P. Guerra

Oncogenic osteomalacia caused by a phosphaturic mesenchymal tumor of the thoracic spine

Phosphaturic mesenchymal tumors that cause the paraneoplastic syndrome known as oncogenic osteomalacia are rare. The authors report on the case of a 57-year-old man with a history of osteomalacia and in whom was diagnosed a thoracic spine tumor at the T-4 level. Complete tumor resection was accomplished. The histological diagnosis was phosphaturic mesenchymal tumor (mixed connective tissue variant). After lesion removal, the paraneoplastic syndrome resolved. At the 24-month follow-up, no recurrence of the disease was observed. The clinical presentation, surgical technique, and follow-up in this case were reviewed in detail.

Severely Symptomatic Craniovertebral Junction Abnormalities in Children

Objectives: The treatment of symptomatic cranio-vertebral junction (CVJ) instability in children affected by CVJ abnormalities is a challenge. A series of severely symptomatic children has been reviewed to understand the controversial long-term effectiveness of the aggressive management of CVJ abnormalities, in terms of clinical improvement, spinal stability and growth. Methods: Three Down syndrome patients, 1 with mucopolysaccharidosis and 1 with os odontoideum (range 3–6 years old) with a CVJ instability determining spinal cord compression with severe neurological deficits (the patients presented at admission a Ranawat III A/III B neurological condition), were consecutively treated at our institution. Medical records, imaging studies, adopted surgical techniques and long-term results were reviewed. Details of the presenting symptoms, clinical and radiological signs were compared to the signs and symptoms at follow-up. Results: The perioperative use of an halo-orthosis, the operative techniques and the timing of rehabilitation were always tailored to the patient’s anatomical features. All the patients showed remarkable neurological improvements, along with construct stability and bone fusion without abnormalities of the developing spine. Conclusions: Considering the effective long-term results, we recommend, even in severely symptomatic children with CVJ abnormalities, a multidisciplinary aggressive tailored treatment of instability with rigid internal fixation.

Primary traumatic benign midbrain haematoma in hyperextension injuries of the head.

Primary traumatic brain stem injury occurring in isolation is not universally recognized as a distinct pathological entity which may follow a head injury. We describe two patients with clinical and radiological evidence of primary posttraumatic midbrain haemorrhage occurring in isolation associated with good recoveries. It is suggested that paramedian midbrain syndromes associated with midbrain haemorrhages should be recognized as a distinct, although unusual, complication of hyperextension injury to the head which may have a benign course.

Diffuse Traumatic Axonal Injury: a Longitudinal Study

Patients who immediately lose consciousness after acceleration-deceleration injuries of the head and whose CTs are characterized by the presence of small hyperdense lesions in the corpus callosum and/or paramedian white matter and/or upper brainstem are generally considered as affected by diffuse axonal injury (DAI). The presence of scattered haemorrhages in the white matter of cerebral hemispheres and posterolateral mesencephalon (tissue tear haemorrhages) would be considered by many authors as a hallmark of severe axonal damage, predictive of less than optimal outcomes.

Relationship Between Clinical Course, CT Scan and ICP in Post-Traumatic Diffuse Lesions

There is strong anatomicopathological and experimental evidence that widespread axonal damage in the white matter (Diffuse Axonal Injury = DAI) is the primary brain lesion in posttraumatic diffuse injuries (Adams et al. 1982, Langfitt et al. 1982). Focal hemorrhages in the corpus callosum, basal ganglia and brain stem (shearing injury) are the distinctive macroscopic features of DAI (Strich 1961). DAI patients are comatose immediately after trauma and the CT scan shows deep hemorrhages that are indicative of the lesion. Patients in coma with a normal CT may belong to the same clinicopathological syndrome as the hemorrhagic components of DAI, demonstrated in these patients by Magnetic Resonance Imaging (Wilberger et al. 1987). DAI may be associated with diffuse brain swelling (Langfitt et al. 1982), hyperemia and increased cerebral blood volume playing the main role in its pathogenesis. The typical CT picture of diffuse brain swelling shows reduction in size of lateral ventricles (slit like ventricles) and absence of the 3rd ventricle and basal cisterns.

Effects of Intracranial Hypertension on Evolution of Post-Traumatic Acute Subdural Hematoma

Acute subdural hematoma (SDH) is a posttraumatic lesion that results in a severe neurological impairment and a high mortality rate due to the effects of the mass expanding lesion, the resulting intracranial hypertension and the presence of injury to brain parenchyma (Becker 1986; Cooper 1982).