RESUMO
BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation (also known as port-wine stain), varicose veins and malformations, and bony and/or soft tissue hypertrophy with or without lymphatic malformation, which are known as the "classic clinical triad". Herein, a rare case of KTS characterized by crossed-bilateral limb hypertrophy accompanied by intermittent hematochezia and hematuria is reported. CASE SUMMARY: We described a 37-year-old female with KTS. She was admitted to our hospital owing to the gradual enlargement of the left lower extremity along with intermittent hematochezia and hematuria. The patient was diagnosed to have hemorrhoid bleeding by other hospitals and treated with conventional hemostatic drugs, but continued to have intermittent gastrointestinal bleeding and hematuria. Therefore, she visited our hospital to seek further treatment. During hospitalization, relevant imaging and laboratory examinations and colonoscopy were performed. In combination with the patient's history and relevant examinations, we considered that the patient had a complex form of KTS. We recommended a combined diagnosis and treatment from the vascular, interventional, anorectal, and other departments, although she declined any further treatment for financial reasons. CONCLUSION: The clinical manifestations of KTS are extensive and diverse and chiefly include the typical triad. However, Vascular malformations of KTS can also involve several parts and systems such as digestive and urogenital systems. Therefore, the atypical manifestations and rare complications necessitate the clinician's attention and are not to be ignored.
RESUMO
STUDY DESIGN: Case report. OBJECTIVE: To report the clinical features, radiographic findings, treatments, and results of 2 children with cervical intervertebral disc calcification combined with ossification of the posterior longitudinal ligament (OPLL). SUMMARY OF BACKGROUND DATA: The calcification of the intervertebral disc, which is more frequent in males with predominant localization to cervical spine, was first reported by Baron in 1924. OPLL of the cervical spine, which is found approximately in the 5th to 7th decade of life, is a disease-causing spinal canal stenosis and spinal cord compression. The etiologies of these 2 diseases still remain unclear. METHODS: An 8-year-old girl presented with progressive neck pain and complained of weakness and numbness of the upper left extremity, and a 6-year-old boy presented with complains of neck pain. X-ray, computed tomography, and magnetic resonance imaging findings of 2 patients confirmed the presence of cervical intervertebral disc calcification combined with OPLL. RESULTS: Two children were treated using conservative treatment. The girl was observed up for 2 years and the boy was observed up for 18 months, respectively. Computed tomography and magnetic resonance imaging revealed that cervical intervertebral disc calcification and OPLL at the C6/7 (case 1) and C3/4 (case 2) level have disappeared completely, only a small calcification at the C2/3 intervertebral disc remained in the second case and both of them were asymptomatic. CONCLUSIONS: Cervical intervertebral disc calcification combined with OPLL was rarely observed in children. Conservative management was carried out and the patients had a full recovery. Our experience suggests that the conservative treatment is an acceptable method.