1/3. Hypochondroplasia.Hypochondroplasia is an autosomal dominant discrete form of short-limbed dwarfism with roentgenographic changes similar to, but distinctive from, achondroplasia. The patients are normal at birth and in early childhood present with short-limbed dwarfism, thick body build, long trunk, normal head, moderate lumbar lordosis and broad, stubby hands and feet. A high incidence of mental retardation and absence of other neurologic complications is distinctive from achondroplasia. diagnosis of hypochondroplasia is primarily radiogrpahic. No single diagnostic radiographic sign is present, and thus, a complete radiographic survey of the whole skeleton is required for diagnosis. Pertinent radiographic findings include a normal skull, short, broad long bones with prominent bony sites of muscle attachment, normal growth plate, prominent styloid process of the ulna and lateral malleolus of the fibula, shortened base of the iliac bones, horizontal hypoplastic low-set sacrum and mild narrowing of the interpediculate distance of the lumbar spine. Differential diagnosis is from other types of short-limbed dwarfism, particularly achondroplasia. Kozlowski, an authority on hypochondroplasia, believes this form of short-limbed dwarfism, if carfully searched for, may be more common than achondroplasia.- - - - - - - - - - ranking = 1keywords = lordosis (Clic here for more details about this article) |
2/3. Pseudoachondroplastic dysplasia.Pseudoachondroplasia is a heterogeneous inherited skeletal dysplasia in which dwarfism is a major feature. We report here a case of a 7 year old girl misdiagnosed as rickets, who presented with short stature, lordosis, genu varum and flexion deformities at both the elbows. Skeletal survey revealed epiphyseal and metaphyseal irregularities. A review of literature is also presented.- - - - - - - - - - ranking = 1keywords = lordosis (Clic here for more details about this article) |
3/3. Epidural anesthesia for extracorporeal shock wave lithotripsy in an achondroplastic dwarf.BACKGROUND AND OBJECTIVES: A 66-year-old achondroplastic male dwarf with right ureteral stones presented for outpatient extracorporeal shock wave lithotripsy under epidural anesthesia. methods: The patient had marked lumbar lordosis and mild thoracic kyphosis. Although the epidural space was located easily at the L2-3 interspace on the first attempt, the catheter could not be advanced beyond the Tuohy needle. On the second attempt, the epidural space was located easily at the L1-2 interspace, and the catheter was advanced without difficulty. RESULTS: A sensory block to the T2 level developed after administration of 2 mL of lidocaine 1.5% with epinephrine 1:200,000 and 9 mL of lidocaine 2.0% with epinephrine 1:200,000. Aside from a short period of mild, asymptomatic hypotension, the patient's intraoperative and postoperative courses were unremarkable. CONCLUSIONS: This case report and the available literature support the feasibility of epidural anesthesia in achondroplastic patients. Careful titration of the local anesthetic dose is recommended since achondroplastic patients may have extensive spread of epidural anesthesia.- - - - - - - - - - ranking = 1keywords = lordosis (Clic here for more details about this article) |