Mark D. Stringer

Choledochal cysts: lessons from a 20 year experience.

Cystic dilatation of the biliary tree is a rare congenital anomaly. To determine mode of presentation, diagnostic pitfalls, and long term outcome after surgery, 78 children (57 girls, 21 boys) with choledochal cyst treated between 1974 and 1994 were reviewed. Anatomical types were: Ic (n = 44), If (n = 28), IVa (n = 4), and V (n = 2); a common pancreaticobiliary channel was identified in 76% patients. Age at presentation ranged from 0-16 (median 2.2) years, six patients being diagnosed by prenatal ultrasonography. Of the 72 patients diagnosed postnatally, 50 (69%) presented with jaundice, associated with abdominal pain in 25 or a palpable mass in three, 13 (18%) presented with pain alone, and two (3%) with a palpable mass. The classic triad of jaundice, pain, and a right hypochondrial mass was present in only four (6%). Four children presented acutely after spontaneous perforation of a choledochal cyst, two presented with ascites and one cyst was discovered incidentally. Plasma and/or biliary amylase values were raised in 30 of 31 patients investigated for abdominal pain; seven had evidence of pancreatitis at operation. In 35 of 67 (52%) patients referred without previous surgery, symptoms had been present for more than one month, and in 14 of them for more than one year, before diagnosis. Delayed referral was due to misdiagnosis as hepatitis (n = 12), incomplete investigation of abdominal pain (n = 6), and failure to note the significance of ultrasonographic findings (n = 10). Two patients referred late died from liver failure. Of the 76 patients with type I or IV cysts, 59 underwent radical cyst excision and hepaticojejunostomy as a primary procedure and 10 as a secondary operation after previously unsuccessful surgery. Sixteen patients have been lost to follow up but most of the remainder are well after a mean period of 4.1 (0.1-13) years. Choledochal cysts are often misdiagnosed, but prognosis is excellent if radical excision is performed.

Randomized, double‐blind, placebo‐controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia

The objective of this study was to evaluate adjuvant corticosteroids after Kasai portoenterostomy for biliary atresia. The study consisted of a prospective, 2‐center, double‐blind, randomized, placebo‐controlled trial of post–Kasai portoenterostomy corticosteroids (oral prednisolone: 2 mg/kg/day from day 7 to day 21 and 1 mg/kg/day from day 22 to day 28). The data were compared with χ 2 or Mann‐Whitney tests, as appropriate. Seventy‐one postoperative infants with type 3 biliary atresia were randomized to receive either oral prednisolone (n = 36) or a placebo (n = 37). At 1 month, the median bilirubin level was lower in the steroid group (66 versus 92 μmol/L, P = 0.06), but no difference was evident at 6 (P = 0.56) or 12 (P = 0.3) months. The proportion of infants with a normal bilirubin level (<20 μmol/L) at 6 (47% versus 49%, P = 0.89) and 12 months (50% versus 40%, P = 0.35) was not significantly different. The need for transplantation by 6 (12% versus 13%, P = 0.99) and 12 months (26% versus 35%, P = 0.47) was not significantly different. The steroid effect was more pronounced in younger infants (less than 70 days at Kasai portoenterostomy, n = 51), with a reduced bilirubin level at 1 month (64 versus 117 μmol/L, P = 0.01) and with a greater proportion with a normal bilirubin level at 12 months (54% versus 37%, P = 0.22). Conclusion: There was a beneficial effect on the rate of reduction of bilirubin in the early postoperative period (specifically in infants less than 70 days old at surgery), but this steroid regimen did not reduce the need for liver transplantation. (HEPATOLOGY 2007;46:1821–1827.)

Endoscopic rectal sparing in children with untreated ulcerative colitis.

Background Ulcerative colitis (UC) typically is associated with a confluent proctitis, whereas rectal sparing may be seen in large bowel Crohn disease (CD). A few studies have reported rectal sparing in UC and suggested that this might indicate a more severe form of the disease. This study aimed to determine the prevalence and prognostic significance of rectal sparing in children with newly diagnosed, untreated UC. Methods The records of all children with untreated UC presenting to a regional pediatric gastroenterology service between January 1996 and December 2001 were retrospectively reviewed. Patients were divided into two groups according to the endoscopic appearance of the rectum: Group 1 (proctitis) and Group 2 (rectal sparing). Clinical features, intractability index (duration of active disease as a proportion of length of follow-up), response to treatment, relapse index (number of recurrences per year), and the need for surgery were compared. Results Thirty children with untreated UC were identified. Seven (23%) had rectal sparing at initial endoscopy, but disease distribution was otherwise similar in both groups. Presenting symptoms were similar in those with and without rectal sparing. In Group 1, 20 (87%) children achieved remission with initial medical treatment, compared with 3 (43%) in Group 2 (P < 0.05). The intractability index was higher in children with rectal sparing, but the difference was not statistically significant (P = 0.22). During a median follow-up period of 2 years, one (4%) child in Group 1 and two (29%) children in Group 2 experienced primary sclerosing cholangitis, and two (29%) children with rectal sparing required colectomy, compared with none in Group 1. Conclusions Endoscopic rectal sparing was seen in 23% of children with newly diagnosed, untreated UC, but this feature did not correlate with presenting symptoms. However, the presence of rectal sparing may indicate more aggressive disease that is less responsive to medical treatment.

Biliary amylase and congenital choledochal dilatation

The relationship between levels of biliary amylase measured at operation and clinical features was studied in a series of 55 children with congenital biliary dilatation (choledochal cyst) who presented between 1976 and 1993. There were 36 cystic and 19 fusiforms dilatations in the series. The most common modes of presentation were painless jaundice (n = 23) and pancreatitis (n = 22). Five infants presented with abnormal antenatal ultrasound examinations. Children with pancreatitis were older than those with painless jaundice (4.2 versus 1.5 years; P = .005), and a higher proportion had raised levels of biliary amylase (100% versus 44%; P < .0001). There was no difference in the age at presentation (P = .32), clinical mode of presentation (P = .3), or the level of biliary amylase (P = .25) between cystic and fusiform dilatations. A correlation was found between age at surgery and biliary amylase in the cystic (rs = 0.55; P = .001) but not in the fusiform group (P = .22). All infants with antenatal diagnoses were cystic dilatations. Choledochal cystic dilatations that were diagnosed antenatally did not have significant amylase reflux, suggesting that the aetiology of this subgroup is truly congenital. Children who present at a later age with pancreatitis invariably have high levels of biliary amylase, which is presumed to occur because of a common channel and reflux of biliary and pancreatic secretions.

Prophylaxis of venous thromboembolism

The objective of prophylaxis in venous thromboembolism is, first, to prevent fatal pulmonary embolism and, second, to reduce the morbidity associated with deep vein thrombosis (DVT) and the postphlebitic limb. This should now be standard practice for most patients over 40 years of age undergoing major surgery and for younger patients with a history of venous thromboembolism. Particularly high-risk groups include patients over 60 years of age undergoing major surgery, those with malignancy, and those requiring hip operations. Low-dose subcutaneous heparin 5,000 IU commencing 2 hours preoperatively and continuing 12 hourly until the patient is fully mobile is unequivocally effective in preventing DVT in medical and surgical patients and, most importantly, significantly reduces the incidence of fatal postoperative pulmonary embolism and total mortality. Such prophylaxis, in the presence of established DVT, also limits proximal clot propagation, which is the precursor of major pulmonary embolism. Low-dose heparin prophylaxis is associated with a small risk of bleeding complications, evidenced mostly by an increased frequency of wound hematoma rather than major clinical hemorrhage. Low molecular weight heparin fragments (e.g., Fragmin®, Choay®, Enoxaparine®) are emerging as useful alternative agents, having the advantage of once daily administration and yet providing similar efficacy in the prevention of DVT. Mechanical methods of prevention which counteract venous stasis, such as graduated elastic compression stockings, are also useful in protecting against DVT but have not been shown to prevent fatal postoperative pulmonary embolism. They are recommended particularly for patients in whom heparin prophylaxis is best avoided (e.g., neurosurgery) and possibly in combination with heparin in very high-risk patients. Le but de la prophylaxie en matière de thrombose et embolie est premièrement de prévenir lembolie pulmonaire mortelle et deuxièmement de réduire la morbidité associée à la thrombose veineuse profonde (TVP) et aux troubles postphlébitiques. La prophylaxie thromboembolique devrait être systématique chez les patients âgés de plus de 40 ans, devant subir une intervention chirurgicale majeure, une intervention sur la hanche ou ceux qui ont un cancer. Lhéparine à faibles doses (5,000 UI) par voie sous cutanée, commencée 2 heures avant lintervention et continuée toutes les 12 heures jusquà ce que le patient soit parfaitement ambulant, est efficace dans la prévention de TVP chez les patients médicaux et chirurgicaux, et elle réduit de façon significative lincidence de lembolie pulmonaire postopératoire fatale ainsi que la mortalité globale. Cette prophylaxie, même lorsque la TVP est établie, limite lextension proximale de la thrombose, souvent phénomène précurseur de lembolie pulmonaire majeure. Les complications hémorragiques dues à la prophylaxie par lhéparine à faibles doses sont peu importantes: essentiellement augmentation de la fréquence dhématome pariétal plutôt que syndrome hémorragique clinique. On emploie de plus en plus actuellement des fragments héparinés à faible poids moléculaire. Ils présentent lavantage dune seule injection par jour pour une même efficacité dans la prévention des TVP. Les méthodes mécaniques de prévention contre la stase veineuse, telles que le bas élastique de compression, sont également utiles dans la prévention de TVP mais il na pas été démontré quils prévenaient lembolie pulmonaire fatale. On recommande surtout chez le patient qui ne doit pas recevoir dhéparinothérapie (par exemple après la neurochirurgie), et on les combine parfois avec lhéparinothérapie chez le patient à très haut risque. El objetivo de la profilaxis del tromboembolismo venoso es, en primer lugar, la prevención de la embolia pulmonar fatal, y en segundo reducir la morbilidad asociada con trombosis venosa profunda (TVP) y el síndrome postflebítico en la enfermedad afectada. La profilaxis debe constituir una práctica estándar en la mayoría de los pacientes mayores de 40 años que sean sometidos a cirugía mayor y en pacientes jóvenes con historia de tromboembolismo venoso. Grupos de particular alto riesgo incluyen los pacientes mayores de 60 años sometidos a cirugía mayor, aquellos con neoplasias malignas, o quienes requieren operaciones de cadera. La heparina de baja dosis admnistrada por vía subcutánea, 5,000 UI, comenzando 2 horas antes de la operación y continuada cada 12 horas hasta que el paciente esté totalmente ambulatorio, es inequívocamente efectiva para prevenir TVP en pacientes médicos y quirúrgicos y, lo más importante, reduce en forma significativa la incidencia de embolismos postoperatorios fatales y la tasa total de mortalidad. Esta profilaxis, en presencia de TVP establecida también limita la propagación proximal del coágulo, fenómeno precursor de una embolia pulmonar mayor. La profilaxis con heparina de baja dosis aparece asociada con un bajo riesgo de complicaciones hemorrágicas, las cuales se evidencia generalmente por una aumentada frecuencia de hematomas de la herida más que por una hemorragia clínica. Los fragmentos químicos de bajo peso molecular de la heparina (por ejemplo, la Fragmina®, el Choay®, y la Enoxaparina®) están surgiendo como agentes alternativos utiles, con la ventaja de requerir ser administrados sólo una vez al día, pero con la misma efectividad en cuanto a la prevención de la TVP. Los métodos mecánicos preventivos que hacen contraparte a la estasis venosa, tales como las medidas de comprensión gradual, son también de utilidad para la protección de la TVP pero no han demostrado prevenir la embolia psotoperatoria fatal. Son particularmente recomendables en pacientes en quienes la profilaxis con heparina debe ser evitada (por ejemplo en la neurocirugía) y posiblemente en combinación con heparina en pacientes de alto riesgo.The objective of prophylaxis in venous thromboembolism is, first, to prevent fatal pulmonary embolism and, second, to reduce the morbidity associated with deep vein thrombosis (DVT) and the postphlebitic limb. This should now be standard practice for most patients over 40 years of age undergoing major surgery and for younger patients with a history of venous thromboembolism. Particularly high-risk groups include patients over 60 years of age undergoing major surgery, those with malignancy, and those requiring hip operations.Low-dose subcutaneous heparin 5,000 IU commencing 2 hours preoperatively and continuing 12 hourly until the patient is fully mobile is unequivocally effective in preventing DVT in medical and surgical patients and, most importantly, significantly reduces the incidence of fatal postoperative pulmonary embolism and total mortality. Such prophylaxis, in the presence of established DVT, also limits proximal clot propagation, which is the precursor of major pulmonary embolism. Low-dose heparin prophylaxis is associated with a small risk of bleeding complications, evidenced mostly by an increased frequency of wound hematoma rather than major clinical hemorrhage.Low molecular weight heparin fragments (e.g., Fragmin®, Choay®, Enoxaparine®) are emerging as useful alternative agents, having the advantage of once daily administration and yet providing similar efficacy in the prevention of DVT. Mechanical methods of prevention which counteract venous stasis, such as graduated elastic compression stockings, are also useful in protecting against DVT but have not been shown to prevent fatal postoperative pulmonary embolism. They are recommended particularly for patients in whom heparin prophylaxis is best avoided (e.g., neurosurgery) and possibly in combination with heparin in very high-risk patients.RésuméLe but de la prophylaxie en matière de thrombose et embolie est premièrement de prévenir lembolie pulmonaire mortelle et deuxièmement de réduire la morbidité associée à la thrombose veineuse profonde (TVP) et aux troubles postphlébitiques. La prophylaxie thromboembolique devrait être systématique chez les patients âgés de plus de 40 ans, devant subir une intervention chirurgicale majeure, une intervention sur la hanche ou ceux qui ont un cancer.Lhéparine à faibles doses (5,000 UI) par voie sous cutanée, commencée 2 heures avant lintervention et continuée toutes les 12 heures jusquà ce que le patient soit parfaitement ambulant, est efficace dans la prévention de TVP chez les patients médicaux et chirurgicaux, et elle réduit de façon significative lincidence de lembolie pulmonaire postopératoire fatale ainsi que la mortalité globale. Cette prophylaxie, même lorsque la TVP est établie, limite lextension proximale de la thrombose, souvent phénomène précurseur de lembolie pulmonaire majeure. Les complications hémorragiques dues à la prophylaxie par lhéparine à faibles doses sont peu importantes: essentiellement augmentation de la fréquence dhématome pariétal plutôt que syndrome hémorragique clinique.On emploie de plus en plus actuellement des fragments héparinés à faible poids moléculaire. Ils présentent lavantage dune seule injection par jour pour une même efficacité dans la prévention des TVP. Les méthodes mécaniques de prévention contre la stase veineuse, telles que le bas élastique de compression, sont également utiles dans la prévention de TVP mais il na pas été démontré quils prévenaient lembolie pulmonaire fatale. On recommande surtout chez le patient qui ne doit pas recevoir dhéparinothérapie (par exemple après la neurochirurgie), et on les combine parfois avec lhéparinothérapie chez le patient à très haut risque.ResumenEl objetivo de la profilaxis del tromboembolismo venoso es, en primer lugar, la prevención de la embolia pulmonar fatal, y en segundo reducir la morbilidad asociada con trombosis venosa profunda (TVP) y el síndrome postflebítico en la enfermedad afectada. La profilaxis debe constituir una práctica estándar en la mayoría de los pacientes mayores de 40 años que sean sometidos a cirugía mayor y en pacientes jóvenes con historia de tromboembolismo venoso. Grupos de particular alto riesgo incluyen los pacientes mayores de 60 años sometidos a cirugía mayor, aquellos con neoplasias malignas, o quienes requieren operaciones de cadera.La heparina de baja dosis admnistrada por vía subcutánea, 5,000 UI, comenzando 2 horas antes de la operación y continuada cada 12 horas hasta que el paciente esté totalmente ambulatorio, es inequívocamente efectiva para prevenir TVP en pacientes médicos y quirúrgicos y, lo más importante, reduce en forma significativa la incidencia de embolismos postoperatorios fatales y la tasa total de mortalidad. Esta profilaxis, en presencia de TVP establecida también limita la propagación proximal del coágulo, fenómeno precursor de una embolia pulmonar mayor. La profilaxis con heparina de baja dosis aparece asociada con un bajo riesgo de complicaciones hemorrágicas, las cuales se evidencia generalmente por una aumentada frecuencia de hematomas de la herida más que por una hemorragia clínica.Los fragmentos químicos de bajo peso molecular de la heparina (por ejemplo, la Fragmina®, el Choay®, y la Enoxaparina®) están surgiendo como agentes alternativos utiles, con la ventaja de requerir ser administrados sólo una vez al día, pero con la misma efectividad en cuanto a la prevención de la TVP. Los métodos mecánicos preventivos que hacen contraparte a la estasis venosa, tales como las medidas de comprensión gradual, son también de utilidad para la protección de la TVP pero no han demostrado prevenir la embolia psotoperatoria fatal. Son particularmente recomendables en pacientes en quienes la profilaxis con heparina debe ser evitada (por ejemplo en la neurocirugía) y posiblemente en combinación con heparina en pacientes de alto riesgo.

Randomised trial of two pharmacological methods of bowel preparation for day case colonoscopy

AIMS To undertake a prospective, single blind, randomised trial comparing the efficacy and tolerance of two outpatient colonoscopy bowel preparation regimens. METHODS Patients aged between 18 months and 16 years being admitted for day case colonoscopy were allocated randomly to receive either Picolax (an oral, sugar free powder containing sodium picosulphate 10u2009mg/sachet with magnesium citrate) and clear fluids or bisacodyl tablets with an unrestricted diet and a phosphate enema just before colonoscopy. Patient compliance, bowel frequency, and associated symptoms were recorded, and the adequacy of the bowel preparation was assessed in a blinded manner. RESULTS 63 of 66 patients completed the trial. Mean age, mean weight, extent of colonoscopy, and distribution of underlying pathology were similar in both groups. Bowel preparation was good or excellent in all of the patients in the Picolax group (nu2009=u200932) compared with 22 patients in the bisacodyl/phosphate enema group (nu2009=u200931). The latter group experienced more abdominal discomfort during bowel preparation but three of the Picolax group vomited and the lack of solid food distressed some children. CONCLUSIONS All bowel preparation methods have limitations and unpleasant side effects but the use of Picolax and clear fluids proved superior to bisacodyl tablets and a phosphate enema in children undergoing day case colonoscopy.

Fixed combinations of low-molecular weight or unfractionated heparin plus dihydroergotamine in the prevention of postoperative deep vein thrombosis

A prospective, double-blind, randomized, controlled clinical trial compared the efficacy and safety of fixed combinations of low-molecular weight heparin or standard unfractionated heparin plus dihydroergotamine mesylate in the prevention of deep vein thrombosis in high-risk patients undergoing elective major abdominal surgery. Two hundred patients, with a mean age of 66.6 years and almost half with malignancy, were allocated to receive either 5,000 IU unfractionated heparin plus 0.5 mg dihydroergotamine mesylate twice daily or 1,500 IU low-molecular weight heparin plus 0.5 mg dihydroergotamine mesylate once daily together with one placebo injection per day. Treatment was commenced 2 hours preoperatively and continued for at least 7 days. The incidence of deep vein thrombosis, determined by radiolabelled fibrinogen uptake and phlebography, was 11 percent in the unfractionated heparin plus dihydroergotamine mesylate group and 11.4 percent in the low-molecular weight heparin and dihydroergotamine mesylate group. Neither these figures nor those for major proximal thrombi proved significantly different. Of the four parameters used to assess hemorrhagic complications, only the decrease in postoperative hemoglobin levels in the low-molecular weight and dihydroergotamine mesylate group reached statistical significance. These results indicate that once-daily prophylaxis with a combination of low-molecular weight heparin and dihydroergotamine is safe, effective, and convenient in high-risk patients undergoing major abdominal surgery.

Gemfibrozil in hyperlipidaemic patients with peripheral arterial disease: some undiscovered actions

The effects of gemfibrozil were assessed in 27 hyperlipidaemic patients with stable peripheral arterial occlusive disease. Gemfibrozil (600 mg twice daily) was administered for 12 weeks after 2 weeks of placebo medication, thus enabling patients to act as their own controls. Serum cholesterol levels were reduced by a mean of 11.3%, triglycerides by 42.3% and low density lipoprotein cholesterol by 19.9%. Small but significant increases in HDL3 and apolipoprotein A-II also occurred. New findings included significant reductions in plasma lipid peroxides and Factor VIIc levels and a mean increase of 19% in antithrombin III concentrations. Furthermore, plasma fibrinogen levels increased by a mean of 17.6%, a potentially adverse effect of gemfibrozil that has not been previously reported.

Hirschsprung'sdisease and allied disorders. 2nd edition.

Hirschsprungs disease and allied disorders. 2nd edition. Edited by Holschneider AM, Puri P. (Pp 503, hardback,

Survival and outcome after hepatic artery thrombosis complicating pediatric liver transplantation

125.00). Australia: Harwood Academic Publishers, 2000. ISBN 90 5702 263 X.nnIn 1887, Harald Hirschsprung published the first detailed description of congenital megacolon, but it was not until 1948 that the true nature of the disease was defined. The discoveries at that time included the finding of aganglionosis in the rectum, the demonstration that peristaltic activity in the colon was propagated only as far as the narrowed aganglionic segment of bowel, and the relief of obstructive symptoms by rectosigmoidectomy. The key histological feature in Hirschsprungs disease is the absence of ganglion cells, which …