Medullary branches of the vertebral artery: microsurgical anatomy and clinical significance.

BACKGROUND: Since the medullary arteries are of a great neurologic and neurosurgical significance, the aim was to perform a detailed microanatomic study of these vessels, as well as of the medullary infarctions in a group of patients. METHODS: The arteries of 26 halves of the brain stem were injected with India ink and gelatin, microdissected and measured with an ocular micrometer. Neurologic and magnetic resonance imaging (MRI) examinations were performed in 11 patients. RESULTS: The perforating medullary arteries, averaging 6.7 in number and 0.26 mm in diameter, most often originated from the anterior spinal artery (ASA), and rarely from the vertebral (VA) (38.5%) and the basilar artery (BA) (11.6%). They supplied the medial medullary region. The anterolateral arteries, 4.8 in number and 0.2 mm in size, most often arose from the ASA and PerfAs, and nourished the anterolateral region. The lateral arteries, 2.2 in number and 0.31 mm in diameter, usually originated from the VA and the posterior inferior cerebellar artery (PICA). They supplied the lateral medullary region. The dorsal arteries, which mainly arose from the PICA and the posterior spinal artery (PSA), nourished the dorsal region, including the roof of the 4th ventricle. The anastomotic channels, averaging 0.3 mm in size, were noted in 42.3%. Among the medullary infarctions, the lateral ones were most frequently present (72.8%). CONCLUSION: The obtained anatomic data, which can explain the medullary infarctions symptomatology, are also important in order to avoid damage to the medullary arteries during neurosurgical and neuroradiologic interventions.

Midline Suboccipital Unilateral Trans-Cerebellomedullary Fissure Approach for Clipping of Ruptured VA-PICA Aneurysm: Two-Dimensional Operative Video.

The vertebral artery-posterior inferior cerebellar artery (VA-PICA) aneurysm poses a technical challenge for microsurgical clipping due to its anatomical complexity, which requires dissection of lower cranial nerves. Endovascular treatment is regarded as a feasible first-line therapeutic option for VA-PICA aneurysm because it has an acceptable aneurysm occlusion rate and is less invasive. However, microsurgical clipping remains an effective treatment option. We present the case of a 62-year-old man who presented with subarachnoid hemorrhage (SAH) due to a ruptured VA-PICA aneurysm. Neuroradiologic examination revealed a 2-3 mm medially pointing left VA-PICA aneurysm with acute obstructive hydrocephalus due to massive SAH in the posterior cranial fossa. As the patient had acute obstructive hydrocephalus and a relatively small aneurysm, we selected clipping over endovascular treatment. Because the aneurysm was located close to the midline and anterolateral to the medulla oblongata, we approached it from the midline. A midline suboccipital craniotomy, C1 laminectomy, and drilling of the left condylar fossa were performed; a unilateral cerebellomedullary fissure opening was added; and the aneurysm was clipped. Postoperative neuroradiologic examinations revealed complete obliteration of the aneurysm. As shown in this video, unilateral cerebellomedullary fissure opening combined with adequate removal of the condylar fossa provides a wide operative field in the cerebellomedullary cistern while avoiding strong retraction of the cerebellum. We believe that this technique makes VA-PICA aneurysm clipping safe and successful. Patient consent was obtained to perform the surgery and to publish the surgical video (Video 1).

Successful microvascular decompression surgery for dolichoectatic vertebral artery compression of medulla oblongata in a patient with hypersomnia disorder.

INTRODUCTION: Hypersomnia is a condition in which a person has trouble staying awake during the day. There are several potential causes of it, including sleep apnea and sleep disorders. CASE PRESENTATION: A 43 year old male was referred to our practice with complaints of hypersomnia, snoring, slurred speech and sleep apnea for more diagnostic and therapeutic interventions. His brain MRI was significant for a vascular loop compression on medulla oblongata. The patient underwent microvascular decompression surgery subsequently and showed improvement in all of his symptoms. CONCLUSION: One of the rare causes of sleep apnea is medulla oblongata compression by a vascular loop. Vertebrobasilar dolichoectasia may cause this phenomenon probably and should be reviewed in imaging examinations more precisely. Microvascular decompression by using a synthetic Teflon patch may be helpful in management of these patients.

A taxonomy for brainstem cavernous malformations: subtypes of medullary lesions.

OBJECTIVE: Medullary cavernous malformations are the least common of the brainstem cavernous malformations (BSCMs), accounting for only 14% of lesions in the authors' surgical experience. In this article, a novel taxonomy for these lesions is proposed based on clinical presentation and anatomical location. METHODS: The taxonomy system was applied to a large 2-surgeon experience over a 30-year period (1990-2019). Of 601 patients who underwent microsurgical resection of BSCMs, 551 were identified who had the clinical and radiological information needed for inclusion. These 551 patients were classified by lesion location: midbrain (151 [27%]), pons (323 [59%]), and medulla (77 [14%]). Medullary lesions were subtyped on the basis of their predominant surface presentation. Neurological outcomes were assessed according to the modified Rankin Scale (mRS), with an mRS score ≤ 2 defined as favorable. RESULTS: Five distinct subtypes were defined for the 77 medullary BSCMs: pyramidal (3 [3.9%]), olivary (35 [46%]), cuneate (24 [31%]), gracile (5 [6.5%]), and trigonal (10 [13%]). Pyramidal lesions are located in the anterior medulla and were associated with hemiparesis and hypoglossal nerve palsy. Olivary lesions are found in the anterolateral medulla and were associated with ataxia. Cuneate lesions are located in the posterolateral medulla and were associated with ipsilateral upper-extremity sensory deficits. Gracile lesions are located outside the fourth ventricle in the posteroinferior medulla and were associated with ipsilateral lower-extremity sensory deficits. Trigonal lesions in the ventricular floor were associated with nausea, vomiting, and diplopia. A single surgical approach was preferred (> 90% of cases) for each medullary subtype: the far lateral approach for pyramidal and olivary lesions, the suboccipital-telovelar approach for cuneate lesions, the suboccipital-transcisterna magna approach for gracile lesions, and the suboccipital-transventricular approach for trigonal lesions. Of these 77 patients for whom follow-up data were available (n = 73), 63 (86%) had favorable outcomes and 67 (92%) had unchanged or improved functional status. CONCLUSIONS: This study confirms that the constellation of neurological signs and symptoms associated with a hemorrhagic medullary BSCM subtype is useful for defining the BSCM clinically according to a neurologically recognizable syndrome at the bedside. The proposed taxonomical classifications may be used to guide the selection of surgical approaches, which may enhance the consistency of clinical communications and help improve patient outcomes.

Chronological Progression and Management of Syringobulbia Caused by Spinal Hemangioblastoma: A Case Series and Review of the Literature.

BACKGROUND: Syringomyelia often accompanies spinal hemangioblastoma (SHB). It often shows progression to the medulla oblongata, dubbed as "syringobulbia", which presents critical symptoms such as dysphagia and respiratory compromise. Appropriate management of chronological syringomyelia progression toward syringobulbia is not set in stone. This study aims to unravel the clinical and chronological behavior of syringobulbia and its management. METHODS: A single-institution case series study of 5 patients operated for SHB with syringobulbia was conducted. Serial preoperative magnetic resonance imaging scans were analyzed in further details, especially focusing on the chronological progression of syringomyelia. A literature review was performed to describe clinical/imaging characteristics. RESULTS: Chronological imaging analyses revealed that despite the relatively steady progression of syringomyelia over years, it accelerated when developing syringobulbia. Intramedullary signal change ("presyringomyelia") was observed in the area where syringomyelia subsequently occurred. Literature review yielded another 15 cases of SHB with syringobulbia, totaling 20 cases. Bulbar dysfunction was seen in 4 cases (20%). Gross total resection was performed in all cases except 1, which underwent just syringotomy. Rapid postoperative symptom improvement was observed in all cases, as well as immediate imaging resolution of syringomyelia. CONCLUSIONS: The symptoms associated with syringobulbia often become life-threatening. Notably, its resolution may be near-synchronous to surgical resection of the spinal lesion. The speed of progression of syringomyelia is usually steady, but it may accelerate when extending to syringobulbia. Regular imaging follow-up is thus highly recommended to determine the best timing of intervention when presyringomyelia and syringomyelia are ascending toward the medulla oblongata.

Microsurgical anatomy of safe entry zones on the ventrolateral brainstem: a morphometric study.

Surgery of the brainstem is challenging due to the complexity of the area with cranial nerve nuclei, reticular formation, and ascending and descending fibers. Safe entry zones are required to reach the intrinsic lesions of the brainstem. The aim of this study was to provide detailed measurements for anatomical landmark zones of the ventrolateral surface of the human brainstem related to previously described safe entry zones. In this study, 53 complete and 34 midsagittal brainstems were measured using a stainless caliper with an accuracy of 0.01 mm. The distance between the pontomesencephalic and bulbopontine sulci was measured as 26.94 mm. Basilar sulcus-lateral side of pons (origin of the fibers of the trigeminal nerve) distance was 17.23 mm, transverse length of the pyramid 5.42 mm, and vertical length of the pyramid 21.36 mm. Lateral mesencephalic sulcus was 12.73 mm, distance of the lateral mesencephalic sulcus to the oculomotor nerve 13.85 mm, and distance of trigeminal nerve to the upper tip of pyramid 17.58 mm. The transverse length for the inferior olive at midpoint and vertical length were measured as 5.21 mm and 14.77 mm, consequently. The thickness of the superior colliculus was 4.36 mm, and the inferior colliculus 5.06 mm; length of the tectum was 14.5 mm and interpeduncular fossa 11.26 mm. Profound anatomical knowledge and careful analysis of preoperative imaging are mandatory before surgery of the brainstem lesions. The results presented in this study will serve neurosurgeons operating in the brainstem region.

Resection of a Medulla Oblongata Hemangioblastoma: 2-Dimensional Operative Video.

Hemangioblastomas are benign vascular tumors that can be sporadic or multiple, as part of Von Hippel-Lindau disease. They develop at any level of the central nervous system, with a predilection for the dorsal medulla among brainstem locations. Radical resection of the solid portion of the tumor is the best treatment option.1,2 The resection should be en bloc to avoid uncontrollable intraoperative hemorrhage hindering safe dissection. Preservation of the venous drainage during the progressive dissection of the tumor of the surrounding structures and interruption of numerous small arterial feeders is a tenet for safe surgical resection.3 Once the tumor is completely disconnected, the large draining veins can be coagulated, and the tumor removed. We demonstrate these technical principles in the surgery of a 30-yr-old female with an exophytic hemangioblastoma from the dorsal medulla obstructing the fourth ventricle outflow. We demonstrate the resection of this lesion through a suboccipital craniotomy in a sitting position.4 The patient consented to the surgery and publication of images. Image at 1:26 from Kadri and Al-Mefty,4 by permission from the Congress of Neurological Surgeons.

Transcondylar Approach for Resection of Medullary Cavernous Malformation: 2-Dimensional Operative Video.

Cavernous malformations located within the brainstem present with a high rate of neurological symptoms and carry a more aggressive course in both pediatric and adult populations.1,2 Cavernomas within the medulla are the rarest form, representing only 5% of all brainstem lesions.3 Repeated hemorrhage of brainstem cavernomas is associated with significant and cumulative neurological deficits and thus requires treatment.4 Microsurgical resection has become the optimal mode of treatment with the aim of resecting the live malformation and not merely the multiaged, organized hematoma.4 This is best achieved by approaching the cavernoma at the location where it projects to the surface and entering the lesion through a safe brainstem anatomic zone. For ventrally located lesions in the medulla, a transcondylar skull base approach provides a direct trajectory to the entry zone through a short surgical distance without the need to manipulate or retract neurovascular structures.5-8 Neuronavigation and intraoperative neurophysiological monitoring of somatosensory evoked potential, motor, and lower cranial nerves are adjuncts to increase patient safety. Radiosurgery for the treatment of brainstem cavernous malformations has been proposed; however, it demonstrates high risk and variable and often poor response rates.9 We present a surgical video demonstrating the transcondylar approach and resection of a medullary cavernoma in a 54-yr-old woman who has had multiple known prior hemorrhages and presented with a new onset of facial numbness and weakness, ataxia, and left body sensory loss. The patient consented to surgery and to photograph publication. Images at 1:28, 1:43 (left), 2:02 from Al-Mefty O, Operative Atlas of Meningiomas, © LWW, 1997,5 with permission. Images at 1:43 (right) from Arnautovic et al,8 with permission from JNSPG.

Stereotactic posterior midline approach under direct microscopic view for biopsy of medulla oblongata tumors: technical considerations.

BACKGROUND: Open and stereotactic transfrontal or transcerebellar approaches have been used to biopsy brainstem lesions. METHOD: In this report, a stereotactic posterior and midline approach to the distal medulla oblongata under microscopic view is described. The potential advantages and limitations are discussed, especially bilateral damage of the X nerve nuclei. CONCLUSION: This approach should be considered for biopsy of distal and posterior lesions. We strongly recommend the use of direct microscopic view to identify the medullary vessels, confirm the midline entry point, and avoid potential shift of the medulla. Further experience is needed to confirm safety and success rate of this approach.

Evolution of cerebellomedullary fissure opening: its effects on posterior fossa surgeries from the fourth ventricle to the brainstem.

Surgical approaches to the fourth ventricle and its surrounding brainstem regions have changed significantly in the previous 30 years, after the establishment of cerebellomedullary fissure (CMF) opening. With the development of CMF opening techniques, CMF opening surgeries have become widely used for the treatment of various pathologies and have contributed to the improvement of surgical results in posterior fossa surgeries. We here review the historical progress of CMF opening surgeries to help the future progression of neurosurgical treatments. The authors studied the available literature to clarify how CMF opening surgeries have developed and progressed, and how much the idea and development of CMF opening techniques have affected the advancement of posterior fossa surgeries. With the establishment of angiography, anatomical studies on CMF in the 1960s were performed mainly to clarify vascular anatomy on radiological images. After reporting the microsurgical anatomy of CMF in a cadaveric study in 1982, one of the authors (T.M.) first proposed the clinical usefulness of CMF opening in 1992. This new method enabled wide exposure of the fourth ventricle without causing vermian splitting syndrome, and it took the place of the standard approach instead of the conventional transvermian approach. Several authors reported their experiences using this method from the end of the twentieth century to the early twenty-first century, and the naming of the approach, "telovelar approach" by Mussi and Rhoton in 2000 contributed to the global spread of CMF opening surgeries. The approach has become widely applied not only for tumors but also for vascular and brainstem lesions, and has assisted in the development of their surgical treatments, and brought up the idea of various fissure dissection in the posterior fossa. Studies of microsurgical anatomy of the fourth ventricle, including the CMF, has led to new surgical approaches represented by the transCMF/telovelar approach. The CMF opening method caused a revolution in posterior fossa surgeries. The idea was developed based on the experience gained while dissecting the CMF (the roof of the fourth ventricle) in the laboratory. Anatomical studies using cadaveric specimens, particularly their dissection by surgeons themselves, together with a deep understanding of brain anatomy are essential for further advancements in neurosurgical treatments.

Cumulative surgical morbidity in patients with multiple cerebellar and medullary hemangioblastomas.

OBJECTIVE: Patients affected with von Hippel-Lindau disease often develop multiple hemangioblastomas in the cerebellum and spinal cord. Timing of surgical intervention is difficult and depends largely on the anticipated surgical morbidity. However, data regarding surgical outcome after multiple cerebellar and medullary surgeries are scarce. Our objective was to evaluate cumulative surgical morbidity in patients operated on multiple cerebellar and medullary hemangioblastomas and to deduce recommendations for treatment. METHODS: We performed a retrospective analysis for a consecutive cohort of von Hippel-Lindau patients with surgical treatment of at least two cerebellar and/or medullary hemangioblastomas. Pre- and postoperative functional grades were reviewed in patients' files and compared by Modified Ranking Scale (cerebellar surgeries) or by Modified McCormick Score (medullary surgeries). RESULTS: Thirty-six patients were surgically treated for at least two cerebellar hemangioblastomas (12 patients), at least two medullary hemangioblastomas (19 patients) or at least two hemangioblastomas in both locations (5 patients). Fourthy-eight cerebellar and 80 medullary procedures were performed in total. On average, multiple cerebellar surgeries caused no clinical deterioration, whereas multiple medullary surgeries led to a slight cumulative deterioration of postoperative functional grades. The severity of this deterioration did not correlate to the number of performed medullary surgeries. CONCLUSION: Resection of multiple cerebellar hemangioblastomas is not associated with cumulative morbidity. Although there is a certain cumulative surgical morbidity caused by medullary surgeries, its extent does not increase with the number of performed surgeries. Microsurgical removal of asymptomatic tumors with radiographic progression can also be considered for patients with multiple tumors and previous surgeries.

Complex Cervicomedullary Junction Malformation and Hypoplastic Cerebellar Tonsils following Fetal Repair of Myelomeningocele: Case Report and Literature Review.

BACKGROUND: Following the publication of the Management of Myelomeningocele study (MOMS), fetal repair of myelomeningocele (MMC) has become increasingly prevalent worldwide. However, limited case presentations exist illustrating the potential mechanical and embryological effects of fetal repair. We present a unique case report of a complex embryological cervicomedullary junction (CMJ) malformation and cerebellar hypoplasia following fetal repair of MMC. CASE DESCRIPTION: A 1-day-old female was referred to the paediatric neurosurgical team after having successful surgical intrauterine closure of MMC abroad at 25 weeks gestation. The patient was born by emergency caesarean section at 33 weeks gestation and had a ventricular-peritoneal shunt inserted at 25 days old due to resulting hydrocephalus. Neonatal MRI scans revealed a complex number of malformations that included a split cord located at the CMJ, hypoplasia of the cerebellum and vermis, and a Chiari type II malformation. CONCLUSION: It is possible that the clefting of the upper cervical spinal cord was undetected at preoperative MRI; however, this is unlikely given the antenatal images. It is our hypothesis that the malformation may have exhibited mechanical change after the repair, as the preoperative MRI showed only a Chiari II malformation without any of the complex abnormalities being present and the split cord was already there but not obvious. There are no existing reports of such a complex malformation following antenatal surgery in the literature. This should be further explored as more cases and trials become available.

Far Lateral Approach for In Situ Occlusion of Craniospinal Cervicomedullary Arteriovenous Malformation Presenting with Subarachnoid Hemorrhage.

A 49-year-old woman presented with sudden-onset headache and meningismus. Computed tomography scan revealed a posterior fossa subarachnoid hemorrhage extending from the foramen magnum to the ambient cistern. Angiography showed a pial extramedullary arteriovenous malformation (AVM) at the lateral ventral surface of the cervicomedullary junction with primary supply from the left posterior inferior cerebellar artery (PICA) and dominant drainage into the anterior median perimedullary vein. Embolization of the AVM was considered a high-risk procedure, as feeding arteries originated from the proximal lateral medullary segment of the left PICA. A far lateral approach with suboccipital craniotomy and C1-C2 laminectomy was performed (Video 1). The PICA was disconnected from the AVM, and multiple small feeders were interrupted with bipolar cautery and aneurysm clips to achieve an in situ occlusion of the pial AVM. Intraoperative indocyanine green video angiography was used to define the AVM and critical en passant vessels before disconnection and to demonstrate no residual early venous filling after the in situ occlusion. Postoperative angiography demonstrated no residual arteriovenous shunting or nidiform vessels, with preservation of patency of the left PICA. The patient had an uneventful postoperative course and was discharged with no significant neurologic deficits.

Upbeat vertical nystagmus after brain stem cavernoma resection: a rare case of nucleus intercalatus/nucleus of roller injury.

INTRODUCTION: CNS cavernomas are a type of raspberry-shaped vascular malformations that are typically asymptomatic, but can result in haemorrhage, neurological injury, and seizures. Here, we present a rare case of a brainstem cavernoma that was surgically resected whereafter an upbeat nystagmus presented postoperatively. CASE REPORT: A 42-year old man presented with sudden-onset nausea, vomiting, vertigo, blurred vision, marked imbalance and difficulty swallowing. Neurological evaluation showed bilateral ataxia, generalized hyperreflexia with left-sided predominance, predominantly horizontal gaze evoked nystagmus on right and left gaze, slight left labial asymmetry, uvula deviation to the right, and tongue deviation to the left. MRI demonstrated a 13-mm cavernoma with haemorrhage and oedema in the medulla oblongata. Surgery was performed via a minimal-invasive, midline approach. Complete excision was confirmed on postoperative MRI. The patient recovered well and became almost neurologically intact. However, he complained of mainly vertical oscillopsia. The videonystagmography revealed a new-onset spontaneous upbeat nystagmus in all gaze directions, not suppressed by fixation. An injury of the rarely described intercalatus nucleus/nucleus of Roller is thought to be the cause. CONCLUSION: Upbeat nystagmus can be related to several lesions of the brainstem, including the medial longitudinal fasciculus, the pons, and the dorsal medulla. To our knowledge, this is the first case of an iatrogenic lesion of the nucleus intercalatus/nucleus of Roller resulting in an upbeat vertical nystagmus. For neurologists, it is important to be aware of the function of this nucleus for assessment of clinical manifestations due to lesions within this region.

Ventrolateral Tonsillar Position Defines Novel Chiari 0.5 Classification.

BACKGROUND: Cervicomedullary compression in young children has been described in the context of Chiari type 1 malformation, with symptoms associated with the extent of tonsillar herniation below McRae line. Historically, Chiari type 1 malformation has been defined by tonsillar herniation of at least 5 mm. However, in certain populations, including very young children, Chiari symptoms may be present without this finding. A new Chiari classification is thus necessary. METHODS: Cases involving patients up to 5 years of age evaluated for possible posterior fossa decompression were retrospectively reviewed. Preoperative symptoms, magnetic resonance imaging findings, surgical management, and short- and long-term outcome and follow-up were recorded. Tonsillar descent and presence of ventral herniation (VH) were recorded. We define VH as the tonsils crossing a line that bisects the caudal medulla at the level of the foramen magnum, thus creating a novel entity, Chiari type 0.5 malformation. Patients with ventrally herniated tonsils were compared with patients exhibiting more typical Chiari morphology. RESULTS: Of 41 cases retrospectively reviewed, 20 met criteria for VH. These differed from cases without VH because of the predominance of medullary symptoms. In the VH cohort, 11 patients underwent surgical decompression with symptom resolution; 9 were initially managed conservatively, but 3 subsequently required surgery. CONCLUSIONS: We define a novel Chiari entity, Chiari type 0.5 malformation, characterized by ventral tonsillar wrapping around the medulla in young children in the absence of classic Chiari type 1 malformation imaging findings. These patients are more likely to present with medullary symptoms than patients without VH. They are also more likely to require surgical decompression and respond favorably to intervention.

A Rare Case of a Pediatric Medullary Intracranial Germinoma.

BACKGROUND: Germinomas are rare intracranial lesions, with medulla germinomas being reported in only approximately 20 cases to date. These tumors are extremely sensitive to radiotherapy, and knowledge of them can allow for accurate diagnosis as well as significantly impact treatment and prognosis. CASE DESCRIPTION: We present a case of medullary germinoma and review the cases previously reported in the literature. CONCLUSIONS: A few imaging characteristics are noted in multiple cases, such as cystic components, enhancement, and absence of hydrocephalus. These features can provide clues to diagnosis of this rare entity.

Rare occurrence of gangliocytoma in the medulla oblongata.

Gangliocytoma is an uncommon low-grade neuroepithelial tumour arising in the cerebral hemispheres, with medulla oblongata being extremely rare site of occurrence. We report a case of an 8-year-old female with recurrent vomiting, hypertension and left hemifacial spasms with a dorsally exophytic lesion arising from the medulla projecting into the fourth ventricle. She underwent excision of the lesion with intra operative cranial nerve monitoring guidance. Histopathology revealed clusters of large mature ganglion cells which were labelled by synaptophysin and CD34. Extreme rarity in this location makes clinical diagnosis and management of gangliocytoma challenging, although prompt diagnosis and total excision has a successful outcome. There are only six previous reports of gangliocytoma in the medulla oblongata.