Preduodenal portal vein and malrotation: what causes the obstruction?

AIM AND METHOD: Preduodenal portal vein is a rare congenital abnormality, and occurs either as a single malformation, in association with other malformations or as part of "polysplenia" syndrome. Preduodenal portal vein has seldom been reported as a cause of intestinal obstruction, however corrective surgery is nearly always performed. We conducted a 25-year retrospective study in a single centre to investigate the cause of obstruction in patients with preduodenal portal vein. Furthermore, we reviewed the literature on preduodenal portal vein. RESULTS: Over a period of 25 years, preduodenal portal vein was diagnosed in five patients. The diagnosis was made during surgery performed because of symptoms of high intestinal obstruction. All five patients had intestinal malrotation as well and, in all patients, another cause for high intestinal obstruction than preduodenal portal vein was found. CONCLUSION: Preduodenal portal vein is mainly asymptomatic. It is often associated with other intestinal congenital abnormalities more likely to cause high intestinal obstruction. Therefore, the (paediatric) surgeon should always be alert for another associated cause of intestinal obstruction. Because of the potential for technical problems from preduodenal portal vein during surgery, it nevertheless should be on the surgeon's mind during surgery when the patient has high intestinal obstruction.

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.

Surgical intervention of intestinal malrotations in paediatric patients without other congenital anatomical abnormalities: overview from a single center.

The subject of malrotation in infants and children without other congenital anatomical abnormalities is reviewed from the perspective of experience with 97 patients operated in 11 years. Fifty-five patients were younger than 6 weeks at operation. They often presented with bilious vomiting, in contrast to older children who presented with non-bilious vomiting or feeding problems. Patients younger than 6 weeks were operated more often acutely than older patients. Volvulus was more common in infants younger than 6 weeks. Two patients with a resulting short bowel syndrome died. In 73 of the surviving 95 (76.8%) children their symptoms disappeared. In the children younger than 6 weeks persisting abdominal problems were significantly less frequent than in older children. In the children presenting with proven gastro-esophageal reflux disease before the malrotation operation, abdominal problems persisted significantly more often. Although there remains considerable controversy over how older children without signs of vascular problems should be managed, failure to respond to radiographic evidence of malrotation could be considered malpractice if volvulus was to occur subsequently. For this reason, every patient with a radiological proven malrotation merits diagnostic laparoscopy.

Diagnosis and treatment of acute appendicitis in children: a survey among Dutch surgeons and comparison with evidence-based practice.

BACKGROUND: Considerable variability exists in the surgical approach to acute appendicitis in children, affecting both quality and costs of care. A national survey provides insight into daily surgical practice and enables comparison of practice with the available evidence. METHODS: A national survey was conducted in all 121 Dutch hospitals asking detailed information concerning diagnosis and treatment of children suspected of acute appendicitis. An evidence-based practice (EBP) score was developed on the basis of a critical appraisal of the literature, allowing for classification of reported practices with regard to the level of evidence and identification of hospitals working in accordance with the best available evidence. RESULTS: The overall response rate was 93%. For the diagnosis of acute appendicitis, respondents relied predominantly on patient history (29%) and clinical examination (31%), followed by laboratory results (22%). Only 20% of the departments routinely measured total white blood cell count (WBC), C-reactive protein (CRP) and leukocyte differential count (proportion of polymorphonuclear cells), being part of the triad that provides diagnostic evidence. Although strong evidence exists in favour of routine prophylaxis for suspected appendicitis, only two thirds of surgical departments reported this as part of their routine practice. For a number of issues, reasonably consistent evidence is available (e.g. primarily versus delayed closure, drainage versus lavage, routine peritoneal culturing). Thirty-eight percent of the departments routinely cultured abdominal fluid despite various reports that it provides no therapeutic advantage. Not more than 22% of the departments closed the skin in perforated appendicitis in spite of clear supportive evidence. Considerable variation exists in cleaning the abdomen in perforated appendicitis, despite evidence favouring lavage. Comparing departments in terms of compliance with available evidence revealed that most paediatric surgery departments worked according to evidence-based medicine. CONCLUSIONS: Available evidence on diagnosis and treatment of acute appendicitis in children is only partly applied in a small proportion of hospitals in the Netherlands. It is recommended that national guidelines be published, which could decrease health care costs and increase more uniform policy, improve quality of care for this group of patients and improve training of residents in general surgery in the Netherlands.

[Pneumatosis intestinalis in 9 children with an oncologic disease]. / Pneumatosis intestinalis bij 9 kinderen met een oncologische aandoening.

OBJECTIVE: To acquire knowledge regarding the rare condition pneumatosis intestinalis (PI) in children treated for malignant disease. DESIGN: Retrospective. METHOD: In 1998-1999 PI was diagnosed in 9 of the 140 children with malignant disease in the department of Paediatric Oncology of the UMC St Radboud, Nijmegen, the Netherlands. By examination of the records of these 9 children, data were collected on the symptomatology, diagnostics, treatment and prognosis of PI. RESULTS: The 9 children included 7 boys and 2 girls, varying in age from 2 to 12 years. In 7 patients the underlying disease was acute lymphocytic leukaemia and in 2 it was a stage IV neuroblastoma. The presenting symptoms were nonspecific and included: a distended abdomen, abdominal pain, diarrhoea and constipation. In all children, PI was located in the colon. Supplemental blood and microbiological analysis did not reveal any typical abnormalities. 8 children were treated with lactitol because of constipation. A laparotomy was performed in the first patient, while the other 8 were treated with gastric suctioning, parenteral nutrition and antibiotics. All 9 children recovered within a few weeks. CONCLUSION: With supportive care, PI in children with malignant disease is mostly a self-limiting condition. A pneumoperitoneum in PI is no indication for surgery, except in the presence of an acute abdomen. Chemotherapy can be continued.

Preventive and curative effects of probiotics in atopic patients.

Normally, the transport of allergens through the intestinal epithelia to the blood is limited. It is hypothesised that if these compounds arrive in the blood circulation, they must percolate through the epithelial cell layer. Thus, food allergy (and thus atopic eczema) implies an increased intercellular leakage of the gut wall. Such increased intercellular leakage is thought to be caused by a slightly changed cellular morphology due to a slight cytopathologic effect because of both a limited decay of the cytoskeleton and a slightly reduced turgor. These events may be due to a reduced production of intracellular metabolic energy in the epithelial cells due to an increased concentration of familiar, frequently occurring, potentially toxic bacterial metabolites, i.e., d-lactic acid and/or ethanol. In this hypothesis we suggest that adequate probiotics can (i) prevent the increased characteristic intestinal permeability of children with atopic eczema and food allergy, (ii) can thus prevent the uptake of allergens, and (iii) finally can prevent the expression of the atopic constitution. The use of adequate probiotic lactobacilli, i.e., homolactic and/or facultatively heterolactic l-lactic acid-producing lactobacilli, reduces the intestinal amounts of the bacterial, toxic metabolites, d-lactic acid and ethanol by fermentative production of merely the non-toxic l-lactic acid from glucose. Thus, it is thought that beneficial probiotic micro-organisms promote gut barrier function and both undo and prevent unfavourable intestinal micro-ecological alterations in allergic individuals.

[Acute appendicitis in children: serious complications when treatment is delayed]. / Appendicitis acuta bij kinderen: ernstige complicaties bij vertraging in de behandeling.

Three children, two boys aged 9 and 6 and a 12-year-old girl, had diffuse abdominal complaints, diarrhoea and a (sub)febrile temperature for several days. On admission, they were found to have a perforated inflamed appendix and peritonitis. Following asystole, intra-abdominal abscesses and an enterocutaneous fistula, the oldest boy showed good recovery after a hospital stay of two months; the girl recovered after one month in hospital following a psoas muscle abscess and two episodes of constrictive pericarditis with threatened tamponade. The younger boy was dead on arrival at the hospital. Appendicitis is not always easy to diagnose. An atypical presentation, very often with diarrhoea, can result in diagnostic delay. Early surgical consultation is mandatory in a child with progressive abdominal pain.

The origin of hydrogen sulfide in a newborn with sulfhaemoglobin induced cyanosis.

This report investigated the origin of H(2)S in a newborn boy with sulfhaemoglobin induced cyanosis, who died because of multiple organ failure. Frozen material was collected and studied after death. The results suggest that enzymes had been released from deteriorating organs into the blood and abdominal fluid, and that the reaction of one of these enzymes with sulfur containing amino acids might have resulted in increased H(2)S concentrations. It is hypothesised that this release of enzymes resulted from a haemolysin produced by an invasive haemolytic Escherichia coli that was found in the blood and organs of this patient.

[Diagnostic image (88). An infant with projectile vomiting]. / Diagnose in beeld (88). Een zuigeling met projectielbraken. Pylorushypertrofie.

A one-month-old infant was admitted with projectile vomiting due to a hypertrophic pyloric stenosis. A pyloromyotomy was successfully performed.

Defecation problems in children with Hirschsprung's disease: a prospective controlled study of a multidisciplinary behavioural treatment.

UNLABELLED: Constipation, faecal incontinence, soiling and difficult toilet training remain significant problems in children with Hirschsprung's disease after corrective surgery. Chronic defecation problems can have various negative implications. At the University Medical Centre Nijmegen, a multidisciplinary behavioural treatment was developed to treat defecation problems. In this paper, a prospective controlled study is presented concerning the effect of this treatment upon children suffering from chronic defecation problems following corrective surgery for Hirschsprung's disease. The effect of treatment was studied in 27 children (21M, 6F, mean age 5.2y, range 2-11 y). Fourteen children were allocated to the experimental treatment group. The 13 children allocated to the waiting-list control group were also treated following a waiting period of 6 mo. On all outcome variables, children in the experimental treatment group had significantly better results after treatment than children in the waiting-list control group after the waiting period. No effect of age upon treatment was found. The effect of treatment remained significant on all outcome variables at a mean follow-up of 7 mo after the end of treatment. CONCLUSION: Multidisciplinary behavioural treatment is successful in decreasing chronic defecation problems in children with Hirschsprung's disease.

Multidisciplinary behavioral treatment of defecation problems: a controlled study in children with anorectal malformations.

BACKGROUND/PURPOSE: The most frequent consequences of being born with an anorectal malformation (ARM) are problems with fecal continence and constipation, which can have various negative implications. In this prospective, controlled study the effect of multidisciplinary behavioral treatment dealing with these problems is evaluated. METHODS: The effect of multidisciplinary behavioral treatment was studied in 24 children (15 boys, 9 girls; mean age 5.8 years). Thirteen children were allocated to the treatment condition. The 11 children allocated to the waiting list control group also were treated after a waiting period of 6 months. Children underwent follow-up after treatment. RESULTS: Compared with a waiting list control group, the experimental treatment group scores significantly better on 2 important measures ("Templeton," "Percentage of feces in toilet"). Although young children had poorer scores than older children before treatment, no significant differences in the favorable outcome of treatment were found between both groups after treatment. No effect of type of ARM on treatment was found either. The results of multidisciplinary behavioral treatment remain stable over a mean follow-up period of 7 months. CONCLUSION: Multidisciplinary behavioral treatment is an important and valuable supplement to the standard medical treatment of children born with ARM suffering from chronic defecation problems.

Clinical letter: epidural analgesia in a newborn with Hirschsprung's disease, associated with congenital central hypoventilation syndrome.

A case is presented of a neonate with Hirschsprung's disease, associated with congenital central hypoventilation syndrome. After an ileostomy (at 2 days) and a stoma revision (at 10 days), postoperative pain management was established by continuous intravenous infusion of morphine, which caused severe postoperative respiratory depression. At 6 weeks a re-exploration and stoma revision was performed using postoperative epidural analgesia with bupivacaine. This caused no respiratory depression. A colectomy under epidural analgesia at 8 months was also uneventful. Respiratory difficulties in children with congenital central hypoventilation syndrome associated with Hirschsprung's disease are discussed in relation to the technique of choice for postoperative pain management.

Defecation problems in children with Hirschsprung's disease: a biopsychosocial approach.

Although most patients with operated Hirschsprung's disease (HD) have good continence in adulthood, a majority have postoperative defection problems during school age. Persistence of chronic constipation and/or incontinence may have considerable consequences for psychosocial development, parent-child interactions, quality of life, and the child's general condition. Considering these consequences, it is important to treat these problems as early as possible. From a biopsychosocial view, we developed a multidisciplinary treatment aimed at resolving defecation problems by teaching the child bowel self-control, primarily by training optimal defecation skills and subsequently toilet behavior. This treatment, carried out by a child psychologist, a pediatric physiotherapist, and a pediatric surgeon, consists of five steps: explanation; extinction of fear and avoidance behavior; learning new defecation behavior; learning an adequate straining technique; and generalization toward daily life. The effect of the treatment was investigated retrospectively in 16 boys with operated HD. The children improved significantly in all aspects during treatment, suggesting that multidisciplinary treatment can significantly reduce the postoperative chronic bowel problems of most children with operated HD. The treatment was as effective in young children (2-5 years) as in older children (5-14 years).