Background: To review the results of Wilms' tumour patients in a tertiary referral hospital in a developing country and to find ways of improving long-term survival. Patients and Methods: Between January 1998 and May 2004, 40 patients with Wilms' tumour (WT) were admitted to Queen Elizabeth Central Hospital. Their files were reviewed and general physical condition on admission, pre-operative investigations, management and outcome were noted. Results: The mean age of presentation was 4.2 years with an equal distribution between the sexes. The mean BMI was 15 kg/m² and more than 80% of the patients were either mildly (PCV <33%) or severely anaemic (PCV <24%). All patients presented with abdominal distension. Half of them had additional complaints including abdominal pain, haematuria, dyspnoea, oedema and or weight loss. Thirty-nine out of the forty patients received pre-operative chemotherapy. Of the 36 patients that underwent surgery, 32 underwent total nephrectomy, one a partial nephrectomy, and in three the tumour was irresectable. There were no intra-operative tumour ruptures. Only 15% of the patients completed their post-operative course of chemotherapy. The 1-year survival lies between 25% and 53%. Fifteen of the 36 patients operated were known to have a recurrence. Conclusion: The patients presented in an advanced stage of the disease. Survival rates are disappointing and recurrence rates are high. Some improvement in outcome may be expected with the implementation of more aggressive treatment protocols but early diagnosis, completion of treatment regimens are needed. Pro-active follow-up is essential to measure outcome.

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Background: Mesenteric cysts are rare intra-abdominal masses in the paediatric age group with varied presentation, ranging from an asymptomatic mass to acute abdomen. This study reviews our experience in the diagnosis and treatment of 17 mesenteric cysts in our centre, with especial reference to acute abdominal symptoms. Patients and Methods: Seventeen patients (age less than 10 years) with mesenteric cysts were managed in our hospital. The age ranged from 15 days to 10 years. Patients were admitted with acute or chronic symptoms. They were evaluated with complete history, clinical examination, blood investigations and radiological investigations (x-ray abdomen erect, ultrasound abdomen (USG) and computed tomography (CT) scan in selected cases) to reach a provisional diagnosis. The diagnosis was proven on laparotomy and histologically confirmed. Results: The main presenting symptoms were abdominal pain or lump. The most common mode of presentation was acute small intestinal obstruction. USG was not conclusive in all. Abdominal CT scan with intravenous contrast was diagnostic in nine patients. Five patients had volvulus on exploration. Cysts were located in small intestinal mesentery in 14 cases and three were in the sigmoid mesentery. Seven patients had complete excision, intestinal resection was required in four and marsupialisation with cauterisation of margins was done in six patients. Histologically, all were lymphangiomatous mesenteric cysts. Conclusion: The diagnosis of mesenteric cysts should be kept in mind in any patient presenting with acute abdominal symptoms. Small bowel volvulus with mesenteric cyst constituted a significant number in children with acute abdominal symptoms. Early diagnosis and treatment yields excellent outcome.

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Background: The management of Hirschsprung's disease remains a problem in developing countries. Our aim is to identify the main epidemiological, clinical, and therapeutic characteristics of Hirschsprung's disease at the University Child Hospital Charles De Gaulle of Ouagadougou (CHUP-CDG). Patients and Method: It is a retrospective study carried out in the period from January 2001 to December 2007 in the Surgery Unit at CHUP-CDG, which is a reference centre for Paediatric Surgery in Burkina Faso. Results: There were 52 patients (M: F=3.3:1). The annual incidence was seven cases. Age at presentation and diagnosis ranged from two days 10 years (median 20 months). Twenty five patients were from poor socio-economic conditions. Presentations were mainly intestinal obstruction, chronic constipation and enterocolitis. There were two cases of associated trisomy 21. Average age at operative intervention was 3.17 months. The rectosigmoidal form was the most frequently encountered. Over two-thirds (67.31%), with no complications at presentation, had benefited from nursing before their final treatment. A temporary colostomy was requested in case of complication. Swenson's technique was practiced for all the patients who underwent surgery operation. The assessment of functional results in eight patients after an average decrease of 3.5 years gave excellent results. Post-surgery complications were mainly enterocolitis in 12% of patients. Mortality rate was 16%. Conclusion: Management of Hirschsprung's disease is a problem in Burkina Faso. It is characterised by its late presentation and difficult diagnosis due to inaccessibility and the non-availability of some investigation services (barium enema, histochemistry, and histology), resulting in high morbidity and mortality rates. Effective technical capacities, adequate staff training, and public education will be necessary to improve care quality.

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Africa has one of the highest neonatal mortalities in the world, for which the commonest causes do not include surgical conditions such as some congenital anomalies that are amenable to surgery but are not often operated on because of a number of challenges. These challenges include cultural beliefs and practices, dearth of human resource capacity, inadequate laboratory and imaging support and lack of consumables and intensive or high dependency care facilities. Some of these challenges will be examined and highlighted using the acronym "ASKS" in this article.

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Background: Laparoscopic appendectomy is a feasible and safe alternative to open appendectomy for uncomplicated appendicitis. In the past decade several laparoscopic procedures have been described using one or more ports. We report our experience in treating acute appendicitis with one-port transumbilical laparoscopic-assisted appendectomy (TULAA). Patients and Methods: We performed 231 TULAA on patients in the period from November 2001 to September 2007. We introduced an 11 mm Hasson's port using open technique; an operative channel with 10 mm telescope and an atraumatic grasper were used. After intra-abdominal laparoscopic dissection, the appendix was exteriorised through the umbilical access. The appendectomy was performed outside the abdomen as in the open procedure; the operation was completed using only one port in 227 patients (98%), two and three ports in two patients (1%) while conversion to open surgery was needed in two patients (1%). Results: The average operating time was 38 minutes and the median time to discharge was three days. Four (1, 7%) early postoperative complications (two suppuration of the umbilical wound) with no major complications were observed. Conclusions: Our results demonstrate that TULAA, which combines the advantages of both open and laparoscopic procedures, is a valid alternative form of treating uncomplicated appendicitis. If appendectomy cannot be completed with only one port, insertion of one or more ports may be necessary to safely conclude the procedure.

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Foregut duplication is commonly found in the posterior mediastinum. 10-20% of these anomalies are associated with oesophageal duplication. It can occur in all parts of oesophageal length. Although duplication of cervical oesophagus has been previously reported, but a majority of them were found in thoracic oesophagus. Infants with oesophageal duplication usually manifested by respiratory distress or asymptomatic thoracic mass, casually, detected in X-ray. A 7-month-old infant weighing about 7.5 kg, with the signboard of respiratory distress, fever and nutritional intolerance was admitted to our hospital. Physical examination showed dehydration, stridor, tachypnoea, intercostal retraction and neck stiffness. Plain chest radiogram showed dubious cystic mass in the distal neck. Cervical and chest computed tomography scan showed neck cystic lesion disseminated to posterior mediastinum, probably propagated to the respiratory system. Cystic lesion connected to oesophagus was partially resected, oesophagus was repaired and remaining mucous of a cyst was removed, then gastrostomy tube was applied. Although cervical oesophageal duplication cysts are rare, but they must be considered as one of the differential diagnoses of cervical mass with respiratory distress in infants.

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Persistent Mullerian duct syndrome is a rare form of internal male pseudohermaphroditism, in which Mullerian duct derivatives (uterus and fallopian tubes) are present in a genotypic (46XY) and phenotypic male. Over 150 cases have been reported, mainly from outside the African setting. This article presents an unexpected case encountered in an African setting. Handicaps in the management were unavailability of necessary diagnostic tools as well as lack of finance to assess those available. Although a diagnosis was eventually arrived at and the parents thoroughly counseled, the patient has not represented for definitive surgery.

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Background: The Care of cleft patients is very challenging. Team cleft care is usually lacking in many developing countries due to shortage of qualified manpower. This study is aimed at highlighting anaesthetic challenges in the management of cleft in children. Patients and Methods: This was a study of cleft lip and palate patients who were managed during team cleft care activities at University of Maiduguri Teaching Hospital and Federal Medical Centre Nguru both in north eastern Nigeria from January to June 2009. Results: One hundred and six cleft patients presented for surgical repair under general or local anaesthesia. Fifteen (14%) patients all of whom children were unfit for general anaesthesia due to various medical reasons. Ninety-one (86%) cleft patients comprising 53(50%) children and 38(36%) adults had cleft repair under halothane general endotracheal anaesthesia and local anaesthesia, respectively. There was no anaesthetic complications recorded under local anaesthesia. Fifteen percent of children who received general endotracheal anaesthesia suffered various anaesthetic complications which included hypoxia (3.8%), laryngospasm (1.9%), kinking of endotracheal tube (5.7%), inadvertent extubation (1.9%) and pulmonary aspiration (1.9%). There was no mortality or anaesthesia-related morbidity at the time of discharge in all the cases. Conclusion: We conclude that anaesthesia for cleft lip and palate repair in hospital based team-cleft care activities in our environment is relatively safe. We recommend general anaesthesia with controlled ventilation for children and local anaesthesia for adult and older children who can cooperate.

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Background: Acute appendicitis is an important differential diagnosis in children with pain in the right iliac fossa. Some patients have equivocal signs that make the diagnosis difficult. Many patients with suspected acute appendicitis are admitted for observation and finally discharged because they did not have appendicitis. We decided to design this study to investigate whether the Alvarado score could be used by emergency room doctors as a criterion for admission to hospital. Patients and Methods: This is a prospective study comprising 350 patients who attended the emergency department with suspected acute appendicitis in the period from May 2007 to April 2009. All patients were scored by Alvarado score in the emergency department before admission. The Alvarado score is based on three symptoms, three signs and two laboratory findings. The decision for admission and surgery was made independent of the score. The diagnosis of patients who underwent appendicectomy was confirmed by both operative findings and postoperative histopathology. Results: We studied the Alvarado scores of 350 patients who presented to the emergency department with pain in the right iliac fossa; their age ranged from 8 to 14 years; 182 patients (52%) operated with the aim to treat acute appendicitis, 168 patients (48%) were discharged without surgical intervention and advised to attend the out-patient clinic after 24 hours for re-evaluation. we have found that patients with a low Alvarado score (less than 6) did not have acute appendicitis. Conclusion: Patients with equivocal signs can present a diagnostic challenge and are very often admitted to the surgical department for observation. The Alvarado score can be used as a scoring system that help in taking the decision for admission of cases with suspected acute appendicitis especially by primary healthcare providers.

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A cleft sternum is a rare congenital anomaly often diagnosed as asymptomatic at birth. Clinical outcome may be unfavourable when an associated anomaly, particularly, an intra cardiac anomaly coexists with the defect. Primary repair should be employed in the neonatal period because the flexibility of the chest wall is maximal and thus the compression of underlying structures is minimal. However, patients with sternal cleft may even present late in the childhood or adolescence period. We herein report two cases of successful repair of sternal clefts with review of the available literature.

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Adrenal cystic lesions are rare and may be associated with both complete and incomplete Beckwith syndrome (BWS). Because the adrenal gland often houses malignant lesions, differentiation between benign and malignant lesions of the gland, although usually difficult, is very necessary from the point of view of management. Here we present our experience in a case of incomplete BWS with adrenal cystic lesion and review of the literature.

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Background: Congenital lobar emphysema (CLE) is characterised by over distension of one lobe and pressure on the adjacent lobe and mediastinum. In this study, we review the pathological results of our paediatric patients with CLE, highlighting the early and late complications that occurred in these patients. Patients and Methods: In a prospective study from 1996 to 2008, we evaluated 30 patients with CLE diagnosis. Variables collected included sex, age at the time of diagnosis, radiological diagnostic method, type of treatment, pathological analysis, surgical findings and early postoperative complications. Parents were asked to refer to our clinic for follow-up and evaluation of late complications. Results: Thirty patients and males accounted for majority of the study population (n = 20, 67%). The mean age of male and female patients (at admission) was 7.2 ± 2.3 and 4.7 ± 1.2 months respectively (P = not significant). The main diagnostic method was chest x-ray (CXR) in all patients. Abnormal bronchial cartilage was found in 71% of patients. The most affected lobe was left upper lobe (50%). Associated anomalies were seen in four patients. Early postoperative periodhadtwo cases of pneumothoraces. At six month follow up, five (25%) males and four females (40%) had delayed weight gain. Permanent oxygen dependency was seen in two patients. Twenty- six patients underwent thoracotomy. Mortality rate was 13%. Base deficit at the time of admission was greater in those patients who eventually died, (-8.6 ± 1.2 versus -3.1 ± 0.4) (P = 0.0003). There were two deaths in the bilobar involvement group and two in the unilobar involvement group (P = 0.07, near significant). Conclusion: This study confirms that the number of affected lobes and base deficit at the time of admission were associated with significantly increased mortality.

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Background: Childhood trauma has become a major cause of mortality and morbidity, disability and socio-economic burden and it is expected by the World Health Organization (WHO) that by 2020 it will be the number 1 disease globally. The WHO and UNICEF have published their third World Report on Child Injury Prevention in December 2008. Materials and Methods: A systematic review was performed on the history and magnitude of paediatric trauma worldwide. Additionally exciting developments and new trends were assessed and summarized. Results: Paediatric trauma is a growing field of clinical expertise. New developments include total body digital imaging of children presenting with polytrauma; targeted management of head injuries; conservative management of abdominal injuries in children and diagnostic laparoscopy, including the laparoscopic management of complications following the conservative management of solid organ injuries. Conclusion: Paediatric trauma has long been neglected by the medical profession. In order to deal with it appropriately, it makes sense to adopt the public health approach, requiring that we view child injuries similarly to any other disease or health problem. The greatest gain in our clinical practice with dealing with child injuries will result from a strong focus on primary (preventing the injury), secondary (dealing with the injury in the most efficient manner) as well as tertiary prevention (making sure that children treated for trauma will be appropriately reintegrated within our society). By actively promoting child safety we will not only achieve a most welcome reduction in medical cost and disability, but also the ever-so-much desired decline of avoidable childhood misery and suffering.

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Purpose: Penoscrotal transposition may be partial or complete, resulting in variable degrees of positional exchanges between the penis and the scrotum. Repairs of penoscrotal transposition rely on the creation of rotational flaps to mobilise the scrotum downwards or transpose the penis to a neo hole created in the skin of the mons-pubis. All known techniques result in complete circular incision around the root of the penis, resulting in severe and massive oedema of the penile skin, which delays correction of the associated hypospadias and increases the incidence of complications, as the skin vascularity and lymphatics are impaired by the designed incision. A new design to prevent this post-operative oedema, allowing early correction of the associated hypospadias and lowering the incidence of possible complications, had been used, whose results were compared with other methods of correction. Materials and Methods: Ten patients with incomplete penoscrotal transposition had been corrected by designing rotational flaps that push the scrotum back while the penile skin remains attached by small strip to the skin of the mons-pubis. Results : All patients showed an excellent cosmetic outcome. There was minimal post-operative oedema and no vascular compromise to the penile or scrotal skin. Correction of associated hypospadias can be performed in the same sitting or in another sitting, without or with minimal complications. Conclusion: This modification, which maintains the penile skin connected to the skin of the lower abdomen by a small strip of skin during correction of penoscrotal transposition, prevents post-operative oedema and improves healing with excellent cosmetic appearance, allows one-stage repair with minimal complications and reduce post-operative complications such as urinary fistula and flap necrosis.

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Objectives: During cleft palate repair, velopharyngeal sphincter reconstruction is still a challenge to plastic surgeons. To improve results of surgical treatment of cleft palate and secondary velopharyngeal incompetence, a carefully designed modified procedure for palatoplasty is presented. Materials and Methods: Thirty patients with incomplete cleft palate corrected by this procedure from April 2003 to October 2007 were included. A u-shaped incision was made in the anterior palate to separate bipedicle flap based on the greater palatine arteries of both sides. After complete dissection of the nasal and palatal mucosa, palatal muscles were carefully dissected from the posterior edge of the bones of the hard palate, wherein the well-mobilised flap receded backward spontaneously and elongated the soft palate. After suturing the nasal mucosa in the midline, the dissected palatal muscles of both sides were sutured together in the anterior third of the soft palate. On suturing the nasal mucosa and the palatal muscles, the soft palate became elongated and the oral mucosa was seen receding backward spontaneously to cover the anterior part of the soft palate. Results: All corrected patients showed good results, with no recurrence, no post-operative fistulae, with accepted speech pattern and no need for further pharyngoplasty. The line of the sutured nasal mucosa and the palatal muscles became covered by the healthy non-sutured oral mucosa and the soft palate became elongated, with narrowing of the vellopharyngeal isthmus after this technique. Conclusion: The designed flap allows covering of sutured nasal mucosa and palatal muscles with a healthy unsutured oral mucosa and elongates the soft palate. Thus, there was no incidence of post-operative fistula and no need for further pharyngoplasties.

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Despite of a number of techniques in the armentarium of the paediatric surgeon, the management of pancreatic pseudocysts remains a challenge. We report on a case of a 5-year-old child with a post-traumatic pancreatic pseudocyst who was successfully treated with endoscopic ultrasound-guided transgastric approach. Endoscopic ultrasound-guided transgastric drainage of a pancreatic pseudocyst is effective and safe in children.

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Blue rubber bleb naevus syndrome (BRBNS) is characterised by vascular malformations of the skin and gastrointestinal tract. We present the rare case of BRBNS in an African child. She presented with large-volume gastrointestinal bleeding and was managed by on-table colonoscopic identification and surgical excision, of all her enteric, vascular malformations.

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Bilateral foetal uropathy is the leading cause of chronic renal failure in childhood. Vesico-amniotic shunting (VAS) is a simple, feasible, and widely used procedure for decompressing the foetal urinary system. We report a case of a boy with bilateral foetal uropathy who underwent VAS at a gestational age of 29 weeks. Vesico-abdominal shunt dislodgement occurred and led to urinary ascites and anhydramnios. Postpartal laparotomy showed a shunt perforation between the urinary bladder and the peritoneal cavity.

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Background: To study the aetiology, management and outcome of biliary perforations in paediatric age group. Patients and Methods: In a retrospective study, the records of patients presented with biliary peritonitis due to biliary perforations, managed from March 2006 to July 2009, are reviewed. Results: Eight male patients with biliary peritonitis due to biliary perforation were managed. These patients were divided in two groups, A and B. Group A, (n = 3) patients, had common bile duct (CBD) perforation, and Group B (n=5) patients had gallbladder perforation. The presenting features were abdominal pain, fever, abdominal distension, vomiting, constipation, jaundice and signs of peritonism. The management of CBD perforations in Group A was by draining the site of perforation and biliary diversion (tube cholecystostomy). In Group B, the gallbladder perforations were managed by tube cholecystostomy in four patients and cholecystectomy in one patient, however, one patient had to be re-explored and cholecystectomy performed due to complete necrosis of gall bladder. There was no mortality in our series. All patients were asymptomatic on regular follow-up. Conclusion: Early optimal management of biliary perforations remarkably improved the very high mortality and morbidity that characterised this condition in the past.

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Female conjoined twins (thoraco-omphalopagus) were delivered by emergency caesarean section in a private nursing home. On examination, one of the twins was dead and was threatening the survival of the surviving twin (twin A). An emergency separation was performed to salvage the surviving twin.

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Spontaneous rupture of hepatocellular carcinoma (HCC) with intraperitoneal haemorrhage is a life-threatening complication with a high mortality rate. The mechanism of spontaneous rupture of HCC is unknown. It may be related to venous congestion, haemorrhage, central necrosis, or trauma. Patients with ruptured tumours confirmed on computerised tomography (CT) scan underwent immediate cardiovascular resuscitation. Depending on the stage of the tumour as seen on the CT scan and the condition of the patient, stoppage of bleeding was accomplished by transcutaneous hepatic artery embolisation, selective hepatic artery ligation, or hepatic resection. Only clinically stable, small tumours were resected as an emergency procedure. We report the case of a 12-year-old child admitted with acute right upper quadrant abdominal pain and signs of hypovolaemia. Ultrasonography revealed free peritoneal fluid and left liver haematoma was suspected. CT scan showed a tumour on the left side of the liver and free peritoneal fluid. Emergency laparotomy revealed haemoperitoneum and a 5-cm diameter left liver tumour which was ulcerated and haemorrhagic. The tumour was completely resected. Histopathological examination confirmed a diagnosis of rupture of differentiated HCC.

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Background: Colostomy site, which is a potentially contaminated wound, is traditionally closed with interrupted skin stitches and placement of intraperitoneal or parietal or both drains; often with poor cosmetic outcome in our country. This study aims at prospective evaluation of colostomy closure wounds by different techniques. Patients and Methods: This study was carried out in all infants and children with colostomy (for different pathologies) admitted for colostomy closure in our institute from August 1, 2006 to February 29, 2008. Patients were divided into two groups: Group A with colostomy closure without any drain and subcuticular skin closure and Group B with colostomy closure with both intraperitoneal and parietal drain and interrupted skin closure. Patient's details, including age, sex, body weight, diagnosis, preoperative bowel preparation, peroperative antibiotics, postoperative wound infection, anastomotic leaks, duration of hospitalisation and postoperative follow-up for wound assessment, were recorded. By the end of February 2008, 151 cases of colostomy closure were recorded, 70 in Group A and 81 in Group B. Results: Statistical analysis of the data showed no statistically significant difference in wound infection and anastomotic leak between the two groups. On postoperative follow-up, wound assessment showed significantly better cosmesis in the no drain subcuticular group. Conclusion: This study shows that closing colostomies without any drain and subcuticular skin closure does not result in any increased incidence of wound infection and has better cosmetic results.

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Background: Surgeries performed for gynaecological conditions in children and adolescents are not common in our environment. Adequate facilities and the skill to perform the required procedures may also be lacking. We were interested in reviewing the practice of paediatric gynaecological surgery in our facility. Patients and Methods: A retrospective study of case files and theatre records of children below the age of 16 years who had surgeries at the Jos University Teaching Hospital over a 10 year period was undertaken. Results: A total of 89 surgeries were performed in this age group during the period under review. Twenty-eight (33.4%) of the patients were below the age of 11. The most common surgical procedure was for the management of septic abortion (21.3%). Correction of congenital malformations of the genital tract accounted for 21.4% (19) of the surgeries performed. Fourteen (15.7%) laparotomies were performed for ovarian cysts. Conclusion: Though the number of surgeries performed on children for gynaecologic reasons may appear small, the skills required to manage them should be enhanced and the requisite facilities provided.

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Background: To analyse a tertiary care experience with various pancreatic problems in the paediatric age group and clarify the frequency of various pancreatic diseases in the same patient population as well as the role of surgical or conservative management in their treatment. Patients and Methods: All patients with clinical suspicion of disease pertaining to the pancreas, substantiated by laboratory investigations or imaging modalities over a period of five years are included in our study. Results: Of the total 228 patients with various pancreatic problems, who presented to us, mostly with acute pancreatitis and pancreatic trauma, 64 needed surgical interventions. The total mortality rate encountered was a meagre 3.5%. Conclusion: Paediatric pancreatic disorders are not so infrequent; and a high index of suspicion aided by the newer modalities of investigation greatly helps in the appropriate and timely management.

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