Recent advances in nerve repair.

Peripheral nerve injuries are extremely devastating, and their management is exceedingly complex. Microsurgical repair is the mainstay of treatment and this includes direct nerve repair, nerve grafts, nerve tubes and nerve transfers. Today, nerve transfers are being widely performed due to an increased understanding of cortical plasticity, motor re-education and perioperative rehabilitation, and they are now known to produce excellent functional outcomes. This manuscript reviews the current strategies for nerve repair, including comments on outcomes, with special emphasis on existing nerve transfer techniques.

Occipitocervical Fetus in Fetu with Extracalvarial Extension: A Case Report.

Fetus in fetu (FIF) is an extremely rare congenital anomaly in the pediatric age group. It is defined by the presence of an axial skeleton and well-formed internal organs in a fetiform mass. Only about 15 cases of intracranial FIF have been reported in the literature worldwide. We report a case of temporo-occipitocervical FIF. This 7-month-old female child presented to us with occipital swelling that she had had since birth. A soft swelling was palpable in the retroauricular region with extension into the neck. A few bony parts were palpable in the swelling. Imaging was suggestive of a multiseptate mass lesion in the right occipital region with intracranial, intraspinal and extracranial components. The patient underwent exploration and excision of the extracranial mass and retromastoid suboccipital craniectomy and decompression of the intracranial component. Different parts, such as a terminal ileum, cecum, appendix, femur, humerus and vertebral body, were identified within the mass. This case meets Willis' criteria as well as 4/5 criteria stated by Spencer for the diagnosis of this condition. To the best of our knowledge, this is the first reported case of temporo-occipitocervical FIF with a well-defined embryological spectrum. This also supports Spencer's hypothesis of abnormal twinning.

Primary pyogenic spinal epidural abscess: How late is too late and how bad is too bad? - A study on surgical outcome after delayed presentation.

Spinal epidural abscess is a rare clinical entity with considerable morbidity. Even with prompt diagnosis and treatment, many patients are left with persistent residual neurological deficits. The present study details the outcome in 23 patients of primary pyogenic spinal epidural abscess, addressing the outcome following late presentation at a neurological facility. At presentation only 2 patients had relatively preserved neurological status. Eleven patients were paraplegic. All the patients underwent laminectomy and evacuation of abscess. A good functional outcome was observed in almost half of the patients, and there was a significant reduction in the number of the patients with severe disability. Factors influencing the outcome are described in this study.

Bilateral carpal tunnel syndrome in two children: common manifestation of an uncommon disease.

Carpal tunnel syndrome (CTS) is a rare entity in children and usually has a different clinical presentation than in adults. The majority of these cases are related to a genetic condition. The most common aetiology is a lysosomal storage disease such as mucopolysaccharidoses or mucolipidoses (ML). Two siblings with bilateral median nerve neuropathy, wrist contractures and skeletal deformity on a background of mild facial coarsening and normal cognition are presented. There was a family history of similar complaints in first cousins. Nerve conduction studies were suggestive of bilateral median neuropathy with conduction block at the wrists. Biochemical investigation showed high levels of plasma lysosomal enzymes. Both patients underwent bilateral flexor retinaculum release. The findings at surgery were severe compression of the median nerve by the thickened flexor retinaculum. The histopathology of the thickened tissue showed a fibrosed and vascularised flexor retinaculum. On electron microscopy, macrophages with membrano-vacuolated inclusion zebra bodies were seen. Both patients had an uneventful recovery with symptomatic improvement. CTS is rarely seen in children except in association with genetic disorders. There is usually a delay in the diagnosis as attention is paid to the contractures rather than to the median nerve compression. The presence of CTS is typically seen in ML types II and III. Surgical intervention helps in improving the quality of life and hand function of these patients.

Efficacy and Safety Profile of Tranexamic Acid in Traumatic Thoracolumbar Fracture Management: A Systematic Review and Meta-Analysis.

OBJECTIVE: In this article, the authors systematically evaluated the efficacy and safety of tranexamic acid (TXA) in surgeries for spinal trauma. METHODS: Potentially relevant academic articles were identified from the Cochrane Library, MEDLINE, PubMed, and Google Scholar. Secondary sources were identified from the references of the included literature. RevMan software was used to analyze the pooled data. RESULTS: A total of 7 randomized controlled trials (RCTs) and 2 non-RCTs were included in the review. There were significant differences in total blood loss (standard mean difference [SMD] = -2.54 [95% CI, -3.72, -1.37], P = 0.0001), intraoperative blood loss (SMD = -0.96 [95% CI, -1.28, -0.64], P < 0.00001), postoperative blood loss (SMD = -1.42 [95% CI, -1.72, -1.11], P < 0.00001), and length of hospital stay (SMD = -3.73 [95% CI, -4.41, -3.06], P = 0.00001). No significant differences were found regarding transfusion requirement, operative duration, deep vein thrombosis, and pulmonary embolism between the 2 groups. CONCLUSIONS: The present meta-analysis indicates that the use of TXA in spinal surgery decreases blood loss and duration of hospital stay while not increasing the risk of side effects such as deep vein thrombosis and pulmonary embolism. CLINICAL RELEVANCE: The study aims to provide clinicians who operate on spine trauma with information on the use of tranexamic acid to decrease blood loss and related complications.

Towards Molecular Classification of Meningioma: Evolving Treatment and Diagnostic Paradigms.

Meningioma, a common primary brain tumor in adults, is graded based on World Health Organization criteria that rely on histology alone. This approach is unable to determine conclusively which tumors, especially benign or atypical, will recur. Molecular characterization of meningioma has identified genetic biomarkers that can predict tumor behavior. Only a few genetic changes are known to classify >85% of all meningioma and clinical trials using targeted therapy to genetic subtypes of meningioma are under way. Immunotherapy is also being trialed in treating high-grade and recurrent meningioma. This review summarizes recent developments characterizing meningioma using genetic and immunologic biomarkers and how these molecular tools may be integrated into existing care together with current World Health Organization grading to improve diagnosis, prognosis, and therapy.

Bobble-head doll syndrome: report of 2 cases and a review of the literature, with video documentation of the clinical phenomenon.

Bobble-head doll syndrome (BHDS) is a rare pediatric movement disorder presenting with involuntary 2- to 3-Hz head movements. Common signs and symptoms also found on presentation include macrocephaly, ataxia, developmental delay, optic disc pallor or atrophy, hyperreflexia, tremor, obesity, endocrinopathy, visual disturbance or impairment, headache, and vomiting, among others. The syndrome is associated with suprasellar cysts, third ventricular cysts, or aqueductal obstruction, along with a few other less common conditions. The cause of involuntary head motions is not understood. Treatment is surgical. The authors present 2 cases of BHDS. The first is a 14-year-old boy with BHDS associated with aqueductal obstruction and triventricular hydrocephalus secondary to a tectal tumor. He was successfully treated by endoscopic third ventriculostomy, and all symptoms resolved immediately in the recovery room. This case is unusual in its late age of symptom onset, the primacy of lateral ("no-no") involuntary head rotations, and the associated tectal tumor. The second case is a 7.5-year-old girl with BHDS associated with a suprasellar cyst. She was successfully treated with an endoscopic fenestration but preexisting endocrinopathy persisted, and the patient was diagnosed with autism spectrum disorder at age 12 years. This second case is more typical of BHDS. A comprehensive and up-to-date review of the literature of BHDS and video documentation of the phenomenon are presented.

Testing the effectiveness and the contribution of experimental supercharge (reversed) end-to-side nerve transfer.

OBJECTIVE: Supercharge end-to-side (SETS) transfer, also referred to as reverse end-to-side transfer, distal to severe nerve compression neuropathy or in-continuity nerve injury is gaining clinical popularity despite questions about its effectiveness. Here, the authors examined SETS distal to experimental neuroma in-continuity (NIC) injuries for efficacy in enhancing neuronal regeneration and functional outcome, and, for the first time, they definitively evaluated the degree of contribution of the native and donor motor neuron pools. METHODS: This study was conducted in 2 phases. In phase I, rats (n = 35) were assigned to one of 5 groups for unilateral sciatic nerve surgeries: group 1, tibial NIC with distal peroneal-tibial SETS; group 2, tibial NIC without SETS; group 3, intact tibial and severed peroneal nerves; group 4, tibial transection with SETS; and group 5, severed tibial and peroneal nerves. Recovery was evaluated biweekly using electrophysiology and locomotion tasks. At the phase I end point, after retrograde labeling, the spinal cords were analyzed to assess the degree of neuronal regeneration. In phase II, 20 new animals underwent primary retrograde labeling of the tibial nerve, following which they were assigned to one of the following 3 groups: group 1, group 2, and group 4. Then, secondary retrograde labeling from the tibial nerve was performed at the study end point to quantify the native versus donor regenerated neuronal pool. RESULTS: In phase I studies, a significantly increased neuronal regeneration in group 1 (SETS) compared with all other groups was observed, but with modest (nonsignificant) improvement in electrophysiological and behavioral outcomes. In phase II experiments, the authors discovered that secondary labeling in group 1 was predominantly contributed from the donor (peroneal) pool. Double-labeling counts were dramatically higher in group 2 than in group 1, suggestive of hampered regeneration from the native tibial motor neuron pool across the NIC segment in the presence of SETS. CONCLUSIONS: SETS is indeed an effective strategy to enhance axonal regeneration, which is mainly contributed by the donor neuronal pool. Moreover, the presence of a distal SETS coaptation appears to negatively influence neuronal regeneration across the NIC segment. The clinical significance is that SETS should only employ synergistic donors, as the use of antagonistic donors can downgrade recovery.

Calvarial defects in the region of the lambdoid suture in neurofibromatosis type-1 patients.

Rare type of calvarial defects seen in patients with neurofibromatosis type-1 (NF1) is presented. The issues of pathogenesis and management are discussed. Two cases of NF1 with skull defects in the region of the lambdoid suture are reported. The possible etiological basis and nature of these type of defects and management issues are discussed. The calvarial skull defects in the lambdoid suture region are rare defects in NF1 patients. The possible reason of the progressive nature of these type of lesions can be the cerebrospinal fluid pulsations behaving like "growing skull fractures," especially when not associated with structural lesions. It leads to progressive enlargement of the small congenital defects in the region of the lambdoid suture and abnormal susceptibility of bones for resorption. For these defects, conservative management is suggested due to its progressive nature and high chances of operative treatment failure.