Small bowel skip segment Hirschprung's disease presenting with perforated Meckel's diverticulum.

Skip segment Hirschprung's disease (SSHD) is an uncommon variant of Hirschprung's disease where normal intestine is interspersed proximally and distally by abnormal, aganglionated intestine. These segmental changes have no well-defined embryological explanation. We present a case of SSHD in the small bowel and concomitant perforated Meckel's diverticulum, with review of the literature relevant to SSHD.

Pharmacodynamic analysis of the interaction between tiagabine and midazolam with an allosteric model that incorporates signal transduction.

PURPOSE: The objective of this study was to characterize quantitatively the pharmacodynamic interaction between midazolam (MDL), an allosteric modulator of the gamma-aminobutyric acid subtype A (GABAA) receptor, and tiagabine (TGB), an inhibitor of synaptic GABA uptake. METHODS: The in vivo concentration-response relation of TGB was determined through pharmacokinetic/pharmacodynamic (PK/PD) modeling. Rats received a single intravenous dose of 10 mg/kg TGB in the absence and the presence of a steady-state plasma concentration of MDL. The EEG response in the 11.5- to 30-Hz frequency band was used as the pharmacodynamic end point. RESULTS: Infusion of MDL resulted in a mean steady-state plasma concentration of 66 +/- 3 ng/ml. A significant pharmacokinetic interaction with TGB was observed. MDL inhibited TGB clearance by 20 +/- 7 ml/min/kg from the original value of 89 +/- 6 ml/min/kg. However, no changes in plasma protein binding of both drugs were observed. The concentration-EEG relation of TGB was described by the sigmoid-Emax model. The pharmacodynamic parameter estimates of TGB were: Emax = 327 +/- 10 microV, EC50 = 392 +/- 20 ng/ml, and nH = 3.1 +/- 0.3. These values were not significantly different in the presence of MDL. Factors that may explain the lack of synergism were identified by a mechanism-based interaction model that separates the receptor activation from the signal-transduction process. High efficiency of signal transduction and the presence of a baseline response were shown to diminish the degree of synergism. CONCLUSIONS: We conclude that the in vivo pharmacodynamic interaction between MDL and TGB is additive rather than synergistic. This strongly suggests that allosteric modulation of the antiseizure activity of a GAT-1 inhibitor by a benzodiazepine does not offer a therapeutic advantage.

Gastrin, somatostatin and infantile hypertrophic pyloric stenosis.

UNLABELLED: Despite multiple and often contradictory research, no firm conclusions regarding the role of hypergastrinaemia in infantile hypertrophic pyloric stenosis (IHPS) have been established. Evaluation of somatostatin, the main physiological antagonist of gastrin, has not been assessed in previous studies. Long-term evaluation following pyloromyotomy suggests persistent abnormalities in gastrin and somatostatin in IHPS. The objective of this case-controlled study was to compare fasting serum gastrin and somatostatin levels in IHPS. Serum sample were collected from 39 children with IHPS at the time of pyloromyotomy and 20 age-matched controls with no evidence of gastrointestinal disease. Standard radioimmunoassay techniques were used to detect circulating levels of the hormones. A two-tailed t-test was used for statistical analysis. The levels of the two hormones (mean +/- SEM) revealed that there was no evidence of hypergastrinaemia in IHPS compared with controls (75.6 +/- 16.1 and 68.1 +/- 7.8 ng l(-1), respectively), but that the level of somatostatin was significantly elevated (38.9 +/- 6.4 and 30.5 +/- 5.8 ng l(-1), p = 0.016). An inverse trend in the gastrin/somatostatin levels could not be identified in IHPS. CONCLUSION: Somatostatin but not gastrin is raised in IHPS. Somatostatin is known to inhibit the actions of inhibitory neurotransmitters in the pylorus and may explain the development of pylorospasm, which is believed to be important in the development of pyloric tumours. These results do not agree with a previous long-term follow-up study, but reflect the hormonal imbalance at the time of pyloric hypertrophy.

Peritoneography in the assessment of peritoneal cerebrospinal fluid absorption potential for distal ventriculoperitoneal shunt catheter placement: technical case report.

OBJECTIVE AND IMPORTANCE: Distal ventriculoperitoneal shunt failure has been associated with absorption failure secondary to previous peritonitis. This assumption has caused surgeons to seek alternate sites for distal catheter placement. We propose that the absorptive potential of the peritoneal cavity should be assessed before that site is discounted for catheter placement. CLINICAL PRESENTATION: The case of a 14-month-old male patient is presented, demonstrating multiple ventriculoperitoneal shunt placement procedures and a diagnostic dilemma with respect to distal shunt placement. Peritoneography was performed to demonstrate peritoneal fluid absorption, allowing subsequent placement of a new distal shunt catheter with good clinical results. TECHNIQUE: Using aseptic technique, a 24-gauge spinal needle was inserted in the midline of the abdomen and water-soluble contrast material was instilled. Delayed radiographs delineated peritoneal adhesions and demonstrated renal excretion of the contrast material, confirming peritoneal absorption. CONCLUSION: The peritoneal cavity remains the site of choice for distal shunt catheter placement. If failure of peritoneal cerebrospinal fluid absorption is suspected as a cause of shunt failure, then peritoneography with water-soluble contrast material may be safely used to demonstrate the adequacy of fluid absorption before a secondary site is chosen.

Laparoscopic fundoplication in children--an audit of fifty cases.

UNLABELLED: Critical evaluation of new laparoscopic procedures in childhood are essential. The aim of this study was to audit fifty laparoscopic fundoplications in children. METHOD: Evaluation of the financial implications, hospital stay, analgesia requirements, operative morbidity and symptom control was undertaken. RESULTS: 50 laparoscopic fundoplications were performed on children (6 months to 13 years) with a median follow-up period of 31.8 months. The conversion rate to an open procedure was 8%. The median length of opiate requirement for opiate analgesia was 1 day (range 1-5), post-operative stay 2 days (range 2-15). The operative morbidity was 8% (respiratory infection, pneumothorax, two patients, oesophageal perforation one patient). The recurrences rate was 6%. Whilst a prospective randomised trial is essential to satisfy the requirements of evidence-based medicine, the results of our review of laparoscopic fundoplication are encouraging.

Xanthogranulomatous pyelonephritis in childhood.

BACKGROUND: Xanthogranulomatous pyelonephritis is a severe, atypical form of chronic renal parenchymal infection accounting for 6/1000 surgically proved cases of chronic pyelonephritis. Its manifestations mimic those of neoplastic and other inflammatory renal parenchymal diseases and, consequently, it is often misdiagnosed preoperatively. AIM: To examine the relation between clinical history and the results of renal investigations performed in children with xanthogranulomatous pyelonephritis. METHOD: A retrospective review of 31 cases presenting with the histopathological diagnosis of xanthogranulomatous pyelonephritis between 1963 and 1999. RESULTS: The mean follow up was 8.2 years. The male:female ratio was 1:1.1. The left kidney was affected in 26 of the 31 patients. The positive findings on examination and investigation at presentation were: fever, 16 children; pyuria, 26 children; positive urine culture, 16 children. A haemoglobin of < 100 g/l was measured in 27 of 31 patients and 15 of 18 patients tested had a raised erythrocyte sedimentation rate of > 20 mm in the first hour. Twenty six children had renal calculi, with a large reduction in the function of the affected kidney on isotope scintigraphy in 27 of the 29 patients tested. Hypertrophy of the contralateral kidney, shown on imaging, was present in 17 of 31 patients. CONCLUSIONS: Increasing awareness of this condition should lead to the diagnosis being suspected preoperatively.

A comparative study of the analgesia requirements following laparoscopic and open fundoplication in children.

To introduce a new procedure, careful evaluation of its effects must be undertaken to assess its impact. Laparoscopic procedures in children are a relatively new phenomenon. A reduction in analgesia is a frequently quoted benefit of a laparoscopic procedure. The introduction of laparoscopic fundoplication into our surgical armoury lead us to evaluate the analgesia requirements of a laparoscopic procedure compared to conventional surgery. Comparative analysis of the analgesia requirements of 40 fundoplication procedures (20 laparoscopic, 20 open) was undertaken. All the pain-relief data was prospectively documented by a pain team as part of an ongoing hospital audit. This pain team was unaware of the comparative study, but were assessing the quality of analgesia within the hospital trust. The total amount of morphine analgesia required was similar for both laparoscopic and open surgery (0.432+/-0.28, 0.427+/-0.28 mg/kg). The period for which analgesia was required was significantly less in the laparoscopic group (1.2+/-0.46, 2.7+/-0.67 days; p = 0.03), yet the requirement for morphine during the first 24 hours was greater in the laparoscopic group (0.399+/-0.19, 0.22+0.11 mg/kg, p = 0.02) despite similar NSAID requirements (18+/-17.28, 18+/-20.16 mg/kg, respectively). The benefit of a laparoscopic over an open fundoplication would appear to be in the decreased duration of pain, as indicated by the decreased duration of analgesia following surgery.

A prospective study of adult inguinal hernia repairs using absorbable sutures.

A prospective study was conducted over a 5-year period to determine whether inguinal hernia repair could be safely performed with absorbable suture material (polydioxanone) with reference to recurrence rates, wound pain, haematoma formation and wound infection. Analysis is available for 111 operations involving 111 patients. Mean follow-up was 36 (range 21-66) months, with 81 procedures monitored for more than 2 years. Two-layered hernia repair was used in all cases with polydioxanone as the chosen suture material. Mean post-operative stay was 2.1 days, with no hospital wound infections and three haematomas. Review identified 1 wound infection. There have been two recurrences. Preliminary results suggest that hernia repair with absorbable suture materials is comparable to traditional non-absorbable repair in terms of recurrence and associated wound complications. The additional benefit is the absence of foreign material in the wound region following degradation of the absorbable material. This does not appear to compromise the integrity of the hernia repair. Mesh repairs are increasingly preferred to Shouldice-style repairs in elective inguinal hernias. However, we believe that polydioxanone should be the suture material of choice in obstructed or strangulated hernia. A larger study is required to verify this, as well as a longer follow-up period. The use of absorbable material warrants further investigation.

Plasma thyroxine concentrations in farmed ostriches in relation to age, body weight, and growth hormone.

Thyroid hormones are of interest in ostriches because, in common with other ratites, ostriches are believed to have evolved through neoteny, a process which, in amphibia, is related to thyroid function. Farmed ostriches show marked differences in growth rates, a problem which also could be related to abnormal thyroid function. In adult farmed ostriches (more than 3 years old), mean plasma thyroxine concentration was 1.8 nmol middle dot liter-1 (range 0.2 to 6.5 nmol middle dot liter-1). This is a lower mean and a much greater range than those in adult starlings or Japanese quail measured at the same time. No sex differences were observed. In 5-month-old ostriches (approximately half grown) mean levels were again low (3.1 nmol x liter-1, range 0.2 to 9.9 nmol x liter-1). There was a correlation (P < 0.0005) between thyroxine and body weight, which ranged from 10.8 to 51.5 kg. Growth hormone in this group ranged from 0.7 to 45.6 microg x liter-1, but there was no correlation with body weight or with thyroxine. In the same group of birds at 10 months of age, plasma thyroxine concentrations were similar, but were not correlated with body weight. In young birds sampled between hatch and 13 weeks, mean thyroxine decreased from 7.6 nmol x liter-1 soon after hatching to less than 2 nmol x liter-1 at 2 weeks and remained less than 2 nmol x liter-1 for the following 3 months. Thyroxine was highly variable within and between individuals. There was no correlation with body weight or growth rate. There were no significant differences between values at 13 weeks, 5 months, 10 months, or in adults. The results do not suggest that slow growth is directly related to low thyroxine. However, the low means and wide range of values do suggest that thyroid function in the ostrich is abnormal compared to other birds.

Antegrade colonic enemas.

Thirteen children aged 6-14 (mean 8) years in whom an antegrade colonic enema procedure was performed were reviewed retrospectively. All presented with refractory constipation or faecal soiling over a 3-year period. Nine of the children had previously undergone pull-through procedures for Hirschsprung's disease or high anorectal malformations. Two were suffering from spina bifida and two from idiopathic functional constipation. The operation was performed through a right iliac fossa incision. A catheterizable conduit was created. The appendix was brought out to the wound edge and made continent by intussuscepting the appendix base into the caecum. When the appendix was absent or unusable, a caecal tube was formed. Five patients suffered minor morbidity, six required a further operative procedure and two eventually required a sigmoid colostomy. However, the eventual outcome of a continent stoma was attained in 11 of the 13 children, all of whom would have been considered for sigmoid colostomy before introduction of the antegrade colonic enema procedure.