Intraspinal characteristics of thoracic spinal nerve roots anomalies.

Purpose: The anomalous anatomical arrangement of the thoracic spinal nerve roots within the spinal canal can complicate the surgical treatment of several pathologies. The aim of this work was to reveal intraspinal anatomical variations of the thoracic spinal nerve roots.Methods: Anatomical study on 43 cadavers with a mean age of 53.7. After opening the spinal canal and dural sac, intradural and extradural anomalies of the thoracic spinal nerve roots were documented. Extradural communicating branches were excised, histologically processed and examined for the presence of nervous tissue.Results: We found 14 cases (32.6%) of intraspinal thoracic nerve root variations: intradural in 8 cases (18.6%), intradural communicating branches in 3 cases (6.97%), extradural anatomical variations occurred 6 cases (13.95%), 2 cases (4.65%) had extradural communicating branches between the nerve roots, 1 case had simultaneous occurrence of intradural and extradural communications (0.23%). All the results are differentiated according to the plexus type. In macroscopic extradural thoracic communicating branch had no nervous tissue on microscopy.Conclusions: This study describes intraspinal anatomical variations of thoracic spinal nerve roots. Knowledge of these variables should help prevent the failure of several medical procedures.

Anatomical features of the cervical spinal canal in Chiari I deformity with presyrinx: A case-control study.

Purpose The relationship between syringomyelia and presyrinx, characterized by edema in the spinal cord, has not been firmly established. Patients with syringomyelia have abnormal spinal canal tapering that alters cerebrospinal fluid flow dynamics, but taper ratios in presyrinx have never been reported. We tested the hypothesis that presyrinx patients have abnormal spinal canal tapering. Materials and methods At six medical institutions, investigators searched the PACS system for patients with Chiari I and spinal cord edema unassociated with tumor, trauma, or other evident cause. In each case taper ratios were calculated for C1 to C4 and C4 to C7. In two age- and gender-matched control groups, Chiari I patients with no syringomyelia and patients with normal MR scans, the same measurements were made. Differences between groups were tested for statistical significance with t tests. Results The study enrolled 21 presyrinx patients and equal numbers of matched Chiari I and normal controls. C4 to C7 taper ratios were positive and steeper in presyrinx patients than in the normal controls ( p = 0.04). The upper cervical spine, C1 to C4, tapered negatively in cases and controls without significant differences between the groups. The difference in degree of tonsillar herniation was statistically significant between presyrinx patients and Chiari I controls ( p = 0.01). Conclusions Presyrinx patients have greater than normal positive tapering in the lower cervical spine and greater degree of tonsillar herniation than the controls.

Defectos de la pared abdominal: complejo OEIS complex. Afectación de uno de los gemelos en una gestación monocorial monoamniótica / Defects of the abdominal wall: OEIS complex. Affection of one of the twins in a monoamniotic monocorial gestation

Los defectos de la pared abdominal engloban un amplio grupo de patologías que pueden clasificarse en distintos tipos según sus características anatomopatológicas y patogenia. El complejo OEIS complex es una rara entidad compuesta por onfalocele, extrofia vesical, ano imperforado y defectos espinales, dando origen a las siglas en inglés (Omphalocele, bladder Exstrophy, Imperforateanu and Spinal defects) y asociándose también a ausencia de genitales externos, deformidad en la flexión de miembros, defectos en pie y arteria umbilical única. Su incidencia estimada es de 1/200.000 a 1/400.000 nacidos en gestaciones únicas, menor en gestaciones gemelares. Su etiología en la mayoría de las ocasiones es desconocida, esporádica y heterogénea, pudiendo asociarse a varios factores. Presentamos el caso de una mujer con gestación monocorial monoamniótica y afectación de uno de los gemelos por este defecto (complejo OEIS complex), presentando un segundo gemelo totalmente sano, lo que demuestra la falta de conocimiento en cuanto a la etiología de esta rara entidad y la improbable implicación de los factores genéticos (AU)

The abdominal wall defects encompass a variety of disorders that can be classified into different types according to their pathologic features and pathogenesis. The OEIS complex is a rare complex composed of omphalocele, bladder exstrophy , imperforate anus and spinal defects, giving rise to the acronym (Omphalocele, bladder Exstrophy, Imperforate anus and Spinal defects) and associating with the absence of external genitalia, flexion deformity member, standing defects, and single umbilical artery. Its incidence is estimated from 1/400,000 to 1/200,000 born in singleton pregnancies, lower in twin pregnancies. The etiology in most cases is unknown, sporadic and heterogeneous it may be associated with several factors. We report the case of a woman with monochorionic monoamniotic gestation and involvement of one of the twins for this defect (OEIS complex complex), presenting one second fully healthy twin, demonstrating the lack of knowledge regarding the etiology of this rare entity and the unlikely involvement of genetic factors (AU)

Cystic Dilation of the Ventriculus Terminalis: Report of 6 Surgical Cases Treated with Cyst-Subarachnoid Shunting Using a T-Catheter.

BACKGROUND: Cystic dilation of the ventriculus terminalis (CDVT) is a rare anatomic variation that and may become symptomatic. Literature regarding CDVT is limited, and thus the clinical characteristics and management strategy of CDVT are unclear. Here we report 6 cases of CDVT seen in our institution, and provide a review of the current literature. METHODS: The study cohort comprised 6 patients with CDVT seen at Beijing Tiantan Hospital between September 2010 and June 2015. All 6 patients were managed surgically. Data on age, sex, clinical presentation, radiologic features, operative methods, and surgical outcomes were reviewed retrospectively. We evaluated the clinical outcomes and postoperative radiologic changes of these patients and compared them with findings of similar previous reports. RESULTS: All 6 patients were female, with a median age of 50.0 years (interquartile range [IQR], 25.8-54.0 years). Surgical management of cyst-subarachnoid shunting using T-catheter after myelotomy was successfully performed in all patients. During an average follow-up of 41 months, relief of clinical symptoms, especially low-back pain and sciatica, were observed in all 6 patients. No patients experienced symptom recurrence during follow-up, and postoperative magnetic resonance imaging revealed no regrowth of the lesion. Patients with focal neurologic deficits and sphincter disorders also achieved symptom relief from the procedure. CONCLUSIONS: CDVT is an extremely rare cause of conus medullaris syndrome with an unclear treatment strategy. Our successful surgical management of 6 cases through cystic-subarachnoid shunting using a T-catheter for CDVT drainage provides a potential option for treating CDVT.

Mobile spinal enterogenous cyst resulting in intermittent paraplegia in a child: case report.

The authors report the case of a mobile spinal enterogenous cyst in a 2-year-old boy, who was admitted to the hospital several times for intermittent paraplegia. Magnetic resonance imaging and CT revealed an isolated cyst in the lumbar spinal canal. The symptoms were caused by transient myelopathy of the conus medullaris and radiculopathy of the cauda equina due to the changing size and location of the cyst. The cyst was surgically extirpated, after which the symptoms resolved. The histopathological diagnosis was enterogenous cyst. The clinical history of intraspinal enterogenous cyst is usually progressive. Mobility and changes in size are rare pathophysiological findings. The authors speculate that the cyst wall did not adhere to the surrounding structures and had ruptured and quickly reformed. Enterogenous cyst should be considered in the differential diagnosis of spinal intradural cysts in children with radiculomyelopathy.

Vertebral, rib, and intraspinal anomalies in congenital scoliosis: a study on 202 Caucasians.

PURPOSE: To investigate vertebral, rib and intraspinal anomalies in patients with congenital scoliosis and their association with each other METHODS: Clinical data and preoperative imaging studies of 202 Caucasians with congenital scoliosis operated on at an educational hospital within 6 years were reviewed for vertebral, rib, and intraspinal anomalies. RESULTS: Rib and intraspinal anomalies were present in 57.4 and 21.8 % of patients, respectively. Most vertebral anomalies were located in the middle-lower thorax. Being the most common vertebral defect (53.5 %), failure of segmentation was significantly more common in males, whereas mixed defects were more frequent in females. Formation and mixed defects were associated with rib changes. Vertebral anomalies were more extensive in males than in females. The presence of multiple hemivertebrae was associated with rib deformity and intraspinal anomaly. Location of the vertebral anomalies varied with gender and rib involvement. Majority of rib changes were of simple type (70.7 %), significantly more common in males. Conversely, females had significantly more fused and bifid ribs. Two most common intraspinal anomalies were diastematomyelia (36.4 %) and syringomyelia (18.2 %). Intraspinal anomalies were located most frequently in the upper and lower thoracic regions. Syringomyelia and low conus were associated with female gender, and patients with rib changes suffered from intraspinal anomalies more frequently. No significant association was found between vertebral and intraspinal anomalies. CONCLUSIONS: The incidences of rib and intraspinal anomalies were 57.4 and 21.8 % in surgical Caucasians with congenital scoliosis, respectively. Unlike vertebral and intraspinal anomalies, rib and intraspinal anomalies were significantly associated. Male gender and intraspinal anomaly were associated with some previously suggested risk factors of curve progression.

[Coffin-Lowry syndrome. Its association with congenitally narrow cervical canal and myelomalacia]. / Síndrome de Coffin-Lowry. Su asociación con canal cervical estrecho congénito y mielomalacia.

BACKGROUND: Typical clinical features of the Coffin-Lowry syndrome include facies with hypertelorism, small nose, wide mouth, full and everted lips; short stature, mental retardation, pectus deformity, mitral valve dysfunction, hippocampal and cerebellar involvement, hearing loss and spinal disorders such as kyphosis and scoliosis. Due to its scarce incidence, it is difficult making an early diagnosis. The aim of this report was to document the anatomical peculiarities identified during the surgical treatment of a patient with this syndrome. CLINICAL CASE: Male patient with Coffin-Lowry syndrome who evolved with narrow cervical canal plus myelomalacia at short age, making decompression from C3 to C6 and instrumentation from C2 to C7 necessary. During the surgery, in addition to calcification of the yellow ligament, adhesions on the dura mater from C4 to C4, dark purplish color in this area and hourglass-shaped thinning were found; the ends at C3 and C6 were normal. The purpose of the surgery was to stop the myopathy. Post-operatively, the patient had pulmonary complications; at the sixth day he passed away due to ventilatory complications and inadequate secretion control. CONCLUSIONS: The Coffin-Lowry syndrome is a rare diagnosis in our country; neurological involvement at the spinal level is characterized by kyphosis or scoliosis; for its diagnosis, an adequate medical history and a karyotype are necessary.

INTRODUCCIÓN: Las características clínicas típicas del síndrome de Coffin-Lowry son facies con hipertelorismo, nariz pequeña, boca amplia, labios amplios y evertidos; estatura corta, retardo mental, deformidad del pectus, disfunción de la válvula mitral, afectación de hipocampo y cerebelo, pérdida de la audición y trastornos de la columna, como cifosis o escoliosis. Debido a su escasa incidencia es difícil realizar el diagnóstico temprano. El objetivo de este informe fue documentar las peculiaridades anatómicas identificadas durante el tratamiento quirúrgico de un paciente con este síndrome. CASO CLÍNICO: varón con síndrome de Coffin-Lowry quien evolucionó con canal cervical estrecho más mielomalacia a corta edad, por lo que fue necesaria descompresión de C3 a C6 e instrumentación de C2 a C7. Durante la cirugía se encontró, además de la calcificación del ligamento amarillo, adherencias a la duramadre desde C4 a C5, color violáceo obscuro en esta área y adelgazamiento en forma de reloj de arena; los extremos en C3 y C6 eran normales. El objetivo de la cirugía fue detener la miopatía. En el posquirúrgico, el paciente presentó complicaciones pulmonares; al sexto día falleció por complicaciones ventilatorias y mal manejo de secreciones. CONCLUSIONES: el síndrome de Coffin-Lowry es un diagnóstico raro en nuestro país, la afección neurológica a nivel de la columna se caracteriza por cifosis o escoliosis, para su diagnóstico es necesario una adecuada historia clínica y un cariotipo.

Tapering of the cervical spinal canal in patients with Chiari I malformations.

BACKGROUND AND PURPOSE: Upper cervical spinal canal dimension may have a role in abnormal CSF dynamics in patients with Chiari I malformation. We attempted to measure spinal canal tapering from anteroposterior spinal canal dimensions in patients with Chiari I. MATERIALS AND METHODS: Twenty-one patients with Chiari I malformation, including 12 with syringomyelia and 7 patients with IS were identified from a local registry. Age- and sex-matched control subjects with cervical spine MR imaging findings reported as normal were selected from the PACS. The anteroposterior diameter of the spinal canal was measured at C1-C7 on T2-weighted sagittal MR images. The taper ratio of the spinal canal was calculated with the regression line. Goodness of fit was calculated as R(2). Differences between patients with Chiari I and other patients were tested for significance with Kruskal-Wallis tests and multivariate analysis. RESULTS: Taper ratios averaged -0.6 ± 0.3 mm/level in the patients with Chiari and syrinx, -0.4 ± 0.2 mm/level (mean ± 1 SD) in the patients with Chiari without syrinx, and -0.3 ± 0.5 mm/level in the patients with IS; control groups had average taper ratios of -0.3 ± 0.2 mm/level. Mean R(2) equaled 0.43. Taper ratios in patients with Chiari and syringomyelia differed significantly from those in the control group (P = .003). Taper ratios in the patients with Chiari without syrinx and in patients with IS did not differ significantly from their matched control groups (P = .60 and 0.76, respectively). CONCLUSIONS: Patients with Chiari I and a syrinx have steeper tapering of the upper cervical spinal canal than matched controls.

Confirmation of microevolutionary increase in spina bifida occulta among Swiss birth cohorts.

Previous studies on the prevalence of spina bifida occulta have indicated a microevolutionary increase in its frequency and possible population differences in the prevalence of the condition. We studied the frequencies of closed and open sacral canals at each sacral level among two birth cohorts in Switzerland. Transverse CT scans and multiplanar reconstruction images of sacra of 95 males and 96 females born in 1940-1950 and 99 males and 94 females born in 1970-1980 in Switzerland were reviewed. We found that individuals born later have significantly more open sacral arches at all sacral levels compared to those born 30-40 years earlier. When results were related to previously published data on Australian cohorts, the trend was the same, but Swiss in both cohorts were less likely to have an open section than Australians at all locations apart from S2. This study confirmed a microevolutionary trend in the opening of sacral canal among two different generations in Switzerland and demonstrated a population difference in the prevalence of spina bifida occulta.

Magnetic resonance imaging in the evaluation of the fetal spinal canal contents.

Ultrasonography (US) remains the first method in the evaluation of fetal central nervous system (CNS) abnormalities but in case of the spinal canal and cord it is often insufficient since the bony structures may obscure these structures. Prenatal magnetic resonance imaging (MRI) is therefore the final noninvasive tool for the assessment of these malformations allowing for correction of sonographic findings, revealing the full extent of complex lesions and choosing the candidates for in utero treatment. The authors present the most frequent anomalies of spinal canal and spinal cord in the consecutive phases of pregnancy, illustrated with their own MR images, with reference to the literature and own experience. In 58 out of 252 fetuses examined due to suspicion of CNS anomalies (23.0%) the spinal canal and spinal cord abnormalities were found on MRI. The cases of diastematomyelia, myelomeningocele, tethered cord, caudal regression syndrome, anterior meningocele, cystic sacrococcygeal teratoma and syringohydromyelia are demonstrated.

Fetal extraperitoneal rectal perforation presenting after duodenal atresia repair.

Fetal extraperitoneal rectal perforation (FERP) is a very rare condition, but prompt diagnosis and appropriate treatment produce overall good outcome. We report the first case of FERP known to be associated with duodenal atresia, which only became clinically apparent after duodenal atresia repair and initially presented a diagnostic difficulty. Our case was successfully treated with a defunctioning colostomy and subsequent excision of the pseudocyst cavity. Proximal atresias can mask the presentation of FERP, and this diagnosis should be considered in cases of neonatal perineal and buttock swelling.

Relationship between spinal magnetic resonance imaging findings and candidacy for spinal surgery.

OBJECTIVE: To compare the prevalence of spinal abnormalities found on magnetic resonance imaging (MRI) in symptomatic surgical candidates and non-surgical patients. DESIGN: Retrospective cohort study. SETTING: A single academic spine surgery practice in Toronto, Ont. PARTICIPANTS: A total of 1586 symptomatic patients referred during a 32-month period; based on chart review, patients were classified as surgical candidates (n=722), non-surgical patients (n=690), or indeterminate regarding surgical candidacy (n=174). MAIN OUTCOME MEASURES: Prevalence rates of different spinal abnormalities between the 2 cohorts, including type, severity, and number of levels of abnormalities detected on lumbar MRI. RESULTS: The total number of abnormalities did not differ between the 2 groups (P=.26). The non-surgical group exhibited more degenerative disk disease (P<.01), while surgical candidates had a higher prevalence of spinal stenosis and spondylolisthesis (P<.01). In multivariate analysis, age (adjusted odds ratio [AOR] per 10-year increase 3.33, 95% confidence interval [CI] 3.32 to 3.33), disk herniation (AOR 1.49, 95% CI 1.16 to 1.89), spinal stenosis (AOR 1.61, 95% CI 1.26 to 2.05), and spondylolisthesis (AOR 2.83, 95% CI 2.08 to 3.88) were independent predictors of surgical candidacy. CONCLUSION: These results might enable physicians without specialty training in spinal disorders to more effectively use MRI reports when deciding on referral to surgical or non-surgical specialists. In jurisdictions with long wait times for elective spinal surgery consultation, a more directed referral is one of many steps necessary to improve patient access and management.

Cervical myelopathy due to single level prolapsed disc and spondylosis: a comparative study on outcome between two groups.

This article describes a retrospective study on myelopathy, induced by monosegmental prolapsed disc and spondylosis. To assess pre- and postoperative clinical and radiological findings related to myelopathy, and factors influencing the outcome, 20 disc herniation (group A) and 11 spondylosis patients (group B) were studied. Average duration of myelopathy in groups A and B were 3 and 8.7 months, respectively. Anterior decompression and fusion were performed. Pre- and postoperative clinical and radiological findings and outcomes were assessed. Average preoperative disc heights were 85.9% of normal in group A and 72.7% in group B. Average anteroposterior canal diameter and Pavlov ratio at diseased level were 13.9 mm and 0.81 in group A, respectively, and 12.1 mm and 0.78 in group B. Five group A (25.0%) and four group B cases (36.4%) had radiculopathy. Cord compressions among 20 group A patients were median in seven and paramedian in 13. In the 11 group B patients, nine were median and two were paramedian. High signal intensity was observed in 19 group A and ten group B patients. Postoperative regression of T(2)-weighted high signal intensity in 14 group A (73.7%) and two group B patients (20.0%) was observed. Preoperative JOA scores in groups A and B were 10.3 and 12.8, respectively, which became 66.2 and 22.5 postoperatively. Neurological recovery was poorer in group B than in group A. Outcome was influenced by chronicity of myelopathy.

Cervical myelopathy accompanied with hypoplasia of the posterior arch of the atlas: case report.

STUDY DESIGN: Case reports of 2 patients having cervical myelopathy accompanied with idiopathic hypoplasia of the posterior arch of the atlas. OBJECTIVE: A standard for the sagittal inside diameter (SID) of the vertebral foramen of the atlas was determined for use in the diagnosis of hypoplasia. From the point of view of short SID, the etiologies of myelopathy in patients with this disease were analyzed. SUMMARY OF BACKGROUND DATA: There is no established definition of hypoplasia of the atlantal vertebral foramen. The mechanism underlying the occurrence of this myelopathy in adulthood is unknown. METHODS: Lateral x-ray views of the cervical vertebrae of 150 Japanese men and 150 Japanese women were investigated, and the standard values of the SID were obtained. The features of myelopathy associated with this disease were analyzed in 2 cases managed at our facility and in the literature. RESULTS: The mean standard value was 34.4 mm in women and 37.1 mm in men, showing a significant sex difference (P<0.0001). When a case with a value -2 SD below the standard was regarded as having hypoplasia, case 1 (female) showed 25 mm, that is, -3.9 SD, and case 2 (male) showed 30 mm, that is, -2.7 SD. The space available for the spinal cord of the atlas was 8.0 mm in case 1 and 9.5 mm in case 2. Both patients showed atlantoaxial subluxation on images, but atlantodental interval on flexion in cases 1 and 2 was 3.8 and 4.5 mm, respectively, showing the degree to be mild. CONCLUSIONS: In the presence of congenital hypoplasia of the posterior arch of the atlas, the spinal cord is highly susceptible to injury because congenital spinal canal stenosis is present. Therefore, the presence of even a mild mechanical compressive lesion leads to myelopathy. Acquired mild atlantoaxial subluxation was associated with this hypoplasia, and the association was considered to be the mechanism underlying the occurrence of myelopathy in adulthood.

The relationship between the cervical spinal canal diameter and the pathological changes in the cervical spine.

A congenitally narrow cervical spinal canal has been established as an important risk factor for the development of cervical spondylotic myelopathy. However, few reports have described the mechanism underlying this risk. In this study, we investigate the relationship between cervical spinal canal narrowing and pathological changes in the cervical spine using positional magnetic resonance imaging (MRI). Two hundred and ninety-five symptomatic patients underwent cervical MRI in the weight-bearing position with dynamic motion (flexion, neutral, and extension) of the cervical spine. The sagittal cervical spinal canal diameter and cervical segmental angular motion were measured and calculated. Each segment was assessed for the extent of intervertebral disc degeneration and cervical cord compression. Based on the sagittal canal diameter, the subjects were classified into three groups: A, subjects with a congenitally narrow canal, diameter of less than 13 mm; B, subjects with a normal canal, diameter of 13-15 mm; C, subjects with a wide canal, diameter of more than 15 mm. When compared with Groups A and B, the disc degeneration grades at the C3-4, C5-6, and C6-7 segments and the cervical cord compression scores at the C3-4 and C5-6 segments showed significant differences. Additionally, when compare with Groups A and C, the disc degeneration grades at all segments, except C2-3, and the cervical cord compression scores at all segments, except C2-3, showed significant differences. With respect to the cervical kinematics, few differences in the kinematics were observed between Groups B and C, however, the kinematics in Group A was different with other two groups. In Group A, the segmental mobility at the C4-5 and C6-7 segments were significantly higher than those observed in Group B, and the segmental mobility at the C3-4 segment was significantly lower than that observed in Groups B or C. We demonstrated the unique pathological and kinematic traits of cervical spine that exist in a congenitally narrow canal. We hypothesize that kinematic trait associated with a congenitally narrow canal may greatly contribute to pathological changes in the cervical spine. Our results suggest that cervical spinal canal diameter of less than 13 mm may be associated with an increased risk for development of pathological changes in cervical intervertebral discs. Subsequently, the presence of a congenitally narrow canal can expose individuals to a greater risk of developing cervical spinal stenosis.

Tetraplegia and respiratory failure following mild cervical trauma in a child with Cornelia De Lange syndrome.

Here we report a 6-year-old female patient with Cornelia de Lange syndrome who developed tetraplegia and respiratory failure after a seemingly trivial spinal trauma due to an spinal malformation that has not yet been described.

Congenital spinal lipomatous malformations: part II--Clinical presentation, operative findings, and outcome.

BACKGROUND: To report this author's experience with patients with a congenital spinal lipomatous malformation with special emphasis on variations in clinical presentation, operative findings, and outcome based on the classification scheme proposed in the first part of this two part article. METHODS: From January 1995 to July 2005, 80 patients with a congenital spinal lipomatous malformation were treated. All patients underwent routine neurological examination, plain radiographs of the spine and all but 10 patients underwent MRI. Ten patients underwent CT-myelography. Hoffman's functional grading scale was used for preoperative and postoperative clinical assessment. The operative findings, complications and outcome were assessed. FINDINGS: Age ranged from 18 days to 19 years. The female: male ratio was 3:2. The malformations were divided into two groups: Group I: Lipomas without a dural defect and, Group II: Lipomas with a dural defect. Included in Group I were: 22 patients out of which there were Caudal lipomas: 10, Filum lipomas:11 and intramedullary lipoma: 1. In Group II there were 58 patients out of which there were Dorsal lipomas: 8, Caudal lipomas with dural defect: 8, Transitional lipomas: 10, lipomyelomeningoceles:28, lipomyeloceles: 4. Most of the group I patients were >5 years of age; cutaneous markers were absent in 60%, older children more often presented with sphincter disturbances. Surgery in group I was straight forward and consisted of sectioning of the filum in filum lipomas, debulking and untethering in caudal lipomas. Duroplasty was seldom required. CSF leak was rare. No patient deteriorated following surgery and no retethering was noted during follow-up. In Group II, all patients had cutaneous markers, most were <2 years of age, 19 were asymptomatic, older children had more severe neurological deficits. Duroplasty was required in most cases. A CSF leak occurred in 12%. Two patients deteriorated temporarily following surgery. Two patients presented with retethering 4 and 8 years after initial surgery. Improvement of more than one Hoffman's functional grade occurred when surgery was done <2 years of age. CONCLUSIONS: Congenital spinal lipomatous malformations do not constitute a single homogenous entity. They can be broadly classified into two groups depending on the presence or absence of a dural defect. These two groups are different from one another embryologically, clinically, surgically and prognostically.

Congenital spinal lipomatous malformations: part I--Classification.

BACKGROUND: Congenital spinal lipomatous malformations constitute a diverse group of lesions. There is considerable confusion in the literature regarding their terminology and a proper classification is long overdue. The first part of this two part report sets out a proposed classification scheme. METHODS: On the basis of this author's experience with 80 patients with a congenital spinal lipomatous malformation treated over a 10 year period, a new classification is proposed. The proposed classification divides congenital spinal lipomatous malformations into two broad groups: 1. Lipomas without dural defect and, 2. Lipomas with dural defect. Within each group, there are several subtypes. These two broad groups differ from one another in their embryology, clinical presentation, operative findings, complications and prognosis FINDINGS: Group I consists of Lipomas without dural defect. Included in this group are: Filum lipoma, caudal lipoma without dural defect, and intramedullary lipoma. Group II consists of lipomas with dural defect. Included in this group are: dorsal lipoma, caudal lipoma with dural defect, transitional lipoma, lipomyelocele, and lipomyelomeningocele. The definitions of the various subtypes and radiological and operative findings of all these lesions are described. CONCLUSIONS: Congenital spinal lipomatous malformations constitute a wide spectrum of lesions ranging from relatively simple lipomas of the filum terminale to complex malformations. These lesions differ from one another in their embryology, clinical presentation, operative strategies, complications and prognosis. Failure to differentiate between the different forms of congenital spinal lipomatous malformations may lead to inaccurate assumptions regarding prognosis and inappropriate management. The proposed classification seeks to address these issues.

Repair of Chiari III malformation using cranioplasty and an occipital rotation flap: technical note and review of literature.

BACKGROUND: Chiari III malformation (CM3) is rare among Chiari malformations (I-IV). Its definition has been expanded to include caudal medullary displacement and hindbrain herniation into encephaloceles in lower occipital and high cervical regions. Prognosis is recorded as dismal, with respect to survival and functional outcome. METHODS: We describe the presentation, radiologic evaluation, and repair of this malformation using methyl-methacrylate cranioplasty and an occipital scalp rotation flap for closure. Outcome after surgery is addressed. RESULTS: Adequate closure of the defect and protection of underlying structures was achieved without undue stress at incision site. CONCLUSIONS: This method of closure can be considered in cases of large occipital and cervical encephaloceles with poor skin cover and added osseous anomalies around the foramen magnum.