Clival chordomas are rare, aggressive, notochord-derived tumors with an incidence
of approximately 8 per 10 million. Management consists of surgery, commonly with an
endoscopic endonasal approach (EEA), and adjuvant proton beam radiotherapy. After
resection, vascularized coverage of the ventral skull base is paramount to avoid complications
such as cerebrospinal fluid (CSF) leak and meningitis. This is often achieved using
a nasoseptal flap (NSF) at the time of initial surgery. NSF failure is estimated to
occur in at least 2% of cases, requiring alternative options using flaps from the
lateral nasal wall, temporoparietal fascia (TPF), nasal turbinates, or anterolateral
thigh (ALT).
We present the case of a 29-year-old patient with a history of intranasal substance
abuse, chronic rhinosinusitis and clival chordoma. She underwent subtotal resection
via EEA, a far lateral craniotomy for residual disease, reconstruction with bilateral
NSF, and adjuvant proton beam radiotherapy at an outside hospital in 2013. Six years
later, the patient presented with purulent meningitis and widespread skull base osteomyelitis
secondary to NSF necrosis, possibly due to insufflation of recreational drugs. She
underwent an EEA for debridement and drilling of the petrous osteomyelitis. A 1.5
cm × 1.5 cm dural defect with egress of CSF was noted. Review of prior proton fields
demonstrated relative sparing (2,600–4,200 cGy) of the bilateral pterygopalatine fossae.
To address this, a right-sided, vascularized, superiorly based inferior turbinate
mucoperiosteal flap was rotated posteriorly over the defect. On follow-up endoscopy,
there was no postoperative CSF leak. Blood and intranasal cultures were negative;
she completed a course of broad-spectrum IV antibiotics and was taken to surgery for
repeat debridement 8 weeks later. The right ITF was well-healed; however, there was
a small region of persistently exposed bone over the left petrous apex. This was drilled
widely, and a left-sided ITF was rotated posteriorly for coverage. At 6-month follow-up,
nasal endoscopy revealed full mucosalization without infection, dehiscence or CSF
leak. The patient's leukocytosis and inflammatory markers had normalized, and she
had returned to her prior neurological baseline with trace CN VII weakness and longstanding
unilateral CN XII weakness.
In this case, we utilized bilateral ITF to reconstruct a clival defect complicated
by acute osteomyelitis despite prior proton therapy to the skull base. Other reconstructive
options such as TPF and ALT flaps were discussed; however, the ITF was considered
to be most appropriate in the setting of infection and for reserving more complex
salvage flap options in case of ITF failure. This case highlights the utility of ITF
in reconstructing the ventral skull base for patients in whom nasoseptal flap reconstruction
has failed.
Fig. 1 T1 MRI image of chordoma prior to treatment. MRI, magnetic resonance imaging.
Fig. 2 Proton beam radiation fields demonstrating relative sparing of the pterygopalatine
fossa (2,600–4,200 cGy).
Fig. 3 (A) Intraoperative image of skull base ORN prior to debridement, (B) right inferior turbinate flap with persistent skull base dehiscence, (C) left inferior turbinate flap with well-healed defect.
Fig. 4 Follow-up MRI demonstrating enhancing bilateral inferior turbinate flaps and closure
of CSF fistula. MRI, magnetic resonance imaging.