Spinal arteriovenous malformations associated with Klippel-Trenaunay-Weber syndrome: a literature search and report of two cases.

SUMMARY: Patients with Klippel-Trenaunay-Weber syndrome present with venous varices, cutaneous capillary malformations, and tissue hypertrophy, usually involving an extremity. A small but important subset also harbors arteriovenous malformations (AVMs) of the spine. We report 2 such cases, 1 with 3 concurrent spinal arteriovenous fistulas. These cases and our review of the literature emphasize the importance of screening the spine for AVMs. In addition, it is also important to investigate for the presence of multiple spinal AVMs.

Failure of a saphenous vein extracranial-intracranial bypass graft to protect against bilateral middle cerebral artery ischemia after carotid artery occlusion: case report.

OBJECTIVE AND IMPORTANCE: We present the case of a patient who experienced bilateral middle cerebral artery infarctions after Hunterian ligation and trapping of a ruptured right cavernous aneurysm, despite a high-flow extracranial-intracranial bypass. This is a rare complication, and it highlights the need for further refinements in our understanding of the hemodynamic insufficiency created by major vessel sacrifice. CLINICAL PRESENTATION: The patient was a 59-year-old woman who experienced multiple episodes of massive epistaxis before undergoing angiography, which revealed left internal carotid artery occlusion and an irregular right cavernous aneurysm. The patient was then transferred to our center for treatment. The patient was neurologically intact at presentation, and her epistaxis was controlled by nasal packing. INTERVENTION: The patient underwent an extracranial-intracranial bypass from the external carotid artery to the M2 segment of the right middle cerebral artery, followed by trapping of the aneurysm. Despite evidence of graft patency, the patient experienced bilateral middle cerebral artery distribution infarctions after surgery. CONCLUSION: Although extracranial-intracranial bypasses protect the majority of patients who undergo carotid artery ligation from ischemic complications, this case demonstrates that hemodynamic insufficiency can occur even with a high-flow saphenous vein graft. Better ways to quantitate the hemodynamic needs of the brain after major vessel sacrifice may facilitate matching of the revascularization strategy to the specific needs of each patient, thus further reducing the likelihood of ischemic complications.

Resection of a large arteriovenous fistula of the brain using low-flow deep hypothermic cardiopulmonary bypass: technical case report.

OBJECTIVE AND IMPORTANCE: We present the second report in the literature on the use of low-flow hypothermic cardiopulmonary bypass to aide in the surgical resection of a large intraparenchymal arteriovenous fistula. CLINICAL PRESENTATION: The patient was a 46-year-old man who was found to have a left sylvian arteriovenous fistula with a giant varix during a workup for chronic left frontal headaches and was referred to our center for management. A cardiac workup revealed a cardiac output of 9 L per minute. INTERVENTION: Endovascular embolization of the lesion was initially attempted without success because of the high flow within the lesion and the large diameter of the feeding arteries. We then planned combined and staged endovascular and surgical approaches to gradually eliminate the fistula. Endovascular embolization, both transarterial and transvenous, could not be performed because of the high flow in the fistula. Despite the stepwise reduction of flow during the course of several weeks via surgical exposures and arterial ligations, the fistula remained difficult to remove because of its size and the turgor of the varix. Once hypothermic low-flow circulatory bypass was used, however, decompression of the sac allowed access to the afferent vasculature. CONCLUSION: The use of low-flow hypothermic circulatory bypass can facilitate the surgical extirpation of certain large intraparenchymal arteriovenous fistulas.

Delayed facial weakness after microvascular decompression of cranial nerve VII.

BACKGROUND: Retromastoid craniectomy and microvascular decompression of cranial nerve VII for hemifacial spasm is a well accepted and effective treatment. Risks of the operation relate to the surgical approach in general and to the seventh nerve in particular. Delayed facial weakness is an unusual and little-described complication of the procedure. The purpose of this review is to describe this complication and the characteristics of the patients so affected. METHODS: Between 1972 and 1996, 985 patients have undergone microvascular decompression for hemifacial spasm. During this time, 28 patients (2.8%) undergoing decompression of the facial nerve and 1 patient undergoing decompression of the cochlear nerve for tinnitus developed delayed facial palsy. RESULTS: The weakness was at least a House Grade III or worse and was complete in 11 of the patients. The time to occurrence averaged 12 days, with a tight range of 7 to 16 days. There were no factors such as duration of symptoms, intraoperative findings, or preoperative botulinum injections that were predictive of this postoperative weakness. In all patients there was almost complete recovery (House Grade I or II). CONCLUSIONS: Delayed facial weakness after MVD of CN VII can occur in up to 3% of cases. The onset of weakness after operation is consistent in its timing, occurring on average 12 days after the procedure. Although the etiology of this complication is uncertain, the palsy spontaneously resolves with a good or excellent outcome.

Intraoperative aneurysm rupture and complication avoidance.

The unexpected rupture of an intracranial aneurysm is a potentially catastrophic event. Strategies to control intraoperative aneurysm hemorrhage are based on sound surgical principles and take into consideration such variables as the timing, location, and severity of the rupture. Proven, successful techniques to prevent or control complications during aneurysm surgery are discussed in this article.

Treatment of aneurysmal subarachnoid hemorrhage.

Subarachnoid hemorrhage is a formidable and common health care problem. Early diagnosis and management are crucial to reduce the morbidity form this complex and multifaceted disease. Open surgery and endovascular techniques both aim at eliminating the source of hemorrhage. The choice of therapy can be made rationally based on an understanding of the merits, risks, and limitations of each therapy. The care of pregnant patients with subarachnoid hemorrhage and patients who harbor both aneurysms and AVMs can be approached rationally with an understanding of the complex pathophysiology behind these clinical scenarios. Familiarity with the signs of mild SAH, and advances in familial screening, noninvasive imaging, and therapies for vasospasm will continue to lessen the toll of this dramatic illness on the public well-being.

Swallowing dysfunction in patients receiving prolonged mechanical ventilation.

Several studies have suggested that swallowing dysfunction and pulmonary aspiration occur in patients receiving prolonged ventilation. However, the incidence of swallowing dysfunction, its rate of resolution, and the sensitivity of tests used to characterize swallowing abnormalities are not well defined. The goals of our study were to evaluate swallowing function in this group of patients by (1) defining the specific swallowing abnormalities that occur in this patient population, (2) comparing the sensitivity of bedside evaluations to modified barium swallow with videofluoroscopy (MBS/VF), (3) performing endoscopic evaluation of the upper airway to characterize glottic function during swallowing, (4) evaluating the relationship between swallowing dysfunction and neuromuscular disorders, and (5) studying the temporal resolution of swallowing abnormalities. Swallowing function was evaluated in 35 patients receiving prolonged ventilation (ie, > or = 3 weeks) admitted to a specialized rehabilitation unit dedicated to the care of patients requiring prolonged ventilation. The average age of the 35 patients was 61 +/- 15 years. The total duration of intubation at the time of the initial swallowing evaluation was 29 +/- 34 days via a cuffed tracheostomy tube and 15 +/- 9 days via an endotracheal tube. Neuromuscular disorders were present in 16 patients (45%). Thirty-four percent of the patients had at least one swallowing abnormality detected by bedside examination. Results of bedside swallowing examination were abnormal in 31% of patients with a neuromuscular disorder and 37% of patients without a neuromuscular disorder. MBS/VF was abnormal in 83% of patients (85% in patients with and 80% in patients without a neuromuscular disorder). Results of early (< 1 month) repeated MBS/VF examinations usually remained unchanged; however, in a small group of patients, later studies (> or = 1 month) revealed significant improvement. In 50% of patients who underwent direct laryngoscopy, important abnormalities were found that contributed to swallowing dysfunction. Our data show that patients requiring prolonged mechanical ventilation have a high incidence of swallowing abnormalities, regardless of the presence or absence of neuromuscular disorders. MBS/VF and direct laryngoscopy can provide useful information about laryngeal action and swallowing dysfunction, and can facilitate the implementation of corrective actions to prevent respiratory complications.

Complications of Swan-Ganz catheterization for hemodynamic monitoring in patients with subarachnoid hemorrhage.

Invasive hemodynamic monitoring has become standard in the management of aneurysmal subarachnoid hemorrhage. This study is a retrospective analysis of 630 Swan-Ganz catheters placed in 184 patients with aneurysmal subarachnoid hemorrhage. Evaluation of complications demonstrated a 13% incidence of catheter-related sepsis (81 of 630 catheters), a 2% incidence of congestive heart failure (13 of 630 catheters), a 1.3% incidence of subclavian vein thrombosis (8 of 630 catheters), a 1% incidence of pneumothorax (6 of 630 catheters), and a 0% incidence of pulmonary artery rupture. In the management of patients with aneurysmal subarachnoid hemorrhage, invasive hemodynamic monitoring continues to be an important tool with acceptable complications.

The outcome and techniques of primary and secondary tracheoesophageal puncture.

OBJECTIVES: To evaluate the outcome of primary vs secondary tracheoesophageal puncture (TEP), in particular the effects of preoperative and postoperative radiotherapy on success and complication rates in primary TEPs, and to highlight modified surgical and management techniques. DESIGN: Retrospective study of case series. SETTING: Chevalier Jackson-Norris Center-Department of Otorhinolaryngology and Bronchoesophagology at Temple University Health Sciences Center Hospital, Philadelphia, Pa. PATIENTS: One hundred six consecutive patients underwent primary TEPs and 30 underwent secondary TEPs for voice restoration after laryngectomy for cancer over a period of 8 years with follow-ups ranging from 6 months to 8.5 years. The group given primary TEP also includes 19 patients who received radiation for cure and salvage laryngectomy and 75 who received full-course postoperative radiotherapy. INTERVENTION: Tracheoesophageal puncture and Blom-Singer prosthesis. MAIN OUTCOME MEASURES: Speech measures including (1) voice intensity, (2) pitch of speech, (3) duration of sustained phonation, and (4) rate of speech. RESULTS: A success rate of 93% was achieved in the group of patients given primary TEP regardless of radiotherapy. An 83% success rate was achieved with patients given secondary TEP. There were no major complications related to TEPs. CONCLUSIONS: Primary TEP for patients requiring total laryngectomy is highly recommended since a second operative procedure can be avoided and speech obtained rapidly. Postoperative radiotherapy does not increase the complication rate from TEP.

Diagnosis and management of occipital condyle fractures.

The authors recently treated three cases involving fractures of the occipital condyle. First described by Bell in 1817, this lesion has proven to be very rare, with only 32 cases previously reported in the literature. Plain films often do not reveal any abnormality, making diagnosis difficult. High-resolution computed tomography has been demonstrated to be very sensitive in diagnosing this lesion. This fact was borne out in the authors' series. All of the authors' patients were managed either with a Philadelphia collar or with halo fixation, with excellent outcomes.

Cerebral syrinx with Chiari I malformation.

A 13-year-old female presented with acute left gaze paralysis. MRI revealed hydrosyringomyelia (HSM) with syringobulbia in the left pons extending cephalad into the centrum semiovale. A suboccipital craniotomy was performed and the foramen of Magendie was imperforate. Ocular movements normalized in 2 months and postoperative MRI confirmed resolution of the cerebral syrinx and syringobulbia and diminished HSM. This case represents an extreme example of the altered spinal fluid dynamics with Chiari I malformation. Theories on HSM are reviewed.