PEDIATRIC/CRANIOFACIAL

Lambdoid Craniosynostosis: The Relationship

with Chiari Deformations and an Analysis of
Surgical Outcomes
Jeffrey A. Fearon, M.D.
Background: A relationship between lambdoid craniosynostosis and Chiari
Vanessa Dimas, M.D.
deformations has been suggested, but the true extent of this association
Kanlaya Ditthakasem,
­remains uncertain. The authors reviewed a single center’s experience treating
M.N.S., R.N. lambdoid synostosis to further elucidate this relationship, examine surgical
Dallas and Corpus Christi, Texas outcomes, and identify associations that might impact future treatments.
Methods: A retrospective chart review was performed of all patients treated
for lambdoid craniosynostosis, excluding the syndromic craniosynostoses.
Operative data, scans, hospitalization, and subsequent surgical procedures
were tracked. All patients were treated with remodeling procedures, but those
with Chiari deformations underwent additional simultaneous suboccipital
decompressions.
Results: Over 22 years, 1006 nonsyndromic craniosynostosis patients were treat-
ed, 45 of whom (4.5 percent) presented with lambdoid involvement: 25 single-
suture and 20 multiple-suture (complex craniosynostosis). Magnetic resonance
imaging revealed that 60 percent of children with unilateral synostosis and
71 percent with a complex synostosis had associated Chiari deformations. The
mean surgical age was 12 months, and the average follow-up was 5.7 years: two
patients developed syringomyelia requiring transcervical decompressions and
two underwent secondary posterior remodeling procedures (one unilateral
and one complex synostosis) while undergoing later Chiari decompressions.
No patients treated with initial suboccipital decompressions have subsequently
developed symptoms requiring treatment.
Conclusions: The majority of children with lambdoid synostosis develop C ­ hiari
deformations; therefore, routine preoperative and postoperative magnetic
­resonance imaging should be considered. The treatment of lambdoid cranio-
synostosis with cranial remodeling procedures, including incontinuity suboc-
cipital decompressions when Chiari deformations were present, was associated
with few complications. Ninety-six percent of those with isolated fusions were
managed with a single procedure. (Plast. Reconstr. Surg. 137: 946, 2016.)
CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

T
he lambdoid suture gets its descriptive name
from the shape created by the intersection
of both sutures coming together producing
an inverted-V shape, similar to the Greek letter
lambda. It is commonly recognized as being one of
the least common sutural fusions, typically quoted
as occurring somewhere between 3 and 4 percent
From The Craniofacial Center; the Driscoll Craniofacial
and Cleft Center; and the Department of Research, Medical
City Dallas Hospital.
Received for publication May 3, 2015; accepted October 22,
2015.
Copyright © 2016 by the American Society of Plastic Surgeons
DOI: 10.1097/01.prs.0000480014.18541.d8
of all single-suture craniosynostoses, although its
incidence (or detection) may be on the rise.1,2
One systematic review, focusing solely on surgi-
cally corrected lambdoid synostosis, identified
only 17 publications (most of these were reviews
of all craniosynostoses), for a combined total of
188 reported surgically treated cases.3 Given the
low frequency of presentation of this condition,
it is not surprising that there is a paucity of out-
comes data following surgical treatment. None
Disclosure: None of the authors has a financial
­interest in any of the products or devices mentioned
in this article.

946 www.PRSJournal.com
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 Volume 137, Number 3 • Lambdoid Craniosynostosis

of the articles captured in this review evaluated

or described any potential relationship between
unilateral lambdoid craniosynostosis and Chiari
deformations. Venes may have been the first to
report the association between the development
of cerebellar tonsillar herniation and syndromic
craniosynostosis; however, based on a review of
their patients with Crouzon syndrome, Cinalli
et al. were the first to suggest a relationship
between fusion of the lambdoid sutures and the
development of Chiari deformations.4,5 Aside
from the syndromic synostoses, these two condi-
tions have also been reported to occur with an
increased frequency in patients with certain sub-
types of complex craniosynostosis.6,7 To our knowl-
edge, the true extent of any potential association
between unilateral lambdoid craniosynostosis and
Fig. 1. A vertex bandeau is removed and swiveled so that the
an acquired Chiari deformation remains uncer-
unaffected (taller) side is moved to the affected side. The ban-
tain. We sought to review our center’s experience
deau is then rotated 90 degrees inferiorly, in a bucket-handle
treating lambdoid synostosis to further explore fashion, inset, and stabilized.
the relationship between these two conditions,
examine our surgical outcomes, and identify asso- addition to performing a posterior cranial vault
ciations that might guide future treatment. remodeling procedure, an in-continuity suboc-
cipital decompression of the foramen magnum
PATIENTS AND METHODS was simultaneously undertaken in all patients,
Following approval from the Institutional also as described previously (Figs. 2 and 3).9 All
Review Board at Medical City Dallas Hospital patients were followed with magnetic resonance
(no. 13.042), a retrospective chart review was per- imaging scans postoperatively, with increasing
formed of all consecutive patients with lambdoid time lags based on age, until skeletal maturity.
craniosynostosis treated by a single surgeon. All The need for secondary operative intervention
patients with syndromic craniosynostoses were was determined by either the development of a
excluded because often these sutures will initially symptomatic Chiari I deformation or a significant
appear patent but later go on to fuse. However, recurrence of the presenting deformity. Categori-
all complex pattern synostoses that initially pre- cal variables were examined using the chi-square
sented with lambdoid fusions were included.
A geneticist evaluated patients suspected of hav-
ing a syndromic presentation, and gene testing
was performed when appropriate. Operative data,
hospital lengths of stay, initial and any subsequent
surgical interventions, and all preoperative and
postoperative scans were tracked. The surgical
technique varied depending on preoperative
magnetic resonance imaging findings. For those
patients with isolated sutural involvement with-
out evidence of cerebellar tonsillar herniation, a
posterior cranial vault remodeling procedure was
performed using a transposed bucket-handle ver-
tex bandeau (flipped 180 degrees so that the unaf-
fected taller side was rotated to the affected side
to increase the posterior projection after rotation
from the vertical to the horizontal plane) and a Fig. 2. When a Chiari deformation is present, a suboccipital
transposed parietooccipital craniotomy bone decompression can be performed to open up the foramen
flap, as described previously (Fig. 1).8 However, magnum by removing the lower occipital bone in the posterior
when cerebellar tonsillar herniation was noted, in midline.

947
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 Plastic and Reconstructive Surgery • March 2016

Fig. 3. (Left) Magnetic resonance imaging scan demonstrating cerebellar tonsillar herniation in a
child with unilateral lambdoid craniosynostosis. (Right) Postoperative magnetic resonance imag-
ing scan showing resolution of the herniation in the same child, 1 month after a posterior cranial
vault remodeling procedure with an in-continuity suboccipital decompression.

or Fisher’s exact test, and continuous variables
were examined using the t test. Categorical vari-
ables are presented as actual incidences and con-
tinuous variables are presented as means. Where
data were missing, we made no assumptions and
performed the analyses without them. Statistical
significance was set at p = 0.05. SAS statistical soft-
ware version 9.4 (SAS Institute, Inc., Cary, N.C.)
was used for all analyses.
RESULTS
Over a 22-year period, 1006 nonsyndromic
patients were treated at our center. Of these, 45
(4.5 percent) presented with lambdoid involve-
ment: 25 (56 percent) of these had isolated sin-
gle-suture involvement and 20 (44 percent) had
multiple-suture involvement as a component of a
complex pattern of craniosynostosis. Among the
isolated presentations, there was a slight male
predominance (male patients, 60 percent; female
patients, 40 percent); however, there were no
significant differences noted between involved
sides (right, 52 percent; left, 48 percent). Various
presentations of complex pattern craniosynosto-
ses with lambdoid involvement were identified,
including 11 patients with Mercedes Benz [sagit-
tal, bilateral lambdoid (55 percent)]; three coro-
nal lambdoid (15 percent); two metopic lambdoid
(10 percent); and two Z-pattern [coronal, sagit-
tal, opposite lambdoid (10 percent)]; one bilat-
eral, and one coronal-sagittal-bilateral lambdoid.
Twenty of 25 patients with isolated unilateral
lambdoid involvement underwent magnetic res-
onance imaging and 12 of 20 (60 percent) were
identified as having cerebellar tonsillar hernia-
tion. A slightly higher incidence of cerebellar
tonsillar herniation was noted among those with
multiple-suture involvement (complex cranio-
synostoses), with 12 of 17 (71 percent) affected;
however, these differences did not reach statisti-
cal significance (Table 1). In addition, when we
evaluated the differing incidences of cerebellar
tonsillar herniation between single lambdoid
fusions and bilateral lambdoid fusions, although
there was a much higher percentage of Chiari
deformations when both sutures were involved
(83 percent versus 40 percent), these differences
also did not reach significance (Table 2). None
of the patients in this series initially presented
with any clinical evidence suggestive for symptom-
atic Chiari deformations. A comparison between
the observed incidences of Chiari deformations
among patients with single-suture lambdoid syn-
ostoses and those with bilateral lambdoid synos-
tosis showed no statistically significant difference.
The diagnosis of synostosis was made primarily
by physical examination in the later part of this
series; when children presented with an uncertain
Table 1. Comparison of the Incidence of Chiari
Deformations between Isolated Lambdoid
Synostoses and Complex Synostoses
Unilateral Complex
Lambdoid Synostosis
(%) (%) Total
Yes 12 (60) 12 (70.59) 24
No 8 (40) 5 (29.41) 13
Total 20 17 37
p (χ2) 0.5014

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 Volume 137, Number 3 • Lambdoid Craniosynostosis

Table 2. Comparison of the Incidence of Chiari
Deformations between One and More Than One
Affected Lambdoid Suture
Multisutural Multisutural
Fusions with Only Fusions with
One Lambdoid Bilateral Lambdoid
Suture (%) Sutures (%)
Yes 2 (40) 10 (83.33)
No 3 (60) 2 (16.67)
Total 5 12
p (χ2) 0.0740
clinical picture, they were reexamined 4 months
later to evaluate changes in skull shape that had
occurred with subsequent growth. If at this later
time the diagnosis remained unclear, a computed
tomographic scan was obtained and, when posi-
tive, often an incomplete fusion was identified
(Fig. 4). Among those children diagnosed without
computed tomographic scan verification, all were
noted to have fused lambdoid sutures at the time
of surgery. The mean surgical age for the isolated
lambdoid synostoses was 12 months (range, 4 to
42 months), and the average hospital stay was 2.3
days. The average surgical age for the complex
synostoses was slightly higher at 12.4 months, and
the average length of stay was 2.4 days. There were
no major complications or mortalities in either
group. Two patients with isolated lambdoid syn-
ostosis required a third hospital day for postop-
erative nausea and vomiting, and one patient with
complex craniosynostosis required an extra day for
bronchodilators and chest physical therapy. With
a mean follow-up of 5.7 years (range, 2 months
to 15 years), two patients (one isolated and one
with multiple-suture involvement) subsequently
developed syringomyelia, requiring transcervical
Total
decompressions. Neither of these two patients
12 had undergone a suboccipital decompression
5
17 during their initial posterior cranial remodeling
procedure. A second posterior remodeling proce-
dure was performed in two patients: one multiple-
suture patient underwent secondary remodeling
procedures during subsequent Chiari decompres-
sion and one single-suture patient (4 percent)
underwent a secondary correction at 40 months,
also for a symptomatic Chiari deformation. This
patient had additional anomalies, including mul-
tiple congenital heart defects and an immuno-
deficiency but, most noteworthy, had undergone
correction primarily at a relatively earlier age
(7 months) compared with others in this series.
Two years postoperatively, this patient presented
with a symptomatic Chiari I deformation (dyspha-
gia and aspiration) and some recurrence of poste-
rior plagiocephaly, and thus a combined posterior
cranial vault remodeling was performed with an
in-continuity suboccipital decompression of the
foramen magnum. Of the 17 patients treated with
simultaneous suboccipital decompression for
associated Chiari deformations (nine with unilat-
eral lambdoid synostosis and eight with complex
synostoses), none has subsequently developed
symptoms associated with these Chiari deforma-
tions or radiologic evidence for progression of
their tonsillar herniation.

Fig. 4. In many children, the diagnosis of lambdoid synostosis
can be made based solely on examination. However, there are
some children who present with less clear clinical diagnoses,
especially those with incomplete fusions. In these cases, further
evaluation with a computed tomographic scan can be helpful in
making the diagnosis.
DISCUSSION
Lambdoid craniosynostosis is the least com-
monly encountered craniosynostosis, constitut-
ing only 4.5 percent of all nonsyndromic cases
treated at our center in Dallas. It is therefore not
surprising that there is a relative paucity of pub-
lished outcomes data following the surgical cor-
rection for this unusual sutural fusion. A number
of different surgical approaches to treatment have
been described for treating lambdoid synostosis,
including strip craniectomies followed by skull-
molding devices, remodeling procedures, and
even distraction osteogenesis.10–13 This current
review suggests that treatment with a single-stage
posterior cranial vault remodeling procedure was
associated with a relatively short hospitalization
(<2.4 days), with the majority of patients spending
only two nights in the hospital, and no significant

949
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 Plastic and Reconstructive Surgery • March 2016
perioperative complications. Furthermore, almost
all those presenting with an isolated lambdoid
fusion (96 percent) have required only a single
procedure, over an average follow-up length of
5.7 years. One child, treated earlier in this series,
did require a secondary vault remodeling proce-
dure that was triggered by the development of a
symptomatic Chiari deformation with recurrence
of the posterior plagiocephaly. We speculate that a
surgical correction performed at too early an age
(7 months) and failure to sufficiently overcorrect
the observed deformity may both have contrib-
uted to this symptomatic recurrence. Based on
studies suggesting superior long-term growth with
later surgical interventions, our recommended
primary treatment age shifted in the later part
of this series to somewhere between 11 and
18 months of age, depending on clinical sever-
ity.14,15 Although the average length of follow-up
for this study falls short of reaching skeletal matu-
rity, these data suggest that with an over 5-year
follow-up and using symptomatic Chiari deforma-
tions, new-onset cerebellar tonsillar herniation,
and significant clinical phenotypic recurrences as
criteria for reoperation, the treatment paradigm
used appears effective with respect to revision
rates. Specifically, the incidence for those with iso-
lated single-suture lambdoid involvement under-
going secondary remodeling procedures is much
lower (4 percent) than has been reported for
other single-suture craniosynostosis repairs with
similar reported lengths of follow-up.16–18 Never-
theless, we recognize that comparisons of second-
ary surgical interventions are complicated by the
subjective nature of any decision to reoperate.
In addition, identification of a phenotypic recur-
rence in the posterior skull may be camouflaged
by the presence of hair, favorably biasing compari-
sons to metopic and coronal fusions.
In spite of the recognized relationship between
lambdoid synostosis and Chiari deformations in
children with syndromic and certain subtypes of
complex craniosynostosis, we are unaware of any
focused reviews examining the potential associa-
tion of cerebellar tonsillar herniation in children
with isolated single-suture lambdoid craniosyn-
ostosis. Within this series of patients, we noted a
surprisingly high incidence of Chiari deforma-
tions (60 percent) in children with single-suture
involvement. We believe that this finding under-
scores the importance of performing preopera-
tive magnetic resonance imaging for all affected
children. If fusion of just a single lambdoid suture
can have an impact on the development of cere-
bellar tonsillar herniation, it would seem intuitive
950
Copyright © 2016 American Society of Plastic Surgeons. Unauthorized reproduction of this article is prohibited.
that involvement of multiple sutures should fur-
ther elevate this risk. Although a higher incidence
of Chiari deformations was noted in our patients
with more than one sutural fusion, this difference
did not attain significance, although one might
speculate that an even larger series might show
such a difference. Experience with syndromic
synostoses suggests that cerebellar tonsillar her-
niation is an acquired deformation; therefore, the
incidence in children with lambdoid synostosis
might be expected to rise with longer lengths of
follow-up, especially in those treated at an early
age or with an insufficient correction, which
might both result in recurrence of the presenting
phenotype.5,19,20 For those patients treated earlier
in this series, or when patients were referred from
outside institutions, evaluations with plain radio-
graphs and computed tomographic scans were
more likely to have been performed, whereas later
in this series, the senior author (J.A.F.) would only
obtain computed tomographic scans for uncer-
tain presentations, such as seen with incomplete
sutural fusions. Our preferred imaging modality
for those presenting with more obvious clinical
signs for lambdoid craniosynostosis is magnetic
resonance imaging, and more recently, we have
transitioned to using a rapid sequence protocol to
avoid the need for general anesthesia. Our pref-
erence for magnetic resonance imaging is based
on the ability of this imaging modality to pro-
vide better visualization of the hindbrain, which
in turn impacted our surgical decision-making.
For example, if preoperative imaging revealed an
absence of cerebellar tonsillar herniation, only a
posterior remodeling procedure was performed.
However, when cerebellar tonsillar herniation
was identified, a suboccipital decompression was
added to the remodeling procedure by extending
a posterior midline osteotomy inferiorly (typically
by approximately 2 cm) to open up the posterior
aspect of the foramen magnum. Performance of
a suboccipital decompression during posterior
remodeling procedures has been shown to provide
radiographic improvement of tonsillar herniation
in 70 percent of asymptomatic patients (with sta-
bility in the remaining 30 percent) without add-
ing any additional morbidity.9 We believe that this
additional step has the potential to prevent the
development of a symptomatic Chiari deforma-
tion, and the need for later surgical intervention,
by preventing future cord compression. Thus
far, of the 17 patients undergoing simultaneous
suboccipital decompressions in this series, none
have required any additional treatment related to
the development of either a symptomatic Chiari
 Volume 137, Number 3 • Lambdoid Craniosynostosis
deformation or a syrinx. However, we recognize
that our retrospective study design, and length of
follow-up of just under 6 years, might limit vali-
dation of this theory. With respect to long-term
patient follow-up, we recommend repeated rapid
sequence magnetic resonance imaging for all
children previously diagnosed with cerebellar
tonsillar herniation or if recurrences of posterior
plagiocephaly are subsequently identified, at ages
5 years, 10 years, and at skeletal maturity, or more
often, depending on clinical severity.
Jeffrey A. Fearon, M.D.
The Craniofacial Center
7777 Forest Lane, Suite C-700
Dallas, Texas 75230
cranio700@gmail.com
REFERENCES
1. Singer S, Bower C, Southall P, Goldblatt J. Craniosynostosis
in Western Australia, 1980-1994: A population-based study.
Am J Med Genet. 1999;83:382–387.
2. Kolar JC. An epidemiological study of nonsyndromal cranio-
synostoses. J Craniofac Surg. 2011;22:47–49.
3. Al-Jabri T, Eccles S. Surgical correction for unilateral
lambdoid synostosis: A systematic review. J Craniofac Surg.
2014;25:1266–1272.
4. Cinalli G, Renier D, Sebag G, Sainte-Rose C, Arnaud E,
Pierre-Kahn A. Chronic tonsillar herniation in Crouzon’s
and Apert’s syndromes: The role of premature synostosis of
the lambdoid suture. J Neurosurg. 1995;83:575–582.
5. Venes JL. Arnold-Chiari malformation in an infant with
Kleeblattschadel: An acquired malformation? Neurosurgery
1988;23:360–362.
6. Rhodes JL, Kolar JC, Fearon JA. Mercedes Benz pattern cra-
niosynostosis. Plast Reconstr Surg. 2010;125:299–304.
7. Schmelzer RE, Fearon JA. ‘Z-pattern’ craniosynostosis:
A novel presentation of trisutural fusion. J Craniofac Surg.
2007;18:568–574.
Copyright © 2016 American Society of Plastic Surgeons. Unauthorized reproduction of this article is prohibited.
8. Fearon JA. Rigid fixation of the calvaria in craniosynos-
tosis without using “rigid” fixation. Plast Reconstr Surg.
2003;111:27–38; discussion 39.
9. Scott WW, Fearon JA, Swift DM, Sacco DJ. Suboccipital
decompression during posterior cranial vault remodeling
for selected cases of Chiari malformations associated with
craniosynostosis. J Neurosurg Pediatr. 2013;12:166–170.
10. Jimenez DF, Barone CM, Cartwright CC, Baker L. Early
management of craniosynostosis using endoscopic-assisted
strip craniectomies and cranial orthotic molding therapy.
Pediatrics 2002;110:97–104.
11. Thaller SR, Hoyt J, Boggan J. Surgical correction of unilat-
eral lambdoid synostosis: Occipital rotation flap. J Craniofac
Surg. 1992;3:12–17; discussion 18.
12. Komuro Y, Yanai A, Hayashi A, Miyajima M, Nakanishi H,
Arai H. Treatment of unilateral lambdoid synostosis with cra-
nial distraction. J Craniofac Surg. 2004;15:609–613.
13. Smartt JM Jr, Reid RR, Singh DJ, Bartlett SP. True lambdoid
craniosynostosis: Long-term results of surgical and conserva-
tive therapy. Plast Reconstr Surg. 2007;120:993–1003.
14. Seruya M, Oh AK, Boyajian MJ, et al. Long-term outcomes of
primary craniofacial reconstruction for craniosynostosis: A
12-year experience. Plast Reconstr Surg. 2011;127:2397–2406.
15. Fearon JA, Ruotolo RA, Kolar JC. Single sutural craniosynos-
toses: Surgical outcomes and long-term growth. Plast Reconstr
Surg. 2009;123:635–642.
16. Pearson GD, Havlik RJ, Eppley B, Nykiel M, Sadove AM.
Craniosynostosis: A single institution’s outcome assess-
ment from surgical reconstruction. J Craniofac Surg.
2008;19:65–71.
17. Taylor JA, Paliga JT, Wes AM, et al. A critical evaluation of
long-term aesthetic outcomes of fronto-orbital advancement
and cranial vault remodeling in nonsyndromic unicoronal
craniosynostosis. Plast Reconstr Surg. 2015;135:220–231.
18. Wes AM, Paliga JT, Goldstein JA, Whitaker LA, Bartlett SP,
Taylor JA. An evaluation of complications, revisions, and
long-term aesthetic outcomes in nonsyndromic metopic cra-
niosynostosis. Plast Reconstr Surg. 2014;133:1453–1464.
19. Fearon JA, Podner C. Apert syndrome: Evaluation of a treat-
ment algorithm. Plast Reconstr Surg. 2013;131:132–142.
20. Fearon JA, Rhodes J. Pfeiffer syndrome: A treatment ­evaluation.
Plast Reconstr Surg. 2009;123:1560–1569.
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