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Surgical treatment for cervicomedullary compression among infants with achondroplasia

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Dr. Shimony's team published on the 17th of February 2015 in the Childs Nerv. Syst. journal, an article about respiratory failure being responsible for most deaths among children with achondroplasia and that it is often related to cervicomedullary compression (CMC). His team presented their experience with early cervicomedullary decompression in infants with achondroplasia.

Here is a summarized and commented version of the abstract:

"METHODS:


Data was retrospectively collected for infants with achondroplasia who underwent CMC decompression between 1998 and 2013. Data included presurgical and postsurgical neurological examinations, MRI scans, and sleep study results."

RESULTS:


- 10  infants were included.


- Ages at surgery were 4 to 23  months (12.5  ±â€‰6.88  months).


- All infants displayed neurological findings prior to surgery, although often subtle." 


This is a crucial point, once many children don ´t show explicit signs of cervicomedullary compression even when it exists already and it ´s compromising the medulla at that point.


- All infants underwent a foramen magnum opening with a wide C1 laminectomy.


- Following surgery, 7  patients (70  %) demonstrated improved neurological status, and one displayed neurological deterioration.


- Seven patients demonstrated improved sleep quality 1  year after surgery. These patients had a good or improved neurological status following surgery.


- Preoperative radiological findings included abnormal hyperintense T2 changes in all children (improved following surgery in six children), brainstem distortion in four children (improved in all), and diminished cerebrospinal fluid (CSF) spaces at the level of the foramen magnum  in eight children (improved in seven).


- One child with extensive preoperative T2 changes accompanied by neurological and respiratory decline, deteriorated following surgery, and remains chronically ventilated.


This is a risk, among others, when the surgery is done after the medulla has been compromised.



"CONCLUSIONS:


Infants with achondroplasia are prone to neurological and respiratory symptoms. We believe that early diagnosis and early surgery for decompression of the foramen magnum and C1 lamina can alleviate respiratory symptoms, improve neurological status, and perhaps prevent sudden infant death in this population."


Source:

  1. Shimony, N., et al., Surgical treatment for cervicomedullary compression among infants with achondroplasia. Child's Nervous System, 2015. 31(5): p. 743-750.

     
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