ESAT-6-specific CD4 T cell responses to aerosol Mycobacterium tuberculosis infection are initiated in the mediastinal lymph nodes.

CD4(+) T cell responses to aerosol Mycobacterium tuberculosis (Mtb) infection are characterized by the relatively delayed appearance of effector T cells in the lungs. This delay in the adaptive response is likely critical in allowing the bacteria to establish persistent infection. Because of limitations associated with the detection of low frequencies of naïve T cells, it had not been possible to precisely determine when and where naïve antigen-specific T cells are first activated. We have addressed this problem by using early secreted antigenic target 6 (ESAT-6)-specific transgenic CD4 T cells to monitor early T cell activation in vivo. By using an adoptive transfer approach, we directly show that T cell priming to ESAT-6 occurs only after 10 days of infection, is initially restricted to the mediastinal lymph nodes, and does not involve other lymph nodes or the lungs. Primed CD4 T cells rapidly differentiated into proliferating effector cells and ultimately acquired the ability to produce IFN-gamma and TNF-alpha ex vivo. Initiation of T cell priming was enhanced by two full days depending on the magnitude of the challenge inoculum, which suggests that antigen availability is a factor limiting the early CD4 T cell response. These data define a key period in the adaptive immune response to Mtb infection.

Spontaneous chronic subdural hematoma due to cerebral cortical artery rupture: First case report and review of pertinent literature.

INTRODUCTION: Chronic subdural hematoma (CSDH) is generally attributed to pathological changes in the dural arterial system, which has led to growing utilization of middle meningeal artery (MMA) embolization in CSDH treatment. There are no case reports of CSDH due to cerebral cortical artery perforation. METHOD: We report a case of spontaneous CSDH caused by cortical artery rupture that was identified on digital subtraction angiography and could be visualized on computed tomographic angiography (CTA). We also present a brief review of the pertinent literature. RESULT: A 56-year-old man presented with daily headache for three weeks with no history of trauma. Neurologic exam was normal except for subtle left pronator drift. A computed tomography scan of the head showed a right CSDH with a midline shift. CTA was suspicious for a dilated vein in the subdural collection. Catheter cerebral angiogram demonstrated contrast extravasation from a small cortical branch of the middle cerebral artery with no aneurysm, arterial dissection, or vascular malformation. In retrospect, CTA findings represented contrast extravasation, not a dilated vein. Burr-hole mini-craniotomy was performed, and the hematoma was evacuated. The patient had complete clinical and radiographic recovery. CONCLUSION: Ruptured cerebral cortical artery can cause CSDH. In atypical cases of CSDH, careful evaluation of selective internal carotid artery angiography is advised to look for alternative pathologies prior to proceeding with MMA embolization. CTA images may demonstrate active extravasation from a small cortical artery.

Subgaleal hematoma evacuation in a pediatric patient: A case report and review of the literature.

BACKGROUND: Subgaleal hematoma (SGH) is generally documented within the neonatal period and is rarely reported as a result of trauma or hair braiding in children. While rare, complications of SGH can result in ophthalmoplegia, proptosis, visual deficit, and corneal ulceration secondary to hematoma extension into the orbit. Although conservative treatment is preferential, expanding SGH should be aspirated to reduce complications associated with further expansion. CASE DESCRIPTION: A 12-year-old African-American female with no recent history of trauma presented with a chief complaint of headache along with a 2-day history of enlarging 2-3 cm ballotable bilateral frontal mass. Hematological workup was negative. The patient's family confirmed a long history of hair braiding. The patient was initially prescribed a period of observation but returned 1-week later with enlarging SGH, necessitating surgical aspiration. CONCLUSION: SGH is rare past the neonatal period, but can be found in pediatric and adolescent patients secondary to trauma or hair pulling. Standard workup includes evaluation of the patient's hematological profile for bleeding or coagulation deficits, as well as evaluation for child abuse. Although most cases of SGH resolve spontaneously over the course of several weeks, close follow-up is recommended. The authors present a case of a 12-year-old female presenting with enlarging subgaleal hemorrhages who underwent surgical aspiration and drainage without recurrence. A literature review was also conducted with 32 pediatric cases identified, 20 of which were related to hair pulling, combing, or braiding. We review the clinical course, imaging characteristics, surgical management, as well as a review of the literature involving subgaleal hemorrhage in pediatric patients and hair pulling.

Pediatric sellar solitary fibrous tumor/ hemangiopericytoma: A rare case report and review of the literature.

BACKGROUND: Solitary fibrous tumor (SFT)/hemangiopericytoma (HPC) is a rare tumor which originates from the walls of capillaries and has historically been thought to be able to occur anywhere in the body that blood vessels are found. It is rarely found in the sellar region. CASE DESCRIPTION: InS this report, we present the first case of this tumor occurring in the sellar region of a pediatric patient. This 12-year-old male presented with progressive vision loss which prompted surgical resection after a sellar lesion was discovered on imaging. The initial transsphenoidal approach resulted in subtotal resection and the patient experienced reoccurrence within 3 months. He underwent an orbitozygomatic craniotomy to achieve gross total tumor resection. CONCLUSION: We conducted a literature review of intracranial SFT/HPC in the pediatric population and found it to be an extremely rare occurrence, with <30 cases reported. The incidence of SFT/HPC occurring in the sellar region for any age group was also found to be a rare entity. Treatment recommendations for this tumor are also scarce, based on retrospective chart reviews from the adult population. The role for adjuvant radiation has mixed results.

Spontaneous Intracranial Hypotension: Case Study and Review of the Literature.

Spontaneous intracranial hypotension (SIH) is a pathology characterized by orthostatic headaches, diffuse pachymeningeal enhancement on magnetic resonance imaging (MRI), and low to normal cerebrospinal fluid (CSF) pressures. We present the case of a 46-year-old male with refractory postural headaches, found to have a diffuse CSF leak throughout the cervicothoracic (C1-T12) spine. His neurological status declined rapidly to a Glasgow Coma Scale (GCS) of eight, necessitating bilateral subdural drain placement. Despite an overall brisk neurologic recovery, the patient remained unable to speak for nearly a week after the return of the remainder of his function. This raised the concern for possible cerebellar mutism. We review the multiple modalities used in this patient's treatment and explore possible explanations for the failure of initial therapy. The placement of bilateral subdural drains was a temporizing measure to treat the patient's neurologic decline, but it was likely the epidural blood patch with prolonged bedrest that hastened the patient's recovery. His speech function also returned with time and repeated therapy.

Pediatric Intracavernous Sinus Lesions: A Single Institutional Surgical Case Series and Review of the Literature.

BACKGROUND: Pediatric intracavernous sinus tumors are exceedingly rare and thus poorly characterized. Their neurosurgical management is challenging and diagnostic, and management guidelines are limited. OBJECTIVE: To report our institutional experience with the surgical resection of pediatric intracavernous sinus tumors. We also compare and contrast our results with the 14 cases of pediatric intracavernous sinus lesions in the current literature. METHODS: A retrospective descriptive analysis of consecutive pediatric patients (ages 0-18 yr) presenting to our institution with a diagnosis of an intracavernous sinus lesion was performed. From January 2012 to January 2017, 5 cases were identified. Eleven patients with secondary invasion of the cavernous sinus (2 meningiomas, 7 pituitary adenomas) or dermoid tumors involving the cavernous sinus (2) were not included in our review. RESULTS: Surgical resection via a frontotemporal orbitozygomatic approach was performed in all cases by a single senior neurosurgeon (M.L.). There were no perioperative or postoperative complications attributable to the surgery or approach. Four of 5 patients remained neurologically stable throughout the perioperative and postoperative period. The fifth patient had a complete resolution of their cranial neuropathies postoperatively. A pathological diagnosis that guided long-term management was obtained in all cases. CONCLUSION: Neurosurgical management of pediatric cavernous sinus lesions can be safely performed and critically guide future therapies. Surgeon familiarity with cavernous sinus and skull-base anatomy is critical to the successful management of these patients. The benefits of surgery should be balanced against the potential complications and need for a tissue diagnosis in children. The senior author had a significant experience with cavernous sinus approaches in adults prior to initiating use of the approach in the pediatric population.

Endoscopic surgery for nonsyndromic craniosynostosis: a 16-year single-center experience.

OBJECTIVE: In this paper the authors review their 16-year single-institution consecutive patient experience in the endoscopic treatment of nonsyndromic craniosynostosis with an emphasis on careful review of any associated treatment-related complications and methods of complication avoidance, including preoperative planning, intraoperative management, and postoperative care and follow-up. METHODS: A retrospective chart review was conducted on all patients undergoing endoscopic, minimally invasive surgery for nonsyndromic craniosynostosis at Rady Children's Hospital from 2000 to 2015. All patients were operated on by a single neurosurgeon in collaboration with two plastic and reconstructive surgeons as part of the institution's craniofacial team. RESULTS: Two hundred thirty-five patients underwent minimally invasive endoscopic surgery for nonsyndromic craniosynostosis from 2000 to 2015. The median age at surgery was 3.8 months. The median operative and anesthesia times were 55 and 105 minutes, respectively. The median estimated blood loss (EBL) was 25 ml (median percentage EBL 4.2%). There were no identified episodes of air embolism or operative deaths. One patient suffered an intraoperative sagittal sinus injury, 2 patients underwent intraoperative conversion of planned endoscopic to open procedures, 1 patient experienced a dural tear, and 1 patient had an immediate reexploration for a developing subgaleal hematoma. Two hundred twenty-five patients (96%) were admitted directly to the standard surgical ward where the median length of stay was 1 day. Eight patients were admitted to the intensive care unit (ICU) postoperatively, 7 of whom had preexisting medical conditions that the team had identified preoperatively as necessitating a planned ICU admission. The 30-day readmission rate was 1.7% (4 patients), only 1 of whom had a diagnosis (surgical site infection) related to their initial admission. Average length of follow-up was 2.8 years (range < 1 year to 13.4 years). Six children (< 3%) had subsequent open procedures for perceived suboptimal aesthetic results, 4 of whom (> 66%) had either coronal or metopic craniosynostosis. No patient in this series either presented with or subsequently developed signs or symptoms of intracranial hypertension. CONCLUSIONS: In this large single-center consecutive patient series in the endoscopic treatment of nonsyndromic craniosynostosis, significant complications were avoided, allowing for postoperative care for the vast majority of infants on a standard surgical ward. No deaths, catastrophic postoperative morbidity, or evidence of the development of symptomatic intracranial hypertension was observed.

Extended middle fossa approach to lateralized pontine cavernomas in children.

OBJECTIVE Treatment of hemorrhagic cavernous malformations within the lateral pontine region demands meticulous surgical planning and execution to maximize resection while minimizing morbidity. The authors report a single institution's experience using the extended middle fossa rhomboid approach for the safe resection of hemorrhagic cavernomas involving the lateral pons. METHODS A retrospective chart review was performed to identify and review the surgical outcomes of patients who underwent an extended middle fossa rhomboid approach for the resection of hemorrhagic cavernomas involving the lateral pons during a 10-year period at Rady Children's Hospital of San Diego. Surgical landmarks for this extradural approach were based on the Fukushima dual-fan model, which defines the rhomboid based on the following anatomical structures: 1) the junction of the greater superficial petrosal nerve (GSPN) and mandibular branch of the trigeminal nerve; 2) the lateral edge of the porus trigeminus; 3) the intersection of the petrous ridge and arcuate eminence; and 4) the intersection of the GSPN, geniculate ganglion, and arcuate eminence. The boundaries of maximal bony removal for this approach are the clivus inferiorly below the inferior petrosal sinus; unroofing of the internal auditory canal posteriorly; skeletonizing the geniculate ganglion, GSPN, and internal carotid artery laterally; and drilling under the Gasserian ganglion anteriorly. This extradural petrosectomy allowed for an approach to all lesions from an area posterolateral to the basilar artery near its junction with cranial nerve (CN) VI, superior to the anterior inferior cerebellar artery and lateral to the origin of CN V. Retraction of the mandibular branch of the trigeminal nerve during this approach allowed avoidance of the region involving CN IV and the superior cerebellar artery. RESULTS Eight pediatric patients (4 girls and 4 boys, mean age of 13.2 ± 4.6 years) with hemorrhagic cavernomas involving the lateral pons and extension to the pial surface were treated using the surgical approach described above. Seven cavernomas were completely resected. In the eighth patient, a second peripheral lesion was not resected with the primary lesion. One patient had a transient CN VI palsy, and 2 patients had transient trigeminal hypesthesia/dysesthesia. One patient experienced a CSF leak that was successfully treated by oversewing the wound. CONCLUSIONS The extended middle fossa approach can be used for resection of lateral pontine hemorrhagic cavernomas with minimal morbidity in the pediatric population.

Robot-assisted endoscopic third ventriculostomy: institutional experience in 9 patients.

OBJECTIVE The endoscopic third ventriculostomy (ETV) is an established and effective treatment for obstructive hydrocephalus. In its most common application, surgeons plan their entry point and the endoscope trajectory for the procedure based on anatomical landmarks, then control the endoscope freehand. Recent studies report an incidence of neural injuries as high as 16.6% of all ETVs performed in North America. The authors have introduced the ROSA system to their ETV procedure to stereotactically optimize endoscope trajectories, to reduce risk of traction on neural structures by the endoscope, and to provide a stable mechanical holder of the endoscope. Here, they present their series in which the ROSA system was used for ETVs. METHODS At the authors' institution, they performed ETVs with the ROSA system in 9 consecutive patients within an 8-month period. Patients had to have a favorable expected response to ETV (ETV Success Score ≥ 70) with no additional endoscopic procedures (e.g., choroid plexus cauterization, septum pellucidum fenestration). The modality of image registration (CT, MRI, surface mapping, or bone fiducials) was dependent on the case. RESULTS Nine pediatric patients with an age range of 1.5 to 16 years, 4 girls and 5 boys, with ETV Success Scores ranging from 70 to 90, underwent successful ETV surgery with the ROSA system within an 8-month period. Their intracranial pathologies included tectal tumors (n = 3), communicating hydrocephalus from hemorrhage or meningeal disease (n = 2), congenital aqueductal stenosis (n = 1), compressive porencephalic cyst (n = 1), Chiari I malformation (n = 1), and pineal region mass (n = 1). Robotic assistance was limited to the ventricular access in the first 2 procedures, but was used for the entirety of the procedure for the following 7 cases. Four of these cases were combined with another procedural objective (3 stereotactic tectal mass biopsies, 1 Chiari decompression). A learning curve was observed with each subsequent surgery as registration and surgical times became shorter and more efficient. All patients had complete resolution of their preprocedural symptoms. There were no complications. CONCLUSIONS The ROSA system provides a stable, precise, and minimally invasive approach to ETVs.

Intraventricular hemorrhage after ventriculoperitoneal shunt revision: a retrospective review.

OBJECT The authors review their ventriculoperitoneal (VP) shunt revisions over a 3-year period to determine the rate of intraventricular hemorrhage (IVH) and subsequent need for re-revision. METHODS Review of medical records identified 35 pediatric patients who underwent 52 VP shunt revisions between 2009 and 2012. The presence and amount of IVH as determined by CT and the time to re-revision were documented. The reason for shunting, catheter position, and time between initial VP shunt placement and subsequent revisions were also recorded. RESULTS After 13 (25%) of the 52 revisions, IVH was evident on postoperative CT scans. The majority of patients had a trace amount of IVH, with only 2% having IVH greater than 5 ml. After 2 (15%) of the 13 revisions associated with IVH, re-revision was required within 1 month. In contrast, the re-revision rate in patients without IVH was 18%. All of the patients who developed IVH had occipital catheters. CONCLUSIONS Some degree of IVH can be expected after approximately one-quarter of all VP shunt revision procedures in pediatric patients, but the rate of significant IVH is low. Furthermore, the presence of IVH does not necessitate an early shunt revision.

Fetal ovarian cyst mimicking a CSF pseudocyst in the setting of shunt failure.

Fetal ovarian cysts are common congenital lesions encountered in the neonatal population. These cysts are typically benign and rarely require any invasive intervention. Abdominal pseudocyst formation as a result of a ventriculoperitoneal shunt is a relatively infrequent occurrence and is similarly an uncommon cause of shunt failure. The authors present the case of a 4-month-old girl with shunted hydrocephalus who presented with shunt failure from a suspected abdominal pseudocyst that was found to be a fetal ovarian cyst.

Endovascular treatment for moyamoya disease in a Caucasian twin with angioplasty and Wingspan stent.

The pathogenesis and genetics of moyamoya disease (MMD) remain a mystery. Here we report a case of female Caucasian adult twins with similar presentations of bilateral MMD that were treated with different surgical modalities. One twin was treated with a bypass and remains event free (defined as either transient ischemic event or stroke) at her 4-year follow-up. The second twin underwent angioplasty to treat a left middle cerebral artery stenosis as well as the placement of a Wingspan stent to treat a right supraclinoid ICA stenosis on separate occasions. The left middle cerebral artery angioplasty thrombosed due to the discontinuation of clopidogrel resulting in recurrent symptoms, principally aphasia, and the Wingspan stent underwent angioplasty for in-stent stenosis. Despite this, the stent has been patent and the patient has remained event free at her 2-year follow-up, post-placement. The relatively new Wingspan stent has been used in the past for intracerebral atherosclerotic lesions, however, this is the first report of it being used in a case of MMD. An overview of the MMD literature in twins and endovascular therapy is given.