Samuel Kocoshis

Neurogenic dysphagia resulting from Chiari malformations

Between 1980 and 1989, 15 of 46 patients (11 children, 4 adults) who underwent suboccipital craniectomy and cervical laminectomy for symptomatic Chiari malformations presented with manifestations of neurogenic dysphagia. Each of these patients had normal swallowing function before the development of dysphagic symptoms. Dysphagia was progressive in all 15 and, in most cases, preceded the onset of other severe brain stem signs. The rate of symptom progression varied depending on the age of the patient. Whereas the six infants (all Chiari II) deteriorated rapidly after the onset of initial symptoms, the five older children (two Chiari I, three Chiari II) and four adults (all Chiari I) showed a more gradual deterioration. In 11 patients with severe dysphagia, barium video esophagograms, pharyngoesophageal motility studies, continuous esophageal pH monitoring, and appropriate scintigraphic studies were useful in defining the scope of the swallowing impairment and determining whether perioperative nasogastric or gastrostomy feedings, gastric fundoplication, and/or tracheostomy were needed to maintain adequate nutrition and avoid aspiration. These patients all had widespread dysfunction of the swallowing mechanism, with a combination of diffuse pharyngoesophageal dysmotility, cricopharyngeal achalasia, nasal regurgitation, tracheal aspiration, and gastroesophageal reflux. The pathophysiology of these swallowing impairments and their relation to the hindbrain malformation is discussed. Postoperative outcome with regard to swallowing function correlated with the severity of preoperative symptoms. The four patients with mild dysphagia showed rapid improvement in swallowing function after surgery. Seven patients with more severe impairment but without other signs of severe brain stem compromise, such as central apnea or complete bilateral vocal cord paralysis, also improved, albeit more slowly. In contrast, the outcome in the four patients who developed other signs of severe brain stem dysfunction before surgery was poor. Early recognition of neurogenic dysphagia and expeditious intervention are therefore crucial in ensuring a favorable neurological outcome.

Coordinated interdisciplinary management of pediatric intestinal failure: A 2-year review

BACKGROUND/PURPOSE Intestinal failure is a complex metabolic process that results from malabsorption and malnutrition and provides challenges for a variety of pediatric subspecialists. The purpose of this study was to evaluate the effect of coordinated interdisciplinary team management of children with intestinal failure on nutritional outcome measures. METHODS The Intestinal Care Center (ICC) is staffed with an interdisciplinary team of pediatric specialists including a gastroenterologist, pediatric surgeon, transplant surgeon, clinical dietitians, and a nutrition support nurse. Using an established registry, the authors conducted a comprehensive evaluation of patient data including anthropometric measures, organ system function, and mode of nutrition support. Disease-associated complications including micronutrient deficiencies, growth delay, and death also were monitored. Nutritional outcome was assessed by transition from enteral to oral feeding, cessation of total parenteral nutrition (TPN), and maintenance of linear growth. RESULTS Since the inception of the ICC in 1996, 103 patients (69 boys, 34 girls) with intestinal failure have been evaluated with a median age of 2.6 years (range, 0.2 to 21.3 years). Mode of nutritional therapy on initial consultation included TPN (n = 76, 74%), enteral feedings (n = 6, 6%) and oral intake (n = 21, 20%). After intensive management of the 76 patients who were TPN dependent, 22 (29%) subsequently have been weaned from TPN (duration, 0.2 to 17.5 years) to oral (n = 14), oral-enteral (n = 4) or enteral feedings (n = 4). Of the 6 patients who were receiving enteral feedings, 4 (67%) were transitioned to oral feedings. Sixty-eight patients (66%) had evidence of hepatic disease. Of these, 10 underwent transplant, and 23 died (2 posttransplant). Linear growth velocity of neither pre- nor postpubescent patients significantly improved during the 2-year study period. CONCLUSION Data registry establishment and concurrent interdisciplinary team management of children with intestinal failure provides for innovative treatment approaches and a foundation for retrospective or prospective assessment of children with disease.

Correlates of depression in new onset pediatric inflammatory bowel disease.

Of thirty six children with new-onset inflammatory bowel disease given a Kiddie-SADS interview, five children were depressed and ten had some depressive symptoms. Depressed children had less severe illness, and were more likely to have a maternal history of depression, more life events, and families characterized by less cohesion and more conflict.

Obsessive-Compulsive Symptoms in Childhood Inflammatory Bowel Disease and Cystic Fibrosis

The Leyton Obsessional Inventory-Child Version was administered to 33 children with Crohns disease, 11 with ulcerative colitis, and 46 with cystic fibrosis. Subjects with ulcerative colitis scored significantly higher on total number of obsessional symptoms and degree of resistance to symptoms than subjects with Crohns disease. Scores of subjects with cystic fibrosis were intermediate between those of the ulcerative colitis and Crohns disease groups. However, neither the ulcerative colitis group nor the Crohns disease group differed significantly from the cystic fibrosis group that were statistically significant. Duration of illness was negatively correlated with symptoms in Crohns disease and ulcerative colitis. The results suggest that obsessive-compulsive symptoms are not specific to Crohns disease or ulcerative colitis and that obsessive-compulsive symptoms in pediatric chronic illness may be secondary to the demands of the illness.

Maternal psychiatric disorders in pediatric inflammatory bowel disease and cystic fibrosis

The mothers of 72 children and adolescents with inflammatory bowel disease (IBD) and 44 mothers of children and adolescents with cystic fibrosis (CF) were given A-SADS interviews. Fifty-one percent of IBD mothers and 41% of cystic fibrosis mothers had a lifetime history of depression. More IBD than CF mothers had a history of suicide gestures or attempts, and were more likely to have a history of obsessive compulsive disorder. However, CF mothers were more likely to have experienced panic attacks.

Characterization of late abdominal accumulation of 99Tcm-HMPAO leukocytes in a large population of children.

We retrospectively evaluated the incidence of late accumulation of 99Tcm-HMPAO leukocytes (99Tcm-WBC) in the right lower quadrant of a large population of children and characterized some predictive patterns that would enable differentiation of active inflammation from this late occasional accumulation of 99Tcm-WBC. We reviewed the charts of 211 children. The first group evaluated consisted of 79 controls: 30 normal children with no gastrointestinal disease, but who underwent 99Tcm-WBC scanning for other medical problems, and 49 children who had non-specific gastrointestinal (GI) complaints, but had no demonstrable inflammatory bowel disease by conventional diagnostic methods. The second group consisted of 132 children with inflammatory bowel disease: 80 children with Crohns disease (CD), 34 with ulcerative colitis (UC) and 18 with indeterminate colitis (IC). Children were imaged at 30 min and 3 h. Fifteen (19%) of the 79 controls scanned showed accumulation of 99Tcm-WBC in the right lower quadrant at 3 h and none at 30 min. Of those 15, 8 were from the control population and 7 from the group with non-specific GI complaints and negative work-ups. There was no uptake in other segments of the bowel. The accumulation was faint, of lesser intensity than in the iliac wing, and diffuse, such that identification of a specific loop of involved bowel was not possible. Migration of the 99Tcm-WBC distal to the terminal ileum was demonstrated. The other 64 children in the control group showed no accumulation of 99Tcm-WBC at any time during their scans. All 79 scans were blindly interpreted as normal studies. There were no false-positive readings encountered in the 132 children with inflammatory bowel disease (80 CD, 34 UC, 18 IC) when the aforementioned characteristics of the late accumulation of 99Tcm were used to differentiate inflammation from this physiological excretion. In conclusion, the late accumulation of 99Tcm-WBC in the right lower quadrant is characterized by (1) accumulation at no less that 3 h, (2) no accumulation in other segments of the bowel, (3) faint accumulation of lesser intensity than in the iliac wing, (4) a diffuse accumulation pattern and (5) migration of the 99Tcm-WBC into the caecum and ascending colon over time. Recognition of this excretion pattern enables differentiation of active Crohns disease of the small bowel from migration and accumulation of 99Tcm-WBC in the right lower quadrant of the abdomen.

Novel primitive swallowing reflex: facial receptor distribution and stimulus characteristics.

We recently described a primitive swallowing reflex: swallowing as a response to a puff of air administered to the face. To identify the facial afferent distribution of this response, the necessary characteristics of the stimulus, and the role of the infants antecedent behavior, we studied 13 infants who had demonstrated this reflex.We evaluated nine infants by clinically observing for swallowing in response to a total of 135 stimulus applications. All nine had consistently positive responses to the maxillary-ophthalmic area and to the maxillary-mandibular area. Two had consistent responses to stimulation of the mandibular area alone; these were positive. Six had consistent responses to stimulation of the ophthalmic area alone; these were negative.Four infants, evaluated by manometric documentation of swallowing (a total of 137 stimulus applications) demonstrated 47 of 79 (59%) positive responses to stimuli applied to facial areas including any parts of the lips, but only 7 of 28 (25%) positive responses to stimuli applied to facial areas excluding all parts of the lips (chi-squareP=0.002).Light touch to any facial area, including the cornea, failed to produce a swallow in any infant. Crying and sleep were incompatible with the reflex. This newly identified primitive swallow reflex seems to require diffuse stimulation, possibly thermal, to the perioral area of the face in an awake and noncrying infant.