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.

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.

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.

[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.

Management of pediatric esophageal perforation.

Esophageal perforation is a serious complication necessitating immediate therapy. In a retrospective study we have evaluated the results in 13 children treated for esophageal perforation. Eleven of 13 perforations could be managed conservatively. In one child with extrapleural effusion, tube drainage was performed. The only death in this series occurred in a child who was brought for treatment after a 60-hour delay. Thoracotomy and multiple abscess drainage eventually proved unsuccessful. On the basis of our experience with children with esophageal perforation or with complications after esophageal atresia repair, we conclude that management of esophageal perforation in children differs substantially from therapy in adults and necessitates restrictive treatment guided by clinical symptoms.

Chronic tunnel infections in children: removal and replacement of the continuous ambulatory peritoneal dialysis catheter in a single operation.

OBJECTIVE: Chronic tunnel infections often necessitate the removal of the continuous ambulatory peritoneal dialysis (CAPD) catheter. Most published studies advocate postponing the insertion of a new catheter for several weeks. For young children it will be particularly difficult to wait this length of time, since vascular access may be cumbersome, and hemodialysis may not be well tolerated. The present study describes the results of the simultaneous removal and replacement of the CAPD catheter. DESIGN: Twenty-three Toronto Western Hospital II catheters were inserted in 17 children because of infectious complications (21 chronic tunnel infections; 2 recurrent peritonitis) in a single operation under appropriate antibiotic prophylaxis. The new catheter was inserted at the contralateral side of the abdomen with the deep cuff in the midline, using the same entrance to the peritoneal cavity. Dialysis was resumed immediately after the operation. SETTING: A university pediatric dialysis unit. PATIENTS: Seventeen children (mean age 3.7 years; range 1.0-8.5 years) were studied. In this group 23 catheters were replaced. RESULTS: In four cases a relapse of the tunnel infection was observed within 3 months. All other cases remained free of infection for a period of at least 6 months. The main causative microorganism was Staphylococcus aureus (15 occurrences). CONCLUSION: It is not necessary to interrupt peritoneal dialysis for the replacement of a CAPD catheter because of infectious complications.

Gallbladder embedded in an accessory liver lobe in umbilical cord hernia.

Two newborns are described, both with a hernia of the umbilical cord, which contained the gallbladder embedded in an accessory lobe of the liver.

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.

The midline cervical cleft.

The midline cervical cleft is a rare congenital anomaly of the ventral neck. With reference to two patients with a midline cervical cleft, we discuss the clinical picture, the embryology, and the surgical treatment.

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.

Neurocristopathy in mother (ganglioneuroblastoma) and daughter (aganglionosis): incidental or causal?

We report on a mother who had been treated for a ganglioneuroblastoma and her daughter who had a long segment aganglionosis. Review of the relevant literature and recent molecular findings warrant the conclusion that most likely, there is a causal relation between these two neurocristopathies.

Continuous arteriovenous hemofiltration (CAVH) in a premature newborn as treatment of overhydration and hyperkalemia due to sepsis.

If renal replacement therapy is required in the neonatal period, peritoneal dialysis is generally applied. In some cases, for example after extensive abdominal surgery, peritoneal dialysis is not possible. Continuous arteriovenous hemofiltration may then be an attractive alternative. The present paper describes the positive results in a 1265 g premature baby.

Long-gap esophageal atresia: experience with reconstruction in 25 patients.

In a series of 183 patients with esophageal atresia in a period of 20 years, 25 had a "long-gap" esophageal atresia. In most of these cases we succeeded in saving the patients' own esophagus but at the cost of one to two years of intensive treatment. The overall survival was 80%. The result compares favorably with the results of esophageal replacement reported in the literature.

Nonsurgical (conservative) treatment of giant omphalocele. A report of 10 cases.

Between January 1970 and December 1984, 65 patients with a congenital anterior abdominal wall defect were admitted to the Pediatric Surgical Center of the St. Radbound Hospital, Nijmegen, The Netherlands. There were 39 cases of omphalocele. Ten had giant omphalocele, defined as omphalocele that could not be closed primarily. In these cases, a conservative method of treatment was used. The results are reported of these 10 cases of giant omphalocele with special emphasis on mortality, complications, and length of hospital stay. Mean hospital stay was 95 days. Local infection did not present serious problems. Sepsis occurred in half the patients but was managed with antibiotics. There was one case of late volvulus of the stomach. All local applications, if used regularly, gave rise to complications. Two cases of hypothyroidism caused by the application of povidon-iodine and one case of alcohol intoxication were observed. In early years, one child was lost due to mercury poisoning. There was one early and one late death unrelated to the procedure.

[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.

[Hemofiltration as kidney function replacement treatment in newborn infants]. / Hemofiltratie als nierfunctie-vervangende behandeling bij pasgeborenen.

Peritoneal dialysis remains the treatment of choice for acute renal failure in infancy. After extensive abdominal surgery peritoneal dialysis often becomes impossible. As haemodialysis is extremely difficult in this age group, continuous arteriovenous haemofiltration (CAVH) is a good alternative. The experience with CAVH in two newborns is reported. Fluid and potassium can easily be removed. Catabolic products such as urea are cleared less effectively than by peritoneal dialysis.

[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.

[Poisoning by disinfectants in the conservative treatment of 2 patients with omphalocele]. / Vergiftiging door desinfectans bij conservatieve behandeling van twee patiënten met omfalokèle.

Two patients with complications of conservative omphalocele-treatment are described. A mercury-intoxication and an alcohol-intoxication were seen, respectively. It is concluded that generally a surgical treatment regimen is preferable, if the cele is not too large. When it is not possible to perform surgical treatment, alcohol application probably is the safest method. Dosage and frequency of application should be as limited as possible.

[Developments in management of esophageal atresia]. / Oesophagusatresie uit de kinderschoenen.

The results of treatment of oesophageal atresia show that attentiveness and knowledge of the referring paediatrician concerning diagnostics, preoperative care and transport, together with concentration of high-standard care and specific experience in centres for paediatric surgery, can lead to a substantial decrease in morbidity and mortality. Between 1955 and 1968 a total of 193 children have been operated for oesophageal atresia. Mortality has declined from 51% in the period 1955-1969 to 10% in the timespan 1980-1986. Mortality is almost solely determined by additional congenital malformations, in which congenital heartdefects form the major cause. Management of oesophageal atresia seems to have evolved to a routine-procedure. New trends in paediatric surgery pose new sorts of specific problems, that require attention.

[Gastroesophageal reflux]. / Gastro-oesofageale reflux.

Gastro-oesophageal reflux (GER) in children, causes sometimes aspecific symptoms in children. Not only in the case of regurgitation but also in respiratory problems or even in the 'near missed' - Sudden Infant Death Syndrome GER is to be considered. The most important method of investigation is pH-monitoring during 24 hours preceded by a barium meal and followed by endoscopy, in most cases. Operative treatment is necessary for a hiatal hernia, or after failure of conservative treatment of an oesophagitis. In mentally retarded children, who continue to vomit, a fundoplication can be necessary to improve general care.