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SPINE Volume 28, Number 8, pp 799–805

©2003, Lippincott Williams & Wilkins, Inc.

The Efficacy of Convex Hemiepiphysiodesis in Patients


With Iatrogenic Posterior Element Deficiency Resulting
from Diastematomyelia Excision

Akin Uzumcugil, MD,* Akin Cil, MD,* Muharrem Yazici, MD,* Emre Acaroglu, MD,*
Ahmet Alanay, MD,* Nejat Akalan, MD,† Pınar Ozisik, MD,† and Adil Surat, MD*

Study Design. Retrospective analysis was performed. efficacy of the technique. [Key words: congenital, convex
Objective. To investigate the safety and efficacy of hemiepiphysiodesis, diastematomyelia, intraspinal pa-
anterior and posterior hemiepiphysiodesis in patients thology, scoliosis, surgery, treatment] Spine 2003;28:
with iatrogenic posterior element deficiency. 799 – 805
Summary of Background Data. Anterior and posterior
convex hemiepiphysiodesis is a well-accepted treatment
method for severe and progressive congenital scoliosis
in young children. Many patients with congenital spinal The longitudinal growth imbalance of anomalous verte-
deformities have intraspinal pathologies that require bra forms the basis of congenital scoliosis. Imbalance
neurosurgical intervention with laminectomy. The effi- originates from normal or nearly normal growth of the
cacy of this method has not been studied in these pa- convex side with respect to the concave side, which has
tient populations.
Methods. Between 1990 and 2001, among 82 patients
little, if any, growth potential. Convex anterior and pos-
with congenital spinal deformity, 38 were treated with terior hemiepiphysiodesis has been a well-accepted treat-
convex epiphysiodesis. This study included 18 of these ment method for stopping the progression or even re-
patients (2 males and 16 females) who underwent di- versing the deformity associated with congenital
astematomyelia excision and had at least 2 years of fol-
scoliosis, provided there is proper adherence to the pre-
low-up evaluation. Diastematomyelia excision was per-
formed before the orthopedic procedure in 8 patients and requisites.1–7 The ideal patient has a pure scoliotic curve
at the same anesthetic setting sequentially in 10 patients. without major kyphosis or lordosis, a progressive curve
The mean age at the time of the fusion was 20 months with a magnitude less than 70°, a curve length consisting
(range, 6 – 60 months), and the average follow-up period of five segments or fewer, an age less than 5 years, no
was 39 months (range, 24 –120 months).
Results. The mean Cobb angle was 54° (range, 31–90°)
cervical spine involvement, and no unilateral unseg-
before surgery and 48° (range, 30 – 84°) at the final fol- mented bar as the etiology of the curve.5,7 Occasionally,
low-up assessment. Any increase of more than 6° was the classic indications have been stretched in terms of age
accepted as progression. Eight patients (44%) had a true at surgery, magnitude of the curve, and etiology with
epiphysiodesis effect: 58° (range, 40 –90°) before sur-
successful outcomes.1,3,6
gery and 39° (range, 30 –70°) at follow-up evaluation.
Eight patients (44%) had a fusion effect: 49° (range, There is a high likelihood of associated anomalies
31– 68°) before surgery and 50° (range, 37–74°) at fol- both inside and outside the spinal column in congenital
low-up assessment. Two patients (12%) had a postop- scoliosis because the developments of the spinal cord and
erative progression of deformity: 63°(range, 54 –72°) vertebral column are closely associated. Diastematomy-
before surgery and 75° (range, 65– 84°) at follow-up
evaluation.
elia, one of the probable coexisting congenital malfor-
Conclusions. Convex epiphysiodesis is an effective mations of the neural axis, is reported to have an inci-
method for patients with midline laminectomy defect as dence of 1.8% to 16% in patients with congenital
those with intact posterior elements. Because the facet scoliosis.8 –11 Diastematomyelia is a sagittal division of a
joints and transverse processes usually are unaffected,
spinal cord or cauda equina segment with a fibrocarti-
the presence of midline defect does not diminish the
laginous or bone spur that protrudes from the posterior
midline portion of one or more vertebral bodies.11–14 To
avoid creating a neural deficit or increasing the preexist-
ing neural deficit, laminectomy to remove the bone spur
From the Departments of *Orthopaedics and †Neurosurgery, Hacet- is indicated for patients with diastematomyelia before
tepe University, Ankara, Turkey. any procedure for correction of the vertebral column
Acknowledgment date: June 11, 2002.
First revision date: August 9, 2002. that might cause traction on the spinal cord.9,11–15
Acceptance date: September 30, 2002. For an effective posterior fusion, availability of poste-
Device status/drug statement: The submitted manuscript does not con- rior elements is an important advantage. One of the rea-
tain information about medical devices or drugs.
Conflict of interest: No funds were received in support of this work. No sons for the increased pseudarthrosis rate associated
benefits in any form have been or will be received from a commercial with posterior fusion in spina bifida ranging from 42%
party related directly or indirectly to the subject of this article. to 76% is thought to originate from inadequate bed for
Address reprint requests to Muharrem Yazici, MD, Department of
Orthopaedics, Hacettepe University, Sihhiye, Ankara, 06100 Turkey. bone grafts.16 –18 The efficacy of convex growth arrest,
E-mail: yazioglu@hacettepe.edu.tr. which is directly proportional to the quantity and

799
800 Spine • Volume 28 • Number 8 • 2003

strength of the posterior fusion mass, in patients with anterior part of the surgery, half of the convex side disc and
iatrogenic posterior element deficiency is unknown. endplates was removed. The corresponding spaces were filled
The purpose of this study was to investigate the safety with chip grafts obtained from the ribs. If one rib was not
and efficacy of convex anterior and posterior he- sufficient to fill in these spaces, an additional rib was taken.
miepiphysiodesis in patients with iatrogenic posterior el- Determined by the presenting anomaly, arthrodesis generally
extended at least one level above and one level below the anom-
ement deficiency.
alous segments posteriorly and along the full length of the curve
anteriorly. Supplemental instrumentation was not used. A
Methods body cast was applied 4 to 7 days after the operation and
Between 1990 and 2000, among 82 consecutive patients with removed at a minimum of 4 months (range, 4 – 6 months).
the diagnosis of congenital scoliosis, 38 were treated with con-
vex growth arrest at our institution. Inclusion criteria specify a
diagnosis of congenital scoliosis without significant kyphosis Results
or lordosis, a curve magnitude greater than 50° or a docu- The profile of the patients is presented in Table 1. In this
mented progression or a pattern known to be progres-
study, 18 patients with congenital scoliosis and concom-
sive,10,19,20 and concomitant diastematomyelia. Excision of di-
astematomyelia and the convex growth arrest should have been
itant diastematomyelia underwent spur excision and an-
performed before the age of 5 years. The patients with pure terior and posterior convex hemiepiphysiodesis and
cervical congenital scoliosis and those with myelomeningocele hemiarthrodesis.
were not included. A total of 18 patients (16 females and 2 The primary symptoms manifested in the children
males) fulfilled the criteria. Their mean age at the time of con- were spinal deformity in 14 patients, foot deformity in 2
vex growth arrest was 20 months (range, 6 – 60 months), and patients, gait disturbance in 1 patient, and back pain and
the average follow-up period was 39 months (range, 24 –120 hairy patch in 1 patient. Cutaneous abnormalities over-
months). lying the spine were present in 10 patients (56%). These
Congenital vertebral anomalies were diagnosed on the su-
included hairy patches in five patients, dermal sinuses in
pine anteroposterior and lateral spine radiographs. The sites of
vertebral anomalies and resultant sites of curvatures were four patients (2 also had hairy patches), hyperpigmented
noted. Deformities were classified according to Winter et al20 nevus in one patient, sacral dimple in one patient. Four
as unsegmented bar with or without contralateral hemiverte- patients (22%) had neurologic abnormalities including
bra, block vertebra, hemivertebra, wedge vertebra, or complex paraparesis in two patients, mild left lower extremity
congenital deformities. Additional vertebral anomalies at other paresis and urinary incontinence in one patient, and
levels that did not contribute to the spinal deformity were not weakness of foot intrinsics in one patient. Sensory and
recorded. Cobb angles21,22 were measured on the anteroposte- motor testing was difficult in this age population, so
rior radiographs of the spine at the initial visit and at the fol- some additional patients may have had sensory or motor
low-up visits every 6 months. As a result, any increase more
problems. Three patients (17%) had foot deformities in-
than 6° was accepted as a progression.22 All patients with con-
genital scoliosis underwent CT myelography or MRI to rule cluding clubfeet in two children and calcaneovalgus de-
out possible concomitant intraspinal anomaly before the sur- formity in one child. One child had acetabular dysplasia,
gery.8,15,23,24 Diastematomyelia was diagnosed in 6 of the pa- and one child had a horseshoe-shaped kidney.
tients using CT myelography and 12 of the patients using MRI. Ten of the patients had complex vertebral anomalies;
The levels of spurs and other intraspinal anomalies, if any, were four patients had unilateral unsegmented bar and con-
recorded. tralateral hemivertebra; one patient had unilateral un-
Ten of the patients sequentially underwent surgery with segmented bar, and three patients had hemivertebra.
neurosurgeons in a single anesthetic setting. Spur excision was
Eight patients (44%) had lumbar spurs; four (22%)pa-
performed via laminectomy, followed by posterior convex he-
tients had thoracic spurs; and seven patients (39%) had
miepiphysiodesis. Anterior convex hemiepiphysiodesis was
performed 1 week to 10 days later. In the remaining eight thoracolumbar spurs. Among the 18 patients, only 1 pa-
patients, laminectomy and spur excision had been performed tient (6%) had double spurs. Eleven of the spurs were
10 days to 5.5 months before the single-anesthetic sequential located at or near the apex of the curve formed by con-
anterior and posterior convex hemiepiphysiodesis. During the genital vertebral anomalies. In 7 of the 18 patients, the
neurosurgical part of the operation, spur excision was per- spur was located one or more level below the curve. To
formed first, then dural tube reconstructed. In some cases, re- reconstruct the dural tube in three of the patients and to
construction was required to extend laminectomy one or more excise the dermal sinuses in four of the patients, laminec-
levels above. Four patients had concomitant dermal sinuses, tomy was extended to the curve, resulting in posterior
which were excised. The operative technique used for convex element deficiency within the fusion area. On the aver-
growth arrest was identical to that described by Winter et al,7
age, three laminas (range, 2–5) had to be removed for
except for the trough on the lateral side of the vertebral bodies
that accommodates the autogenous rib graft, which was not
excision of the spur or dural tube reconstruction. Spina
used in these patients. Posterior elements at the convex side, bifida occulta was seen in almost all of the patients.
including the remnants of the laminae, facet joints, and trans- However, in some patients, it was extensive, involving
verse processes were exposed subperiosteally. Inferior articular several vertebrae, whereas in others, it was small. Eleven
processes were excised, and articular cartilages were removed. patients had rib anomalies including fusion on the con-
After decortication, ample bone grafting was used. During the cave side.
The Efficacy of Convex Hemiepiphysiodesis • Uzumcugil et al 801

Table 1. Patient Profiles


Age at Level of Neurologic
Case Sex Anomaly Level of Curve Surgery (months) Diastematomyella Manifestations

1 F Complex T11–L4 10 L3 No
2 F UB⫹H T6–L1 13 T10 No
3 M Complex T8–L2 9 L3 Bilateral weak foot
intrinsics
4 F Complex T1–L3 60 T6 Left weakness,
urinary
incontinence
5 F UB⫹H T8–T12 11 L1 No
6 F H T5–T12 7 L4 No
7 F H T7–L1 18 T11–L1 No
8 M Complex T6–T10 9 T12–L1 No
9 F UB⫹H T4–L2 24 T10–12 No
10 F Complex T2–T8 24 T12–L1 No
11 F Complex T8–L5 24 L2 Paraparesis
12 F UB T5–T12 36 T5–7 No
13 F Complex T5–L2 20 DUAL (T11–12 No
and L1–2)
14 F UB⫹H T4–T8 21 L2 Paraparesis
15 F Complex T9–L2 6 T9 No
16 F Complex T4–T10 12 T12 No
17 F Complex T7–T12 14 L1 No
18 F H T12–L5 42 L3 No
UB ⫽ Unsegmented bar, H ⫽ Hemivertebra

The indications for the surgery were documented pro- Discussion


gression in 7 patients and magnitude of the curve (⬎50°)
at admission in 11 patients. The delineation of the natural history and the difficulties
The mean Cobb angle was 54° (range, 31–90°) before in correcting the rigid curves of the congenital scoliosis
surgery and 48° (range, 30 – 84°) at the final follow-up have led spine surgeons to attempt stopping the defor-
assessment. Because more than 6° difference is accepted mity before it becomes severe.1–7,15,19,20,25 For patients
as a change in curve magnitude, the patients fell into with congenital scoliotic deformity that becomes evident
three groups. Eight patients (44%) had a true epiphys- before the age of 5 years, there are some treatment op-
iodesis effect, with an average curve magnitude of 58° tions including hemivertebra excision, posterior fusion,
(range, 40 –90°) before surgery and 39° (range, 30 –70°) and convex growth arrest.
at follow-up evaluation (Figure 1 A and B). Eight pa- Hemivertebral excision seems to be the ideal treat-
tients (44%) had a fusion effect, with an average curve ment of choice because it results in acute and presumable
magnitude of 49° (range, 31– 68°) before surgery and correction in one stage. However, it has some significant
50° (range, 37–74°) at follow-up evaluation (Figure 2 A disadvantages. Klemme et al26 reported six children with
and B). Two patients (12%) had a progressive deformity an average age of 19 months (range, 13–33 months) who
with an average curve magnitude of 63° (range, 54 –72°) had undergone hemivertebra excision. In their series, the
before surgery and 75° (range, 65– 84°) at follow-up duration of surgery averaged 435 minutes, and the blood
evaluation (Table 2, Figure 3 A and B). The behavior of loss averaged 362 mL. In another study, hemivertebra
the compensatory curves followed that of the treated excision was demonstrated to have higher risks than
primary curves. Sagittal alignments within the operated
other procedures with regard to neurologic injury.27 The
segments were not observed to change.
main indication for excision is the hemivertebra at lum-
Three patients had a curve magnitude exceeding 70°
bar level (preferably at L3 or L4), which causes signifi-
(cases 6, 9, and 13), which constituted an extended indi-
cant decompensation that cannot be corrected by other
cation of the procedure. Two of these patients had a true
epiphysiodesis effect, and one patient had progression. means.25,28,29
Five patients had unilateral unsegmented bars (cases 2, 5, Winter and Moe30 reported their results from poste-
9, 12, and 14), four of which also had contralateral hemi- rior fusion in 32 patients with congenital scoliosis who
vertebra constituting an extended indication as well. One were younger than 5 years. Of the 32 patients, 12
of these patients had progression; two had a true (37.5%) were shown to demonstrate bending of fusion
epiphysiodesis effect; and two had fusion effects. mass even after the fusion was solid. Winter and Moe30
No complications occurred during spur removal or attributed this to weak fusion mass, and others attrib-
convex growth arrest. In none of the patients the neuro- uted it to crankshaft effect produced by anterior spinal
logic deficit progress or a new neurologic deficit occur growth in the presence of posterior tether created by the
after the operation. solid fusion mass.31,32
802 Spine • Volume 28 • Number 8 • 2003

Figure 1. A 35-month-old girl (case 5) with an unsegmented bar (thick arrow) and contralateral multiple hemivertebrae (thin arrows)
concomitant with rib fusions. The Cobb angle was 56° before surgery (A) and 38° at a 2-year follow-up assessment (B).

To negate late bending, convex growth arrest both formity or the age group that underwent surgery and
anteriorly and posteriorly had been considered a remedy the response to treatment. Also, in the literature, this
for this small age population of patients.1–7 Although a issue is not highlighted.1–7
number of series have reported the outcome of convex The treatment of unilateral unsegmented bar with
growth arrest, the great majority of the patients in these convex growth arrest was reported to be useless because
studies were not followed until skeletal maturity (Table it was believed to be impossible for the concavity of the
3). The reported epiphysiodesis effect ranges from 20% curve to grow.7,29 Winter6 operated on two patients with
to 77%. The fusion effect reportedly is 17% to 70%, and unilateral unsegmented bar and obtained a fusion effect
progression after this procedure reportedly is 6% to in both patients. Andrew and Piggott1 operated on three
21%.1,3,5–7 Recently, transpedicular anterior and pos- patients with unsegmented bar, two of which also had
terior convex growth arrest had been reported with contralateral hemivertebra, and achieved an epiphys-
success comparable to that for classic convex growth iodesis effect in two patients and a fusion effect in one
arrest3 (Table 3). patient. Keller et al3 operated on seven curves with uni-
In this series, 18 patients with congenital scoliosis and lateral unsegmented bar, and obtained fusion in five, but
diastematomyelia underwent spur excision and convex two patients progressed. In the current series, five pa-
anterior and posterior hemiepiphysiodesis at an average tients had unilateral unsegmented bar, four of whom had
age of 20 months and were followed for a minimum of 2 contralateral hemivertebra. An epiphysiodesis effect was
years. Among these patients, 44% had epiphysiodesis obtained in two patients and a fusion effect in two pa-
effect; 44% had fusion effect; and 12% deteriorated. The tients, whereas one patient experienced progression. As
88% success rate is comparable with that of the afore- the operation extends above and below the bar, there still
mentioned studies on convex growth arrest. However, is some possibility of correction, with growth, or at least
some questions still need to be answered, such as why a fusion effect, to be obtained.1
some patients responded well to the treatment and A curve magnitude less than 70° was reported to be
others did not. With a limited number of patients in one of the prerequisites for a successful convex growth
this study, statistically significant correlations could arrest.7 Winter6 operated on two patients with a curve
not be drawn between the type or location of the de- magnitude exceeding 70° and obtained a fusion effect in
The Efficacy of Convex Hemiepiphysiodesis • Uzumcugil et al 803

Figure 2. An 18-month-old girl (case 7). The curve was stabilized at 24 months after surgery. The Cobb angle was 40° before surgery (A)
and 42° at the final follow-up assessment (B).

both. Andrew and Piggott1 operated on one patient and a contralateral hemivertebra complicating the situation,
obtained a true epiphysiodesis effect. Keller et al3 oper- experienced progression. Although the number of pa-
ated on one patient with more than 70° of curve magni- tients treated was limited in the current study and in the
tude and obtained a fusion effect. The current authors literature, the authors think that whenever the other pre-
obtained two true epiphysiodesis effects, whereas one requisites of convex growth arrest are fulfilled in a pa-
patient, who also had a unilateral unsegmented bar with tient, the curve magnitude exceeding 70° can be of sec-
ondary importance.
Table 2. Patient Cobb Angles Although none of the 4 patients with neurologic def-
icit had progression and no neurologic deficits were iden-
Cobb angle(°) Age at Follow-up tified in the remaining 14 patients, it was decided to
follow-up time
Case Sex Preoperative Follow-up (months) (months)
remove the spurs. One of the objectives was to prevent
possible neural deterioration in patients with a deficit (4
1 F 31 37 34 24 patients).9,11–15 Neural deficit progression was seen pre-
2 F 68 74 73 60
3 M 58 63 33 24
dominantly in patients before the age of 5 years.9 Pro-
4 F 60 49 100 40 gression of neural symptoms was reported for 70% of
5 F 56 38 35 24 the patients at the subsequent follow-up assessment.14
6 F 90 70 57 50
7 F 40 42 42 24
Another objective was to prevent possible development
8 M 55 55 33 24 of neural abnormality in patients who were neurologi-
9 F 72 84 51 27 cally normal (14 patients).9,11–15 The last but most im-
10 F 54 65 144 120
11 F 45 32 48 24 portant objective was to prevent neural complications
12 F 54 60 96 60 during prospective and gradual curve correction after
13 F 74 30 80 80 convex growth arrest.9,11–15 McMaster9 advised re-
14 F 40 32 57 36
15 F 46 32 30 24 moval of intraspinal anomaly as a prophylactic measure
16 F 45 40 45 33 in all children younger than 6 years with a congenital
17 F 53 30 38 24 scoliosis regardless of their neural status. Excision of the
18 F 39 33 66 24
spur did not result in a dramatic improvement in neuro-
804 Spine • Volume 28 • Number 8 • 2003

Figure 3. A 2-year-old girl (case 10). At the 10-year follow-up assessment, a curve progression of 11° was observed. A, Preoperative
radiograph. B, Follow-up anteroposterior radiographs.

logic status, but at least none of the patients were made the patients treated with convex growth arrest. This
worse. This also was a common finding in the literature could result from the integrity of the facet joints and
reporting on diastematomyelia excision.9,11–13 The pa- transverse processes, which were not resected during re-
tients with neural lesions comprised 22% of the patients moval of the spur.28
of the current study. This is a significantly lower inci- Convex growth arrest is a safe procedure that generally
dence than the reported 50% to 89% neural deficit does not result in serious complications except for wound
among patients with diastematomyelia in the litera- or chest infections and traction neuropraxias of the inter-
ture.9,11–14 This may be the result of diagnosing and costal or thigh cutaneous nerves.1–7 The patients in the cur-
treating the patients at an early age. rent study had no major or minor complications.
The presence of iatrogenic laminectomy defect, al- In conclusion, convex epiphysiodesis is as effective for
though theoretically thought to decrease the bony sur- treating patients with midline laminectomy defects as for
face available for an effective solid fusion posteriorly, treating patients with intact posterior elements. How-
was found to have no negative effect on the outcome in ever, for a better analysis of the results from this tech-

Table 3. Previously Reported Outcomes of Convex Growth Arrest


Number of Age at Follow-up Epiphysiodesis
Study Patients Surgery Period Effect Fusion Effect Progression

Winter [29] 10 3 yr. 10 mo. 2 yr. 9 mo. 2 (20%) 7 (70%) 1 (10%)


Andrew [1] 13 5 yr. 10 mo. 4 yr. 4 mo. 6 (46%) 6 (46%) 1 (8%)
Winter [31] 13 3 yr. 6 mo. 6 yr. 6 mo. 5 (38%) 7 (54%) 1 (8%)
Keller [13] 16 4 yr. 8 mo. 4 yr. 8 mo. 7 (37%) 8 (42%) 4 (21%)
Thompson [25] 30 6 yr. 4 mo. 8 yr. 10 mo. 23 (77%) 5 (17%) 2 (6%)
Present Series 18 1 yr. 8 mo. 3 yr. 3 mo. 8 (44%) 8 (44%) 2 (12%)
The Efficacy of Convex Hemiepiphysiodesis • Uzumcugil et al 805

nique, the patients should be followed until skeletal 10. Shahcheraghi GH, Hobbi MH. Patterns and progression in congenital sco-
liosis. J Pediatr Orthop 1999;19:766 –75.
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deformities. J Bone Joint Surg [Am] 1974;56:27–39.
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● A retrospective review was conducted for 18 pa- [Am] 1973;55:1425–35.
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