R.S.V.M. Severijnen

Complex regional pain syndrome type I in children.

Background: Complex Regional Pain Syndrome type I (CRPS I) is a potentially incapacitating syndrome which can occur after a minor injury or operation to a limb. It is a disorder characterized by pain, sensory and motor disturbances. CRPS I is well known in adults, but a relatively new diagnostic entity in children. The clinical presentation of CRPS I in children is, to some extent, different from adults and therefore sometimes not recognized early. The aim of this study was to search for differences in patient characteristics between children and adults with CRPS I.

Additional congenital defects in anorectal malformations

From 1974 until 1995 a total of 264 (141 ♂, 123 ♀) patients born with an anorectal malformation (ARM) were referred to the University Hospital Nijmegen in the Netherlands. All additional congenital defects (ACDs) were registered. Special attention was paid to whether the ACDs take part in associations, syndromes, or sequences. One or more ACDs were observed in 67% of the patients. In decreasing order the defects concerned the uro-genital tract (43%), skeleton (38%), gastrointestinal tract (24%), circulation (21%), extremities (16%), face (16%), central nervous system (15%), respiratory tract (5%), and remaining defects (5%). Associations were observed in 49% of the patients, mostly (in 44%) the Vertebral, Anorectal, Cardial, Tracheo-Esophageal, Renal and Limb association. In 5% of the patients syndromes were recognized. Sequences were seen in 2% of the patients. Remarkable is the combination of trisomy 21 and ARM without a fistula. The combination of ARM and the Zellweger syndrome has not been reported before.ConclusionAlmost all combinations of ARM and ACDs can be classified as an association, syndrome or sequence. ARM-causing agents affect males and females in equal numbers but lead to different expression in the sexes. The origin of the Omphalocele, Extrophia of the bladder, Imperforate anus, Sacral anomalies complex probably differs from that of other forms of ARM.

Pancreatic injury in children: good outcome of nonoperative treatment.

PURPOSE Treatment of blunt injury of the pancreas in children remains controversial. Some prefer nonoperative treatment, whereas others prefer operative management in selected cases. This report reviews the treatment of patients with blunt pancreatic trauma admitted to a level I pediatric trauma center in The Netherlands. METHODS Medical records of all children less than 15 years with blunt pancreatic trauma admitted to the University Medical Center St Radboud in the period 1975 to 2003 were retrospectively analyzed. RESULTS Thirty-four children were included, age 3 to 14 years. Most injuries were because of bicycle accidents (58%). On admission, amylase was raised in 90% of the patients. Five patients had pancreatic duct injuries identified by imaging (endoscopic retrograde cholangiopancreaticography was used once, magnetic resonance cholangiopancreaticography twice) or at surgery. Thirty-one children were initially managed nonoperatively. Pancreatic surgery was performed in 3 children (1 Roux-Y, 2 drainage only). Mean hospital stay was 29 days in the operative group and 24 days in the nonoperative group. Fluid collections developed in 2 operated patients. Both resolved spontaneously. In 14 of the 31 nonoperated patients, a pseudocyst developed. Only 6 of these needed secondary intervention. Of these, 3 were drained percutaneously. There was no mortality and no long-term morbidity in both groups. CONCLUSIONS Nonoperative management of pancreatic injury in children has good clinical outcome. Only 10% need secondary surgery. In 50%, pseudocysts develop of which half can be managed nonoperatively. The reliability of computed tomographic scan grading is of limited value to decide whether to operate primarily. There is little to gain with ERCP and stenting. The place of MRCP as a noninvasive diagnostic tool remains to be determined.

Enteral drug absorption in patients with short small bowel : a review.

Drug therapy may become difficult when a significant amount of the small intestine is resected, as happens in patients with a short small bowel. Drug absorption from the gastrointestinal tract is altered in these patients; however, this effect is variable in patients and differs with each drug. Literature regarding clinical outcomes of normal or alternative administration routes in patients with a short small bowel is limited. We explored what is written about the normal absorption of commonly used drugs and what difference the resection of different but substantial parts of the small intestine makes. Changes in the gastrointestinal tract after resection of >50% of the small intestine causes malabsorption of macronutrients and micronutrients, and may alter the drug absorption process. The metabolic activity of the abundantly present intestinal lactobacilli can also affect the enteral drug absorption in patients with short small bowel as this results in the production of lactic acid, gaseous CO2, ethanol and an increased bile acid deconjugation. Accelerated intestinal luminal transit time causes a reduction in absorption of certain antimicrobial agents, digoxin, hydrochlorothiazide, Ciclosporin, Cimetidine, mesalazine (5-aminosalicylic acid), oral contraceptives and levothyroxine. Gastric hypersecretion and lack of sufficient contact time with the intestinal mucosa in patients with short small bowel leads to insufficient absorption of drugs such as omeprazole. Successful treatment with warfarin, tricyclic antidepressants, metronidazole, fluconazole, procainamide, Sotalol and pindolol are reported in several studies. Many different factors cause this variability in drug absorption in such patients. Monitoring the serum drug concentration in these patients may ease dealing with the management problems.

Quality of life in adults with childhood-onset of Complex Regional Pain Syndrome type I.

INTRODUCTION The clinical presentation of Complex Regional Pain Syndrome type I (CRPS I) in children differs compared to the presentation in adults. Reported results of treatment of CRPS I in children are usually more favourable and seem better than the reported treatment of adults with CRPS I. We investigated the quality of life (QoL) in adults who have been treated for childhood-onset CRPS I. METHODS We performed a retrospective chart review on signs, symptoms and treatment of all patients, seen and treated for CRPS I in childhood (age <16 years). At one time point a survey was sent by mail to all adult patients with onset CRPS I in childhood with a postal reminder after one month. The first part of the survey consisted of questions focused on the experience of chronic pain and other current complaints in the affected extremity. The second part consisted of a generic-health-related quality of life instrument (SF-36). RESULTS Forty-two patients (75%) responded to our survey. The median follow-up period was 12 years (SD 4.7; range 2-22). Fifty-two percent of all patients complained about pain at the time of follow-up. Of the 12 symptoms and signs, 4 are improved, 1 is worse and the remainder are unchanged. Fifteen patients experienced one or more documented relapses. General health and physical functioning (2 out of 8 scales on the SF 36) were lower in patients compared to those of the literature. CONCLUSION In contrast to the literature, the prognosis of childhood-onset CRPS I seems less favourable than usually reported, and is comparable to the prognosis of the adult-onset CRPS I in view of a decreased quality of life and a large relapse percentage (33%) at long-term follow-up.

Bile acid deconjugation by Lactobacilli and its effects in patients with a short small bowel

As early as 1986, Ohkohchi et al.1 reported on the nutritional condition and the absorptive capacity of infants with a short small bowel (SSB), especially with respect to severe steatorrhea, disrupted absorption of bile acids, and subnormal concentrations of serum vitamin D and of total cholesterol, with the intake of normal meals. In 1997, the same group2 specifically reported on the disturbed bile acid metabolism in children with SSB. In children without diarrhea, no severe fat malabsorption was recognized, and the content of total bile acids in the feces was normal to slightly increased.2 In patients with intractable diarrhea, fat malabsorption was observed, and the fecal content of total bile acids in these patients exceeded by more than tenfold that of controls.2 In healthy persons, the primary bile acids, cholic acid and chenodeoxycholic acid, account for 60%–70% of the total serum bile acids, but in SSB children with intractable diarrhea, they account for more than 95%. In these children, the (taurineand glycine-) conjugated bile acids accounted for only 10% of the total serum bile acids. Some children with and without diarrhea had hyperbile acidemia.2 In the same year, 1997, we published our findings on the intestinal flora in patients with a SSB.3 Most remarkable was the intestinal predominance of lactobacilli (up to 95%) in the fecal flora of orally fed SSB patients without antibiotic therapy.3 Ohkohchi et al.2 assumed that unconjugated bile acids from an indistinct origin affected the growth of intestinal bacteria such as lactobacilli. It is clear now that the contrary is true: the intestinal lactobacilli are bile acid-resistant, and they deconjugate the bile acids,4,5 thus strongly disturbing bile acid and lipid metabolism. An essential factor in the reported disorder of patients with SSB is bacterial bile salt hydrolase (BSH) activity,6,7 a specific property of many Lactobacillus spp, especially the intestinal ones.

A bronchogenic cyst under the scapula with a fistula on the back

A 20-year-old patient with a chronic fistula located at the left scapular region caused by a deep located subscapular bronchogenic cyst is described.

A reassessment of the PROPATRIA study and its implications for probiotic therapy

The PROPATRIA (Probiotics in Pancreatitis Trial) study was a multicenter, double-blind, placebo-controlled clinical trial that aimed to reduce infectious complications in patients with predicted severe acute pancreatitis by the enteral use of a multispecies probiotic preparation. An unprecedented 24 of 152 patients (16%) in the group receiving probiotics died versus 9 of 144 (6%) in the placebo group. This high mortality rate in the probiotic-treated group contrasts strongly with observations from a previous smaller study and from our observations regarding the effects of abundant intestinal lactobacilli in patients with short small bowel (SSB) syndrome. We argue here that a lethal combination of mainly proteolytic pancreas enzymes and probiotic therapy resulted in the high mortality rate of the PROPATRIA trial and that elevated levels of lactic acid produced by bacterial fermentation of carbohydrates were a key contributing factor. We suggest that probiotic therapy may not be counter-indicated for the prevention of secondary infections associated with acute pancreatitis, provided that future clinical studies (i) start probiotic therapy immediately after first onset of disease symptoms, (ii) limit the supply of fermentable carbohydrates, (iii) prevent bacterial (over)growth of patients own intestinal flora and (iv) massively increase the dose of probiotic bacteria.

Early closure of enterostomy after exteriorization of the small intestine for abdominal catastrophies.

Little has been written about the follow-up after temporary exteriorization of the small bowel for intraabdominal catastrophies in neonates and small infants. This report summarizes our results in 27 patients who had as a rule intestinal continuity restored electively within 4 weeks. There was a leakage rate of 10% and no mortality.

Congenital cardiovascular defects in children with intestinal malrotation.

Intestinal malrotation (IM) and cardiovascular defects (CCVD) are both common congenital defects. We investigated the prevalence and types of CCVD in a 25-year IM population, and its association with post-IM-operative morbidity and mortality. Data on the type of CCVD, other congenital defects, syndromes, associations, post-IM-operative morbidity and mortality were retrospectively reviewed from the records of IM patients born between 1980 and 2005. Data were analyzed on (significant) differences between CCVD subgroups, and risk factors for both morbidity and mortality were calculated. Seventy-seven of 284 IM patients (27.1%) were diagnosed with a major or minor CCVD (37 and 40 patients, respectively). Syndromes and associations were more frequently diagnosed in patients with major than with a minor CCVD (67.6 vs. 40%, respectively). Post-IM-operative complications, although frequently observed (61%), did not differ between patients with major and minor CCVD. Physical CCVD signs before IM surgery increased post-IM-operative morbidity significantly (OR 4.0, 95% CI 1.4–11.0). Fifteen patients died (19.5%), seven due to cardiovascular cause. Mortality risk was increased by intestinal ischemia and post-IM-operative complications and by major CCVD after correction for age at weight at the time of IM operation. Congenital cardiovascular defects in children with intestinal malrotation are common, with high morbidity and mortality rates after IM operation. Elective IM surgery in young patients with CCVD should be performed in a centre with adequate paediatric cardiac care. Benefits of laparoscopic intervention need further study.