti-smooth muscle antibodies (ASMA) and two liver markers (gamma-glutamyl transpeptidase [GGT] and alkaline phosphatase [ALP]) for differentiating between patients with extrahepatic biliary atresia (EHBA) and idiopathic neonatal hepatitis (INH). Materials and Methods: During April 2010-2011, all infants at 2 weeks of age who were diagnosed with cholestasis and admitted to Children's Hospital of Tabriz were enrolled. Based on the results of physical examination, laboratory, imaging and pathological studies, neonates were divided into two groups (EHBA and INH). Receiver operating characteristics analysis was used to define sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy for ASMA, GGT and ALP. Results: Thirty neonates with cholestasis (18 with EHBA and 12 with INH) and mean age of 54.66 ΁ 25.86 days were enrolled. Total and direct bilirubin, serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase and ASMA titres were highly not significant (P > 0.05) in patients with INH. GGT (P = 0.008) and ALP (P = 0.01) had statistically significant differences that were higher in patients with EHBA. The sensitivity, specificity, PPV and NPV, accuracy, LR+ and LR− of SMA in differentiating cases with BA were 66.7%, 75%, 80% 60%, 70%, 2.68 and 0.44, respectively. For GGT, the values were 88.9%, 66.7%, 80%, 80%, 79.1%, 3.08 and 0.31, respectively. Finally, for ALP, the values were 77.8%, 75%, 82.4%, 69.2%, 80%, 2.66 and 0.24, respectively. Conclusion: Our study showed that ASMA may be a useful biomarker for differentiation of EHBA from INH. Further studies with larger samples are recommended for confirming the results of this study.
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Corrections of diverse forms of lower limb deformities in patients with mucopolysaccharidosis type IVA (Morquio syndrome)
Ali Al Kaissi, Vladimir Kenis, Eugeniy Melchenko, Maher Ben Ghachem, Robert Csepan, Franz Grill, Rudolf Ganger
April-June 2016, 13(2):88-94
DOI:10.4103/0189-6725.182563  PMID:27251659
Background: Thoracolumbar kyphosis has been considered as the first presenting deformity and is often a key diagnostic clue noted in children with mucopolysaccharidosis (MPS) type IV (Morquio's syndrome). However, we observed that the progressive irregularities of the epiphyses of the long bones were the most prominent skeletal pathology, causing effectively the development of diverse forms of lower limbs deformities with extreme variation in age of onset. Materials and Methods: Ten patients (seven children and three adults) with an average age of 15 years have been enrolled in this study. Age of diagnosis of MPS IVA has a variable age of onset and a MISLEADING rate of severity. Hip dislocations, genu valgum, protrusio acetabuli and osteoarthritis were the most common lower limbs deformities in these patients. Clinical and radiographic phenotypes were the baseline tools of documentation. Urinary screening and genotypic characterizations have been applied accordingly. Results: Combined pelvic and femoral procedures for hip dislocation, epiphysiodeses and supracondylar osteotomy for genu valgum and hip arthroplasty for protrusio acetabuli have been performed. All patients manifested insufficient activity of N-acetylgalactosamine-6-sulphate sulphatase, an enzyme that degrades keratin sulphate and chondroitin-6 sulphate. Conclusion: The extensive clinical heterogeneity contributed significantly in the delay in establishing the diagnosis particularly in adult patients with MPS IV. The epiphyseal irregularities of the long bones and the progressive flattening pathology of MPS IV A were the reason to falsely diagnose some patients as spondyloepiphyseal dysplasia congenital and/or tarda. Proximal femoral osteotomy, realignment osteotomy and total hip arthroplasty have been performed for coxa vara, genu valgum and protrusio acetabuli, respectively, in children and adult group of patients. The importance of early diagnosis on MPS IV A is to receive enzyme replacement therapy and plan for other therapeutic measures.
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Paediatric day-case neurosurgery in a resource challenged setting: Pattern and practice
Afolabi Muyiwa Owojuyigbe, Edward O Komolafe, Anthony T Adenekan, Muyiwa A Dada, Chiazor U Onyia, Ibironke O Ogunbameru, Oluwafemi F Owagbemi, Ademola O Talabi, Fola A Faponle
April-June 2016, 13(2):76-81
DOI:10.4103/0189-6725.182561  PMID:27251657
Background: It has been generally observed that children achieve better convalescence in the home environment especially if discharged same day after surgery. This is probably due to the fact that children generally tend to feel more at ease in the home environment than in the hospital setting. Only few tertiary health institutions provide routine day-case surgery for paediatric neurosurgical patients in our sub-region. Objective: To review the pattern and practice of paediatric neurosurgical day-cases at our hospital. Patients and Methods: A prospective study of all paediatric day-case neurosurgeries carried out between June 2011 and June 2014. Results: A total of 53 patients (34 males and 19 females) with age ranging from 2 days to 14 years were seen. Majority of the patients (77.4%) presented with congenital lesions, and the most common procedure carried out was spina bifida repair (32%) followed by ventriculoperitoneal shunt insertion (26.4%) for hydrocephalus. Sixty-eight percentage belonged to the American Society of Anesthesiologists physical status class 2, whereas the rest (32%) belonged to class 1. General anaesthesia was employed in 83% of cases. Parenteral paracetamol was used for intra-operative analgesia for most of the patients. Two patients had post-operative nausea and vomiting and were successfully managed. There was no case of emergency re-operation, unplanned admission, cancellation or mortality. Conclusion: Paediatric day-case neurosurgery is feasible in our environment. With careful patient selection and adequate pre-operative preparation, good outcome can be achieved.
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CASE REPORTS
Laparoscopic treatment of adrenal masses in children: Report of two cases and review of literature
Antonio Orofino, Cosetta Maggipinto, MariaPaola Lanzillotto, Michele D'Amato, Massimo Ronzini, Guglielmo Paradies
April-June 2016, 13(2):98-102
DOI:10.4103/0189-6725.182565  PMID:27251661
Laparoscopic adrenalectomy has become a common alternative to open surgery for the resection of adrenal lesions in adults: The advantages are to provide better exposure of the adrenal gland, diminish soft tissue dissection, decrease morbidity and postoperative pain; however, reporting on the laparoscopic adrenalectomy in paediatric patients has been limited. We present two cases of laparoscopic adrenalectomy performed at our institution in two children, for left adrenal neuroblastoma in a first patient with opsomyoclonus syndrome, and for a right incidentaloma in the second case. According to recent literature, our experience has demonstrated that the laparoscopic adrenalectomy is a feasible procedure in children with small, well-circumscribed adrenal masses: It can be used a safety to treat suspected benign and malignant adrenal masses in children, with minimal morbidity and short hospital stay. The lateral trans-peritoneal approach offers optimal visualisation and good outcomes in terms of minimal discomfort, rapid recovery and excellent cosmesis. However, in the paediatric field, the number of patients is limited, making the learning curve longer.
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ORIGINAL ARTICLES
Is there any correlation between radiologic findings and eradication of symptoms after pyloromyotomy in hypertrophic pyloric stenosis?
Davoud Badebarin, Saeid Aslanabadi, Fereshteh Yazdanpanah, Sina Zarrintan
April-June 2016, 13(2):73-75
DOI:10.4103/0189-6725.182560  PMID:27251656
Background: Hypertrophic pyloric stenosis (HPS) is one of the most common gastrointestinal disorders during early infancy, with an incidence of 1-2:1000 live births in the world. In this study, we aimed to investigate the correlation between radiologic findings and eradication of symptoms after pyloromyotomy in HPS. Materials and Methods: One hundred and twenty-five (102 boys and 23 girls) patients with suspected infantile HPS were treated surgically by Ramstedt pyloromyotomy between March 21, 2004 and March 20, 2014 at paediatric surgery ward of Tabriz Children's Hospital, Iran. The demographic features, clinical findings, diagnostic work-up, operation type and postoperative specifications of the patients were studied retrospectively. Results: Male to female ratio was 4:1. The patients were 16-90 days of old and the mean age was 39 ± 1.42 days. The range of pyloric canal length was 7.60-29.00 mm and the mean length was 19.54 ± 3.42 mm. Pyloric muscle diameter was 2.70-9.00 mm, and the mean diameter was 4.86 ± 1.14 mm. Seventy-two percent of patients had episodes of vomiting after operation. Mean time of persistence of vomiting after pyloromyotomy was 15.73 ± 0.15 h. Mean discharge time was 55.22 ± 0.08 h. Radiologic findings did not show any significant correlation with persistence of vomiting or discharge time. Conclusion: The present study revealed that radiographic findings could not predict postoperative symptom eradication after pyloromyotomy in HPS.
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Our experience with pre-operative haemostatic assessment of paediatric patients undergoing adenotonsillectomy at Federal Medical Centre, Makurdi
Amali Adekwu, Agida Samuel Adoga, Terna Ambrose Gav
April-June 2016, 13(2):69-72
DOI:10.4103/0189-6725.182559  PMID:27251655
Background: In 2-4% of all patients requiring adenoidectomy, tonsillectomy or adenotonsillectomy, pre-operative screening tests for coagulation disorders are indicated to detect surgical bleeding complications. However, because of cost effect on the patients, the usefulness of these tests is being challenged. We therefore highlight our experience in paediatric patients undergoing adenoidectomy, tonsillectomy or both in our centre. Patients and Methods: This is a 3½-year analysis of the data of 165 paediatric patients who had adenoidectomy, tonsillectomy or both over the study period. The data collected included age, sex, procedure done and detailed clinical bleeding history. Results: A total of 165 children had either adenoidectomy or tonsillectomy, or both. There were 76 males and 89 females giving a male to female ratio of 1:1.2. Their ages ranged from 10 months to 18 years. Eighty-five (51.5%) patients had adenotonsillectomy, 48 (29.1%) and 32 (19.4%) had only tonsillectomies and adenoidectomies, respectively. Only 11 (6.7%) families volunteered the history of either prolonged bleeding with minor injury on the skin or occasional slight nose bleeding. Six (3.6%) patients including 3 of the children with positive family history had posttonsillectomy bleed, out of which 4 (66.7%) were moderate whereas the remaining 2 (33.3%) were severe bleeding, which was not statistically significant (P = 0.041). The two cases of severe bleeding had fresh whole blood transfused whereas the rest that had no bleeding issues were discharged home 48 h postoperatively. Conclusion: Our experience in this study suggests that detailed bleeding history is necessary as well as pre-operative haemostatic assessment, if available and affordable for paediatric patients undergoing adenotonsillectomy.
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CASE REPORTS
Volvulus of the jejunum on cystic lymphangioma: About a clinical case
Yacaria Coulibaly, Soumaila Keita, Aliou Doumbia, Adegne Togo
April-June 2016, 13(2):95-97
DOI:10.4103/0189-6725.182564  PMID:27251660
Intestinal volvulus on mesenteric cysticum lymphangioma (CL) is rare in children. The clinical picture is not very suggestive. We report a case of intestinal volvulus on CL in a 7-year-old girl after an abdominal trauma. Resection and anastomosis were made. The confirmation diagnosis was done by anatomopathological examination. Early diagnosis of intra-abdominal CL will allow avoiding complication.
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LETTER TO THE EDITOR
A glance at rabies pre-exposure and post-exposure prophylaxis for dog bites
Mirko Bertozzi, Victoria Elisa Rinaldi, Giuseppe Di Cara, Antonino Appignani
April-June 2016, 13(2):107-108
DOI:10.4103/0189-6725.182569  PMID:27251663
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