Çağlar YILGÖR, İrem Nur EROĞLU, Nuray SÖĞÜNMEZ, Kadir ABUL, Ahmet ALANAY

Konjenital Skolyoz Tanı ve Tedavisi

Omurganın en sık görülen konjenital deformitesi konjenital skolyozdur. Deformiteyi oluşturan sebep, oluşum veya ayrışma kusuru veya bu iki bozukluğun birlikte olduğu karma tiptir. Kliniğe yansıması, stabil hemivertebradan, pulmoner, kardiyak, genitoüriner ve nörolojik komplikasyonlara yol açabilen karmaşık ve ilerleyici deformitelere kadar geniş bir aralıktadır. Tabloya sıklıkla diğer organ sistemlerindeki anomaliler eşlik eder. Tanıda; prenatal ultrason, fizik muayene ve röntgen kullanılır. Manyetik rezonans eşlik eden intradural patolojilerin ortaya konulmasında yardımcıdır. Bilgisayarlı tomografi cerrahi planlamada yardımcı olabilir. Tedavi planı eğriliğin yeri ve derecesi, hastanın yaşı, deformitenin tipine ve öngörülen ilerleme beklentisine göre her hastaya özel olarak yapılır. Tedavi temel olarak kontrollü gözlem, konservatif ve cerrahi tedavilerden oluşur. Tek hemivertebra varlığında genellikle erken yaşta hemivertebrektomi ve limitli füzyon önerilir. Daha karmaşık deformitelerde hemiepifizyodez, distraksiyon temelli füzyonsuz cerrahi yöntemler veya bunların kombinasyonları kullanılabilir. Günümüzde teknolojik ilerlemeler sayesinde spinal deformite ve intradural patolojilerin cerrahisi eş zamanlı olarak güvenli şekilde yapılabilmektedir. İhmal edilmiş ileri deformitelerde düzeltici osteotomiler gerekebilir. Düzeltici osteotomiler barındırdıkları nörolojik yaralanma riski ve uygulamadaki teknik zorluklar nedeniyle donanımlı merkezlerde deneyimli cerrahlar tarafından gerçekleştirilmelidir

Diagnosis and Treatment of Congenital Scoliosis

Congenital scoliosis is the most common congenital spinal deformity caused by a failure in the formation or segmentation or both. Clinical manifestations may vary from stable hemivertebra to complex deformities that can lead to pulmonary, cardiac, genitourinary and neurological complications. Other system anomalies may accompany as well. Prenatal ultrasound, physical examination and x-rays are used for diagnosis. MR is helpful in detecting accompanying pathologies. CT may be useful in preoperative planning. The treatment plan is tailored to each patient according to location and magnitude of the curve, patient age, type of the deformity and the anticipated natural history. Treatment consists of controlled observation, conservative and surgical methods. Hemivertebrectomy and limited fusion are recommended at an early age for single hemivertebra. Hemiepiphysiodesis, distraction-based non-fusion methods or combinations of these may be used for more complex deformities. Technological advances today allow for concurrent surgeries for spinal deformity and intradural pathologies. Corrective osteotomies may be required in neglected severe cases. Due to the challenging technique and risk of neurological injury, corrective osteotomies necessitate well-equipped centers and experienced surgeons

Keywords:

Spine, congenital abnormalities, scoliosis, kyphosis, hemivertebrae,

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