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José Gonzalez Tortosa, Juan F. Martínez-Lage, and Máximo Poza

Object. Most craniocerebral injuries are caused by mechanisms of acceleration and/or deceleration. Traumatic injuries following progressive compression to the head are certainly unusual. The authors reviewed clinical and radiological features in a series of patients who had sustained a special type of cranial crush injury produced by the bilateral application of rather static forces to the temporal region. Their aim was to define the characteristic clinical features in this group of patients and to assess the mechanisms involved in the production of the cranial injuries and those of the associated cerebral and endocrine lesions found in this peculiar type of head injury.

Methods. Clinical records of 11 patients were analyzed with regard to the state of consciousness, cranial nerve involvement, findings on neuroimaging studies, endocrine symptoms, and outcome. Furthermore, an experimental model of bitemporal crush injury was developed by compressing a dried skull with a carpenter's vice.

Seven of the 11 patients were 16 years old or younger. All patients presented with a characteristic clinical picture consisting of no loss of consciousness (six patients), epistaxis (nine patients), otorrhagia (11 patients), peripheral paralysis of the sixth and/or seventh cranial nerves (10 patients), hearing loss (five patients), skull base fractures (11 patients), pneumocephalus (11 patients), and diabetes insipidus (seven patients). Ten patients survived the injury and most recovered neurological function.

Conclusions. Static forces applied to the head in a transverse axis produce fractures in the skull base that cross the midline structures without producing significant cerebral damage. Stretching of cranial nerves at the skull base explains the nearly universal finding of paralysis of these structures, whereas an increase in the vertical diameter of the skull accounts for the occurrence of diabetes insipidus in the presence of an intact function of the anterior pituitary lobe. The association of clinical, endocrine, and neuroimaging findings encountered in this peculiar type of head injury supports the idea that this subset of injured patients has a distinctive clinical condition, namely the syndrome of bitemporal crush injury to the head.

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Juan F. Martínez-Lage

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Juan F. Martinez-Lage, José Masegosa, Joaquin Sola, and Máximo Poza

✓ The authors report the case of an infant who presented with an epidermoid tumor contained in a lumbosacral myelomeningocele. The association of spina bifida aperta and congenital intraspinal tumors is rare. Only two cases of teratoma and two cases of epidermoid tumors arising within a meningocele have been documented previously.

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Juan F. Martínez-Lage, Francisco López, Claudio Piqueras, and Máximo Poza

✓ The authors present the case of a 6-year-old girl who developed a right frontal intradiploic meningoencephalocele following an accidental dural tear produced during surgery for craniosynostosis. Although rare, growing skull fractures have been described in at least eight cases following the accidental laceration of the dura mater in the course of craniosynostosis repair. These cases closely resemble those produced by accidental trauma to the growing skull. However, the intradiploic location of a meningoencephalocele following surgery for craniosynostosis has not been documented previously. This patient presented with headache and a frontal tumor of bonelike consistency. Radiographs and computerized tomography scans of the skull revealed an intraosseous cyst, whereas magnetic resonance imaging demonstrated cerebral tissue herniation within the intradiploic tumor. Surgical treatment consisted of duraplasty and cranioplasty, which achieved good functional and cosmetic results. The pathogenesis of this unusual lesion is discussed and compared with the hypotheses advanced for explaining posttraumatic intradiploic cysts.

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Juan F. Martínez-Lage, Antonio Ruiz-Espejo, Encarna Guillén-Navarro, and María-José Almagro

The authors report the case of a patient with Chiari malformation Type I (CM-I) and syringomyelia probably caused by a retrocerebellar arachnoid cyst. The patient's phenotype corresponded to trichorhinophalangeal syndrome Type I. The authors attributed the origin of both the retrocerebellar cyst and the abnormal posterior fossa to endochondral ossification anomalies that occur in this syndrome. The patient's spinal pain was most likely a result of the combination of CM-I and syringomyelia. To the best of the authors' knowledge, this is the first report on the association of CM-I and syringomyelia with a retrocerebellar arachnoid cyst occurring in a patient with trichorhinophalangeal syndrome Type I. The authors discuss the pathogenetic mechanisms involved in the production of tonsillar descent and syringomyelia in this patient, and review the current literature on related conditions that can result in this association.

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Marcelo Galarza, Anthony P. Fabrizi, Raffaella Maina, Roberto Gazzeri, and Juan F. Martínez-Lage

Object

The aim of this study was to evaluate whether clinical improvement is noticeable after a minimally invasive procedure such as that used with the Aperius PercLID System in patients with degenerative lumbar spinal stenosis (DLSS) and neurogenic intermittent claudication (NIC).

Methods

The patients were treated with the aforementioned system at 3 different centers. The initial requirement to be included in the study was a minimum follow-up of 12 months. The authors studied 40 cases of DLSS in patients with NIC (age 72.7 ± 8.08 years). Symptom severity, physical function, quality of life, and self-rated pain were assessed preoperatively and at the 12-month follow-up using the Zurich Claudication Questionnaire (ZCQ) and a visual analog scale. The procedure was conducted under spinal (35 patients) or local (5 patients) anesthesia, using biplanar fluoroscopy for visualization.

Results

Single-level treatment was performed in 28 patients and 2-level treatment was performed in 12 patients. Based on time recordings in 24 cases, the mean procedural time was 19.9 ± 5.0 minutes. The mean pain visual analog scale score improved significantly from 8.1 ± 2.19 at baseline to 3.44 ± 2.89 at the 1-year follow-up. The ZCQ score for patient satisfaction showed 90% of the patients being satisfied with the procedure. The mean rates of improvement in ZCQ score for symptom severity and physical function at 1 year were 38.7 ± 33.3% and 33.8 ± 29.7%, respectively, and both proved to be statistically significant. Most improvement was seen in mobility, pain/discomfort, and ability for self-care.

Conclusions

In this preliminary study, the Aperius system provided clinically significant improvement after 1 year of follow-up in patients older than 65 years with DLSS and NIC.

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José González-Tortosa, Javier Ros de San Pedro, Guillermo Parrilla, Belen Ferri-Ñiguez, and Juan F. Martínez-Lage

The authors report the case of a 23-year-old woman with café-au-lait spots and axillary and inguinal freckling who presented with a diploic chronic spontaneous hematoma of the left parietal bone. To the authors' knowledge, this case represents the first description of a diploic hematoma in a patient with stigmata of neurofibromatosis Type 1 unrelated to head trauma. Plain skull radiography showed an osteolytic lesion with well-circumscribed margins, corresponding to the hematoma, together with exuberant perilesional vascular markings. Angiography demonstrated an incidental aneurysm of the left supraclinoidal internal carotid artery and an unusual cortical venous drainage toward the diploic vessels. The blood flow of these vessels on the right hemicranium was sluggish and exhibited enlarged diploic venous lacunas. The authors hypothesize that the hematoma was formed by both an abnormal venous drainage toward the diploic vascular net, together with a vasculopathy that caused stenosis and obstruction of the normal drainage pathways from these vessels.

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Juan F. Martinez-Lage, Joaquin Sola, Carlos Casas, M´ximo Poza, Maria J. Almagro, and Diego G. Girona

✓ Atretic cephalocele appears as an unimportant and benign lesion. This malformation consists of meningeal and vestigial tissues (arachnoid, glial, or central nervous system rests). The authors report the findings in 16 cases (seven parietal and nine occipital) of rudimentary cephaloceles. Twelve patients presented with associated brain abnormalities detected by either computerized tomography (CT) or magnetic resonance imaging (MR). Nine lesions also exhibited an anomalous vascular component demonstrated by CT or MR imaging or at surgery. The existence of this tiny malformation in five cases was the main diagnostic clue to a severe complex of cerebral anomalies, namely cerebro-oculomuscular (Walker-Warburg) syndrome. An occipital location of the atretic cephalocele was associated with the worst prognosis, with only two children developing normally. However, a parietal location carried a better prognosis, which is contrary to the outcome reported in the current literature.

The authors classify atretic cephaloceles into two types based on histological examination of the surgical specimens, and suggest that these types represent different stages in the development of this malformation. It is concluded that, in the evaluation of the atretic cephalocele, the neurosurgeon is obliged to proceed to a detailed neuroradiological study of the patient and that the prognosis does not depend on the existence of the cephalocele itself, but rather on associated “occult” brain anomalies.

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Claudio Piqueras, Juan F. Martínez-Lage, María José Almagro, Javier Ros De San Pedro, Pedro Torres Tortosa, and Agueda Herrera

✓ The authors report the case of a 10-year-old boy who sustained an injury to the cauda equina as a result of the accidental penetration of a wooden pencil into the spinal canal. After neuroimaging evaluation to exclude visceral and vascular lesions, the foreign body was removed and the wound was repaired. This is the first report of a cauda equina injury caused by a pencil.

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Juan F. Martínez-Lage, Matías Felipe-Murcia, Encarna Guillén Navarro, María-José Almagro, Antonio López López-Guerrero, and Miguel A. Pérez-Espejo

Niikawa-Kuroki, or Kabuki syndrome (KS), is characterized by distinctive facial features, skeletal anomalies, persisting fingertip pads with dermatoglyphic abnormalities, short stature, and mental retardation. Neurological manifestations and CNS anomalies have been described in some patients with this condition. However, craniosynostosis has been documented in only 4 patients with KS who did not undergo operations. The authors report a case of KS with unicoronal synostosis that constitutes the first documented instance of a patient with this syndrome submitted to surgery. Previous reported instances of craniosynostosis occurring in KS are briefly reviewed. Although rarely documented, craniosynostosis might represent a relatively frequent feature of this syndrome. Kabuki syndrome should be considered at the time of evaluating children with craniosynostosis. The diagnosis of KS can be suspected from the patients' characteristic facial features. Kabuki syndrome appears to be an underdiagnosed condition in the craniosynostosis population. Given that most patients with this syndrome suffer from only mild to moderate mental retardation, surgical correction can be considered in instances of KS with craniosynostosis.