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Original Article

Redefining the Treatment of Lumbosacral Transitional Vertebrae for Bertolotti Syndrome:


Long-Term Outcomes Utilizing the Jenkins Classification to Determine Treatment
Arthur L. Jenkins III1-3, Richard J. Chung3, John O’Donnell3, Charlotte Hawks3, Sarah Jenkins1,3, Daniella Lazarus3,
Tara McCaffrey3, Hiromi Terai3, Camryn Harvie3, Stavros Matsoukas1

- OBJECTIVE: Using the Jenkins classification, we pro- preoperatively (n [ 27), 21 (78%) had improvement of hip
pose a strategy of shaving down hypertrophic bone, uni- pain postoperatively.
lateral fusion, or bilateral fusion procedures to achieve - CONCLUSIONS: The Jenkins classification system pro-
pain reduction and improve quality of life for patients with
vides a strategy for patients with Bertolotti syndrome who
Bertolotti syndrome.
fail conservative therapy. Patients with Type 1 anatomy
- METHODS: We reviewed 103 patients from 2012 through respond well to resection procedures. Patients with Type 2
2021 who had surgically treated Bertolotti syndrome. We and Type 4 anatomy respond well to fusion procedures.
identified 56 patients with Bertolotti syndrome and at least These patients respond well in regard to hip pain.
6 months of follow-up. Patients with iliac contact preop-
eratively were presumed to be more likely to have hip pain
that could respond to surgical treatment, and those patients
were tracked for those outcomes as well.
- RESULTS: Type 1 patients (n [ 13) underwent resection. INTRODUCTION
Eleven (85%) had improvement, 7 (54%) had good outcome,
1 (7%) had subsequent surgery, 1 (7%) was suggested
additional surgery, and 2 (14%) were lost to follow-up. In
Type 2 patients (n [ 36), 18 underwent decompressions
L umbosacral transitional vertebra (LSTV) anatomy is defined
as a congenital anomaly with incomplete sacralization or
lumbarization of lumbosacral junction.1 In recent studies,
LSTVs have a prevalence of 15.8% to 35.6% within the general
population.2-5 Bertolotti syndrome is a clinical finding where an
and 18 underwent fusions as a first line. Of the 18 patients
LSTV is the source of one or more of: back pain, hip pain,
treated with resection an interim analysis saw 10 (55%)
sacroiliac pain, groin pain, or radiating leg pain in a classically L5
with failure and needing subsequent procedures. With
distribution.6 We believe that having a full understanding of a
subsequent procedure, 14 (78%) saw improvement. For patient’s LSTV anatomy improves management. Currently, the
fusion surgical patients, 16 (88%) saw some improvement diagnosis of Bertolotti syndrome is inconsistent and
and 13 (72%) had a good outcome. In Type 4 patients (n [ unclear—often a diagnosis of exclusion.7
7), 6 (86%) did well with unilateral fusion, with durable Many studies utilize the Castellvi classification system estab-
benefit at 2 years. In patients who had hip pain lished in 1984 for LSTV identification and subsequent Bertolotti

Key words TP: Transverse process


- Bertolotti syndrome VAS: Visual analog scale
- Castellvi classifications
- Lumbosacral transitional vertebrae From the Departments of 1Neurosurgery, and 2Orthopedics, Icahn School of Medicine at
- Minimally invasive surgery Mount Sinai; 3Jenkins NeuroSpine, Private Practice, New York, New York, USA
- Neurosurgery
To whom correspondence should be addressed: Arthur L. Jenkins III, M.D.
- Spine
[E-mail: alj@arthurjenkinsmd.com]

Abbreviations and Acronyms Citation: World Neurosurg. (2023) 175:e21-e29.


https://doi.org/10.1016/j.wneu.2023.03.012
CT: Computed tomography
LSTV: Lumbosacral transitional vertebrae Journal homepage: www.journals.elsevier.com/world-neurosurgery
MIS: Minimally invasive surgery Available online: www.sciencedirect.com
MRI: Magnetic resonance imaging 1878-8750/ª 2023 The Authors. Published by Elsevier Inc. This is an open access article under
PSIC: Posterior superior iliac crest the CC BY license (http://creativecommons.org/licenses/by/4.0/).
SI: Sacro-Iliac

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ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

syndrome diagnosis. Yet Castellvi’s hypothesis was that pain was tomography [CT] reconstructions) that causes the pain, and that
derived primarily from disk herniations at the transitional level, separating these surgically to a minimum of 10 mm separation
instead of contact between elements of the transitional anatomy at would be adequate distance to mitigate bone-on-bone rubbing
the L5-S1 level.8 However, the proximity of the transverse process without potential regrowth. This was based on the observation
(TP) and ala, or the pseudoarticulation itself, as well as in some that no patients (without a hypermobility diagnosis) were found to
cases bone spurs coming off the facet joints, may directly be symptomatic at this anomaly if the transverseeala gap was
irritate the exiting L5 nerve.2 Direct bone-on-bone contact may larger than 10 mm.
induce inflammatory mediators that irritate the exiting L5 nerve, By the same logic, we initially anticipated that for patients with
in addition to nerves on the surfaces of the bones of the transverse Type 2 anatomy and favorable response to injections into the
process, ala, and iliac wing.2 A significant number of patients have pseudoarticulations, they would also respond to shaving down the
also reported mechanical hip region pain, which may be alleviated bone on the painful side(s), but also considered that this strategy
by treatment of the transitional anatomy in which the pain may require re-evaluation over time. We also considered that Type
mechanism may be from the contact or instability of the 2 anatomy might benefit from a fusion procedure, as patients who
sacroiliac (SI) joint caused by extra bones being nearby.6 Many had decompression-type procedures might eventually develop
patients with Bertolotti syndrome also report groin pain, and bone regrowth or have an inadequate resection that results in
this may be partially related to contributions of the L5 nerve to recurrence of symptoms and potential reoperation, or instability
the genitofemoral nerve.9 from “releasing” a relatively unstable level (given the prevalance of
Thus, we proposed the anatomic basis for our new classification LSTV hypoplastic disks, lack of muscle development at the LSTV
system in the parallel paper, which we will refer to as the Jenkins level, and other associated findings).11,12 Our initial hypothesis
classification (Figure 1, Table 1). This new description of LSTV was that we could try the shaving first, a much smaller
anatomy is based on the concept of a reduced gap between the “minimally invasive” procedure, and if that failed, proceed to a
transverse process and sacral ala being the primary responsibility fusion as a second stage procedure.
for Bertolotti syndrome pain, rather than disk herniations (which Type 3 anatomy is predicted to have no symptoms referable to
we consider to be at most an epiphenomenon of the LSTV, not the LSTV level itself, and therefore no need for surgical manage-
Bertolotti syndrome). This physical gap in symptomatic patients ment at that level, given the bilateral stability of the sacroiliac
with Bertolotti syndrome, which in normal patients is typically junction. For these patients, we proposed looking at alternate
greater than or equal to 20 mm, is almost always less than 10 levels or locations for their primary pain generator, and not calling
mm.9 In addition, when the transverse process is less than 5 mm it “Bertolotti syndrome”, even if they have associated adjacent level
from the iliac wing, this iliac contact can also be part of the disease known to be associated with LSTV anatomy.
symptomatic LSTV anatomy as well. And finally, we proposed that patients with Type 4 anatomy and
Although an LSTV can be radiologically diagnosed, current favorable response to injections targeting the painful side of the
diagnostic and clinical treatment of symptomatic LSTVs, or Ber- LSTV will benefit most from fusion surgery of the non-fused side,
tolotti syndrome, is unclear.10 Often, the source of the pain can be given the existing unilateral stability.
identified by targeted injections to the LSTV anatomy, confirmed The concept of whether an LSTV is already a “fusion”, or that a
by positive responses to specific and sensitive relief. For partial fusion within an LSTV can even generate any pain, has
treatment options, the current literature recommends caused considerable discussion and dissent among providers and
conservative management with medical management, physical researchers, and many patients report hearing from their other
therapy, and a combination of local and steroid injections into clinicians that a unilateral fusion (a Type 4 LSTV) “cannot cause
the pseudoarticulation as non-surgical therapeutic measures.10 pain”. We hypothesize that given the presence of hypoplastic but
There have long been 2 primary surgical treatments for symp- partially functional disks, bilateral mobile facet joints, and only a
tomatic Bertolotti syndrome patients: decompressions or fusions unilateral fusion on one side, and either a gap or a “pseudoarti-
of the transitional articulation at the lumbosacral junction.10 culation” on the other side, pain can be generated in these pa-
Determining the appropriate treatment for a particular patient tients either from flexing of the “fused” side and bone contact or
depends upon matching the clinical presentation with the irritation of the nerve causing pain on the “open” side, or from
radiologic findings to optimize the treatment plan for each flexing of the “fused” side and stress on that flexing side leading
patient. However, there has been no large-scale study that estab- to pain on the “fused” side.
lishes a consensus of decompressions or fusions procedures based The purpose of this study is to establish a preliminary guideline
on LSTV presentation or other diagnostic measures. In our sur- based on our collective experience with these patients who only
gical outcomes analysis of 56 patients with Bertolotti syndrome, have Bertolotti syndrome and not any other confounding or
we proposed the following treatment strategies based on the pa- coincident spinal conditions. Heretofore, there has been little
tient’s specific type. consensus regarding the surgical management of Bertolotti syn-
For patients with Type 1 LSTVs who had favorable responses to drome. Of note, in discussions with many surgeons who have
injections between the transverse process and ala at the closest attempted to treat this syndrome, a pattern of doing resections for
point of proximity, we proposed to perform decompressions all classifications as the treatment has been noticed among sur-
(resection of a portion of the hypertrophic or prominent transverse geons who have treated fewer than 5 patients (unpublished data).
process, ala, or in some cases, both) as we hypothesize that it is With our proposed surgical recommendations, we hope to in-
the proximity (less than 10 mm separation between the transverse crease inter-surgeon reliability, and in return, improve care in
processes and the ala, as seen on multiplanar computed axial patients presenting with Bertolotti syndrome.

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ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

Figure 1. Schematic presentation of the classification of unilateral and bilateral LSTV according to the proposed Jenkins classification.

METHODS underwent surgical treatment that included surgical treatment for


their Bertolotti syndrome on their LSTV plus 1 or more surgical
Patient Selection procedures at a different level/location, including suprajacent
We performed a retrospective cohort study, under the exemption lumbar fusions, diskectomies, laminectomies, or removal of
of the Center for IRB Intelligence review board, of patients hardware and exploration of fusion at 1 or more levels above the
operated on from 2012 through 2021 with clinically verified target level. This resulted in almost half of patients with Bertolotti
Bertolotti syndrome, who all met the following criteria: Symp- syndrome having a second significant diagnosis at a different
tomatic patients (1) with 1 or more of back pain, L5 radiculop- lumbar level that required simultaneous treatment. In addition to
athy, SI pain, hip pain, groin pain; (2) having transitional those, a small population had insufficient follow-up data to be
anatomy as described by the Jenkins classification; (3) having used for outcome result, such as being lost to follow-up, providing
favorable injection results targeting the LSTV, and in many cases, incomplete pain information, and death of the patient.
also with no response to targeting other spinal and non-spinal
anatomy including epdidural injections, hip injections, facet or
medial branch block injections (with no or minimal response to Collection of Patient-Reported Pain
these latter types); (4) who failed other forms of conservative Patient-reported pain data were collected utliizing the visual
treatment; and (5) who subsequently underwent a surgical pro- analog scale (VAS) pain scale (0e10), and pain was recorded when
cedure to address their LSTV by the senior author. it was present in their back or legs or hips, and specifically re-
Of the original 103 surgical patients reviewed in the parallel ported as to location in the upper lumbar, lower midline lumbar,
article, only 56 had only Bertolotti syndrome as their initial and and SI regions, as well as specific dermatomal distribution, hip
sole surgical diagnosis and therefore underwent only surgical pain, groin pain, or other regions.
treatment of their transitional lumbosacral anatomy. That means Patients were screened for a combination of symptoms, ranging
that 47 patients were excluded from this analysis because they from myelopathy, radiculopathy, mechanical back pain, hip pain,

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ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

Table 1. Description of the Classification of Unilateral and Bilateral LSTV According to the Proposed Jenkins Classification
Laterality (More
Classification Abnormal Side) Anatomic Description

Primary Classification
1A (L)/(R) Unilateral dysplastic transverse process [<10 mm between TP and ala; e.g., Type 1A(L) gap is >2 mm, <10 mm, only on left,
>10 mm on right]
1B (L)/(R) Bilateral dysplastic transverse process [both sides <10 mm gap; e.g., Type 1B(R) closer on right than left]
2A (L)/(R) Incomplete unilateral lumbarization/sacralization with enlarged transverse process that has a diarthrodial joint between
itself and the sacrum [<2 mm separation, with planar surface on transverse process parallel to the opposite surface of the
ala, but >10 mm gap on the opposite side; e.g.: Type 2A(L) is Type 2 on the left, with >10 mm on the right]
2B (L)/(R) Incomplete bilateral lumbarization/sacralization with enlarged transverse process that has a pseudo-joint between itself and
the sacrum [<2 mm of separation on both sides, with planar surfaces on transverse process parallel to the opposite side of
the ala; e.g., Type 2B is symmetrical while 2B(R) has a bigger interface on right]
2C (L)/(R) Dysplastic transverse process on one side and incomplete lumbarization/sacralization on the other side [<10 mm but >2 mm
on one side, and <2 mm on the other side; e.g., Type 2C(L), Type 1 on right, Type 2 on left]
3 (L)/(R) Bilateral lumbarization/sacralization with complete osseous fusion of the transverse process to the sacrum [no radiographic
fusion plane viable on MRI, CT, or Ferguson views of roentogram, either side]
4A (L)/(R) Lumbarization/sacralization with complete osseous fusion on one side dysplastic transverse process on the other side [Type 1
on one side, Type 3 on the other side; e.g., Type 4A(L) Type 1 is open on right, Type 3 on left]
4B (L)/(R) Lumbarization/sacralization with complete osseous fusion on one side and incomplete lumbarization/sacralization on the
other side [Type 3 on one side Type 2 on other side; e.g., Type 4B(R), Type 2 side is open on right, Type 3 on left]
4C (L)/(R) Lumbarization/sacralization with complete osseous fusion on one side, >10 mm gap on other side [Type 3 on one side, Type
1 on other side; e.g., Type 4C(L) is open on left, Type 3 on right]
Secondary Classification
þL/R/B þL/R/B Denotes which side has contact between transverse process and iliac crest [e.g., Type 2C(L)þL]

CT, computed tomography; L, Left side more prominent; MRI, magnetic resonance imaging;R, Right side more prominent; TP, transverse process;þ, transverse process touches (within 2 mm of)
the iliac bone; C, Type 1 LSTV on one side and Type 2 LSTV on the other side.

and were screened for other spinal, orthopedic, or medical con- While we offered a choice of instrumented fusion or “simple”
ditions that might be contributing to their pain. Only those with decompression procedures to the Type 2 patients, we left the
both transitional anatomy and the fact that the transitional anat- choice to the individual patients; in all cases the patients elected to
omy was the primary pain generator were considered to have undergo a resection instead of a fusion as the first-line treatment.
“Bertolotti syndrome”. After an initial internal analysis, we determined that there was a
We identified a “good” outcome to be either a reduction of the high rate of recurrent surgery for Type 2 patients (n ¼ 18 in this
original VAS pain level from their preoperative level to 50% of that group) undergoing resection/decompression procedures. The
level, or to a 3 out of 10 or less; an “acceptable” outcome was any majority of these patients, after undergoing instrumented fusion
reduction in that pain that was sustained, even if not a the “good” of the LSTV level, had resolution or improvement in symptoms
level. Patient outcomes for this analysis were appraised at 6 month, that was sustained. As a result, we then changed our algorithm to
1 year, 2 years, and time of last follow-up. Presence of additional instead recommend resection/decompression only for Type 1
surgical treatments was noted, and whether this surgical treatment anatomy, and instrumented fusion for Type 2 anatomy of all
was for return of the original symptoms (a failure of treatment) or subtypes. This recommendation and strategy has been maintained
for new and unrelated treatments (like a new L2-L3 disk herniation). for the rest of the cohort.
Particular notice was made as to whether those “unrelated” surgical No patients with Type 3 anatomy were identified with injections
treatments were at the suprajacent level. to have pain coming from the LSTV level, and therefore no sur-
gical procedures were offered to treat this level.
Surgical Treament Strategies Patients with Type 4 anatomy were offered surgical instru-
Our initial treatment paradigm consisted only surgical resection or mented fusions of the unfused side only, except where a stress
decompression procedures, and only on the symptomatic sides, of fracture in the “fused” side was identified (n ¼ 1), for that patient
all patients with Type 1 or Type 2 anatomic findings (Type 1A, an instrumentation of the fractured side and an instrumentation
Type 1B, Type 2A, Type 2B, Type 2C). and fusion of the open side was performed.

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ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

Surgical Technique Type 1 anatomy is described as a dysplastic transverse process


Decompression procedures, whether unilateral or bilateral, were that shows a gap of less than 10mm, but greater than 2mm across
performed using an operating microscope and the METRx dilators the lumbosacral junction.
(Medtronic, Memphis, TN), drilling out the bone of the inferior Type 2 anatomy is identified as pseudo-articulation between
half or so of the transverse process, the superior portion of the adjacent transverse processes with a gap of less than 2 mm.
sacral ala, or both, so that the gap between the 2 measured at least Type 3 anatomy is the complete fusion lateral to the facet at the
10 mm at all points from tip to foramen. The intertransverse ala, without spontaneous fusion of the disk or facet joints, of the
membrane, the attached periosteum, and any scar in the area, lumbosacral junction.
were removed completely from pars interarticularis and facet Type 4 anatomy is the presence of unilaterally fused side with
medially to the end of the transverse process laterally, and the varying pseudoarticulation on the contralateral side (Type 4A, 4B,
bleeding surfaces of the bone edges so exposed were covered first or 4C).
with bone wax, and then a layer of fibrin glue sprayed on top of
that to prevent bone regrowth.
Instrumented fusion procedures (unilateral or bilateral) in this RESULTS
series were performed utilizing a Wiltse approach, utilizing The 56 patients had an average age of 46.8  17.4 years with an
microsurgical dissection, and a dorsal fusion performed by average body mass index of 25.1 4.8. There were 29 female and
decorticating the surfaces of the sacral ala, the transverse process, 27 male patients. Using the Jenkins classification, 13 patients with
and the facet joint from the LSTV level, as follows: an incision 2e3 Type 1 anatomy (all with Type 1B), 36 patients with 1 or more
cm was utilized, parallel to but just medial to the posterior su- subsets of Type 2 anatomy (Type 2A, n ¼ 2; Type 2B, n ¼ 14; Type
perior iliac crest (PSIC) in line with the angles of the pedicles of 2C, n ¼ 20), no patients with Type 3, and 7 patients with Type 4
the lowest lumbar and highest sacral levels.13 The fascia and anatomy (Type 4A, n ¼ 3, and Type 4B, n ¼ 4).
muscle were split, exposing the ala and transverse process. In each subtype, surgical outcomes were defined as “good”,
If instrumentation was to be placed, pedicle screws were placed at “adequate”, and “no” improvement. Good improvement was
the bottom lumbar (usually L5, but could be either L4 or L6) as well as defined as patients with a 50% reduction in postoperative VAS
either pedicle screw or alar screw placed at S1, and a rod placed be- score compared with preoperative or a VAS score  3. Adequate
tween them, and locked into place. In cases where there was a sig- improvement was defined as patients displaying clinical
nificant focal scoliosis, distraction or compression was performed on improvement but less than a 50% reduction in their VAS score. No
1 side to attempt to level the L5 body against the S1 body, or to improvement was defined as patients with no change or even
compensate for any other asymmetry that might have been present. worsening of their symptoms postoperatively.
Bone grafting was performed with either locally harvested
autogenous iliac crest, or Infuse rhBMP-2 product (Medtronic),
Type 1
and then allograft cancellous bone chips being placed over the
The 13 total patients with Type I anatomy generally did well with
decorticated surfaces. All patients were informed that, if the
decompression surgery by resection of the inferior portions of the
Infuse was utilized, this was considered an “off-label” indication.
transverse process, the superior sacral ala protrusions, or both to
The choice of allograft iliac crest or rhBMP-2 product was based
create at least 1 cm gap between the 2. A total of 13 patients un-
on several factors including price and insurance coverage issues,
derwent decompression procedures, with 6 initial bilateral and 7
or potential side effects of the rhBMP-2, but was left to the indi-
unilateral decompression surgeries (7 left).
vidual patient; in most cases the patients elected to use the
Of these patients, 11 of 13 (85%) improved from their decom-
rhBMP-2 instead of harvesting bone from their PSIC.
pression procedure. Within these 13 patients, 7 (54%) patients had
All patient who had hardware placed had intraoperative re-
good improvement, 5 (38%) patients reported adequate improve-
constructions to verify final positioning of the screws prior to the
ment, and 1 (8%) showed no improvement. The patient with no
fusion part of the procedure, utilizing 1 of 3 systems, including
improvement developed supra-adjacent issues and underwent an
Ziehm Vision 3D (Ziehm Imaging, Orlando FL), Siemens C-ios or
L4-S1 decompression and L5-S1 fusion to manage her recurrent
Siemens ISO-C (Siemens, Erlangen, Germany).
Bertolotti type pain, followed by an L4-L5 discectomy. She still
reported residual pain but was lost to follow-up at the 6-month
Jenkins Classification mark.
When discussing patient types, we use the Jenkins classification None of the other Type I patients underwent subsequent
Type 1, 2, 3, and 4 (Figure 1), with subclassifications of: resection/decompression surgery. Three patients considered to be
a successful outcome with regard to their Bertolotti related surgery
- L (left) or R (right) prominence subsequently developed pain that was different from their pre-
operative Bertolotti pain, and are being managed by injections, or,
- A (unilateral), B (bilateral), or C (Type 2 with Type 1 and
in 1 case, being managed for her endometriosis pain. Notably, of
contralateral 2 anatomy or Type 4 with a gap > 10 mm and
the 7 patients with good improvements (54%), 4 patients (31%)
contralateral Type 3 anatomy)
had no back or leg pain at the last follow-up ranging from 4 to 10
- þ L (left) or R (right) iliac contact years after surgery.

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ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

Type 2 All subsequent patients with Type 2 anatomy were then rec-
Of 36 patients who underwent surgical procedures, 18 underwent ommended to undergo fusion bilaterally instead of decompres-
decompressions and 18 underwent fusions as their first treatment. sion. An option was given to them to undergo decompression
first, with the caveat that there was a high likelihood for revision
MIS Decompression/Reduction Procedures procedure based on our analysis, and no patient subsequently
Our initial surgical management of Type 2 patients (n ¼ 18) was opted for that option given the high likelihood of failure.
similar to Type 1 patients, with the performance of a decom- Overall, the pain reduction for all patients in the initial MIS
pression/reduction of the transverse process and/or ala with a goal resection/decompression treatment group for Type 2 anatomy with
of 1 cm gap between the ala and the transverse process, only on any improvement was 15/18 (83%), yet the good improvement
the symptomatic side (right ¼ 9, left ¼ 7) or bilaterally (n ¼ 2). (50% pre-op pain or VAS  3) was limited to 6/18 (33%) patients.
Around 2017, at the time of the initial evaluation of efficacy of Patients with the initial treatment failures tended to do well with a
this technique, we noticed that many of these initial cohort of 18 second procedure if they had one.
patients had undergone a subsequent re-operation for recurrent
Bertolotti’s Syndrome symptoms (contralateral decompression MIS Fusion Procedures
(n ¼ 2), ipsilateral revision decompression (n ¼ 1), diskectomy at MIS bilateral fusion procedures as described above in Methods
L5/S1 (n ¼ 1), and revision with fusion (n ¼ 4)) within the first 2 were then performed on the next 18 patients with Type 2 anatomy
years after their index procedure. Therefore, we performed an of any subtype.
initial subgroup analysis and determined that in the first 2 years, Bilateral fusion surgery helped 16/18 (89%) patients. 13/18 (72%)
12/18 (67%) patients who underwent initial resections for Type 2 showed good improvement (more than 50% pre-op pain or VAS 
anatomy failed to have durable relief and recurrence of their initial 3), 3/18 (17%) reported adequate improvement (less than 50% pre-
pain, with 7 of the 12 patients undergoing another procedure at op reduction), and only 2/18 (11%) had no improvement at the last
the index level (3 repeat decompressions and 4 fusions). follow-up.
Of the 3 patients who underwent repeat decompression/reduc- All 3 patients with only “adequate” improvement had one or
tion Bertolotti’s procedures, 1 was successful in keeping pain 2/10 more surgical complications from their initial fusion procedure,
or less, 1 was still a treatment failure but refused further surgical and none of them had surgical hardware (screws and rods) placed.
intervention, and 1 showed some improvement in pain but did not 2 of the 3 patients had pseudoarthroses, and then subsequent
fall under the criteria for good improvement. revision fusion surgeries with the senior author, and proved suc-
Of the 4 patients who subsequently underwent fusion at the cessful with good reduction in their pain. The last patient had a
lumbosacral junction, all 4 had good improvement based on the non-union and was recommended a revision fusion, but declined
earlier criteria of reduction in pre-operative Bertolotti’s pain. any further surgical treatment, given their advanced age and
However, 1 patient developed a new pain from the proximity of the “adequate” improvement. All three of these patients had under-
S1 pedicle screw to the iliac wing but refused removal of that screw gone un-instrumented fusions (using rhBMP-2 and allograft, but
despite a solid fusion. 1 went on to develop L4/5 spondylolisthesis without screws). After this third failure of uninstrumented fu-
but refused surgical treatment of that level despite 6/10 pain that sions, no further patients were offered uninstrumented fusions as
was 80% reduced with L4/5 medial branch blocks. The remaining an options for Type 2 anatomy.
2 patients had adequate improvement in their pain following The 2 patients that did not see any initial improvement post-
surgery at 2 years after surgery. operatively had subsequent surgeries with different providers and
Of the 5 remaining decompression for Type 2 patients who were lost to follow-up in spite of repeated attempts to reach them.
refused either “Bertolotti’s” reoperation procedure, all had some Patients in this group, even with good outcomes, were not
persistent pain, refractory to conservative treatments. 1 patient without subsequent adject level back pain “generators” in future
underwent subsequent laminotomies and diskectomy at the L5/S1 follow-up data. Confirmed or suspected adjacent level diagnoses
level due to a delayed new disk herniation at the index level, and developed in 8/16 patients: 4 L4/5 spondylolistheses; 1 confirmed
after that procedure was noted to have 1/10 pain (a “good” and 1 possible cluneal nerve entrapment; 1 confirmed and 1 sus-
outcome eventually) at the last follow-up; this patient was pain- pected possible SI Joint aggravation; 1 additional patient under-
free initially after the Bertolotti’s decompression until a sudden went a removal of previously placed SI fusion screw with excellent
onset of leg pain 5 months after surgery, and a new MRI resolution of those symptoms; 1 solid fusion but new L4/5 spon-
demonstrated a new L5/S1 disk herniation that was not present dylolisthesis, refusing intervention as pain improved from 8/10 to
prior to their decompression procedure; this was considered a 4.5/10; 1 patient developed both target level pseudoarthrosis and
treatment failure at the index level of the initial decompression suprajacent L4/5 spondylolisthesis; 1 patient had a solid fusion at
procedure because it was believed a fusion would have prevented L5/S1 but developed L4/5 spondylolisthesis and did well after
this disk herniation. 1 patient developed a delayed fracture in their fusion L4/5; and 1 patient developed an unclear combination of
remaining L5 transverse process tip, but refused surgical resection L4/5 and possible cluneal nerve or SI joint symptoms and declined
of the fractured portion despite their 8/10 VAS pain level; it was further follow-up with us after 2 years.
also believed that a fusion at this level would have prevented the
fracture. 1 patient had a hip replacement after their decompres- Minimally Invasive Surgery Decompression versus Fusion
sion but still had the same pre-operative pain afterward and was Analysis
lost to follow-up. The remaining 2 patients were recommended A total of 36 patients with Type 2 anatomy underwent minimally
fusions at the index level but refused surgical treatment. invasive surgery (MIS) decompressions (n ¼ 18) or fusion (n ¼ 18)

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ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

form of iliac contact between the transverse process. Of those with


Table 2. Breakdown of Patients With or Without Iliac Contact hip pain, 59% (16 of 27) had iliac contact with the transverse
Patients with Hip Pain and Patients with Hip Pain process. Of those patients with hip pain preoperatively, 78% (n ¼
Status No Iliac Contact and Iliac Contact 21) had their hip pain improve after surgery for Bertolotti syn-
drome. The breakdown of those with or without iliac contact is
Improved 9 (33%) 12 (44%) shown in Table 2.
Did not improve 2 (7%) 4 (15%) Thus, patients with hip pain and iliac contact had a 75% chance
of improving, while those with hip pain and no iliac contact had
an 82% chance of improving with surgery treating just the
lumbosacral transitional anatomical variation (P ¼ not signifi-
procedures as their initial treatment. From the initial treatment, cant). Of the patients with hip pain, 11 had severe hip pain (pain of
the Type 2 decompression group had good improvement in 6 of 18 at least 8 on VAS scale). All 11 with severe pain had at least
(33%) patients, while the fusion group had good improvement in adequate improvement in this hip pain, with the average being a
13 of 18 (72%) patients, showing a significant difference between 5.6-point improvement. A “good” outcome occurred in 6 (55%)
the procedures (P ¼ 0.044). patients with regard to hip pain.

Type 3 DISCUSSION
No Type 3 patients were identified as having pain from the tran-
From the original cohort of 103 surgical patients with Bertolotti
sitional level itself, so none were operated upon.
syndrome with or without other spinal comorbidities, 47 (or
45.6%) had more than 1 surgical diagnosis and underwent a more
Type 4 complex procedure than isolated Bertolotti syndrome treatments
Seven patients underwent surgery for their symptomatic Type 4 and were excluded from this analysis, to focus exclusively on pa-
LSTV anatomy (right open ¼ 4, left open ¼ 3) with a unilateral tients with isolated Bertolotti syndrome requiring treatment of the
instrumented fusion, although 1 patient also underwent instru- lumbosacral transitional level. These other patients will be
mentation without fusion for possible fracture developing on the analyzed separately. However, this incidence is consistent with
“fused” side. Six patients did well with unilateral fusion of the other authors, who identified about a 40% incidence of clinically
“open” (or Type 1/Type 2 side) as treatment of the pain that was significant issues at adjacent levels in patients with LSTVs.5 This
improved by the injection. One patient did not have satisfactory does not include those patients who were found to also have
response to surgery (even though he did well with preoperative distant neurologic issues including cervical myelopathy, thoracic
injections into the open side), was found on CT scan to have a outlet syndrome, and who underwent treatments for those
solid fusion on the operated side, underwent exploration of the entities. An analysis of other associated congenital and
(solid) fusion and removal of the hardware, still with no degenerative anomalies in this patient population (symptomatic
improvement in any of his symptoms. All Type 4 patients had LSTV) is ongoing.
solid fusion of the operated sides of these procedures which The 56 patients in this cohort underwent a total of 66 proced-
resulted in the equivalent of Type 3 anatomy. ures, which were performed by the senior author over this same
Three of 7 of these Type 4 patients developed 1 or more adjacent 10-year period and resulted in an overall improvement in the pa-
level pathology. One (whose index procedure was an L6-S1 tran- tients functioning. The treatment paradigm for Type 1, Type 3, and
sitional level) developed Baastrup syndrome 2 levels above the Type 4 LSTVs with only Bertolotti syndrome as their pain gener-
fusion as well as having pain in his SI fusion screw sites; this ator did not vary over the time of collection of this data (2012 to
patient did well with subsequent removal of the SI fusion screws the beginning of 2022).
as well as removal of the L4 and L5 (of 6) spinus processes. One The treatment paradigm for symptomatic Type 2 LSTVs shifted.
was found to have cluneal nerve and other peripheral nerve Initially, treatment was effectively limited to MIS partial resections
entrapment syndromes, although that workup is still ongoing. (also known as “reduction” or “decompression”) of the transverse
One had persistent but slowly improving (and different location) process and/or ala of the transitional levels, but after a high rate of
low back pain, which coincided with the radiographic subsidence clinical failures of the resections for the Type 2 patients, we moved
of his previously placed suprajacent (L5-L6) artificial lumbar disk. to a minimally invasive fusion paradigm for all 3 of the Type 2
This pain was not present prior to his Bertolotti surgery. anatomic variants (Type 2A, Type 2B, Type 2C), based on the
surgical outcomes from those initial 20 patients with that
Iliac Contact anatomic finding; additionally, after 3 patients failed to fuse with
Patients with contact between their transverse process and iliac un-instrumented procedures, all fusions were performed with at
wing and hip pain preoperatively were very likely to have least 1-sided instrumentation.
improvement or resolution of that hip pain postoperatively. In Initially, in this series, the first 31 patients all underwent
some cases, patients who had undergone hip surgery for this “hip minimally invasive or open decompressions for Type 1 and Type 2
pain” had resolution of symptoms that were not improved by that anatomy, regardless of symptomatic subtype (Type 3 patients were
prior hip surgery. excluded from surgery as none were found to have the LSTV level
Of 56 surgical patients, 27 (48%) presented with some be the pain generator, and no Type 4 patients had presented for
component of hip pain. Of 56 patients, 50% (n ¼ 28) had some surgery by then), but after the first 18 patients with Type 2

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ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

anatomy were operated on, we performed an interim subgroup for hip surgery for (what might be incidental) findings in the
analysis, which suggested a high rate of failure and subsequent re- hip joint first.
operation. Three patients with Type 2 anatomy underwent Patients who underwent successful treatment of their Bertolotti
attempted fusion without instrumentation, and all 3 failed to fuse. syndrome are still subject to adjacent level or joint degeneration,
After this, all patients with favorable medical conditions and Type and should be observed over time for SI, lumbar, and hip pa-
2 anatomy were recommended to undergo the minimally invasive thology developing over time. More longitudinal and larger patient
fusion and instrumentation procedure. series will be necessary to look at what impact the surgical
Six patients underwent unilateral surgery for symptomatic Type treatment of Bertolotti’s Syndrome patients has on these joints,
4 anatomy, 1 of those patients was previously reported in a case compared to their natural untreated history, and to patients
report.14 One additional Type 4 patient presented with a fracture without these congenital anomalies.
of her Type 3 anatomy and the Type 2 “pseudoarticulation” on the By further describing the additional clinical presentations of
other side (Type 4B with fracture) and underwent bilateral Bertolotti syndrome, which may overlap with other potential pain-
instrumentation but only unilateral bone grafting on the Type 2 generating joints (such as sciatica, referred hip and SI pain
side, with good outcome of this pain. potentially coming from the facets or disks of the spine, hip pa-
thology, or SI instability), we hope to avoid misdiagnoses that
result in inappropriate nonsurgical and surgical treatments (such
CONCLUSIONS as SI fusions, hip replacements, and diskectomies, all of which
Bertolotti syndrome is much more common than previously were noted in our series to have preceded the diagnosis and
thought, and many patients may go misdiagnosed for years, but in treatment of some Bertolotti syndrome patients, and to have not
others the diagnosis and treatment can proceed in less than 6 benefited the patients’ symptoms).
months from initial suspicion to surgical treatment and resolution Keeping an open mind among pain management, physiatric,
of symptoms. Using the new Jenkins nomenclature system for orthopedic, and neurosurgery colleagues to the possibility of
LSTVs, we feel this both helps to identify patients who might be Bertolotti syndrome causing these overlapping symptoms will
missed in the diagnosis (compared with the older Castellvi sys- be the key to reducing the likelihood of failures of those sur-
tem), as well as directing the treatment strategy based on both gical procedures, and direct surgical treatments to the causative
anatomy type and the above evidence-based guidelines. etiology for these patients—the LSTV itself. The use of differ-
Bertolotti syndromeecaused sciatica can be differentiated from ential injections for patients with possible overlap can be used
herniated disk or foraminal stenosisecaused sciatica by the dif- to their advantage with a high degree of success, if the in-
ferential response to epidural steroids and Bertolotti targeted in- jections are done properly. Although not all patients with an
jections. For those who fail conservative management of their LSTV have pain from it, when noticed and when the condition
Bertolotti syndrome symptoms, surgery to correct or address the could also cause the symptoms the patient is suffering from, it
anatomic and biomechanical anomalies was highly successful, should be eliminated from the diagnosis prior to proceeding
although following a variable time course in recovery. Surgical with any other surgical procedures that do not address the
treatments (56 patients, 66 surgeries) fell into 2 categories: re- LSTV directly.
sections (of the transverse process and/or alar prominence) and Fortunately, surgical treatment of their Bertolotti syndrome
fusions. In some cases, patients who underwent resections and resulted in improvement or resolution of the same symptoms the
then failed to have durable relief underwent subsequent fusions at patients had, even if previously “unsuccessfully” treated, even
those levels, and in those cases most did well with the fusions. when the symptoms persisted for years. We hope these data
Within the surgical subgroup, we found after an evolution of provide guidance for those early in their Bertolotti syndrome
our treatment paradigm that patients with Type 1 anatomy did treatment experience to consider instrumented fusions for all but
better with resection surgery, while patients with Type 2 or Type 4 Type 1 patients who fail conservative management.
anatomy did better with instrumented fusions (unilateral for Type
4, bilateral for Type 2). Type 3 patients (bilateral fusions of the
transverse-ala junctions) were not found in this series to have pain CRediT AUTHORSHIP CONTRIBUTION STATEMENT
derived from the LSTV anatomy directly, but that their pain Arthur L. Jenkins: Conceptualization, Methodology, Validation,
generator was from another level or location and were never Investigation, Data curation, Resources, Writing e original draft,
identified as having pain from the LSTV itself. Writing e review & editing, Visualization, Supervision, Project
Over the course of our treatments in the last 10 years, we administration. Richard J. Chung: Validation, Formal analysis,
noticed not only that hip pain was a component of Bertolotti Investigation, Data curation, Writing e original draft, Writing e
syndrome, but that patients with contact between their trans- review & editing, Visualization. John O’Donnell: Validation,
verse process and iliac wing were more likely to have hip pain Formal analysis, Investigation, Data curation, Writing e original
preoperatively, and more likely to have resolution of that hip draft, Writing e review & editing, Visualization, Project admin-
pain postoperatively, so that aspect of the anatomy was added istration. Charlotte Hawks: Validation, Formal analysis, Investi-
to the classification system. Severe hip pain also showed a gation, Data curation, Writing e original draft, Writing e review
larger improvement postoperatively than more mild cases, & editing, Visualization, Project administration. Sarah Jenkins:
suggesting that patients diagnosed with Bertolotti syndrome Conceptualization, Methodology, Validation, Investigation, Data
and severe hip pain could see durable relief with treatment curation, Resources, Writing e original draft, Writing e review &
aimed at their transitional anatomy alone, rather than opting editing, Visualization, Supervision, Project administration.

e28 www.SCIENCEDIRECT.com WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2023.03.012


ORIGINAL ARTICLE
ARTHUR L. JENKINS ET AL. SURGICAL OUTCOMES OF BERTOLOTTI’S SYNDROME

Daniella Lazarus: Validation, Formal analysis, Investigation, Data analysis, Investigation, Data curation, Writing e original draft,
curation, Writing e original draft, Writing e review & editing, Writing e review & editing, Visualization. Camryn Harvie: Vali-
Visualization. Tara McCaffrey: Validation, Formal analysis, dation, Formal analysis, Investigation, Data curation, Writing e
Investigation, Data curation, Writing e original draft, Writing e original draft, Writing e review & editing, Visualization. Stavros
review & editing, Visualization. Hiromi Terai: Validation, Formal Matsoukas: Writing e review & editing.

biomechanical continuum. Med Hypotheses. 2013; 12. Bahadir Ulger FE, Illeez OG. The effect of
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