Neurologic problems caused by vertebral stenosis in the thoracolumbosacral (TLS) region are common in achondroplasia. Surgical decompression by means of laminectomy is recommended often, but its long-term results have not been assessed. We reviewed the clinical history of 22 achondroplastic patients who had at least one TLS laminectomy performed before 1981. Symptoms predated the first TLS laminectomy by a mean of 2.3 years (range 0.1-17 years). Preoperatively, 91% of patients had motor function impairment, 86% had sensory dysfunction, 86% had neurogenic claudication, 77% had radicular pain, 59% had symptomatic bladder dysfunction, and 32% had fecal incontinence. Only upper motor neurons were affected in 45%, only lower motor neurons in 27%, and both in 27%. Follow-up after the first TLS laminectomy averaged 8 years. Of the 20 patients who initially improved neurologically, 12 had functional improvement for more than 5 years. However, 11 of these 12 subsequently regressed and 10 had additional laminectomies. Long-term neurologic and functional improvement was associated with both a short duration of symptoms preoperatively and absence of cervical stenosis. Because of hypertrophic scarring, 9 patients developed compression at the site of the initial TLS laminectomy and required re-operation 6.4 years (range 1-11 years) later. We conclude that TLS laminectomy is an effective treatment for spinal stenosis if performed early in the course of the neurologic syndrome. However, some patients have, or later develop, compression adjacent to the myelographic site of stenosis, and some develop hypertrophic scarring at the site of initial decompression. We therefore suggest that the first TLS laminectomy extend (1) 3 levels cephalad to the myelographic block, (2) at least to S2, and (3) laterally at least to the facets.

Currently accepted modes of clinical and radiologic evaluation were analyzed retrospectively in 55 patients with "normal-pressure" hydrocephalus on whom a cerebrospinal fluid shunting procedure was done. When applied alone, each criterion neither reliably differentiated normal-pressure hydrocephalus from cortical atrophy nor indicated in a significant number of cases which patients would benefit from shunting. Therefore, future prospective evaluations should include clinical history, physical and neurologic examination, skull radiography, echoencephalography, psychometric testing, brain scanning, lumbar puncture with cerebrospinal fluid laboratory analysis, isotope cisternography, pneumoencephalography, and constant-infusion manometric testing. Cerebral angiography may add optional support to the diagnosis of cortical atrophy but always should be done before lumbar puncture if there is evidence of intracranial mass and/or increased pressure is revealed on neurologic examination, skull radiographs, echograms, or brain scans. Patients with seizures should undergo electroencephalography. Postoperative improvement should be evaluated using serial neurologic and psychometric examinations. Echoencephalography may confirm postshunt reductions in ventricular size.

Twelve patients between the years of 1964 and 1971 underwent surgical treatment for the so-called Dandy-Walker syndrome at the Johns Hopkins Hospital. Five patients belonged to an early group (1–8 weeks); four patients to an intermediary group (3–20 months); and a late group consisted of three patients (3 years, 14 years and 34 years). The value of the various diagnostic studies is discussed on the basis of an analysis of the plain skull X-rays, cerebral angiograms, and combined air studies. The importance of a careful delineation of the underlying pathological condition is stressed to enable the surgeon to choose the appropriate technique. The diagnostic studies confirmed the diagnosis of a true Dandy-Walker syndrome in six of the twelve cases. The remaining six proved to have either an independent posterior fossa cyst with communication of the subarachnoid space and occasionally with the ventricles; or an isolated posterior fossa cyst with no communication but with impingement on the aqueduct and on the midline structures. In six patients, a primary simple shunt procedure was performed which had to be followed by a posterior fossa craniectomy in one and by a double-shunt (ventricle and posterior fossa cyst) in another. Four patients underwent a posterior fossa craniectomy; as the primary and only procedure (2), craniectomy followed by a ventriculoperitoneal shunt (1), or an unsuccessful shunt followed by craniectomy (1). In two patients, a so-called ‘double shunt’ was performed as a second operation, draining simultaneously the right lateral ventricle and the posterior fossa cyst. There was no death in this series. Three children had evidence of infected shunts; they responded to adequate therapy. Four had excellent results, with normal neurological examination and normal development. Four had good results, performing in school (working) was markedly improved; two had fair results with residual neurological deficit and some mental retardation. Two patients had to be considered as poor results. Both of them, however, have shown evidence of concomitant severe congenital abnormalities of the cerebellum on the diagnostic studies. The follow-up period ranged from 1½ to 7½ years.

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Spinal cord compression by epidural extramedullary hematopoiesis (EMH) is a rare phenomenon. A case of acute compressive myelopathy is reported in a 72-year-old man with EMH secondary to sideroblastic anemia. Technetium colloid scanning was used to document extensive ectopic marrow formation. The patient improved following surgery and radiotherapy. A review of the literature revealed 23 other cases of symptomatic spinal epidural EMH. The underlying hematological disorder varied but was always of long duration. Eighty-eight percent of the patients were males. Symptoms lasted longer than 1 week in 90% of cases, and 91% demonstrated incomplete neurological deficits. Plain x-ray films were rarely helpful in establishing the diagnosis. Technetium sulfur colloid bone marrow scanning has been used successfully to detect EMH and has led to preoperative diagnosis in one case. Decompressive laminectomy with or without postoperative irradiation is the suggested therapy, although there is evidence that radiotherapy alone may be adequate in some cases. Good recovery is the rule despite long-standing neurological deficits.

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