Initial experience with combined endoscopic third ventriculostomy and choroid plexus cauterization for post-hemorrhagic hydrocephalus of prematurity: the importance of prepontine cistern status and the predictive value of FIESTA MRI imaging.

PURPOSE: Post-hemorrhagic hydrocephalus of prematurity (PHHP) is among the most common causes of infant hydrocephalus in developed nations. This population has a high incidence of shunt failure, infection, and slit ventricle syndrome. Although effective for other etiologies of infant hydrocephalus, the efficacy of combined endoscopic third ventriculostomy and choroid plexus cauterization (ETV/CPC) in PHHP has not been investigated. This pilot study reports the initial experience. METHODS: Ten patients (four grade III and six grade IV intraventricular hemorrhage) requiring definitive treatment for PHHP underwent ETV/CPC within 6 months of birth. Seven had a prior ventriculo-subgaleal shunt. Mean age at birth was -12.8 weeks, or 25.2 weeks gestation (24-28 weeks), and at surgery was -1.6 weeks (-11 to +11 weeks). Mean weight at surgery was 3.3 (1.0-5.5 kg). Each patient had preoperative magnetic resonance imaging (MRI) with fast imaging employing steady-state acquisition (FIESTA). RESULTS: Four of ten (40%) required no further operations related to hydrocephalus (mean follow-up, 29.7 months). Six required another procedure (five ultimately shunted). Prepontine cistern status correlated with outcome (p = 0.033). Procedures in all infants with unobstructed cisterns were successful but failed in six of seven with cisternal obstruction, with the one success having an alternative lamina terminalis endoscopic third ventriculostomy. Preoperative MRI FIESTA images correlated well with intraoperative assessment of the cistern. CONCLUSIONS: Results from this small homogenous cohort suggest cistern status is an important determinant of outcome. FIESTA imaging correlated with endoscopic observation. Preliminary analysis suggests ETV/CPC as an effective treatment for PHHP, but only when the cistern is unscarred. This information should guide patient selection for future study protocols.

Limb length discrepancy and congenital limb anomalies in fibular hemimelia.

The aim of this study was to determine the prevalence of ipsilateral congenital musculoskeletal anomalies associated with fibular hemimelia. We also attempted to determine the corelation between the eventual limb length discrepancy at maturity and these associated anomalies, as well as the Achterman-Kalamchi class of these patients. The records and roentgenograms of 45 patients with fibular hemimelia were reviewed retrospectively. All patients were classified into three groups (types I-A, I-B, and II) according to the Achterman-Kalamchi system. The prevalence of congenital limb anomalies, lower extremity discrepancy percentage, and ultimate limb length discrepancy at skeletal maturity were evaluated. There was no significant statistical association between the number of congenital limb anomalies and severity of the limb length discrepancy, but patients included in the mildly affected group (type I-A) had a higher average prevalence of congenital limb anomalies than those classified as types I-B and II. There was a substantial correlation between congenital short femur as well as the Achterman-Kalamchi classification system and the predicted limb length discrepancy. A similar percentage of limb length discrepancy in types I-B and II was seen. In conclusion, the numbers of associated congenital limb anomalies are not predictive of the eventual limb length discrepancy. Presence of a congenital short femur and ball and socket ankle are predictive of a higher limb length discrepancy. Achterman-Kalamchi types I-B and II show a similar percentage of limb length discrepancy and this is significantly higher than that seen in type I-A.

The role of the dynamic pedobarograph in assessing treatment of cavovarus feet in children with Charcot-Marie-Tooth disease.

We used the dynamic pedobarographs to study pressure distribution patterns in the foot after surgical correction of cavovarus feet. We also assessed the influence of ankle power generation on pressure distribution in these feet. Nine children (14 feet) diagnosed with Charcot-Marie-Tooth disease who had undergone operative treatment with a combination of osteotomies and muscle transfers were the subjects of this study. Preoperative and postoperative pedobarographic measurements recorded included pressure over the medial forefoot, lateral forefoot, medial midfoot (MMF), lateral midfoot (LMF), and heel segments. In 6 patients (9 feet) who had a complete gait analysis, the power generation of the ankle was also obtained both preoperatively and postoperatively. Lateral radiographic measurements included the (1) talus-first metatarsal angle, (2) calcaneus-first metatarsal angle, and (3) calcaneal pitch. The radiographs showed significant improvements in all 3 angles. Increased LMF and decreased forefoot pressures were seen on preoperative pedobarographic measures. Postoperatively, improvement in pressure at the LMF was seen. When postoperative measurements were compared with the normal values, only the LMF was similar; the other 4 segments showed decreased forefoot and MMF pressures and increased heel pressures (P = 0.000 for the lateral forefoot and MMF; 0.040 for the heel and medial forefoot). The heel pressures displayed an inverse relationship to ankle power generation. The amount of correction achieved radiographically did not correlate with pedobarographic measurements. The increased heel pressure that was noted was not addressed by treatment. Normalization of pressure patterns should be the goal in treating children with symptomatic cavovarus feet. Although the foot deformity is corrected completely in neuromuscular disorders, pressure distribution was not normalized, and therefore, symptoms might persist. Both patients and parents should be informed about this possible problem before surgical intervention.

Knee arthritis in congenital short femur after Wagner lengthening.

Knee anomalies are common in patients with congenital short femurs who require lengthening to correct limb-length discrepancies. We retrospectively reviewed the incidence of knee arthritis and the factors influencing its occurrence after femoral lengthening using the Wagner method. Twenty-three patients with congenital short femurs treated with the Wagner method were followed up until skeletal maturity (minimum, 5 years postoperatively). The mean age of the patients at lengthening was 10.8 years (range, 8.4-14.5 years). The mean leg-length discrepancy at the time of surgery was 9.7 cm (femur, 7.6 +/- 3.7 cm; tibia, 2.1 +/- 1.8 cm). Femoral lengthening (mean, 7.9 cm) was performed in 17 patients. Femoral lengthening and tibial lengthening were performed simultaneously in six patents (mean, 11.8 cm). The mean age of the patients at the last followup was 16.8 years (range, 14-20.3 years). Eighteen patients had arthritis at followup. Nine patients had severe arthritis develop, seven of whom had knee instability preoperatively and temporary subluxation during the lengthening procedure. Seventy-eight percent of patients had arthritis develop in the knee after lower-limb lengthening using the Wagner method for congenital short femurs. Patients who had an unstable knee before surgery had temporary knee subluxation develop during the lengthening procedure, and patients who had simultaneous lengthening of the femur and tibia had a high association with degenerative arthritis changes in the knee.

Residual deformities following successful initial bone union in congenital pseudoarthrosis of the tibia.

The pathology of congenital pseudoarthrosis of tibia is an enigma and the treatment is challenging. Despite achieving union of the pseudoarthrosis, these patients may have compromised function secondary to residual deformities. The purpose of this study is to analyze the prevalence of these deformities, the morbidity caused by them, and the methods to overcome the problems. Sixteen patients who had a successful union were retrospectively reviewed. Clinically, the following parameters were assessed: pain, joint stiffness, and limb length discrepancy (LLD). Anteroposterior and lateral lower extremity radiographs were performed to analyze (1) union of the tibia and fibula, (2) deformity of the tibia, (3) degree of ankle valgus, (4) degree of calcaneus of the os calcis, (5) LLD, and (4) refracture. The average follow-up was 16 years from the first and 8 years from the last surgical procedure. Residual deformities included valgus of tibia (average 11.4 degrees) and procurvatum (average 19.4 degrees) deformities of the tibia or valgus deformity of the ankle (average 21.3 degrees) and calcaneus of the os calcis (average 46 degrees). There were 9 refractures in 8 patients. Nine patients had an LLD (average 3.5 cm). The study demonstrates that careful follow-up of these patients is necessary and residual problems are to be treated as they occur.

Coxa vara: a novel measurement technique in skeletal dysplasias.

Coxa vara can be a progressive deformity in children with skeletal dysplasia. Preoperative anteroposterior pelvic radiographs of 30 children with spondyloepiphyseal dysplasia congenita and spondyloepimetaphyseal dysplasia were used to test the reliability of a new radiographic measure of coxa vara, the Hilgenreiner-trochanteric angle. An additional 10 patients (20 hips) with coxa vara deformities needing valgus-producing proximal femoral osteotomies also were reviewed. Interobserver reliability with plain radiographs was 0.929 for the left side and 0.914 for the right side using interclass correlation coefficients. Intraobserver reliability also was high, with an interclass correlation coefficient of 0.875. Twelve hips corrected by osteotomy had adequate ossification to measure the Hilgenreiner-epiphyseal angle, head-shaft angle, and Hilgenreiner-trochanteric angle. Only one of these hips had a recurrence. The results were good in all of the other ossified hips. Eight hips had limited ossification; only two of these hips maintained acceptable alignment. Six hips had less postoperative correction and progressive deformity at the final followup. We present a novel measurement technique to determine the degree of coxa vara deformity in children with delayed or absent ossification of the capital femoral epiphysis.

Arthrofibrosis involving the middle facet of the talocalcaneal joint in children and adolescents.

BACKGROUND: Pain over the anterolateral aspect of the ankle in a patient with a history of repeated ankle sprains and with restricted subtalar movement may be associated with a tarsal coalition. Nineteen patients presented with such a history, but conventional imaging did not reveal a cartilaginous or osseous coalition. Since symptoms persisted despite nonoperative treatment, the middle facet was explored surgically. The purpose of this study was to discuss the operative findings and to report the results of treatment. METHODS: Nineteen patients (twenty-three feet) with pain over the anterolateral aspect of the ankle or a history of repeated ankle sprains had restricted subtalar joint motion and inconclusive findings on diagnostic imaging, except for bone-scanning. Their ages ranged from 9.1 to 18.5 years. The middle facet of the subtalar joint was explored surgically through a 3 to 4-cm-long incision centered over the sustentaculum tali. The results at a mean of 5.8 years were classified as good, fair, or poor on the basis of pain, talocalcaneal joint motion, and shoe wear. RESULTS: Routine radiographs, computed tomography, and magnetic resonance imaging revealed no major abnormality, whereas technetium-99m bone scintigraphy consistently showed slightly increased isotope uptake in the middle facet. Surgical removal of a hypervascular and thickened capsule and synovium in the area of the middle facet of the subtalar joint decreased pain and improved subtalar motion. The final result was good in seventeen patients (twenty feet) and fair in two patients (three feet). There were no poor results. CONCLUSIONS: A diagnosis of inflammatory arthrofibrosis should be considered when a patient with a painful rigid flatfoot has normal findings on radiographs and hematological studies but increased isotope uptake in the middle facet of the talocalcaneal joint on bone scintigraphy. Excision of the hypervascular capsule and synovium from this area can result in resolution of the symptoms. LEVEL OF EVIDENCE: Therapeutic Level IV.

Congenital vertical talus in arthrogryposis and other contractural syndromes.

No practical classification system exists to identify which patients may have successful outcome following treatment of congenital vertical talus in arthrogryposis. We classified 229 patients into five distinct groups: Group I had amyoplasia or so-called classic arthrogryposis, Group II had distal arthrogryposis, Group III had a specific syndrome as a diagnosis, Group IV had severe systemic or neurologic involvement, and Group V had unclassifiable contracture syndromes. No patient with amyoplasia (Group I) had congenital vertical talus. The congenital vertical talus seen in distal arthrogryposis (Group II) was milder than that seen in Groups III or IV and feet in this group responded well to early one-stage surgical correction. Congenital vertical talus that occurred in association with a generalized syndrome or with extensive systemic and neurologic involvement (Groups III and IV) was severe and refractory to treatment. Most children in these groups were unable to walk; therefore, the goal of treatment should be to achieve a pain-free foot to allow fitting of normal shoes. Most children in Group V were able to walk and responded well to operative correction; they should be treated before walking age.

Correction of cavovarus foot deformity in Charcot-Marie-Tooth disease.

Operative correction of cavovarus foot deformity in Charcot-Marie-Tooth disease (CMT) is challenging. This progressive peripheral sensory and motor neuropathy commonly involves the forefoot, midfoot, hindfoot, and toes. The authors present a new imaging technique that allows the surgeon to assess the flexibility of the hindfoot in patients with CMT to determine the best operative procedure to correct the deformity. Twenty-five patients (41 feet) with CMT and cavovarus foot deformity were evaluated and a new radiographic technique was studied in some of these patients to determine the usefulness of this imaging modality. The authors believe that this new imaging method will aid in determining the optimal operation for correcting this complex deformity.

The duPont kyphosis brace for the treatment of adolescent Scheuermann kyphosis.

The purpose of this study was to evaluate the effectiveness of a newly designed brace in the treatment of adolescent Scheuermann thoracic kyphosis. Twenty-two children who met the roentgenographic criteria of Scheuermann kyphosis and were compliant with treatment were followed until skeletal maturity. Sixteen patients (73%) showed nonprogression of their kyphosis (nine patients demonstrated an improvement, seven patients remained unchanged), and had a mean improvement of 9 degrees (64 degrees to 55 degrees). Six patients (27%) demonstrated progression of the kyphosis and had a mean increase in their kyphosis of 9 degrees (59 degrees to 68 degrees). One patient underwent posterior spinal fusion for progressive thoracic kyphosis despite bracing. It was recommended that this brace be worn until skeletal maturity; in this study this time period was determined to be at least 16 months to induce improvement or halt progression of this disease. Flexible curves are a positive predictor of a successful outcome of bracing with the kyphosis brace. These results are comparable to previous reports in the literature describing the effectiveness of the modified Milwaukee brace in the treatment of Scheuermann thoracic kyphosis prior to skeletal maturity, and the kyphosis brace has the advantage of concealability under normal attire.

Transphyseal fracture-dislocation of the femoral neck: a case report and review of the literature.

We describe a case of transphyseal hip fracture-dislocation in a 7.5-year-old patient who was treated initially by open reduction and internal fixation. Soon after the injury, the femoral head developed avascular necrosis. The treatment was focused on maintaining adequate hip range of motion and providing femoral head containment with a combined subtrochanteric femoral osteotomy and shelf acetabuloplasty. The patient's young age and good hip remodeling potential contributed to the favorable clinical outcome 3 years after the injury. The long-term prognosis remains guarded, however.

Type IV congenital deficiency of the tibia.

Type IV congenital deficiency of the tibia is associated with two major problems: the equinovarus position of the foot with the talus incarcerated in the distal tibiofibular mortise and the limb length inequality due to the shortened dysmorphic tibia. The purpose of this study was to formulate guidelines for the management of this rare but complex condition. Eleven patients treated for type IV congenital deficiency of the tibia between 1963 and 2000 were evaluated. The treatment consisted of a Boyd or Syme amputation of the foot in seven patients, ankle reconstruction surgery in three patients, and heel cord lengthening for correction of the equinovarus deformity of the foot in one patient. Even though seven patients treated by Boyd or Syme amputation were community ambulators with a prosthesis, parents and patients preferred ankle reconstruction. All three patients treated by ankle reconstruction surgery presented with a stable ankle, a plantigrade foot, and independent ambulation. Reconstruction of the ankle mortise should be the treatment of choice for type IV congenital deficiency of the tibia.

Bilateral Köhler's disease in identical twins.

Köhler's disease is an uncommon idiopathic osteochondrosis of the tarsal navicular bone. The diagnosis is clinical and the natural history is benign. The treatment is symptomatic, including shoe supports when the symptoms are mild and initial cast immobilization for at least 8 weeks when the symptoms are more intense. The final clinical outcome is always favorable. This article reviews the development of bilateral Köhler's disease in identical twin brothers with simultaneous onset and parallel clinical course. Bilateral involvement in identical twins, to the authors' knowledge, has not been reported in the literature. Although this is a preliminary finding, it can imply that an unknown genetic predilection may exist and relate to the appearance of this rare disease.

A neurovascular complication after scapulothoracic arthrodesis.

Facioscapulohumeral muscular dystrophy is a progressive disorder characterized by weakness in the muscles of the face, shoulder girdle and upper limbs, and variable lower extremity weakness. The muscles that stabilize the scapula are significantly weak, although the deltoid usually is preserved. With attempted shoulder abduction, the unstable scapula protrudes, elevates, and internally rotates. Scapulothoracic arthrodesis stabilizes the scapula and improves active range of motion and function of the shoulder. Appropriate scapular positioning on the chest wall has been described previously. The current authors review a neurovascular complication after scapulothoracic arthrodesis in which the scapula was positioned as described in the literature. Immediate repositioning resulted in an excellent long-term outcome. Previous recommendations as to scapular position must be taken simply as guidelines. Intraoperative monitoring of neurovascular function in the upper extremity should prevent this complication.

Macrodactyly of the foot.

BACKGROUND: The purpose of this study was to focus on the problems associated with macrodactyly of the foot and to formulate guidelines for optimum treatment. METHODS: Seventeen feet (fifteen patients) with macrodactyly formed the basis of this retrospective review. The feet were classified into one of two groups, depending on whether the macrodactyly involved only the lesser toes (group A) or involved the great toe with or without involvement of the lesser toes (group B). Toe amputation or ray resection was usually done to reduce the length and width of the foot in group A, whereas the length of the first ray was reduced by epiphysiodesis or amputation of the phalanx in four of the five feet in group B. In both groups, soft-tissue debulking was an integral part of the treatment. The severity of the macrodactyly and the effect of treatment were documented radiographically by measurement of the so-called metatarsal spread angle. At the latest follow-up evaluation, each foot was graded with regard to pain and shoe wear. RESULTS: Toe amputation was performed in six of the twelve feet in group A and toe shortening was performed in two, but only three of those procedures had a good result. Ray resection was performed in five feet (as an initial or secondary procedure) in Group A, and all had a good result. The mean reduction of the metatarsal spread angle was 10.0 degrees following resection of a single ray in Group A. In Group B, four of the five feet were rated as having only a fair result because shortening alone did not effectively reduce the size of the great toe. The macrodactyly of the great toe was not treated in the fifth foot, which also had a fair result. CONCLUSIONS: Toe amputation, which is cosmetically unappealing, is not effective for treating macrodactyly of the lesser toes and does not address the enlargement of the forefoot. Ray resection results in the best cosmetic and functional outcomes in feet with involvement of the lesser toes. When the great toe is involved, the result is often only fair, and repeated soft-tissue debulking may be necessary.

Displaced fractures of the lateral humeral condyle: criteria for implant removal.

Displaced fractures of the lateral humeral condyle have been successfully treated with closed or open reduction and pinning. However, there is no consensus as to when the implants should be removed, with the recommended time ranging from 3 to 8 weeks. The purpose of this study was to assess the results after treatment of displaced lateral condyle fractures of the humerus in children and formulate guidelines for removing the implants. Fifty-five patients with displaced fractures were included in this study. A modified Hardacre functional rating system was used to grade the final clinical result. In 50 patients the fracture was clinically and radiographically healed at 6 weeks, in 4 patients at 5 weeks, and in only 1 patient at 4 weeks. The implants should not be removed until healing can be demonstrated radiographically. This time averaged 6 or more weeks in most of the patients in this study.