How common is colonic elongation in children with slow-transit constipation or anorectal retention?

PURPOSE: Colonic elongation is reported as a possible cause for slow colonic transit, as it is observed in patients with slow-transit constipation (STC). This study aimed to determine the frequency of colonic elongation in children with STC or anorectal retention using radioimaging. We hypothesized that transverse colon elongation may occur in patients with STC, whereas sigmoid colon elongates in patients with anorectal retention. METHODS: Nuclear transit scintigraphy performed for chronic constipation (1999-2011) was analyzed qualitatively for elongated transverse colon or sigmoid colon. Three major colonic transit patterns were identified: slow transit in the proximal colon (STC), normal proximal colonic transit with anorectal retention (NT-AR), and rapid proximal transit ± anorectal retention (RT). χ(2) Test was used for statistical analysis (P < .05 significant). RESULTS: From 1999 to 2011, 626 children had nuclear transit scintigraphy. Transverse colon elongation occurred more frequently in STC (73/322, or 23%) compared with NT-AR (9/127, or 7%) and RT (5/177, or 3%; P < .0001). Sigmoid colon elongation was equally common in NT-AR (8/127, or 6%) compared with RT (10/177, or 6%) and STC (14/322, or 4%; P < .9). CONCLUSION: Transverse colon elongation is more common in STC (23%), whereas sigmoid colon elongation is not more common in anorectal retention. Colonic elongation may be the cause or the result of the underlying slow colonic transit.

Nuclear transit studies of patients with intractable chronic constipation reveal a subgroup with rapid proximal colonic transit.

AIMS/BACKGROUND: Nuclear transit studies (NTS) allow us to follow transit through the stomach and the small and large intestines. We identified children with chronic constipation with rapid proximal colonic transit and characterized their clinical features. METHODS: We reviewed NTS from 1998 to 2009 to identify patients with chronic constipation and rapid proximal colonic transit, defined as greater than 25% of tracer beyond hepatic flexure at 6 hour and/or greater than 25% of tracer beyond end of descending colon at 24 hour. This was correlated with clinical symptoms and outcome from patient records. RESULTS: Five hundred twenty children with chronic constipation underwent investigation by NTS, and 64 (12%) were identified with rapid proximal colonic transit. The clinical history, symptoms, and outcome in 55 of 64 available for analysis frequently showed family history of allergy (10.9%) and symptoms associated with food allergy/intolerance: abdominal pain (80%), anal fissure (27.3%), and other allergic symptoms (43.6%). Eighteen children were treated with dietary exclusion, with resolution of symptoms in 9 (50%). CONCLUSIONS: Some children with intractable chronic constipation have rapid proximal colonic transit, have symptoms consistent with possible food allergy/intolerance, and may respond to dietary exclusion. The NTS can identify these patients with rapid proximal transit that may be secondary to food intolerance.

¹²³I-MIBG scintigraphy/SPECT versus ¹8F-FDG PET in paediatric neuroblastoma.

PURPOSE: To analyse different uptake patterns in (123)I-MIBG scintigraphy/SPECT imaging and (18)F-FDG PET in paediatric neuroblastoma patients. METHODS: We compared 23 (123)I-MIBG scintigraphy scans and 23 (18)F-FDG PET scans (mean interval 10 days) in 19 patients with a suspected neuroblastic tumour (16 neuroblastoma, 1 ganglioneuroblastoma, 1 ganglioneuroma and 1 opsomyoclonus syndrome). SPECT images of the abdomen or other tumour-affected regions were available in all patients. Indications for (18)F-FDG PET were a (123)I-MIBG-negative tumour, a discrepancy in (123)I-MIBG uptake compared to the morphological imaging or imaging results inconsistent with clinical findings. A lesion was found by (123)I-MIBG scintigraphy and/or (18)F-FDG PET and/or morphological imaging. RESULTS: A total of 58 suspicious lesions (mean lesion diameter 3.8 cm) were evaluated and 18 were confirmed by histology and 40 by clinical follow-up. The sensitivities of (123)I-MIBG scintigraphy and (18)F-FDG PET were 50% and 78% and the specificities were 75% and 92%, respectively. False-positive results (three (123)I-MIBG scintigraphy, one (18)F-FDG PET) were due to physiological uptake or posttherapy changes. False-negative results (23 (123)I-MIBG scintigraphy, 10 (18)F-FDG PET) were due to low uptake and small lesion size. Combined (123)I-MIBG scintigraphy/(18)F-FDG PET imaging showed the highest sensitivity of 85%. In 34 lesions the (123)I-MIBG scintigraphy and morphological imaging findings were discrepant. (18)F-FDG PET correctly identified 32 of the discrepant findings. Two bone/bone marrow metastases were missed by (18)F-FDG PET. CONCLUSION: (123)I-MIBG scintigraphy and (18)F-FDG PET showed noticeable differences in their uptake patterns. (18)F-FDG PET was more sensitive and specific for the detection of neuroblastoma lesions. Our findings suggest that a (18)F-FDG PET scan may be useful in the event of discrepant or inconclusive findings on (123)I-MIBG scintigraphy/SPECT and morphological imaging.

Slow-transit constipation with concurrent upper gastrointestinal dysmotility and its response to transcutaneous electrical stimulation.

PURPOSE: Transcutaneous electrical stimulation (TES) speeds up colonic transit in children with slow-transit constipation (STC). This study examined if concurrent upper gastrointestinal dysmotility (UGD) affected response to TES. METHODS: Radio-nuclear transit studies (NTS) were performed before and after TES treatment of STC as part of a larger randomised controlled trial. UGD was defined as delayed gastric emptying and/or slow small bowel transit. Improvement was defined as increase of ≥1 Geometric Centre (median radiotracer position at each time [small bowel = 1, toilet = 6]). RESULTS: Forty-six subjects completed the trial, 34 had NTS after stimulation (21 M, 8-17 years, mean 11.3 years; symptoms >9 years). Active stimulation increased transit in >50% versus only 25% with sham (p = 0.04). Seventeen children also had UGD. In children with STC and either normal upper GI motility (NUGM) and UGD, NTS improved slightly after 1 month (57 vs. 60%; p = 0.9) and more after 2 months (88 vs. 40%; p = 0.07). However, mean transit rate significantly increased with NUGM, but not UGD (5.0 ± 0.2: 3.6 ± 0.6, p < 0.01). CONCLUSION: Transcutaneous electrical stimulation was beneficial for STC, with response weakly associated with UGD. As measured by NTS, STC children with NUGM responded slightly more, but with significantly greater increased transit compared to those with UGD. Higher numbers are needed to determine if the difference is important.

Familial digital arthropathy-brachydactyly.

We report a large family with a previously undescribed, dominantly inherited condition comprising arthropathy of the hands and feet and progressive shortening of the middle and distal phalanges. We have designated the condition familial digital arthropathy-brachydactyly (FDAB). Onset of FDAB is in the first decade and the arthropathy is progressive, resulting in deformity and pain in adult life. The remainder of the skeleton is not affected. It is hypothesized from the radiological appearance of patients at different ages that FDAB might result from subchondral pathology primarily affecting the heads of the phalanges, metacarpals, and metatarsals, with the arthropathy and brachydactyly being secondary effects.