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Byline: G. Fusai (1), R. Steinberg (1), A. Prachalias (1), N. D. Heaton (1), L. Spitz (2), M. Rela (1) Keywords: Ex vivo; Extracorporeal; Ex situ; Pheochromocytoma; Bench surgery Abstract: A case of a 15-year-old girl with a malignant extraadrenal pheochromocytoma situated between the inferior vena cava and the portal triad is described. Using ex vivo technique, the tumour was successfully resected on the bench, as a previous attempt to remove the tumour in situ had been abandoned. The surgical aspects and implications of ex vivo surgery are discussed, highlighting the increased operative risk, perioperative mortality, and poor long-term results in patients with malignant tumours. Author Affiliation: (1) Institute of Liver Studies and Liver Transplant Surgical Service, King's College Hospital, Denmark Hill, SE5 9RS, London, UK (2) University Department of Paediatric Surgery, Great Ormond Street Hospital, London, UK Article History: Registration Date: 11/03/2005 Accepted Date: 16/07/2004 Online Date: 22/11/2005

Aims: To compare the long term functional and psychosocial outcomes following surgical treatment for total colonic aganglionosis (TCA) with those in an age and gender matched group of patients with [...]

Byline: E. Towu (1), G. Boxer (2), R. Begent (2), J. Zweit (3), L. Spitz (4), K. Hobbs (1), M. Winslet (1) Keywords: Keywords Lipiodol targeted radiotherapy; Hepatoblastoma cell line Abstract: Lipiodol, a stable iodine addition product of fatty ethyl esters derived from poppyseed oil, has been used as a vehicle for targeted cytotoxic or radiotherapeutic treatment in adults with unresectable hepatocellular carcinoma and in a few children with advanced hepatoblastoma (HB). Prolonged retention of lipiodol by the cancer cells might enable more effective targeted therapy to advanced, multifocal, or metastatic HB. To investigate the uptake and cytotoxic efficacy of beta and Auger electron-emitting radioconjugates on HB cells, monolayers of HB (C3a, Hep Tow1) and normal human hepatocyte cell lines were exposed to lipiodol.sup.131 (L131), lipiodol.sup.125 (L125), or a cocktail of both isotopes. Uptake of radioactivity was assessed autoradiographically using phosphoimages and the cytotoxicity assessed by trypan blue exclusion and clonogenic assay. The uptake of the different radioconjugates was identical in both HB cell lines. The `cocktail' of both radioactive lipiodols caused the greatest cytotoxicity to HB C3a, and HepTow1. L125 alone had a similar level of uptake and cytotoxicity as L131. Radioactive iodine alone did not show any cytotoxicity on any of the liver cell lines in culture for up to 72 h. These experimental results provide support for a clinical strategy of combinations (`cocktails') of radioconjugates in targeted radiotherapy in patients with HB. Author Affiliation: (1) University Department of Surgery, The Royal Free Hospital School of Medicine, London, UK, GB (2) Department of Clinical Oncology, The Royal Free Hospital School of Medicine, London, UK, GB (3) Cyclotron Unit, Hammersmith Hospital, London, UK, GB (4) Department of Paediatric Surgery, Great Ormond Street Hospital for Children, London, UK, GB Article note: Accepted: 22 September 2000

Byline: V. Baraldini (1), L. Spitz (1), A. Pierro (1) Keywords: Key words Randomised controlled trials; Paediatric surgery; Evidence-based medicine Abstract: It has been assumed that only 10% of medical interventions are supported by solid scientific evidence. The aim of this study was to determine the type of research evidence supporting operations in a tertiary referral paediatric surgical unit. All patients admitted over a 4-week period to two surgical firms were enrolled in the study. All major operations carried out on each patient since birth were evaluated. Patients for whom a diagnosis was not reached were excluded. A bibliographic database (MEDLINE) was used to search for the articles published between January 1986 and December 1995 on the analysed operations. The type of evidence supporting the operations was classified as follows: I=evidence from randomised controlled trials (RCTs) II=self-evident intervention (obvious effectiveness not requiring RCTs) III=evidence from prospective and/or comparative studies IV=evidence from follow-up studies and/or retrospective case series and V=intervention without substantial evidence for or against results of randomised trials. Seventy operations (32 individual types) were performed on 49 patients (1--5 operations/patient) 18 (26%) were supported by RCTs (type of evidence I). Two patients (3%) received a self-evident intervention (type II) 48 operations (68%) were based on non-randomised prospective or retrospective studies (type III=13% type IV=55%). Two patients (3%) received an operation not supported by or against convincing scientific evidence (type V). A significant proportion of operations in paediatric surgery is supported by RCTs. However, the vast majority of these trials were conducted on adult patients. Sixty-eight per cent of the operations were based on prospective follow-up studies or retrospective case series, which may not represent solid scientific evidence. More RCTs are needed in paediatric surgery. Author Affiliation: (1) Institute of Child Health and Great Ormond Street Hospital for Children, 30 Guilford Street, London WC1N 1EH, U.K., GB Article note: Accepted: 24 November 1997

Byline: G. Azzie (1), A. Bensoussan (1), L. Spitz (2) Keywords: Oesophageal leiomyomatosis; Anorectal leiomyomatosis; Alport's syndrome Abstract: Anorectal leiomyomatosis is exceedingly rare in children. Its association with the more common diffuse oesophageal leiomyomatosis has been documented in only two previous cases. This report describes the presentation, diagnosis and treatment of two further cases of diffuse oesophageal combined with anorectal leiomyomatosis. Author Affiliation: (1) Departement De Chirurgie Pediatrique, Hopital Sainte Justine, 3175 Cote Sainte Catherine, Montreal, Quebec, H3T 1C5, Canada (2) Department of Paediatric Surgery, Institute of Child Health, 30 Guilford Street, WC1 N 1EH, London , UK Article History: Accepted Date: 08/10/2002 Online Date: 05/08/2003

Byline: W. Cheng (1), A. E. Bishop (2), L. Spitz (3), J. M. Polak (2) Keywords: Key words Esophageal atresia; Gastroesophageal reflux; Adriamycin; Fetal rat Abstract: Gastroesophageal reflux is common in children after successful repair of esophageal atresia (EA), and may be related to a congenital neuronal abnormality of the esophagus. This study employed a fetal rat model of adriamycin-induced EA to investigate whether the innervation of the esophagus is abnormal in EA. The fetal rats were divided into four groups: (1) normal controls (2) a saline-injected controls (3) adriamycin administered but without the development of EA and (4) adriamycin-induced EA. The distal esophageal segments were immunostained with a general neural marker, protein gene product 9.5 (PGP). Immunoreactivity per cross-sectional area (/xsa) was measured with an image analyzer. The extent of the esophageal circumference encircled by PGP-stained nerve tissue was assessed. While there was no significant difference in PGP immunoreactivity/xsa between the groups, the near-complete ring of nerve tissue along the plane of the myenteric plexus was replaced by clusters of nerve tissue in the atretic group (normal vs EA, P=0.001, Mann-Whitney U test). The abnormal distribution of nerve tissue in the atretic esophagus may be contributing factor in the esophageal dysmotility seen in EA. Author Affiliation: (1) Division of Paediatric Surgery, Department of Surgery, The Hong Kong University Medical Centre, Queen Mary Hospital, Pok Fu Lam Road, Hong Kong, HK (2) Department of Histochemistry, Royal Postgraduate Medical School, Hammersmith Hospital, London, United Kingdom, GB (3) Paediatric Surgical Unit, Institute of Child Health and Department of Surgery, Great Ormond Street Hospital for Children, London, United Kingdom, GB

Byline: C. Kimber (1), A. Pierro (1), D. Drake (1), E. Kiely (1), L. Spitz (1) Keywords: Key words Hemisplenectomy; ; Epithelial cysts; Splenic cysts; ; Children Abstract: To analyse the clinical features and outcome following surgery of giant splenic cysts inaPSchildren, all patients presenting to a children's hospital over the years 1987--1996 were reviewed. Variables analysed included presenting symptoms, method of diagnosis, operative procedure, and complications. Six patients (aged 8--16 years) presented with abdominal pain or a large abdominal mass. Ultrasound examination demonstrated large (&gt 5cm) unilocular splenic cysts. Five of the six children underwent hemisplenectomy without complication or blood transfusion. The remaining patient had an infected cyst, for which total splenectomy was necessary because of major intraoperative haemorrhage. Hemisplenectomy is based on accurate dissection of the splenic hilum with clear knowledge of the intracapsular vascular anatomy of the spleen. Preoperative vaccination is recommended in the event that splenectomy is required. This retrospective review concludes that hemisplenectomy for splenic cysts is a safe procedure in children. Author Affiliation: (1) Department of General Surgery, Great Ormond Street Hospital for Children and Institute of Child Health, London, U.K., GB Article note: Accepted: 3 November 1997

A boy with a long history of dysphagia attributed to neurological impairment was shown to have a large oesophogeal foreign body. It had remained undetected for five years as a [...]

Byline: Majella McCullagh (1), A. S. Bhuller (2), A. Pierro (2), L. Spitz (2) Keywords: Key wordsaOesophageal duplication; Antenatal diagnosis Abstract: aDuplications of the alimentary tract can occur from mouth to anus, and often are undetected until they present with complications. We report duplication of the cervical oesophagus presenting as dyspnoea and stridor in a two year old. In retrospect, this had been detected on ultrasound antenatally, but no specific diagnosis had been made. We review the present literature to raise awareness of duplication as one cause of cysts identified antenatally in this region. Author Affiliation: (1) Children's Hospital, Lewisham, London SE13 6LH, UK, GB (2) Hospital for Sick Children, Great Ormond Street, London WC1N, UK, GB Article note: Accepted: 23 February 1999

Byline: P. Pushparani (1), L. Spitz (1), J. Somerville (2), M. R. de Leval (3) Keywords: Key words Infant; Lower gastrointestinal haemorrhage; Necrotising enterocolitis; colectomy Abstract: An infant developed massive lower gastrointestinal haemorrhage secondary to necrotising enterocolitis after cardiac surgery. The haemorrhage was only controlled following a colectomy. Author Affiliation: (1) Department of Paediatric Surgery, Institute of Child Health, 30 Guilford Street, London WCIN 1EH, United Kingdom, GB (2) Royal Bromptom National Heart and Lung Hospital, Sydney Street, London SW3 6NP, United Kingdom, GB (3) Cardiothoracic Unit, Great Ormond Street Hospital for Children, London WCIN 3JH, United Kingdom, GB Article note: Accepted: 10 August 1998

Byline: M. McCullagh (1), J. Harper (1), M. C. Pitt (1), D. Jones (1), L. Spitz (1) Keywords: Key words Proteus syndrome; Median-nerve compression Abstract: Proteus syndrome is a multi--organ disorder, a prime feature of which is localized gigantism, usually clinically obvious. Symptoms secondary to hypertrophy of nerves has not been previously recognized as a part of the syndrome. Author Affiliation: (1) Great Ormond Street Hospital for Children NHS Trust, Great Ormond Street, London WC1N 3JH, UK, GB Article note: Accepted: 16 May 1997

A 12 year old boy with Proteus syndrome (macrodactyly, plantar/palmar cerebriform hyperplasia, lipomata, verroucous naevi, macrocephaly, and/or vascular abnormalities) presented with gross abdominal distension and severe intractable constipation. Magnetic resonance [...]