Endonasal Endoscopic Fenestration of Rathke's Cleft Cysts: Whether to Leave the Fenestration Open or Closed?

Introduction Rathke's cleft cysts (RCC) are generally treated with transsphenoidal fenestration and cyst drainage. If no cerebrospinal fluid (CSF) leak is created, the fenestration can be left open. If CSF is encountered, a watertight closure must be created to prevent postoperative CSF leak, though sellar closure has theoretically been linked with higher recurrence rate. In this study, we investigate the relationship between sellar closure, rate of postoperative CSF leak, and RCC recurrence. Methods Retrospective review of a prospective database of all endoscopic endonasal RCC fenestrations and cases were divided based on closure. The "open" group included patients who underwent fenestration of the RCC, whereas the "closed" group included patients whose RCC was treated with fat and a rigid buttress ± a nasoseptal flap. The rate of intra- and postoperative CSF leak and radiographic recurrence was determined. Results The closed group had a higher rate of suprasellar extension (odds ratio [OR]: 8.0, p = 0.032) and intraoperative CSF leak ( p ≤ 0.001). There were 54.8% intraoperative CSF leaks and no postoperative CSF leaks. Radiologic recurrence rate for the closed group (35.0%) was three times higher than the open group (9.1%; risk ratio [RR] = 3.85, p = 0.203), but not powered to show significance. None of the radiologic recurrences required reoperation. Conclusion Maintaining a patent fenestration between an RCC and the sphenoid sinus is important in reducing the rate of radiographic recurrence. Closure of the fenestration may be required to prevent CSF leak. While closure increases the rate of radiographic recurrence, reoperation for recurrent RCC is still an uncommon event.

Low incidence of true Sternberg's canal defects among lateral sphenoid sinus encephaloceles.

BACKGROUND: Spontaneous sphenoid sinus cerebrospinal fluid (CSF) encephaloceles have been postulated to arise from a persistent Sternberg's canal. However, recent evidence has questioned this embryological etiology. We examined the anatomic location of a series of lateral sphenoid sinus encephaloceles to determine if they corresponded with the location of Sternberg's canal. METHODS: We queried a prospectively acquired database of surgically treated spontaneous CSF leaks and identified those arising from the sphenoidal sinus. Images were reviewed to characterize the leaks with respect to the foramen rotundum (FR) and the vidian canal (VC). Four leak types were classified of which Type I (medial to FR and VC entering nasopharynx) was theoretically located in the precise location of Sternberg's canal. Type II was medial to FR; Type III was lateral to FR; Type IV passed through an enlarged FR into sphenoid sinus. Demographic data were analyzed. RESULTS: Of 103 repaired CSF leaks, 17 arose from the lateral sphenoid sinus. There were no true Type I leaks, 3 Type II leaks, 12 Type III leaks, and 2 Type IV leaks. No differences were found with respect to sphenoid pneumatization, BMI, age, sex, arachnoid pits, or postoperative leak between different types. CONCLUSIONS: No evidence was found to support the existence of a classic Sternberg canal CSF leak, supporting the hypothesis that most sphenoid spontaneous leaks likely occur secondary to chronically elevated ICP. Rare cases may be related to a weakness in the sphenoid wall in the region of Sternberg's canal.

Preserve or sacrifice the stalk? Endocrinological outcomes, extent of resection, and recurrence rates following endoscopic endonasal resection of craniopharyngiomas.

OBJECTIVEGross-total resection (GTR) of craniopharyngiomas (CPs) is potentially curative and is often the goal of surgery, but endocrinopathy generally results if the stalk is sacrificed. In some cases, GTR can be attempted while still preserving the stalk; however, stalk manipulation or devascularization may cause endocrinopathy and this strategy risks leaving behind small tumor remnants that can recur.METHODSA retrospective review of a prospective cohort of patients who underwent initial resection of CP using the endoscopic endonasal approach over a period of 12 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, was performed. Postresection integrity of the stalk was retrospectively assessed using operative notes, videos, and postoperative MRI. Tumors were classified based on location into type I (sellar), type II (sellar-suprasellar), and type III (purely suprasellar). Pre- and postoperative endocrine function, tumor location, body mass index, rate of GTR, radiation therapy, and complications were reviewed.RESULTSA total of 54 patients who had undergone endoscopic endonasal procedures for first-time resection of CP were identified. The stalk was preserved in 33 (61%) and sacrificed in 21 (39%) patients. GTR was achieved in 24 patients (73%) with stalk preservation and 21 patients (100%) with stalk sacrifice (p = 0.007). Stalk-preservation surgery achieved GTR and maintained completely normal pituitary function in only 4 (12%) of 33 patients. Permanent postoperative diabetes insipidus was present in 16 patients (49%) with stalk preservation and in 20 patients (95%) following stalk sacrifice (p = 0.002). In the stalk-preservation group, rates of progression and radiation were higher with intentional subtotal resection or near-total resection compared to GTR (67% vs 0%, p < 0.001, and 100% vs 12.5%, p < 0.001, respectively). However, for the subgroup of patients in whom GTR was achieved, stalk preservation did not lead to significantly higher rates of recurrence (12.5%) compared with those in whom it was sacrificed (5%, p = 0.61), and stalk preservation prevented anterior pituitary insufficiency in 33% and diabetes insipidus in 50%.CONCLUSIONSWhile the decision to preserve the stalk reduces the rate of postoperative endocrinopathy by roughly 50%, nevertheless significant dysfunction of the anterior and posterior pituitary often ensues. The decision to preserve the stalk does not guarantee preserved endocrine function and comes with a higher risk of progression and need for adjuvant therapy. Nevertheless, to reduce postoperative endocrinopathy attempts should be made to preserve the stalk if GTR can be achieved.

The rectus capitis lateralis and the condylar triangle: important landmarks in posterior and lateral approaches to the jugular foramen.

OBJECTIVE The rectus capitis lateralis (RCL) is a small posterior cervical muscle that originates from the transverse process of C-1 and inserts onto the jugular process of the occipital bone. The authors describe the RCL and its anatomical relationships, and discuss its utility as a surgical landmark for safe exposure of the jugular foramen in extended or combined skull base approaches. In addition, the condylar triangle is defined as a landmark for localizing the vertebral artery (VA) and occipital condyle. METHODS Four cadaveric heads (8 sides) were used to perform far-lateral, extended far-lateral, combined transmastoid infralabyrinthine transcervical, and combined far-lateral transmastoid infralabyrinthine transcervical approaches to the jugular foramen. On each side, the RCL was dissected, and its musculoskeletal, vascular, and neural relationships were examined. RESULTS The RCL lies directly posterior to the internal jugular vein-only separated by the carotid sheath and in some cases cranial nerve (CN) XI. The occipital artery travels between the RCL and the posterior belly of the digastric muscle, and the VA passes medially to the RCL as it exits the C-1 foramen transversarium and courses posteriorly toward its dural entrance. CNs IX-XI exit the jugular foramen directly anterior to the RCL. To provide a landmark for identification of the occipital condyle and the extradural VA without exposure of the suboccipital triangle, the authors propose and define a condylar triangle that is formed by the RCL anteriorly, the superior oblique posteriorly, and the occipital bone superiorly. CONCLUSIONS The RCL is an important surgical landmark that allows for early identification of the critical neurovascular structures when approaching the jugular foramen, especially in the presence of anatomically displacing tumors. The condylar triangle is a novel and useful landmark for identifying the terminal segment of the hypoglossal canal as well as the superior aspect of the VA at its exit from the C-1 foramen transversarium, without performing a far-lateral exposure.

Intradiploic Epidermoid Tumors of the Cranium: Case Report with Review of the Literature.

Introduction Epidermoid cysts are a rare intracranial tumor, mostly arising from the cerebellar pontine angle but sporadically arising from the cranial diploe. Intradiploic epidermoids were first described in 1838 and have since been reported in sporadic case reports and case series. Due to the scarcity of cases, no institution has a large enough case series to fully characterize this tumor. We review numerous case series to better describe intradiploic epidermoids. Methods Using a search for intradiploic epidermoid, several case reports and series were found. References from these articles were reviewed for further cases. Results A total of 169 cases of intradiploic epidermoids were reviewed. The average age of patients presenting with intradiploic epidermoids was 38.1 years (standard error of the mean: 1.56). Overall, 68.9% of these patients presented with localized swelling over the scalp, 32.3% with headaches, and 42.7% with other neurologic symptoms. The most common location of the tumor was the frontal bones, with the least common the sphenoid, zygomatic, and maxillary bone. Surgical resection was curative in most cases, with a 3.2% mortality. Recurrence rate was only 5.8%, with nearly all occurring before 1999. Of the recurrent cases, malignant transformation to squamous cell carcinoma was estimated at 44% (4/9). Most of these cases are thought to have occurred as a result of incomplete resection of a primary intradiploic epidermoid. Conclusions To date, this is the largest review of intradiploic epidermoids reported in the literature. In the reported cases, most had a benign course. However, there are occasional malignant transformations, and some patients have neurologic sequelae from mass effect or tumor infiltration. This is a surgically curative tumor in most cases.

Cranio-orbito-zygomatic approach for a previously coiled/recurrent giant MCA aneurysm in a hybrid angio/OR suite.

We present surgical clipping of a giant middle cerebral artery aneurysm. The patient is a 64-year-old woman who suffered subarachnoid hemorrhage in 2005. She was treated with coiling of the aneurysm at an outside institution. She presented to our clinic with headaches and was found on angiography to have giant recurrence of the aneurysm. To allow adequate exposure for clipping, we performed the surgery through a cranio-orbito-zygomatic (COZ) skull base approach, which is demonstrated. The surgery was performed in an operating room/angiography hybrid suite allowing for high quality intraoperative angiography. The technique and room flow are also demonstrated. The video can be found here: http://youtu.be/eePcyOMi85M.

Spring-mediated sagittal craniosynostosis treatment at the Children's Hospital of Philadelphia: technical notes and literature review.

OBJECT Sagittal craniosynostosis has been treated using both cranial remodeling techniques and modification of the sagittal strip craniectomy. A more recent technique is to implant springs in conjunction with a suturectomy to transversely expand the parietal bones to accommodate the growing brain. In this paper the authors describe and evaluate several modifications to the spring-mediated cranioplasty (SMC) technique, most notably use of an ultrasonic scalpel to limit dural dissection and maximize opening of the stenosed suture by placement of multiple spring devices. In addition, the literature is reviewed comparing SMC to other surgical treatments of sagittal synostosis. METHODS The authors retrospectively reviewed patients who presented to the Children's Hospital of Philadelphia with a diagnosis of sagittal synostosis from August 2011 to November 2014. A pooled data set was created to compare our institutional data to previously published work. A comprehensive literature review was performed of all previous studies describing the SMC technique, as well as other techniques for sagittal synostosis correction. RESULTS Twenty-two patients underwent SMC at our institution during the study period. Patients were 4.2 months of age on average, had a mean blood loss of 56.3 ml, and average intensive care unit and total hospital stays of 29.5 hours and 2.2 days, respectively. The cranial index was corrected to an average of 73.7 (SD 5.2) for patients who received long-term radiological follow-up. When comparing the authors' institutional data to pooled SMC data, blood loss and length of stay were both significantly less (p = 0.005 and p < 0.001, respectively), but the preoperative cranial index was significantly larger (p = 0.01). A review of the SMC technique compared with other techniques to actively expand the skull of patients with sagittal synostosis demonstrated that SMC can be performed at a significantly earlier age compared with cranial vault reconstruction (CVR). CONCLUSIONS The authors found that their institutional modifications of the SMC technique were safe and effective in correcting the cranial index. In addition, this technique can be performed at a younger age than CVRs. SMC, therefore, has the potential to maximize the cognitive benefits of early intervention, with lower morbidity than the traditional CVR.

Medical and surgical management of spinal epidural abscess: a systematic review.

OBJECT: Spinal epidural abscess (SEA) is a rare condition that has previously been treated with urgent surgical decompression and antibiotics. Recent availability of MRI makes early diagnosis possible and allows for the nonoperative treatment of SEA in select patients. The first retrospective review of medically and surgically managed SEA was published in 1999, and since that time several other retrospective institutional reports have been published. This study reviews these published reports and compares pooled data with historical treatment data. METHODS: A PubMed keyword and Boolean search using ("spinal epidural abscess" OR "spinal epidural abscesses" AND [management OR treatment]) returned 429 results. Filters for the English language and publications after 1999 were applied, as the first study comparing operative and nonoperative management was published that year. Articles comparing operative to nonoperative treatment strategies for SEA were identified, and the references were further reviewed for additional articles. Studies involving at least 10 adult patients (older than 18 years) were included. Case reports, studies reporting either medical or surgical management only, studies not reporting indications for conservative management, or studies examining SEA as a result of a specific pathogen were excluded. RESULTS: Twelve articles directly comparing surgical to nonsurgical management of SEA were obtained. These articles reported on a total of 1099 patients. The average age of treated patients was 57.24 years, and 62.5% of treated patients were male. The most common pathogens found in blood and wound cultures were Staphylococcus aureus (63.6%) and Streptococcus species (6.8%). The initial treatment was surgery in 59.7% of cases and medical therapy in 40.3%. This represented a significant increase in the proportion of medically managed patients in comparison with the historical control prior to 1999 (p < 0.05). Patients with no neurological deficits were significantly more likely to be treated medically than surgically (p < 0.05). There was no statistically significant difference overall between surgical and nonsurgical management, although several risk factors may predict failure of medical management. CONCLUSIONS: Since the first reports of nonoperative treatment of SEA, there has been a substantial trend toward treating neurologically intact patients with medical management. Nevertheless, medical therapy fails in a fair number of cases involving patients with specific risk factors, and patients with these risk factors should be closely observed in consideration for surgery. Further research may help identify patients at greater risk for failure of medical therapy.

Scale to predict survival after surgery for recurrent glioblastoma multiforme.

PURPOSE: Despite initial treatment with surgical resection, radiotherapy, and chemotherapy, glioblastoma multiforme (GBM) virtually always recurs. Surgery is sometimes recommended to treat recurrence. In this study, we sought to devise a preoperative scale that predicts survival after surgery for recurrent glioblastoma multiforme. PATIENTS AND METHODS: The preoperative clinical and radiographic data of 34 patients who underwent re-operation of recurrent GBM tumors were analyzed using Kaplan-Meier survival analysis and Cox proportional hazards regression modeling. The factors associated with decreased postoperative survival (P < .05) were used to devise a prognostic scale which was validated with a separate cohort of 109 patients. RESULTS: The factors associated with poor postoperative survival were: tumor involvement of prespecified eloquent/critical brain regions (P = .021), Karnofsky performance status (KPS) < or = 80 (P = .030), and tumor volume > or = 50 cm(3) (P = .048). An additive scale (range, 0 to 3 points) comprised of these three variables distinguishes patients with good (0 points), intermediate (1 to 2 points), and poor (3 points) postoperative survival (median survival, 10.8, 4.5, and 1.0 months, respectively; P < .001). The scale identified three statistically distinct groups within the validation cohort as well (median survival, 9.2, 6.3, and 1.9 months, respectively; P < .001). CONCLUSION: We devised and validated a preoperative scale that identifies patients likely to have poor, intermediate, and good relative outcomes after surgical resection of a recurrent GBM tumor. Application of this simple scale may be useful in counseling patients regarding their treatment options and in designing clinical trials.

Experimental approaches for the treatment of malignant gliomas.

Malignant gliomas, which include glioblastomas and anaplastic astrocytomas, are the most common primary tumors of the brain. Over the past 30 years, the standard treatment for these tumors has evolved to include maximal safe surgical resection, radiation therapy and temozolomide chemotherapy. While the median survival of patients with glioblastomas has improved from 6 months to 14.6 months, these tumors continue to be lethal for the vast majority of patients. There has, however, been recent substantial progress in our mechanistic understanding of tumor development and growth. The translation of these genetic, epigenetic and biochemical findings into therapies that have been tested in clinical trials is the subject of this review.

Elevation of matrix metalloproteinases 3 and 9 in cerebrospinal fluid and blood in patients with severe traumatic brain injury.

OBJECTIVE: Traumatic brain injury (TBI) causes an increase in matrix metalloproteinases (MMPs), which are associated with neuroinflammation, blood-brain barrier disruption, hemorrhage, and cell death. We hypothesized that patients with TBI have an increase in MMPs in ventricular cerebrospinal fluid (CSF) and plasma. METHODS: Patients with TBI and a ventricular catheter were entered into the study. Samples of CSF and plasma were collected at the time of catheter placement and at 24 and 72 hours after admission. Seven TBI patients were entered into the study, with 6 having complete data for analysis. Only patients who had a known time of insult that fell within a 6-hour window from initial insult to ventriculostomy were accepted into the study. Control CSF came from ventricular fluid in patients undergoing shunt placement for normal pressure hydrocephalus. Both MMP-2 and MMP-9 were measured with gelatin zymography and MMP-3 with Western immunoblot. RESULTS: We found a significant elevation in the levels of the latent form of MMP-9 (92-kD) in the CSF obtained at the time of arrival (P < 0.05). Elevated levels of MMP-2 were detected in plasma at 72 hours, but not in the CSF. Using albumin from both CSF and blood, we calculated the MMP-9 index, which was significantly increased in the CSF, indicating endogenous MMP production. Western immunoblot showed elevated levels of MMP-3 in CSF at all times measured, whereas MMP-3 was not detected in the CSF of normal pressure hydrocephalus. CONCLUSION: We show that MMPs are increased in the CSF of TBI patients. Although the number of patients was small, the results were robust and clearly demonstrated increases in MMP-3 and MMP-9 in ventricular CSF in TBI patients compared with controls. Although these preliminary results will need to be replicated, we propose that MMPs may be important in blood-brain barrier opening and hemorrhage secondary to brain injury in patients.