Syng Nathi A

SYNGNATH
IA
Dr Azhar Imran
FCPS-II Trainee
OMFS
Agenda
Introduction
Clinical Features/Diagnosis
Associated Syndromes
Etiology
Classification
Literature
Management/Surgery
Conclusion
Introduction
• Syngnathia (‘‘syn’’ = fusion and ‘‘gnathos’’ = jaw) in its
literal sense means fusion of jaws.
• This fusion could be fibrous, bony or a combination of
both.
• Rare craniofacial disorder seen in infants. (Till date
~120+ cases reported in literature).
• Associated with various syndromes.
• Varying in severity from a single mucosal band
(synechiae) to complete bony fusion (synostosis).
Features
• Congenital Syngnathia primarily affects mouth opening
causing difficulty in feeding and respiration.
The coexisting head and neck abnormalities include:
• cleft lip.
• cleft palate (most common)
• clefts of mandible, oblique facial clefts.
• tongue anomalies (bifid, small or absent).
• glossopalatine ankylosis.
• mandibular hypoplasia, coloboma, hypophyseal
duplication and frontonasal malformations.
• (can be confused with TMJ ankylosis)
Associated
Syndromes
Van der Woude
• Lip pits
• Cleft lip/palate
• Hypodontia
• Syngnathia
Popliteal pterygium
• characterized by skin webs on the

popliteal fossa
• Syngnathia
• Syndactylyl
• Bifid scrotum
Dobrow Syndrome
• A rare multiple congenital defect/dysmorphic
syndrome
• characterized by variable degrees of

bony syngnathia
• growth retardation
• Microcephaly
• Iris coloboma, nystagmus
Craniofacial microsomia
• one or both sides of the face (facial) is
underdeveloped
Fryns syndrome
• Fryns syndrome is characterized by
diaphragmatic defects. LETHAL birth defect.
• characteristic facial appearance (coarse facies,
wide-set eyes, a wide and depressed nasal bridge
with a broad nasal tip, long philtrum, low-set and
anomalous ears, tented vermilion of the upper lip,
wide mouth, and a small jaw).
• short distal phalanges of the fingers and toes
Etiology
The etiopathology of syngnathia
remains unknown, and various
hypotheses have been proposed such
as:
1. depressed fetal swallowing reflexes (Humprey)
2. failure of tongue protrusion, especially in a small jaw (Walker and Fraser)
3. period and magnitude of the traumatic forces (Laster)
4. uncontrolled expansion of ectoderm (Snijmann)
5. abnormality of the stapedial artery (Poswillo)
6. premature loss of neural crest cells (Hegtvedt)
7. teratogenic agent, persistence of the buccopharyngeal membrane (Kamata)
8. amniotic constriction bands in the developing brachial arch region, drugs such as
large doses of vitamin A and mutilating environmental insults (Nanda)
Classification
Laster et al.
Management
Management
• Primarily goal = adequate mouth opening
• Treatment = excision of tissue
• Parameters = site and extent of lesion
• Computed tomography plays a pivotal role in
treatment planning.
Surgery
• Fibrous adhesions = electrocautery/scalpel
• Bony fusion = osteotomies
• Post-op vigorous mouth opening/jaw exercises
Surgical approach
• Intraoral/extraoral
• Submandibular, preauricular
• TMJ ankylosis = interpositional gap arthroplasty
(to prevent recurrence).
Literature on Surgical
Procedure/Guidelines
• Approaches in literature: temporo-

preauricular approach, submental approach,
intraoral approach .
• Interpositional flap using temporalis muscle
or buccal mucosa, silastic sheets to prevent
recurrence.
• Post-op appliances
• Choi et al. reported use of radial forearm

free flap to prevent refusion (adequate
mouth opening)
• However, secondary TMJ surgery & lip
surgery using temporalis for relapse.
• To prevent relapse = detachment of
the affected temporalis muscle and
coronoidectomy.
• Radial forearm free flap = with the
separation of the maxilla and the
mandible.
• Secure airway and feeding.
• Removal/resection of fusion.
• Post-op exercises/treatment
• Early surgery = to avoid the TMJ ankylosis of
both TMJs and prevent secondary
developmental deformity.
Literature/Historical perspective
(surgery)
Stenberg (1983) used gradual mechanical
separation of gingiva over a 2-week period.
Blunt dissection of the bands under GA.
Randall (1984) Division of bands with

scissors, bleeding control using LA with
epinephrine.
Daskin and Sawyer (1988) divided the

bands on the second day of life.
Follow-up
• Regular follow-up of this condition is necessary
along with active periodic jaw movements,
physiotherapy.
CASE
REPORT
A 12-year-old boy with a soft tissue
band connecting upper and lower jaws.
Pain on mouth opening.
Difficulty in swallowing.
History of incomplete cleft palate that
had been repaired at another clinic
when he was three years old, but that
the intra-oral bands had been left to be
excised later.
On oral examination, a scar was seen
on the soft palate midline as the result
of the incomplete cleft palate operation, Case Report (Dept. of Plastic and
as well as two symmetrical mucosal
bands of about 5 cm length from the
Reconstructive Surgery, Etlik
palate to the inferior alveolar arch. Training Hospital, Ankara, Turkey)
Continued
Mucosal bands excised = electrocautery.

Histopathology = a fibroepithelial lesion, with
vascularity and slight perivascular inflammation in
the polypoid structure.
No post-op problems, mouth opening and swallowing
restored.
Post-op exercises such a vigorous mouth opening
were advised.
Conclusion
References
Thank You.

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SYNGNATH

• characterized by skin webs on the

• characterized by variable degrees of

• Approaches in literature: temporo-

• Choi et al. reported use of radial forearm

Randall (1984) Division of bands with

Daskin and Sawyer (1988) divided the

Mucosal bands excised = electrocautery.

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