Kaudal regresyon sendromu: Olgu sunumu
Küçük Resim Yok
Tarih
2001
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Erişim Hakkı
info:eu-repo/semantics/openAccess
Özet
Kaudal regresyon sendromu sakral ve/veya lumbar omurganın kısmen veya tamamen yokluğuyla karekterize, nadir bir konjenital spinal anomalidir. Çoğu olguda üriner ve fekal inkontinans eşlik eder. Diğer sistemlerle ilgili ek anomaliler de vardır. Burada klinik ve radyografik bulguları kaudal regresyon sendromuyla uyumlu, idrar ve gaita inkontinansı olan yedi yaşındaki kız olgu sunulmaktadır. Ek olarak meningosel, unikorn uterus, sol over ve tuba yokluğu ve sol alt ekstremite yokluğunun olması, olguyu diğer kaudal regresyon olgularından ayırmaktadır. Sakral meningosel nedeniyle önce nöroşirurjikal girişim uygulanan olguya, Mitrofanoff ve Malone prosedürleri, sentetik yama ile karın duvarı rekonstrüksiyonu, dermal sinüs traktı eksizyonu uygulanmış ve tam kontinans sağlanmıştır. Bu hastalar nadir olması ve patolojinin pek çok sistemi ilgilendirmesi bakımından ilginç olup multidisipliner yaklaşımla tedavi edilmelidirler. Erken tanı ve tedavi üriner traktusta geri dönüşü olmayan hasarı önlemede anahtardır ve yaşam konforunu artırır.
Caudal regression syndrome is a rare congenital spinal malformation that is characterized by partial or total absence of sacral and/or lumbar spine. Urinary and fecal incontinence coexist with most of the cases. Associated anomalies are also present with this syndrome. A seven years old female patient who had clinically and radiologically established caudal regression syndrome with urinary and fecal incontinence is presented. In addition, the presence of meningocele and unicorn uterus with the absence of left overy, tube and left lower limb distinguished this case from other types of caudal regression. Mitrofanoff and Malone procedures, reconstruction of the abdominal wall with synthetic material and excision of the dermal sinus tract were performed for the patient who had been previously operated for sacral meningocele, and complete continence was achieved. The rarity of the malformation and its associated anomalies involving multiple systems, makes this syndrome an intriguing situation, thus requiring multidisciplinary management. Early diagnosis and treatment is the key to prevent irreversible damage in the urinary tract and to improve the quality of life.
Caudal regression syndrome is a rare congenital spinal malformation that is characterized by partial or total absence of sacral and/or lumbar spine. Urinary and fecal incontinence coexist with most of the cases. Associated anomalies are also present with this syndrome. A seven years old female patient who had clinically and radiologically established caudal regression syndrome with urinary and fecal incontinence is presented. In addition, the presence of meningocele and unicorn uterus with the absence of left overy, tube and left lower limb distinguished this case from other types of caudal regression. Mitrofanoff and Malone procedures, reconstruction of the abdominal wall with synthetic material and excision of the dermal sinus tract were performed for the patient who had been previously operated for sacral meningocele, and complete continence was achieved. The rarity of the malformation and its associated anomalies involving multiple systems, makes this syndrome an intriguing situation, thus requiring multidisciplinary management. Early diagnosis and treatment is the key to prevent irreversible damage in the urinary tract and to improve the quality of life.
Açıklama
Anahtar Kelimeler
Konu Ataması Yapılmamış
Kaynak
Pediatrik Cerrahi Derg.
WoS Q Değeri
Scopus Q Değeri
Cilt
15
Sayı
3
