William E. Bolger

Nasal Cerebrospinal Fluid Leaks: Critical Review and Surgical Considerations

Otolaryngologists have assumed a major role in the evaluation and management of anterior skull base defects that result in cerebrospinal fluid (CSF) leaks and meningoencephaloceles. To achieve the best possible results for patients with CSF leaks, a thorough understanding of the underlying pathophysiology and a critical analysis of management principles and treatment options is essential. Surgical and medical management of these patients is highly individualized and depends on a number of factors, including etiology, anatomic site, patient age, and underlying intracranial pressure. This review article will highlight the history, physiology, pathophysiology, diagnosis, surgical techniques, and postoperative care relevant to nasal CSF leaks and encephaloceles.

Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks.

Background Spontaneous cerebrospinal fluid (CSF) leak is a condition that previously has been considered idiopathic and classified as having “normal” intracranial pressure (ICP). We present clinical and radiographic evidence that indicates elevated ICP in this group. In addition, we review the pathophysiology and unique management issues in caring for patients with spontaneous CSF leak. Methods We present a retrospective review of medical records, imaging studies, ICP measurements, and surgical treatment of patients with spontaneous CSF leaks. Results Sixteen patients with spontaneous CSF leaks were surgically treated from 1996 to 2002. Ten patients underwent postoperative lumbar puncture with CSF pressure measurement during clinically indicated computed tomography cisternograms. Intracranial pressures were elevated in all 10 patients, with a mean of 26.5 cm H2O and a range of 17.3–34 cm H2O, (normal, 0–15 cm H2O). Demographically, 13/16 patients were women, all were middle-aged with a mean age of 49.6 years, and 15/16 patients were obese with a mean body mass index of 35.9 kg/m2. Radiographically, 15 patients had imaging of the sella turcica, 10 patients had completely empty sellas, and 5 patients had partially empty sellas. Surgical repair was 100% successful in leak cessation with a mean follow-up of 14.1 months. Conclusion Although the precise cause and mechanism of spontaneous CSF leaks is not fully understood, this study sheds light on important factors to consider. Patients with this condition have similar physical and radiographic findings such as middle-aged, female gender, obesity, and empty sella. Additional investigation is needed to determine the exact cause of the condition, its relationship to elevated ICPs, and if further medical or surgical treatments to correct the intracranial hypertension are warranted.

Significance of Empty Sella in Cerebrospinal Fluid Leaks

OBJECTIVE: The role of elevated cerebrospinal fluid (CSF) pressures in the pathophysiology of various CSF leaks is not clear. Empty sella syndrome (ESS) is a radiographic finding that can be associated with elevated CSF pressures and may represent a radiographic indicator of intracranial hypertension. We present our experience with CSF leaks of various causes, the prevalence of ESS in the spontaneous and nonspontaneous categories, and the potential pathophysiology and unique management issues of the spontaneous CSF leak group. METHODS: We conducted a retrospective review of medical records, imaging studies, and surgical treatment of CSF leaks in patients treated by the senior author. RESULTS: Sixteen patients with spontaneous CSF leaks and 12 patients with nonspontaneous CSF leaks were surgically treated from 1996 through 2002. In the spontaneous group, 15 patients had complete imaging of the sella turcica. Ten had completely empty sellae and 5 had partially empty sellae, for a total of 100% (15 of 15). In the nonspontaneous group, 9 patients had complete imaging of the sella. Only 11% (1 of 9) had a partially empty sella and that was a congenital leak. Comparison of proportions between these 2 groups was significant (P = 0.01). The spontaneous group consisted primarily of obese, middle-aged females (13 of 16 patients). CONCLUSION: Empty sella probably represents a sign of elevated intracranial pressure that leads to idiopathic, spontaneous CSF leaks. Spontaneous CSF leaks are strongly associated with the radiographic finding of an empty sella and are more common in obese females, similar to benign intracranial hypertension. This unique population may require more aggressive surgical and medical treatment to prevent recurrent or multiple leaks.

Endoscopic transpterygoid approach to the lateral sphenoid recess: surgical approach and clinical experience.

OBJECTIVE: Pathology within a lateral recess of a widely pneumatized sphenoid sinus is difficult to access with the use of traditional open and current endoscopic surgical approaches. A new surgical procedure, the endoscopic transpterygoid approach, directly accesses this region. A clinical experience over several year with this approach is reported as well as a refined and updated description of the technique. SETTING: Tertiary care center. METHODS: Retrospective review of medical records of patients treated with the endoscopic transpterygoid approach to the lateral sphenoid recess. RESULTS: An endoscopic transpterygoid approach was performed in 9 patients, 6 to resect a middle fossa meningoencephalocele and repair the CSF leak and associated skull base defect, 2 for possible invasive fungal sinusitis, and 1 to marsupialize a symptomatic epidermoid cyst. Patients tolerated the approach well and no significant complications occurred. CONCLUSIONS AND SIGNIFICANCE: In selected cases, the endoscopic transpterygoid approach enables the otolaryngologist to meet modern demands to treat conditions in the lateral sphenoid using minimally invasive techniques that are well-tolerated by patients. The surgical approach and the initial experience described herein is presented in the hopes that it can aid fellow otolaryngologists to help patients with challenging conditions in this region of the body.

Cerebrospinal Fluid Pressure Monitoring after Repair of Cerebrospinal Fluid Leaks

OBJECTIVE: To measure intracranial pressures (ICPs) via lumbar drains after surgical repair of cerebrospinal fluid (CSF) leaks. METHODS: We conducted a retrospective review of ICP measurements through lumbar drains during the immediate postoperative period after CSF leak repair. RESULTS: Eight patients with spontaneous CSF leaks underwent surgery and postoperative CSF pressures were measured via lumbar drains. ICP was elevated in 7/8 patients (mean, 32.5 cm H2O). Diuretics reduced ICP (mean, 10 cm H2O). Three traumatic CSF leaks patients served as controls (mean, ICP 14 cm H2O). CONCLUSION: Measurement of ICP through lumbar drains provides important information regarding the pathophysiology of CSF leaks that has an impact on subsequent medical and surgical treatment. Although the precise cause and mechanism of spontaneous CSF leaks are not fully understood, this study indicates that elevated ICP plays a role and that further medical or surgical treatment to correct the intracranial hypertension may be warranted. (Otolaryngol Head Neck Surg 2004;130:443-8.)

Sphenoid Encephaloceles: Disease Management and Identification of Lesions Within the Lateral Recess of the Sphenoid Sinus†

Objectives Encephaloceles usually involve herniation of frontal lobe tissue through an anterior cranial fossa defect into the ethmoid sinus or nasal cavity. Encephaloceles can also result from temporal lobe herniation through a middle fossa defect into the sphenoid sinus. Within the sphenoid, encephaloceles are thought to occur most commonly in the central or midline aspect of the sinus. Lateral sphenoid encephaloceles, especially within the lateral aspect of the sphenoid sinus when the sphenoid sinus has pneumatized extensively into the pterygoid recess, are considered exceedingly rare. The objectives of the study were to review our experience with sphenoid encephaloceles to understand the relative frequency and the locations in which they occur within the sphenoid sinus and to report our experience in caring for patients with this condition.

Endoscopic resection of sinonasal malignancies: a preliminary report.

Background A minimally invasive endoscopic approach for the management of sinonasal malignancy offers several advantages including excellent illumination, maximal preservation of uninvolved vital structures, and sparing of facial incisions. The purpose of this study was to evaluate the outcome and morbidity of endoscopic resection with or without combined radiotherapy and/or chemotherapy. Methods Forty-seven patients with sinonasal malignancy were diagnosed and/or treated with an endoscopic approach at The Cleveland Clinic Foundation and The University of Pennsylvania Medical Center from 1996 to 2003. Nineteen patients fulfilled the study criteria and had a minimally invasive endoscopic resection. Fifteen patients were treated with curative intent and four patients underwent palliative resection. Results The mean age was 56.9 (9–78 years) years and the mean follow-up period was 26.4 months. Combined radiation with or without chemotherapy pre- or postoperatively was given to 15 of 19 (78.9%) patients. Thirteen patients were resected strictly with an endoscopic approach and six patients were resected in combination with neurosurgery. There were no peri- and postoperative deaths. The local recurrence (LR) rate was 26.3% (5/19) and the distant metastasis rate was 15.8% (3/19). Overall survival rate (OS) was 78.9% (15/19) at a mean follow-up duration of 32.1 (4–74 months) months. The disease-free survival (DFS) rate was 68.4% (13/19) by clinical, endoscopic, and radiographic surveillance at a mean follow-up duration of 33.1 months. Patients treated with curative intent had LR, OS, and DFS rates of 21.4, 85.7, and 85.7%, respectively, and the patients treated for palliation had LR, OS, and DFS rates of 40, 60, and 15%, respectively. Conclusion Minimally invasive endoscopic resection of sinonasal malignancy in combination with adjunctive therapies reduces treatment morbidity and yields LR, OS, and DFS rates that are comparable with traditional anterior craniofacial approaches.

Endoscopic and Endoscope-Assisted Resections of Inverted Sinonasal Papillomas

OBJECTIVE: To evaluate the success of endoscopic and endoscope-assisted resection of inverted sinonasal papillomas and to assess the indications for adjunctive external procedures. STUDY DESIGN: Retrospective chart review. RESULTS: Fifty patients were treated surgically during the 10-year study period and had greater than 1 year of follow-up care. These patients had a recurrence rate of 14% (7/50) with a mean follow-up of 31.1 months. Of these 7 patients with recurrences, 3 were treated primarily at this institution, and 4 had been treated prior to referral. Recurrence was 11% (3/27) for primary resections and 17% (4/23) for secondary resections. Average time to recurrence was 11.7 months. Of the patients, 38% (19/50) had adjunctive external procedures either during the initial resection or for the management of recurrent disease. CONCLUSIONS: Endoscopic and endoscope-assisted resections of inverted papilloma are effective techniques for managing inverted papilloma. The intraoperative findings, most importantly the site(s) of tumor attachment, dictate whether an endoscopic procedure is sufficient to complete resect the inverted papilloma or whether an adjunctive external procedure is required. Appropriate patient selection and an aggressive surgical approach are necessary for the management of these neoplasms.

Spontaneous nasal cerebrospinal fluid leaks and empty sella syndrome: a clinical association.

Background Spontaneous, idiopathic nasal meningoencephaloceles are herniations of arachnoid/dura and cerebrospinal fluid (CSF) through anatomically fragile sites within the skull base. Empty sella syndrome occurs when intracranial contents herniate through the sellar diaphragm filling the sella turcica with CSF and giving the radiographic appearance of an absent pituitary gland. The objective of this study was to examine the association between spontaneous encephaloceles/CSF leaks and empty sella syndrome because of their similar clinical features and potential common pathophysiology. Methods Retrospective. Results Sixteen patients were treated for spontaneous encephaloceles between 1996 and 2001. All 16 patients had associated CSF leaks. Five patients had multiple simultaneous encephaloceles. Fifteen patients with imaging of the sella turcica had empty (10 patients) or partially empty (5 patients) sellas. One patient did not have complete imaging of the sella. Three patients had lumbar punctures with measurement of CSF pressure during computed tomography cisternograms because of multiple skull base defects. Mean CSF pressure was 28.3 cm of water (range, 19–34 cm; normal, 0–15 cm). Thirteen of 16 patients (81%) were obese women (mean body mass index, 35.9 kg/m2; normal, <25 kg/m2). Mean follow-up was 14.2 months with 100% success in closure of the defects after one procedure. Conclusion Spontaneous meningoencephaloceles and CSF leaks are strongly associated with radiographic findings of an empty sella and suggest a common pathophysiology. The underlying condition probably represents a form of intracranial hypertension that exerts hydrostatic pressure at anatomically weakened sites within the skull base. Otolaryngologists should be familiar with this disease entity and the implications intracranial hypertension has on patient management.

Outcomes for endoscopic resection of sinonasal squamous cell carcinoma

Objectives: Preliminary data suggest efficacy for the endoscopic approach for sinonasal malignancy. The purpose of this study is to evaluate the effectiveness of the endoscopic approach for the resection of sinonasal squamous cell carcinoma (SCC).