OBJECTIVES

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HYPOTHESIS:

Evaluation of long-term patient-perceived functional outcomes and quality of life (QOL) related to communication and eating with an emphasis on voice, speech production, and swallowing after cerebello-pontine angle (CPA) surgery.

STUDY DESIGN:

Prospective cross-sectional study.

METHODS:

The MD Anderson Dysphagia Inventory (MDADI), Voice Handicap Index (VHI), and Facial Clinimetric Evaluation (FaCE) surveys were distributed to patients who underwent CPA surgery between January 2008 and December 2010. Immediate postoperative cranial nerve function extracted from medical records was compared to long-term patient-perceived function and associated QOL.

RESULTS:

There was a 61% response rate with a mean postoperative period of 31.6 months (range 15-49). The presence of facial palsy in the postoperative period and the corresponding House-Brackmann (H-B) score were the strongest predictors of patient-perceived long-term function and QOL in all three domains (P <.005). Postoperative vagal palsy by comparison was not associated with long-term disturbance of voice or speech function. Postoperative dysphagia had a particularly large association with perceived long-term facial function and related QOL (P <.0005), with a smaller but significant impact on perceived swallow outcome (P <.05). After adjusting for other variables, the postoperative H-B score remained a significant predictor of perceived long-term facial and voice function and related QOL.

CONCLUSIONS:

Patients with severe facial dysfunction following surgery to the CPA are at increased risk for long-term self-reported difficulties with communication and eating, even with improvement of vagal function. Speech and swallow therapy should therefore be provided to these patients whether or not they also have pharyngeal dysphagia or voice disturbance.

LEVEL OF EVIDENCE:

2b. Laryngoscope, 2013.

PURPOSE:

To estimate the proportion of patients presenting with isolated third, fourth, or sixth cranial nerve palsy of presumed microvascular origin versus other causes.

DESIGN:

Prospective, multicenter, observational case series.

PARTICIPANTS:

A total of 109 patients aged 50 years or older with acute isolated ocular motor nerve palsy. TESTING: Magnetic resonance imaging (MRI) of the brain.

MAIN OUTCOME MEASURES:

Causes of acute isolated ocular motor nerve palsy (presumed microvascular or other) as determined with early MRI and clinical assessment.

RESULTS:

Among 109 patients enrolled in the study, 22 had cranial nerve III palsy, 25 had cranial nerve IV palsy, and 62 had cranial nerve VI palsy. A cause other than presumed microvascular ischemia was identified in 18 patients (16.5%; 95% confidence interval, 10.7-24.6). The presence of 1 or more vasculopathic risk factors (diabetes, hypertension, hypercholesterolemia, coronary artery disease, myocardial infarction, stroke, and smoking) was significantly associated with a presumed microvascular cause (P = 0.003, Fisher exact test). Vasculopathic risk factors were also present in 61% of patients (11/18) with other causes. In the group of patients who had vasculopathic risk factors only, with no other significant medical condition, 10% of patients (8/80) were found to have other causes, including midbrain infarction, neoplasms, inflammation, pituitary apoplexy, and giant cell arteritis (GCA). By excluding patients with third cranial nerve palsies and those with GCA, the incidence of other causes for isolated fourth and sixth cranial nerve palsies was 4.7% (3/64).

CONCLUSIONS:

In our series of patients with acute isolated ocular motor nerve palsies, a substantial proportion of patients had other causes, including neoplasm, GCA, and brain stem infarction. Brain MRI and laboratory workup have a role in the initial evaluation of older patients with isolated acute ocular motor nerve palsies regardless of whether vascular risk factors are present. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

For the past three decades, surgery of glomus jugulare tumors (GJTs) has been characterized by extensive combined head and neck, neuro-otologic, and neurosurgical approaches. In recent years, the authors have modified the operative technique to a less invasive approach for preservation of cranial nerves while achieving satisfactory tumor resection. We evaluated and compared the clinical outcomes of our current less invasive approach with our previous more extensive procedures. The clinical records of 39 cases of GJT surgically treated between 1992 and 2011 were retrospectively reviewed. The less invasive transjugular approach with Fallopian bridge technique (LI-TJ) was used for the most recent five cases. The combined transmastoid-transjugular and high cervical (TM-HC) approach was performed in 30 cases, while four cases were treated with a transmastoid-transsigmoid approach with facial nerve translocation. Operative technique, extent of tumor resection, operating time, hospital stay, and morbidity were examined through the operative records, and a comparison was made between the LI-TJ cases and the more invasive cases. No facial nerve palsy was seen in the LI-TJ group while the TM-HC group demonstrated six cases (17.6 %) of facial palsy (House-Brackmann facial nerve function grading scale grade II and III). The complication rate was 0 % in the LI-TJ group and 16.7 % in the more invasive group. The mean operative time and hospital stay were shorter in the LI-TJ group (6.4 h and 4.3 days, respectively) compared with the more invasive group (10.7 h and 8.0 days, respectively). The LI-TJ approach with Fallopian bridge technique provided adequate tumor resection with cranial preservation and definitive advantage over the more extensive approach.

Glomus tumors known as paragangliomas are neoplasms arising from the neural crest. They are named according to the place they originate from. Tumors originating from the carotid body at the carotid bifurcation are called Carotid Body Tumors (CBT). Surgical intervention is planned according to the Shamblin classification. 17 patients were operated after being diagnosed with CBT in our clinic between February 2007 and June 2010. 12 (70.5%) of the patients were male, and 5 (29.4%) of the patients were female. The average age was 42 (ages ranging between 32 and 47). Nine of the patients were diagnosed and treated with Shamblin type I tumor, seven of the patients with type II and one patient with type III. Only one patient had bilateral carotid tumor. In all patients with Shamblin type I and II, blunt dissection of the tumor was conducted smoothly by means of thermal cautery in the subadventitial plane. The patient with Shamblin type III had tumor invasion in the carotid artery and adjacent tissues were in an adherent state. Therefore mass resection was carried out by resecting 2 cm of the distal portion of the common carotid artery and 3 cm of the proximal portion of the internal carotid artery. 6 mm of synthetic polytetrafluoroethylene graft was interpositioned between the common carotid artery and the internal carotid artery. External carotid artery was anastomosed to this graft in an end-to-end fashion. The patient developed vocal cord paralysis postoperatively on the lesion side. The patient who underwent bilateral tumor excision developed Baroreflex Failure Syndrome. In the two patients thrombus developed in the internal carotid artery in the early postoperative period. These patients underwent thrombectomy and developed hemiplegia on the lesion side. One of them died on the seventh post-operative day while in follow-up in the intensive care unit. Surgical resection is the recommended treatment for carotid body tumors. Shamblin I and II type tumors' dimensions and pathological characteristics allow dissection. However Shamblin III tumors may require carotid artery resection and reconstruction due to tissue invasion. The possibility of post-operative cranial nerve paralysis and arterial thrombosis should be taken into account.

Object The purpose of this study was to evaluate the incidence, radiographic findings, relationship between presenting symptoms for treatment and drainage pattern, and treatment outcomes of hypoglossal canal dural arteriovenous fistula (HC-dAVF). Methods During a 16-year period, 238 patients underwent endovascular treatment for cranial dAVF at a single center. The incidence, radiographic findings, relationship between presenting symptoms for treatment and drainage pattern, and treatment outcomes of HC-dAVF were retrospectively evaluated. Results The incidence of HC-dAVF was 4.2% (n = 10). Initial symptoms were tinnitus with headache (n = 6), tinnitus only (n = 1), ocular symptoms (n = 1), otalgia (n = 1), and congestive myelopathy (n = 1). Presenting symptoms requiring treatment included ocular symptoms (n = 4), hypoglossal nerve palsy (n = 4), aggravation of myelopathy (n = 1), and aggravation of tinnitus with headache (n = 1). While the affected HC was widened in 4 of 10 patients, hypersignal intensity on source images was conspicuous in all 7 patients who underwent MR angiography (MRA). All ocular symptoms and congestive myelopathy were associated with predominant drainage to superior ophthalmic or perimedullary veins due to antegrade drainage restriction. All patients who underwent transvenous coil embolization (n = 8) or transarterial N-butyl cyanoacrylate (NBCA) embolization (n = 1) improved without recurrence. One patient who underwent transarterial particle embolization had a recurrence 12 months posttreatment and was retreated with transvenous embolization. Conclusions The incidence of HC-dAVF was 4.2% of all cranial dAVF patients who underwent endovascular treatment. Source images of MRA helped to accurately diagnose HC-dAVF. More aggressive symptoms may develop as a result of a change in the predominant drainage route due to the development of venous stenosis or obstruction over time. Transvenous coil embolization appears to be the first treatment of choice.

Objectives:

The aim of this investigation is to examine the causes, clinical picture, treatment, and prognosis of spontaneous intracranial hypotension, a rare cause of orthostatic headache, among the cases presenting in our clinic.

Methods:

Thirteen cases (5 males and 8 females), diagnosed with spontaneous intracranial hypotension in our clinic between January 1st, 2009 and October 30th, 2011, were included in this study. The presenting symptoms, treatment, findings on cranial magnetic resonance imaging, cerebrospinal fluid pressure measured at lumbar puncture (in available patients), and the healing period of the patients were recorded.

Results:

Five patients with orthostatic headache and accompanying symptoms were treated with bed rest, increase in oral fluid intake, intravenous hydration and caffeine, and experienced a complete recovery. Complete recovery was observed in two patients (15.3%) within 10 days, in another two (15.3%) within 15 days and in one patient (7.6%) within 21 days. Headache and other clinical symptoms significantly regressed within 30 days in four patients (37.6%) who received similar treatment, but a mild headache persisted intermittently during follow-up in these individuals. As the headache had not resolved after 30 days, an epidural blood patch was applied in these four cases (37.6%) and the clinical picture completely improved within 10 to 15 days.

Conclusion:

Spontaneous intracranial hypotension should primarily be suspected in cases complaining about postural headache and contrast-enhanced cranial imaging should be performed. The presence of cranial nerve paralysis and pyramidal tract signs should b considered. Conservative treatments should be considered initially, however if conservative treatments fail, epidural blood patches must be applied.

Traumatic carotid-cavernous sinus fistula (TCCF) is a rare but significant vascular abnormality in the skull base found after craniomaxillofacial trauma. Although the direct type is usually caused by trauma, the onset of symptoms in TCCF may present several weeks after injury. We present the case of a patient who sustained a blunt head injury from falling down and was hospitalized with skull base fracture associated with zygomatic complex fractures on the right side. After surgery, the recovery was uneventful and the patient was discharged without any problems. On the eighth week postoperatively, the patient returned to hospital presenting tinnitus, bruit on the right orbital area, diplopia, eye pain, and headache. The patient also had severe limitation of ocular movement on lateral gaze. After having brain angio-CT, which showed a dilated superior ophthalmic vein, the diagnosis of CCF with cranial nerve VI was confirmed. The fistula was occluded successfully by using coils. After the procedure, tinnitus, bruit, and headache were reduced immediately. On following up 4 months after coiling, cranial nerve VI palsy and related symptoms-diplopia and limit of ocular movement-were improved significantly. Although TCCFs usually manifest symptoms early after trauma, in this case, the patient presented clinical signs 8 weeks post-injury, while the longest time that was acknowledged in another previously released article was 6 weeks. According to this case, we recommend a careful follow-up until 2 months for patients with skull base fracture in order to rule out the risk of CCF.

Bone resorption is a known complication of cranioplasty after decompressive craniectomy (DC). A peculiar group of insidious, progressive, invalidating neurological symptoms was observed in patients presenting with incomplete resorption and abnormal mobility of the re-implanted bone. Such symptoms were similar, but with time more severe, to those encountered in the sinking flap syndrome. Are we facing a sort of Sinking Bone Syndrome? We accurately analyze these cases and review the literature. Over a 7-years period, 312 DCs were performed at our Institution. In 7 patients, headache, vertigo, gait ataxia, confusion, blurred speech, short-term memory impairment, hemiparesis, sudden loss of consciousness, and third cranial nerve palsy were observed in a time period ranging from 18 months to 5 years after cranioplasty. Clinical and neuroradiological examinations were performed to disclose the possible etiopathogenesis of this condition. Collected data showed partial resorption of the repositioned bone and its unnatural inward movements during postural changes. Bone movements were interpreted as the major cause of the symptoms. A new cranioplasty was then performed in every case, using porous hydroxyapatite in 6 patients and polyetherketone implant in the other. Full resolution of symptoms was always obtained 3 to 20 days after the second surgery. No further complications were reported. We believe that long-term follow up in patients operated on by cranioplasty after DC will be needed regularly for years after skull reconstruction and that newly appearing symptoms should never go underestimated or simply interpreted as a long-term consequence of previous brain damage.

Tick paralysis is a rare, but readily treatable condition that if missed can lead to significant morbidity and death. The classic clinical presentation of tick paralysis is the development of an unsteady, ataxic type gait followed by an acute symmetric ascending flaccid paralysis. Symptoms generally begin within 2 to 6 days of tick attachment. If the tick continues to feed, the weakness ascends to the upper extremities over a matter of hours, followed by cranial nerve involvement. Due to the similarity in its presentation, tick paralysis is often misdiagnosed as Guillain-Barré's syndrome, particularly the Miller Fisher's subtype, given its cranial nerve involvement. However, the weakness seen in tick paralysis progresses more quickly than what is generally seen in Guillain-Barré's syndrome and the protein concentration is not elevated in the cerebrospinal fluid. The mainstay of treatment for tick paralysis is tick removal. The time to full neurologic recovery after tick removal is estimated to be around 1.5 days with initial improvement generally within hours.

Abstract

Purpose:

In patients referred with blepharoptosis, the possibility of an underlying systemic cause for their ptosis can warrant a more detailed evaluation. The purpose of this study is to determine both the incidence and demographic characteristics associated with different types of ptosis in patients referred to the oculoplastics division at a tertiary care center.

Methods:

A retrospective chart review was performed on all patients referred to the oculoplastics division between 2007 and 2010. Final etiology for each patient's ptosis was determined based on history, standard eyelid measurements, and ancillary testing. Based on etiology, ptosis was categorized as aponeurotic, neurogenic, myogenic, traumatic, congenital, or mechanical. Demographics, including median age and sex were analyzed for patients in each category of ptosis.

Results:

Of the 251 patients, aponeurotic ptosis was the most common type of ptosis (60.2%), followed by traumatic (11.2%), congenital (10.4%), mechanical (8.8%), neurogenic (5.6%), and myogenic (4.0%). Of the neurogenic group, 35.7% of patients had cranial nerve 3 (CN 3) palsy, 28.6% had myasthenia gravis, 14.3% had aberrant regeneration, and 7.1% had Horner's syndrome. Thirty percent of the myogenic group had chronic progressive external ophthalmoplegia (CPEO). The congenital group had the youngest median age (10.5 years), yet the aponeurotic group had the oldest (62 years).

Conclusions:

A significant proportion of patients referred with ptosis had more serious conditions such as neurogenic or myogenic ptosis. Thus, clinicians should maintain a high degree of suspicion and thoroughly evaluate all patients with ptosis in order to properly assess for underlying systemic associations.