Maurice Klippel and Andre Feil first to describe syndrome in 1912 -Characterized by patients with: Short neck Low hairline Decreased cervical motion -Fewer than 50% with congenital defects of cervical spine have all three signs Wheeless' Textbook of Orthopaedics

- scoliosis occurs in the majority of patients w/ Marfan's syndrome, (over 60%) but curvatures significant enough to require treatment occur in only 20% of pts; - risk of progression: - scoliosis often occurs before age of 10 & may progress rapidly; - progression is more likely w/ curves greater than 20 deg in growing patients, and is more likely w/ curves more than 30-40 deg in adults; - associated conditions: - high-grade spondylolisthesis is additional spinal deformity that reportedly occurs with this syndrome. - dural ectasia and anterior myelomeningocele may be present & are thought to be caused by CSF pulsations against weakened dura; Wheeless' Textbook of Orthopaedics

Michael Vitale, MD
Surgical treatment of spinal conditions is changing at a rapid pace--perhaps more than any other field of orthopaedics. Minimally invasive techniques, new spinal implants, and genetically engineered biologic agents have already altered the practice of spinal surgery and promise to improve patient outcomes. Many studies presented at the 68th Annual Meeting of the American Academy of Orthopaedic Surgeons offered exciting new findings in this field.

Use of epidural analgesia for pain management after major spinal surgery Kumar et al JOS 2003 11(1) 67-72

Wheeless' Textbook of Orthopaedics
- cardiopulmonary problems (thoracic curves> 60-65 deg alter PFT's; - curves> 90 deg may cause myelopathy & may affect mortality; - progression is unlikely in curves <30 deg - single major thoracic curves> 60-80 deg are likely to increase in size even after growth was complete; - rate of progression is about one degree per year; - Indications for Fusion: - adult patients with scoliosis, chronic pain & curve> 60 deg; - because of higher complication rate in adults, radiograph or cosmetic appearance alone are not indications for surgery;

Spine fusion remains a common means of achieving stability in spinal surgery. Methods for obtaining fusion involve decortication of the host bed and introduction of a grafting material. Autologous bone has served as the standard material for these procedures in that its three cardinal properties encourage effective bone healing: osteogenic cells, an osteoconductive structure, and an osteoinductive matrix. Further, both cancellous bone with its increased surface area and osteogenic potential and cortical bone with its structural properties may be harvested. However, autologous bone suffers certain drawbacks, particularly a high rate of donor site morbidity, limited amounts of available bone, and the additional operative time required for harvest. For these reasons, intensive efforts have been directed toward developing alternative substances to either augment or substitute for autologous bone in spinal surgery. In this paper, we will examine some of the commonly used and emerging materials and their indications in spinal surgery.

Bone Healing and Spinal Fusion from Neurosurgical Focus Posted 02/10/2003 Julie G. Pilitsis, M.D., Ph.D., David R. Lucas, M.D., Setti R. Rengachary, M.D. Abstract Bone is a tissue that constantly undergoes deposition, resorption of stromal matrix, and remodeling. These processes may be altered by a variety of chemical, mechanical, cellular, and pathological mechanisms. Understanding the physiology of bone healing and the mechanisms affecting this process is important not only when evaluating normal skeletal development but also when initiating fracture repair. Because the ultimate success of spinal fusions involves creation of an osseous union, we focus this review on the anatomy and physiology of bone under physiological conditions, normal bone healing and mechanisms that alter it, and available adjuvant therapies that may enhance healing potential in a clinical setting.

Wheeless' Textbook of Orthopaedics
bracing has been the mainstay of non operative treatment of significant curves which have a potential to progress; - progression is related to size of curve, area of spine involved, & physiologic age of child;

Wheeless' Textbook of Orthopaedics
- vital capacity <75% (of predicted values) is seen in thoracic curves> 75 deg; - significant reductions in pulmonary function are seen in curves> 80 deg; - although some effect on pulmonary function can be seen at 60 deg, significant compromise is not seen until the curve is> 90 deg; - curves greater than 100 deg have total lung capacity of about 50% of normal; - w/ curves between 100 - 140 deg, average vital capacity is 60%; - restrictive lung disease and right sided heart failure become manifest at curves about 120 deg; - Effect of Bracing: (see bracing in scoliosis) - application of brace results in sig reduction in vital capacity (14%), f(x) residual capacity (22%), & total lung capacity (12%); - bracing will reduce lung function by 10 to 15%

Wheeless' Textbook of Orthopaedics
are most frequent at C 6-7 level but also occur at C 5-6 & to a lesser extent at C4-5 & other levels; - in relatively younger persons soft disk protrussion is more common than hard disk protrussion;

Wheeless' Textbook of Orthopaedics
Notes on treatment and references

Wheeless' Textbook of Orthopaedics
See also: - Crankshaft Phenomenon: - Epiphysiodesis - Hemivertebrae: - Pathogenesis - Spine Development - Segmented Vertebrae: - Spina Bifida - Spondylosis - Unilateral Bar - Associated Malformations

Wheeless' Textbook of Orthopaedics
Sciolosis: - w/ a mobile lumbar or thoracolumbar curve, CD instrumentation can provide permanent derotation and restoration of lumbar lordosis and thoracic kyphosis; - in other words there is improved saggital plane correction

Wheeless' Textbook of Orthopaedics
in skeletally immature pt, isolated posterior arthrodesis w/ instrumentation of a lordotic curve may act as a posterior tethering bar, producing lordosis & bending of the fusion mass as the unfused anterior vertebral bodies continue to grow;

Wheeless' Textbook of Orthopaedics
also known as Forestier's disease; - disease involves non-marginal syndesmophytes present at at three successful vertebral levels; - commonly there will be no disc & joint narrowing & nor osteoarthritis; - differentiated from AS, w/ marginal syndesmophytes; - more common in elderly men; - DISH is associated w/ low back pain and is more common in patients w/ diabetes and gout; - prevelance of DISH may be as high as 28%;