Background: Tibial aplasia is of heterogeneous aetiology, the majority of reports are sporadic. We describe the reconstruction procedures in two subjects - a daughter and father manifested autosomal dominant (AD) inheritance of the bilateral tibial aplasia and split hand-foot syndrome. Materials and Methods: Reconstruction of these patients required multiple surgical procedures and orthoprosthesis was mandatory. The main goal of treatment was to achieve walking. Stabilization of the ankle joint by fibular-talar-chondrodesis on both sides, followed by bilateral Brown-procedure at the knee joint level has been applied accordingly. Results: The outcome was with improved function of the deformed limbs and walking was achieved with simultaneous designation of orthotic fitting. Conclusion: This is the first study encompassing the diagnosis and management of a father and daughter with bilateral tibial aplasia associated with variable split hand/foot deformity without foot ablation. Our patients showed the typical AD pattern of inheritance of split-hand/foot and tibial aplasia.

Introduction: The elastic stable intramedullary nailing (ESIN) presently seems the best technique in the surgical treatment of femoral shaft fractures (FSF) in >6-year-old children. We hereby report technical difficulties and therapeutic results after our first 8 years of experience. Patients and Methods: It's a retrospective study over a period of 8 years from January 2005 to December 2012 in the Paediatric Surgery Department of Sylvanus Olympio Teaching Hospital of Lomé. Indications, technical particularities and results were studied. Results: There were 32 patients, 17 were boys and 15 were girls, with a mean age of 11 years old. The mean time to surgery was 21 days (range: 14 and 51 days). A callus was always removed before reduction. The osteosynthesis was stable in 22 cases, but in 10 others, it had been completed with a cast immobilisation. The operation needed a blood transfusion in 18 cases. With a mean follow-up of 3.5 years, the results were excellent in 29 patients (90.63%) and good in 3 patients (9.37%). Conclusion: Although undertaking an ESIN can be difficult, due to the lack of adequate equipment the procedure produces satisfactory outcome.

Background: The treatment of the persistent peritoneo-vaginal duct (PPVD) or persistent processus vaginalis (PPV) in children by inguinal access carries a real testicular risk that can be avoided using the laparoscopic treatment, mostly in the case of recurrence. We hereby present the results of our first series treated by laparoscopy at Sylvanus Olympio Teaching Hospital. Patients and Methods: This is a descriptive and prospective study on a 6 months period. It concerned 21 boys with mean age of 4 years and 3 months, weighing 15.70 Kg. They clinically presented 12 unilateral inguinal hernias and 9 hydroceles among which one recurrence. We used the Montupet and Esposito technique. The results were considered good in absence of recurrence and bad if recurrence occurred. Results: With 4 over the 21 patients (19.04%), the diagnosis of a controlateral PPV was done per operatory. In those cases, the bilateral closure was systematic in the same operative time. The mean duration of the unilateral treatment was 57.6 minutes (extremes: 45 and 70 min). The mean pneumo-peritoneum pressure was 7.62 mm Hg (extremes: 5 and 8 mm Hg). Seventeen boys (80.95%) got out on the same day. There was neither infection nor parietal hematoma. With a mean follow-up of 9.3 months, there was no recurrence. Conclusion: The laparoscopic treatment of the PVDP in children is an efficient procedure. It permits to diagnose and treat at the same time, a controlateral PVDP that had not been found during the clinical examination; it also helps to avoid the traumatisms of the spermatic cord.

Background: The objective of the following study is to determine and to compare the different morphological parameters with duration of obstruction created experimentally in unilateral upper ureters of rats. Materials and Methods: Unilateral upper ureteric obstruction was created in 60 adult Wistar rats that were reversed after predetermined intervals. Rats were sacrificed and ipsilateral kidneys were subjected for analysis of morphological parameters such as renal height, cranio-caudal diameter, antero-posterior diameter, lateral diameter, volume of the pelvis and average cortical thickness: Renal height. Results: Renal height and cranio-caudal diameter of renal pelvis after ipsilateral upper ureteric obstruction started rising as early as 7 days of creating obstruction and were affected earlier than antero-posterior and lateral diameter and also were reversed earlier than other parameters after reversal of obstruction. Renal cortical thickness and volume of the pelvis were affected after prolonged obstruction (> 3 weeks) and were the late parameters to be reversed after reversal of obstruction. Conclusions: Cranio-caudal diameter and renal height were the early morphological parameters to be affected and reversed after reversal of obstruction in experimentally created ipsilateral upper ureteric obstruction.

Background: The objective of this study was to assess the outcome in children who had undergone pyeloplasty by lumbotomy approach using infant feeding tube (IFT; 5 Fr) as a single stent. Materials and Methods: During January 2000 and December 2010, 134 pyeloplasty were performed by the lumbotomy approach. The procedure involves single layer anastomosis at pelvi-ureteric junction using vicryl 5-0/6-0 (reduction of pelvis if required). An IFT 5 Fr with multiple holes used as a single stent to serve as nephrostomy and as transanastomotic stent also. Results: There were 109 males and 25 females with M: F ratio of 4.3:1. Left-side pelvi-ureteric junction obstruction (PUJO) was seen in 117 (87.3%) while right side PUJO in 17 (12.7%). Mean age of presentation was 52.7 months (range 9-120 months). Postoperative complications included infection 2 (1.5%), urinoma formation 1 (0.7%), urine leak 3 (2.2%), non drainage 2 (1.5%), accidental removal of the stent 2 (1.5%). Follow-up scan done at 3 and 9 months showed improved drainage in 124 (92.5%), preserved renal function in 129 (96.2%) cases. Overall success rate 97.5%. Conclusion: Transanastomotic stent using IFT not only provide an effective drainage but also avoid the complications associated with double-J stents and nephrostomies, with the added benefit of being cheaper and availability.

Background: Peptic oesophageal stricture (PES) is a serious complication of gastroesophageal reflux disease (GERD) in childhood. The treatment of PES is still controversial, ranging from simple oesophageal dilations to resection/anastomosis of the stenotic portion of the oesophagus. In this study, we want to share our experience with 11 children with GERD and PES. Patients and Methods: A retrospective review of clinical data obtained from children who underwent dilation and antireflux surgery for PES was performed. Results: A total of 11 patients were diagnosed with PES. The clinical picture was dominated by dysphagia. Barium swallow showed hiatal hernia in nine cases (82%). Oesophageal strictures were located most commonly in the lower third of the oesophagus (91%). Three Children (27%) with PES had a neurologic impairment and patients had a mean duration of symptoms of 20 months (range, 3 month to 6.2 years) before intervention. Children received a median of four dilations (range, 1-21 dilations) for PES. Time to first dilation from age of diagnosis was a mean of 4.5 months (range, 2-14 months). Antireflux surgery was performed in all patients. Post-operatively, seven patients required repeat oesophageal dilation. Patients were followed with serial dilation for a median of 6 years (range, 1-9 years) and only one patient has a continued requirement of oesophageal dilation for PES. Conclusion: GERD complicated by PES is an important condition affecting a significant number of children. Early and effective treatment of both stricture and GERD is required to improve the prognosis of this serious condition.

Background: Granulomatous appendicitis (GA) is a rare entity, mostly mentioned in adults. There have been anecdotal case reports describing GA in the paediatric population. This study was aimed at reviewing the cases of appendectomies to assess the incidence and characteristics of GA in children in a tertiary care University hospital. Materials and Methods: Records of children (<13 years age) with biopsy proven granulomatous lesions in the appendectomy specimen, treated during 1991-2011, were analysed. Data regarding demography, clinical presentation, radiological findings, intra-operative finding, histology, diagnosis and follow-up were recorded and descriptively analysed. Results: Twelve out of 1150 (1.04%) appendectomies were biopsy proven GA. Male to female ratio was 8:4. Four had Yersinia enterocolitis, two had Crohn's disease (CD; one isolated Crohn's Appendicitis, one Ileo-cecal Crohn' with appendicitis) and five were idiopathic. Remaining one case, initially diagnosed as idiopathic GA, developed full blown ileo-cecal CD at 2 ^nd month post-operative. Age ranged between 4 and 11 years with inflammatory bowel disease (IBD) affecting older children and Yersinia, seen in younger children. Majority (10/12) remained asymptomatic at a maximum of 5 years of follow-up. Two patients had recurrent symptoms; one with sub-acute obstruction (2 years follow-up) and another with flaring of Crohn's ileitis (2 months follow-up). Conclusions: GA in children is a rare entity, with incidence of 1.04% and male preponderance in our series. Idiopathic causes were the most common followed by Yersinia enterocolitis and CD. Although majority remained asymptomatic, IBD should be ruled out in case of recurrence of pain or alteration of bowel habit. Therefore, a long-term follow-up (at least for 5 years) of idiopathic GA is suggested in children.

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.

Background: Management of long gap oesophageal atresia with tracheoesophageal fistula (OA TEF) is challenging. Various intra-operative and pre-operative manures have been described to tackle this challenge. We reviewed our experiences with livaditis circular myotomy. The aim of this study was to evaluate long-term outcomes in cases of long gap OA TEF managed primarily with livaditis circular myotomy. Materials and Methods: This is a cross-sectional study including cases of long gap oesophagus managed by livaditis circular myotomy between January 1998 and October 2012. Their case records were evaluated for operative and post-operative data. The anthropometric data of these cases were collected. All these cases were subjected to barium swallow and manometry. Those cases with other associated neurological anomalies, multiple congenital anomalies, parents refusing consent for the study, less than 6 months of follow up or incomplete data were excluded from the study. Results: Out of the total of 109 patients of OA TEF managed, long gaps OA TEF were 37. Out of the 37 cases, 13 were managed by primary repair with livaditis circular myotomy. Of these 13 cases, 11 formed the study group. Mean age at evaluation was 36 ± 9 months. Mean age at primary surgery was 3 ± 2.5 days of life. Minor leak in the immediate post-operative period was present in 2/11 cases. Manometry was done in all the cases and revealed motility disorder in the form of un-coordinated contraction in 4/11 cases. Remaining 7/11 cases were normal. Conclusion: Livaditis circular myotomy is a viable option in the management of long gap OA TEF with good comparable long-term results.

Background: Congenitalmal formation represents defects in morphogenesis during early foetal life. Congenital anomalies account for 8-15% of perinatal deaths and 13-16% of neonatal deaths in India. The proportion of perinatal deaths due to congenital malformations is increasing as a result of reduction of mortality due to other causes owing to the improvement in perinatal and neonatal care. Materials and Methods: A retrospective record based study was conducted in the Paediatric Surgery Department of a Tertiary Care Institute of North India. The records of over a decade (2003-2012) were analysed. A total of 4305 cases of congenital anomalies were recorded in the study. All the data were entered in the excel spread sheet and analysed in SPSS version 17. (Statistical Package for the Social Sciences, a software package used for statistical analysis, officially named "IBM SPSS Statistics"). Results: Over the decade, a total of 14264 children were admitted in the paediatric surgery department with various problems. Out of these about one-third children (30.18%) had one or other type of congenital anomalies. This trend remained almost constant over the decade within a range of 26.8-33.6%. About half of the total congenital anomalies belonged to the gastro-intestinal tract, followed by genitourinary tract, central nervous system and other anomalies. All the anomalies were more common in males and were found in children belonging to rural communities. Conclusions: All the neonates should be examined with scrutiny for overt as well as occult congenital anomalies and Paediatric surgical care should be considered as an essential component of child health programmes in developing populations. Moreover, it is necessary to establish a registry system for congenital anomalies.

Background: To observe the histopathological spectrum of primary cardiac tumours in paediatric population those came in Pathology Department over a period of last 16 years. Materials and Methods: During the time period of 16 years (1995-2010), we had received 16 cases of primary cardiac tumours in paediatric patients. The tumour diagnosis and subtyping was done by histopathological examination along with cytochemistry and immunohistochemistry. Results: Benign cardiac tumours were much more common (15 cases) than the malignant tumours. Among these, myxoma was the most frequent (13 cases). The other benign cardiac tumours were rhabdomyoma (one case) and fibroma (one case). A primary malignant cardiac tumour was diagnosed in one case and was labelled as undifferentiated sarcoma. Conclusions: The present study reveals the pathological spectrum of surgically excised cardiac tumours in the paediatric population in northern India. Although the diagnosis of cardiac masses can be made by routine imaging techniques, but the role of pathologist is important for exact characterisation of tumour subtype.

Background: In cases of portal hypertension with splenic infarcts, splenectomy with proximal splenorenal shunt has been recommended. We are sharing our experience with distal splenorenal shunt in these cases contrary to the popular belief. Materials and Methods: Splenic infarcts were graded as mild, moderate and severe according to the pre-operative CT portogram. Mild, moderate and severe infarcts were defined as an infarct involving < 25%, 25-50% and > 50% area of the spleen, respectively. Mild and moderate infarcts were managed by spleen-preserving distal splenorenal shunt while those with extensive infarcts were subjected to splenectomy and proximal splenorenal shunt. Those with spleen-preserving shunts were closely followed in the post-operative period according to a uniform protocol. Clinical examination was regularly done to assess the size of the spleen and note the presence of pain, tenderness in the left intercostal space. An ultrasound Doppler was done after 7 days to assess shunt patency while CT portogram was repeated at 6 monthly intervals. Results: Fourteen cases with splenic infarcts formed the study group. Eight cases had mild infarcts, 3 had moderate infarcts and 3 had severe infarcts. Four underwent proximal splenorenal shunt, and 10 underwent warren's shunt (8 with mild and 2 with moderate infarcts). In 9/10 (90%), spleen could eventually be retained. Spleen completely regressed in them and so did the infarct. Conclusions:Spleen-preserving distal splenorenal shunt can be considered as a viable option in the management of cases with mild and carefully selected moderate splenic infarcts.

Background: Van der Woude syndrome (VWS), an autosomal dominant condition associated with clefts of the lip and/or palate and lower lip pits and is caused by mutations in interferon regulatory factor six gene. It is reported to be the most common syndromic cleft world-wide. Non-penetrance for the lip pit phenotype is found in at least 10% of affected individuals and those without the pits are phenocopies for non-syndromic clefting. The aim of this study is to present the phenotypic characteristic of VWS seen at the Lagos University Teaching Hospital (LUTH) cleft clinic. Materials and Methods: A review of cases of patients with VWS that attended the cleft lip and palate clinic at the LUTH Idi-Araba, Lagos, from January 2007 to December 2012 was conducted. Data analyses included sex of affected patients, types of cleft, presence of lower lip pits and history of lower lip pits/cleft in the family. Results: A total of 11 cases were seen during the period (male = 4; female = 7). Age at presentation ranged between 1 week and 12 years, with majority (n = 10) less than 2 years of age. Bilateral cleft lip and palate (BCLP) was seen in six patients, isolated soft palatal cleft (n = 3) and unilateral cleft lip and alveolus (n = 1) and cleft of hard and soft palate (n = 1). Bilateral lower lips were presented in 10 out of the 11 cases. The mother of the only patient without lip pits presented with bilateral lower lip pits. No family history of cleft/lip pits was elicited in 10 other cases. Conclusion: Most of the cases of VWS presented with BCLP and lower lip pits. Non-penetrance for the lip pits was seen in one out of 11 cases. Our study emphasizes the need to screen family members in all cleft cases, especially clinically diagnosed non-syndromic cases who may be VWS with no lip pits. Future studies are required to investigate the genetic causes of this syndrome in our population.

Background: Neonatal circumcision is one of the oldest and most frequently performed surgical procedures on males. Newborns demonstrate strong endogenous reaction to pain and therefore modalities are being explored for optimum pain relief during circumcision. Pediatric nurses have a vital role for the use of these modalities and minimising the pain response during the neonatal minor procedures. Aim: The aim of this study was to assess the effectiveness of eutectic mixture of local anaesthetic (EMLA) cream compared with oral sucrose and both in alleviating pain in neonatal circumcision. Materials and Methods: This study was conducted in the Day Care Surgery Department of Maternity and Children Hospital, Dammam City, KSA. 90 full-term newborn males who underwent circumcision were divided randomly into three groups (30 each). Each group was assigned to receive a different type of analgesics such as EMLA cream (Group A), oral sucrose (Group B) or combination of EMLA cream and oral sucrose (Group C). Neonatal pain agitation and sedation scale (N-PASS) was used 5 min before, during and 5 min after the circumcision procedure to assess the neonatal response to pain. Results: N-PASS scores were significantly lower in Group C (median Group C = 5.2, Group A = 5.8, Group B = 8.5; P< 0.001). The endogenous response to pain in terms of escalation of heart rate and reduction in O ₂ saturation were minimal among Group C (P < 0.0001). Duration of crying was comparable among all the groups. Conclusion: The combination of sucrose and EMLA cream revealed a higher analgesic effect and minimal adverse response to pain than either EMLA cream or sucrose alone during neonatal circumcision.

Pancreaticoduodenal injuries are rare in children. They pose challenges for the treating surgeon in decision making with respect to diagnosis, surgical and post-surgical management. The management plan must be tailored to the nature and severity of trauma, patient profile, surgeon's expertise and the resources available at surgeon's disposal. We describe a pancreaticoduodenal injury in 3-year-old child with successful outcome and follow-up of a decade.

Haemangioma is the most common benign neoplasm of infantile age. It is most commonly located in head and neck region, trunk and extremities but very rarely it can be located at clitoris. However, it is very important to differentiate clitoral haemangioma from enlargement of the clitoris secondary to androgen excess. Only four cases of clitoromegaly caused by cavernous haemangioma have been reported in the literature so far. Herein, we report our experience with a 10-year-old girl who presented with clitoromegaly and normal hormonal assay that turned out to be clitoral cavernous haemangioma after histopathological examination of the clitoral mass.

Splenic cysts are uncommon lesions and are classified as true or pseudocysts based on the presence of an epithelial lining. True congenital cysts can be epidermoid, dermoid or endodermoid and require surgery when large, sympthomatic or complicated. Ultrasonography and computerised tomography scan are useful for diagnosis and serum test for echinococcosis should be done. Several procedures, open or laparoscopic, have been described, with emphasis on the spleen-preserving surgery, when possible. We present a boy with a giant symptomatic epidermoid cyst in whom total splenectomy by open technique was carried out.

Carcinoid tumours of the appendix are uncommon incidentally detected tumours during histopathological examination following appendicectomy for acute appendicitis. Even though considered rare in children, they are the most frequently encountered tumours of the gastrointestinal tract. To our knowledge, carcinoid tumour of appendix in childhood has not yet been reported from Indian Subcontinent. The clinical presentation is similar to acute appendicitis and the signs and symptoms of carcinoid syndrome have not been reported in children. The prognosis of carcinoid tumour of appendix is excellent in children as the tumour is generally small in size and less aggressive with no metastasis. Simple appendicectomy is curative in most of the patients and long term follow up is debatable. We present here a case of carcinoid tumour of the body of appendix, which is an uncommon location in a 6-year-old child.

Congenital hernia of the umbilical cord (CHUC) is a rare congenital entity compared to more common post-natally occurring umbilical hernia. Although recognized as a distinct entity since 1920s, CHUC is often misdiagnosed as a small omphalocele, resulting in its underreporting. We present the first case report of CHUC associated with extracelomic colonic atresia, complicated by perinatal perforation in a newborn. We also discuss the differentiating features from other anterior abdominal wall defects such as omphalocele and gastroschisis including its embryogenesis.

Ileocecal valve atresia is a very rare anomaly with only a few cases reported in the literature. The conventional therapy includes ileocecal resection and ileocolic anastomosis. Here, we present a patient with inborn ileocecal valve atresia in whom we successfully preserved the ileocecal valve and the terminal ileum through opening a valve and tapering the ileum in one operation.

Gastrointestinal malignancies are extremely rare in the paediatric population and duodenal cancers represent an even more unusual entity. It represents 0.3-1% of all gastrointestinal tumours. A case report of a 10-year-old boy with duodenal adenocarcinoma is reported and the difficulties of diagnosing and treating this rare tumour are discussed.

Large palatal fistulas are a challenging problem in cleft surgery. Many techniques are used to close the defect. The tongue flap is an easy and reproductible procedure for managing this complication. The authors report a case of a large palatal fistula closure with anteriorly based tongue flap.

Multiple magnet ingestion is rare, but can cause serious gastrointestinal complications. We report a case of 7-year-old girl with multiple intestinal perforations caused by multiple magnet ingestion. The aim of this report is to draw attention to magnetic toys, results of magnet ingestion and the importance of timing of operation.

Mesenchymal chondrosarcomas are rare malignant tumours in children, especially, in neonates. The authors present a case of congenital mesenchymal chondrosarcoma in a 1-day neonate located in sacrum. According to the authors' literature searches, this case is the first congenital sacral mesenchymal chondrosarcoma. We also reviewed the papers published in English literatures.

A handmade snare is designed from easily available materials to treat bleeding per rectum in children due to rectosigmoid polyps. A total of 29 polypectomies were done in 24 patients. It is simple, effective, safe and economic.