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Biomedical subjects

R Shane Tubbs

Publications and source records attributed to R Shane Tubbs.

At least 127 records · Page 7Linked to original sources

Russell Silver syndrome and tethered spinal cord.

CASE REPORT: We report a 20-year-old male with Russell Silver syndrome and tethered cord syndrome. This patient presented with non-painful scoliosis. MRI demonstrated a conus medullaris at the superior aspect of the L3 vertebral body. Axial images demonstrated no fat within the filum terminale. There was a horizontal sacrum. The patient had no cutaneous stigmata over the lumbosacral spine indicative of occult spinal dysraphism. At operation, the filum terminale was found to be quite large at approximately 5.0 mm in diameter. Grossly, the filum was found to have several large vessels both anterior and posterior to it. Following transection of the filum the distal cord was noted to withdraw cephalad indicating caudal traction. DISCUSSION: We believe this to be the first report of Russell Silver syndrome in conjunction with tethered cord syndrome. Following additional reports, Russell Silver syndrome may be added to the differential diagnoses for tethered cord syndrome.

Adult↗

Complications of ventriculosubgaleal shunts in infants and children.

INTRODUCTION: The ventriculosubgaleal shunt has been used for the temporary bypass of the normal cerebrospinal fluid (CSF) pathways. To date, a large series of complications from this procedure has not been elaborated upon in the literature. PATIENTS AND METHODS: We retrospectively reviewed all such shunts (170) placed at our institution over the last 6 years and documented all complications from this procedure. The majority of patients operated upon were premature infants with intraventricular hemorrhage and subsequent hydrocephalus. This technique was used in a much smaller group of patients in whom the peritoneal cavities were not currently candidates for distal shunt implantation but would have been with time. Other patients in whom this technique was used were those with malignant brain tumors, intraventricular abscesses, chronic truncal wounds, chronic subdural hygromas, and meningitis. RESULTS: Complications from subgaleal shunting included infection (5.9%), intracranial hemorrhage (1.1%), and wound leakage (4.7%). CONCLUSIONS: We believe the benefits afforded by ventriculosubgaleal shunting significantly outweigh the risks of the procedure and greatly ease the burden of care for this select population of children. Based on the literature and our own experience, the complications from this procedure are not excessive or extraordinarily unique compared with other neurosurgical CSF diversion techniques.

Child, Preschool↗

Isolated atlantal stenosis in a patient with idiopathic growth hormone deficiency, and Klippel-Feil and Duane's syndromes.

OBJECTIVE AND IMPORTANCE: Isolated atlantal stenosis is very rare with less than ten cases reported, mostly in adult Asians; however, this pathology should be recognized by the neurosurgeon. An unusual case of this rare clinical entity is reported in conjunction with multiple other anomalies. CLINICAL PRESENTATION: We report a Caucasian girl with symptomatic stenosis of the first cervical vertebrae who presented with episodes of loss of tone with subsequent falling, facial cyanosis, urinary incontinence, hand weakness, and difficulties with swallowing. This patient also had the diagnoses of Duane's and Klippel-Feil syndromes, and idiopathic growth hormone deficiency. In addition, this patient was found to have retroflexion of the odontoid process, which further compromised the spinal canal. Flexion-extension radiographs failed to identify cervical spine instability. INTERVENTION: Following suboccipital craniectomy and the removal of the posterior arch of the atlas, the patient's symptoms were resolved and her urinary incontinence improved. CONCLUSIONS: We theorize that our case represents a variant of Wildervanck syndrome. Further case reports are necessary to verify that isolated atlantal stenosis is a component of this constellation of findings. Furthermore, our case demonstrates that degenerative changes of the spine are not necessary for the production of symptoms in cases of isolated stenosis of the atlas.

Child↗

Treatment and management of the Chiari II malformation: an evidence-based review of the literature.

OBJECTIVE: Multiple surgical strategies exist for the management of the symptomatic Chiari II malformation. To date, no comprehensive analysis of this medical literature in an attempt to seek out standards or guidelines has been performed, thus serving as the impetus for this present review. METHODS: A computerized search of the database of the National Library of Medicine was performed on the English-language medical literature between 1966 and 2003. Terms searched for included hindbrain hernia, Chiari II, Arnold-Chiari, surgery, decompression, syringomyelia, and CSF shunts. All literature found was reviewed with associated references from these sources. Each piece of literature reviewed was scrutinized and guidelines developed utilizing the methodology used by the Guidelines for the Management of Acute Cervical Spine and Spinal Cord Injuries. RESULTS: All data reviewed were deemed to be Class III in nature. No standards or guidelines could be established from the available literature. There is significant debate and variable results in the current neurosurgical literature regarding the evaluation of shunt function vs. Chiari II decompression in patients with symptomatic hindbrain herniation. CONCLUSIONS: Multi-institutional prospective randomized trials are needed before any conclusions can be drawn regarding symptomatic Chiari II and its treatment paradigm. Until this time, clinical practice will be based on individual surgeons' experience, training, and personal beliefs.

Arnold-Chiari Malformation↗

Left-sided vagus nerve stimulation decreases intracranial pressure without resultant bradycardia in the pig: a potential therapeutic modality for humans.

INTRODUCTION: The medical literature is lacking in data regarding intracranial pressure in an animal model with concomitant vagus nerve and spinal cord stimulation. METHODS: Ten pigs were anesthetized and placed in the supine position and an intracranial pressure monitor was inserted. Each study animal had a small laminectomy performed in the lumbar region and the left vagus nerve exposed within the carotid sheath. Intracranial pressure was monitored for 3 h in all animals. Eight animals at the end of 3 h of monitoring underwent vagus nerve stimulation and or spinal cord stimulation. Study and control animals had their intracranial pressure monitored for an additional 1 and 2 h respectively. RESULTS: Following vagus nerve stimulation, all animals had significant (p<0.05) decreases in their intracranial pressure with a lasting effect of 15-35 min (mean 18.5 min). No significant change in blood pressure was noted during stimulation of the vagus nerve. After spinal cord stimulation all animals responded with acute increases in their intracranial pressure (p<0.05). CONCLUSIONS: Left vagus nerve stimulation reliably decreases intracranial pressure in the pig. The mechanism of this action remains unclear and does not appear to be due to resultant bradycardia. Conversely, stimulation of the upper lumbar spinal cord increases intracranial pressure with simultaneous increases in heart rate. Following additional studies and with close observation of cerebral perfusion pressure, we believe that left vagus nerve stimulation may represent a novel adjunctive therapy for decreasing elevated intracranial pressure in posttraumatic human patients with head injuries. Additionally, according to this animal data, minimizing spinal cord stimulation should be considered in the acute setting following head injury so as to minimize iatrogenic elevation of intracranial pressure.

Animals↗

Degree of tectal beaking correlates to the presence of nystagmus in children with Chiari II malformation.

INTRODUCTION: Tectal beaking is a common finding in children born with a myelomeningocele. Quantitation of the presence or absence of this finding correlated with normal or abnormal eye movements is lacking in the medical literature. MATERIALS AND METHODS: We prospectively evaluated 50 children in our multidisciplinary spina bifida clinic. Each child was observed for the presence or absence of nystagmus at rest or with extraocular movement. Grades of nystagmus and severity of tectal beaking (Types I-III) on MRI were then correlated. RESULTS: Overwhelmingly, children with greater dysmorphology of the mesencephalic tectum were most likely to have nystagmus. Higher grades of nystagmus were associated with Type III tectal beaking. CONCLUSIONS: We have demonstrated that there is a correlation between the presence and severity of nystagmus and the severity of tectal beaking and have quantified this association in patients with the Chiari II malformation. This information may act as a guide in predicting which children with the Chiari II malformation will have significant nystagmus. These data may aid the clinician in the interpretation of oculomotor abnormalities in this cohort of patients.

Adolescent↗

Urinary incontinence in a patient with Duchenne muscular dystrophy and cord in the normal position with fatty filum terminale.

INTRODUCTION: To our knowledge, muscular dystrophy and specifically, Duchenne muscular dystrophy, has not previously been associated with gross intrathecal abnormalities. CASE REPORT: We report a pediatric patient with Duchenne muscular dystrophy that was diagnosed a long time ago and recent onset urinary incontinence. MRI revealed a conus medullaris in a normal position with a fatty-infiltrated filum terminale. Operative intervention was performed entailing sectioning of the filum terminale. This resulted in resolution of the child's urinary incontinence. CONCLUSION: Although seemingly rare and perhaps associated by serendipity, new, unusual neurological deficits in a patient with muscular dystrophy, especially of an upper motor neuron nature, should warrant MRI to rule out pathology of the spinal cord such as tethering.

Adolescent↗

Clostridium infection resulting in paralysis in a child.

CASE REPORT: We report an 11-year-old boy who fell from an All Terrain Vehicle and sustained multiple minor soft tissue contusions and a small midthoracic laceration. Irrigation and closure of the small wound was performed at another hospital. There was no history of a penetrating wound. Within 48 h of injury, the patient developed profound dysesthesia and paralysis of the lower extremities and was transferred to our hospital. MRI disclosed a paraspinal abnormality without bony involvement. At exploration a portion of a tree branch was removed. Wound cultures were positive for Clostridium botulinum, tetani, and perfringens. CONCLUSIONS: To our knowledge, this is the first case of direct Clostridium intoxication of the spinal cord in man. Moreover, this report demonstrates the invasive manner in which Clostridium toxins may breach both the intact ligamentum flavum and the dura mater to deliver their toxicity to the intradural contents. Although the patient's dysesthesia resolved and paraplegia improved to ambulation he is still left with a significant motor deficit.

Child↗

Horner's syndrome resulting from agenesis of the internal carotid artery: report of a third case.

INTRODUCTION: Only two previous reports of agenesis of the internal carotid artery with a coexistent Horner's syndrome are to be found in the medical literature. CASE REPORT: We report a case of a male child seen initially for what was presumed to be a traumatic superficial temporal artery aneurysm. MRA incidentally revealed ipsilateral agenesis of the internal carotid artery. This child's physical exam included ipsilateral Horner's syndrome. CONCLUSION: This case report illustrates that agenesis of the internal carotid artery does indeed cause Horner's syndrome in some cases. The clinician who diagnoses a patient with Horner's syndrome should remember that agenesis of the internal carotid artery may be the cause as this entity can be associated in itself with other pathologies of the central nervous system.

Aneurysm↗

Eyebrow incision with supraorbital trephination for endoscopic corpus callosotomy: a feasibility study.

OBJECTIVE: We describe a novel technique for the partial bisection of the corpus callosum in order to increase the minimally invasiveness of this procedure. METHODS: Brow incisions with midline trephinations were performed in six adult cadavers. An endoscope was next introduced and used to transect approximately the anterior two-thirds of the corpus callosum. RESULTS: No complications such as injury to the superior sagittal sinus or anterior cerebral artery were encountered in any of our cadaveric specimens. The corpus callosum was easily transected in each specimen. CONCLUSION: As a feasibility study, we believe this technique could provide a less invasive mechanism for patients who require corpus callosotomy and will minimize much of the morbidity associated with the traditional methods of sectioning the corpus callosum.

Aged↗

Split cord malformation and situs inversus totalis: case report and review of the literature.

INTRODUCTION: Situs inversus is a rare condition of visceral transposition in which the spinal axis is rarely affected. CASE REPORT: The authors report a patient with situs inversus totalis and Type II split cord malformation. The patient had no symptoms and presented with scoliosis. CONCLUSIONS: Recent compelling evidence from animal models and human case reports has lead to hypotheses that defects of the midline and laterality defects (e.g., situs inversus) are etiologically related. Confirmation from additional case reports of situs inversus and split cord malformation could prove useful in determining a genetic locus for split cord malformations or implicating various chemical agents that are known to produce situs inversus as potential causative factors in the production of split cord malformations.

Child↗

Fenestration of the superior medullary velum as treatment for a trapped fourth ventricle: a feasibility study.

We developed a novel approach for fenestration of the trapped fourth ventricle utilizing the superior medullary velum (valve of Vieussens). Trapped fourth ventricles, which are seen often in the pediatric hydrocephalic population, are troublesome entities surgically. A right burr hole was carried out in 10 adult cadavers with no gross intracranial pathology and the superior medullary velum was fenestrated to the quadrigeminal cistern with the aid of an endoscope. This technique was carried out easily in all cadaveric specimens. With endoscopy, no vascular insult was appreciated either before or after fenestration of the superior medullary velum. These preliminary findings demonstrate that fenestration of the superior medullary velum may provide a good alternative to the present therapy of shunting trapped fourth ventricles, a therapy wrought with complications.

Aged↗

Femoral head of the rectus femoris muscle.

Variability of the muscles of the anterior thigh is minimal. After review of the extant medical literature, we believe this to be the first reported case of a femoral head of the left and right rectus femoris muscles. In addition to this anomaly, our anatomic specimen demonstrated an accessory head of the right brachialis muscle and the quadratus plantae muscles inserted into the tendons of both the flexor hallucis longus and flexor digitorum longus. In clinical practice, knowledge of the possibility of an anomalous accessory femoral head of the rectus femoris muscle in clinical practice would be useful for those who might palpate this unusual muscle or encounter it surgically.

Aged↗

Unusual origin of the omohyoid muscle.

An unusual origin of the right omohyoid muscle was found during cadaveric dissection. The muscle originated from the transverse process of C6 and inserted into the scapula. No other muscular anomalies of the neck were found. Although many anomalies of the omohyoid muscle have been described, a proximal attachment to the cervical spine is apparently quite rare. Knowledge of the many anomalies that can potentially occur in the cervical region is necessary in routine surgical intervention of this area. We believe this to be the first reported instance of the superior belly of the "omo" hyoid originating from the cervical region as we have described.

Cadaver↗

Duane's syndrome and giant parietal foramina.

We report a 14-month-old male with Duane's syndrome and bilateral parietal calvarial defects. This child had no other known medical conditions. The Duane's syndrome involved the left eye. Imaging was demonstrative of bilateral enlarged parietal foramina. We believe this to be the first reported case of simultaneous Duane's syndrome and bilateral giant parietal foramina. Further case reports of our current association are required to determine if these simultaneous findings are spurious or a low-frequency association.

Duane Retraction Syndrome↗

Continuation of the ascending cervical artery as the occipital artery in man.

We report an elderly male cadaver with unilateral anomalous continuation of the ascending cervical artery as the occipital artery. The ascending cervical artery was enlarged from its origin and supplied segmental branches through the intervertebral foramina. At the upper cervical spine, the ascending cervical artery continued as the occipital artery providing the branches normally seen stemming from this artery. No other vascular anomalies were noted in this specimen. This anatomical curiosity should be kept in mind by the clinician or academic who may manipulate this anatomical area.

Cadaver↗

Retroesophageal right subclavian artery with persistent ductus arteriosus.

We report an adult male cadaver with persistence of the ductus arteriosus and a right retroesophageal artery. The retroesophageal artery was first appreciated as a retropharyngeal mass during the dissection of this area. Both the left and right vertebral arteries originated in normal fashion. The thoracic duct of our specimen drained into the junction of the left internal jugular and left subclavian veins. We believe this to be the first report of simultaneous right retroesophageal subclavian artery and patent ductus arteriosus. The anatomy and embryology of these two anomalies in the same specimen are discussed.

Ductus Arteriosus, Patent↗