Distribution and imaging characteristics of spina bifida occulta in young people with low back pain: a retrospective cross-sectional study.

PURPOSE: Spina bifida occulta (SBO) is one of the most common congenital spinal deformities. Although many studies have demonstrated the influence of lumbosacral dysplasia on low back pain (LBP) in young athletes, there have been few studies on SBO among young people in other occupations. The purpose of this study is to investigate the distribution of SBO in young people with LBP and to classify SBO from the perspective of lamina development. METHODS: The X-ray films of 148 young patients with LBP were analyzed to quantify the distribution of SBO and classify abnormal laminae. RESULTS: Of the 148 patients, 93 (61.49%) had SBO: 83 cases involved S1 alone, 2 involved L5-S1, 5 involved S1-2, 2 involved S1-4, and 1 involved L4-S4. According to the degree of the defect, the patients with SBO were divided on the basis of five grades: 9 patients with grade I, 53 with grade II, 23 with grade III, and 8 with grade IV. The cases were classified by the shape of the laminae into 4 types: 15 cases of type a, 11 cases of type b, 37 cases of type c, and 30 cases of type d. CONCLUSION: Among the young people with LBP that we surveyed, SBO is the most common lumbosacral dysplasia, which frequently involves the S1 segment. Most laminae in SBO are in the developmental stage of the spinous process, and an abnormal laminar growth direction and laminar stenosis are the most common laminar morphologies in SBO.

[Congenital defects of the spinal part of the neural tube]. / Wady wrodzone czesci rdzeniowej cewy nerwowej.

The abnormal closure of the neural tube results in defects of the nervous system development, which are referred to as dysraphism. Considering successive steps of the development of the human foetus, it can be estimated that spinal cord malformations arise from pathologies of early foetal development between 17th and 28th day gestational age. This time period comprises a development of the neural plate and subsequently neural tube. The development is completed with a closure of a posterior aperture of the neural tube (caudal neuropore). Congenital malformations are often caused by defective closure of the caudal neuropore. The neural plate develops about 17th day gestational age, the cerebral vesicle appears about 21st day and the neural tube forms between 17th and 20th day. The rostral neuropore closes on the day 25th as does the caudal neuropore on the day 28th. The embryo is 2.5 mm long at the time. Noxious factors acting during that period can affect normal closure of the caudal neuropore and distort the process of spinal cord canalization. The resultant defect is called spina bifida. This is the most severe form of dysraphism-rachischisis. Meninges are also affected in this defect. They cannot cover the neural canal and on the margins of the lesion are replaced by epithelium. In milder types of the defect lack of a complete bony framework is concealed by soft tissues, forming a sac of variable size over the lesion. Spina bifida can be subdivided according to the sac structure: meningocoele, meningocysticoele, meningomyelocoele, meningomyelocystocoele. Central canal pathologies constitute another form of spinal cord malformations presenting as hydromyelia. Abnormal closure of the neural tube may affect development of the vertebral column and spinal cord along their entire length or only at a certain portion. Malformations are seen most frequently in the lumbo-sacral and then cervical regions.

The tethered spinal cord: diagnosis, significance, and management.

Tethered cord is the result of fixation of the spinal cord, which limits motion in the caudal-rostral direction. Most children either have cutaneous markers or a predisposing condition. Prophylactic release of the spinal cord can prevent the long-term disabilities associated with this condition. Although delayed release, following the onset of a neurological deficit, may reverse some lost function, it is unlikely to restore bladder and bowel function.

[Tethered spinal cord syndrome in adults]. / Das Tethered-spinal-cord Syndrom beim Erwachsenen.

The tethered spinal cord syndrome is more often encountered in children, but does also occur in adults. Its clinical spectrum comprises low back pain, neurological deficits such as distal motor weakness and trophic and sensory disturbances in the legs, urological symptoms and such musculoskeletal signs as scoliosis or foot deformities. In addition, cutaneous lesions or subcutaneous lipomas in the lumbosacral region may be indirect signs of an intraspinal pathology. This consists in a tight, thickened and sometimes shortened filum terminale, an intraspinal lipoma, intradural scar formation or other lesions that lead to conus fixation. The common mechanism of injury of these types of pathologies is an impairment of longitudinal movement of the spinal cord, especially the conus medullaris, which subsequently leads to chronic local ischemia. Diagnosis is most readily achieved by magnetic resonance imaging. Treatment is aimed at the restoration of cord mobility by means of microsurgical release of the conus, the cauda equina and the filum terminale with the aid of cauda equina neuromonitoring. Further progression can be effectively halted; in fact almost half of the patient actually improve. Therefore, every patients presenting with the clinical diagnosis of tethered cord syndrome should be offered specialized surgical treatment.

Occult spinal dysraphism--the common combination of lesions and the clinical manifestations in 50 patients.

Fifty patients (26 males, 24 females) aged from birth to 51 years with occult spinal dysraphism were identified. All had Magnetic Resonance (MR) scans carried out. The MR scans were examined to determine the vertebral level of the conus and to see if one or more of the following were present; lipoma, syrinx, dermoid, diastematomyelia, and meningocele. In 43 patients the conus lay below the level of L3, 23 had a lipoma, 23 a meningocele, 20 an open central canal in the spinal cord or a syrinx, 15 a diastematomyelia and 4 a dermoid. The commonest combination of lesions was a long cord, a syrinx or an open central canal in the spinal cord and a lipoma. The diastematomyelias were always associated with a long cord and had the highest incidence of vertebral body anomalies (60%). The most frequent recorded signs were deformities of the feet, short legs, wasting of the calf muscles, weakness of the legs, back pain and bladder dysfunction. Combinations of these occurred with all of the lesions although some had none. Whilst deterioration was seen in about half of the patients, acute deterioration was uncommon and was associated most frequently with a dermoid or an expanded syrinx.

Evaluation of surgery for the tethered cord syndrome using a new grading system.

A new grading system is presented to assess the degree of untethering achieved at surgery for the 'tethered cord syndrome' based on intraoperative observation at the end of the procedure. Various pathophysiological mechanisms responsible for the 'tethered cord syndrome', as well as possible factors causing retethering were considered in developing this grading system. In Grade I the cord is considered to be fully untethered and the factors potentially responsible for retethering are eliminated, in Grade II partial untethering is performed and in Grade III untethering is unsuccessful. This grading system was used to assess the results of 22 consecutive operations performed to release a tethered cord between June 1991 and February 1995. The tethering factors encountered at surgery were: spinal lipoma in 14, diastematomyelia in five, a tight filum terminale in 10 and intradural adhesions in three instances. The grade of untethering was correlated with the type of pathology encountered, postoperative results, and whether previous surgery was performed or not. Previous surgery was found not to affect the rate of subsequent successful untethering.

Cutaneous stigmata of occult spinal dysraphism.

We describe a 4-year-old Hispanic boy with a dermal pit and an overlying macular vascular malformation in the lumbosacral area. Magnetic resonance imaging of the region revealed an intraspinal lesion at L1-L2. A fibrous tract was excised. A benign lipoma intrinsic to the roots of the cauda equina was noted at surgery. The cutaneous stigmata of occult spinal dysraphisms are reviewed.

Tethered cord syndrome: the low-lying and normally positioned conus.

We have previously proposed the existence of the tethered cord syndrome in the presence of a conus medullaris in the normal position. Our 12-year series of 73 patients with the tethered cord syndrome included 13 patients in whom the spinal cord terminated at or above the L1-L2 disc space. We compare the characteristics of these 13 with those of the 60 patients whose spinal cord terminated below the L1-L2 disc space. The parameters for comparison included neurological status at presentation and follow-up, the presence of cutaneous stigmata of occult spinal dysraphism, vertebral anomalies, and others. The frequency of occurrence of each parameter in the normally positioned group was essentially the same as its occurrence in the low-lying group.

Oto-palato-digital syndrome type II.

We report a patient with a sporadic case of oto-palato-digital (OPD) syndrome type II. Parents and five previous sibs are normal. At 26 years of age the patient had conductive hearing impairment, cleft palate, a prominent forehead, a flat facies, and a broad nasal base resulting in the characteristic "pugilistic" appearance. Extension and supination were limited at the elbows; thumbs and halluces were broad. Many radiological abnormalities were noted: malformations of the cervical spine, pelvic abnormalities, bilateral coxa valga, genu valgum, small fibulae, pes equino varus, and 15 carpal bones. IQ improved dramatically from 65 to 95.

Confirmation of a new MR/male pseudohermaphroditism syndrome, Verloes type.

In this report we describe a moderately mentally retarded adult male with a remarkable association of additional clinical symptoms, severe hearing loss, ocular colobomata, hypogonadism of central origin, distinct craniofacial features resembling the Borjeson-Forssman-Lehmann syndrome and skeletal anomalies with cervical spina bifida, hyperkyfosis and thoracic deformity. The findings in the present patient are very similar to the observation made by Verloes et al. in two male siblings with a hitherto undescribed MCA/MR syndrome.

Trends in clinical characteristics of infants with spina bifida--Atlanta, 1972-1979.

In Atlanta, the birth prevalence of spina bifida declined from 10.1 per 10,000 live births in 1972-1973 to 5.8 per 10,000 live births in 1978-1979. This trend cannot be explained by use of prenatal diagnosis, because it was unavailable in Atlanta until 1976, and even in the late 1970s was not used widely. To determine if this decrease was associated with changes in the distribution of clinical characteristics among infants with spina bifida, we reviewed the medical records of a population-based group of 154 infants with spina bifida, born in Atlanta during the eight-year period from 1972 through 1979. Distribution by the highest level of the defect on the spine did not change during these years. The proportions of infants with isolated spina bifida (ie, no other major malformations) and with open spina bifida (ie, not covered by skin) did, however, decrease. When we examined these two characteristics simultaneously, we found declines in both the proportion of infants with open-isolated spina bifida and the birth prevalence of infants with open-isolated spina bifida. If this trend persists, it will have important implications for workers involved with prenatal alpha-fetoprotein screening and researchers investigating the etiology of spina bifida.

[Lipomeningocele]. / Les lipomeningocèles.

In a revision of 473 spina Bifida cystica, 19 lumbosacral lipomas during the last 13 years is presented. It corresponds to a 4,01% of total cases. The results are commented pointing out the embryology, symptomatology and diagnostic. The treatment is surgical before symptomatology appear. It is very important early surgery for the risk is very low if we compare it with the serious neurologic symptoms that can be established. Tumor must be exirpated as much as possible, liberalizing the medullar adherences that avoid its free ascend. All surgical cases presented an intramedullar communication and compresion. Only three of them, under 3 months of life, are not operated. Postsurgery evolution is satisfactory.