Management of Thoracic Insufficiency Syndrome in Patients With Jeune Syndrome Using the 70 mm Radius Vertical Expandable Prosthetic Titanium Rib.

BACKGROUND: Jeune syndrome (JS) often results in lethal thoracic insufficiency syndrome. Since 1991, vertical expandable prosthetic titanium rib Dynamic PosteroLateral Expansion Thoracoplasty was used at our institution for treatment of JS. This study assesses the safety and efficacy of this procedure. METHODS: Twenty-four JS patients were treated, 2 lost to follow-up, 17 with a minimum of 2-year follow-up retrospectively reviewed for clinical course: Assisted Ventilation Rate, respiratory rate, capillary blood gases, pulmonary function testings, and complications. Upright anteroposterior/lateral radiographs were measured for Cobb angle, kyphosis, lordosis, thoracic width, and thoracic/lumbar spinal height. Computed tomography scan lung volumes were obtained in 12 patients. RESULTS: Mean age at initial implant was 23 months (7 to 62 mo) with an average 8.4 years (2.3 to 15.6 y) of follow-up. Average chest width increased from 121 to 168 mm at follow-up (P<0.001). Preoperatively, 7/17 (41%) patients had scoliosis. The remainder developed scoliosis during treatment, 8 requiring additional implants. Thoracic and lumbar spinal height was normal preoperatively and stayed normal during treatment. Thoracic kyphosis/lumbar lordosis was stable. Average computed tomography scan total lung volumes increased 484 to 740 mm3 (P<0.001), and Assisted Ventilation Rate status tended to improve (P=0.07). Average forced vital capacity was 34% predicted at first test and 27% predicted at last follow-up. Early demise after surgery was common with multisystem disease. Mean respiratory rate decreased from 35 to 24 bpm at last follow-up (P<0.05). Survival rate of the 22 patients was 68%. Migration of the rib cradles/titanium slings occurred in 12 patients, superficial infections in 5 patients, deep infections in 4 patients, and wound dehiscence in 5 patients. Infection rate was 4.6% per procedure. CONCLUSIONS: The survival rate in JS with surgery was nearly 70% (compared with 70% to 80% mortality without treatment) with less ventilator dependence. Both C1 stenosis and scoliosis are common in JS. Spinal height in JS is normal. Complications are frequent, but tolerable in view of the clinical gains and increase in survival.

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.

Use of a biodegradable patch for reconstruction of large thoracic cage defects in growing children.

BACKGROUND/PURPOSE: At our institution, procedures were developed to enlarge and stabilize the thoracic cavity of children born with severe-enough abnormalities of the thoracic cage as to result in lack of normal lung growth and function. In addition to the device known as the vertical expandable prosthetic titanium rib (VEPTR), some type of patch material was needed to cover large congenital defects of the chest cage owing to rib absence or to cover defects created because of the expansion process. Initially, synthetic material, polytetrafluorethylene (Gore-Tex, WL Gore and Associates, Flagstaff, Ariz) was used, but this proved itself to be restrictive over time and required removal. Thus, a nonsynthetic biodegradable patch material was adopted for coverage of the defects (Surgisis, Cook, Bloomington, Ind). METHODS: From October 2001 through October 2004, 26 growing children undergoing thoracic cage reconstruction received the biodegradable extracellular matrix patch derived from porcine SIS (Cook). A patch was deemed necessary if any one of the following conditions was obtained: (a) herniation of the lung was likely; (b) chest wall musculature was significantly diminished; (c) injury to the lung was likely at reoperation; (d) the defect was greater than 2 x 2 cm. RESULTS: During the follow-up period, 41 months thus far, each of the 26 children has undergone routine, scheduled expansions or change-out procedures every 6 months. The SIS has not required removal for any reason and has not restricted growth of the thoracic cage. CONCLUSIONS: Compared with synthetic soft tissue patch material, the nonsynthetic, biodegradable soft tissue patch (SIS) has proven to be a superior alternative, thus far, to others for use in our population of patients for reconstruction of the thoracic cage in the growing child.

The effect of opening wedge thoracostomy on thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.

BACKGROUND: Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth and is seen in patients who have severe congenital scoliosis with fused ribs. Traditional spinal surgery does not directly address this syndrome. METHODS: Twenty-seven patients with congenital scoliosis associated with fused ribs of the concave hemithorax had an opening wedge thoracostomy with primary longitudinal lengthening with use of a chest-wall distractor known as a vertical, expandable prosthetic titanium rib. Repeat lengthenings of the prosthesis were performed at intervals of four to six months. Radiographs were analyzed with respect to correction of the spinal deformity, as indicated by a change in the Cobb angle, and lateral deviation of the spine, as indicated by the interpedicular line ratio. Spinal growth was assessed by measuring the change in the length of the spine. Correction of the thoracic deformity and thoracic growth were assessed on the basis of the increase in the height of the concave hemithorax compared with the height of the convex hemithorax (the space available for the lung), the increase in the thoracic spinal height, and the increase in the thoracic depth and width. The thoracic deformity in the transverse plane was measured with computed tomography, and the scans were analyzed for spinal rotation, thoracic rotation, and the posterior hemithoracic symmetry ratio. Clinically, the patients were assessed on the basis of the relative heights of the shoulders and of head and thorax compensation. Pulmonary status was evaluated on the basis of the respiratory rate, capillary blood gas levels, and pulmonary function studies. RESULTS: The mean age at the time of the surgery was 3.2 years (range, 0.6 to 12.5 years), and the mean duration of follow-up was 5.7 years. All patients had progressive congenital scoliosis, with a mean increase of 15 degrees /yr before the operation. The scoliosis decreased from a mean of 74 degrees preoperatively to a mean of 49 degrees at the time of the last follow-up. Both the mean interpedicular line ratio and the space available for the lung ratio improved significantly. The height of the thoracic spine increased by a mean of 0.71 cm/yr. At the time of the last follow-up, the mean percentage of the predicted normal vital capacity was 58% for patients younger than two years of age at the time of the surgery, 44% for those older than two years of age (p < 0.001), and 36% for those older than two years of age who had had prior spine surgery. In a group of patients who had sequential testing, all increases in the volume of vital capacity were significant (p < 0.0001), but the changes in the percentages of the predicted normal vital capacity were not. There was a total of fifty-two complications in twenty-two patients, with the most common being asymptomatic proximal migration of the device through the ribs in seven patients. CONCLUSIONS: Opening wedge thoracostomy with use of a chest-wall distractor directly treats segmental hypoplasia of the hemithorax resulting from fused ribs associated with congenital scoliosis. The operation addresses thoracic insufficiency syndrome by lengthening and expanding the constricted hemithorax and allowing growth of the thoracic spine and the rib cage. The procedure corrects most components of chest-wall deformity and indirectly corrects congenital scoliosis, without the need for spine fusion. The technique requires special training and should be performed by a multispecialty team.

Expansion thoracoplasty: the surgical technique of opening-wedge thoracostomy. Surgical technique.

BACKGROUND: Children with congenital thoracic scoliosis associated with fused ribs with a unilateral unsegmented bar adjacent to convex hemivertebrae will invariably have curve progression without treatment. Surgery has been thought to have a negligible growth-inhibition effect on the thoracic spine in such patients because it has been assumed that the concave side of the curve and the unilateral unsegmented bar do not grow, but we are unaware of any conclusive studies regarding this assumption. METHODS: The changes in the length of the concave and convex sides, anterior and posterior vertebral edges, posterior arch, and unilateral unsegmented bars of the thoracic spine were measured in the twenty-one children with congenital scoliosis and fused ribs after expansion thoracoplasty had been carried out with use of a vertical, expandable titanium prosthetic rib. Three of these children had undergone posterior spinal fusion previously. Measurements were made with use of a three-dimensional software program that analyzed baseline and follow-up computed tomography scans. The technique was validated through measurement of the thorax of a small female adult cadaver. RESULTS: The patients without spine fusion had an average age of 3.3 years at the time of the baseline computed tomography scan, and the average duration of follow-up was 4.2 years. On the average, these patients showed significant growth (p < 0.0001) of the concave side of the thoracic spine (an increase in length of 7.9 mm/yr, or 7.1%/yr) and the convex side (8.3 mm/yr, or 6.4%/yr) compared with the baseline lengths. There was no significant difference in the increases in length (p = 0.38) between the concave and convex sides. Eleven patients with an unsegmented bar had an average 7.3% increase in the length of the bar (p < 0.0001). In the three children with prior spinal fusion, the increase in length averaged only 4.6 mm/yr (3%/yr) on the concave side of the thoracic spine and 3.7 mm/yr (2.2%/yr) on the convex side; both increases were significant (p < 0.0001). CONCLUSIONS: Longitudinal growth of the thoracic spine in a normal child has been estimated to be 0.6 cm/yr between the ages of five and nine years. After expansion thoracoplasty, growth of the thoracic spine was approximately 8 mm/yr in our series of children with congenital scoliosis and fused ribs. After expansion thoracoplasty, both the concave and the convex side of the thoracic spine and unilateral unsegmented bars appeared to grow in these patients. When a thorax is already foreshortened by congenital scoliosis, control of spine deformity with expansion thoracoplasty allows growth of the thoracic spine, and it is likely that the longer thorax provides additional volume for growth of the underlying lungs with probable clinical benefit.

The characteristics of thoracic insufficiency syndrome associated with fused ribs and congenital scoliosis.

1. Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration or lung growth. 2. The rare condition of fused ribs and congenital scoliosis may result in a three-dimensional thoracic deformity with adverse effects on thoracic growth and function with development of thoracic insufficiency syndrome. 3. The normal thorax is defined by two characteristics: normal, stable volume and the ability to change that volume. Volume depends on the width and depth of the rib cage, and the thoracic spine provides height. The ability to change volume, termed thoracic function, is provided by the diaphragm and the secondary muscles of respiration. 4. On radiographs, the loss of the vertical height of the lung of the concave, restricted hemithorax is defined by the percentage of space available for the lung. 5. Spine rotation causes a windswept thorax, with both restriction of the volume of the convex hemithorax and restriction of the motion of the involved ribs. 6. Constrictive three-dimensional deformity of the thorax may cause extrinsic, restrictive lung disease. 7. Progressive thoracic insufficiency syndrome is diagnosed on the basis of clinical signs of respiratory insufficiency, loss of chest wall mobility as demonstrated by the thumb excursion test, worsening indices of three-dimensional thoracic deformity on radiographs and computed tomography scans, or a relative decline in percent predicted vital capacity due to thoracic "failure to thrive," as demonstrated by pulmonary function tests. 8. Treatment of progressive thoracic insufficiency syndrome should provide an acute increase in the thoracic volume with stabilization of any flail chest-wall defects and maintain these improvements as the patient grows, without the need for spine fusion.