Ann Thorac Surg 2001;72:872-877
© 2001 The Society of Thoracic Surgeons
Original article: general thoracic
Lateral thoracic expansion for Jeune’s syndrome: midterm results
J. Terrance Davis, MDa,
Jonathan B. Heistein, MDa,
Robert G. Castile, MDa,
Brent Adler, MDa,
Khaled H. Mutabagani, MD, PhDa,
Rafael E. Villalobos, DOa,
Robert L. Ruberg, MDa
a Department of Cardiac Surgery, Columbus Children’s Hospital, The Ohio State University School of Medicine and Public Health, Columbus, Ohio, USA
Accepted for publication May 10, 2001.
Address reprint requests to Dr Davis, Department of Cardiac Surgery, Columbus Children’s Hospital, 700 Children’s Dr, Columbus, OH 43205
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Abstract
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Background. In 1995, we reported the use of lateral thoracic expansion in a patient with symptomatic Jeune’s asphyxiating thoracic dystrophy. We have subsequently used lateral thoracic expansion 16 times on 10 patients during 7 years. This article reports our outcomes and provides surgical details.
Methods. Charts of all patients undergoing lateral thoracic expansion were reviewed. Eight of the 10 patients had symptomatic Jeune’s syndrome. The other 2 had similar thoracic deformities limiting thoracic capacity. In half of the patients the procedures were performed bilaterally.
Results. All patients older than 1 year of age were symptomatically benefited by lateral thoracic expansion. Functional and anatomic measurements documented thoracic enlargement in several patients who had comparable preoperative and postoperative studies. However, 2 infants with significant underlying airway disease did not improve and went on to succumb to that aspect of their disease despite enlargement of the thorax. Fracture of the titanium ministruts has been a recurrent problem, and we now use larger struts.
Conclusions. Lateral thoracic expansion is a safe and effective procedure in selected patients with Jeune’s syndrome older than 1 year of age as judged by short-term and midterm follow-up. More experience and longer follow-up are required to discern the place of the lateral thoracic expansion in the overall management of these patients.
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Introduction
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Jeune’s syndrome, or asphyxiating thoracic dystrophy, was first described by Jeune and colleagues [1, 2] in a pair of siblings. It is an autosomal recessive disease that involves deformities of the thoracic cage, pelvis, and phalanges in addition to varying degrees of renal dysfunction [3–5]. These children have a polychondrodystrophy characterized by broad, short, horizontal ribs and irregular costochondral junctions, which results in a bell-shaped, almost completely rigid thorax. The dimensions of the thorax are markedly reduced [6], and the chest wall disease causes restriction of the lungs by preventing normal intercostal muscle excursion [7, 8].
Jeune’s disease varies along a spectrum, and the age at presentation correlates with the severity of the disease [9–11]. The intrinsic function of the lungs may initially be anywhere on the spectrum from completely normal to severely hypoplastic with pulmonary hypertension [12, 13]. In the most severe form, the disease is fatal in the newborn period because of respiratory distress and failure. In milder forms, the child typically develops recurrent bouts of pneumonia with progressive, indolent respiratory failure usually within the first year of life [7, 9–11]. Typically, this leads to ventilator dependence and death without treatment. In contrast, some patients diagnosed later in life may have adequate ventilation at rest and their respiratory capacity may improve with age [5, 14]. With respect to outcome, relative contributions to the pathophysiology from the lungs, chest wall, or other organ systems have yet to be determined [5, 13] and may vary from patient to patient.
The only part of the disease that is theoretically correctable is the size of the thorax. Therefore, a variety of surgical approaches have been attempted. The sternum can be split longitudinally and widened with metal plates, acrylic plates, stainless steel struts, rib or other bone grafts, or other prostheses [14–17]. Although there are advantages and disadvantages to each approach, the surgical approach remains controversial.
In 1995 we described the lateral thoracic expansion (LTE) [18]. This approach reflected our attempt to actually create an enlarged chest wall on each side not ultimately dependent on prosthetic material to maintain the expansion. We have now performed 16 procedures on 10 patients during 7 years. This report documents the short-term and midterm results of this series, the technical modifications that have evolved during the years, and observations about the related pulmonary pathologic process.
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Material and methods
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Patient data
Clinic and hospital charts were reviewed retrospectively. Data collected from these charts included demographic information, procedural and postoperative details, and lengths of stay. The families were contacted and questioned regarding events before hospitalization, perception of clinical outcome, and whether or not they would recommend this procedure to others with similar problems.
Surgical technique
Sixteen procedures were performed on 10 patients. The technique evolved throughout the series, and the method described in this paper is our current preference. Single-lumen endotracheal anesthesia is used with the patients in the lateral position. Ribs number four through nine are approached. After elevating the skin, subcutaneous tissue, and muscles overlying the chest wall, the ribs are separated from their underlying rib beds as well as the remainder of the chest wall, the intercostal muscles, and parietal pleura. The ribs are then divided in a staggered fashion, as illustrated in Figure 1. The underlying chest wall with the rib bed is also divided in a staggered fashion in the opposite direction from the rib divisions. The long ends of the fifth and sixth ribs as well as the seventh and eighth ribs (Fig 2) are opposed and secured with titanium plates (until recently, Titanium Mini Bone Plating System [Walter Lorenz, Jacksonville, FL], for 15 of the 16 patients), thus expanding the section. The divided fourth and ninth ribs allow for expansion. The periosteal bridges are then brought together underneath the ribs to create new areas for calcification, creation of new chest wall, and stability for the ultimate enlargement. A single chest tube is then placed, and the incision is closed primarily. Postoperative management is similar to that for children who undergo a standard thoracotomy.
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Fig 1. Staggered osteotomies of a six-rib segment with opposite division of underlying periosteum, chest wall, and parietal pleura as one layer.
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Fig 2. Fifth rib bent down and sixth bent up and opposed to create expansion. Titanium plate maintains bone opposition for healing. Exposed periosteum calcifies in time. The process is repeated for ribs seven and eight. Ribs four and nine allow for expansion of the overall segment.
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To obtain objective data regarding the effectiveness of LTE we attempted to measure lung and thoracic volumes preoperatively and postoperatively on the last 5 children undergoing LTE. Functional residual capacity was measured by nitrogen washout [19] in 4 patients and by plethysmography [20] in patient H. Inspiratory capacity was measured by inflating the lungs to 30 cm H2O pressure, and total lung capacity was estimated as the sum of functional residual capacity and inspiratory capacity.
Thoracic air space volumes were also estimated using computed tomography imaging of the chest. Using the controlled-ventilation computed tomographic method [21], a pause in respiration was induced, the lungs were inflated to 25 cm H2O, and spiral computed tomographic images of the chest were obtained. Volumes were calculated using standard lung algorithms (Advantage Windows 3.1, GE Medical Systems, Milwaukee, WI) and measuring the volume of pixels between -50 and -900 Hounsfield unit thresholds. These data included all airspaces in the thorax including trachea. The volume of the mediastinum and hilar vessels were excluded.
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Results
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Table 1 lists the characteristics of all 10 patients who have undergone LTE at Columbus Children’s Hospital. Eight of the 10 patients had Jeune’s syndrome. Patient E was a 17-year-old patient with a combination of scoliosis with a neuromuscular disease that caused severe flattening of his right thorax. This resulted in chronic respiratory insufficiency, pulmonary hypertension, and ventilator dependency. Patient F had a diagnosis of Ellis-van Creveld syndrome, and was mainly symptomatic from a grossly restrictive thoracic deformity very similar to Jeune’s syndrome. All of our patients had a past medical history consistent with chronic progressive respiratory insufficiency. Symptomatology ranged from intermittent bouts of respiratory distress requiring hospitalization to severe respiratory insufficiency requiring mechanical ventilation. Four had varying degrees of renal dysfunction. One child was classified as severe (requiring dialysis) and 3 were not severe. Three children manifested some type of cardiac abnormality. Eight of 10 patients had tracheostomies whereas 5 of 9 had gastrostomy tubes.
Table 2 details the results of 16 procedures in the 10 patients in our series since 1993. The ages range from 3 months to 17 years. There were two mortalities in the series, one at 20 days and one at 78 days. Both were infants receiving ventilation. The cause of death was respiratory failure in both instances. Both had preoperative pulmonary parenchymal and malacic airway changes on chest computed tomographic scan. Although they both tolerated the procedure well, they were not helped by the operation, and they succumbed to progressive tracheomalacia and bronchomalacia.
The operation lasted a mean of 167 minutes, and the mean blood loss was 97 mL. The patients returned to their preoperative respiratory support level within an average of 0.9 days. The chest tube remained in place an average of 2.5 days. Length of stay at Columbus Children’s Hospital ranged from 3 to 78 days. Because some patients were from distant cities, they were transferred back to their home hospital, so the average 12.4-day postoperative length of stay does not reflect the total time in hospital for those patients. Excluding the two mortalities, the average length of stay at Children’s Hospital was 7.2 days. There were no readmissions for respiratory problems or infections. In all Jeune’s cases, the procedure was done on both sides, with the procedures done separately from 6 months to 1 year apart.
Follow-up ranged from 8 months to 7 years. At the time of follow-up, surviving patients were more functional in activities of daily living. All of the children had better exercise tolerance, and none had trouble with frequent pulmonary infections. Oxygen requirements decreased, as did respiratory episodes. In only 1 of our earliest patients has there been a recent, slow increase in symptomatology, suggesting he may be beginning to outgrow the repair. The chest roentgenographs taken at follow-up show riblike calcification in the region of the subperiosteal rib advancement. Physical examination typically reveals a normal consistency to the chest wall in the operated area.
Figure 3 shows typical changes on the plain chest roentgenogram as a result of the LTE procedure. Figure 3A shows a typical preoperative chest film, and Figure 3B shows typical postoperative changes.
Figure 4 demonstrates computed tomography changes resulting from the LTE procedure. Figure 4A presents a preoperative computed tomographic slice of the chest performed at full inspiration using suspended respiration technique. Figure 4B presents a postoperative computed tomography performed through the same area, using the same technique. Notice the rib fixation on the right. There has been expansion both laterally and anterior to posterior. The left side does not demonstrate significant change.
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Fig 4. Changes in computed tomography. (A) Preoperative computed tomography in inspiration. (B) Postoperative computed tomography with same technique through the same area.
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Table 3 demonstrates the changes in functional volume measurements after LTE in 3 patients who had both preoperative and postoperative studies. Measurements on patient F were made before and 3.5 and 5 months postoperatively. Patient I was studied 10.5 months after his first LTE. Patient J was restudied in the immediate postoperative period, only 9 days after LTE. It can be appreciated that measured volumes increased after LTE in patients F and I but decreased in patient J. It is likely that the reduction of volumes in patient J reflected the immediate postoperative timing of the study, because 6 months later the patient has had a 50% reduction in ventilator requirements.
Thoracic air space volumes measured by computed tomographic imaging both preoperatively and postoperatively were accomplished in 5 patients. These results are shown in Table 4. Volumes increased in 4 of 5 patients after the operation. These increases in volume ranged from 16% to 93%. As was observed in the physiologic data, thoracic air space volume decreased in patient J. This decrease may be related to the fact that studies were done before complete recovery from the LTE.
All of the families contacted were happy with the outcome after the procedure. Five of the 9 families reported no serious problems postoperatively. Of the 4 who stated that they had complications, 2 were those patients previously discussed who experienced symptomatic plate fractures. The other 2 were the families of the patients who died. There was a unanimous consensus among the families that they would recommend this procedure to others with the same disease. They all said that the patient’s quality of life was improved.
The most important long-term complication has been fracture of the titanium miniplates, which occurred in 7 patients. In 5 the fractures were asymptotic and only noted on late chest films. In 2 patients, a portion of the fractured plate stuck up against the back of the skin, causing pain. Removal of the offending fragments as an outpatient procedure solved the problem. In 6 of the 7 patients with fractured plates, the expansions were maintained despite the fractures, suggesting the fractures occurred late after healing of the repair and that the plates were no longer required to maintain the expansion. However, in patient I the fractures occurred in the first week, and there was a loss of expansion. We reoperated on this patient, reexpanded the segment, and secured the expansion with much larger plates (Mandibular Modular Fixation System, 2.4/3.0 locking reconstruction plate; Synthas Systems, Paoli, PA). These larger struts have yet to fracture at 6 months. We currently favor the use of the larger plates.
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Comment
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Jeune’s syndrome is an infrequently occurring disorder with varying degrees of severity, and the entire spectrum is represented in this series. As one would expect, the more severe manifestations of the disease present earlier in life. In each of our patients, medical treatment modalities including intravenous antibiotics and various forms of mechanical ventilation had become less effective, and an operation was recommended to halt the progress or reverse the disease. Pathologic studies suggest that alveolar growth potential may be normal in these children and that pulmonary changes are secondary to the severe chest wall hypoplasia. One would hope that surgical expansion of the thorax would allow for further growth of the lungs and improve the respiratory status of the afflicted children. Although these data do not prove that hypothesis, there is suggestive evidence that capacity can be improved.
We believe the LTE has several theoretical advantages. Unlike other approaches, the repairs actually heal with bone-to-bone healing reinforced by regeneration of bone in newly exposed periosteum, thus actually increasing the total size of the thoracic wall entirely with living tissue. This is demonstrated by the maintenance of the expansion in multiple cases of late strut fracture. Therefore, indefinite integrity of the titanium is not required for success. Nonetheless, based on the acute fracture experienced in our last patient, we are recommending use of the considerably larger struts. The fact that significant expansion can be obtained laterally means that the expansion potential is twice what can be obtained by a sternal approach, because, as we demonstrated, the procedure can easily be performed bilaterally. To date we have arbitrarily staged the procedures 1 year apart, except for patient I. At this point we have no evidence to suggest the optimal timing of the staging and continue to evaluate on a case by case basis.
Although the expanded segment is entirely living tissue, we do not have evidence with respect to actual growth over time. Longer follow-up will be necessary to answer that question. However, theoretically, a second procedure could be performed at a later date on the oher side, although that is yet to be accomplished.
Our experience in infants makes us less enthusiastic for patients younger than 1 year of age who are being ventilated. We believe that underlying lung disease, particularly malacic airway disease, is not helped by the procedure and will progress. Therefore, we believe that there must be imaging evidence of normal underlying lung to approach these patients surgically. In the 1 infant with normal lungs to start, the follow-up is too short to speculate.
In summary, we believe the LTE is a safe and effective method of treating symptomatic patients with Jeune’s syndrome older than 1 year of age. Patients can be expected to have symptomatic improvement, and, in some cases, a measurable increase in lung capacity. Further testing and longer follow-up of these patients will be necessary to provide more objective evidence of the success of the LTE.
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Acknowledgments
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The authors acknowledge the contributions of Anthony Baker for creating the illustrations and Mary Lou Naftzger for editorial and database assistance.
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References
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-
Jeune M., Carron R., Beraud C., Loaec Y. Polychondrodystrophie avec Blcage Thoracique d’ Evolution fatale. Pediatrie 1954;9:390-392.[Medline]
-
Jeune M., Beraud C., Carron R. Dystrophie thoracique asphyxiante de caractere familial. Arch Fr Pediatr 1955;12:886-891.[Medline]
-
Langer L.O. Thoracic pelvic phalangeal dystrophy: asphyxiating thoracic dystrophy of the newborn, infantile thoracic dystrophy. Radiology 1968;91:447-456.
-
Herdman R.C., Langer L.O. The thoracic asphyxiant dystrophy and renal disease. Am J Dis Child 1977;52:192-201.
-
Oberklaid F., Danks D.M., Mayne V., Campbell P. Asphyxiating thoracic dysplasia. Arch Dis Child 1977;52:758-765.[Abstract/Free Full Text]
-
Ravitch M.M. Congenital deformities of the chest wall and their operative correction. Philadelphia: WB Saunders, 1977:273-284.
-
Borland L.M. Anesthesia for children with Jeune’s syndrome (asphyxiating thoracic dystrophy). Anesthesiology 1987;66:86-88.[Medline]
-
Fruchter Z., Enachescu I. Asphyxiating thoracic dystrophy (Jeune’s syndrome). Radiological verification. Paediatrician 1969;18:46-50.
-
Tahernia A.C., Stamps P. "Jeune’s syndrome" (asphyxiating thoracic dystrophy). Clin Pediatr 1977;16:903-907.
-
Schmidt R., Pajewshi M., Mundel G. Unusual features in familial asphyxiating thoracic dysphasia (Jeune’s disease). Clin Genet 1972;3:90-98.[Medline]
-
Canepa G., Saputo V., Gallina M., et al. La distrofia toracica di Jeune. Chir Organi Mov 1978;64:11-49.[Medline]
-
Williams A.J., Vawter G., Reid L.M. Lung structure in asphyxiating thoracic dystrophy. Arch Pathol Lab Med 1984;108:658-661.[Medline]
-
Forrest P., Harkes A., D’Souza S.W. Hypoplastic lungs and abnormal phospholipids in asphyxiating thoracic dystrophy. Aust Pediatr J 1987;23:47-51.
-
Weber T.R., Kurkchubasche A.G. Operative management of asphyxiating thoracic dystrophy after pectus repair. J Pediatr Surg 1998;33:262-265.[Medline]
-
Barnes N.D., Hull D., Milner A.D., Waterston D.J. Chest reconstruction in thoracic dystrophy. Arch Dis Child 1971;46:833-837.
-
Karjoo M., Koop C.E., Cornfeld D., et al. Pancreatic exocrine enzyme deficiency associated with asphyxiating thoracic dystrophy. Arch Dis Child 1973;48:143-146.[Free Full Text]
-
Todd D.W., Tinguely S.J., Norberg W.J. A thoracic expansion technique for Jeune’s asphyxiating thoracic dystrophy. J Pediatr Surg 1986;21:161-163.[Medline]
-
Davis J.T., Ruberg R.L., Leppink D.M., et al. Lateral thoracic expansion for Jeune’s asphyxiating dystrophy: a new approach. Ann Thorac Surg 1995;60:694-696.[Abstract/Free Full Text]
-
Tepper R.S., Reister T. Forced expiratory flows and lung volumes in normal infants. Pediatr Pulmonol 1993;15:357-361.[Medline]
-
Castile R., Filbrun D., Flucke R., Franklin W., McCoy K. Adult-type pulmonary function in infants without respiratory disease. Pediatr Pulmonol 2000;30:215-237.[Medline]
-
Long F., Castile R., Brody A., et al. Lungs in infants and young children: improved thin-section computed tomography of the lungs in infants and young children using a non-invasive controlled-ventilation technique: initial experience. Radiology 1999;212:588-593.[Abstract/Free Full Text]
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Abstract
Introduction
