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Split cord malformation with partial eventration of the diaphragm. Case report.

The authors describe the case of a 3-year-old girl who presented with a dorsal split cord malformation (SCM) and was found to have eventration of the diaphragm. Although the child did not undergo surgery for eventration, its presence suggests a need for careful preoperative planning and clinical evaluation to rule out or confirm the anomalies associated with spinal dysraphism or SCM.

Child, Preschool↗

Retrograde intraventricular hemorrhage caused by a traumatic sacral pseudomeningocele in the presence of spina bifida occulta. Case report.

The authors present the case of a large, posttraumatic sacral pseudomeningocele in the presence of spina bifida occulta. A pseudomeningocele in the sacral region is associated with trauma and with Marfan syndrome, but only one occurrence has been reported in association with spinal dysraphism. Trauma resulted in bleeding into the pseudomeningocele and retrograde passage of blood and fat into the ventricles. An oculomotor nerve palsy subsequently developed in the patient. The authors suspected a subarachnoid hemorrhage caused by a posterior communicating artery aneurysm, although this hypothesis was refuted on further investigation. The pseudomeningocele was drained by direct exposure of the neck and opening of the sac. Postoperatively, communicating hydrocephalus developed and the patient underwent ventriculoperitoneal shunt placement, resulting in resolution of the cranial nerve palsies. This first report of intradural bleeding from direct trauma to a pseudomeningocele illustrates the rare phenomenon of retrograde passage of blood from the sacral region to the brain. It also illustrates a possible but unlikely differential diagnosis of intraventricular blood and fat.

Cerebral Hemorrhage↗

Cavus foot deformity in children.

A cavus deformity of the foot is easily recognizable, but appropriate neurologic assessment can help to determine the etiology. Cavovarus, the most frequent type of cavus foot, presents with an elevated medial longitudinal arch, first ray plantarflexion, and, if rigid, a fixed heel varus. Common causes include progressive motor sensory conditions, typically Charcot-Marie-Tooth disease, and nonprogressive conditions such as cerebral palsy and poliomyelitis. A calcaneocavus foot may be seen in poliomyelitis, spinal dysraphism, and peripheral neuropathy. Initially, the cavus deformity is flexible, but if left untreated, it becomes a fixed bony deformity. Physical examination should include the cavovarus block test, which assesses flexibility of the hindfoot deformity and can direct surgical treatment. Standing radiographs of the feet and spine, magnetic resonance imaging, and electrodiagnostic studies may be useful. Management goals are to obtain a plantigrade, mobile, pain-free, stable, motor-balanced foot. Surgical options include soft-tissue and plantar fascia releases for a flexible deformity, osteotomy for a fixed deformity, and tendon transfers to restore muscle balance. Triple arthrodesis has poor long-term results in patients with progressive deformity and sensory impairment.

Child↗

What does 2-dimensional imaging add to 3- and 4-dimensional obstetric ultrasonography?

OBJECTIVE: The purpose of this study was to determine whether 2-dimensional (2D) ultrasonography adds diagnostic information to that provided by the examination of 3-dimensional/4-dimensional (3D/4D) volume data sets alone. METHODS: Ninety-nine fetuses were examined by 3D/4D volume ultrasonography. Volume data sets were evaluated by a blinded independent examiner who, after establishing an initial diagnostic impression by 3D/4D ultrasonography, performed a 2D ultrasonographic examination. The frequency of agreement and diagnostic accuracy of each modality to detect congenital anomalies were calculated and compared. RESULTS: Fifty-four fetuses with no abnormalities and 45 fetuses with 82 anomalies diagnosed by 2D ultrasonography were examined. Agreement between 3D/4D and 2D ultrasonography occurred for 90.4% of the findings (123/136; intraclass correlation coefficient, 0.834; 95% confidence interval, 0.774-0.879). Six anomalies were missed by 3D/4D ultrasonography when compared to 2D ultrasonography (ventricular septal defect [n = 2], interrupted inferior vena cava with azygous continuation [n = 1], tetralogy of Fallot [n = 1], horseshoe kidney [n = 1], and cystic adenomatoid malformation [n = 1]). There were 2 discordant diagnoses: transposition of the great arteries diagnosed as a double-outlet right ventricle and pulmonary atresia misinterpreted as tricuspid atresia on 3D/4D ultrasonography. One case of occult spinal dysraphism was suspected on 3D ultrasonography but not confirmed by 2D ultrasonography. When compared to diagnoses performed after delivery (n = 106), the sensitivity and specificity of 3D/4D ultrasonography (92.2% [47/51] and 76.4% [42/55], respectively) and 2D ultrasonography (96.1% [49/51] and 72.7% [40/55]) were not significantly different (P = .233). CONCLUSIONS: Information provided by 2D ultrasonography is consistent, in most cases, with information provided by the examination of 3D/4D volume data sets alone.

Female↗

MR imaging of congenital anomalies of the pediatric spine.

Congenital anomalies of the pediatric spine are ideally evaluated with MR imaging. The wide spectrum of pathology in this category of disease is dramatically displaced for both diagnosis and treatment planning for the neurosurgeon. An understanding of embryology and knowledge of the clinical presentation combined with the MR imaging findings allows the radiologist to play a key role in the evaluation of these complex anomalies.

Child↗

The management of infants with scoliosis.

This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis.

Braces↗

[Syringohydromyelia--coexistence or complication following removal of meningioma of forth ventricle].

Numerous theories have been presented to explain the pathogenesis of syringohydromyelia. Among these is Gardner's hydrodynamic theory with later modifications. Syrinx is usually located in cervical cord and is often associated with Arnold-Chiari, Dandy-Walker malformation or other anomalies of cranio-cervical region. Other theories suggest that syringohydromyelia may be secondary to arachnoiditis, neoplasms or spinal cord trauma. The distal location of the syrinx is very often associated with occult spinal dysraphic lesion. The authors present the case of 28 years old woman operated on meningioma of fourth ventricle. One and a half year later syrinx on Th11-Th12 and coexistent arachnoiditis were recognized. They stress diagnostic and therapeutic difficulties in this case and try to explain of etiology of syringohydromyelia.

Adult↗

[Imaging of the painful cervical spine].

Neck pain can occur in several circumstances: traumatic, spontaneous, associated or not with motion, with or without head or upper limb irradiations. Each case requires appropriate clinical examination and radiographs. CT and MRI can be used to obtain additional information. Myelography and arteriography are exceptionally used. Cervical discography and facet joint arthrography are used therapeutically. After a brief anatomical review, normal and pathological patterns will be reviewed using radiographs. Each circumstance is studied: traumatic, degenerative, inflammatory and tumoral. It is emphasized that discogenic cervico-brachial neuralgia usually has a favorable spontaneous outcome. A special chapter is dedicated to calcifying and ossifying diseases of the cervical spine. Cervico-occipital neuralgia is also discussed.

Adolescent↗

Ischiopubic hypoplasia: a rare constituent of congenital syndromes.

Hypoplasia of the ischiopubic region is described in four patients. An adolescent was referred for spinal dysraphism and scoliosis, another one for bilateral aplasia of the patella and scoliosis, and finally two children were treated for congenital dislocation of the hip. The osseous malformation of the ischiopubic bones was not associated with any other intrapelvic disorders, and remained unchanged until skeletal maturity. The appearance of this extremely rare anomaly is usually reported as a syndromic constituent of a limited number of congenital malformation syndromes that can be widened to include congenital dislocation of the hip.

Bone Diseases↗

[Vascular tumors as syndromic indicators].

Haemangiomas can be indicators or clue signs for serious syndromes. Although less well known than those related to vascular malformations, there are some syndromes of important diagnostic value that are associated with haemangomias. Early recognition of problematic haemangiomas, together with a prompt intervention, may help to minimise their future morbidity. Neonatal haemangiomas warrant a special follow-up, since their growth pattern might be unpredictable at such an early age. Several clinical presentations are relevant because of their risk of syndromic association: cervicofacial haemangiomas, especially the extensive ones, may be markers for severe dysmorphic conditions like the PHACE(S) syndrome. Those distributed in the beard area are occasionally associated with haemangiomas of the airway. Lumbosacral haemangiomas usually hide an underlying spinal dysraphism or anorectal and urogenital anomalies. Multiple cutaneous haemangiomas may be a sign of visceral haemangiomatosis, most often hepatic, which becomes complicated by cardiac insufficiency or thyroid disease. Finally, there are two vascular neoplasms of rapid and invasive growth - kaposiform haemangioendothelioma and angioblastoma or tufted angioma - which, unlike infantile haemangioma, are markers for the Kasabach-Merritt syndrome.

Hemangioma↗

Gains of cardiorespiratory fitness with arm-crank training in spinally disabled men.

Cardiorespiratory responses to four patterns of arm-crank training (thrice weekly sessions at 50 or 70% of peak oxygen intake, 20 or 40 min per session) were examined over 8, 16, and 24 weeks in 24 initially inactive subjects with paraplegia. Training was associated with a significant increment of the peak oxygen intake during arm-crank tests except in control subjects and those combining a low intensity (50% of peak) with short-duration training (20-min sessions). There were associated increases in cardiac stroke volume, as assessed by a carbon dioxide rebreathing technique during submaximal exercise. It is suggested that the performance of inactive wheelchair users is limited by a pooling of blood in paralysed regions, with a reduction of cardiac preloading; nevertheless, substantial gains of performance are possible through prolonged arm-crank exercise of moderate intensity.

Adolescent↗