A 12-year-old boy with progressive trunk deformity and a huge subcutaneous tumor was referred to our department. At the age of three, the subcutaneous tumor was found in the back, and biopsy of the tumor indicated a diagnosis of cavernous hemangioma. During that biopsy, a massive hemorrhage occurred, and the patient required a blood transfusion (3000 ml). Scoliosis was diagnosed at age 11, and the patient underwent brace treatment. However, the scoliosis worsened progressively, and the patient was finally referred to our department. Upon physical examination, we found significant protrusion of the right back ribs. On the left back was a huge subcutaneous tumor, measuring approximately 15 cm in diameter, and a scar from a previous surgical wound that was approximately 5 cm long (Figure ). The mass was not tender or throbbing, and no neurological abnormalities were observed.
Figure 1 Clinical appearance of the patient. On physical examination, significant protrusion of the right ribs, as well as a huge subcutaneous tumor measuring approximately 15 cm in diameter and a surgical wound measuring approximately 5 cm in length on the left (more ...)
Full-length, standing radiographs demonstrated a scoliosis of 85° at T6-L1 and a kyphosis of 58° at T4-T10. The Risser sign was grade zero, and the triradiate cartilages were open (Figure ). Traction radiography showed a correction rate of 6%, indicating extremely low flexibility. On the axial CT images, vertebral body atrophy was recognized at T7-T10, where the vertebral bodies were surrounded by the cavernous hemangioma (Figure ). No congenital deformity of the vertebral bodies was observed. MR images revealed that the hemangioma extended from the subcutaneous region to the paraspinal muscles and the retroperitoneal space at T6-L1, primarily on the left side. Invasion of the hemangioma into the spinal canal and compression of the dura by the hemangioma were recognized at the T8-T10 level (Figure ).
Standing X-ray films. Radiographs indicated a scoliosis of 85° at T6-L1 and a kyphosis of 58° at T4-T10. The Risser sign was grade zero, and the triradiate cartilages were open.
Axial CT image. Axial CT image at T10 indicating the atrophy of vertebral bodies and invasion of the hemangioma.
Figure 4 MR images of the hemangioma. The huge hemangioma extending from the subcutaneous region to the paraspinal muscles and the retroperitoneal space was observed at T6-L1 primarily on the left side. Invasion into the spinal canal and compression of the dura (more ...)
The diagnosis of severe progressive scoliosis associated with cavernous hemangioma was made. Prior to the surgery, we decided against the use of radiotherapy, which may reduce the size of the tumor or blood circulation through it, because irradiation of the spine can lead to pseudoarthrosis. In planning the surgery, an anterior approach was considered first, since the hemangioma was mainly located on the concave side of the curve. However, we abandoned this idea because damage to the hemangioma during disc removal or screw placement would have required us to manage the hemorrhage from inside the narrow thoracic cage, which might not have been successful. Although bleeding from hemangioma was inevitable if we used the posterior approach, we had the option of stopping the bleeding by the tamponade effect of closing the wound. Therefore, we used the posterior approach for correction surgery and fusion with pedicle screws at T2-L3.
A midline skin incision was made on the patient's back. Soon after the incision was made, the hemangioma hemorrhaged massively, and hemostasis by coagulation failed using bipolar forceps, electrocautery, and ligation. Thus, the surgery was continued with gauze packing at the bleeding sites. The soft tissues were carefully detached from the spinous processes or laminae subperiosteally using a spatula to minimize damage to the pseudocapsule of the hemangioma, and consequently, the hemorrhage greatly decreased. Pedicle screws were placed segmentaly, except from T8 to T10, where the hemangioma invaded the spinal canal, to avoid spinal cord injury from intracanalar hemorrhage of the hemangioma. Since we encountered massive hemorrhage at T11, 12 and L1, we could not place pedicle screws at these levels, where we had hoped to place them to increase the number of anchor points. The correction force was applied carefully to avoid screw failure, since fewer screws could be placed than were needed and the flexibility of the curves was quite low. After the correction, abundant bone grafting was performed using the iliac crest and local bone. No drainage tube was placed inside the wound, since it could have increased the risk of postoperative hemorrhage by penetrating or damaging the surrounding hemangioma (figure ) during insertion of the tube. After surgery, the scoliosis was corrected to 59°, and kyphosis to 45°, with correction rates of 31% and 22%, respectively (Figure ). The intraoperative time was 314 minutes, and the intraoperative blood loss was 2800 ml.
Postoperative radiographs. After surgery, scoliosis was corrected to 59°, and kyphosis to 45°, with correction rates of 31% and 22%, respectively.
The patient's hemodynamics stabilized with blood hemoglobin level of 9.2 mg/dl when the surgery finished at 7 pm after the transfusion of 400 ml of preoperative donated autologous blood and 1350 ml of intraoperative and postoperative cell saver autologous blood. The patient was admitted to intensive care unit at 8 pm. At 11 pm (four hours after surgery), his blood pressure was 92/52 mmHg with stable hemodynamics, and his blood hemoglobin level was 8.5 mg/dl. However, seven hours after surgery, after the administration of diazepam to control agitation, his blood pressure suddenly decreased to an unmeasurable level, and the patient became unconscious with apnea. At that time, the hemoglobin level was 5.9 mg/dl. Endotracheal intubation was immediately performed, along with blood transfusion, which resulted in his recovery from hypovolemic shock. The following day, however, the patient developed disseminated intravascular coagulation (fibrinogen degradation products [FDP] 32.3 μg/ml, platelet count 95,000/μl, prothrombin time international normalized ratio [PT-INR] 2.28), and received fresh frozen plasma and gabexate mesylate. Seven days after the surgery, when his general condition had stabilized, the patient was extubated. However, as the patient became conscious, a language disorder became apparent, and a head MRI revealed ischemic lesions in the bilateral frontal and temporal lobes. Sensory and conduction aphasia was diagnosed, caused by cerebral hypoxia during the hypovolemic shock on the day of the surgery (Figure ). The patient gradually recovered from the aphasia, and the ischemic lesions became smaller on MRI (Figure ). At present, two years after the surgery, although the patient has completely recovered from the aphasia.
MRI indicating brain ischemia. a: MRI revealed ischemic lesions in the bilateral frontal and temporal lobes. These lesions caused postoperative sensory and conduction aphasia. b: MRI revealed that the ischemic lesions had become much smaller.