Summary
A five-and-one-half-year-old boy, referred for orthopaedic evaluation of spinal curvature, was diagnosed as having neurofibromatosis (von Recklinghausen's disease) with a 44-degree right thoracolumbar curve from T-10 to L-3. A soft-tissue mass was identified in the area of T-7 to T-12 and, when neurological signs developed, the mass was removed by extraperitoneal laparothoracotomy. Tumor root was noted to extend into the spinal canal, and subsequent surgery was undertaken to remove this tissue. There was no postoperative increase in the neurologic deficit, although the scoliosis progressed to the point where spinal fusion was required. This was followed by brace treatment to maintain correction during the growth years. Long-term follow-up (12 years) revealed that the patient's spine was balanced, the fusion mass appeared to be solid radiographically, and the neurological examination was normal.
Zusammenfassung
Bei einem \(5{\raise0.5ex\hbox{$\scriptstyle 1$}\kern-0.1em/\kern-0.15em\lower0.25ex\hbox{$\scriptstyle 2$}}\) Jahre alten Knaben, der zu einer orthopädischen Beurteilung seiner Wirbelsäulenverkrümmung überwiesen worden war, wurde eine Neurofibromatose (von Recklinghausen'sche Krankheit) diagnostiziert, mit einer rechtskonvexen thorakolumbalen Krümmung von 44 Grad, die sich von Th X his L III erstreckte. Im Bereich von Th VII bis Th XII wurde eine Weichteilmasse festgestellt, die, als sich neurologische Symptome entwickelten, durch eine extraperitoneale Laparo-Thorakotomie entfernt wurde. Es wurde dabei festgestellt, daß sich die Wurzel des Tumorgewebes bis in den Wirbelkanal erstreckte, weshalb anschließend auch eine operative Entfernung dieses Gewebes durchgefülhrt wurde. Postoperativ war keine Zunahme der neurologischen Ausfallserscheinungen zu verzeichnen, obwohl die Skoliose bis zu einem Schweregrad zunahm, der eine operative Fusion erforderte. Anschließend erfolgte eine Korsettbehandlung, um die Korrektur während der Wachstumsjahre zu erhalten. Die Langzeitbeobachtung (über 12 Jahre) ließ erkennen, daß die Wirbelsäulenverkrümmung kompensiert war. Die Versteifungsmasse stellte sich röntgenologisch als stabil dar and die neurologische Untersuchung ergab normale Befunde.
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Castelein, R.M., MacEwen, G.D. A dumbbell (hourglass) neurofibroma of the spine in a patient with von Recklinghausen's disease. Arch. Orth. Traum. Surg. 102, 216–220 (1984). https://doi.org/10.1007/BF00436132
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DOI: https://doi.org/10.1007/BF00436132