We're going to move on with our abstract presentations. The next speaker is Dr. Janjua. Chiari malformation clusters describe differing presence of concurrent anomalies based on Chiari type. Thank you again for the opportunity. So I'm going to talk about Chiari malformations in terms of presence of concurrent anomalies based on Chiari types. We have nothing to disclose. So Chiari malformations are essentially a structural defect resulting from congenital hyperplasia of posterior fossa with transdermal herniation. Classified into almost now six types, but I'm just going to primarily focus on four types because of the study. So commonly associated with other neurological abnormalities such as reported of syringomyelia from 23 to 80 percent in Chiari 1. Chiari malformation type 1 has been reported to be more prevalent in 0.8 to 3.7 percent of children and about 2.4, 0.24 to 0.9 percent of adult population undergoing MR imaging. So objective was to elucidate the nationwide incidence of different types of Chiaris and the concurrent anomalies associated with each type. So primarily our data source was nationwide inpatient sample which is primarily developed as part of healthcare cost and utilization project from 2002 to 2012. And all four Chiari subtypes primarily were included in this study and we utilized ICD-9 codes for that. Additionally for cardiac, gastrointestinal and genitourinary anomalies were also studied utilizing ICD-9 codes. Congenital and other anomalies associated with Chiari were studied using a logarithm and generating a contingency set of tables. Association in about 0.5 percent of the population were utilized and NIS hospital and year-adjusted rates were allowed for accurate assessment. So here are our results. Breakdown of Chiari by different types. We can see essentially 300,000 cases for Chiari type 1, about 120,000 cases for Chiari 2 and about 15,000 cases for 3 and 80,000 for Chiari 4. Overall about 44 percent of Chiari patients have at least one concurrent anomaly. Most commonly neuro-tube defects about 15 percent, 2 percent congenital hydrocephalus, 1.6 percent ASD, 1 percent PDA and about 0.8 percent BSD. Looking at main three types of concurrent conditions by Chiari type. Type 1 we can see for about 4.6 percent of serine gomelia and 0.5 percent of hydrocephalus and ASD defects. While looking at Chiari 2, serine gomelia about 2.8 percent. Looking at type 3 and type 4, significantly increased risk of congenital hydrocephalus and neuro-tube defects which ranges up to 3.7 percent type 3 and about microcephaly about 6.7 percent in type 4. Here are the results looking at other association with other conditions like scoliosis and serine gomelia in type 1 about 0.6 percent with telecoresyndrome and Ehlers-Danlos about 0.6. In looking at Chiari type 2, we could see scoliosis and telecore about 0.7 percent. As we have seen previously, type 3 and type 4 essentially had more neuro-tube defects associated with hydrocephalus ranging up to 11.4 to about 5 percent type 3 and about 16 to 10 percent in type 4. So in conclusion, Chiari malformations have strong association with other abnormalities outside of known relationships. In regards to neurologic anomalies, we can see serine gomelia is commonly associated with Chiari type 1 and type 2. Hydrocephalus and other neuro-tube defects like encephalocetamol frequently seen in Chiari type 3 and 4. Neurologic involvement is commonly encountered, but additional body systems are often involved with ever-seeing hindbrain hypoplasia and progressive malformations. Thank you.

Chiari malformations

concurrent anomalies

structural defects

posterior fossa

nationwide incidence