1/8. Resection of talocalcaneal middle facet coalition. Interposition with a tensor fascia lata allograft: a case report.Tensor fascia lata is utilized in the management of complex soft-tissue injuries and defects, but has not been described in the literature in the use of tissue interposition with resection of talocalcaneal middle facet coalitions. This article is a case presentation of a resection of a middle facet coalition with interposition of an allograft of tensor fascia lata. At 14 months postoperative follow-up, range of motion of the subtalar joint was noted to be 20 degrees, and without pain or crepitus. There was no radiographic evidence of degenerative changes in Chopart's joint. The patient returned to all routine and sports activities without pain. He was satisfied with the outcome of the procedure.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
2/8. Complete bony fusion of the mandible to the zygomatic complex and maxillary tuberosity: case report and review.Congenital craniofacial disorders represent approximately 20% of all birth defects. One of these disorders is syngnathia, of which only 24 cases have been reported since 1936. Twenty cases involved fusion of the alveolar processes of the maxilla and mandible. Only four are similar to the presented case, which includes bony fusion of the ascending ramus of the mandible to the zygomatic complex and the posterior part of the maxilla. This case report will present details from the 23rd week of gestation to 8 months of age when the infant underwent the first attempt to free the syngnathia. The literature is discussed and a causative mechanism and new classification are proposed.- - - - - - - - - - ranking = 5keywords = complex (Clic here for more details about this article) |
3/8. Humeroradial synostosis, ulnar aplasia and oligodactyly, with contralateral amelia, in a child with prenatal cocaine exposure.Humeral "bifurcation" due to humeroradial synostosis, and amelia are both very rare limb anomalies. We report on a Canadian. Aboriginal boy with both these limb deficiencies. The family history was unremarkable, but he was exposed prenatally to cocaine at the time of limb development. Humeroradial synostosis with ulnar aplasia has been reported by several authors. The majority of cases are unilateral. When both upper limbs arms are involved, cases with oligodactyly often have asymmetrical limb deficiencies and have all been sporadic to date. Some appear to represent cases of the femur-fibula-ulna or FFU complex. Affected individuals with normal hands usually have symmetrical defects and show an autosomal recessive pattern of inheritance. Limb deficiencies have been reported in several infants exposed prenatally to cocaine and have been inducible in animal models. Most are terminal transverse defects or deficiencies of middle digits. When more than one limb is involved, the defects are usually asymmetric. Our case appears to be one of the most severely affected children reported to date.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
4/8. Congenital maxillomandibular fusion: a case report and review of the literature.Congenital fusion of mandible to maxilla (syngnathia) is rare. Only 25 cases of syngnathia have been reported up to date since the first case was reported in 1936. Of these cases only six have involved fusion of the ascending rami of mandible to the maxilla and zygomatic complex. This report is an additional case of syngnathia involving bilateral bony fusion of mandibular horizontal and ascending rami to zygomatic complex and maxilla in a female neonate. Delay in treatment afforded the opportunity to observe the effects on the growth of the patient as she presented again at the age of 3 years.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
5/8. Intrathoracic kidney and vertebral fusion: a model of combined misdevelopment.We report a case of right intrathoracic kidney associated with a complex somite malformation that comprised vertebral fusion and right intrathoracic supernumerary ribs. The interpretation of this association as a disease model of nature allows us to propose a unifying hypothesis on the pathogenesis of this form of renal dystopia.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
6/8. Congenital fusion of the maxilla and mandible.Congenital fusion of the maxilla and mandible (syngnathia) is rare and can present in a wide range of severity from single mucosal bands (synechiae) to complete bony fusion (synostosis). Congenital synostosis of the mandible and maxilla is even less common than synechiae, with only 25 cases reported in the literature. Most of them presented as an incomplete, unilateral fusion. A 4-year-old boy was referred to the authors' emergency unit with asphyxia after vomiting. The authors found the child could not open his mouth. His upper and lower jaws were fused, with only a 2- to 3-mm gap in the anterior part. x-rays and computed tomography scans showed that there was a bony fusion of the ramus of the mandible to the zygomatic complex and the posterior part of the maxilla. In addition, there was significant mandible hypoplasia. After performing an osteotomy (to treat the fusion between the bilateral ramus mandible, maxilla, and zygoma), the authors performed a temporomandibular joint reconstruction using a silicon block. After the completion of these procedures, they observed that the mouth could be opened 32 mm. After 2 years of mandibular lengthening performed with an external distracter, the patient's facial appearance and occlusion became more acceptable. An extremely rare case is described, and the existing literature is reviewed.- - - - - - - - - - ranking = 1keywords = complex (Clic here for more details about this article) |
7/8. Cavovarus foot deformity with multiple tarsal coalitions: functional and three-dimensional preoperative assessment.In rare instances, tarsal coalition leads to cavovarus foot deformity, although the pathologic mechanism leading to this deformity is not clear. This article reports a case of a 14-year-old boy presenting a severe cavovarus deformity of the right foot with talocalcaneal and calcaneonavicular coalitions, and a mild cavus deformity of the left foot with a single talocalcaneal coalition. Computed tomography and postoperative histologic analysis demonstrated a synostosis between talus and calcaneus and a fibrous calcaneonavicular coalition with partial ossification. Instrumented gait analysis revealed a limited range of ankle plantar flexion and increased external rotation of the ankle. Associated skeletal malformations including incomplete hemimelia of the forearm and scoliosis raised the possibility of a teratologic condition, but neurologic examination, spinal magnetic resonance imaging, and nerve conduction velocities were normal. The progressive ossification of combined coalitions during growth of the foot may have been one factor leading to this complex foot deformity. The fine-wire electromyogram showed normal tibialis anterior and posterior muscle activity. Small soft tissue tears in the sinus tarsi may have led to a mild reflexive increase of the muscle tone and tendon shortening, which pulled the forefoot into adduction and the heel into varus, and raised the medial arch. Mechanical alterations of the ankle appear secondary to the heel varus and to the progressive deformity of the talus. Three-dimensional computed tomography reconstruction and gait analysis appeared to be helpful additional parameters to understanding the pathomechanics of this complex foot deformity and for preoperative planning of triple arthrodesis.- - - - - - - - - - ranking = 2keywords = complex (Clic here for more details about this article) |
8/8. Physical mapping of the t(12;22) translocation breakpoints in a family with a complex type of 3/3'/4 synpolydactyly.We previously reported clinical and radiological findings in a Belgian family with a complex type of synpolydactyly associated with metacarpal and metatarsal synostoses, cosegregating with a balanced t(12;22). Recently, expansions of a polyalanine stretch within the first exon of the HOXD13 gene, which resides on chromosome 2q31, have been shown to cause synpolydactyly (SPD). Using exon amplification followed by direct sequencing, we were able to exclude the direct involvement of the HOXD13 gene in this family. As a first step toward the positional cloning of a candidate disease gene on chromosome 12 and/or 22 responsible for the type of complex synpolydactyly observed in this family, we report here the construction of a somatic cell hybrid retaining only the der(22) of the t(12;22)(p11.3;q13.3). STS content mapping and FISH experiments allowed us to position the chromosomal breakpoints between markers D12S1596 and D12S1034 on chromosome 12 and markers N73F4 and D22S158 on chromosome 22.- - - - - - - - - - ranking = 6keywords = complex (Clic here for more details about this article) |