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Anies Mahomed
Department of Paediatric Surgery
Royal Alexandra Children's Hoapital
Brighton, BN2 5BE
anies.mahomed@bsuh.nhs.uk

Journal articles

2007
 
DOI   
PMID 
Martin Tousek, Amy Hughes-Thomas, Anies Mahomed (2007)  Calcific parasite or surgical miscellany?   J Pediatr Surg 42: 1. 267-269 Jan  
Abstract: This article highlights the difficulties posed by initial radiological investigations that misleadingly interpreted a linear opacity in the ascending colon of a 2-year-old to be a complicating calcified Ascaris. Final surgical management revealed an unusual cause for the tram tracking effect so pathognomonic of Ascaris infestation. Of interest are the images of the abdominal x-ray, ultrasound scan, and barium study.
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Chan, Currie, Malik, Mahomed (2007)  Paediatric cholecystectomy: Shifting goalposts in the laparoscopic era.   Surg Endosc Jun  
Abstract: BACKGROUND: Laparoscopic cholecystectomy is the treatment of choice in symptomatic paediatric cholelithiasis. However, controversy exists about its role in asymptomatic cholelithiasis and biliary dyskinesia. We have reviewed the experiences of two UK paediatric centres with laparoscopic cholecystectomy over an 8.5 year period and critically evaluated the indications and outcomes of surgery. METHODS: Patients who underwent laparoscopic cholecystectomy by a single surgeon at the Royal Aberdeen and Royal Alexandra Hospitals between May 1996 to August 2003 and September 2003 to December 2005, respectively, were studied. Information was extracted from prospectively held databases and analysed. RESULTS: A total of 27 cholecystectomies were performed during the period of study. The mean age of patients was 11.7 years with a female preponderance. Symptomatic idiopathic cholelithiasis was the main indication for surgery (14). Cholecystectomy was also performed for haemolytic disease (3), acute recurrent pancreatitis of unknown cause (2), gallbladder trauma (1), and for asymptomatic calcific non-resolving stones (7). All patients were investigated with ultrasound scans with four patients undergoing magnetic resonance cholangiopancreatography (MRCP) for suspected common bile duct (CBD) stones. A standard four-port approach was used with the gallbladder extracted through the umbilical port. The mean operative time in the latter 13 cases was 105 minutes with a median postoperative stay of one day for the whole series. Histology revealed chronic cholecystitis in all but three cases. All patients were discharged after a six-month follow-up period. CONCLUSIONS: The advent of laparoscopy has resulted in an expansion of the traditional indications for cholecystectomy. MRCP is a useful investigation in selected children to exclude choledocholithiasis and avoid intraoperative cholangiography. There appears to be no clear correlation between histology and presenting symptoms. The natural history of asymptomatic gallstones in children is not known although a consensus is emerging to support cholecystectomy for all calcific non-resolving gallstones.
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A A Mahomed, C Hoare, F Welsh, C P Driver (2007)  A two-center experience with the exclusive use of laparoscopic transperitoneal nephrectomy for benign renal disease in children.   Surg Endosc 21: 9. 1532-1536 Sep  
Abstract: BACKGROUND: This study aimed to evaluate a two-center experience with pediatric transperitoneal laparoscopic nephrectomy, specifically focusing on the outcome parameters of operative time, complication, analgesic requirement, and postoperative stay. METHODS: This ambispective study was conducted over a 4-year period between May 2001 and May 2005 in two tertiary pediatric surgical centers. Data were prospectively recorded from an in-house expanded medical audit system (EMAS) and a Microsoft Excel database. Information on patient demographics, operative time, complications, analgesic requirement, and length of hospital stay were retrieved and analyzed. RESULTS: A total of 30 consecutive patients with a mean age of 4.43 years (range, 3 months to 15 years) underwent laparoscopic nephrectomy. All the patients underwent unilateral nephrectomy/nephroureterectomy for multidysplastic kidney (n = 12), reflux nephropathy (n = 13), pelvicoureteric junction obstruction (n = 4), or cystic disease of indeterminate cause (n = 1). The mean operative time was 93 +/- 30 min. The principal hemostatic devices used were the Harmonic Scalpel (20 cases), liga clips (5 cases), and hook diathermy and endoshears exclusively (4 cases). There were no conversions, but the intraoperative complications of bleeding (n = 2), difficult location (n = 1), difficult extraction (n = 1), and requirement for a liver retractor (n = 2) were encountered. An additional five patients had problems in the immediate postoperative period, two of whom went on to have long term difficulties with recurrent urinary tract infections resulting from a residual ureteric stump, which required surgery. Nearly one-third of the patients required morphine for analgesia in the immediate postoperative period, with the figure falling to 20% by day 1. The median postoperative hospital stay was 1 day (range, 0-16 days). At this writing, all the patients remain under surveillance with a mean follow-up period of 2.88 years, and no patients have experienced complications secondary to intraabdominal adhesions. CONCLUSION: Transperitoneal laparoscopic nephrectomy is technically feasible in most cases of benign renal disease. The intraoperative complications are minimal, and recovery for most is robust. Two-thirds of the patients are discharged within 24 h. In this study, narcotic analgesics were prescribed in about a one-third of all the cases for a limited period. Further problems may be seen when refluxing ureters are incompletely excised. However, the transperitoneal approach does not mitigate against complete excision because the exposure to the pelvis is adequate. At the midterm follow-up assessment, adhesive obstruction was not encountered, confirming this approach as a tenable alternative to other laparoscopic approaches for nephrectomy.
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PMID 
Anies A Mahomed, Vanessa McLean (2007)  Cost analysis of minimally invasive surgery in a pediatric setting.   J Laparoendosc Adv Surg Tech A 17: 3. 375-379 Jun  
Abstract: AIMS: The aims of this study was to determine whether an active policy of cost curtailment would impact on the theater cost of laparoscopic surgery in a pediatric setting; to document the extent of cost changes over time and to identify factors that adversely influence expenditure; and to investigate whether the surgeon is a significant factor in the price of the procedure. MATERIALS AND METHODS: A prospective audit of laparoscopic procedures was performed in a single unit over a 36-month period. Detailed costs of theater inventory for all procedures were compiled on a case-by-case basis and recorded on a database. The cost of six index procedures were collated and changes over the period of the study analyzed. The factors responsible for increased expenditure were flagged and appraised to enable the implementation of cost-saving measures. The prices of the laparoscopic equipment were based on invoiced figures provided by hospital managers, and no long-term outcome measures were taken into account. RESULTS: A total of 179 cases were performed by six surgeons over a 3-year period between January 1, 2003 and December 31, 2005, with no adverse intraoperative events. The procedures studied in further detail were appendicectomy (n = 50), fundoplication (n = 25), cholecystectomy (n = 12), nephrectomy (n = 10), Fowler Stevens for undescended testes (n = 10), and modified Palomo operations for varicocoele (n = 7). The mean cost of these procedures fell year by year over the period of study but was significant only in appendicectomy (P = 0.017). For this procedure, there was a significant difference in costs between the various surgeons (P = 0.007), but this trend was not noted with the other procedures. There were no major intraoperative events, although 2 patients required conversion owing to technical difficulties posed by the cases. Among the factors that influenced costs were the use of disposables, particularly for hemostasis and suctioning, and an inability to procure reuseable instruments. CONCLUSIONS: The costs of commonly performed laparoscopic procedures are falling year by year. The surgeon is a factor in the costs of some procedures. A cost-saving strategy has not been compromised of patient safety; however, some cost-saving measures, though attractive, are labor intensive and are not practical. An overall commitment to the sensible use of health care resources translates into savings for hospitals, thereby strengthening the case for laparoscopic surgery.
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2006
 
DOI   
PMID 
Ross J Craigie, Neil Forrest, Shay Nanthakumaran, Anies A Mahomed (2006)  Laparoscopy in diagnosis and management of Meckel's diverticulum.   J Laparoendosc Adv Surg Tech A 16: 1. 70-73 Feb  
Abstract: OBJECTIVE: The aim of this paper is to demonstrate that laparoscopy can successfully diagnose otherwise elusive Meckel's diverticuli and at the same time offer a therapeutic access for successful resection. MATERIALS AND METHODS: Two patients with anemia of unknown cause and a further patient with acute small bowel obstruction underwent diagnostic laparoscopy. A Meckel's diverticulum was identified in each case and successfully resected through a minilaparotomy. RESULTS: All three patients were successfully investigated and treated with laparoscopy with no morbidity or mortality. CONCLUSION: Laparoscopy is a safe and effective surgical modality for diagnosis of atypically presenting Meckel's diverticulum and has a therapeutic role that results in an excellent cosmetic result.
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Anies A Mahomed, Linga Panchalingum, Yiannis Nikolopoulos (2006)  The squeeze technique to assist transumbilical delivery of pyloric tumor.   J Pediatr Surg 41: 8. 1492-1494 Aug  
Abstract: BACKGROUND/PURPOSE: The popularity of the transumbilical approach for the treatment of infantile hypertrophic pyloric stenosis has increased over recent years mainly because of its superior cosmetic result. However, delivering a large pyloric tumor through a small incision can be technically demanding and is associated with significant complications. Described in this article is a novel yet simple technique to facilitate delivery of the pyloric tumor. METHOD: This is a prospective report of an 8-year experience of a single surgeon on consecutive patients with pyloric stenosis who underwent transumbilical pyloromyotomy assisted by the squeeze technique. Information on patient demographics, perioperative performance, and postoperative stay were recorded and analyzed. A standard surgical approach and postoperative feeding regimen were used in all cases. RESULTS: Forty-six patients were operated on at a mean of 5.5 weeks and discharged 2.5 days postsurgery. There were no significant intra- or postoperative complications, and all patients were reviewed at 6 weeks postdischarge. CONCLUSION: The success of the squeeze technique in facilitating delivery of the pylorus strengthens the case for the continued use of the transumbilical over traditional and laparoscopic approaches for infantile hypertrophic pyloric stenosis.
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Mairi Macarthur, Anies Mahomed (2006)  Rare association of female pseudohermaphroditism, phallic urethra, and posterior cloaca.   J Pediatr Surg 41: 3. 576-579 Mar  
Abstract: We describe a child with the rare clinical entity of female pseudohermaphroditism, accessory phallic urethra, and posterior cloaca who was successfully treated with posterior sagittal anorectovaginourethroplasty. Masculinization was limited to the external genitalia, and no chromosomal, metabolic, or adrenal abnormalities were detected. Associated pathology included bilateral vesicoureteric reflux, a non functioning dysplastic kidney, and bicornuate uterus. The investigation and surgical management of this particularly challenging combination of anomalies is detailed.
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2004
 
PMID 
R K Mackenzie, G G Youngson, A A Mahomed (2004)  Laparoscopic decapsulation of congenital splenic cysts: a step forward in splenic preservation.   J Pediatr Surg 39: 1. 88-90 Jan  
Abstract: PURPOSE: The aim of this study was to evaluate the role of laparoscopic decapsulation in the management of congenital splenic cysts in children. METHODS: Patients who presented over the last decade with congenital splenic cysts and were treated with laparoscopic decapsulation were reviewed retrospectively. The authors performed 4 procedures in 3 patients aged 10, 11 (and later 13), and 13 years. In all cases there was progressive enlargement of a single cyst, which lay in the upper pole of the spleen in 3 instances and in the lower pole in the fourth. Pneumoperitoneum was induced using the Veress needle technique, and 3 or 4 ports were utilized. Cysts were aspirated initially before marsupialization with excision of around two thirds of the cyst wall. Both endoshears and the harmonic scalpel were used with good hemostatic effect. RESULTS: All patients had a good outcome with cyst resolution on long-term follow-up using serial ultrasound scanning. The first patient (in 1993) remained 3 days as an in patient postoperatively, and the others stayed overnight only. One patient had a new cyst near the first 30 months after the initial procedure. This again was dealt with laparoscopically, with patient discharge the following day. There were no complications in the short or long term. Histology in all cases confirmed a simple epithelial cyst. Median follow-up was 2 years (range, 6 months to 8 years). CONCLUSIONS: Our experience shows that laparoscopic decapsulation is an effective means of managing congenital splenic cysts and that both harmonic scalpel and endoshears are satisfactory dissecting instruments. Extensive marsupialization is probably unnecessary in these larger cysts, because long-term follow-up has shown no increased risk of recurrence after leaving a significant proportion of the cyst wall behind. This approach helps avoid major surgery in these cases.
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2003
 
PMID 
G G Youngson, C P Driver, A A Mahomed, V A Duddalwar (2003)  Computed tomographic cholangiography in the diagnosis of bile duct injury in children.   J Pediatr Surg 38: 11. E18-E20 Nov  
Abstract: A case of avulsion of the extrahepatic bile duct from the duodenum is described in association with severe liver trauma in a child. The value of computed tomographic (CT) cholangiography and laparoscopy in the diagnosis of this rare condition is outlined, and a paradigm for management is described.
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A A Mahomed, R N MacKenzie, L S Carson, J A Jibril (2003)  Enterobius vermicularis and perianal sepsis in children.   Pediatr Surg Int 19: 11. 740-741 Dec  
Abstract: Specific aetiological factors are responsible for a significant proportion of cases of perianal sepsis in children. A rarely implicated pathogen is Enterobius vermicularis, an obligate parasite with a ubiquitous presence in children. The authors describe two unequivocal instances of threadworm involvement in perianal sepsis, suggesting a pathophysiological basis.
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2002
 
PMID 
R K Mackenzie, G G Youngson, J K Hussey, A A Mahomed (2002)  Is there a role for balloon dilatation of pelvi-ureteric obstruction in children?   J Pediatr Surg 37: 6. 893-896 Jun  
Abstract: PURPOSE: The aim of this study was to examine the effectiveness of balloon dilatation with double J stenting in the management of children with pelvi-ureteric junction (PUJ) obstruction. METHODS: A retrospective study of a 4-year experience of PUJ balloon dilatation in children was conducted in a single unit. Information was collected on patient demographics, clinical presentation, the procedure of balloon dilatation, renal function, and antero-posterior renal pelvis diameter both pre- and postoperatively. RESULTS: There were 10 patients with a median age of 12.5 years (range, 21 months to 15 years). Four cases presented with ipsilateral flank pain, 3 with urinary tract infection, and 3 were discovered incidentally. Retrograde dilation was performed in all cases. Dilation was considered an initial success in 8 of 10 cases, and 1 patient deteriorated after 17 months. Double J stents were placed in all cases and remained in situ for a median of 7.5 weeks (range, 7 to 8.5). Median in-hospital stay was 2 days. Median follow-up was 34 months (range, 7 to 48). Ipsilateral renal excretion times (on MAG III isotope scanning) improved markedly from a median of 84 minutes (range 13 to 200) preoperatively to 7 minutes (range, 1 to 200) at 3 months and 7 minutes (range 1 to 113) at 12 months postoperatively. Median pelvic AP diameter reduced from 30 mm (range 22 to 74) to 16 mm (range, 8 to 36) at 2 months and 10 mm (range, 4 to 41) at 7 months. There were 2 immediate failed procedures, both occurred in patients who went on to open pyeloplasty and were found to have aberrant renal vessels causing extrinsic PUJ compression. One child deteriorated between 10 and 17 months and went on to open pyeloplasty. Twenty-two patients underwent open pyeloplasty in the unit during the same time period with a 95% success rate. Median stay was longer at 5 days and median age younger at 22 months (range, 6 weeks to 13 years). CONCLUSION: Balloon dilatation is a viable option in the management of PUJ obstruction, carrying minimal morbidity in experienced hands.
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DOI   
PMID 
A J McKinley, A A Mahomed (2002)  Laparoscopy in a case of pediatric blunt abdominal trauma.   Surg Endosc 16: 2. Feb  
Abstract: The use of laparoscopy in the assessment and treatment of blunt abdominal trauma has been reported for both adults and children. With increasing surgical experience and improvements in equipment, an expanding range of therapeutic interventions is available. The management of a 7-year-old boy who sustained blunt abdominal trauma during a fall from a bicycle is described. He was hemodynamically stable, but his abdomen remained tender, with guarding in the right upper quadrant. Serial ultrasound scans showed a thick-walled gallbladder and increasing intraabdominal fluid. Laparoscopy was used both as a diagnostic tool, and then as a therapeutic method. Diagnostic laparoscopy showed a ruptured gallbladder treated by laparoscopic cholecystectomy. A 3-cm grade 1 liver laceration also was identified, which required no intervention. Free bile was aspirated and the peritoneal cavity thoroughly lavaged. The child made a rapid postoperative recovery. The ultimate aim in the management of blunt trauma is to avoid unnecessary laparotomy while preventing undue delay in the detection of significant intraabdominal pathology. Computed tomography scanning, ultrasound scanning, and diagnostic peritoneal lavage all have strengths for detecting different injury types, but they also have a low specificity in determining the need for laparotomy. The advantages and limitations of laparoscopy in hemodynamically stable children who have sustained injuries from blunt trauma are discussed. The authors recommend laparoscopy in experienced hands as an additional tool for the optimal management of selected trauma cases.
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PMID 
A J Watson, A T Johnston, P M Barker, G G Youngson, W M Bisset, A A Mahomed (2002)  The presentation and management of juvenile-onset chronic inflammatory bowel disease in Northeastern Scotland.   J Pediatr Surg 37: 1. 83-86 Jan  
Abstract: PURPOSE: This study reviews the presentation and management of juvenile onset chronic inflammatory bowel disease and identifies changes in incidence of the disease over a 20-year period. METHODS: This was a retrospective study of all patients aged 16 and under with chronic inflammatory bowel disease diagnosed in 1 health region between 1980 and 1999. The patients were identified from computer records and the following variables studied: age, sex, mode of presentation, medical and surgical management, and length of follow-up. RESULTS: One hundred seven patients were identified: 77 with Crohn's disease and 30 with ulcerative colitis. The incidence of ulcerative colitis and Crohn's disease has risen from 0.7 in 100,000 and 2.2 in 100,000, respectively, in the years 1980 through 1989 to 1.5 in 100,000 and 4.4 in 100,000 in the period 1990 through 1999. The median age at presentation was 10.1 years for ulcerative colitis and 10.8 years for Crohn's disease. The majority of disease was diagnosed within 1 year of the onset of symptoms, which were principally abdominal pain, diarrhea, and rectal bleeding. The average length of follow-up was 6.9 years. Analysis of the surgical management of Crohn's patients has shown a low rate of surgical intervention. CONCLUSIONS: This study has shown an increasing incidence of chronic inflammatory bowel disease in the Grampian region of Scotland coupled with a low rate of surgical intervention in Crohn's disease. These findings could be the result of early referral and diagnosis, with the disease being documented earlier in its course or more aggressive preemptive medical therapy.
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2001
 
PMID 
A A Mahomed, P Bachoo, D King, G G Youngson (2001)  Recurrent cervical abscess: life-threatening presentation of chronic granulomatous disease in twin infants.   Pediatr Surg Int 17: 5-6. 478-480 Jul  
Abstract: Non-identical male twins presented at 12 and 13 weeks of life, respectively, with recurrent cervical abscesses and signs of imminent upper-airway obstruction (UAO). Despite adequate initial surgical drainage, airway patency became seriously jeopardised in both cases by recurrent abscess formation requiring further intervention. Chronic granulomatous disease was eventually diagnosed in both cases. The unusual presentation of this rare condition with life-threatening UAO in twin infants, a scenario not previously described, is reported and a management protocol suggested.
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PMID 
P Bachoo, A A Mahomed, G K Ninan, G G Youngson (2001)  Acute appendicitis: the continuing role for active observation.   Pediatr Surg Int 17: 2-3. 125-128 Mar  
Abstract: We present the results of a 6-year review of appendicitis. In the event of diagnostic doubt, a policy of active observation was instituted. This review endorses the validity of such a policy, indicating that it does not expose patients to increased morbidity. Data were collected prospectively over a 6-year period on 1,479 children admitted with suspected acute appendicitis (AA); 1,028 (69.5%) were discharged with a diagnosis of non-specific abdominal pain after a mean observation period of 2.5 days, whilst in the remaining 451 a clinical diagnosis of AA was confirmed. The male-to-female ratio was equal, with no difference in the mean age of males (11 years) or females (12 years); 95% of patients were over the age of 5 years. In 324 (72%) cases surgery was performed on the day of admission, whilst in the remaining 126 (28%) it was deferred for 1 to 6 days because the clinical diagnosis of AA remained doubtful. The mean hospital stay was 4 days (range 1-32). Analysis of the histological reports of all 451 cases confirmed a positive predictive value for clinical assessment alone of 97.9% and a normal appendicectomy rate of 2.6%. No mortality was observed; surgical morbidity was recorded at 6% with no correlation between postoperative morbidity and timing of surgery evident (Spearmans correlation coefficient = -0.079, p = 0.9). Active observation for suspected AA thus remains a valid technique for achieving an accurate diagnosis and successful outcome.
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2000
 
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A A Mahomed (2000)  Primary cricopharyngeal achalasia in infancy--myotomy treatment of choice.   S Afr J Surg 38: 2. 28-30 May  
Abstract: Dysphagia secondary to primary cricopharyngeal achalasia (PCA) is infrequently seen in paediatric patients. Two female children with PCA who had recurrent attacks of aspiration pneumonia are presented. A cricopharyngeal myotomy was successfully performed in both cases, confirming its efficacy. Investigation and management of this rare condition are reviewed.
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1998
 
PMID 
A Mahomed, G Youngson (1998)  Congenital lateral cervical cysts of infancy.   J Pediatr Surg 33: 9. 1413-1415 Sep  
Abstract: The etiology of lateral cervical cysts in infancy is complex. The cysts are congenital in origin and are derived from the third or fourth branchial apparatus. The authors describe two cases of congenital lateral cervical cysts with an internal opening in the pyriform sinus and possible origin in the third branchial arch. The embryology, clinical presentation, and operative management of this condition is reviewed.
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PMID 
A Mahomed, C Merry, E J Guiney (1998)  Splenic cysts--aspiration or partial splenic decapsulation?   S Afr J Surg 36: 3. 84-86 Aug  
Abstract: We report on 2 children who had partial splenic decapsulation for splenic cysts--a pseudocyst and a congenital epidermoid cyst. Ultrasound-guided percutaneous needle aspiration with drainage was attempted in both patients but resulted in rapid cyst reaccumulation. Used on its own in the management of splenic cysts, percutaneous drainage has limited therapeutic value and delays definitive surgery. Partial splenic decapsulation is a simple and safe procedure.
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PMID 
A A Mahomed, P Beale, P Puri (1998)  Mediastinal lipoma in children.   Pediatr Surg Int 13: 2-3. 218-219 Mar  
Abstract: Mediastinal masses are uncommonly encountered in childhood. These tumours are often interesting because of the varied mediastinal tissues from which they arise. Despite extensive investigations, a large percentage of these lesions remain undiagnosed prior to operation. We describe two rare cases of mediastinal lipoma. The first was incidentally diagnosed whereas the second patient presented with respiratory symptoms. The definitive diagnosis was established at surgery, which was curative.
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A Mahomed, A Jibril, G Youngson (1998)  Laparoscopic management of a large ovarian cyst in the neonate.   Surg Endosc 12: 10. 1272-1274 Oct  
Abstract: Laparotomy has become the preferred approach to the excision of large, complex abdominal cysts in the neonate. We describe a laparoscopic-assisted decapsulation of an antenatally diagnosed abdominal cyst that was noted on postnatal ultrasound scan to have a complex echo pattern. This limited procedure allows for accurate verification of the diagnosis, institution of appropriate therapy, and organ salvage. It represents a superior management option that obviates the significant complications associated with conservative management.
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PMID 
A A Mahomed, E J Stockdale, J Varghese, G G Youngson (1998)  Abdominoscrotal hydrocoeles: little place for conservatism.   Pediatr Surg Int 13: 2-3. 186-188 Mar  
Abstract: Abdominoscrotal hydrocoeles (ASH) are infrequently reported in children. The presence of bilateral ASH is even rarer, with fewer than five cases reported to date. ASH are present at birth and tend to be progressive in nature. Spontaneous resolution has not been documented. The pathogenesis is uncertain and their presence in the neonatal period suggests a preformed abnormality of the processus vaginalis. Diagnosis is suspected on clinical examination and confirmed by ultrasonography, which is a reliable diagnostic tool. Undescended testes frequently coexist. Early surgery is the preferred management, and complications are frequent if left unattended. Surgery is simply an extension of a hernia operation, without the need for a laparotomy.
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1997
 
PMID 
A Mahomed, G Youngson (1997)  Endoscopic restoration of esophageal continuity in caustic burn.   J Pediatr Surg 32: 12. 1747-1748 Dec  
Abstract: Total obliteration of the esophageal lumen after caustic ingestion is an uncommon event. Esophageal continuity was reestablished using endoscopic resection of scar tissue followed by serial dilatations.
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1996
 
PMID 
H Kobayashi, A Mahomed, P Puri (1996)  Intestinal neuronal dysplasia in twins.   J Pediatr Gastroenterol Nutr 22: 4. 398-401 May  
Abstract: Intestinal neuronal dysplasia (IND) has been described proximal to the aganglionic segment in Hirschsprung's disease and less frequently as an isolated condition. Familial occurrence of IND is rare. We report a case of monozygotic twins affected with IND. One-year-old male monozygotic twins presented with a history of chronic constipation and abdominal distension since birth. At presentation, both twins had fecal impaction with palpable fecal-filled loops of bowel. Barium enema in both patients showed gross fecal loading of the sigmoid colon and rectum. Delayed films taken at 48 h showed marked barium retention. Suction rectal biopsy specimens examined by acetylcholinesterase (AChE) histochemistry in both patients showed moderately increased AChE-positive nerve fibers in the lamina propria, muscularis mucosae, and around submucosal blood vessels, as well as hyperplasia of submucous plexus with formation of giant ganglia. Both children underwent extended internal sphincter myectomy. Six months later, both patients had normal bowel function. The occurrence of IND in twins suggests that genetic factors are involved in the etiology of this disease.
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