James K. Liu, Derald E. Brackmann and Johnny B. Delashaw Jr.
Ahmed M. Raslan, James K. Liu, Sean O. McMenomey and Johnny B. Delashaw Jr.
Staged resection of large vestibular schwannomas (VSs) has been proposed as a strategy to improve facial nerve outcomes and morbidity. The authors report their experience with 2-stage resections of large VSs and analyze the indications, facial nerve outcomes, surgical results, and complications. The authors compare these results with those of a similar cohort of patients who underwent a single-stage resection.
A retrospective review of all patients (age > 18 years) who underwent surgery from 2002 to 2010 for large (≥ 3 cm) VSs at the authors' institution with a minimum of 6 months follow-up was undertaken. A first-stage retrosigmoid approach (without meatal drilling) was performed to remove the cerebellopontine angle portion of the tumor and to decompress the brainstem. A decision to stage the operation was made intraoperatively if there was cerebellar or brainstem edema, excessive tumor adherence to the facial nerve or brainstem, a poorly stimulating facial nerve, or a thinned or splayed facial nerve. A second-stage translabyrinthine approach was performed at a later date to remove the remaining tumor. The single-stage resection consisted of a retrosigmoid approach with meatal drilling. Patient charts were evaluated for tumor size, extent of resection, tumor recurrence, House-Brackmann facial nerve function grade, and complications.
Twenty-eight and 19 patients underwent 2- or single-stage resection of a large VS, respectively. The average tumor size was 3.9 cm (range 3.2–7 cm) in the 2-stage group and 3.9 cm (range 3.1–5 cm) in the single-stage group. The mean follow-up was 36 ± 19 months in the 2-stage group versus 24 ± 14 months in the single-stage group. Gross-total or near-total resection was achieved in 27 (96.4%) of 28 patients in the 2-stage group and 15 (79%) of 19 patients in the single-stage group (p < 0.01). Anatomical facial nerve preservation was achieved in all but 1 patient (94.7%), and there were no recurrences on follow-up imaging in the 2-stage group. Good facial nerve functional outcome (House-Brackmann Grades I and II) at last follow-up was achieved in 23 (82%) of 28 patients in the 2-stage group and 10 (53%) of 19 patients in the single-stage group (p < 0.01). Cerebrospinal fluid leak–related complications (intracranial hypotension, blood patch, and lumboperitoneal shunt for pseudomeningocele) were more common in the 2-stage group. There were no postoperative strokes, hemorrhages, or deaths in either group.
The authors' results suggest that staged resection of large VSs may potentially achieve better facial nerve outcomes. There does not appear to be added neurological morbidity with staged resections.
James K. Liu, Yuan Lu, Ahmed M. Raslan, S. Humayun Gultekin and Johnny B. Delashaw Jr.
Cavernous malformations (CMs) of the optic pathway and hypothalamus (OPH) are extremely rare. Patients with these lesions typically present with chiasmal apoplexy, characterized by sudden visual loss, acute headaches, retroorbital pain, and nausea. Surgical removal is the recommended treatment to restore or preserve vision and to eliminate the risk of future hemorrhage. However, the anatomical location and eloquence of nearby neural structures can make these lesions difficult to access and remove. In this study, the authors review the literature for reported cases of OPH CMs to analyze clinical and radiographic presentations as well as surgical approaches and neurological outcomes.
A MEDLINE/PubMed search was performed, revealing 64 cases of OPH CMs. The authors report an additional case in the study, making a total of 65 cases. Each case was analyzed for clinical presentation, lesion location, radiographic features, treatment method, and visual outcome.
In 65 patients with OPH CMs, the optic chiasm was affected in 54 cases, the optic nerve(s) in 35, the optic tract in 13, and the hypothalamus in 5. Loss of visual field and acuity was the most common presenting symptom (98%), followed by headache (60%). The onset of symptoms was acute in 58% of patients, subacute in 15%, and chronic progressive in 26%. Computed tomography scans revealed hyperdense suprasellar lesions, with calcification visible in 56% of cases. Magnetic resonance imaging typically demonstrated a heterogeneous lesion with mixed signal intensities suggestive of blood of different ages. The lesion was often surrounded by a peripheral rim of hypointensity on T2-weighted images in 60% of cases. Minimal or no enhancement occurred after the administration of gadolinium. Hemorrhage was reported in 82% of cases. Most patients were surgically treated (97%) using gross-total resection (60%), subtotal resection (6%), biopsy procedure alone (6%), biopsy procedure with decompression (23%), and biopsy procedure followed by radiation (2%). Those patients who underwent gross-total resection had the highest rate of visual improvement (85%). Two patients were treated conservatively, resulting in complete blindness in 1 patient and spontaneous recovery of vision in the other patient.
Cavernous malformations of the OPH are rare and challenging lesions. Gross-total resection of these lesions is associated with favorable visual outcomes. Emergent surgery is warranted in patients presenting with chiasmal apoplexy to prevent permanent damage to the visual pathway.
James K. Liu, Aclan Dogan, Dilantha B. Ellegala, Jonathan Carlson, Gary M. Nesbit, Stanley L. Barnwell and Johnny B. Delashaw Jr.
Surgical intervention may be required if endovascular embolization is insufficient to completely obliterate intracranial dural arteriovenous fistulas (DAVFs). The authors report their 14-year experience with 23 patients harboring diverse intracranial DAVFs that required surgical intervention.
Between 1993 and 2007, 23 patients underwent surgery for intracranial DAVFs. The following types of DAVFs were treated: superior petrosal sinus (in 10 patients); parietooccipital (in 3); confluence of sinuses and ethmoidal (in 2 each); and tentorial, falcine, occipital, transverse-sigmoid, superior sagittal, and cavernous sinuses (in 1 patient each). In all cases, the authors' goal was to obliterate the DAVF venous outflow by direct surgical interruption of the leptomeningeal venous drainage. Transarterial embolization was used primarily as an adjunct to decrease flow to the DAVF prior to definitive treatment.
Complete angiographic obliteration of the DAVF was achieved in all cases. There were no complications of venous hypertension, venous infarction, or perioperative death. There were no recurrences and no further clinical events (new hemorrhages or focal neurological deficits) after a mean follow-up of 45 months.
The authors' experience emphasizes the importance of occluding venous outflow to obliterate intracranial DAVFs. Those that drain purely through leptomeningeal veins can be safely obliterated by surgically clipping the arterialized draining vein as it exits the dura. Radical excision of the fistula is not necessary.
James K. Liu, Kelly Mahaney, Stanley L. Barnwell, Sean O. McMenomey and Johnny B. Delashaw Jr.
The anterior condylar confluence (ACC) is located on the external orifice of the canal of the hypoglossal nerve and provides multiple connections with the dural venous sinuses of the posterior fossa, internal jugular vein, and the vertebral venous plexus. Dural arteriovenous fistulas (DAVFs) of the ACC and hypoglossal canal (anterior condylar vein) are extremely rare. The authors present a case involving an ACC DAVF and hypoglossal canal that mimicked a hypervascular jugular bulb tumor.
This 53-year-old man presented with right hypoglossal nerve palsy. A right pulsatile tinnitus had resolved several months previously. Magnetic resonance imaging demonstrated an enhancing right-sided jugular foramen lesion involving the hypoglossal canal. Cerebral angiography revealed a hypervascular lesion at the jugular bulb, with early venous drainage into the extracranial vertebral venous plexus. This was thought to represent either a glomus jugulare tumor or a DAVF.
The patient underwent preoperative transarterial embolization followed by surgical exploration via a far-lateral transcondylar approach. At surgery, a DAVF was identified draining into the ACC and hypoglossal canal. The fistula was surgically obliterated, and this was confirmed on postoperative angiography. The patient's hypoglossal nerve palsy resolved.
Dural arteriovenous fistulas of the ACC and hypoglossal canal are rare lesions that can present with isolated hypoglossal nerve palsies. They should be included in the differential diagnosis of hypervascular jugular bulb lesions. The authors review the anatomy of the ACC and discuss the literature on DAVFs involving the hypoglossal canal.
James K. Liu, Maria Fleseriu, Johnny B. Delashaw Jr., Ivan S. Ciric, William T. Couldwell and Ph.D.
✓Cushing disease is considered an aggressive pituitary endocrinopathy because of the devastating effects from untreated hypercortisolemia. Although they are histologically benign, these adrenocorticotropic hormone (ACTH)-secreting pituitary tumors are associated with significant morbidity and premature death. Currently, transsphenoidal surgery is the primary treatment of Cushing disease associated with an ACTH-secreting pituitary tumor, resulting in remission rates ranging from about 50 to 90%. Some patients, however, will not achieve sustained remission after transsphenoidal surgery and can exhibit persistent or recurrent Cushing disease that requires multimodal treatment to achieve remission. In these patients, options for treatment include repeat transsphenoidal resection, radiation therapy (including conventional fractionated radiation therapy and stereotactic radiosurgery), and medical therapy. Despite undergoing multiple treatment modalities, some patients may ultimately require bilateral adrenalectomy for definitive treatment to eliminate hypercortisolemia associated with Cushing disease. In this article, the authors review the treatment options for patients who have persistent or recurrent Cushing disease after unsuccessful transsphenoidal surgery. The indications, current results reported in the literature, and complications of each treatment modality are discussed.
Eric H. Sincoff, James K. Liu, Laura Matsen, Aclan Dogan, Ilman Kim, Sean O. Mcmenomey and Johnny B. Delashaw Jr.
✓ The authors report a novel technique for the treatment of cholesterol granulomas. An extradural middle fossa approach was used to access the granuloma, with drainage through silastic tubes into the sphenoid sinus via the anteromedial triangle between V1 and V2.
Cholesterol granulomas occur when the normal aeration and drainage of temporal bone air cells is occluded, resulting in vacuum formation and transudation of blood into the air cells. This process results in anaerobic breakdown of the blood with resulting cholesterol crystal formation and an inflammatory reaction. Traditional treatment of this lesion involves extensive drilling of the temporal bone to drain the granuloma cyst and establish a drainage tract into the middle ear. Such drainage procedures can be time consuming and difficult, and potentially involve structural damage to the inner ear and facial nerve. An extradural middle fossa approach provides easy access to the granuloma and anterior petrous bone entry into the granuloma for resection. Granuloma drainage is then achieved using shunt tubing in the sphenoid sinus via a small hole in the anteromedial triangle between V1 and V2.
Five patients with symptomatic cholesterol granuloma were treated without complication using this novel extradural middle fossa approach. One patient required reoperation 1-year postoperatively for cyst regrowth and occlusion of the drainage tube. At the 5-year follow-up examination, no patient reported recurrent symptoms.
Extradural middle fossa craniotomy and silastic tube drainage into the sphenoid sinus is a viable alternative method for treatment of cholesterol granuloma.