JBJS | Cervical Kyphosis in Patients Who Have Larsen Syndrome*

The Journal of Bone & Joint Surgery, Volume 78, Issue 4

Articles | April 01, 1996

Cervical Kyphosis in Patients Who Have Larsen Syndrome*

CHARLES E. JOHNSTON II, M.D.†; JOHN G. BIRCH, M.D†; JOHN L. DANIELS, M.D.‡, DALLAS, TEXAS

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Investigation performed at Texas Scottish Rite Hospital for Children, Dallas

The Journal of Bone & Joint Surgery. 1996; 78:538-45

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Abstract

Four patients who had Larsen syndrome and cervical kyphosis were managed operatively and followed for an average of seventy months (range, forty to ninety-two months). The preoperative cervical kyphosis ranged from 35 to 65 degrees. The patients had had a posterior cervical arthrodesis alone when they were infants, at an average age of fourteen months (range, ten to sixteen months). In three infants, the kyphosis either stabilized (one patient) or reversed into lordosis (two patients). Thus, the kyphosis corrected gradually by continued anterior growth in the presence of a solid posterior fusion. In the fourth infant, the kyphosis progressed to 110 degrees because of pseudarthrosis. This child had anterior decompression and arthrodesis for an acute neurological deficit.We believe that cervical kyphosis is sometimes present but not diagnosed in patients who have Larsen syndrome. Early diagnosis followed by operative stabilization should help such patients avoid neurological deficits. Posterior cervical arthrodesis alone, performed in infancy, provided stability and the opportunity for the gradual correction of the deformity by continued anterior growth in three of our four patients.

Figures in this Article

Introduction

Patients who have Larsen syndrome usually need staged orthopaedic management for congenital dislocations of the hip and knee as well as for deformities of the foot such as clubfoot, serpentine or so-called z-foot, or pes equinovalgus. Abnormalities of the cervical spine, specifically cervical kyphosis, were not emphasized in the original description of the syndrome⁵, but they may be potentially the most serious and, indeed, a life-threatening manifestation of the syndrome because of the impingement on the spinal cord at the apex of the kyphosis. However, with the exception of a few sporadic reports of the treatment of the cervical kyphosis^1,3,8-10, we found little information in the literature regarding this most hazardous and serious skeletal manifestation of Larsen syndrome.

Of nine patients with documented Larsen syndrome who were being followed at Texas Scottish Rite Hospital for Children, five were noted to have cervical kyphosis and were managed operatively. In the present report, we review the results of treatment, with an emphasis on the early recognition of cervical kyphosis and on the results of posterior arthrodesis, which was performed in four of these five patients.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, Texas 75219.

‡Crystal Lake Orthopaedic Surgery and Sports Medicine, Limited, 700 East Terra Cotta (Route 176), Crystal Lake, Illinois 60014-3606.

*No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. No funds were received in support of this study.

†Texas Scottish Rite Hospital for Children, 2222 Welborn Street, Dallas, Texas 75219.

‡Crystal Lake Orthopaedic Surgery and Sports Medicine, Limited, 700 East Terra Cotta (Route 176), Crystal Lake, Illinois 60014-3606.

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Figs. 1-A, 1-B, and 1-C: The patient, first seen in 1977, who had cervical kyphosis but was not included in our series because the kyphosis was not treated until he was twelve years old, at which time he had severe myelopathy. Fig. 1-A: Lateral radiograph, made when the patient was first seen at the age of two years and nine months, showing hypoplasia of the fifth cervical vertebra and spondylolysis of several segments.

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Fig. 1-B: Lateral radiograph, made with the cervical spine in the neutral position when the patient was twelve years old, showing the kyphosis, which measured 48 degrees. The patient had never walked independently and had recently become dependent on a wheelchair.

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T1-weighted magnetic resonance images showing severe impingement on the spinal cord.

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Figs. 2-A through 2-E: Case 1. The patient, first seen at the age of four days, had a 20-degree kyphosis from the third to the sixth cervical vertebra at the age of four months and was simply observed. Figs. 2-A and 2-B: Lateral radiographs, made when the patient was sixteen months old, showing progression of the kyphosis to 35 degrees (Fig. 2-A), with correction to 0 degrees in extension (Fig. 2-B).

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Figs. 2-A and 2-B: Lateral radiographs, made when the patient was sixteen months old, showing progression of the kyphosis to 35 degrees (Fig. 2-A), with correction to 0 degrees in extension (Fig. 2-B).

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Fig. 2-C: Lateral radiograph, made two months after the posterior arthrodesis, showing correction of the kyphosis to 4 degrees with immobilization in a halo vest.

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Fig. 2-D: Lateral radiograph, made six months postoperatively, showing a solid posterior fusion. However, the kyphosis had returned to its preoperative magnitude.

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Lateral radiograph, made six years postoperatively, showing normal cervical lordosis (21 degrees), with apparent extension of the fusion to include the fourth to the seventh cervical vertebra.

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Figs. 3-A through 3-F: Case 4. When first seen at the age of two weeks, the patient had a kyphosis that measured 30 degrees from the third to the sixth cervical vertebra. Fig. 3-A: Lateral radiograph, made when the patient was ten months old, showing progression of the kyphosis to 65 degrees, with correction to only 48 degrees in extension.

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Fig. 3-B: Lateral radiograph, made immediately after the posterior arthrodesis, showing the patient in the orthosis and correction of the kyphosis to 39 degrees.

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The first echo of a T2-weighted magnetic resonance imaging sequence that was made fifteen months postoperatively, showing severe impingement on the spinal cord. The kyphosis had progressed to 110 degrees, and the patient was quadriparetic after a fall.

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Fig. 3-D: Lateral radiograph, made one week after a decompressive vertebrectomy of the fourth cervical vertebra and an anterior arthrodesis with tibial strut-grafting, showing the position in the halo vest.

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Fig. 3-E: Lateral radiograph, made ten months postoperatively, showing non-union of the anterior fusion site with a dramatic increase in the kyphosis. The patient remained quadriparetic and hypotonic.

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Lateral radiograph, made one year after the second anterior decompression and arthrodesis with strut-grafting from the second to the seventh cervical vertebra, showing a solid fusion. The patient had recovery of neurological function.

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TABLE I DATA ON THE PATIENTS

*The kyphosis in extension is given in paretheses.

Case	Sex, Age (Mos.)	Level of Kyphosis	Preop. Kyphosis* (Degrees)	Operation	Type of Immobil.	Kyphosis (Degrees)		Duration of Follow-up (mos.)	Neurolog. Status
Postop.	At Latest Eval.	Preop.	Postop.
1	M, 16	C3-C6	35 (0)	Post. arthrodesis	Halo vest	4	-21	75	Normal	Normal
2	M, 16	C3-C6	37 (26)	Post. arthrodesis	Orthosis	31	18	72	Normal	Normal
3	M, 14	C3-C6	35 (-25)	Post. arthrodesis	Orthosis	35	-68	92	Normal	Normal
4	M, 10	C3-C6	65 (48)	Post. arthrodesis	Orthosis	39	110	15	Normal?	Quadriparetic
				Ant. arthrodesis, decomp.	Halo vest		145	13	Quadriparetic, hypotonic	Improved, then worsened
				Ant. arthrodesis, decomp.	Halo vest		20	12	Hypotonic	Improved, walking

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TABLE I DATA ON THE PATIENTS

*The kyphosis in extension is given in paretheses.

Case	Sex, Age (Mos.)	Level of Kyphosis	Preop. Kyphosis* (Degrees)	Operation	Type of Immobil.	Kyphosis (Degrees)		Duration of Follow-up (mos.)	Neurolog. Status
Postop.	At Latest Eval.	Preop.	Postop.
1	M, 16	C3-C6	35 (0)	Post. arthrodesis	Halo vest	4	-21	75	Normal	Normal
2	M, 16	C3-C6	37 (26)	Post. arthrodesis	Orthosis	31	18	72	Normal	Normal
3	M, 14	C3-C6	35 (-25)	Post. arthrodesis	Orthosis	35	-68	92	Normal	Normal
4	M, 10	C3-C6	65 (48)	Post. arthrodesis	Orthosis	39	110	15	Normal?	Quadriparetic
				Ant. arthrodesis, decomp.	Halo vest		145	13	Quadriparetic, hypotonic	Improved, then worsened
				Ant. arthrodesis, decomp.	Halo vest		20	12	Hypotonic	Improved, walking

Materials and Methods

From 1977 through 1994, nine patients were managed at our institution for orthopaedic deformities related to Larsen syndrome. All of them had the stigmata originally described by Larsen et al., including hypertelorism with a depressed nasal bridge, a spatulate thumb, multiple dislocations of joints, and deformities of the foot. All of the patients also had the accessory ossification center of the calcaneus, an essential radiographic finding for the diagnosis of Larsen syndrome⁴. Five of the patients (all male) had kyphosis of the cervical spine. Two other patients (also male) had spondylolisthesis of the seventh cervical on the first thoracic vertebra associated with an obvious spondylolysis of the seventh cervical vertebra; one of them had already had a posterior cervical arthrodesis at another institution. In addition, two patients (one male and one female) had no cervical kyphosis. One of the patients who had cervicothoracic spondylolisthesis and one who had no kyphosis had ossification anomalies of the cephalad part of the cervical spine, and they were followed for the possible development of instability^1,2.

The five patients who had cervical kyphosis all had deformity because of marked hypoplasia of one or two vertebral bodies (usually the fourth or fifth cervical vertebra, or both) at the apex of the kyphosis, combined with spondylolysis and spina bifida occulta of at least one cervical segment (Figs. 1-A, 2-A, and 3-A). With the exception of one patient who was first seen in 1977 at the age of two years and nine months, all of the patients who had cervical kyphosis were first diagnosed in infancy, between the ages of two and four months. All five of the patients had bilateral dislocation of the knee that was treated operatively. Four of the patients had unilateral or bilateral congenital dislocation of the hip that was treated with open reduction. All had deformities of the foot (bilateral clubfoot in four patients and pes equinovalgus in one patient) as well as various dislocations of the radiohumeral or ulnohumeral joints. None of the dislocations of the elbow were treated.

All five patients had operative treatment of the cervical kyphosis. Four patients who were diagnosed in infancy had posterior arthrodesis only, at an average age of fourteen months (range, ten to sixteen months). They form the basis of this report (Table I). The fifth patient (Figs. 1-A, 1-B, and 1-C), who was not included in this series, had cervical kyphosis when he was seen initially at the age of two years and nine months. However, this deformity was not treated until he was twelve years old, by which time he had severe myelopathy that necessitated anterior and posterior arthrodesis.

The four infants had a posterior arthrodesis performed with an autogenous bone graft from the iliac crest, followed by immobilization in either a halo vest (Case 1) or a Minerva-type custom orthosis for the occipital, cervical, thoracic, and lumbar spine (Cases 2, 3, and 4) for a minimum of three months. The patient who was managed with the halo vest had eight pins inserted at reduced torque (two to three inch-pounds [0.23 to 0.34 newton-meter]) and tolerated the immobilization without incident. The three patients who were managed with the custom orthosis needed frequent modifications of the orthosis and had problems with irritation of the skin and scalp, but the course of postoperative immobilization was completed for all and was believed to be satisfactory. There were no infections, acute perioperative neurological deficits, or other general complications.

Results

The preoperative kyphosis, measured from either the third or the fourth to the sixth cervical vertebra, ranged from 35 to 65 degrees in the four patients (Table I). The kyphosis could be substantially corrected by extension in two patients, decreasing from 35 to 0 degrees in one patient (Case 1) and from 35 degrees of kyphosis to 25 degrees of lordosis in the other (Case 3). The kyphosis could be only partially corrected by extension in the other two patients, decreasing from 37 to 26 degrees in one (Case 2) and from 65 to 48 degrees in the other (Case 4).

The initial postoperative position in the halo vest or the orthosis decreased the kyphosis to a variable degree in all patients, although correction was not vigorously attempted. Postoperatively, at an average of seventy months (range, forty to ninety-two months), three patients (Cases 1, 2, and 3) had a solid posterior fusion and one (Case 4) had a solid anterior fusion after three attempts. The kyphosis had reversed into lordosis in two patients (Cases 1 and 3), had stabilized at 18 degrees in one (Case 2), and had progressed dramatically in one (Case 4) who had had a pseudarthrosis after an attempted posterior arthrodesis and who needed two additional anterior arthrodeses.

It was of interest that the two patients (Cases 1 and 3) who had had substantial correction of the kyphosis in extension preoperatively had reversal of the deformity into lordosis at the time of the follow-up evaluation (at seventy-five and ninety-two months, respectively) and that the three patients (Cases 1, 2, and 3) who had had a successful posterior arthrodesis in infancy had a gradual correction of the kyphosis (either reversal into lordosis or, in Case 2, stabilization of the kyphosis at 18 degrees six years postoperatively). In the fourth patient (Case 4), the attempted posterior arthrodesis was followed by pseudarthrosis and progression of the kyphosis.

At the most recent follow-up examination, the neurological function of the three patients who had a solid posterior fusion was thought to be normal, despite several operations on the lower extremities with incomplete correction of the deformities in some instances. Transient quadriparesis developed, after a fall, in the patient (Case 4) who had progressive kyphosis and pseudarthrosis. He had two additional anterior decompressions and arthrodeses. At the most recent examination, the kyphosis appeared to be stabilized at 20 degrees and, coincidentally, the patient had begun to walk.

The patient who was first seen in 1977 and was not included in this series because of the long delay in the treatment of the kyphosis had definite myelopathy and weakness preoperatively. We believe that the case of this patient probably represents the natural history of the untreated deformity (Figs. 1-A, 1-B, and 1-C ). After anterior and posterior arthrodesis, the neurological function of the patient had not changed or improved.

Case Reports

CASE 1. A four-day-old male infant was found to have dislocation of the knee bilaterally, clubfeet, a 90-degree flexion contracture of both elbows with dislocated radial heads, and the typical facies of Larsen syndrome. The patient's father had a similar facial appearance and had been managed for dislocated knees and clubfeet when he was a child. When the patient was two months old, the dislocations of the knees were successfully reduced operatively. Radiographs of the cervical spine, made when he was four months old, demonstrated mild dysplasia and 20 degrees of kyphosis from the third to the sixth cervical vertebra. The kyphosis was simply observed at that time. The clubfeet were corrected operatively when the patient was one year old.

When he was sixteen months old, cervical radiographs demonstrated progression of the kyphosis to 35 degrees, correcting to 0 degrees in extension (Figs. 2-A and 2-B). There was no detectable neurological deficit. A magnetic resonance image demonstrated mild anterior impingement on the dural sac. The patient had posterior in situ arthrodesis from the third to the seventh cervical vertebra with an autogenous bone graft from the iliac crest. Postoperative positioning in a halo vest corrected the kyphosis to 4 degrees (Fig. 2-C). At six months postoperatively, the posterior fusion appeared well incorporated, although the kyphosis had collapsed back to the original degree of deformity after the removal of the halo vest (Fig. 2-D). Six years postoperatively, because of the continued anterior growth in the presence of a posterior fusion, the patient had a cervical lordosis that measured 21 degrees (Fig. 2-E). The patient was fully active and asymptomatic, and the neurological function was normal.

CASE 4. A male infant who had the typical facies of Larsen syndrome, severe flexion contracture of the elbows, ulnohumeral dislocations, bilateral dislocation of the hip and knee, and clubfeet was first seen when he was two weeks old. Cervical kyphosis was also diagnosed. However, because he had had numerous recurrent respiratory infections and an uncertain prognosis, no operative treatment was possible until the patient was ten months old. At that time, the kyphosis measured 65 degrees from the third to the sixth cervical vertebra (Fig. 3-A). Posterior arthrodesis from the third to the sixth cervical vertebra was performed, and the kyphosis was corrected to 39 degrees with use of a custom orthosis extending to the trunk (Fig. 3-B). Staged open reductions of the knees and hips were then performed. However, by fifteen months after the operation, the kyphosis had progressed dramatically to 110 degrees because of pseudarthrosis and the inadequate extent of the posterior arthrodesis. At that time, the patient fell from a highchair and was transiently quadriparetic (Fig. 3-C). He had an anterior decompression, on an urgent basis, with a complete vertebrectomy of the body of the fourth cervical vertebra, and an anterior arthrodesis was performed from the third to the fifth cervical vertebra with a tibial bone graft. The kyphosis was reduced to 75 degrees (Fig. 3-D). However, despite immobilization in a halo vest, the anterior arthrodesis failed because of resorption of the graft, with dramatic progression of the kyphosis again, to 145 degrees (Fig. 3-E). The patient remained hypotonic compared with the neurological status before the fall. Therefore, he had a repeat anterior decompression of the third to the sixth cervical vertebra and arthrodesis from the second to the seventh cervical vertebra with a fibular strut graft. One year after this operation, the fusion appeared solid and the kyphosis had decreased to 20 degrees (Fig. 3-F). The patient walked for the first time in his life.

Discussion

The orthopaedic manifestations of Larsen syndrome are well known and are usually not difficult to recognize because of the multiple dislocations of the joints (hips, knees, and elbows), the deformities of the foot (equinovarus or equinovalgus), and the dysmorphic facies, which were all originally described in 1950^4,5. However, with the exception of the two patients described by Micheli et al., the one patient each reported on by Bellon and Filipe, Lefort et al., and Francis and Noble, and the quadriparetic patient discovered in retrospect by Muzumdar et al., kyphosis of the cervical spine, noted in five of our nine patients, has not been identified or discussed, to our knowledge. Indeed, Laville et al. reviewed the cases of thirty-eight patients with Larsen syndrome who came from an isolated geographical area and did not find any who had cervical kyphosis. In that series, however, the absence of the accessory calcaneal apophysis, which is a characteristic radiographic finding in classic Larsen syndrome, casts doubt on the diagnosis and, hence, on the validity of the failure to observe cervical deformity. Although spina bifida occulta, spondylolysis, and segmentation defects have been previously identified^2,4-6,12,15, there is no explanation for the increased frequency of cervical kyphosis in our series unless it is assumed that this deformity has been underdiagnosed⁴.

The potential morbidity and mortality from this deformity are obvious. Indeed, one of the patients of Micheli et al. died at the age of twenty-six months specifically as a result of compression of the spinal cord before the deformity of the neck had been treated. Also, one of the original patients of Larsen et al. died at the age of one year after open reduction of a dislocation of the knee. The death was attributed to a so-called respiratory anesthetic complication. The latter patient had had an apparently uneventful operative reduction of the contralateral knee one month before the fatal procedure. Although tracheomalacia and bronchomalacia have been documented in patients who have Larsen syndrome^11,13,14, and although early death from other cardiopulmonary anomalies is not uncommon in these patients^4,7, it may be reasonably speculated that manipulation of an undiagnosed kyphosis in the neck of the patient while he was under anesthesia may have directly contributed to the perioperative death^1,9. Quadriplegia may also occur after trauma in patients who have kyphosis, as it did in one of our patients (Case 4), or in those who have atlanto-axial instability^1,4,10.

Many patients who have Larsen syndrome are described as being hypotonic, a condition that contributes to a delay in the achievement of motor skills, such as the ability to walk. Hyperlaxity is a central feature of the syndrome and of the characteristic dislocations of the joints. Because dislocations of the major joints of the lower extremity, particularly anterior dislocation of the knee, are essentially always present, it is easy to attribute the delay in walking and in the achievement of other developmental milestones to the hypotonia, the hyperlaxity with instability, and the multiple orthopaedic deformities. However, the reason that the patient who was first seen at our institution in 1977 and who had cervical kyphosis that was neglected until he was twelve years old was not able to walk independently was most probably a chronic myelopathy that had been present for years before it was recognized. The myelopathy was not recognized until the patient, who had been able to walk with use of braces, had further deterioration of the ability to walk at the age of twelve years (Figs. 1-B and 1-C). We believe that the hypotonia that is characteristic of Larsen syndrome may be a result of early chronic compression of the spinal cord that remains undetected because of the absence of obvious long-tract signs, its low-grade chronicity, and the attention drawn by the often dramatic dislocations of the joints and deformities of the feet.

The three infants (Cases 1, 2, and 3) who had had a successful arthrodesis when they were sixteen months old or less were all active and walked independently at the most recent follow-up examination, despite the deformities of the lower extremities. The cervical spine of two of these patients (Cases 1 and 3) had changed to a lordotic alignment. We continue to follow these patients for possible overcorrection into hyperlordosis as a result of the potential anterior growth remaining. To our knowledge, progressive cervical hyperlordosis due to continued anterior growth and its neurological consequences have not been reported. The one infant (Case 4) in whom an attempted posterior arthrodesis failed had had a more rigid and severe kyphosis, with less correction in extension, than the other patients. In retrospect, the arthrodesis may not have been performed at an adequately cephalad level of the spine, as the kyphosis subsequently progressed to include the second cervical vertebra. In this patient, the kyphosis progressed for fifteen months, presumably because of pseudarthrosis, at which time an acute quadriparesis resulting from a fall necessitated immediate anterior decompression for a neurological deficit. This kyphosis was not stabilized, despite the posterior arthrodesis when the patient was ten months old and the anterior arthrodesis when the patient was twenty-five months old. The performance of a second, more extensive decompression and strut-grafting achieved stability and, concomitantly, neurological recovery (Figs. 3-C, 3-D, 3-E through 3-F).

Initial treatment of the cervical kyphosis with an anterior procedure is less attractive for very young children who have Larsen syndrome because anterior growth and, hence, the potential for correction are eliminated. Theoretically, late stenosis of the cervical canal could occur after early circumferential fusion, by virtue of the lack of enlargement of the diameter of the vertebral column after such arrest of growth. For patients in whom myelopathy has been established, anterior decompression may be necessary to obtain maximum neurological recovery. However, early anterior arthrodesis in patients who have no neurological deficit essentially produces fusion in situ in a degree of kyphosis and eliminates possible subsequent correction by anterior growth. Such a kyphosis theoretically would follow a course of chronic myelopathy similar to that in our patient who had a deformity that was neglected until he was twelve years old (Figs. 1-A, 1-B, and 1-C). The chronic myelopathy and quadriparesis that developed in the patient represent the natural history of cervical kyphosis.

In conclusion, the prevalence of cervical kyphosis in Larsen syndrome is probably underestimated. Because of its inherent ability to produce neurological morbidity and mortality by as young an age as two years, diagnosis of this deformity should be a priority in the initial evaluation of patients who have this syndrome. Although other causes of myelopathy, such as atlanto-axial instability, have been reported¹, cervical kyphosis can be identified on a lateral radiograph in a very young infant. No dynamic studies or cooperation by the patient are necessary. When early posterior arthrodesis of the cervical spine was successful in our patients (Cases 1, 2, and 3), anatomical and functional results were optimum. Before the development of a severe rigid kyphosis or myelopathy, or both, posterior arthrodesis for cervical kyphosis should take precedence in the staging of the multiple orthopaedic procedures that are necessary for a patient who has Larsen syndrome.

References

Bellon, J. M., and |and |Filipe, G.: Problèmes rachidiens recontrés au cours du syndrome de Larsen. A propos de 3 cas. Rev. chir. orthop,73: 57-62, 1987.7357 1987 [PubMed]

Bowen, J. R.; Ortega, K.; Ray, S.; and |and |MacEwen, G. D.: Spinal deformities in Larsen's syndrome. Clin. Orthop.,197: 159-163, 1985.197159 1985 [PubMed]

Francis, W. R., Jr., and |and |Noble, D. P.: Treatment of cervical kyphosis in children. Spine,13: 883-887, 1988.13883 1988 [PubMed][CrossRef]

Goldberg, M. J.: The Dysmorphic Child: an Orthopedic Perspective, pp. 10-16. New York, Raven Press, 1987.

Larsen, L. J.; Schottstaedt, E. R.; and |and |Bost, F. C.: Multiple congenital dislocations associated with characteristic facial abnormality. J. Pediat.,37: 574-581, 1950.37574 1950 [PubMed][CrossRef]

Latta, R. J.; Graham, C. B.; Aase, J.; Scham, S. M.; and |and |Smith, D. W.: Larsen's syndrome: a skeletal dysplasia with multiple joint dislocations and unusual facies. J. Pediat,78-A: 291-298, 1971.78-A291 1971 [CrossRef]

Laville, J. M.; Lakermance, P.; and |and |Limouzy, F.: Larsen's syndrome: review of the literature and analysis of thirty-eight cases. J. Pediat. Orthop.,14: 63-73, 1994.1463 1994 [CrossRef]

Lefort, G.; Mourad, H.; de Niscault, G.; and |and |Daoud, S.: Dislocation du rachis cervical supérieur dans le syndrome de Larsen. Chir. Pédiat.,24: 211-212, 1983.24211 1983

Micheli, L. J.; Hall, J. E.; and |and |Watts, H. G.: Spinal instability in Larsen's syndrome. Report of three cases. J. Bone and Joint Surg.,58-A: 562-565, June 1976.58-A562 1976

Muzumdar, A. S.; Lowry, R. B.; and |and |Robinson, C. E.: Quadriplegia in Larsen syndrome. Birth Defects,13: 202-211, 1977.13202 1977 [PubMed]

O'Herlihy, C., and |and |O'Brien, N.: Neonatal stridor with Larsen's syndrome. Israel J. Med. Sci.,13: 912-914, 1977.13912 1977 [PubMed]

Oki, T.; Terashima, Y.; Murachi, S.; and |and |Nogami, H.: Clinical features and treatment of joint dislocations in Larsen's syndrome. Report of three cases in one family. Clin. Orthop.,119: 206-210, 1976.119206 1976 [PubMed]

Rock, M. J.; Green, C. G.; Pauli, R. M.; and |and |Peters, M. E.: Tracheomalacia and bronchomalacia associated with Larsen syndrome. Pediat. Pulmonol.,5: 55-59, 1988.555 1988 [CrossRef]

Ronningen, H., and |and |Bjerkreim, I.: Larsen's syndrome. Acta Orthop. Scandinavica,49: 138-142, 1978.49138 1978 [CrossRef]

Steel, H. H., and |and |Kohl, E. J.: Multiple congenital dislocations associated with other skeletal anomalies (Larsen's syndrome) in three siblings. J. Bone and Joint Surg.,54-A: 75-82, Jan. 1972.54-A75 1972

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T1-weighted magnetic resonance images showing severe impingement on the spinal cord.

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Fig. 2-C: Lateral radiograph, made two months after the posterior arthrodesis, showing correction of the kyphosis to 4 degrees with immobilization in a halo vest.

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Fig. 2-D: Lateral radiograph, made six months postoperatively, showing a solid posterior fusion. However, the kyphosis had returned to its preoperative magnitude.

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Lateral radiograph, made six years postoperatively, showing normal cervical lordosis (21 degrees), with apparent extension of the fusion to include the fourth to the seventh cervical vertebra.

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Fig. 3-B: Lateral radiograph, made immediately after the posterior arthrodesis, showing the patient in the orthosis and correction of the kyphosis to 39 degrees.

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TABLE I DATA ON THE PATIENTS

*The kyphosis in extension is given in paretheses.

Case	Sex, Age (Mos.)	Level of Kyphosis	Preop. Kyphosis* (Degrees)	Operation	Type of Immobil.	Kyphosis (Degrees)		Duration of Follow-up (mos.)	Neurolog. Status
Postop.	At Latest Eval.	Preop.	Postop.
1	M, 16	C3-C6	35 (0)	Post. arthrodesis	Halo vest	4	-21	75	Normal	Normal
2	M, 16	C3-C6	37 (26)	Post. arthrodesis	Orthosis	31	18	72	Normal	Normal
3	M, 14	C3-C6	35 (-25)	Post. arthrodesis	Orthosis	35	-68	92	Normal	Normal
4	M, 10	C3-C6	65 (48)	Post. arthrodesis	Orthosis	39	110	15	Normal?	Quadriparetic
				Ant. arthrodesis, decomp.	Halo vest		145	13	Quadriparetic, hypotonic	Improved, then worsened
				Ant. arthrodesis, decomp.	Halo vest		20	12	Hypotonic	Improved, walking

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TABLE I DATA ON THE PATIENTS

*The kyphosis in extension is given in paretheses.

Case	Sex, Age (Mos.)	Level of Kyphosis	Preop. Kyphosis* (Degrees)	Operation	Type of Immobil.	Kyphosis (Degrees)		Duration of Follow-up (mos.)	Neurolog. Status
Postop.	At Latest Eval.	Preop.	Postop.
1	M, 16	C3-C6	35 (0)	Post. arthrodesis	Halo vest	4	-21	75	Normal	Normal
2	M, 16	C3-C6	37 (26)	Post. arthrodesis	Orthosis	31	18	72	Normal	Normal
3	M, 14	C3-C6	35 (-25)	Post. arthrodesis	Orthosis	35	-68	92	Normal	Normal
4	M, 10	C3-C6	65 (48)	Post. arthrodesis	Orthosis	39	110	15	Normal?	Quadriparetic
				Ant. arthrodesis, decomp.	Halo vest		145	13	Quadriparetic, hypotonic	Improved, then worsened
				Ant. arthrodesis, decomp.	Halo vest		20	12	Hypotonic	Improved, walking