A Unique Case of Bilateral Recurrent Sphenoid Sinus Cerebrospinal Fluid Leaks: Primary Acquired Leak Within the Lateral Sphenoid Sinus Recess, Followed by a Leak via Sternberg's Canal.

OBJECTIVES: This case presents a previously undescribed clinical scenario of spontaneous cerebrospinal fluid (CSF) leaks secondary to a lateral sphenoid sinus recess skull base dehiscence and contralateral Sternberg's canal. This case report aims to characterize the presentation and successful management of these lesions. METHODS: The electronic medical record was used to collect information pertaining to the patient's clinical history. RESULTS: The patient was a middle-aged, obese female with persistent clear rhinorrhea as her only presenting symptom. Neuroradiologic studies localized the defect to the lateral sphenoid sinus recess. CSF opening pressures were within normal limits, but radiographic findings were consistent with elevated intracranial pressure. After an endoscopic transnasal transsphenoidal approach failed to resolve the CSF leak, a transpterygoid approach facilitated CSF leak resolution. The patient then did well for the following 2 years, but later developed a CSF leak through a contralateral Sternberg's canal. An endoscopic suprapterygoid procedure and ventriculoperitoneal shunt placement led to CSF leak resolution. CONCLUSION: This case demonstrates one of the only published examples of a sphenoid sinus CSF leak secondary to Sternberg's canal as it was originally described in the literature. Wide endoscopic surgical exposure and intracranial pressure management ultimately led to CSF leak resolution.

Spontaneous Resorption of a Penetrating Orbital Bone Fracture Fragment.

The authors describe a 20-year-old man who sustained multiple facial fractures in a high-speed motor vehicle crash, including a bone fragment from a skull base fracture that penetrated the orbital soft tissues superomedially. Serial CT scans documented spontaneous resorption over a 6-month period. While it is known that autologous bone grafts used in craniofacial reconstruction exhibit variable amounts of bone resorption, the complete resorption of an intraorbital fracture fragment has not been documented in the literature. His clinical care and the report of his case were undertaken in a fashion in accordance with the principles of the Health Insurance Portability and Accountability Act regulations.

Application of the Mimic Valsalva Maneuver with the Help of a Saccule in Cerebrospinal Fluid Rhinorrhea Reconstruction Surgery.

OBJECTIVE: To explore the mimic Valsalva maneuver with the help of a saccule handled by an anesthesiologist in order to locate the leakage channel and repair the fistula during intranasal endoscopic reconstruction surgery of cerebrospinal fluid rhinorrhea. METHODS: From 2012 to 2014, 8 patients were diagnosed with cerebrospinal fluid rhinorrhea by medical histories, physical and biochemical examination. All patients were treated with intranasal endoscopic reconstruction surgery of cerebrospinal fluid rhinorrhea. During the surgery, the mimic Valsalva maneuver with the help of a saccule was carried out once or twice by an anesthetist during the operation. Intranasal endoscopy was used to accurately locate the leakage site as shown by the exact fistula. Temporal fascia, fascia lata, middle turbinate mucosa and nasal septum mucosa were all used to repair the fistula. RESULTS: After the surrounding mucosa was removed, the exact leakage sites were accurately found. Fascia materials were used in all 8 patients. All patients were successfully treated after their first operation, and 1 patient was successfully treated by two operations with no complications and recurrences. All the patients were followed up for 1 month to 2 years. CONCLUSION: The convenient method of the mimic Valsalva maneuver with the help of a saccule handled by an anesthesiologist has a good prospect in cerebrospinal fluid rhinorrhea reconstruction surgery.

High-pressure headaches, low-pressure syndromes, and CSF leaks: diagnosis and management.

BACKGROUND: Headache resulting from idiopathic intracranial hypertension (IIH) in a population of moderately to obese women of childbearing age. The causes overall remain unclear. With this review, we provide an overview of clinical treatment and management strategies. RESULTS: IIH management is dependent on the signs and symptoms presented. Symptomatic treatment should attempt to lower intracranial pressure, reduce pain, and protect the optic nerves. Consideration for lumbar puncture and draining fluid as an option for reducing pressure may be helpful; however, repeated treatment is not usually favored by patients. Traditional prophylactic medications used in migraine may help reduce the primary headache often induced by raised intracranial pressure. We suggested surgical intervention for patients experiencing visual loss or impending visual loss and not responding to medication therapy. CONCLUSION: In this review, we discuss headache associated with IIH and spontaneous intracranial hypotension. Much needs to be learned about treatment options for patients with cerebrospinal fluid leaks including methods to strengthen the dura.

Tension pneumocephalus as a result of endonasal surgery: an uncommon intracranial complication.

Tension pneumocephalus (TP) is a clinical entity characterized by continued build-up of air within the cranial cavity, leading to abnormal pressure exerted upon the brain and subsequent neurologic deterioration, due to development of a mass effect and potentially a herniation syndrome. Intracranial complications of endoscopic sinus surgery (ESS) and other endonasal procedures are fortunately very rare, occurring in less than 3% of cases. We report 4 cases of small bone defects (<3 mm) in the anterior cranial base accompanied by TP, caused by ESS and other endonasal procedures. The pathophysiology and management of this clinical entity is discussed with a pertinent literature. Four patients with small (<3 mm) skull base defects were identified. All patients presented with active cerebrospinal fluid leaks. CT scans showed intracranial tension pneumocephalus. Using image-guided endoscopic techniques, all defects were addressed with multi-layer repair. Closure was achieved in all patients on the first attempt, with an average follow-up of 36 months. Tension pneumocephalus is a rare event that can occur as a result of traumatic or iatrogenic violation of the dura and should be considered in all patients presenting with altered mental status after endoscopic sinus surgery or other surgical and diagnostic procedures that violate either the cranial or spinal dura. Because of the potential for rapid clinical deterioration and death, prompt brain imaging is warranted to rule out the diagnosis, and urgent neurosurgical consultation is indicated for definitive management.

Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks.

Although for the vast majority of patients with spontaneous intracranial hypotension knowledge of the exact site of the underlying spinal CSF leak is not necessary, it is for patients with recalcitrant symptoms. Such patients may require directed treatments such as percutaneous fibrin glue injections or surgery. A variety of MRI techniques have been shown to be able to detect CSF leaks as well and sometimes better than the "gold standard" - CT-myelography. For unusually rapid CSF leaks - particularly those ventral to the spinal cord - digital subtraction myelography or dynamic CT-myelography are indicated. Some patients with spontaneous intracranial hypotension verified by intracranial MRI are never found to have a spinal CSF leak using current techniques.

Lack of increase in intracranial pressure after epidural blood patch in spinal cerebrospinal fluid leak.

BACKGROUND AND IMPORTANCE: Epidural blood patch (EBP) is one therapeutic measure for patients suffering from spontaneous intracranial hypotension (SIH) or post-lumbar puncture headaches. It has been proposed that an EBP may directly seal a spinal cerebrospinal fluid (CSF) fistula or result in an increase in intracranial pressure (ICP) by a shift of CSF from the spinal to the intracranial compartment. To the best of our knowledge this is the first case of a patient with SIH and neurological deterioration in whom ICP was measured before, during, and after spinal EBP. CLINICAL PRESENTATION: This 52-year old previously healthy man presented with holocephal headaches. MRI showed a left hemispheric subdural fluid collection causing a significant mass effect. Myelography revealed a CSF leak with epidural contrast at the left side of the L-2 level. To seal the CSF leak, we performed an EBP procedure targeted at left L-2 level and recorded ICP. After applying the epidural blood patch (15 cc) the patient improved rapidly, ICP however remained unchanged before, during, and after the procedure. One day post-treatment, he had a GCS score increase from 12 to 15 and no headache or neurological deficits. CONCLUSION: A shift of CSF from the spinal to the cranial compartment with a subsequent rise in ICP might not be a beneficial therapeutic mechanism of spinal epidural blood patching.

Endoscopic repair of cerebrospinal fluid rhinorrhoea in a developing country.

The objectives of the study was to determine the causes and outcome of endoscopic repair of cerebrospinal fluid (CSF) leak in a developing country. A total of five patients were recruited in the study. The age of patients ranged from 8 to 65 years. Four patients were male and one was female. In two cases of iatrogenic injury, the first was in the sphenoid sinus. The second was following functional endoscopic sinus surgery (FESS). Fascia lata was used to repair all cases. Beriplast was used as sealing agent in four cases and clotted blood was used in remaining case. Despite the small number, CSF rhinor rhoea was resolved in all cases. The patients were followed up for 2.5 to 6.5 years. Endoscopic repair is a viable option even in developing countries. It is cost effective and has a very low morbidity rate with no mortality at all.

[The substantiation of the approach for endoscopic surgery of nasal liquorrhea].

The objective of the present study was to develop a rationale for the approach to be employed in endoscopic surgery of nasal liquorrhea. A total of 69 patients presenting with this condition were available for the observation. Traumatic liquorrhea accounted for 80% of the cases, iatrogenic liquorrhea for 10%, liquorrhea associated with malformations for 5%, and spontaneous liquorrhea for 5% of the cases. The diagnostic algorithms elaborated for the identification of liquor cartilages were used to develop the criteria for the choice of the operative approach. A total of 69 surgical interventions were performed including 54 transnasal, 20 transethmoidal, 29 transsphenoidal, 5 endonasal, and 15 transcranial operations. The liquor fistulas were closed using tissue fragments and artificial materials, viz. muscles, broad fascia of thigh, periosteum and cartilage of the nasal septum, the mucous membrane from the nasal cavity, TachoComb plates, hemostatic sponge, fibrin-thrombin glue, bone crumbs, elements of bone marrow, and stem cells. The study has demonstrated the advantages of transnasal surgical treatment of liquor fistulas. The principles of postoperative care for the patients have been developed.

Combined simultaneous transcranial and transsphenoidal resection of large-to-giant pituitary adenomas.

BACKGROUND: While large-to-giant pituitary adenomas (PAs) may be safely removed by experienced surgeons through a single route, the procedure is technically challenging. We present the outcome of a simultaneous combined transcranial and transsphenoidal approach and discuss its applications. METHODS: A retrospective review was conducted on 12 consecutive patients. Surgical complications, visual and endocrinological functions, and tumour control were reviewed. RESULTS: There were four men and eight women, with a mean age of 47.6 years. All but one patient had non-functioning PAs. The mean tumour height was 4.1 cm (range: 2.3-5.5). The predominant presenting symptoms were visual field loss in eight patients, headache in three patients and mental confusion in one patient. There was no operative mortality. Post-operative cerebrospinal fluid leakage occurred in one patient. Five of the eight patients who presented with visual field loss achieved full recovery, and three had partial improvement. Two patients developed permanent diabetes insipidus after surgery. Panhypopituitarism occurred in one patient. Gross total removal (GTR) was achieved in five, and subtotal removal (STR) in seven patients. Seven patients received post-operative external irradiation. All patients who had GTR remained tumour-free and all those with STR had stable diseases after a mean follow-up period of 53.1 months (range: 14.1-92.1). CONCLUSION: The simultaneous 'above and below' approach is a safe and effective surgical strategy for large-to-giant PAs, particularly when expertise in endoscopic transsphenoidal surgery is unavailable. Its use, however, should be limited to a carefully selected group of patients, and tailored to individual user's expertise and experience.

Cerebrospinal fluid dynamics along the optic nerve in patients with spontaneous cerebrospinal fluid rhinorrhea: a retrospective computed tomographic cisternography study.

PURPOSE: Spontaneous cerebrospinal fluid (CSF) rhinorrhea is related to intracranial pressure (ICP) and dynamic changes. This study investigated CSF dynamics along the optic nerve (ON) in patients with spontaneous CSF rhinorrhea. METHODS: The computed tomographic (CT) cisternographies of 66 patients (132 eyes) with spontaneous CSF rhinorrhea were analysed. The contrast-loaded CSF (CLCSF) density was measured in Hounsfield units (HU) at three regions of interest (ROIs) along the ON and adjusted by the basal cistern density. The CLCSF density and ON sheath diameter (ONSD) were analysed between both sides in the different ICP groups. RESULTS: When comparing the density of CLCSF along the ON, no significant differences were found between the ipsilateral and contralateral sides of the leakage. The distribution of CLCSF along the ON showed a highly significant density reduction from the canalicular segment to the bulbar segment on both sides. The CLCSF density significantly decreased on the ipsilateral ON in the canalicular segment and tended to decrease on the ipsilateral ON in the bulbar and canal segments compared with that on the contralateral ON in the low-ICP group. The ONSD tended to decrease on the ipsilateral side of leakage. CONCLUSIONS: According to the CLCSF density on CT cisternography, CSF dynamics along the ON may bilaterally decrease from the optic canal to the retrobulbar segment. Cerebrospinal fluid (CSF) dynamics are possibly influenced by differences in ICP, and a lower ICP may cause more obvious differences or impairments in CSF dynamics along the ipsilateral ON.

Spontaneous intracranial hypotension-hypovolemia associated with tacrolimus.

There is little precedent for a medication-induced spontaneous intracranial hypotension/cerebrospinal fluid (CSF) hypovolemia (SIH). This case history of a woman with low CSF pressure, orthostatic headache, and radiographic findings consistent with SIH but without a detectable leak was notable for its association, both onset and resolution, with the use of the calcineurin inhibitor tacrolimus (FK506). A literature review for potential causes of a tacrolimus-induced CSF hypotension suggests many potential mechanisms of action, including effects on blood brain barrier and dural compliance, and supports further vigilance for this condition in the medically complex setting of tacrolimus use.

Natura abhorret a vacuo--use of fibrin glue as a filler and sealant in neurosurgical "dead spaces". Technical note.

OBJECTIVE: The objective of this study is to report our experience and illustrate our technique in the use of fibrin glue in the treatment of post-operatory cerebrospinal fluid (CSF) leaks and collections following different neurosurgical procedures. METHODS: In a 3-year period, 40 subjects underwent endoscopic endonasal approach for different sellar and skull base lesions (three tuberculum sellae meningiomas, six craniopharyngiomas, three Rathke's cleft cysts and 28 pituitary macroadenomas), in which an intraoperative CSF leakage was evident. In such subjects, the fibrin glue was used as a first step of the final phase of the procedure-i.e. the reconstruction of the skull base defect-followed by the other materials employed. Furthermore, ten other patients, who had undergone transsphenoidal (four cases), spinal (two cases), posterior fossa (three cases) and transcortical intraventricular tumour removal (one case) neurosurgical procedures and developed CSF leaks or collections, were conservatively treated by single or repeated in situ injections of "modified" fibrin glue under local anaesthesia according to different described techniques. In total, 50 patients constitute the clinical material of the present study. TECHNIQUE: In the cases where the fibrin glue was used during the reconstruction phase of the procedure (40 cases), the glue was injected inside the tumour cavity to fill the dead space left by the removal of the lesion. In case of post-operative CSF leak or CSF fluid collection (ten cases), after discarding 50-80% of the thrombin solution to obtain prevalence of the product's adhesive properties, fibrin glue was injected directly in the path of the CSF leak or into the collection cavity after aspiration of the collection's content. This was performed with the provided application system or through lumbar or Tuohy needles. Applications were repeated every 48 h until the disappearance of the leak. In all the treated cases, the disappearance of CSF leaks or collections was obtained with a number of applications ranging from one to five. Successful results are stable with a follow-up ranging from 6 months to 3 years. CONCLUSIONS: In our experience, the injection of fibrin glue has proved to be effective in filling or sealing post-operative "dead spaces" and treating minor or initial CSF leaks resulting from procedures of transsphenoidal, cranial and spinal surgery, adding another possibility in the management of many of these dreadful complications.

Dural suturing for repair of cerebrospinal fluid leak in transnasal transsphenoidal surgery.

OBJECTIVE: Repair of a cerebrospinal fluid (CSF) leak after transsphenoidal surgery (TSS) is usually accomplished using various graft materials. These methods are effective in most, but not all, cases. METHODS: Since 2006, we have been directly suturing the sellar floor dura in patients with an intraoperative CSF leak. Fat and/or fascial grafts were utilized only when a major CSF leak developed. The incidence of postoperative CSF rhinorrhea was compared before and after the suture. RESULTS: Postoperative CSF rhinorrhea developed in 3.7% (7 out of 188) of cases before 2005, but never since the dural suture was introduced (0 out of 136, 0%; P = 0.0229). Although watertight closure was not achieved in some cases, narrowing the dural defect and supporting the intrasellar graft was attained in every case. Surgical time was approximately 30 min longer in patients who underwent dural suture (148 +/- 42 min) than those who did not (119 +/- 37 min; P = 0.0001). CONCLUSION: Direct suturing of the sellar dura is a simple, safe, and reliable surgical technique for repairing CSF leaks after TSS. Using this procedure, more than 70% of patients with an intraoperative CSF leak can avoid autologous tissue grafts.

Endonasal approaches to the sellar and parasellar regions: closure techniques using biomaterials.

PURPOSE: We reviewed the clinical outcomes resulting from various closure techniques used following endoscopic endonasal surgery for lesions in the sellar and parasellar regions. We compared our current closure technique, which uses a biological matrix of native equine collagen (TissuDura) fixed with fibrin sealant (Tisseel), with the technique we employed previously, using autologous materials, in order to assess the comparative efficacy and tolerability of both methods over the medium- to long-term. METHODS: A review was conducted of all cases of endonasal endoscopic intervention carried out in our institution between 1997 and 2007. Operations performed between January 1st 1997 and December 31st 2003 involved a sellar closure technique using autologous materials, either alone or supported by fibrin sealant. From January 1st 2004, sellar reconstruction techniques involving resorbable heterologous materials were used in the closure phases. Post-operatively, clinico-endoscopic assessments took place at 15 days, 1, 3, and 6 months and yearly thereafter, supplemented by magnetic resonance imaging (MRI) scanning at 3 months and annually. RESULTS: Between January 1st 1997 and December 31st 2003, 79 operations were performed in which the sellar closure technique involved the use of autologous materials. Between January 1st 2004 and January 1st 2008, 125 operations were performed in which biomaterials were used for sellar closure. The incidence of complications (fluid fistula) was 2.5% in the autologous materials closure group and 1.6% in the biomaterials closure group. The most marked difference between the two approaches was seen at 1-month follow-up, when restoration of mucociliary transport in the sphenoidal sinus and physiological functionality of the nasal mucosa and paranasal sinuses were observed to be superior in the biomaterials patient cohort. CONCLUSIONS: The development of biomaterials for closure of the sellar floor offers a viable alternative to traditional techniques using autologous materials.

Pneumococcal meningitis secondary to intra-sphenoidal encephalocoele.

Basal encephalocoeles are rare congenital abnormalities of the skull base. The authors describe a rare case of spontaneous CSF leak and meningitis secondary to an intra-sphenoidal encephalocoele. Initial endoscopic transsphenoidal repair was unsuccessful, necessitating a combined subtemporal and transsphenoidal approach to close the defect.

Endoscopic repair of supraorbital ethmoid cerebrospinal fluid leaks.

OBJECTIVE: To examine the clinical and anatomical characteristics of patients with supraorbital ethmoid (SOE) cerebrospinal fluid (CSF) leaks and encephaloceles and identify specific considerations unique to their management. METHODS: Retrospective review of patients who underwent repair of SOE CSF leaks at our institution from 2003 to 2007. RESULTS: The majority of patients were women (5/8), middle-aged (mean: 54.9 years) and had a high body mass index (mean 42.3). Intracranial pressures (ICPs) were elevated in 6/8 patients. Anatomically, 6/8 patients had defects medial to the medial orbital wall (MOW; mean distance: 4.15 mm) and 2/8 had defects lateral to the MOW (mean distance: 8.14 mm). Seven out of 8 were successfully repaired endoscopically, and 1 patient with a lateral defect required an adjunctive trephination. CONCLUSIONS: Patients with spontaneous SOE CSF leaks have unique clinical characteristics that include obesity and elevated ICP. Extension of a skull base defect lateral to the MOW and a narrow anterior-posterior diameter of the frontal recess are technical obstacles to endoscopic repair and may necessitate an adjunctive external approach.

Current imaging of cerebrospinal fluid leaks.

A CSF leak results from a defect in the dura and skull base. Due to the risk of potentially devastating central nervous system infection, it is an important entity. Timely diagnosis of CSF leaks is crucial. Laboratory testing of nasal or aural fluid drainage for the presence of the protein Beta (beta)-2 transferrin plays a key role in establishing the presence of a CSF leak. Such assays are not always available, making imaging pivotal in the diagnosis of this entity. The development of minimally invasive endoscopic repair further underscores the importance of imaging as precise anatomic localization is important for repair In this article, we review the literature and make suggestions for the appropriate radiological investigation of patients with suspected CSF leaks.

[Primary empty sella: symptoms, physiopathology, diagnosis and treatement]. / Silla turca vacía primaria: clínica, fisiopatología y tratamiento.

Bibliographical review on the primary empty sella: concept, symptomatology, diagnosis, physiopathology and management.

Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension.

OBJECTIVE: To report our outcomes with the repair of spontaneous cerebrospinal fluid (CSF) leaks and to demonstrate how management of underlying intracranial hypertension improves outcomes. STUDY DESIGN: Retrospective review of spontaneous CSF leaks treated at the University of Pennsylvania Health System from 1996 to 2006. Data collected included demographics, nature of presentation, body mass index (BMI), site of skull base defect, surgical approach, intracranial pressure, and clinical follow-up. RESULTS: Fifty-six patients underwent repair of spontaneous CSF leaks. Eighty-two percent (46 of 56) were obese (average BMI 36.2 kg/m(2)). Nine patients had multiple CSF leaks. Fifty-four patients (96%) had associated encephaloceles. Fifty-three CSF leaks (95%) were successfully repaired at first attempt (34 months of follow-up). Intracranial pressures averaged 27 cm H(2)O. Patients were treated with acetazolamide or, in severe cases, with a ventriculoperitoneal shunt. CONCLUSIONS: Spontaneous CSF leaks have the highest recurrence rate of any etiology. With treatment of underlying intracranial hypertension coupled with endoscopic repair, the success rate (95%) approaches that of other etiologies of CSF leaks.