26 PhiliPPine Journal of otolaryngology-head and neck Surgery
PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 28 no. 2 July – december 2013
CASE REPORTS
ABSTRACT
Objective: To present a rare case of congenital maxillomandibular fusion or syngnathia.
Methods:
Study Design: Case Report
Setting: Tertiary Public Teaching Hospital
Participant: One patient
Results: A 3-year-old girl with oral adhesion (syngnathia) caused by a mandibular to maxillary
fibrous band with bony fusion underwent successful surgical division and release. Subsequent
monitoring and serial oral dilations were performed post operatively, resulting in mouth opening
of 24mm over a period of three months. Currently, the patient is able to tolerate a general liquid
diet.
Conclusion: Congenital maxillomandibular fusion is a very rare condition with few cases reported.
We hope this report contributes to its diagnosis and management in other children.
Keywords: congenital maxillomandibular fusion, syngnathia
From birth, the upper aerodigestive tract of neonates plays an important role in simultaneous
nose-breathing, suckling and swallowing. It is crucial for this mechanism to function properly to
ensure survival. Problems that interfere with this design, such as bilateral choanal atresia, may
result in death, unless attended to emergently. This report a case that could have had similar
consequences over a slightly longer course.
CASE REPORT
A newborn girl from Nueva Ecija, Philippines was noted to have maxillo-mandibular fusion
after full-term spontaneous home delivery to a 31-year-old G3P3 (3003) mother attended by
a midwife. The mother was a non-smoker and non-alcoholic beverage drinker with irregular
prenatal check-ups but had multivitamins and ferrous sulfate during pregnancy. She took
unrecalled medications for an upper respiratory tract infection at 1 to 2 months of pregnancy
and denied exposure to viral exanthems, chemicals, radiation or teratogenic drugs.
On examination, there was complete bilateral fusion of the bony upper and lower jaws. A
nasogastric tube was inserted through the right nasal cavity. The rest of the physical examination
findings were normal. Complete blood count revealed a white cell count of 18.45 with segmenter
predominance. She was started on Ampicillin 60 mg/IV every 8 hours, Gentamicin 14 mg/IV
Congenital Maxillomandibular Fusion: A Rare
Case of Isolated True Bony Syngnathia
Patrick O. Aguiling, MD1
Nikki Lorraine Y. King-Chao, MD1
Lyra V. Veloro, MD1,2
1Department of Otorhinolaryngology
Head and Neck Surgery
The Medical City
2Department of Child Neuroscience
Section of Pediatric Otorhinolaryngology
Philippine Children’s Medical Center
Correspondence: Dr. Patrick Aguiling
Department of Otolaryngology- Head and Neck Surgery
The Medical City
Ortigas Avenue, Pasig City 1600
Philippines
Phone (632) 6356789 local 6250
Email: patrickaguiling@yahoo.com
Reprints will not be available from the author.
The authors declared that this represents original material
that is not being considered for publication or has not been
published or accepted for publication elsewhere, in full or
in part, in print or in electronic media; that the manuscript
has been read and approved by all the authors, that the
requirements for authorship have been met by each author,
and that each author believes that the manuscript represents
honest work.
Disclosures: The authors signed disclosures that there are no
financial or other (including personal) relationships, intellectual
passion, political or religious beliefs, and institutional affiliations
that might lead to a conflict of interest. Philipp J Otolaryngol Head Neck Surg 2013; 28 (2): 26-28 c Philippine Society of Otolaryngology – Head and Neck Surgery, Inc.
PhiliPPine Journal of otolaryngology-head and neck Surgery 27
PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 28 no. 2 July – december 2013
CASE REPORTS
OD, Vitamin K 0.5 mg/IM and Oxytetracycline eye ointment, and she
eventually improved. The assessment was congenital maxillomandibular
fusion or syngnathia. (Figure 1)
CT scan of the facial bones with 3D reconstruction showed
complete bony fusion of the maxilla and the mandible with hypoplasia
of the temporomandibular joints. (Figure 2) Radiographs showed
no definite lung infiltrates, cardiothymic shadow within normal
limits, intact diaphragm and costophrenic angles, gastrointestinal
tract patterns within normal, no abnormal mass or calcifications, no
pneumoperitoneum or organomegaly and no osseous, joint space or
soft tissue abnormalities appreciated.
At two months of age, elective tracheostomy under local anesthesia
was performed due to dyspneic episodes associated with desaturations
exacerbated by recurrent upper respiratory tract infections. Anesthesia
was maintained through the tracheostomy tube while a gastrostomy
was carried out. She tolerated the procedure well and was eventually
discharged, breathing spontaneously and well oxygenated. Several
follow-up visits included a repeat CT scan of the facial bones with 3D
reconstruction (including reference markers) at 1 year and 5 months of
age, but she was not seen again until 3 years of age.
At 3 years and 6 months of age, she underwent surgical division of
the bony fusion. An extended right pre-auricular incision and dissection
of the superficial temporal fascia over root of zygoma were carried to
the fascia and periosteum which were incised over the zygomatic root.
The skin flap and parotid were retracted anteriorly and the coronoid
process and zygomatico-maxillo-mandibular area were exposed. With
maximum retraction to facilitate optimal exposure of a long tunnel
medial to the masseter muscle, the mandibular fusion was released
and the mucosal incision was gradually extended to the midline. The
bony fusion was exposed by dissecting the mucosa away from the bone
using a periosteal and freer elevator. Osteotomy of zygomatico-maxillo-
mandibular fusion was achieved by tunneling from the zygomatic area
to the midline by alternately using an osteotome, oscillating saw and
high-speed surgical drill with different-sized cutting bur tips. The same
procedure was replicated on the left side up to the midline until the
division from the right side was reached. The bony division was then
pried open until all fibrous bands were detached. Sharp edges were
smoothened using a surgical drill with diamond bur tips. Avulsed central
and lateral incisors were removed. The oral cavity was established and
packed with medicated gauze impregnated with antibiotic ointment.
The patient underwent serial oral dilation on succeeding follow-ups,
eventually achieving mouth opening of 24mm within 3 months. (Figures
3, 4) Currently, the patient is able to tolerate a general liquid diet.
Figure 1. Complete bilateral fusion of the bony and soft-tissue components of the upper and
lower jaws.
Figure 2. 3D reconstruction CT scan of the facial bones showing complete bony fusion of the
maxilla and the mandible with hypoplasia of the temporomandibular joints.
Figure 3. Oral dilation at 15mm (4 weeks postoperative)
28 PhiliPPine Journal of otolaryngology-head and neck Surgery
PhiliPPine Journal of otolaryngology-head and neck Surgery Vol. 28 no. 2 July – december 2013
CASE REPORTS
REFERENCES
1. Mir M, Iqbal S, Hafeez A, Zargar H, Rasool A, Mohsin M, Darzi A. Syngnathia without any other
associated anomaly: A very rare case report. The Internet Journal of Plastic Surgery. 2006;4(1).
Cited 2013 March 24. Available at:http://ispub.com/IJPS/4/1/13380.
2. Bali R, Sharma P, Jain S, Thapar D. Congenital fibrous maxillomandibular fusion. J Maxillofac Oral
Surg. 2010 Sep; 9(3): 277–279.
3. Puvabanditsin S, Garrow E, Sitburana O, Avila FM, Nabong MY, Biswas A. Syngnathia and Van der
Woude syndrome: a case report and literature review. Cleft Palate Craniofac J. 2003 Jan; 40(1):
104-6.
4. Dawson KH, Gruss JS, Myall RW. Congenital bony syngnathia: a proposed classification. Cleft
Palate Craniofac J. 1997 Mar; 34(2):141–146.
5. Goodacre TE, Wallace AF. Congenital alveolar fusion. Br J Plast Surg. 1990 Mar; 43(2): 203–209.
6. Poovazhagi V, Vijayakumar V, Kumudha J, Balachandran K. Congenital fusion of maxilla and
mandible (bony syngnathia). Pediatric Oncall 2009. Cited 2009 December 1; 6(12) Art 63.
Available at http://www.pediatriconcall.com/fordoctor/casereports/syngnathia.asp
DISCUSSION
Congenital causes of limited mouth opening involving fusion of
the maxilla and mandible (syngnathia) are a rare group of anomalies.
Cases are classified into those involving bony tissue and those involving
soft tissue alone. Occurrence may be unilateral or bilateral, partial or
complete.1,2 Among the abovementioned classifications, cases involving
solely soft tissue and bilateral fusion in the posterior region are more
common.2
Isolated occurrence of bony syngnathia is a very rare condition with
few reported cases. In the literature, the congenital defect is associated
with other anomalies like Van der Woude syndrome, popliteal pterygium
syndrome2,3 and aglossia–adactylia syndrome. Our patient has none of
these syndromes or any other intraoral or maxillofacial abnormalities.
No sex predilection has been reported. 2,4 However, it is interesting
to note that eight out of 11 cases reviewed for this study have male
subjects.
The exact pathogenesis of congenital bony fusion is unknown.
Some of the etiologic hypotheses proposed by Goodacre and Wallace5
include “persistence of buccopharyngeal membrane, amniotic
constriction bands in the region of the developing first branchial arch,
environmental insults, drugs such as meclozine and large doses of
Vitamin A.”1,2 According to a review by Dawson et al., there is no familial
tendency, history of drug or toxin exposure and consanguinity.1,2,4
However, there is a possibility of autosomal recessive inheritance. Mir
et al. and Poovazhagi et al. reported cases which revealed a history of
consanguinity.1,6
Our patient presented with maxillomandibular fusion discovered
immediately after birth. Physically, oral cavity deformity was evident
and the baby was not able to open her mouth or feed normally. Once
recognized, the diagnosis was confirmed by CT scan. These events are
congruent with the observation that congenital bony fusion is clinically
diagnosed soon or after birth as the neonate presents with airway and
feeding difficulties.1 The modality of choice is high resolution or spiral
CT scan which has the advantage of revealing the condition of the
temporomandibular joints or any hypoplasia of the other facial bones.1
The usual accompanying problems in such patients include airway
and respiratory impairment, feeding difficulties, speech limitation,
poor oral hygiene, interference with salivation and mastication, and
induction of anesthesia.1 In spite of these complications, studies show
that functional results especially in isolated occurrence are likely to be
good.1 For our patient, feeding problems were addressed initially by
insertion of nasogastric tube and subsequently, by gastrostomy tube.
Treatment consists of surgical division of the maxillomandibular
fusion under anesthesia.1,6 Awake blind nasal intubation is the ideal
manner of inducing anesthesia, although a tracheostomy may be
required if blind intubation fails.1,2 A less invasive technique using
fiberoptic nasotracheal intubation may also be administered.3,6
Our patient underwent tracheostomy insertion for maintenance of
airway and anesthesia. Following surgery, active physical therapy is
recommended and the infant should be encouraged to feed normally
as soon as possible1,3,4 to achieve adequate mandibular range or motion
and function. Acceptable mouth opening may be appreciated 1-2 weeks
post operatively. 6 The mother of our patient was advised to perform
such manual therapy techniques as carefully opening the baby’s mouth
with a tongue depressor and rotating fingers to press the gums.
Indeed, congenital maxillomandibular fusion is a very rare condition
with few cases reported. We hope this report contributes to its diagnosis
and management in other children.
Figure 4. Mouth opening of 22 mm (8 weeks postoperative)