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Diastematomyelia is a rare but potentially devastating spinal dysraphism classically characterized as a bony or fibrous spur separating two hemispinal cords. This study reviews our experience with diastematomyelia over a 40-year period from 1947 through 1987, and suggests that the neurologic and orthopedic deficits of diastematomyelia remain stable during extended follow-up after surgery.
Using an "electronic nappy" of improved design and a miniature pressure manometer of the strain-gauge type mounted at the tip of a catheter, the case of a patient with spina bifida and a large lipoma has been investigated. Some retention exists, but areas on the lipoma surface can be touched lightly, causing an increase in bladder pressure with subsequent voiding as measured by the nappy. Investigations of the same area by electrical stimulation produced rises in bladder pressure sustained for only a few seconds, as in the case of manual stimulation. Local anaesthetic in the "trigger" area prevented bladder pressure changes and urine loss, but triggering returned as the anaesthetic wore off.
231 patients with neurogenic bladder dysfunction were evaluated for bladder deformities. Bladder deformity implies bladder trabeculation and deformity of bladder shape such as pine tree shape. Bladder deformity was judged radiographically at maximum cystometric capacity and classified into grade 0 (none), grade I (mild), grade II (moderate) and grade III (severe). Upper urinary tract deterioration (hydronephrosis and/or reflux) was found in 2% of grade 0, 8% of grade I, 52% of grade II and 62% of grade III. I suggested that bladder deformity was one of the risk factors for upper tract deterioration. High grade deformity (grade II and III) was found more frequently in traumatic spinal cord injury and spinal dysraphism than brain disease. Most patients with low compliance bladder had high grade bladder deformity. The management program including clean intermittent catheterization was effective in preventing bladder deformities.
Herein, the diagnosis and management of 14 cases with sacral agenesis experienced in the last 15 years were reviewed. Age at first presentation tended to be older than that of meningomyelocele patients. All the children displayed partial sacral agenesis or hemisacrum. Occult spinal dysraphism was found in 8. Frequent association of genitourinary and anorectal anomalies required important alterations in the management. Vesicoureteral reflux was found in 8 patients but most of them were treated conservatively. Thirteen children underwent urodynamic evaluation on more than 1 occasion and 12 displayed neurogenic bladder dysfunction (upper motor type 6, lower motor type 5, upper and lower type 1). However, the diagnosis of bladder dysfunction was not final. Clean intermittent catheterization was most frequently assigned but it was difficult in some cases with urethral abnormalities. Overall, 5 children required surgical intervention for their urinary tract, 4 of whom needed multiple procedures. As a result, half the children achieved continence and 1 child, who awaited reconstructive surgery, had slightly elevated serum creatinine. In summary, early diagnosis, complete neurourological evaluation and adequate management with careful follow-up monitoring risk factors to the upper urinary tract are mandatory.
Spina bifida was diagnosed in four English Bulldogs and one Collie dog. These cases and the examination of records from the Veterinary Medical Data Program suggested a high incidence of spina bifida in the English Bulldog. Urinary and fecal incontinence was the most common reason for initial examination for the five dogs. Radiographs and myelography confirmed the diagnosis of spina bifida. Blood, cerebrospinal fluid and urine analyses did not aid diagnosis but urocystitis was identified. In addition to spina bifida, clinical signs and morphologic changes in the spinal cords of all five dogs fulfilled the criteria for an additional diagnosis of spinal dysraphism. A common pathogenesis for the two conditions is suggested.
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The intent of this article is to propose the existence of the tethered cord syndrome in patients whose conus is in a normal position. The tethered cord syndrome has been a well-recognized entity, occurring in the pediatric and adult population. A central tenet to this syndrome is that the conus medullaris must be "abnormally" low, regardless of the tethering lesion. Our 12-year series of 73 patients with tethered cord syndrome included 13 patients in whom the cord terminated at or above the L1-L2 space. These patients otherwise displayed characteristics usually associated with the patient with an abnormally low conus. Most patients with progressive neurological deterioration responded to surgery. The preoperative radiographic evaluation and the operative findings usually demonstrated a thickened filum. Tethered cord syndrome may occur in the patient who has a conus in the "normal" position.
A case of roentgenographic identification of a decomposed, visually unidentifiable, edentulous deceased person is reported. Comparative evaluation of ante-mortem and post-mortem plain radiographs provided positive identification, confirming the presence of a transitional lumbar vertebra with concordance in dysmorphic features of a spinal dysraphism, and the configuration of sternotomy sutures used for surgical reconstruction of the sternum post-coronary artery bypass surgery. In addition, comparison of ante-mortem and post-mortem dental orthopantomograms confirmed the presence of iatrogenic dental evidence, in the form of amalgam tattooing. The use of amalgam tattooing in establishing identification has only been reported once previously in the literature (Slabbert et al., 1991). The stability in morphology and the location of the amalgam particle was sufficient to confirm the identification of the deceased as a single trait or as an adjunct to other identification criteria.
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In this study, interobserver and intraobserver variations in the interpretation of plain radiographs of the lumbosacral spine were evaluated. Three radiologists independently interpreted the radiographs from 200 consecutive outpatients, aged 13-93 years, mostly referred from general practitioners. Interobserver agreement was best for vertebral fractures, osteopenia, spondylolisthesis at L5-S1, lumbosacral junctional vertebra, reduced disc height at L4-S1 and osteophytes at L2-S1 (kappa 0.61-0.95), and poorest for spina bifida of S1, degenerative spondylolisthesis and facet joint arthrosis at T12-L4, sacroiliac joint arthrosis, narrow central spinal canal, film quality, and for decisions concerning evaluation of facet joints and spinal canal (kappa < 0.34). For several diagnoses, the number of abnormal findings differed significantly between observers (p < 0.05, McNemar's test), indicating different diagnostic thresholds. Intraobserver agreement in 36 reevaluated patients was fair to excellent for almost all variables (kappa > 0.46). Although some diagnoses related to low back pain were quite consistently evaluated, the substantial disagreement on many findings should alert clinicians and radiologists against overestimating the validity and usefulness of the examinations. To improve diagnostic consistency, it is important to reduce variation caused by different thresholds for abnormality.
A radiographic suvey has been carried out of 147 first-degree relatives of forty-seven patients treated in Edinburgh for spondylolisthesis of the fifth lumbar vertebra; twelve patients had the dysplastic (congenital) type and thirty-five an isthmic defect. The survey identified 19 per cent of relatives with spondylolysis, and index patients with each type of spondylolisthesis had relatives with the opposite type. Index patients with the dysplastic form had a higher proportion of affected relatives (33 per cent) than had those with the isthmic type (15 per cent), but both figures were significantly in excess of the estimated frequency for the general population of under 1 per cent and 5 per cent respectively. Spina bifida occulta at the fifth lumbar or first sacral level or both, and lumbosacral segmental defects were commoner amongst all individuals with spondylolysis than amongst unaffected relatives (dysplastic form 94 per cent, isthmic type 32 per cent, unaffected relatives 7 per cent). However, there was no single instance of a neural tube defect (anencephaly, spina bifida with or without meningocele, other generalised vertebral anomalies or spinal dysraphism) amongst 826 first-, second- or third-degree relatives. It is concluded that the developmental defects of the vertebrae associated with spondylolysis are not aetiologically related to the neural tube defects. The one in three risk of spondylolysis to near relatives of patients with the dysplastic form of spondylolisthesis is emphasised in order that the deformity in their sibs and children can be recognised at any early age.
Growth retardation and precocious puberty are frequently found in children with meningomyelocele (MMC). Lower limb contractions, spasticity and kyphoscoliosis may lead to disproportionate short stature. Most of these patients have structural brain defects or hydrocephalus which can cause growth hormone deficiency. In this study, 19 children aged between 3.5 and 12.8 years with MMC and growth hormone (GH) deficiency were treated with recombinant human GH for a period of 12 months. Supine length, arm span and growth velocity were compared before, and after 6 and 12 months of treatment with rhGH (daily dose 2.0 IU/m2 BSA s.c.). Mean supine length standard deviation score (SDS) increased by +0.8 SDS after 6 months and +1.2 SDS after 12 months of therapy. Mean arm span standard deviation score increased by +0.9 SDS and +1.3 SDS. Growth velocity increased in supine length from 3.3 cm/yr (-2.1 SDS) to 8.4 cm/yr (+2.4 SDS) and in arm span from 4.8 cm/yr (-1.3 SDS) to 8.6 cm/yr (+3.1 SDS) in the first 6 months and was 8.1 cm/yr (+2.4 SDS) and 8.3 cm/yr (+2.6 SDS) after 12 months of therapy. Linear correlation between SDS growth velocity supine length and SDS growth velocity arm span during one year of treatment was excellent (r = 0.65, p < 0.0025). We surmise that body proportions do not deteriorate when growth velocity is stimulated in MMC patients. Both supine length and arm span measurements are necessary to document growth in children with spinal dysraphism.
We reviewed the results for forty-three patients who had a diastematomyelia. All of the patients had been skeletally immature when the diagnosis was made, the mean age being six years (range, birth to thirteen years), and were skeletally mature by the time that they were evaluated by us. When they were first seen at our institution, twenty-four patients (56 per cent) had a cutaneous lesion, such as hairy patch, dimple, hemangioma, subcutaneous mass, or teratoma at or near the level of the diastematomyelia; thirty-four patients (79 per cent) had congenital scoliosis; and forty-two patients (98 per cent) had at least one associated musculoskeletal anomaly, such as spinal dysraphism, asymmetry of the lower extremities, club foot, or a cavus foot. In twenty-seven patients (63 per cent), the diastematomyelia was located in the lumbar spine. Thirty-six patients had eighty-four neurological manifestations. Resection of the spur was performed in thirty-three patients at a mean age of seven years (range, three months to seventeen years). Twenty-two patients who had a resection had no change in neurological condition, nine patients had improvement, and one patient had one symptom improve and another symptom worsen after the operation. We believe that resection of the spur should be performed in patients who have progressive neurological manifestations. Patients who do not have progressive neurological manifestations should be observed; if progression is noted, a resection should then be performed.
A 2-year-old boy presented with a rare sacral arachnoid cyst manifesting as gait disturbance. Neuroimaging revealed an intradural cyst in the sacral nerve root sheath associated with spina bifida occulta and a lipoma at the same level. At surgery, the conus medullaris was situated at the L-1 level and not tethered. The highly pressurized arachnoid cyst had exposed the dural sheath of the left S-2 nerve root and compressed the adjacent nerves. An S-2 nerve root pierced through the cyst. There was no communication between the cyst and spinal arachnoid space. We thought the one-way valve mechanism had contributed to the cyst enlargement and the nerve compression. Radical resection of the cyst was not attempted. A cyst-subarachnoid shunt was placed to release the intracystic pressure. Postoperatively, his gait disturbance improved and no deterioration occurred during the 4-year follow up. Both tethered cord syndrome and sacral arachnoid cyst in the nerve root sheath should be considered in pediatric progressive gait disturbance. Cyst-subarachnoid shunt is an alternative method to cyst resection or fenestration to achieve neurological improvement.
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In an attempt to prevent tethering following intradural procedures in the spine or at the foramen magnum silastic film has been inserted as a dural substitute. Magnetic resonance imaging has enabled us to judge the effectiveness of this material in preventing tethering at the operative site. Our experience of Silastic dura substitute in 43 paediatric and adult cases is discussed, and illustrated together with a description of the technique of insertion and an analysis of the complications associated with its use.
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The authors report a case of primary brainstem tethering, a component of a spinal dysraphic state, occurring in a 13-year-old girl. This patient also had associated hydrocephalus, a low-lying tethered cord, and a syrinx in her conus medullaris. The significance of imaging surveys of the craniospinal region and head in a case involving a low-lying tethered cord is highlighted. The insertion of a ventriculoperitoneal shunt, leading to traction on the tethered brainstem, proved nearly fatal for this patient. In cases involving a multiple-level tethered cord, the sequence of detethering should be decided on with caution. It seems prudent to detether the vital neural structure first, so as to minimize the traction force on that structure. All four previously reported cases of secondary tethered brainstem and cerebellar vermis in the literature are reviewed and compared with the present case of primary brainstem tethering. The literature describing brainstem dysfunction caused by ventricular drainage is also reviewed.