Cervical Dermal Sinus Tract: A Case Report and Comprehensive Literature Review.

The cervical and thoracic dermal sinuses are rare entities, conforming epithelium-lined tracts that extend from an opening in the skin through a corridor to the layers of the spinal cord. They are commonly detected in early childhood; however, adult reports are singularly rare, especially in cervical regions. We report a very unusual case of a 45-year-old Mexican female who developed progressive left-side weakness and dexterity suffered from childhood, getting worse in the last year. Physical examination revealed a soft, congenital round cystic lesion in the dorsal-midline skin at the level of C4-C5 vertebrae with no previous treatment received. MRI showed a dermal sinus tract at the C4 level from the skin tethering to the spinal cord and syringomyelia. CT scan showed a dysraphism corresponding to spina bifida at the C4 level and an incomplete closure at the C3 and C5 vertebrae. We surgically managed the lesion by microscopic resection with C3-C5 laminectomy preserving strength and sensitivity. Follow-up MRI showed no residual lesion and contained fistula with no further complications. Cervical dermal sinus lesions are unusual entities, even less prevalent in adulthood. It represents a possible delay in diagnosis and an increased rate of complications. Early suspicion of the condition is required to make an accurate diagnosis since it is a potentially treatable lesion with a high risk of sequelae without surgical treatment.

Giant Posterior Inferior Cerebellar Artery Aneurysm Mimicking a Brainstem Tumor.

Aneurysms from the vertebrobasilar system are rare, accounting for only 5%-10% of all intracranial aneurysms. The most common sites in which these lesions occur are the bifurcation of the basilar artery and the origin of the posterior inferior cerebellar artery (PICA). When the aneurysms present in the distal portion of the PICA, they represent from 0.5% to 6%. These aneurysms are called giant when they exceed 25 mm in diameter. We present a case of a 49-year-old male who presented with acute obstructive hydrocephalus, which required ventriculoperitoneal shunting and left hemispheric cerebellar syndrome. The magnetic resonance imaging study revealed an occupative mass located in the fourth ventricle, and diagnostic angiography showed a partially thrombosed giant saccular aneurysm in the posterior inferior cerebellar artery. He underwent surgical management via a lateral suboccipital approach. The aneurysm was remodeled and clipped successfully without complications, with an uneventful postoperative course. Although rare, PICA aneurysms should always be considered when posterior fossa syndrome occurs, including brainstem and cranial nerve compression symptoms. It can easily be misdiagnosed as a neoplastic lesion, especially when the aneurysm reaches big or giant size. Therefore, complete diagnostic studies, such as cerebral angiography, must be performed. Surgical clipping must be offered as the first line of treatment. It provides occlusion of the aneurysm and relieves compressive symptoms.

Sodium Fluorescein Videoangiography as an Adjunct to Resection of Cerebral Arteriovenous Malformations.

OBJECTIVE: To characterize dynamic changes of arteriovenous malformation using a microscope-integrated technique at the moment of performing intraoperative fluorescein videoangiography (FL-VAG) at each of the resection phases. METHODS: We prospectively recruited 12 patients with arteriovenous malformations and used FL-VAG as an ancillary technique for resection of the lesion. We analyzed transit time (TT) of FL in arterial feeders (arterial transit time [TTa]) and draining veins (venous transit time [TTv]) during the different stages of resection. To achieve this, we recorded 3 values of TT of FL (TTa, initial TTv, final TTv); when final TTv was markedly slower than initial TTv, we hypothesized that the nidus was devascularized enough and could be safely removed. RESULTS: No mortality or morbidity was related to use of FL. In most cases, TT values of arterial feeders and draining veins allowed an easier distinction between them. At advanced stages of resection, FL-VAG assessed increase in TTv (venous blood slower or absent), suggesting that most feeding arteries had been obliterated, indicating the appropriate moment for nidus removal. Optimal dose of FL was a 75-mg bolus followed by injection of 20 mL of saline solution. CONCLUSIONS: FL-VAG allows a distinction of normal from abnormal flow in draining vessels and might help the surgeon to decide when the nidus can be safely removed. This is the first study prospectively evaluating this technique, and it proposes an ideal dose for brain arteriovenous malformation surgery, in contrast to doses used in tumor cases.

Reoperation for growth hormone-secreting pituitary adenomas: report on an endonasal endoscopic series with a systematic review and meta-analysis of the literature.

OBJECTIVE Surgery is generally the first-line therapy for acromegaly. For patients with residual or recurrent tumors, several treatment options exist, including repeat surgery, medical therapy, and radiation. Reoperation for recurrent acromegaly has been associated with poor results, with hormonal control usually achieved in fewer than 50% of cases. Extended endonasal endoscopic approaches (EEAs) may potentially improve the results of reoperation for acromegaly by providing increased visibility and maneuverability in parasellar areas. METHODS A database of all patients treated in the authors' center between July 2004 and February 2016 was reviewed. Cases involving patients with acromegaly secondary to growth hormone (GH)-secreting adenomas who underwent EEA were selected for chart review and divided into 2 groups: first-time surgery and reoperation. Disease control was defined by 2010 guidelines. Clinical and radiological characteristics and outcome data were extracted. A systematic review was done through a MEDLINE database search (2000-2016) to identify studies on the surgical treatment of acromegaly. Using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, the included studies were reviewed for surgical approach, tumor size, cavernous sinus invasion, disease control, and complications. Cases were divided into reoperation or first-time surgery for comparative analysis. RESULTS A total of 44 patients from the authors' institution were included in this study. Of these patients, 2 underwent both first-time surgery and reoperation during the study period and were therefore included in both groups. Thus data from 46 surgical cases were analyzed (35 first-time operations and 11 reoperations). The mean length of follow-up was 70 months (range 6-150 months). The mean size of the reoperated tumors was 14.8 ± 10.0 mm (5 micro- and 6 macroadenomas). The patients' mean age at the time of surgery was younger in the reoperation group than in the first-time surgery group (34.3 ± 12.8 years vs 49.1 ± 15.7 years, p = 0.007) and the mean preoperative GH level was also lower (7.7 ± 13.1 µg/L vs 25.6 ± 36.8 µg/L, p = 0.04). There was no statistically significant difference in disease control rates between the reoperation (7 [63.6%] of 11) and first-time surgery (25 [71.4%] of 33) groups (p = 0.71). Univariate analysis showed that older age, smaller tumor size, lower preoperative GH level, lower preoperative IGF-I level, and absence of cavernous sinus invasion were associated with higher chances of disease control in the first-time surgery group, whereas only absence of cavernous sinus invasion was associated with disease control in the reoperation group (p = 0.01). There was 1 case (9%) of transient diabetes insipidus and hypogonadism and 1 (9%) postoperative nasal infection after reoperation. The systematic review retrieved 29 papers with 161 reoperation and 2189 first-time surgery cases. Overall disease control for reoperation was 46.8% (95% CI 20%-74%) versus 56.4% (95% CI 49%-63%) for first-time operation. Reoperation and first-time surgery had similar control rates for microadenomas (73.6% [95% CI 32%-98%] vs 77.6% [95% CI 68%-85%]); however, reoperation was associated with substantially lower control rates for macroadenomas (27.5% [95% CI 5%-57%] vs 54.3% [95% CI 45%-62%]) and tumors invading the cavernous sinus (14.7% [95% CI 4%-29%] vs 38.5% [95% CI 27%-50%]). CONCLUSIONS Reoperative EEA for acromegaly had results similar to those for first-time surgery and rates of control for macroadenomas that were better than historical rates. Cavernous sinus invasion continues to be a negative prognostic indicator for disease control; however, results with EEA show improvement compared with results reported in the prior literature.

Transorbital endoscopic eyelid approach for resection of sphenoorbital meningiomas with predominant hyperostosis: report of 2 cases.

Sphenoorbital meningiomas (SOMs) are slow-growing tumors that originate from the sphenoidal wing and are associated with visual deterioration, extrinsic ocular movement disorders, and proptosis caused by hyperostosis of the lateral wall of the orbit. In some cases, the intracranial component is quite small or "en plaque," and the majority of the symptoms arise from adjacent hyperostosis. Craniotomy has traditionally been the standard of care, but new minimally invasive multiportal endoscopic approaches offer an alternative. In the current study, the authors to present their experience with the transorbital endoscopic eyelid approach for the treatment of 2 patients with SOMs and sphenoid wing hyperostosis. Clinical and radiological data for patients with SOMs who underwent a transorbital endoscopic eyelid approach were retrospectively reviewed. Surgical technique and clinical and radiographic outcomes were analyzed. The authors report the cases of 2 patients with SOMs and proptosis due to sphenoid wing hyperostosis. One patient underwent prior craniotomy to debulk the intracranial portion of the tumor, and the other had a minimal intracranial component. Both patients were discharged 2 days after surgery. MR images and CT scans demonstrated a large debulking of the hyperostotic bone. Postoperative measurement of the proptosis with the aid of an exophthalmometer demonstrated significant reduction of the proptosis in one of the cases. Persistence of intraconal tumor in the orbital apex limited the efficacy of the procedure in the other case. A review of the literature revealed 1 publication with 3 reports of the transorbital eyelid approach for SOMs. No measure of relief of proptosis after this surgery had been previously reported. The transorbital endoscopic approach, combined with endonasal decompression of the medial orbit, may be a useful minimally invasive alternative to craniotomy in a subset of SOMs with a predominantly hyperostotic orbital wall and minimal intracranial bulky or merely en plaque disease. In these cases, relief of proptosis and optic nerve compression are the primary goals of surgery, rather than gross-total resection, which may have high morbidity or be unachievable. In cases with significant residual intraconal tumor, orbital bone removal alone may not be sufficient to reduce proptosis.

Do craniopharyngioma molecular signatures correlate with clinical characteristics?

OBJECTIVE Exome sequencing studies have recently demonstrated that papillary craniopharyngiomas (PCPs) and adamantinomatous craniopharyngiomas (ACPs) have distinct genetic origins, each primarily driven by mutually exclusive alterations: either BRAF ( V600E), observed in 95% of PCPs, or CTNNB1, observed in 75%-96% of ACPs. How the presence of these molecular signatures, or their absence, correlates with clinical, radiographic, and outcome variables is unknown. METHODS The pathology records for patients who underwent surgery for craniopharyngiomas between May 2000 and March 2015 at Weill Cornell Medical College were reviewed. Craniopharyngiomas were identified and classified as PCP or ACP. Patients were placed into 1 of 3 groups based on their genomic mutations: BRAF mutation only, CTNNB1 mutation only, and tumors with neither of these mutations detected (not detected [ND]). Demographic, radiological, and clinical variables were collected, and their correlation with each genomic group was tested. RESULTS Histology correlated strongly with mutation group. All BRAF tumors with mutations were PCPs, and all CTNNB1 with mutations and ND tumors were ACPs. Preoperative and postoperative clinical symptoms and radiographic features did not correlate with any mutation group. There was a statistically significant relationship (p = 0.0323) between the age group (pediatric vs adult) and the mutation groups. The ND group tumors were more likely to involve the sella (p = 0.0065). CONCLUSIONS The mutation signature in craniopharyngioma is highly predictive of histology. The subgroup of tumors in which these 2 mutations are not detected is more likely to occur in children, be located in the sella, and be of ACP histology.

Limitations of the endonasal endoscopic approach in treating olfactory groove meningiomas. A systematic review.

OBJECTIVE: To review current management strategies for olfactory groove meningioma (OGM)s and the recent literature comparing endoscopic endonasal (EEA) with traditional transcranial (TCA) approaches. METHODS: A PubMed search of the recent literature (2011-2016) was performed to examine outcomes following EEA and TCA for OGM. The extent of resection, visual outcome, postoperative complications and recurrence rates were analyzed using percentages and proportions, the Fischer exact test and the Student's t-test using Graphpad PRISM 7.0Aa (San Diego, CA) software. RESULTS: There were 444 patients in the TCA group with a mean diameter of 4.61 (±1.17) cm and 101 patients in the EEA group with a mean diameter of 3.55 (± 0.58) cm (p = 0.0589). GTR was achieved in 90.9% (404/444) in the TCA group and 70.2% (71/101) in the EEA group (p < 0.0001). Of the patients with preoperative visual disturbances, 80.7% (21/26) of patients in the EEA cohort had an improvement in vision compared to 12.83%(29/226) in the TCA group (p < 0.0001). Olfaction was lost in 61% of TCA and in 100% of EEA patients. CSF leaks and meningitis occurred in 25.7% and 4.95% of EEA patients and 6.3% and 1.12% of TCA patients, respectively (p < 0.0001; p = 0.023). CONCLUSIONS: Our updated literature review demonstrates that despite more experience with endoscopic resection and skull base reconstruction, the literature still supports TCA over EEA with respect to the extent of resection and complications. EEA may be an option in selected cases where visual improvement is the main goal of surgery and postoperative anosmia is acceptable to the patient or in medium-sized tumors with existing preoperative anosmia. Nevertheless, based on our results, it seems more prudent at this time to use TCA for the majority of OGMs.

Transorbital endoscopic approach for exposure of the sylvian fissure, middle cerebral artery and crural cistern: an anatomical study.

BACKGROUND: The availability of minimal access instrumentation and endoscopic visualization has revolutionized the field of minimally invasive skull base surgery. The transorbital endoscopic approach using an eyelid incision has been proposed as a new minimally invasive technique for the treatment of skull base pathology, mostly extradural tumors. Our study aims to evaluate the anatomical aspects and potential role of the transorbital endoscopic approach for exposure of the sylvian fissure, middle cerebral artery and crural cistern. METHODS: An anatomical dissection was performed in four freshly injected cadaver heads (8 orbits) using 0- and 30-degree endoscopes. First, an endoscopic endonasal medial orbital decompression was done to facilitate medial retraction of the orbit. An endoscopic transorbital approach through an eyelid incision, with drilling of the posterior wall of the orbit and lesser sphenoidal wing, was then performed to expose the sylvian fissure and crural cisterns. A stepwise anatomical description of the approach and visualized anatomy is detailed. RESULTS: A superior eyelid incision followed by orbital retraction provided a surgical window of approximately 1.2 cm (range 1.0-1.5 cm) for endoscopic transorbital dissection. The superior (SOF) and inferior (IOF) orbital fissures represent the medial limits of the approach and are identified in the initial part of the procedure. Drilling of the orbital roof (lateral and superior to the SOF), greater sphenoidal wing (lateral to the SOF and IOF) and lesser sphenoidal wing exposed the anterior and middle fossa dura. A square-shaped dural opening provided visualization of the posterior orbital gyri, sylvian fissure and temporal pole. Intradural dissection allowed exposure of the sphenoidal portion of the sylvian fissure, M1, MCA bifurcation and M2 branches and lenticulostriate perforators. Dissection of the medial aspect of the sylvian and carotid cisterns with a 30-degree endoscope allowed exposure of the mesial temporal lobe and crural cistern. CONCLUSIONS: The transorbital endoscopic approach allows successful exposure of the sphenoidal portion of the sylvian fissure and M1 and M2 segments of the middle cerebral artery. Angled endoscopes may provide visualization of the mesial temporal lobe and crural cistern. Although our anatomical study demonstrates the feasibility of intradural dissection and closure via an endoscopic transorbital approach, further studies are necessary to evaluate its role in the clinical scenario.

Transtubular excisional biopsy as a rescue for a non-diagnostic stereotactic needle biopsy-case report and literature review.

Stereotactic needle biopsy, a standard of care for acquiring deep-seated pathology, has limitations and risks in some situations. We present an uncommon case with basal ganglia dematiaceous mycetoma. Due to the firm consistency of the lesion, the initial stereotactic needle biopsy failed to provide a diagnosis. In a second operation, transtubular excisional biopsy was successfully performed to remove the entire mycetoma. We reviewed recent case series of transtubular approaches to deep-seated brain lesions and suggest this method could be a rescue for a non-diagnostic stereotactic needle biopsy and even may be the approach of choice in some cases.

Predictors and incidence of orthostatic headache associated with lumbar drain placement following endoscopic endonasal skull base surgery.

BACKGROUND: Orthostatic headache (OH) is a potential complication of lumbar drainage (LD) usage. The incidence and risk factors for OH with the use of lumbar drainage during endoscopic endonasal procedures have not been documented. OBJECTIVE: To investigate the incidence of post-procedure OHs associated with placement of LD in patients undergoing endoscopic endonasal procedures. METHODS: We prospectively noted the placement of LDs in a consecutive series of endoscopic endonasal skull base surgeries. Charts were retrospectively reviewed, and patients were divided into two groups: those with OH and those without. The patient demographics, drain durations, imaging findings of intracranial hypotension, pathologies and need for a blood patch were compared between the two groups. RESULTS: Two hundred forty-nine patients were included in the study. Seven patients (2.8%) suffered post-dural puncture OH, which was mild to moderate and disappeared 2-8 days (median 3 days) after treatment. Blood patches were used in four patients. Significant predisposing factors were age (33.0 vs. 53.5, P = 0.014) and a strong trend for female gender (85.7% vs. 47.9%, P = 0.062). BMI and drain duration were not significant. Postoperative intracranial hypotension was diagnosed radiographically in 43% of OH patients and in 5.4% of those without OH (P = 0.003). Four (1.6%) patients required treatment with an epidural blood patch. CONCLUSION: OH associated with intracranial hypotension in patients undergoing endoscopic endonasal procedures with LDs is an infrequent complication seen more commonly in young female patients. Radiographic signs of intracranial hypotension are a specific but not sensitive test for OH.

[Association of Doppler flowmetry with perinatal outcome in patients with oligohydramnios]. / Asociación de la flujometría Doppler con la evolución perinatal en pacientes con oligohidramnios.

BACKGROUND: Oligohydramnios is sometimes associated with poor perinatal outcome. Our aim was to determine the association of Doppler flowmetry with perinatal outcome in patients with oligohydramnios. METHODS: We carried out a prospective cross-sectional study that included 130 patients with pregnancy between 30 to 41 weeks of gestation; we compared the measurement of the resistance index in the umbilical artery by Doppler flowmetry and the measurement of amniotic fluid in patients with oligohydramnios. A follow-up of patients after birth was made, recording the Apgar score and whether they required special attention. The data were analyzed using descriptive statistics and we calculated the sensitivity, specificity, accuracy, and predictive values of the tests. RESULTS: Sensitivity, specificity and accuracy were obtained for oligohydramnios associated to perinatal death and for five-minute Apgar, with results of 100, 0, 50 % and 0, 0, 0 % respectively. The resistance index of umbilical artery related to perinatal death had sensitivity, specificity and accuracy of 100, 96 and 98 %, while in the Apgar were 0, 95 and 47.5 %, respectively. CONCLUSIONS: Doppler flowmetry of the umbilical artery showed a higher sensitivity, specificity and accuracy to diagnose perinatal death than the measurement of oligohydramnios and the Apgar score.

Introducción: el oligohidramnios en ocasiones se asocia a mala evolución perinatal. El objetivo fue determinar la asociación de la flujometría Doppler con la evolución perinatal en pacientes con oligohidramnios. Métodos: se realizó un estudio transversal en el que se analizaron 130 pacientes con embarazo de 30 a 41 semanas con oligohidramnios; se comparó la medición del índice de resistencia de la arteria umbilical por flujometría Doppler y la medición del líquido amniótico. Se hizo un seguimiento postnatal en el que se registró el Apgar y si los pacientes ameritaron cuidados especiales. Se utilizó estadística descriptiva y se calculó la sensibilidad, especificidad, exactitud y los valores predictivos de las pruebas. Resultados: para el oligohidramnios en muerte perinatal se obtuvo sensibilidad, especificidad y exactitud de 100, 0 y 50 %; y para Apgar de 0, 0 y 0 %, respectivamente. El índice de resistencia de la arteria umbilical referente a la muerte perinatal tuvo sensibilidad, especificidad y exactitud de 100, 96 y 98 %, en tanto que en el Apgar los valores fueron de 0, 95 y 47.5 %, respectivamente. Conclusiones: la flujometría Doppler de la arteria umbilical mostró mayor sensibilidad, especificidad y exactitud que la medición del oligohidramnios y la prueba de Apgar para diagnosticar muerte perinatal.

[Results of the application of modified Hamilton scale in the diagnosis of maternal anxiety during immediate puerperium]. / Resultados de la aplicación de la escala de Hamilton modificada en el diagnóstico de ansiedad materna durante el puerperio inmediato.

BACKGROUND: Anxiety during pregnancy is a risk factor of maternal and fetal morbidity with adverse results in newborn at short and long term. OBJECTIVE: To determine, with the modified Hamilton Anxiety Scale, the level of anxiety at the immediate postpartum and its association with maternal and fetal morbidity. MATERIAL AND METHOD: A cross-sectional study including 384 patients at the immediate postpartum to whom modified Hamilton Anxiety Scale was applied to determine the level of anxiety and its association with maternal factors and fetal and neonatal morbidity. Data were analyzed with statistical logistic regression analysis; an alpha value was set at 0.05. RESULTS: One hundred thirty-five patients had anxiety associated at immediate postpartum, the significant factors were scholarship (p = 0.0034), number of pregnancies (p = 0.0001), occupation (p = 0.0001). Women with anxiety had more frequency of threatened abortion (p = 0.0061), preterm labor (p = 0.0001), neonatal sepsis (p = 0.0123) and transient tachypnea of the newborn (p = 0.0016). CONCLUSIONS: A significant percentage of pregnant women had anxiety at immediate postpartum. Women with anxiety suffer more frequently maternal and fetal morbidity. It is recommended the opportune identification of this disorder in order to avoid the repercussion in the neonatal care.

[High concentrations of serum estradiol in assisted reproduction]. / Altas concentraciones de estradiol sérico en reproducci6ón asistida.

OBJECTIVES: To analyze the influence of the levels of estradiol on the day of HCG in the pregnancy rate (PR) of ICSI and oocyte donation. STUDY DESIGN: Retrospective, comparative. MATERIAL AND METHODS: 333 patients underwent ICSI and 66 in oocyte donation were included dividing them according to the level of estradiol: a) < 1,000 pg/mL, b) 1,001-3,000 pg/mL and c) > 3,000 pg/mL. Therapeutic protocol: Down regulation with acetate leuprolide in late luteal phase, COH with FSHr and/or HMG, ultrasonographic monitoring and estradiol blood levels, HCG application with > 3 follicles > 18 mm, oocyte retrieval 34 hours later. We analyzed: PR, age (including receptors), FSH and LH. Number, mature grade and fertilized oocytes; luteal support, transfer quality and type of catheter. Statistical analysis (SPSS 11) with chi square, ANOVA and Kruskall-Wallis. RESULTS: ICSI: Older patients in group A (p < 0.001), but without difference between B and C groups (p = 0.08). Statistical difference in number of follicles, number of oocytes, fertilized oocytes and transferred embryos being less in the A group (p < 0.001). Statistical difference in PR 21.7, 35.6 and 25.7% in A, B and C groups respectively (p = 0.032). Oocyte donation: Group A has younger patients (p = 0.005), FSH and LH were similiar among groups. Major number of follicles were observed to increase estradiol levels, but major quantity of metaphase II and fertilized oocytes were observed in group B (p = 0.05). PR without significant differences: 50, 51.5 and 52.3% in groups A, B and C (p = 0.977). Without statistical difference in the age of receptors; transferred embryos, type of catheter and quality on ICSI and oocyte donation groups. CONCLUSION: High estradiol levels at the day of HCG application affect the PR in patients submitted to ICSI. The best results were obtained with estradiol levels between 1,000 and 3,000 pg/mL. In oocyte donation the high concentrations of estradiol do not affect the PR of the receptors.