Zimbler S

Segmental spinal dysgenesis.

Segmental spinal dysgenesis is characterized by focal agenesis or dysgenesis of the lumbar or thoracolumbar spine, with focal abnormality of the underlying spinal cord and nerve roots. Children are symptomatic at birth with lower limb deformities and neurological deficits that may be segmental. Myelography and computed tomography disclose hypoplastic or absent vertebrae and atrophic or absent neural elements adjacent to the bony deformity; the spinal column distal to the abnormality may be partially bifid, but is otherwise normal. Spinal ultrasonography was a helpful diagnostic adjunct in one patient. Surgery may be helpful in decompressing partially functioning spinal cord or nerve roots, but may exaggerate the tendency toward spinal instability. The embryology of this abnormality is not clear, but two children had other anomalies suggesting a spinal dysraphic syndrome, and its cause is probably related to a segmental maldevelopment of the neural tube.

Hindbrain Stroke in Children Caused by Extracranial Vertebral Artery Trauma

Hindbrain transient ischemic attacks (TIAs) culminating in posterior circulation stroke are described in five children. Atlanto-axial subluxation and angiographical documentation of C1 to C2 level arterial pathology are documented in one patient. Four additional patients with nearly identical clinical presentations, posterior fossa TIAs, stroke and basilar angiographical pathology are reviewed. A mechanical traumatic etiology is suggested. Unexplained transient repeated brain stem and/or cerebellar symptomatology may be due to extracranial vertebral artery stenosis or occlusion by atlanto-axial instability. After appropriate documentation, stabilization may prevent further TIAs or strokes.

Subtalar arthrodesis for stabilization of valgus hindfoot in patients with cerebral palsy

All patients with spastic cerebral palsy who underwent correction of valgus hindfoot by Grice extraarticular subtalar arthrodesis between 1971 and 1986 performed by two surgeons using an identical technique were reviewed. Twenty-nine patients (53 feet) were followed at an average of 8.9 years after operation. Traditional radiographic criteria for measurement of hindfoot alignment in skeletally mature individuals have poor reliability. Talar head uncovering is a useful and reproducible method for evaluation of hindfoot valgus in these patients. Five patients had progressive hindfoot or ankle deformity at latest follow-up. All five of these patients were spastic quadriplegics. There was no recurrence in the 17 patients who were less severely involved than the quadriplegic patients.

The Arthrogrypotic Foot Plan of Management and Results of Treatment

Fifty-one arthrogrypotic feet have been treated and followed by the Pediatric Orthopaedic Unit, Tufts New England Medical Center, (1970–1980). Forty of the 51 feet presented as equinovarus with the residual divided among metatarsus adductus, vertical tali, and calcaneo-valgus. Equinovarus deformities are the most resistant in all cases. Corrective casts are applied for at least the first 3 months of life. Surgical procedures were then initiated with any evidence of lack of progression of treatment. Varus and equinus were treated by an extensive posterior and medial release. Lateral soft tissue releases in addition to calcaneocuboid fusion or cuboid osteotomy were necessary in 24 of the 70 operations. Recurrence rate has been a problem in the simple type of posterior release including only an Achilles tendon lengthening, and posterior capsulotomy of the ankle and subtalar joint. Tal-ectomy has been carried out in four feet and appears to be one type of reasonable salvage procedure in smaller children with recurrent varus. Treatment is difficult in these patients but a plantigrade foot should be achieved in all cases.

Air embolism as a complication of hip arthrography

As an aid to correctly placing the needle tip within the hip joint during arthrography, injection of small amounts of air has been used to outline the joint space. Over a period of seven years, air embolus has been noted in three pediatric patients, twice with minor symptoms, and once with cardiac arrest.Even small amounts of air (less than 5 cc) injected accidentally intravenously may cause dangerous complications in small infants. If this technique is to be used, it is recommended that small amounts of carbon dioxide or oxygen be used instead of air.

Chondromyxoid fibroma of the ala of the sacrum presenting as a cause of lumbar pain in an adolescent

We report a case of chondromyxoid fibroma of the ala of the sacrum: its presentation, diagnosis, treatment, and resolution. Although this tumor is admittedly rare, our case demonstrates the need for careful evaluation of pack pain in an adolescent.

Effectiveness of surgical and non-surgical management of crouch gait in cerebral palsy: A systematic review

BACKGROUND Cerebral palsy (CP) is a prevalent group of neuromotor disorders caused by early injury to brain regions or pathways that control movement. Patients with CP exhibit a range of functional motor disabilities and pathologic gait patterns. Crouch gait, characterized by increased knee flexion throughout stance, is a common gait pattern in CP that increases energy costs of walking and contributes to ambulatory decline. Our aim was to perform the first systematic literature review on the effectiveness of interventions utilized to ameliorate crouch gait in CP. METHODS Comprehensive searches of five medical databases yielded 38 papers with 30 focused on orthopaedic management. RESULTS Evidence supports the use of initial hamstring lengthenings and rectus femoris transfers, where indicated, for improving objective gait measures with limited data on improving gait speed or gross motor function. In contrast, evidence argues against hamstring transfers and revision hamstring lengthening, with recent interest in more technically demanding corrective procedures. Only eight studies evaluated alternatives to surgery, specifically strength training, botulinum toxin or orthoses, with inconsistent and/or short-lived results. CONCLUSIONS Although crouch in CP is recognized clinically as a complex multi-joint, multi-planar gait disorder, this review largely failed to identify interventions beyond those which directly address sagittal plane knee motion, indicating a major knowledge gap. Quality of existing data was notably weak, with few studies properly controlled or adequately sized. Outcomes from specific procedures are confounded by multilevel surgeries. Successful longer term strategies to prevent worsening of crouch and subsequent functional decline are needed. LEVEL OF EVIDENCE Systematic review.

Metastatic Sweat Gland Carcinoma in an Adolescent: A Case Report

Sweat gland carcinomas are rare tumors that metastasize frequently. The diagnosis is seldom suspected prior to histologic examination. A long interval between onset of the disease and diagnosis is characteristic of the disorder. The benign clinical and histologic appearance of some of these tumors can obscure their full malignant potential. This report illustrates an unusual instance of sweat gland carcinoma metastatic to bone carcinoma in an adolescent. This case stresses the necessity for those involved in the care of the foot to be familiar with a condition of this type.

Osteoid Osteoma of the Cervical Spine

Osteoid osteoma is a benign tumor of bone that occurs in children and adolescents, particularly in the femur and tibia. Osteoid osteoma of the spine accounts for approximately 10% of these lesions. There frequently is a delay in diagnosis because of its difficult visual appearance. Pain in the spine occurring at night that is relieved by aspirin, or painful scoliosis, should alert the physician to the possibility of its presence. Bone scan and tomograms are essential in making the diagnosis. Complete surgical excision of the lesion is the procedure of choice for immediate, lasting pain relief.

A ten-year-old boy with cerebral palsy and femoral anteversion. How much does it hurt to break a leg?

DW, an active, well-adjusted 10-year-old boy of normal intelligence with spastic left hemiplegic cerebral palsy, had developed severe internal rotation of his left leg from femoral anteversion. His spasticity had thus far been managed conservatively, with braces and exercises. The orthopedic surgeon who had provided care for the past two years felt that continued conservative measures could not reverse or arrest the rotational deformity and that a left femoral derotational osteotomy with appropriate muscle and tendon releases was indicated. Surgery would place the leg in a more straightforward or neutral position, providing an immediate cosmetic benefit. More importantly, the surgeon believed that without corrective therapy DW would begin to exhibit a declining functional efficiency of the leg, manifested by increased energy expenditure for daily activities and increased fatigue. Furthermore, the derotational osteotomy would improve the mechanical function of the leg and delay the onset of degenerative changes of the hip and knee, which would otherwise begin to develop over the next 20 to 30 years. In addition to causing perioperative pain, surgery would require three to six weeks of hospitalization and an additional three to five months of rehabilitation before the child could return to his current level of function. If the operation was deferred for more than a year, the surgeon