Diagnosis and management of gastro-esophageal reflux disease in children with congenital diaphragmatic hernia—a nationwide assessment of practices

European Surgery - Although gastro-esophageal reflux disease (GERD) is the most frequent long-term morbidity in congenital diaphragmatic hernia (CDH) survivors, no consensual guidelines exist...     

A variant pattern of Calretinin immunohistochemistry on rectal suction-biopsies is fully specific of short-segment Hirschsprung’s disease

Background/purpose

Calretinin immunohistochemistry is now widely used to diagnose Hirschsprung’s disease (HD), since loss of calretinin expression within the mucosa and muscularis mucosae of rectal suction-biopsy is pathognomonic of HD. However, a stippled staining may be observed within hypertrophic nerves in the submucosae in some HD patients. The aim of the study was to test the hypothesis that such findings may announce the beginning of the transitional zone.

Methods

We retrieved 44 consecutive patients (10 girls and 34 boys; median age 6.5 days), diagnosed with aganglionosis on rectal suction biopsies, followed by surgery. According to calretinin immunohistochemistry performed on all paraffin-embedded rectal biopsies, we defined two HD groups: P? showing an absence of any staining within mucosa, muscularis mucosae and submucosa et P+ showing an absence of staining within the mucosa and muscularis mucosae, but a positivity of some submucosal hypertrophic nerves. These data were correlated to the length of total pathological segment (aganglionic and transitional zones) obtained from the original surgery reports.

Results

18/44 patients (40.9 %) belonged to the P+ group and 26/44 (59 %) patients were within the P? group. In the P+ group, the maximal length of the aganglionic zone was 9 cm [mean 4 (1–9)] and the total pathological zone never exceeded 14 cm [mean 8 (3.8–14)]. In the P? group, the maximal length of aganglionic zone was 55.5 cm [mean 11.3 (2.5; 55.5)] and the total pathological zone extended to 59.5 cm [mean 17.75 (4.5; 59.5)]. Aganglionic segment was significantly shorter in the P+ group (p < 0.0001).

Conclusion

Staining of some hypertrophic nerves in the submucosa in suction rectalbiopsy of HD patients using calretinin immunohistochemistry is only encountered in short-segment aganglionosis with a pathological zone always restricted to rectal and sigmoid colon. This information could be crucial for the surgeons in the decision to choose a transanal procedure.     

Focal cortical atrophy in multiple sclerosis: relation to lesion load and disability

Multiple sclerosis (MS) is thought to predominantly affect white matter (WM). Recently, however, loss of cortical gray matter has also been described. Little is known about the cause of cortical atrophy in MS, whether it occurs early in the disease course, and whether it affects all cortical regions equally or if there is a preferential pattern of focal cortical atrophy. An automated method was used to compute the thickness at every vertex of the cortical surface of the brains of 425 early relapsing-remitting MS patients. We correlated cortical thickness with the WM lesion load and the Expanded Disability Status Scale score. Mean cortical thickness correlated with WM lesion load and disability. The correlations of cortical thickness with total lesion load and disability were most significant in cingulate gyrus, insula, and associative cortical regions. Conversely, primary sensory, visual, and motor areas showed a less significant relationship. The highest amount of atrophy per lesion volume or disability scale unit was in the anterior cingulate cortex. This study confirms the relation between cortical atrophy, WM lesion load, and disability in MS, and suggests that cortical atrophy occurs even in MS patients with only mild disability. Most interestingly, we show a specific regional pattern of focal atrophy in MS that is distinctively different from the one in normal aging. The predilection of the atrophic process for areas that are heavily inter-connected with other brain regions suggests that interruption of WM tracts by MS plaques contributes, at least in part, to the development of cortical atrophy.     

Central venous-to-arterial carbon dioxide difference: an additional target for goal-directed therapy in septic shock?

Objective  To test the hypothesis that, in resuscitated septic shock patients, central venous-to-arterial carbon dioxide difference [P(cv-a)CO₂] may serve as a global index of tissue perfusion when the central venous oxygen saturation (ScvO₂) goal value has already been reached. Design  Prospective observational study. Setting  A 22-bed intensive care unit (ICU). Patients  After early resuscitation in the emergency unit, 50 consecutive septic shock patients with ScvO₂ > 70% were included immediately after their admission into the ICU (T0). Patients were separated in Low P(cv-a)CO₂ group (Low gap; n = 26) and High P(cv-a)CO₂ group (High gap; n = 24) according to a threshold of 6 mmHg at T0. Measurements  Measurements were performed every 6 h over 12 h (T0, T6, T12). Results  At T0, there was a significant difference between Low gap patients and High gap patients for cardiac index (CI) (4.3 ± 1.6 vs. 2.7 ± 0.8 l/min/m2, P < 0.0001) but not for ScvO₂ values (78 ± 5 vs. 75 ± 5%, P = 0.07). From T0 to T12, the clearance of lactate was significantly larger for the Low gap group than for the High gap group (P < 0.05) as well as the decrease of SOFA score at T24 (P < 0.01). At T0, T6 and T12, CI and P(cv-a)CO₂ values were inversely correlated (P < 0.0001). Conclusion  In ICU-resuscitated patients, targeting only ScvO₂ may not be sufficient to guide therapy. When the 70% ScvO₂ goal-value is reached, the presence of a P(cv-a)CO₂ larger than 6 mmHg might be a useful tool to identify patients who still remain inadequately resuscitated. Electronic supplementary material  The online version of this article (doi:) contains supplementary material, which is available to authorized users.