Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome.

INTRODUCTION: Although surgical treatment of spondylothoracic dysplasia (STD) is controversial, we have found that an expansion thoracoplasty using a Vertical Expandable Prosthetic Titanium Rib (VEPTR; DePuy Synthes) results in favorable outcomes, including 100% survivability (at an average follow-up of 6.2 years), increased thoracic spinal length, and decreased requirements for ventilation support. STEP 1 PREOPERATIVE PREPARATION: Make anteroposterior and lateral radiographs of the spine. STEP 2 POSITION THE PATIENT FOR THE PROCEDURE: The patient is placed in the prone position. STEP 3 THE INCISION: A curvilinear skin incision is made, starting proximally between the spine and the medial edge of the scapula. STEP 4 THE OSTEOTOMY: Perform the v-osteotomy. STEP 5 PLACEMENT OF THE VEPTR DEVICE: A number-4 VEPTR-I device is wedged in, starting laterally within the osteotomy sites, wedging the osteotomies apart, distracting the superior ribs proximally and the inferior ribs distally, lengthening the hemithorax, and stopping approximately at the posterior axillary line, when there is maximum stress on the superior and inferior ribs, to avoid fracture, and the lamina spreaders are then removed. STEP 6 WOUND CLOSURE: Insert drains and local anesthetic catheters and close the wound. STEP 7 EXPANSION AND REPLACEMENT PROCEDURES: Lengthen the devices with the standard VEPTR technique of limited 3-cm incisions every three to six months. RESULTS: VEPTR treatment in patients with STD is associated with increased thoracic spine height and reduced thoracic width-to-height ratio, suggesting a greater gain in height than in width. WHAT TO WATCH FOR: IndicationsContraindicationsPitfalls & Challenges.

Can VEPTR(®) control progression of early-onset kyphoscoliosis? A cohort study of VEPTR(®) patients with severe kyphoscoliosis.

BACKGROUND: Kyphoscoliosis is considered a relative contraindication to treatment with the Vertical Expandable Prosthetic Titanium Rib (VEPTR(®); Synthes Inc, Paoli, PA). Nevertheless, patients do present with early-onset kyphoscoliosis and thoracic insufficiency syndrome, and no suitable alternative treatments are currently available. However, it is unclear whether VEPTR(®) is reasonable for treating patients with kyphoscoliosis. QUESTIONS/PURPOSES: We determined whether VEPTR(®) controls progression in patients with kyphoscoliosis and, if so, what methods might be used to improve control of deformity progression in these patients. PATIENTS AND METHODS: We retrospectively reviewed 14 patients who had VEPTR(®) treatment of early-onset kyphoscoliosis. Degrees of kyphosis and scoliosis before, during, and after treatment were measured, and levels of instrumentation, thoracic dimensions, and complications were recorded. Minimum followup was 1.7 years (average, 5.8 years; range, 1.7-12.8 years). RESULTS: While scoliosis was stabilized, kyphosis increased a mean of 22° at last followup. Supple kyphosis became rigid during treatment. Proximal cradle cutout was a recurring problem. Distal anchors placed too proximally had inadequate lever arms to control kyphosis. CONCLUSIONS: Progression of kyphosis can be minimized during VEPTR(®) treatment by early extension of the construct to the second ribs bilaterally, distal extension of hybrid constructs to the pelvis, use of bilateral hybrid VEPTR(®) implants, and use of redesigned VEPTR(®) constructs that enhance fixation at the upper end. While our early results suggest these devices control progression of kyphosis, longer followup with more patients will be required to confirm the concept in these patients.

The effect of mid-thoracic VEPTR opening wedge thoracostomy on cervical tilt associated with congenital thoracic scoliosis in patients with thoracic insufficiency syndrome.

STUDY DESIGN: Prospective clinical trial of vertical expandable prosthetic titanium rib (VEPTR) surgery in patients with congenital thoracic scoliosis with thoracic insufficiency syndrome and cervical tilt. OBJECTIVE: Report efficacy and safety of VEPTR mid-thoracic opening wedge thoracostomy in treatment of cervical tilt and head/truncal decompensation in children with thoracic insufficiency syndrome associated with thoracic congenital scoliosis. SUMMARY OF BACKGROUND DATA: Cervical tilt in children is a rare problem, usually associated with congenital cervical or cervical-thoracic scoliosis, and in progressive deformity, surgical fusion or hemi-vertebrectomy risks neurologic injury, loss of motion of the cervical spine, and unknown effect on pulmonary function. VEPTR patients with congenital scoliosis can have severe neck tilt with poor cosmesis, with the risk of neck pain in adulthood. VEPTR mid-thoracic opening wedge thoracostomy treats thoracic insufficiency syndrome, and paradoxically also appears to improve the neck alignment cosmesis. MATERIALS METHODS: Patients with fused ribs and congenital scoliosis, thoracic insufficiency syndrome, and cervical tilt were treated with VEPTR mid-thoracic thoracostomy. Complication rates, respiratory outcome, pulmonary function tests in older children, Cobb angles, head/truncal decompensation, T1 oblique take-off, space available for lung, height of the thoracic spine, and cervical tilt angle were measured. RESULTS: Fourteen patients with cervical tilt, fused ribs, progressive congenital scoliosis, and TIS were treated. The primary thoracic scoliosis and space available for lung improved, cervical tilt stabilized, and head and truncal decompensation improved. One child was weaned off a ventilator. Complications included device migration, infection, and transient brachial palsy. CONCLUSION: Mid-thoracic VEPTR opening wedge thoracostomy can stabilize cervical tilt associated with thoracic congenital scoliosis and fused ribs and may be an alternative to cervicothoracic spine fusion or hemi-vertebrectomy in some young children.

Pseudarthrosis after lumbar spine fusion: nonoperative salvage with pulsed electromagnetic fields.

We studied 100 patients in whom symptomatic pseudarthrosis had been established at more than 9 months after lumbar spine fusion. All patients were treated with a pulsed electromagnetic field device worn consistently 2 hours a day for at least 90 days. Solid fusion was achieved in 67% of patients. Effectiveness was not statistically significantly different for patients with risk factors such as smoking, use of allograft, absence of fixation, or multilevel fusions. Treatment was equally effective for posterolateral fusions (66%) as with interbody fusions (69%). For patients with symptomatic pseudarthrosis after lumbar spine fusion, pulsed electromagnetic field stimulation is an effective nonoperative salvage approach to achieving fusion.

Small bowel motility and transit after aortic surgery.

OBJECTIVE: The inability to tolerate feedings after aortic surgery prolongs hospitalization. The aim of this study was to define jejunal manometric and small bowel transit characteristics associated with the ileus that follows transperitoneal aortic surgery. METHODS: Five male patients who underwent transperitoneal infrarenal aortobifemoral bypass had intraoperative placement of a jejunal multilumen catheter. The open abdomen allowed precise placement of pressure recording ports at 20, 22, 24, 26, 28, and 38 cm past the ligament of Treitz. Three-hour manometric studies were done after surgery and for 3 postoperative days. The migrating motor complex was identified visually on the manometric tracings, and pressure waves were identified with computer and a motility index calculated. Motility data were compared with healthy control data previously reported in the literature. Small bowel transit was determined with barium and serial abdominal radiographs. RESULTS: All patients had ileus develop with return of bowel sounds at 2 to 7 days (median, 6 days) and flatus at 3 to 9 days (median, 7 days) after surgery. Jejunal motor activity was present within 6 hours of surgery, but the motility index was less in patients then in control subjects. The postoperative migrating motor complexes differed from control subjects in having more phase I, less phase II, and more frequent phase IIIs. Phase III retrograde migration was common in the patients but not in the control subjects. Small bowel transit was 2 days or greater in all patients. CONCLUSION: Motor activity is present in the jejunum shortly after aortic surgery. However, the activity is decreased in intensity and the fasting cycle differs from control subjects. Retrograde migration of phase III is the most likely abnormality, resulting in delayed small bowel transit. The data would predict a high rate of enteral feeding intolerance early after surgery. Future studies should focus on pharmacologic manipulation to rapidly return small bowel motility to a more normal state after aortic surgery.