1/64. Unilateral tibial hemimelia with leg length inequality and varus foot: external fixator treatment.A 15-year-old girl with type II unilateral hemimelia presented with a 13.5-cm shortening of her right leg, absence of the distal half of the tibia, tibiofibular synostosis, and medial dislocation of a cavus and varus foot. She was treated by means of an external fixator. The shortening was significantly corrected, and realignment of the foot with the limb was achieved. An arthrodesis of the talus and lower end of the fibula was carried out operatively and stabilized with an external fixator. In the same surgical procedure, we performed an osteotomy of the tibiofibular synostosis, and progressive distraction was done with another external fixator. We emphasize the advantages of progressive distraction for the correction of congenital deformities of the limbs.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
2/64. Confirmation of arterial deficiencies in a limb with necrosis following clubfoot surgery.This study describes postoperative necrosis of the hallux and first ray in a child with clubfoot. Arteriography performed on this child's lower limbs demonstrated, in the operated leg, hypoplasia of both the anterior and posterior tibial arteries and failure of the dorsalis pedis artery to traverse the tarsus and complete the deep plantar arch. Previously, congenital vascular deficiency was suggested to predispose such operated limbs to necrosis. These findings confirm the association between vascular deficiency and necrosis. In this present study, the metabolic demands of wound healing were sufficient in a limb with vascular deficiency to cause localized distal hypoperfusion leading to cyanosis and necrosis of the hallux and medial foot.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
3/64. Characterization of a small supernumerary ring marker derived from chromosome 2 by forward and reverse chromosome painting.A small ring-shaped supernumerary marker chromosome (SMC) was detected in 50% of metaphase cells in an 18-month-old boy with mental retardation and multiple congenital anomalies. Conventional cytogenetic methods had failed to identify the origin of the marker. When the patient was age 11.5 years, we defined the origin of the SMC by fluorescence in situ hybridization using a battery of centromere-specific dna probes. The marker was positive with the probe for locus D2Z. More detailed characterization was achieved by using chromosome 2 arm-specific and marker-specific DNA libraries, which were constructed by microdissection of the two arms chromosome 2 and SMC with subsequent amplification of the chromosomal material by a degenerate oligonucleotide-primed polymerase chain reaction (DOP-PCR). The marker was identified as r(2)(p11.2-->q14.1). The propositus had dolichocephaly, coarse hair, low-set ears, exophthalmos, epicanthal folds, strabismus, depressed nasal bridge, high-arched palate, excess of skin on the neck, tapered fingers with mild clinodactyly, talipes varus on the right, inguinal hernia, hypogenitalism, muscular hypotonia, and mental retardation. This is the first case of SMC derived from chromosome 2 that was characterized by forward and reverse chromosome painting.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
4/64. Laurin-Sandrow syndrome--a surgical challenge.We describe the sixth reported case of Laurin-Sandrow syndrome. This spectrum of congenital abnormalities includes complex syndactyly, often associated with ulnar duplication, mirror feet and columella deformities. We discuss Laurin-Sandrow syndrome and report on its surgical management.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
5/64. Congenital bowing of the long bones associated with camptodactyly, talipes equinovarus and agenesis of the corpus callosum.We report a baby with congenital bowing of the long bones, camptodactyly, talipes equinovarus and radiological features resembling both Stuve-Wiedemann syndrome and Schwartz-Jampel syndrome type 2. The baby had, in addition, agenesis of the corpus callosum. This feature has not been reported in either of these syndromes. It is possible that this baby has a previously undescribed syndrome.- - - - - - - - - - ranking = 1keywords = congenita (Clic here for more details about this article) |
6/64. Congenital fiber-type disproportion presenting antenatally with clubfoot and hydramnios.This is a case of congenital fiber-type disproportion that presented prenatally with bilateral clubfoot, hydramnios, and reduced fetal movements. Although prognosis is generally good for this condition, the neonate presenting at birth may have a more severe form of congenital fiber-type disproportion with a high rate of mortality.- - - - - - - - - - ranking = 2keywords = congenita (Clic here for more details about this article) |
7/64. The natural history and longitudinal study of the surgically corrected clubfoot.Surgical treatment for clubfoot has been largely directed at finding the best one-stage operation for the resistant clubfoot. Eighteen patients with 27 clubfeet (average follow-up 11 years since first surgery; range, 3.5-24 years) were reviewed. More than one clubfoot operation was required in 56% of cases. Forty-six percent were corrected after one surgery; 33% required a second surgery and 14% required a third operation. One patient with particularly severe feet required a fourth operation on each foot. The mean age at the time of surgery was 1.26 years, 5.12 years, and 8 years for the first, second, and third operations, respectively. The first operation consisted of a soft-tissue release. The second and third operations consisted of more extensive soft-tissue release and various rearfoot and forefoot procedures. Radiographic values revealed an AP talocalcaneal angle of 18 degrees, AP talo-first metatarsal angle of 6 degrees, lateral talocalcaneal angle of 29.6 degrees, lateral talo-first metatarsal angle of 15 degrees, and calcaneo-first metatarsal angle of 143 degrees. At follow-up all patients had adequate function as determined by personal interview and clinical examination. We conclude that correction of resistant congenital clubfoot often requires more than one surgery, not because of a "failed first operation," but due to dynamic muscle imbalances that may not be fully recognized in infancy and early childhood. Thus, the need for a second operation should not be perceived as a failure of the first, but as part of the natural history of congenital clubfoot.- - - - - - - - - - ranking = 2keywords = congenita (Clic here for more details about this article) |
8/64. Accessory soleus muscle as a cause of resistance to correction in congenital club foot: a case report.A 14-month-old female with bilateral clubfeet was initially treated by serial casting and percutaneous tenotomy of the achilles tendon, bilaterally. Both clubfeet subsequently underwent surgical treatment with a posteromedial release through a Cincinnati incision. At surgery on one clubfoot, an accessory Soleus muscle was found anterior to the achilles tendon with a distinct insertion on the upper surface of calcaneus, anterior and medial to the insertion of achilles tendon. This accessory Soleus muscle may have been the cause of resistance to correction in this congenital clubfoot.- - - - - - - - - - ranking = 5keywords = congenita (Clic here for more details about this article) |
9/64. A 30 year functional follow-up of a neglected congenital clubfoot in an adult: a case report.Neglected congenital clubfoot in adults has been described in literature, but is not common to see adult patients with this orthopaedic disorder in developed countries with 30 years of follow-up. We report an asymptomatic case of neglected congenital clubfoot in an adult, who is incidentally seeking treatment for her 18-month-old son with congenital clubfoot. Although the cosmetic appearance is unacceptable, this mother remarkably has no functional limitations and for this reason she refuses any surgical treatment for her foot. To our knowledge, this is the first report of an untreated congenital clubfoot with 30 years follow-up.- - - - - - - - - - ranking = 8keywords = congenita (Clic here for more details about this article) |
10/64. magnetic resonance imaging study of the congenital clubfoot treated with the Ponseti method.Little information exists about the degree of efficacy of the several nonoperative treatments, such as manipulation and casting, used in correcting the pathology of the virgin clubfoot deformity. The steps in the correction of the displacements and anomalies of the skeletal components have never been visualized. The method reported to have the best long-term results is that of Ponseti. A magnetic resonance imaging protocol was devised to image the described chondroosseous abnormalities of the virgin clubfoot deformity and to illustrate the changes that occur with the Ponseti method of treatment. Scans were performed at the beginning of, in the middle of, and at the end of treatment. Images obtained with this protocol largely agree with postmortem studies of clubfeet. All of the major chondroosseous pathology could be visualized in vivo. With Ponseti treatment, all the abnormalities seen on the initial scans either improved markedly or corrected completely. Treatment resulted in correction not only of the abnormal relationships of the tarsal bones, but also of the abnormal shapes of the individual tarsal osteochondral anlages, probably because of the changes in growth resulting from the changes in mechanical loading of fast-growing tissues.- - - - - - - - - - ranking = 4keywords = congenita (Clic here for more details about this article) |
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