ESCOBAR SYNDROME MULTIPLE PTERYGIUM SYNDROME ASSOCIATED WITH SCOLIOSIS AND KYPHOSIS : A CASE REPORT

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ESCOBAR SYNDROME (MULTIPLE PTERYGIUM SYNDROME)
ASSOCIATED WITH SCOLIOSIS AND KYPHOSIS A CASE
REPORT
M. Bulent Balioglu, Aysegul Bursali, M.Akif
Kaygusuz, Ali Oner, U.Selcuk Aykut Ministry of
Health Metin Sabanci Baltalimani Bone Disease
Hospital, Istanbul, TURKEY
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Summery

• Escobar Syndrome (ES) is a rare hereditary
  disease that can affect the spine and lead to
  scoliosis and kyphosis.
• In this case report we evaluated the early
  clinical and radiological results of an 11 year
  old male patient who received posterior
  stabalization and fusion treatment.

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Introduction

• Escobar syndrome is an infrequent congenital
  anomaly characterized by pterygia (webbing),
  respiratory distress, and scoliosis.
• Other variable characteristics are intrauterine
  death, faciocranial dysmorphism, short stature,
  ptosis, low-set ears, arachnodactyly,
  cryptorchism in males, and vertical talus.
• This study presents a scoliosis patient with ES
  and the surgical treatment of this patients
  scoliosis.

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Methods

• An 11 year old patient was diagnosed with ES at a
  medical genetics clinic.
• His physical examination revealed that he had
  scoliosis, a high palate, ptosis, low-set ears,
  arachnodactyly, faciocranial dysmorphism, mild
  deafness, joint contractures and vertical talus.

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Methods

• The Cobb method measurements were 23 for T2
  T5, 78 for T5 L3 segments.
• The MRI study showed that L5 was sacralised, the
  cauda equine level was at L3.
• Surgical treatment was performed with T3 L1
  vertebrae posterior segmental instrumentation.

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Results

• Follow-up was performed nine months aftersurgery.
  
• Postoperative AP Cobb method measurements were
  33 for T5 L3 segments (correction 48.8).
• Lateral Cobb measurements were 53 for T3 - L1
  and 36 for T1 - T3 thereby showing the
  development of junctional Kyphosis at T1 - T3.

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Post-Op
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Conclusion

• This case demonstrates treatment of scoliosis of
  multiple pterygium syndrome.
• This patient's proximal thorasic area was
  effected by congenital bone fusion and thereby
  caused junctional Kyphosis after the surgery.

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Conclusion

• We believe that careful preoperative planning
  for specific patients and cautious posterior
  surgical techniques will result in a balanced
  spinal correction and restoration of pulmonary
  function.