Page 28 - Delaware Medical Journal - March/April 2019
P. 28
Hip Evaluation in Children With Congenital Central Hypotonia: A Preliminary Report
Oussama Abousamra, MD; Maria del Pilar Duque Orozco, MD; Mehmet Serhan Er, MD; Kenneth J. Rogers, PhD, ATC; Julieanne P. Sees, DO; Kirk W. Dabney, MD;
Freeman Miller, MD; Justin Connor, MD
PURPOSE: The possibility of hip instability in hypotonic conditions has been reported. The aim of this study was to report the natural history of the hip in children with congenital central hypotonia.
METHODS: A cross-sectional analysis was performed on 53 radiographs (53 children). Measurements were compared between age groups (<2, 2-4.9, 5-7.9, ≥8 years). A longitudinal subgroup (33 children) had at least two radiographs with a minimum one-year interval. Changes of measurements over time were recorded.
RESULTS: In the cross-sectional group, significant increase of migration percentage after 8 years of age was noted. Higher migration percentage was noted with higher Gross Motor Function Classification System level (levels IV and V).
In the longitudinal group, 10 hips had a higher migration percentage (change >8%) over follow-up. At the last visit, higher migration percentage was noted in Gross Motor Function Classification System levels IV and V.
CONCLUSION: In congenital central hypotonia, hip subluxation can develop without pain or movement limitation. Even with early normal radiographic screening (<5 years of age), the risk of subluxation remains. Consistent radiographic follow-up is recommended with longer intervals than in spastic hips (every 2-4 years) since displacement in most hypotonic hips tends not to progress.
KEYWORDS: congenital central hypotonia, hip subluxation, hip surveillance, migration percentage
INTRODUCTION
resistance to passive movement.1 Tone assessment has been reported as part
of the clinical neurologic examination
of infants.1,2 Congenital hypotonia can result from different pathologies along
the neural pathway that connect the brain with the muscles.1,2 When congenital hypotonia is diagnosed, the next goal becomes to localize the underlying cause.2 Central hypotonia results from a lesion
or condition that affects the upper motor neuron, whereas peripheral hypotonia
is diagnosed when the underlying cause involves the lower motor neuron.2 Despite the advances in diagnostic techniques, challenging.1,2 Central causes of hypotonia
1
Hypotonia has attracted the attention
of pediatricians and neurologists since the early 1900s.3 Recent advances in molecular and genetic technology have 4 Since the classic article by Little about spastic rigidity in 1862,5 the orthopaedic literature has focused more on studying the spastic form of muscle tone abnormalities. Although many reports have described the orthopaedic disorders in children with Down syndrome, which is considered a form of congenital central hypotonia, no studies have reported the less common forms of central hypotonia.
The possibility of hip instability in hypotonic conditions has been reported.6
Muscle imbalance around the hip joint has
based on the available reports about the hip in children with Down syndrome.7
The aim of this study was to report the natural history of the hip in a group of children with congenital central hypotonia for diagnoses other than Down syndrome. The clinical characteristics, as well as the surgical procedures and outcomes, are reported.
PATIENTS AND METHODS
After obtaining the approval of our Institutional Review Board, records
of all children with congenital central hypotonia, seen at our institution between 2004 and 2014, were reviewed. Children with Down syndrome were
76
Del Med J | March/April 2019 | Vol. 91 | No. 2
Abstract
