1/8. encephalocele as a late complication of cranial vault reconstruction in a patient with Crouzon's syndrome.encephalocele is a rare late complication of craniosynostosis repair. An undiagnosed nick to the dura is the etiology of this complication. It usually presents within a few months to years after repair. This case report describes a patient with Crouzon's disease who had a craniosynostosis repair as an infant and presents 22 years later with an encephalocele. The patient had a thorough physical exam, 2D, and 3D CT Scans as a preoperative workup. Surgical intervention included repair of the encephalocele, cranialization of the frontal sinus with bone grafting, and Lefort III osteomies for mid face advancement. The patient benefited from a two-team simultaneous approach between neurosurgery and Plastic Surgery. The patient also benefited from an aggressive one-stage repair of all her defects and deformities.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/8. Antenatal evaluation of an encephalocele in a dizygotic twin pregnancy using fast magnetic resonance imaging.We report a case of an encephalocele in a dizygotic twin pregnancy, following ovulatory induction. In the involved fetus, an abnormal shadow like an encapsulated-solid tumor located on the occiput was found by routine maternal transabdominal ultrasonography at 17 weeks of gestation. The parents did not accept induced abortion because of the presence of another fetus with no abnormality on ultrasonography. At 35 weeks of gestation, transabdominal ultrasound examination showed a large occipital cyst, composed of protrusive fetal brain and cerebrospinal fluid. Fast-scanning magnetic resonance imaging delineated more clearly the inside of the abnormal lesion and thus allowed confirmation of the putative diagnosis of fetal encephalocele during pregnancy. Surgical report was possible in this case, and the patient had no severe physical or neurological abnormalities 10 months after birth. Since the prognosis appears to depend primarily on how prominent the brain tissue is inside the herniated sac, this approach had benefit for clinical decision making.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/8. Large anterior skull base lesions in patients presenting with obstructive sleep apnea. Report of two cases and review of the literature.patients with sleep apnea often are treated by sleep disorder specialists and are studied in a sleep laboratory. The authors present two such patients who ultimately were found to harbor large benign anterior skull base lesions that caused their obstructive sleep apnea (OSA). The first patient had a massive pituitary tumor and had undergone a tracheostomy before the lesion was diagnosed. The second patient had a large frontoethmoidal encephalocele that was diagnosed at the same time as a recommendation for continuous positive airway pressure therapy was being considered. Such therapy in the presence of an encephalocele can be dangerous and even fatal. Although there are case reports of tumors causing OSA, nearly all of these lesions have been large pharyngeal lipomas (some of which were palpable in the neck during physical examination) or growth hormone-secreting pituitary adenomas. The patients reported here were completely unaware of the presence of these large lesions until imaging studies and/or nasal endoscopy were performed. These cases illustrate the need to perform nasopharyngeal endoscopy and also to obtain magnetic resonance images of the head before prescribing therapy for OSA. Neurosurgeons must be aware that large skull base lesions sometimes present only with OSA.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
4/8. Is there an upper limit of intracranial pressure in patients with severe head injury if cerebral perfusion pressure is maintained?Authors of recent studies have championed the importance of maintaining cerebral perfusion pressure (CPP) to prevent secondary brain injury following traumatic head injury. Data from these studies have provided little information regarding outcome following severe head injury in patients with an intracranial pressure (ICP) greater than 40 mm Hg, however, in July 1997 the authors instituted a protocol for the management of severe head injury in patients with a glasgow coma scale score lower than 9. The protocol was focused on resuscitation from acidosis, maintenance of a CPP greater than 60 mm Hg through whatever means necessary as well as elevation of the head of the bed, mannitol infusion, and ventriculostomy with cerebrospinal fluid drainage for control of ICP. Since the institution of this protocol, nine patients had a sustained ICP greater than 40 mm Hg for 2 or more hours, and five of these had an ICP greater than 75 mm Hg on insertion of the ICP monitor and later experienced herniation and expired within 24 hours. Because of the severe nature of the injuries demonstrated on computerized tomography scans and their physical examinations, these patients were not aggressively treated under this protocol. The authors vigorously attempted to maintain a CPP greater than 60 mm Hg with intensive fluid resuscitation and the administration of pressor agents in the four remaining patients who had developed an ICP higher than 40 mm Hg after placement of the ICP monitor. Two patients had an episodic ICP greater than 40 mm Hg for more than 36 hours, the third patient had an episodic ICP greater than of 50 mm Hg for more than 36 hours, and the fourth patient had an episodic ICP greater than 50 mm Hg for more than 48 hours. On discharge, all four patients were able to perform normal activities of daily living with minimal assistance and experience ongoing improvement. Data from this preliminary study indicate that intense, aggressive management of CPP can lead to good neurological outcomes despite extremely high ICP. Aggressive CPP therapy should be performed and maintained even though apparently lethal ICP levels may be present. Further study is needed to support these encouraging results.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
5/8. The prenatal diagnosis of the walker-warburg syndrome.On the basis of physical features and autopsy findings, a child with congenital hydrocephalus, bilateral microphthalmia, myopathy, severe developmental retardation and multiple brain malformations was diagnosed to have the walker-warburg syndrome (WWS). During a subsequent pregnancy in this family, a fetus at risk for this autosomal recessive condition was evaluated with serial ultrasound examinations. At 15 weeks of gestation an encephalocele was noted. Disproportionately slow growth of the head compared to the body was noted at 36 weeks. At birth, the diagnosis of WWS was confirmed in the child due to the presence of microcephaly, an encephalocele, a meningocele and bilateral microphthalmia. This is the first reported case of the early prenatal diagnosis of this recently categorized genetic condition, in which the major features are hydrocephalus, multiple central nervous system malformations, microphthalmia with ocular malformations, severe psychomotor retardation, congenital myopathy and a very limited life expectancy.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
6/8. Congenital midline nasofrontal mass. Two case reports with a clinical review.Because of aberrant embryologic development, encephalocele, nasal dermoid, and glioma may present as a nasofrontal midline mass in a newborn infant. Though uncommon, vascular malformation or hemangioma in the nasofrontal region may present similarly. The correct diagnosis and early management of a nasofrontal mass is imperative because complications such as brain herniation, hypertelorism, or cerebrospinal fluid rhinorrhea may ensue, resulting in poor facial or visual development, recurrent meningitis, and brain abscess. Nasofrontal encephalocele and midline frontal vascular malformation are rare congenital abnormalities presenting as a midline nasofrontal mass. We report two neonatal cases, one a frontoethmoidal encephalocele and another, a frontal vascular malformation. Both had uncomplicated surgical interventions. The physical findings and clinical review are presented. The diagnostic approach toward midline nasofrontal mass is discussed.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
7/8. Morning glory syndrome in children. association with endocrine and central nervous system anomalies.OBJECTIVE: To identify morning glory syndrome, an uncommon optic disc anomaly. Generally, it is an isolated ocular abnormality; however, some cranial facial and neurologic associations have been reported. patients: We herein report two patients with morning glory syndrome and associated pituitary dwarfism. In one patient, the pituitary insufficiency was secondary to compression of the pituitary gland by a basal encephalocele; in the second patient, causative factors were not identified. CONCLUSIONS: patients with morning glory syndrome should have a complete general physical examination and growth evaluation so that early recognition and treatment of the patient with pituitary dwarfism can occur.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
8/8. Vertebrobasilar distribution stroke mimicking transtentorial herniation.Strokes in the vertebrobasilar arterial distribution may result in a variety of physical findings and can be challenging to diagnose. We report a case of a 60-year-old woman with infarction of the left midbrain, right pons, and bilateral thalami with physical findings resembling transtentorial herniation.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |