What is the role of surgery in the treatment of Down syndrome?

Updated: Apr 30, 2018
  • Author: Gratias Tom Mundakel, MBBS, DCH; Chief Editor: Maria Descartes, MD  more...
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Answer

Timely surgical treatment of cardiac anomalies, detected during the newborn period or early infancy, may be necessary to prevent serious complications and is crucial for optimal survival.

Prompt surgical repair is necessary for GI anomalies, most commonly duodenal atresia and Hirschsprung disease. Other GI anomalies include tracheoesophageal fistula, pyloric stenosis, annular pancreas, aganglionic megacolon, and imperforate anus.

Surgical intervention may be necessary to reduce atlantoaxial subluxation and to stabilize the upper segment of the cervical spine if neurologic deficits are clinically significant.

Congenital cataracts occur in about 3% of children and must be extracted soon after birth to allow light to reach the retina. Afterward, appropriate correction with glasses or contact lenses helps to ensure adequate vision.

Surgical intervention in children with Down syndrome has a high risk of complications, particularly infection and wound healing problems. [66] Careful anesthetic airway management is needed because of the associated risk of cervical spine instability. Preoperative evaluation for anesthesia must include adequate evaluation of the airway and the patient’s neurologic status. Cervical radiography (with flexion and extension views) should be performed when any neurologic deficit suggests spinal-cord compression.

During laryngoscopy and intubation, the patient’s head should be maintained in a neutral position, and hyperextension should be avoided. Anticholinergics can be prescribed to control hypersecretion in the airways. Other airway complications include subglottic stenosis and obstructive apnea, which may result from a relatively large tongue, enlarged adenoids, and midfacial hypoplasia. Adenotonsillectomy may be performed to manage obstructive sleep apnea.


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