Annals of 3D Printed Medicine 6 (2022) 100053

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Technical note

3D surgical planning of neonatal mandibular distraction osteogenesis in

children with Pierre-Robin sequence
rez Ferna
Eduardo Pe ndeza,*, Marta Ayats Solerb, Marta Go
mez Chiaric, Irene Martínez Padillad,
d d
Albert Malet Contreras , Josep Rubio-Palau
a
Department of Oral and Maxillofacial Surgery, Hospital de Cruces, Spain
b
3D for Health Department (3D4H), Hospital Sant Joan de Deu (HSJD), Spain
c
Department of Pediatric Radiology, HSJD, Spain
d
Division of Maxillofacial Surgery, Department of Pediatric Surgery, HSJD, Spain

A R T I C L E I N F O A B S T R A C T

Article History: Mandibular distraction osteogenesis (MDO) is a surgical procedure that can successfully treat the microgna-
Received 8 November 2021 thia in neonates with Pierre Robin Sequence (PRS), avoiding glosoptossis and airway obstruction. While vir-
Revised 9 February 2022 tual surgical planning (VSP) and three-dimensional (3D) printing is a common technique in Oral &
Accepted 19 February 2022
Maxillofacial Surgery, it has not been widely used in neonates with this condition. The objective of this study
Available online 21 February 2022
is to describe how we employ 3D technology on MDO for neonates with mandibular hypoplasia.
© 2022 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY-NC-ND
Keywords:
license (http://creativecommons.org/licenses/by-nc-nd/4.0/)
Distraction osteogenesis
Micrognathia
Neonate
Pierre Robin sequence
3D surgical planning
3D printing

Introduction Preoperative planning and preparation improves accuracy and

Described by French stomatologist Pierre Robin in 1923, the
Pierre-Robin Sequence (PRS) consists of the clinical triad of micro-
gnathia, glosoptossis and airway obstruction [1]. The incidence of
this congenital condition ranges from 1/8000 and 1/14000 births
[2,3]. Although PRS can be an isolated finding, nearly 50% are associ-
ated with a genetic syndrome, the most common being Stickler,
22q11.2 deletion and Treacher-Collins syndrome [4−6]. Cleft palate
is frequently associated with PRS due to the malposition of the
tongue, which prevents the descent and fusion of the palatal shelves
[7]. The mechanical upper airway obstruction can lead to hypoxia
and apnea, and feeding is compromised by the hypoplastic mandible,
the cleft palate and the airway obstruction.
Mandibular hypoplasia can be treated with distraction osteogene-
sis (DO). This technique was firstly described in 1905 by Codivilla,
and it is based on new bone formation between two bony fragments
under gradual tension [8]. Nevertheless, MDO can be technically diffi-
cult, and the procedure is not without risk of complications.
This research did not receive any specific grant from funding agencies in the public,
commercial, or not-for-profit sectors.
* Corresponding author at: C/ Gregorio de la Revilla, 14, 7° Derecha, 48011, Bilbao,
Vizcaya, Spain.
E-mail address: cmfperezfernandez@gmail.com (E. Pe rez Fernandez).
reduces intraoperative bleeding and surgical time, which is especially
important in infants with PRS that are small and poorly developed
because of malnutrition. Three‑dimensional (3D) planning is a stan-
dardized technique in other maxillofacial fields like dental implants
or orthognathic surgery. Some of its advantages are better precision,
simulate different approaches or procedures; compare different vec-
tors and hardware in DO; avoid damage of neurovascular structures
and teeth; reduce complications and reoperations; lower surgical
and hospitalization time; precision and predictable results. Moreover,
3D printing of anatomical models allows better diagnosis, surgical
training, and awareness of the patient’s anatomy [9]. The aim of this
article is to present how we use 3D surgical planning and printing in
MDO in children with PRS and explain the step by step of our surgical
technique.
Methods
CT scan
The CT protocol was performed on a Brilliance 256channel iCT
(Brilliance iCT, Philips Medical Systems, Haifa, Israel), following imag-
ing parameters with dose reduction, limited FOV and Idose

https://doi.org/10.1016/j.stlm.2022.100053
2666-9641/© 2022 The Authors. Published by Elsevier Masson SAS. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/)
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al.
reconstruction protocol: 100 kV, 87 mA, 4 cm x 4 cm collimation.
Radiation dose: DLP: 82.5 mGy*cm; CTDI vol (mGY): 7.2. (Fig 1)
Virtual surgical planning (VSP)
The general 3D VSP and 3DP process is shown in Fig. 2.
The first step of the process is the image acquisition, which, in
most of the cases will be the same image used for diagnostic pur-
poses. A CT is used for surgical planning.
Once the CT is performed and the quality of the image is analyzed,
the next step of the process is image segmentation. This part of the
process consists of converting a group of 2D images from the CT
series into a 3D volume of the anatomical structures that will be
needed for the surgical planning. Different segmentation techniques
are available in the different software for image postprocessing. Usu-
ally these techniques are:
Manual segmentation: it is usually used for soft tissues that are
segmented from a CT or an MRI and for every tissue segmented
from an MRI.
Semiautomatic segmentation: it is the one that is done by thresh-
old of Hounsfield Units (HU) and is basically used to segment
structures that have HUs considerably different from the struc-
tures near them.
Automatic segmentation: This is the most used to segment bony
structures from CT series. It is mainly used to hyperdense struc-
tures from a CT, such as bone or teeth.
For the described case, the software IntelliSpace Portal from Phil-
lips is used for both, manual and automatic segmentation. Segmenta-
tion was done automatically for the bone and teeth, as the medical
image was a CT and the desired structures were hyperdense struc-
tures. Regarding to the dental nerve, the segmentation was manual
and from the same CT scan. In total, the segmentation process takes
approximately 60 min and was done by the engineer responsible for
surgical planning and reviewed by the specialized radiologist of the
Hospital. A 3D volume of the cranium, mandible, the teeth, and skin
contour of the patient is obtained (Fig. 3).
When the segmentation is done, the surgical virtual simulation
starts. The aim of this process is to simulate all the steps of the sur-
gery to be able to detect the main difficulties and define some param-
eters such as the angle of position of the distractors or the position of
the osteotomy in this specific case. For surgical planning, the first
Fig. 1. Ten-month-old infant who has isolated Pierre Robin sequence. (A) Preoperative 3DCT showing retromicrognathic mandible and abnormalities of the mandibular condyle and
coronoid process. (B) Sagittal CT scan demonstrating glossoptosis. (C) Coronal and (D1 − D2) parasaggital CT scan shows bilateral abnormal TMJs with dysplastic condylar processes.
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Annals of 3D Printed Medicine 6 (2022) 100053
step is the definition of the ideal final position of the mandible, by
defining the wanted relation between the maxilla and the mandible
in the final position. This ideal final position will define the position
of the osteotomy and the distraction vector. The next step then, is the
simulation of the osteotomy, that should be perpendicular to the dis-
traction vector and positioned taking into account the relative posi-
tion of the teeth and the needed space in each side of the osteotomy
so that the distractor can be positioned (Fig. 4). After the simulation
of the osteotomy is performed, a 3D volume of the distractor that will
be used during surgery is imported in the software to be used for sur-
gical simulation. The distractor is then positioned considering the
ideal angle of the distraction to be performed and the distance
between the distractor and the teeth (Fig. 5). Finally, a simulation of
the mandibular distraction is done to ensure that the final position of
the mandible for the defined distraction vector is creating a proper
final result (Fig. 6). VSP takes approximately 4 h, not including the
segmentation processes, and it is performed completely in house by
the 3D Unit of the Hospital (3D for Health Department (3D4H), Hospi-
tal Sant Joan de De u).
Once the virtual simulation process is finished, a 3D Printing of
the mandible is performed so that the surgical team can practice the
positioning of the Kirshner wires and the distractors before surgery.
In this case, since no patient specific surgical instruments such as cut-
ting and positioning guides were used, the anatomical model was
printed using a Fused Deposition Modeling (FDM). A replica of the
initial mandible of the patient and a second replica of mandible with
the osteotomies performed and the Kirschner wires positioned were
printed using Polylactic acid (PLA). EPSILON printer from BCN3D
Technologies was used.
Results
Surgical technique
We perform MDO in neonates under general anesthesia and naso-
tracheal intubation. After intraoral infiltration with lidocaine and
adrenaline at 1:100.000, we raise an intraoral mucoperiosteal flap at
each mandibular body. Mandibular body is exposed and osteotomies
on buccal and basilar mandibular cortex are performed using piezo-
electric surgery device (VarioSurg3, NSK. Nakanishi Inc. Japan). The
osteotomy path line and the position of the needles are carefully
determined in the virtual planning in order not to damage dental
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al.
Fig. 2. 3D Virtual Surgical Planning and 3D Printing process.
germs and neurovascular structures (i.e., inferior alveolar nerve). We
use two mini-Molina unidirectional external distractors (KLS-Martin.
Ludwigstaler Str. 132. D-78,532 Tuttlingen, Germany) and two
Kirschner needles 1.5 mm diameter (Fig. 7). No surgical splints or
positioning guides are used in these patients, as there are no teeth to
fix them. The needles are inserted percutaneously in a parallel posi-
tion, passing through buccal and lingual cortex on each side. Anterior
traction of the tongue is performed when the posterior needle is
inserted, relieving the glossoptosis. The osteotomy is completed on
the lingual cortex. Separation between the two bone fragments must
be accomplished after untensional activation of the device; if not,
osteotomies are reviewed with osteotomes and piezoelectric. The
device is deactivated until a distracted distance of 2 mm on each side.
The wound is irrigated and closed in layers with resorbable monofila-
ment 5/0. MDO surgical time takes approximately 60 min, depending
on the complexity of the case.
Fig. 3. STL file obtained from CT scan. Initial situation with open mouth (A) and closed mouth (B).
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Annals of 3D Printed Medicine 6 (2022) 100053
Postoperative care & follow-up
After the operation, the patient is hospitalized intubated in the
neonatal intensive care unit. Antibiotics, corticosteroids, and analge-
sic treatment is administrated. We start the distractor activation on
the 2nd or 3rd postoperative day at a rate of 1 mm per day. If good
evolution, activation can raise to 2 mm daily separated into morning
and evening activations. Intubation is usually maintained for 3
−4 days after the surgery to ensure the airway until the swelling is
resolved. Distraction is continued until the mandibular alveolar ridge
is 2−4 mm anterior to the maxilla. Resolution of OSA is performed
with a PSG. Adequate nutrition is ensured with an enteral nutrition.
A percentage of nutrition is orally administrated to stimulate suction
reflex. Parents are instructed to correctly activate the distractors at
home. The patient is followed ambulatory every week. At 6th-8th
week postoperative we extract needles and the distractors in the
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al. Annals of 3D Printed Medicine 6 (2022) 100053

Fig. 4. Osteotomy design regarding erupted and included teeth.

office. The procedure is unpainful so there is no need of general or
local anesthesia.
Discussion
The management of PRS children depends on the severity of the
airway obstruction. For mild to moderate, conservative therapy for
PRS include lateral or prone positioning, placement of nasopharyn-
geal airways and continuous positive airway pressure (CPAP) [10].
Severe neonatal upper airway obstruction that fails conservative
management demands surgical intervention. Tracheostomy has been
traditionally the most effective and definitive treatment, but it is
associated with high morbidity and mortality rates [11]. Tongue lip
adhesion (TLA), which consists of the adhesion of the ventral tongue
to the lower lip, was later popularized as an effective alternative to
tracheostomy, with high rates of success and a simple surgical tech-
nique. However, complications as dehiscence and the need of extra
procedures have been described, since TLA does not treat the micro-
gnathia [12].
DO was introduced in the craniofacial skeleton by McCarthy in
1992 [13] and mandibular distraction osteogenesis (MDO) two years
later by Molina [14]. An osteotomy in the mandibular body, angle or
ramus is performed and external or internal distractors are placed
and activated to length the mandible. This way, the floor of the

Fig. 5. Mandibular distractor positioning over the mandible (A) and on the soft tissues (B).

4
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al. Annals of 3D Printed Medicine 6 (2022) 100053

Fig. 6. Mandibular distraction of 7 mm simulation (A) and final results (B).

mouth and the tongue is moved anteriorly, improving the airway
obstruction. DO procedure is divided in three phases. Latency period
is the time needed for fibrous callus formation, between 2 and
7 days. Distraction period when the mandible actively lengthens
around 1 mm/day. Consolidation period lasts some weeks, time in
which the stretched callus mineralizes [15]. The MDO is considered
successful if apnea resolves, index apnea-hypopnea decreases, decan-
nulation or avoidance of tracheostomy and breathing improvements
[16]. Nevertheless, MDO can be technically difficult, and the proce-
dure is not exempt of complications such as surgical site infection,

Fig. 7. Neonate with PRS sequence and micrognathia in the operating room, before (A) and after (B, C) the surgical procedure.

5
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al.
scars, facial or mandibular alveolar nerve damage, fracture accidents,
teeth injury, bone nonunion, temporomandibular joint (TMJ) ankylo-
ses, dental injury or malocclusion [17].
Virtual surgical planning has improved the accuracy in many cra-
nio-maxillo-facial procedures. However, its use in mandibular dis-
traction in neonates with PRS has been rarely reported in the
literature [18,19,20]. In those papers, VSP is applied to plan the
osteotomies, the growth vector of DO, and the placement of internal
semi-buried distractors using, in some cases, custom cutting guides.
In our experience, we prefer external distractors since the hypoplas-
tic mandible in PRS patients can present insufficient bone support to
place a device, and use internal distractors in older patients with
hemifacial microsomia or bilateral mandibular hypoplasia [21]. The
technique with external distractors results in little tissue dissection
and short operation time, minimal skin scars and the avoidance of a
second surgical intervention to explant the distractors, since the
Kirschner needles and the mini-Molina external distractors are
removed unpainfully in the office with no need of local nor general
anesthesia. In our experience, the design of a cutting and positioning
guide has been tested in simulation models but its application is diffi-
cult in the clinical practice. The small mandible and the flat and
Fig. 8. 3D printed facial skeleton and soft tissue for surgical training.
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Annals of 3D Printed Medicine 6 (2022) 100053
edentulous bone surface make difficult the exact placement of a cut-
ting guide. Furthermore, Kirschner needles have some flexibility,
which makes it difficult for a perforating guide to lead the exact vec-
tor through both mandibular bodies.
Although further clinical data and research is needed, we think
that VSP in these patients would present several benefits: customized
osteotomies can potentially avoid damage to dental structures; it
would shorten intraoperative time; it can help to obtain the correct
direction for distraction vector; 3D printing of the hypoplastic mandi-
bles would improve the recognition of the patient’s anatomy. We can
trace the surgical steps over the anatomical model and predict the
technical challenges. It enables surgical training to surgeons of any
experience: drilling, cutting, and manipulating the mandibles
improves the surgeon’s knowledge of the technique (Figs. 8-9). 3D
printed models are useful in counseling and explaining the parents
the pathology and the intervention; understanding the procedure
may increase the acceptance of the treatment. On the other side, VSP
has some disadvantages in this population such us the exposure to
ionizing radiation. However, in our opinion, the benefit that VSP
could offer and the screening for other associated malformations jus-
tifies the radiation.
 ndez, M. Ayats Soler, M. Go
E. Perez Ferna mez Chiari et al.
Fig 9. 3D printed simulator for surgical training.
Conclusions
Treating mandibular hypoplasia in neonates can be technically
demanding, especially in PRS children who usually associate other
malformations and are poorly developed. Virtual surgical planifica-
tion and 3D-printing is widely used in many cranio-maxillo-facial
surgeries, and it is useful in shortening surgical times, limiting poten-
tial surgical complications and pitfalls, and improving the training
and knowledge of the patient’s anatomy by the surgical team. With
this paper, we show that 3D technology can be applied on neonates
with mandibular hypoplasia.
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