Persistent gastrointestinal symptoms after correction of malrotation.

Devane, S P; Coombes, R. C.; Smith, Virpi V.; Bisset, W M; Booth, I W; Lake, B D; Milla, P J

doi:10.1136/adc.67.2.218

Cited by 58 publications

(41 citation statements)

References 12 publications

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“…These patients improved slowly, although now aged 3 and 5 years, respectively, they continue to have sporadic episodes of constipation without mechanical obstruction, which can be managed with senokot or lactulose. Patients 5 and 17 were similar to a group of patients with feeding problems after a Ladd’s procedure for midgut malrotation described by Devane et al ,7 some of whom had enteric neuropathies. Developmental delay is described as one of the features in this syndrome,3 4 and perhaps the delay in the development of the central nervous system is paralleled by delay in the development of the enteric nervous system.…”

Section: Discussionsupporting

confidence: 58%

“…In our series we found severe gastrointestinal symptoms in 16 of 25 patients. All had such poor feeding that they required nasogastric feeding and in three of these patients after completion of the investigations feeding gastrotomies were placed, although the presence of a feeding gastrostomy appears not to influence the investigations performed 7. Investigation of the most severely affected patients showed their poor feeding to be associated with gastro-oesophageal reflux and greatly impaired acid clearance, providing evidence of an underlying oesophageal dysmotility.…”

Section: Discussionmentioning

confidence: 99%

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Feeding difficulties and foregut dysmotility in Noonan's syndrome

Shah

Rodríguez

Louis

et al. 1999

Archives of Disease in Childhood

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Purpose-Noonan's syndrome is a common dysmorphic syndrome in which failure to thrive and gastrointestinal symptoms are frequent but poorly understood. Design-Twenty five children with Noonan's syndrome were investigated by contrast radiology, pH monitoring, surface electrogastrography (EGG), and antroduodenal manometry (ADM). Results-Sixteen had poor feeding and symptoms of gastrointestinal dysfunction. All 16 required tube feeding. Seven of 25 had symptoms of foregut dysmotility and gastro-oesophageal reflux. In the most symptomatic children (four of seven) EGG showed fasting frequency gradient loss along the stomach fundus and pylorus with antral postprandial frequency loss. ADM showed shortened fasting cycle length, with abnormal phase III and shortened postprandial activity containing phasic contractions. Implications-Gastroduodenal motor activity was reminiscent of 32-35 week preterm patterns. The feeding diYculties appear to resolve as gut motility matures. In Noonan's syndrome, feeding problems appear to be the result of delayed gastrointestinal motor development. (Arch Dis Child 1999;81:28-31)

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Section: Discussionsupporting

confidence: 58%

Section: Discussionmentioning

confidence: 99%

Feeding difficulties and foregut dysmotility in Noonan's syndrome

Shah

Rodríguez

Louis

et al. 1999

Archives of Disease in Childhood

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“…It seems that among these patients there are alterations of intestinal motility, leading to short and long term persistent episodes of vomiting, diarrhoea, anorexia, and food intolerance. This persistence of symptoms could be due to an intrinsic innervation defect associated with some of these malformations [66] and not to the surgical techniques that were used. These symptoms have been described as affecting 25% of patients who were operated on as neonates affected by volvulus, with vomiting being the most frequent symptom (40%) and sub-obstructive clinical manifestations with abdominal distension being the least frequent (21%) [67].…”

Section: Discussionmentioning

confidence: 87%

Anomalies of intestinal rotation and fixation: consequences of late diagnosis beyond two years of age

et al. 2007

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The malrotation and no well fixation anomalies of the digestive tract is also frequent in older child, young and adult, with characteristic and specific clinical presentation. Actually, the diagnostic and treatment seem to be late, after suffering prolonged symptoms and/or in emergency. We present nine cases of anomalies in the embryonic development of the digestive tract which were diagnosed and treated in infants or young, all above 2 years old. Eight cases were of more or less complete intestinal malrotation; one of them was a complete malrotation with an intrinsic duodenal stenosis associated (no bands of Ladd) and another one was a right paramesocolic hernia, always accompanied by malrotation. The association with other extra-digestive anomalies, especially urological, was 70%. The predominant symptom was intermittent abdominal pain (IAP)-80%-sometimes accompanied by vomiting (35%) and episodes of diarrhoea (25%). In all the cases, while the clinical background was early, diagnosis was late. Indeed, in 60% of the cases diagnosis was made intra-operatively in emergency surgical interventions. The imaging procedures employed were scanning and Doppler ultrasound, CT scan, and contrast gastrointestinal series (GIS). Up to 30% of errors in interpretation occurred, although they were eventually corrected with other tests. The most reliable diagnostic procedures were GIS and CT scan with contrast, although partial interpretation errors occurred with the latter procedure. Surgery was essential in 80% of the pre-operative cases, and in another two it was required as a preventative measure. Post-operatively, there was notable persistence of SBS in the cases of intestinal necrosis, and of other lesser symptoms in the rest. We conclude that: intestinal malrotations and malfixations are still being diagnosed very late, with serious systemic consequences such as intestinal obstructions or necroses, and prolonged clinical suffering. This could all be avoided if more attention were paid to the digestive symptoms associated with IAP, and to subocclusion or other abdominal phenomena (distension,...), together with, in the case of doubt about the findings with the previous procedures, the opportune imaging tests (e.g., abdominal Doppler ultrasound, CT scan with contrast, and barium GIS). Unlike other authors, we consider that the morbidity/mortality associated with cases of late diagnosis of these anomalies is high, and calls for earlier surgical treatment.

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“…The mechanism that determines the orientation of the intestines is not known. 9 The bowel normally returns to the abdominal cavity at 12 weeks' gestation. By this time, enteric neurons have populated the gastrointestinal tract from mouth to anus.…”

Section: Patients Materials and Methodsmentioning

confidence: 99%