Waking up from Coronary Bypass Surgery and One Eye does not Move Right

Introduction

Complications of coronary artery bypass graft surgery (CABG) include acute oculomotor nerve palsy secondary to ischemic stroke and pituitary apoplexy. These can present with impairment of extraocular muscle function as well as involvement or sparing of the pupil.

Case Report

We report the case of a 58-year-old male admitted for elective CABG surgery for severe coronary artery disease and found to have a pupil-sparing partial oculomotor palsy post-procedure. Neurological examination revealed left pupil-sparing isolated medial rectus and levator palpebrae superioris paresis. Magnetic resonance imaging demonstrated acute midbrain infarction.

Conclusion

Acute pupil-sparing partial oculomotor nerve palsy should be recognized as a neurological complication of cardiac surgery. Pupillary involvement can be helpful in identifying the underlying etiology.     

Ultra-Early Aneurysmal Rebleeding and Brainstem Destruction

Background

Early secondary neurologic deterioration after aneurysmal subarachnoid hemorrhage (SAH) may have many causes including rebleeding, hydrocephalus, parenchymal hematoma, or seizures.

Methods

Case report.

Results

A 69-year-old woman presented with thunderclap headache and nausea. A head computed tomography (CT) showed SAH. On initial evaluation she was awake, alert, and confused without focal neurologic deficits. Two episodes of marked clinical deterioration occurred, manifesting as acute unresponsiveness and fixed anisocoria. Serial head CTs showed massive extension of hemorrhage into the brainstem parenchyma and ventricles.

Conclusions

Sudden clinical deterioration after SAH with coma and a fixed ??blown?? pupil may result from hemorrhage extension into the brainstem parenchyma rather than oculomotor nerve injury from compression or stretch.     

Suprasellar granular cell tumor of the neurohypophysis in a child: unusual presentation in pediatric age of a rare tumor

Purpose

Granular cell tumors (GCT) of the neurohypophysis are rare, solitary, nodular-shaped lesions, mostly presenting in the adult age with a female predilection. They rarely grow to a sufficient size to cause mass effect related symptoms and they may be found in most cases incidentally at autopsy of older patients. Few cases of symptomatic GCT of the neurohypophysis have been reported in the literature and only one of these in a pediatric patient in the first decade of life, who presented with central precocious puberty.

Methods

We report the case of a 11-year-old boy with a large suprasellar GCT of the neurohypophysis, complaining severe headache and pituitary insufficiency. Before our referral, the child was operated at another insitution through a pterional approach for tumor biopsy and underwent chemotherapy because of the misleading diagnosis of glioma.

Results

The patient was operated on by a fronto-orbito-zygomatic approach with subtotal tumor resection. At last follow-up examination, a partial hypopituitarism was detected. The quality of life with replacement therapy was excellent. Fractionated radiotherapy on tumor remnant was advised.

Conclusions

The reported case is exceptional because the tumor developed in a male pediatric patient, causing clinical symptoms related to intracranial hypertension and unusual endocrinological features. GCT has to be considered in the differential diagnosis of suprasellar masses, to avoid misleading interpretation and consequent wrong therapeutic management.     

The Jugular Foramen Schwannomas: Review of the Large Surgical Series

Objective

Jugular foramen schwannomas are uncommon pathological conditions. This article is constituted for screening these tumors in a wide perspective.

Materials

One-hundred-and-ninty-nine patients published in 19 articles between 1984 to 2007 years was collected from Medline/Index Medicus.

Results

The series consist of 83 male and 98 female. The mean age of 199 operated patients was 40.4 years. The lesion located on the right side in 32 patients and on the left side in 60 patients. The most common presenting clinical symptoms were hearing loss, tinnitus, disphagia, ataxia, and hoarseness. Complete tumor removal was achieved in 159 patients. In fourteen patients tumor reappeared unexpectedly. The tumor was thought to originate from the glossopharyngeal nerve in forty seven cases; vagal nerve in twenty six cases; and cranial accessory nerve in eleven cases. The most common postoperative complications were lower cranial nerve palsy and facial nerve palsy. Cerebrospinal fluid leakage, meningitis, aspiration pneumonia and mastoiditis were seen as other complications.

Conclusion

This review shows that jugular foramen schwannomas still have prominently high morbidity and those complications caused by postoperative lower cranial nerve injury are life threat.     

Cavernous hemangiomas of the cranial vault in infants: a case-based update

Background

Mass lesions arising in the scalp or the cranial vault of children require a significant diagnostic work-up but their nature, in most instances, can only be clarified after histopathological study. Tumors of osseous consistency or merging from within cranial bones are seldom susceptible of fine needle aspiration biopsy and require excision. Ossified cephalhematoma is a rare entity that is usually related to perinatal or accidental trauma. Exceptionally, ossified cephalhematoma has a non-traumatic origin and arises from a different condition, as vascular tumor or malformation.

Discussion

Cavernomas of the skull are infrequent and occur in middle-aged individuals. Skull cavernomas in infants lack the sunburst radiographic pattern seen in adults and may present as a bone-like tumor whose appearance is identical to that of ossified cephalhematoma. A literature search showed only two previous reports of patients younger than 1 year with skull cavernomas presenting with associated ossified cephalhematomas. We hypothesize that this type of skull cavernomas are congenital in origin and that they manifest precociously due to the occurrence of early bleeding.

Case illustration

We report a 12-month-old boy presenting with a hard tumor arising on his right parietal bone that was present since birth. After diagnostic work-up, the bone tumor and the associated intraosseous hematoma were removed. Interestingly, the source of the hematoma was a cavernous hemangioma originating in the skull.

Conclusions

Calvarial cavernous hemangiomas may bleed precociously, even before birth, and manifest as large intraosseous hematomas. Differential diagnosis against ossified cephalhematoma can only be established through histopathological study.     

Intraoperative pre- and post-craniofacial reconstruction intracranial pressure (ICP) monitoring in children with craniosynostosis

Introduction

One of the goals of cranial vault expansion performed in patients with craniosynostosis (CS) is to reduce the harmful effects associated with elevated intracranial pressure (ICP). Until now, clear guidelines on when cranial vault expansion should take place have not been established except in unacceptable cosmetic deformities.

Materials and methods

This paper illustrates the potential benefit of ICP monitoring in determining the time of surgery. The ICP of six patients (ranging from 7 months to 8 years) was measured before and after surgery. For the first time, we regulated end-tidal carbon dioxide, the position and movements, the level of sedation and the monitoring site of our patients under anesthesia to report accurate ICP readings.

Results

The mean pre- and postoperative ICPs were 14.7 and 4.2 mmHg, respectively. Pressure sensor was placed through a burr hole under general anesthesia and remained through all stages of recording. Though ICP monitoring has been reported before, the physiological fluctuations of ICP and patient’s condition affected results. Under our ICP monitoring protocol, the six-patient study represents a suggestion to standardize ICP measurements under certain conditions in order to improve the reproducibility of ICP monitoring and therefore establish the need for optimal timing of cranial vault expansion in pediatrics.

Conclusion

Although we cannot clearly define the indications and establish normal pediatric ICP values from the result of this study because of the small number of cases and some other limitations, this is a new approach to define ICP increase as a potential indication for surgery in CS.     

Pediatric intracranial aneurysms—our experience and review of literature

Purpose

Intracranial aneurysms in children are not as common as in adults and there are many differences in the etiology, demographic variables, aneurysm location, aneurysm morphological characteristics, clinical presentation, and outcome in pediatric and adult intracranial aneurysms.

Methods

All children (≤18 years) suffering from intracranial aneurysm managed at our center from July 2001 through June 2013 were included in the study, and the details of these patients were retrieved from the computerized database of our hospital.

Observations

A total of 62 pediatric patients were treated for 74 aneurysms during the study period and constituted 2.3 % of all intracranial aneurysms treated during the same period. The mean age at presentation was 13.5 years. Headache (82 %) was the commonest presenting feature; other symptoms included seizures (21 %), ictal loss of consciousness (27 %), and motor/cranial nerve deficits (22.6 %). Computed tomogram revealed subarachnoid hemorrhage in 58 % of patients. Eighty-two percent of aneurysms were in anterior circulation. Sixty-seven percent of aneurysms were complex aneurysms. Fifty-eight percent of patients underwent surgical intervention while 30 % underwent endovascular procedures. Twenty-one percent of the patients developed vasospasm. There was no postoperative mortality. Favorable outcome was seen in 72 % of the patients.

Conclusions

Pediatric intracranial aneurysms are uncommon as compared to in adult patients. Seizures and cranial nerve involvement are seen more often as the presenting features in children. Posterior circulation aneurysms are more common in children, as are the internal carotid artery bifurcation aneurysms. There is high incidence of giant, posttraumatic, and mycotic aneurysms in children.     

Tectal plate gliomas: a review

Introduction

Tectal plate gliomas are generally benign neoplastic lesions arising in the brainstem which can, with local extension, obstruct the aqueduct of Sylvius and lead to hydrocephalus.

Anatomy

Diagnosis is based on initial suspicion fostered by the presentation of an obstructive hydrocephalus followed by physical exam which may potentially reveal indications of pyramidal tract dysfunction or cranial nerve palsies.

Discussion

MRI studies reveal a characteristic well-circumscribed, isodense or hypodense mass on T1-weighted images, with hyperdensity on T2 imaging. Yet current radiological methods insufficiently distinguish tectal plate gliomas from brainstem tumors or gliomas in the neighboring structures, and a definitive diagnosis requires biopsy and histopathological analysis. Management is planned according to the degree of associated signs and symptoms, and may range from diligent observation and periodic screening for advancing tumor development, to cerebrospinal fluid shunting in an effort to resolve obstructive hydrocephalus, to radio- and chemotherapy. A wide range of minimally invasive approaches using endoscopy is available for the neurosurgeon, including endoscopic third ventriculostomy and endoscopic aqueductoplasty.     

The fallopian canal: a comprehensive review and proposal of a new classification

Introduction

The facial nerve follows a complex course through the skull base. Understanding its anatomy is crucial during standard skull base approaches and resection of certain skull base tumors closely related to the nerve, especially, tumors at the cerebellopontine angle.

Methods

Herein, we review the fallopian canal and its implications in surgical approaches to the skull base. Furthermore, we suggest a new classification.

Conclusions

Based on the anatomy and literature, we propose that the meatal segment of the facial nerve be included as a component of the fallopian canal. A comprehensive knowledge of the course of the facial nerve is important to those who treat patients with pathology of or near this cranial nerve.     

Total ipsilateral C7 root neurotization to the upper trunk for isolated C5-C6 avulsion in obstetrical brachial plexus palsy: a preliminary technical report

Background

C5-C6 root avulsion in obstetrical brachial plexus palsy (OBPP) is a rare injury with poor prognosis usually associated with breech delivery. The treatment is challenging and requires high microsurgical skills. The triple nerve transfer (spinal accessory nerve, ulnar fascicles, and triceps long or lateral head branch) represents the gold standard treatment. The total ipsilateral C7 nerve root neurotization is a promising technique, which has never been described in OBPP.

Objective

The total ipsilateral C7 nerve root is used as a neurotizer transferred to the upper trunk as an alternative method to other intra- or extra-plexual reconstruction techniques.

Methods

During brachial plexus surgical exploration, an intraoperative neurostimulation was performed to confirm the integrity of C7 and the lesion of C5 and C6. The entire C7 nerve root and the upper trunk are cut. The C7 root was transferred to the upper trunk with a fibrin sealant.

Result

This technique was easily performed with a single approach and avoided intercalated nerve grafts. The C7 nerve root provided a large number of nerve fibers with an adequate diameter to be transferred to the upper trunk. We illustrated this technique with a typical case of a child at 8 years of follow-up.

Conclusion

The total ipsilateral transfer of the C7 root to the upper trunk is a viable alternative procedure for newborns with C5-C6 avulsion.     

Deep renal ectopia causing sciatic mononeuropathy

Introduction

Sciatic mononeuropathy resulting from compression by an adjacent pelvic ectopic kidney has not been reported in the literature. Here, the authors present a case of a patient presenting with unilateral lower extremity paresthesia correlating to the sciatic nerve distribution.

Results

MRI examination demonstrated lumbosacral plexus compression by a very unusual deeply situated renal ectopia. Physicians should consider other rare causes of mechanical lumbosacral plexus compression in patients with sciatic symptomatology.     

Routine sectioning of the C2 nerve root and ganglion for C1 lateral mass screw placement in children: surgical and functional outcomes

Purpose

Adult studies have shown that sectioning the C2 nerve root and ganglion may facilitate placement of C1 lateral mass screws and lead to decreased operative time and blood loss. We report the functional outcomes and complications following routine sectioning of the C2 nerve root and ganglion, which have not been reported in pediatric patients.

Methods

Fifteen consecutive pediatric patients underwent C1 lateral mass screw insertion and bilateral C2 nerve root and ganglion sectioning. Clinical and radiographic assessments were performed at follow-up. Numbness in the C2 distribution and/or occipital neuralgia, operative times, estimated blood loss (EBL), length of stay (LOS), and complications were recorded.

Results

Average follow-up time was 35.7 months. Overall mean operative time was 250.5 min, LOS was 8.46 days, and EBL was 337 cc. When considering only atlantoaxial fusions, mean operative time was 180.7 min and EBL was 97.1 cc. There were no intraoperative complications, and no patient reported new onset occipital neuralgia or numbness in the C2 distribution that would interfere with daily living. Of the patients, 93 % achieved Lenke fusion grade A; one achieved Lenke fusion grade B.

Conclusions

Routine C2 nerve root sectioning and ganglionectomy enhanced surgical exposure of the C1 lateral mass and C1–2 facet joint, potentially maximizing fusion rate and minimizing intraoperative complications. This technique may yield favorable operative times, EBL, and LOS in children undergoing C1 lateral mass screw insertion without negatively affecting functional outcome.     

Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome

Introduction

Posterior fossa tumors are the most common brain tumor of children. Aggressive resection correlates with long-term survival. A high incidence of posterior fossa syndrome (PFS), impairing the quality of life in many survivors, has been attributed to damage to bilateral dentate nucleus or to cerebellar output pathways. Using diffusion tensor imaging (DTI), we examined the involvement of the dentothalamic tracts, specifically the superior cerebellar peduncle (SCP), in patients with posterior fossa tumors and the association with PFS.

Methods

DTI studies were performed postoperatively in patients with midline (n?=?12), lateral cerebellar tumors (n?=?4), and controls. The location and visibility of the SCP were determined. The postoperative course was recorded, especially with regard to PFS, cranial nerve deficits, and oculomotor function.

Results

The SCP travels immediately adjacent to the lateral wall of the fourth ventricle and just medial to the middle cerebellar peduncle. Patients with midline tumors that still had observable SCP did not develop posterior fossa syndrome (N?=?7). SCPs were absent, on either preoperative (N?=?1, no postoperative study available) or postoperative studies (N?=?4), in the five patients who developed PFS. Oculomotor deficits of tracking were observed in patients independent of PFS or SCP involvement.

Conclusion

PFS can occur with bilateral injury to the outflow from dentate nuclei. In children with PFS, this may occur due to bilateral injury to the superior cerebellar peduncle. These tracts sit immediately adjacent to the wall of the ventricle and are highly vulnerable when an aggressive resection for these tumors is performed.     

Dose-dependent relapse of hiatus hernia after administration of intrathecal baclofen treatment—a rare complication

Introduction

Intrathecal baclofen treatment (ITB) is widely used in children with cerebral palsy. Although this treatment effectively reduces spasticity, diverse side effects are reported.

Case report

We report about a boy with severe asphyxia-induced encephalopathy with bilateral cerebral palsy. After starting the intrathecal baclofen treatment, he episodically showed symptoms of severe gastroesophageal reflux with pale skin color, vomiting, massive drooling, acid regurgitation, and reduced well-being. An open ventral semifundoplication was done some years ago to treat a gastroesophageal reflux. These symptomatic episodes occurred strongly dose-dependent and were not observed during the short test procedure.

Conclusion

For the first time, a strong dose-dependent treatment with ITB was documented as a cause for the above episodes and relapsing re-herniations.     

Johan Georg Ræder (1889–1959) and paratrigeminal sympathetic paresis

Introduction

Johan Georg Ræder (1889–1959) was the most eminent Norwegian ophthalmologist in the early decades of the last century. Ræder made significant contributions to our current understanding of glaucoma. He is remembered for a syndrome he described, that of trigeminal nerve neuralgia and/or paresis and incomplete Horner’s syndrome (oculopupillary sympathetic paresis).

Discussion

Here, Ræder's biography, scientific contributions, and a thorough review of his original report on paratrigeminal sympathetic paresis are presented. Ræder's syndrome may reflect a lesion of the middle cranial fossa, which involves oculopupillary sympathetic fibers that originate from the internal carotid artery plexus and travel with the trigeminal and oculomotor nerves.     

Oberlin transfer and partial radial to axillary nerve neurotization to repair an explosive traumatic injury to the Brachial Plexus in a child: Case report

Purpose

Explosive injuries to the pediatric brachial plexus are exceedingly rare and as such are poorly characterized in the medical literature.

Methods

Herein, we describe an 8-year-old who was struck in the neck by a piece of shrapnel and suffered multiple vascular injuries in addition to a suspected avulsion of the cervical 5 and 6 ventral rami. The patient had a complete upper brachial plexus palsy and failed to demonstrate any clinical improvement at 6-months follow-up. He was taken to the operating from for a partial ulnar to musculocutaneous nerve neurotization as well as a partial radial to axillary nerve neurotization.

Results

The patient’s motor exam improved from a Medical Research Council scale 1 to 4+ for biceps brachii and 0 to 4 deltoid function with greater than 90° of shoulder abduction.

Conclusions

This outcome supports complex neurotization techniques as viable treatment options for persistent motor deficits following an upper brachial plexus injury in older, non-infant age, children.     

Histone deacetylases expression in atypical teratoid rhabdoid tumors

Purpose

Atypical teratoid rhabdoid tumors (ATRTs) are rare, highly malignant central nervous system tumors that occur during infancy and early childhood. Their poor outcome and resistance to conventional chemotherapies and radiotherapy, urges the development of new therapies. Recent studies have evaluated the effects of histone deacetylase inhibitors (HDACi) as a new potential treatment for ATRTs. However, most HDACi act unselectively against all, or at least several, histone deacetylase (HDAC) family members. We hypothesized that specific HDAC family members are deregulated in ATRT and therefore a more selective class of HDACi would be beneficial to patients with ATRT.

Methods

To test our hypothesis, we evaluated the expression level of different HDAC family members in ATRTs. Eight ATRTs were compared to six medulloblastoma samples in regards to the level of expression of the 18 HDAC family members as determined by microarray gene expression profiling.

Results

HDAC1 was the only member of the HDAC family to be significantly differentially expressed in ATRTs (FC?=?4.728; p value?=?0.00003).

Conclusions

A class of HDACi specifically targeting HDAC1 may allow for the desired therapeutic benefits with fewer side effects for children with ATRT.     

The role of neuroimaging in the evaluation process of children with isolated sixth nerve palsy

Purpose

The purpose of this study was to assess the role of neuroimaging in identifying the etiology of pediatric isolated sixth nerve palsy (SNP).

Methods

A retrospective cohort study was conducted of all patients younger than 18 years of age with an isolated SNP seen at our medical center between 2003 and 2011.

Results

Sixteen children (nine girls; mean age, 4.5 years) with isolated SNP were identified during an 8-year period. Only cases with normal optic disk appearance and otherwise normal neurological examination were included into this study. Thus, 12 other children with SNP were excluded: ten children with papilledema, one child who developed a SNP following a resection of a brain tumor, and one with hydrocephalus and a shunt malfunction. All cases of isolated SNP were unilateral (ten left eyes). The most common cause for the SNP in these children was a tumor that was found in five patients. Other etiologies encountered in decreasing frequencies were: benign recurrent SNP (in four children), postviral or vaccination (in three children), and one case each of post trauma, Chiari malformation, congenital, and undetermined. Children who were found to have a tumor (9.9?±?5.5 years) were significantly older (P?=?0.019) than children who did not have a tumor (2.1?±?1.8 years).

Conclusions

Isolated SNP can be the presentation of a brain tumor in children, and therefore, early neuroimaging of the brain is recommended, especially in older children.