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Bilateral Congenital Facial Paralysis
IOTI
BRIEF REPORTS
Bilateral congenital facial paralysis
R ic h a r d A. Fogg, DDS
I he seventh cranial nerve consists of both sensory (and secretory) fibers and a larger motor root. The sensory fibers primarily contribute to the salivary and lacrimal glands, to the anterior two thirds of the tongue, and to the glands of the nose and soft pal ate. The motor root emerges from the cranial cavity via the stylomastoid foramen, extends forward to the parotid gland, and then divides into two main segments. Most of the fi bers serve the muscles of facial ex pression: the orbicularis oculi closes the eyelids, the nasal muscles dilate and contract, and the orbicularis oris and associated muscles retract, con tract, and protract the lips.1 The ab sence of the motor function of these
muscles is the primary focus of this report.
A 1 7-year-old white girl (Fig 1) was exam ined in early 1979. A medical history dis closed palsy of the right and left facial nerves that had been diagnosed at birth. When the patient was 7 years old, she was reexamined at the University of Nebraska and the diagnosis was confirmed. The prognosis was poor; little or nothing could be done. Except for the paralysis, the patient had a noncontributory medical history. Taste sensations were active, as were salivary and lacrimal flow. Excellent occlusion, m ucosa, gingiva, and dental hygiene were evident. No family members had had this rare condition. Approximately 125 cases are
known to have existed.2 The letters B and P were the most dif ficult to pronounce, because it was neces sary for the patient to compress the lower lip against the maxillary incisors to pro nounce these consonants (Fig 2, 3). The third cran ial nerve serves the strong superior levator muscle that is responsi ble for opening the upper eyelids. How ever, dysfunction of the facial nerve does not permit total contraction of the orbic ularis oculi muscle; maximum closure of the patient’s eyelids was approximately 80% (Fig 4). Through contraction of the buccinator muscle, the lips could move in a lateral direction (Fig 5). The long buccal branch of the fifth nerve ordinarily inter laces with the facial nerve that serves the buccinator m uscle.1 In Gray's Anatomy, a footnote states, “There seems to be no rea son to doubt that the branch supplying the buccinator muscle is entirely a nerve
Fig 1 ■ Patient w ith Mobius syndrome. Notice
Fig 2 ■ Patient m aking a B sound— notice po
Fig 3 ■ Patient m aking a P sound.
lack o f expression.
sition of teeth and lower lip.
Report of case
JADA, Vol. 100, January 1980 ■ 71
B R IE F
REPO RTS
Fig 4 ■ M axim um closure of eyes.
Fig 5 ■ Only facial expression w as movement
Fig 6 ■ Patient w histling.
o f lips in lateral direction.
of ordinary sensation, and that the true motor supply of this m uscle is from the facial.”3 In this case, it appears that the fifth cranial nerve serves the buccinator motor. W histling was done with tongue and teeth (Fig 6); sucking on a straw was ac complished by using the corner o f the mouth (Fig 7); and removal of soft food, such as pudding, from a spoon was ac complished by using the tongue and teeth (Fig 8). The patient compensated for the loss of lip function with facile motion.
Discussion This condition is known as the Mobius syndrome. Other characteris tics, in addition to bilateral facial paralysis, include anomalies, such as upper facial paralysis only; club foot; deformed hands, ears, or both; and aplasia or hypoplasia of pectoral or brachial musculature. Paralysis of the lateral rectus muscle (crossed eyes) is the most common accom panying condition. However, in a re view of 61 cases, Henderson4 noticed that not all cases were associated with abducens palsy. Neuropathologists have not agreed whether the cause of the syndrome is a hypoplasia of the central nervous system (involving the third, fifth, sixth, seventh, ninth, tenth, eleventh, or twelfth cranial nerves, or all of these) or an absence of primary pe ripheral muscle, or a defect that causes d egen eration of nerves through atrophy,5 To correct the lack of expression characterized by this syndrome, transposition of the temporalis mus cle has been done.5 In this procedure, 72 ■ JADA, Vol. 100, January 1980
Fig 7 ■ N otice position o f straw in sucking.
Fig 8 ■ Patient removing pudding with teeth and tongue.
a broad band of the temporal muscle is exposed above the hairline. Inci sions are then made at the outer canthi of the mouth and eye. Small strands of the muscle are separated and inserted into the upper and lower eyelids, and larger groups of muscle fibers are attached to the outer canthi of the lips. When teeth are clenched, with training, the tem poral muscle contracts; the eyelids can then be closed and a smile can be achieved. Specialists will decide whether this type of surgery is indi cated for the patient in this study.
Summary A 17-year-old girl who has the Mobius syndrome showed compen satory motions in sucking, speech, and eating. Movements of facial ex-
pression were absent (except for con traction of the buccinator muscle and some closure of the eyelids). The pa tient has been referred to a specialist for restoration of additional facial expression. Dr. Fogg is in private practice, 2226 G ulf Gate Dr, Sarasota, Fla 33581. Address requests for reprints to Dr. Fogg. 1. Schaeffer, J.P. M orris’ hum an anatomy. Philadelphia, Blakiston Co., 1946. 2. Gorlin, R .F., and Pindborg, J.J. Syndromes of the head and neck. New York, McGraw-Hill, 1964. 3. Gray, H. Anatomy of the hum an body. Philadelphia, Lea & Febiger, 1973. 4. Henderson, J.L. T he congenital diplegia syndrom e: c lin ic a l features, pathology and aetiology; a review of sixty-one cases. Brain 62:381 -4 0 3 ,1 9 3 9 . 5. Converse, J.M . R eco n stru ctiv e p lastic surgery. Philadelphia, W. B. Saund ers Co., 1977.
BRIEF REPORTS
Bilateral congenital facial paralysis
R ic h a r d A. Fogg, DDS
I he seventh cranial nerve consists of both sensory (and secretory) fibers and a larger motor root. The sensory fibers primarily contribute to the salivary and lacrimal glands, to the anterior two thirds of the tongue, and to the glands of the nose and soft pal ate. The motor root emerges from the cranial cavity via the stylomastoid foramen, extends forward to the parotid gland, and then divides into two main segments. Most of the fi bers serve the muscles of facial ex pression: the orbicularis oculi closes the eyelids, the nasal muscles dilate and contract, and the orbicularis oris and associated muscles retract, con tract, and protract the lips.1 The ab sence of the motor function of these
muscles is the primary focus of this report.
A 1 7-year-old white girl (Fig 1) was exam ined in early 1979. A medical history dis closed palsy of the right and left facial nerves that had been diagnosed at birth. When the patient was 7 years old, she was reexamined at the University of Nebraska and the diagnosis was confirmed. The prognosis was poor; little or nothing could be done. Except for the paralysis, the patient had a noncontributory medical history. Taste sensations were active, as were salivary and lacrimal flow. Excellent occlusion, m ucosa, gingiva, and dental hygiene were evident. No family members had had this rare condition. Approximately 125 cases are
known to have existed.2 The letters B and P were the most dif ficult to pronounce, because it was neces sary for the patient to compress the lower lip against the maxillary incisors to pro nounce these consonants (Fig 2, 3). The third cran ial nerve serves the strong superior levator muscle that is responsi ble for opening the upper eyelids. How ever, dysfunction of the facial nerve does not permit total contraction of the orbic ularis oculi muscle; maximum closure of the patient’s eyelids was approximately 80% (Fig 4). Through contraction of the buccinator muscle, the lips could move in a lateral direction (Fig 5). The long buccal branch of the fifth nerve ordinarily inter laces with the facial nerve that serves the buccinator m uscle.1 In Gray's Anatomy, a footnote states, “There seems to be no rea son to doubt that the branch supplying the buccinator muscle is entirely a nerve
Fig 1 ■ Patient w ith Mobius syndrome. Notice
Fig 2 ■ Patient m aking a B sound— notice po
Fig 3 ■ Patient m aking a P sound.
lack o f expression.
sition of teeth and lower lip.
Report of case
JADA, Vol. 100, January 1980 ■ 71
B R IE F
REPO RTS
Fig 4 ■ M axim um closure of eyes.
Fig 5 ■ Only facial expression w as movement
Fig 6 ■ Patient w histling.
o f lips in lateral direction.
of ordinary sensation, and that the true motor supply of this m uscle is from the facial.”3 In this case, it appears that the fifth cranial nerve serves the buccinator motor. W histling was done with tongue and teeth (Fig 6); sucking on a straw was ac complished by using the corner o f the mouth (Fig 7); and removal of soft food, such as pudding, from a spoon was ac complished by using the tongue and teeth (Fig 8). The patient compensated for the loss of lip function with facile motion.
Discussion This condition is known as the Mobius syndrome. Other characteris tics, in addition to bilateral facial paralysis, include anomalies, such as upper facial paralysis only; club foot; deformed hands, ears, or both; and aplasia or hypoplasia of pectoral or brachial musculature. Paralysis of the lateral rectus muscle (crossed eyes) is the most common accom panying condition. However, in a re view of 61 cases, Henderson4 noticed that not all cases were associated with abducens palsy. Neuropathologists have not agreed whether the cause of the syndrome is a hypoplasia of the central nervous system (involving the third, fifth, sixth, seventh, ninth, tenth, eleventh, or twelfth cranial nerves, or all of these) or an absence of primary pe ripheral muscle, or a defect that causes d egen eration of nerves through atrophy,5 To correct the lack of expression characterized by this syndrome, transposition of the temporalis mus cle has been done.5 In this procedure, 72 ■ JADA, Vol. 100, January 1980
Fig 7 ■ N otice position o f straw in sucking.
Fig 8 ■ Patient removing pudding with teeth and tongue.
a broad band of the temporal muscle is exposed above the hairline. Inci sions are then made at the outer canthi of the mouth and eye. Small strands of the muscle are separated and inserted into the upper and lower eyelids, and larger groups of muscle fibers are attached to the outer canthi of the lips. When teeth are clenched, with training, the tem poral muscle contracts; the eyelids can then be closed and a smile can be achieved. Specialists will decide whether this type of surgery is indi cated for the patient in this study.
Summary A 17-year-old girl who has the Mobius syndrome showed compen satory motions in sucking, speech, and eating. Movements of facial ex-
pression were absent (except for con traction of the buccinator muscle and some closure of the eyelids). The pa tient has been referred to a specialist for restoration of additional facial expression. Dr. Fogg is in private practice, 2226 G ulf Gate Dr, Sarasota, Fla 33581. Address requests for reprints to Dr. Fogg. 1. Schaeffer, J.P. M orris’ hum an anatomy. Philadelphia, Blakiston Co., 1946. 2. Gorlin, R .F., and Pindborg, J.J. Syndromes of the head and neck. New York, McGraw-Hill, 1964. 3. Gray, H. Anatomy of the hum an body. Philadelphia, Lea & Febiger, 1973. 4. Henderson, J.L. T he congenital diplegia syndrom e: c lin ic a l features, pathology and aetiology; a review of sixty-one cases. Brain 62:381 -4 0 3 ,1 9 3 9 . 5. Converse, J.M . R eco n stru ctiv e p lastic surgery. Philadelphia, W. B. Saund ers Co., 1977.