https://doi.org/10.1177/2732501620949195
FACE
2020, Vol. 1(1) 70–73
© The Author(s) 2020
DOI: 10.1177/2732501620949195
journals.sagepub.com/home/fac
Article
A combination of cranial vault and midfacial growth distur-
bances exist with many craniofacial syndromes. Different
mutations in the fibroblast growth factor receptor-2 gene
may lead to Crouzon or Apert or Pfeiffer syndromes, which
display restriction of the skull base, turribrachycephaly and
cranial vault restriction. This restriction may lead to elevated
ICP, which has negative consequences on long-term cogni-
tive, psychological and other functional outcomes. Midfacial
hypoplasia results in reduced orbital volume, exorbitism,
corneal exposure, and loss of vision.
1
The midface hypopla-
sia similarly produces functional problems that often need to
be addressed. Narrowing of the nasopharynx and class III
malocclusion produces sleep apnea, which affects respiration
and overall well-being.
2
Malocclusion affects mastication
and produces an aesthetic deformity.
Monobloc advancement separates the anterior portions of
the skull base, orbits, maxilla and dental arch, which remain
in continuity, and are advanced anteriorly. The procedure can
be effective in improving exorbitism, airway constriction,
and overall aesthetic appearance (Figure 1). The procedure is
not without significant complications, including bleeding,
CSF leak, wound/pin site infection, meningitis, bone loss,
and death. A mortality range of 0% to 4.5% has been reported,
with current rates less than 1%.
2
Evaluation by all members of the multidisciplinary team
permits complete assessment of the patient and improves
preoperative planning. A pre-mission genetics evaluation
will confirm the diagnosis, evaluate for associated medical
problems, and identify other risks. A pre-mission ophthalmo-
logic exam will identify papilledema secondary to elevated
intracranial pressure and increase the urgency for surgery.
Regarding preoperative imaging, which may or may not
be difficult to obtain, computerized tomography (CT) is the
gold standard. Many international centers have this capabil-
ity and are able to reformat the images in 3-dimensions. A
thorough review of the images at the time of patient screen-
ing should identify prior attempts to address the underlying
synostosis, as well as hydrocephalus, persistent ICP eleva-
tion, posterior pharyngeal space narrowing and possibly a
Chiari malformation. A lateral cephalogram may assist with
advancement planning if distraction is considered. Magnetic
resonance imaging is not necessary unless a brain abnormal-
ity requires evaluation.
In the mission setting, non-operative interventions may be
required before the team arrives to manage the patient surgi-
cally. Patching, moisturizing eye drops and tarsorraphy can
protect the cornea against orbital exposure. The benefit from
tarsorraphy may be self-limited and ultimately ineffective
due to tissue stretch and dehiscence. Continuous positive air-
way pressure (CPAP) or supplemental oxygen may be
required for mild airway obstruction, while tracheostomy is
indicated in more severe instances.
At the time of the screening, team members need to evalu-
ate the indications for surgery, all noted above. Patients with
949195FAC XX X 10.1177/2732501620949195FACE: Journal of the American Society of Maxillofacial SurgeonsTaub et al
research-article 20202020
1
Icahn School of Medicine at Mount Sinai, New York, NY, USA
2
Komedyplast Surgical Charity, Chicago, IL, USA
Corresponding Author:
Peter J. Taub, Surgery, Pediatrics, Dentistry, and Neurosurgery, Division
of Plastic and Reconstructive Surgery, Icahn School of Medicine at Mount
Sinai, 5 East 98th Street, Box 1259, New York, NY 10029, USA.
Email: peterjtaub@gmail.com
Success With Monobloc
Advancement in Mission Surgery
Peter J. Taub
1
, Anand Kumar
2
, Alex Lin
2
, Chris Bonfield
2
,
Cheryl Gooden
2
, Franklyn Cladis
2
, and Jeffrey Weinzweig
2
Abstract
Syndromic hypoplasia of the orbit and midface results in noticeable dysmorphology. Correction involves advancement of
the orbito-facial complex either directly or by distraction osteogenesis. The surgery is challenging no matter where it is
performed, however, the challenges are amplified on a surgical mission. Komedyplast Surgical Charity (Chicago, IL) has
been performing complex craniofacial procedures, including midfacial advancement at the Monobloc level, for 13 years with
safety and success. As such, key points need specific attention to avoid complications in a remote location, where staff and
resources may be less than adequately equipped to handle them.
Keywords
monobloc, maxillary hypoplasia, distraction