A Rare Case of Sagittal Sinus Obstruction Following Posterior Cranial Vault Distraction Osteogenesis.

Posterior cranial vault distraction osteogenesis (PCVDO) is a relatively new paradigm in the treatment of syndromic craniosynostosis, having first been introduced in 2009. PCVDO directly addresses the underdeveloped cranial vault and appears to allow for a larger increase in intracranial volume when compared to traditional techniques. Although reported as safe in the literature, critical appraisal is still required as PCVDO is a relatively uncommon procedure that may require greater numbers to detect true complication rates. The overall reported incidence of serious complications in PCVDO to date is low. This presentation highlights a rare case of sagittal sinus obstruction following posterior cranial vault distraction and raises questions as to the safest technical considerations when planning the operation.

Management of Unicoronal and Metopic Synostoses: Minimally Invasive Approaches.

Early endoscopic-assisted correction of unicoronal and metopic synostosis is an excellent, safe, cost-effective, and highly effective option for affected patients. Although open calvarial remodeling has a place in the armamentarium of the craniofacial team, the skull base changes seen in endoscopic-assisted techniques are unparalleled. The procedures are associated with low morbidity and no mortality. There is minimal blood loss, decreased operating time, significantly reduced blood transfusion rates, decreased hospitalization length, decreased cost, and less pain and swelling. Early diagnosis and referral for surgical evaluation are critical to obtaining these results.

Lambdoid synostosis: endoscopic craniectomy.

Lambdoid craniosynostosis leads to significant deformational changes of the calvaria and cranial fossae. Surgery used to treat the condition typically consists of a calvarial vault remodeling (CVR) procedure whereby the entire occiput is removed and reshaped along with a bandeau advancement to give the patient a rounded occiput. As an option, this video presents the minimally invasive endoscopic craniectomy used at the author's institution, which was developed there and has been successfully used for 25 years. This procedure is simple and can be done rapidly, with minimal to no blood loss. The video details the key steps necessary to successfully perform the procedure. The video can be found here: https://vimeo.com/515746378.

Endoscopy-assisted early correction of single-suture metopic craniosynostosis: a 19-year experience.

In BriefThe long-term results of treating infants with metopic craniosynostosis by using endoscopic, minimally invasive techniques are reported. The impetus arose from the lack of consistent and favorable outcomes associated with calvarial vault remodeling techniques and from the very traumatic and invasive nature of these procedures. The results presented show excellent and consistent long-term outcomes that are superior to traditional methods and are associated with minimal trauma, blood loss, and anesthetic exposure, and with short surgical times.

Does progesterone show neuroprotective effects on traumatic brain injury through increasing phosphorylation of Akt in the hippocampus?

There are currently no federally approved neuroprotective agents to treat traumatic brain injury. Progesterone, a hydrophobic steroid hormone, has been shown in recent studies to exhibit neuroprotective effects in controlled cortical impact rat models. Akt is a protein kinase known to play a role in cell signaling pathways that reduce edema, inflammation, apoptosis, and promote cell growth in the brain. This study aims to determine if progesterone modulates the phosphorylation of Akt via its threonine 308 phosphorylation site. Phosphorylation at the threonine 308 site is one of several sites responsible for activating Akt and enabling the protein kinase to carry out its neuroprotective effects. To assess the effects of progesterone on Akt phosphorylation, C57BL/6 mice were treated with progesterone (8 mg/kg) at 1 (intraperitonally), 6, 24, and 48 hours (subcutaneously) post closed-skull traumatic brain injury. The hippocampus was harvested at 72 hours post injury and prepared for western blot analysis. Traumatic brain injury caused a significant decrease in Akt phosphorylation compared to sham operation. However, mice treated with progesterone following traumatic brain injury had an increase in phosphorylation of Akt compared to traumatic brain injury vehicle. Our findings suggest that progesterone is a viable treatment option for activating neuroprotective pathways after traumatic brain injury.

Spatial and temporal expression levels of specific microRNAs in a spinal cord injury mouse model and their relationship to the duration of compression.

BACKGROUND CONTEXT: MicroRNAs, a class of small nonprotein-coding RNAs, are thought to control gene translation into proteins. The latter are the ultimate effectors of the biochemical cascade occurring in any physiological and pathological process. MicroRNAs have been shown to change their expression levels during injury of spinal cord in contusion rodent models. Compression is the most frequent mode of damage of neural elements in spinal cord injury. The cellular and molecular changes occurring in the spinal cord during prolonged compression are not very well elucidated. Understanding the underlying molecular events that occur during sustained compression is paramount in building new therapeutic strategies. PURPOSE: The purpose of our study was to probe the relationship between the expression level changes of different miRNAs and the timing of spinal cord decompression in a mouse model. STUDY DESIGN: A compression spinal cord injury mouse model was used for the study. METHODS: A laminectomy was performed in the thoracic spine of C57BL/6 mice. Then, the thecal sac was compressed to create the injury. Decompression was performed early for one group and it was delayed in the second group. The spinal cord at the epicenter of the injury and one level rostral to it were removed at 3, 6, and 24 hours after trauma, and RNA was extracted. Expression levels of six different microRNAs and the relationship to the duration of compression were analyzed. This work was supported in part by the University Research Council Grants Program at the University of Texas Health Science Center San Antonio (Grant 130267). There are no specific conflicts of interest to be disclosed for this work. RESULTS: Expression levels of microRNAs in the prolonged compression of spinal cord model were significantly different compared with the expression levels in the short duration of compression spinal cord injury model. Furthermore, microRNAs show a different expression pattern in different regions of the injured spinal cord. CONCLUSIONS: Our findings demonstrate that spinal cord compression causes alterations in the expression of different miRNAs in the acute phase of injury. Their expression is related to the duration of the compression of the spinal cord. These findings suggest that early decompression of the spinal cord may have an important modulating effect on the molecular cascade triggered during secondary injury through the changes in expression levels of specific microRNAs.

Spatial distribution of insulin-like growth factor binding protein-2 following hypoxic-ischemic injury.

BACKGROUND: Insulin-like growth factor binding protein-2 (IGFBP-2) regulates the bioavailability, transportation, and localization of insulin-like growth factor-I (IGF-I), an effective neuroprotectant in animal stroke models especially when administered intranasally. Therefore, determining IGFBP-2's endogenous distribution in the normal and ischemic brain is essential in maximizing the neuroprotective potential of the intranasal IGF-I treatment approach. However, current data on IGFBP-2 is limited to mRNA and in situ hybridization studies. The purpose of this study was to determine if there are any changes in IGFBP-2 protein levels and distribution in ischemic brain and also to determine if IGFBPs play a role in the transportation of intranasally administered IGF-I into the brain. RESULTS: Using an in vitro approach, we show that ischemia causes changes in the distribution of IGFBP-2 in primary cortical neurons and astrocytes. In addition, we show using the transient middle cerebral artery occlusion (MCAO) model in mice that there is a significant increase in IGFBP-2 levels in the stroke penumbra and core after 72 h. This correlated with an overall increase in IGF-I after stroke, with the highest levels of IGF-I in the stroke core after 72 h. Brain sections from stroke mice indicate that neurons and astrocytes located in the penumbra both have increased expression of IGFBP-2, however, IGFBP-2 was not detected in microglia. We used binding competition studies to show that intranasally administered exogenous IGF-I uptake into the brain is not receptor mediated and is likely facilitated by IGFBPs. CONCLUSIONS: The change in protein levels indicates that IGFBP-2 plays an IGF-I-dependent and -independent role in the brain's acute (neuroprotection) and chronic (tissue remodeling) response to hypoxic-ischemic injury. Competition studies indicate that IGFBPs may have a role in rapid transportation of exogenous IGF-I from the nasal tissue to the site of injury.

Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative cranial orthosis therapy: 16-year experience.

OBJECT: The objective of this study was to present the authors' 16-year experience treating coronal craniosynostosis in infants using endoscopy-assisted techniques and postoperative cranial orthoses. METHODS: A total of 128 synostosed coronal sutures in 115 patients were treated between 1996 and 2012 by endoscopically resecting a strip of bone containing the stenosed suture via a 2-3 cm incision made at the ipsilateral stephanion. Data were obtained from a prospective database. Following surgery, patients were fitted with custom cranial orthoses to help correct preoperative craniofacial deformities. All patients were followed closely with cranial anthropometric measurements and photographs. RESULTS: The estimated mean blood loss was 20 ml (range 5-120 ml) and the estimated mean strip size was 0.6 cm × 10.7 cm. The mean surgical duration was 55 minutes (range 22-150 minutes). One patient underwent an intraoperative blood transfusion and 1 had a postoperative blood transfusion, for a total transfusion rate of 1.7%. Ninety-seven percent of patients were discharged on the first postoperative day. There were no deaths. Vertical dystopia correction of more than 80% from baseline was obtained in almost two-thirds of patients, with 51% achieving 100% correction. Nasal and sagittal craniofacial deviation (vertex-nasion-gnathion) correction greater than 80% was achieved in 80% of patients, with 77% achieving 100% correction. Supraorbital rim advancement of the ipsilateral eye was obtained in 98% of cases, with correction of frontal plagiocephaly the last deformity to achieve correction. CONCLUSIONS: Early treatment of coronal synostosis with endoscopy-assisted craniectomy and postoperative molding helmets leads to significant correction of craniofacial abnormalities, including vertical dystopia, nasal deviation, sagittal misalignment, and ipsilateral proptosis. This treatment method is associated with minimal trauma, blood loss, and transfusion rates, and typically only requires 1 overnight stay. This surgical approach is safe, effective, and associated with excellent results.

Rapamycin treatment improves neuron viability in an in vitro model of stroke.

Ischemic stroke is the leading cause of serious, long-term adult disability and is associated with sensorimotor and cognitive impairments due to neuronal degeneration. Currently, recombinant tissue plasminogen activator (rTPA) is the only FDA-approved medical therapy for treatment of patients with acute ischemic stroke. However, rTPA can only be given within 3 hours of symptom onset, and only 2% of patients are eligible. Therefore, there is an urgent need for novel neuroprotective treatment options for ischemic stroke. An emerging treatment for a diverse range of neurological disorders associated with neurodegeneration is rapamycin, a key modulator of the mammalian target of rapamycin (mTOR) pathway. The mTOR pathway is the primary regulator of the cellular response to nutrient availability, changes in energy status and stress as seen following ischemia and reperfusion. However, rapamycin's effects on mTORC1 and mTORC2 are poorly understood in neurons. In the current study we show that rapamycin can prevent the activation of both mTORC1 and mTORC2 in cortical neurons and improve cell survival following oxygen glucose deprivation (OGD), an in vitro model of ischemic stroke. This work further supports the investigation of rapamycin as a novel neuroprotectant for ischemic stroke.

Calcifying pseudoneoplasm of the atlantoaxial joint in a child.

Calcifying pseudoneoplasm of the spine is a rare nonneoplastic lesion of unknown origin described in adolescents and adults. Its clinical manifestations include axial pain, myelopathy, or radiculopathy. Surgery is the preferred method of treatment. The authors report the occurrence of calcifying pseudoneoplasm at the C1-2 cervical segment in a 22-month-old child who became completely asymptomatic 2 months after open biopsy. A review of the literature is presented, emphasizing the uniqueness of the presented case in comparison with the previously published cases. The 22-month-old healthy girl presented with sudden onset of neck pain. Due to persistence of the symptoms 2 weeks after onset, imaging studies were performed that revealed an inhomogeneous calcified mass extending from the transverse ligament to the C1-2 interlaminar space and facet joint on the left side. Open biopsy of the mass at the C1-2 lamina was performed. The histological features were consistent with calcifying pseudoneoplasm. The child's neck pain progressively improved and she remained asymptomatic at the 1-year follow-up. The postoperative MRI at 8 months did not reveal any progression of the lesion. Contrary to reported cases, calcifying pseudoneoplasm of the spine may occur as early as 2 years of age and should be included in the differential diagnosis of calcified lesions in this age group. Complete resection is not a prerequisite to clinical improvement when there is no compromise of neural structures; conservative management is appropriate.

The history of autologous fat graft use for prevention of cerebrospinal fluid rhinorrhea after transsphenoidal approaches.

Presented herein is a review of the history of fat graft use in preventing iatrogenic cerebrospinal fluid (CSF) rhinorrhea after transsphenoidal surgery. Since the first transsphenoidal surgeries were described in the early 1900s, the techniques of sellar packing to prevent CSF leak have evolved. Kanavel, Halstead, and Cushing used bismuth- or iodine-soaked gauze. Under Dandy's influence, fascia lata was the first autologous material to be used for the repair and prevention of CSF rhinorrhea. The use of autologous fat graft for this purpose has only been reported in recent decades. Montgomery was the first to use abdominal fat to obliterate the middle ear cavity in 1964, and Collins reported the first transsphenoidal application of fat graft in 1973. Other reports by Kirchner, Tindall, and Wilson followed.

Endoscopic technique for sagittal synostosis.

PURPOSE: This study aimed to present a 16-year experience of treating sagittal synostosis with endoscopic-assisted techniques and postoperative cranial orthotic therapy. In 1996, we introduced the use of endoscopes for the management of sagittal synostosis in four young infants. During the subsequent years, we have treated a total of 256 patients with great success and long-term follow-up. Presented herein are the techniques and results of such clinical experience. METHODS: A total of 256 patients with sagittal synostosis have been treated between May 1996 and April 2012. There were 187 males and 69 females. Mean age at time of surgery was 3.9 months. A wide-vertex craniectomy with bilateral barrel stave osteotomies of the temporal and parietal bones using small scalp incisions and endoscopic viewing techniques was performed. Instruments have been developed to assist with the operation. All patients were placed in postoperative molding cranial orthosis. RESULTS: Mean estimated blood loss was 27 cc. Mean transfusion rate was 7 %. Mean surgical time was 57 min. Mean length of stay was 1.1 days. Using cephalic index (CI) as an anthropometric measurement to judge head shape, our results were classified as excellent (CI>80), good (CI 80-70), or poor (CI<70). A total of 87 % were classified as excellent, 9 % as good, and 4 % as poor. CONCLUSIONS: Endoscopic-assisted management of sagittal synostosis is a safe, efficacious, and excellent option for treating this condition with long-lasting, superb results. It is associated with minimal morbidity and complications and improved results over traditional procedures.

Endoscopic technique for coronal synostosis.

PURPOSE: This paper aims to present a 15-year experience treating coronal craniosynostosis with endoscopic-assisted techniques and postoperative cranial orthotic therapy. METHODS: A total of 100 patients with coronal craniosynostosis were treated between 1996 and 2010. There were 36 males and 64 females. A single 2-cm incision was made halfway between anterior fontanelle and the squamosal on the affected side. Using endoscopic-assisted visualization, a strip of bone was removed between the aforementioned points. Following surgery, all patients were placed in cranial orthoses to assist in the correction of the craniofacial deformity. RESULTS: Mean estimated blood loss was 20 cm(3); only one patient required a transfusion. Mean length of stay was 1 day. Mean surgery time was 54 min. There were no mortalities. There was significant correction of vertical dystopia (66 % from baseline) and midsagittal plane deviation (80 % from baseline). CONCLUSIONS: Endoscopic-assisted craniectomy for treatment of coronal craniosynostosis in very young infants followed by cranial molding is associated with excellent long-lasting results and minimal morbidity and no mortality.

Bilateral endoscopic craniectomies in the treatment of an infant with Apert syndrome.

Patients with Apert syndrome commonly present with ocular proptosis due to bilateral coronal craniosynostosis and midfacial hypoplasia. Severe proptosis can cause visual compromise and damage, which is most commonly treated with bilateral orbital frontal advancement. The authors present the case of a patient who was treated at 8 weeks of age with endoscope-assisted bilateral coronal craniectomies followed by treatment with a custom-made postoperative cranial orthosis. The patient underwent the procedure without any complications. Over the ensuing months, the patient's proptosis corrected, the forehead and orbital rims advanced without the need for an orbital frontal advancement and craniotomies. This approach may provide an alternative treatment modality for these patients.