Rett Syndrome: Meeting the Challenge of This Gender-Specific Neurodevelopmental Disorder
from Medscape General Medicine 1(2), 1999 Posted 01/13/1997 Daniel G. Glaze, MD, Rebecca J. Schultz, MSN, RN, CPNP, The Rett Center, Baylor College of Medicine, Houston, Tex. Abstract and Introduction Abstract Rett syndrome (RS) is an incurable neurological disorder that occurs in females. Although the biological basis is unknown, there is substantial evidence suggesting a genetic basis. RS is characterized by an initial period of apparently normal psychomotor development followed by loss of communication skills and purposeful hand movement. Then, hand stereotypies, gait dyspraxia, and deceleration of head growth become apparent. Other problems include scoliosis, growth failure and epilepsy. There is no biological marker for RS; the diagnosis is based on well-delineated clinical criteria. The prevalence of RS is 1:23,000 live female births. Survival to 30-40 years or beyond is the rule rather than the exception. Treatment is both palliative and supportive. A vigorous approach to all aspects of care, including educational, medical, and psychosocial issues, is recommended.

Scoliosis Associated With Typical Mayer-Rokitansky-Küster-Hauser Syndrome
from South Med J 93(2), 2000 Keri Fisher, PA-S, Richard H. Esham, MD, Ian Thorneycroft, PhD, MD, Departments of Physicians Assistant Studies, Medicine, and Obstetrics and GynecologyUniversity of South Alabama, Mobile.
Abstract
Disorders that cause congenital scoliosis include Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. We present the case of a 46-year-old karyotypical (XX) woman with müllerian agenesis (MRKH type A, typical form), a rudimentary bicornate uterus, a blind vaginal pouch, and adenocarcinoma of both ovaries with subsequent bilateral salpingo-oophorectomy. She also had scoliosis of the thoracic and lumbar spine, an association thus far seen only among patients with type B (atypical) MRKH. We describe typical and atypical forms of MRKH and emphasize how these various anomalies associated with müllerian agenesis have affected the classification of the syndrome. We also outline possible embryologic etiologies of müllerian agenesis.

Orthopaedics, June 2001 Journal Scan Review of article from Spine March 2001 (Volume 26, Number 5)
Radiologic Findings and Curve Progression 22 Years After Treatment for Adolescent Idiopathic Scoliosis: Comparison of Brace and Surgical Treatment With Matching Control Group of Straight Individuals
Danielsson AJ, Nachemson AL Spine. 2001;26(5):516-525
This article follows a consecutive series of patients treated for scoliosis between 1968 and 1977. One hundred thirty nine patients were surgically treated and 109 were brace-treated. The follow-up rates were very high at 89% and 86%, respectively, at an average follow-up period of 22-years. Treated patients were compared with a group of age- and sex-matched individuals without scoliosis. This article reports the radiologic results; functional and back pain evaluations will be published later.

Clinical Cases From Hospital for Joint Diseases
Adolescent Idiopathic Scoliosis
from Medscape Orthopaedics & Sports Medicine Posted 09/20/2002 Gerard K. Jeong, MD; Thomas J. Errico, MD
Introduction Scoliosis is defined as a spinal curvature in the coronal plane greater than 10° as measured by the Cobb method. However, scoliosis is a deformity that generally occurs in not only the coronal, but also the sagittal and transverse planes. Adolescent idiopathic scoliosis represents a subset of idiopathic scoliosis, which is categorized by the age of onset. By definition, the etiology of infantile (0-3 years old), juvenile (4-10 years old), and adolescent (> 10 years old) idiopathic scoliosis is unknown.

Effect of Grafting Technique on the Maintenance of Coronal and Sagittal Correction in Anterior Treatment of Scoliosis
from Spine 27(19):2129-2136, 2002 Posted 10/25/2002 Jean A. Ouellet, FRCS, Charles E. Johnston II, MD
Conclusions: Although the rib strut grafting technique improves the pseudarthrosis rate, as compared with morsellized graft, it did not affect the maintenance of correction or sagittal alignment. Adjunctive measures to provide truly structural interbody support (fusion cages, allograft rings, two-rod construct) appear to be required to address the shortcomings of anterior single-rod instrumentation.

Relations Between Standing Stability and Body Posture Parameters in Adolescent Idiopathic Scoliosis
from Spine 27(17):1911-1917, 2002 Posted 10/14/2002
Marie-Lyne Nault, BSc; Paul Allard, PhD, PEng; Sébastien Hinse, MSc; Richard Le Blanc, PhD; Olivier Caron, PhD; Hubert Labelle, MD; Heydar Sadeghi, PhD
Conclusions: The use of backward stepwise multiple correlations highlighted the interaction between several body parameters and their relation to standing stability in both able-bodied girls and scoliotic subjects. The scoliotic group displayed a much larger number of correlations between standing stability and body posture parameters than the nonscoliotic group. Standing imbalance was related to altered body posture parameters measured in the frontal and horizontal planes only. Although the correlation coefficients were relatively high, factors other than body posture parameters appeared related to standing imbalance in adolescent idiopathic scoliosis. These findings support the concept of either a primary or a secondary dysfunction in the postural regulation system of scoliotic subjects.

Reports of papers presented to the 17th Annual Meeting of the North American Spine Society
Spinal Deformity Update: The Lenke Classification of Adolescent Idiopathic Scoliosis CME Disclosures Thomas J. Errico, MD Gerard K. Jeong, MD Introduction The selection of fusion levels for spinal arthrodesis remains one of the most critical elements of preoperative planning in the surgical management of adolescent idiopathic scoliosis. Proper selection of fusion levels allows the spinal surgeon to achieve the preoperative goals of curve correction, maintenance of truncal balance, and preservation of motion segments. As there has been an increasing recognition and understanding of scoliosis as a 3-dimensional deformity occurring in the coronal, sagittal, and axial planes, a more comprehensive classification system has been introduced by Lenke and colleagues[1] to determine the extent of spinal arthrodesis. The Lenke classification system consists of 3 components: curve type (1-6), lumbar spine modifier (A, B, C), and thoracic sagittal modifier (-, N, +).

From Spine 28(15):1672-1677, 2003
Boston Brace Correction in Idiopathic Scoliosis: A Biomechanical Study
Posted 09/23/2003 Delphine Périé, PhD , Carl-Eric Aubin, PhD, Yvan Petit, PhD, Marie Beauséjour, MSc, Jean Dansereau, PhD, Hubert Labelle, PhD, MD
Conclusion: High thoracic pads reduced more effectively both thoracic and lumbar scoliotic curves than lumbar pads only. The study suggests that mechanisms other than brace pads produce correction and contribute to the force equilibrium within the brace.

Changes in Alignment of the Scoliotic Spine in Response to Lateral Bending
from Spine 28(7):693-698, 2003 Posted 04/23/2003 Murray J. Beuerlein, MSc, Vincent J. Raso, MASC, Douglas L. Hill, MBA, Marc J. Moreau, MD, James K. Mahood, MD
Conclusions: Lateral bending does not improve axial rotation in severe scoliosis (scoliosis for which surgical correction is advised). Structural changes including disc and vertebral wedging may be responsible for the lack of rotational correction of the scoliotic spine. Lack of axial flexibility in the thoracic region may hamper surgical attempts to correct the deformities of the trunk.

Correction of Adult Scoliosis Via a Posterior-Only Approach
from Neurosurg Focus 14(1), 2003. Posted 02/14/2003
Caleb R. Lippman, M.D., Caple A. Spence, M.D., A. Samy Youssef, M.D., Ph.D., David W. Cahill, M.D.
Conclusions: In many cases of adult scoliosis, a satisfactory multiplanar correction may be obtained via a single posterior approach and by using extended PLIF techniques. Cephalad adjacent-segment failure remains a significant problem in patients with osteoporosis, and routine extension of posterior instrumentation to the upper thoracic spine should be considered in these cases.

Evaluation and Management of Scoliosis from J Pediatr Health Care 17(1):42-44, 2003 Posted 02/24/2003 Ellen Taft, RN, MSN, CPNP, Richard Francis, MD

Idiopathic Scoliosis As a Presenting Sign of Familial Neurologic Abnormalities from Spine 28(1):40-45, 2003 Posted 02/05/2003 Masatoshi Inoue, MD, Yoshinori Nakata, MD, Shohei Minami, MD, Hiroshi Kitahara, MD, Yoshinori Otsuka, MD, Keijiro Isobe, MD, Masashi Takaso, MD, Makoto Tokunaga, MD, Takashi Itabashi, MD, Shinsuke Nishikawa, MD, Hideshige Moriya, MD
Conclusions: It is suggested that familial neurologic abnormalities may have a wide range of expression, and that some patients with "idiopathic" scoliosis present with genetically determined craniovertebral malformations such as syringomyelia, Chiari 1 malformation, and tonsillar ectopia.

Outlook Good in Untreated Idiopathic Scoliosis
Medscape Medical News
Feb. 4, 2003 — A landmark trial reported in the Feb. 5 issue of the Journal of the American Medical Association assessed the natural history of untreated late-onset idiopathic scoliosis (LIS) and showed that patients had little functional impairment, aside from back pain and cosmetic concerns. However, those with large Cobb angles may develop pulmonary problems. Report on JAMA. 2003;289:559-567, 608-609

Repair of Adult Scoliosis Effective Long Term
Medscape Medical News Laurie Barclay, MD
Feb. 28, 2003 — Repair of adult scoliosis is effective in providing pain relief, functional restoration, and patient satisfaction over the long term, according to a case series published in the February issue of Spine. This is the first report of long-term follow-up evaluation in adults since the advent of modern segmental instrumentation.

Aminocaproic Acid Reduces Perioperative Blood Loss in Scoliosis Repair Surgery
Medscape Medical News
Feb. 12, 2004 — Aminocaproic acid (Amicar) is a safe, effective, and inexpensive method of significantly reducing perioperative blood loss in patients undergoing scoliosis repair surgery, according to the Feb. 1 issue of Spine.