Endoscopic Resection of Skull Base Teratoma in Klippel-Feil Syndrome through Use of Combined Ultrasonic and Bipolar Diathermy Platforms

Abstract

Klippel-Feil syndrome (KFS) is associated with numerous craniofacial abnormalities but rarely with skull base tumor formation. We report an unusual and dramatic case of a symptomatic, mature skull base teratoma in an adult patient with KFS, with extension through the basisphenoid to obstruct the nasopharynx. This benign lesion was associated with midline palatal and cerebral defects, most notably pituitary and vertebrobasilar arteriolar duplications. A multidisciplinary workup and a complete endoscopic, transnasal surgical approach between otolaryngology and neurosurgery were undertaken. Out of concern for vascular control of the fibrofatty dense tumor stalk at the skull base and need for complete teratoma resection, we successfully employed a tissue resection tool with combined ultrasonic and bipolar diathermy to the tumor pedicle at the sphenoid/clivus junction. No CSF leak or major hemorrhage was noted using this endonasal approach, and no concerning postoperative sequelae were encountered. The patient continues to do well now 3 years after tumor extirpation, with resolution of all preoperative symptoms and absence of teratoma recurrence. KFS, teratoma biology, endocrine gland duplication, and the complex considerations required for successfully addressing this type of advanced skull base pathology are all reviewed herein.

Occipital dermal sinus associated with infectious teratoma in an adult patient affected by Klippel–Feil syndrome: Rare case report and literature review

“…In addition, most were subjected to surgical treatment; in two cases, sub-craniectomy was performed, seven cases reported total resection, two cases of partial resection without specifying the technique, two cases of puncture and resection, and one was not described. [ 3 ] In contrast, among the provided results, only two cases associate KFS with teratoma. This highlights the rarity of such occurrences.…”

“…Teratomas usually occur in the first two decades of life; however, few cases have been reported in adults. [ 2 , 3 ] Thus, among many particularities, the occurrence of teratoma in the posterior fossa in an adult is a rare feature of this case. It is composed of nongerminomatous germ cells that present different types of tissues derived from the endodermal, mesodermal, and ectodermal layers.…”

“…This anomaly may be associated with other anatomical abnormalities such as cleft palate, renal agenesis, malformation of the aortic arch, spina bifida, and other structural alterations in the brain. [ 3 ]…”