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Impact of Abdominal Muscle Weakness on Motor Performance in Children with Prune Belly Syndrome
Catherine J. Chen, MD1, Lorena Marichal, PT2, Susan Simpkins, PT1, Niccolo Passoni, MD1, Alice Anderson, PT2, Deanna Lusty, PT2, Daniel Wong, MS1, Nida Iqbal, PhD1, Thomas Jascur, PhD1, Emma Sanchez, CRC2, Irina Stanasel, MD1, Linda A. Baker, MD1.
1University of Texas Southwestern Medical Center, Dallas, TX, USA, 2Children's Health, Dallas, TX, USA.

Background: The characteristic wrinkled abdominal appearance in Prune Belly Syndrome (PBS) is due to varying degrees of ventral abdominal wall musculature deficiency ranging from hypoplasia to complete absence. The weak abdominal musculature contributes to a diminished cough strength, increasing the risk of pneumonia and atelectasis, impaired bladder and bowel emptying, and poor balance and posture. The quantitation of these functional deficiencies is lacking. Furthermore, while surgical correction of the abdominal wall laxity improves appearance, improvement in these functional deficiencies has not been well-characterized. This study assesses trends in physical therapy (PT) usage and characterizes motor skills of children with PBS with commonly available standardized PT assessment tests, comparing children with PBS to matched normative data.
Methods: 27 children with PBS were recruited at the 2017 Dallas PBS Network Convention and divided into 2 groups based on age, completing one of two widely available standardized tests of motor performance administered by pediatric physical therapists. Children ≤4 years old (group A) underwent testing with the Peabody Developmental Motor Scales, 2nd ed. (PDMS-2) while children >4 years old (group B) were assessed with the Bruininks-Oseretsky Test of Motor Proficiency, 2nd ed. (BOT2) and GaitRITE tests. Results were compared to matched normative data. Care-providers participated in a questionnaire regarding motor function impairment and prior PT treatments.
Results: In the younger cohort (group A), 10 males and 1 female, median age 10.4 months (IQR 16.1 months), completed the PDMS-2 assessment. There was no prior history of abdominoplasty. 73% (n=8) scored below the 20th percentile compared to normative values in the stationary category, which measures balance. 55% (n=6) scored below the 20th percentile compared to normative values in the locomotion category, which quantifies ability to move from one place to another. In the older cohort (group B), 16 males, median age 10.0 years (IQR 5.9 years), completed the BOT2 assessment. 75% (n=12) had prior history of an abdominoplasty. Overall, 69% (n=11) scored below the 20th percentile compared to normative values in the strength and agility testing, a measure of running speed/agility and trunk, upper and lower body strength. 94% (n=15) were not able to perform a sit-up. 13/16 males completed the GaitRITE, which measures gait velocity. All had a slower gait speed compared to that of age-matched peers. Out of the 15 anonymous questionnaires received, 67% (n=10) underwent PT at some point, with only 5 patients still undergoing therapy. 9/11 responders reported PT as beneficial.
Conclusions: While 67% of children with PBS have participated in PT, the majority still scored below the 20th percentile in motor proficiency compared to matched typically developing children. The PDMS-2, BOT-2, and GaitRITE may serve as functional measures in children with PBS. Longitudinal studies are needed to assess the value of prolonged PT and the efficacy of abdominoplasties in improving functional deficiencies from ventral abdominal wall weakness.

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