University of the Philippines Manila

College of Medicine-Philippine General Hospital

Department of Otorhinolaryngology

LOSING FACE

A SELF-INSTRUCTIONAL MANUAL ON DISORDERS OF

FACIAL EXPRESSION: DIAGNOSIS, PATHOPHYSIOLOGY
AND PRINCIPLES OF THERAPY
FOR YEAR LEVEL IV MEDICAL STUDENTS

SYNOPSIS
This module will enable the year level IV student to adopt a basic approach to the
diagnosis and principles of management of disorders of facial expression by
correlating signs, symptoms and pathyphysiology.

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 INTRODUCTION

Dear Medical Student,

We are easily concerned about “losing face” in social situations. Have you
ever considered what it must be like to actually “lose face” in terms of form and
/or function? This module will equip you to explore the various ways facial
expression can be altered by such disorders. We begin by reviewing skills in
obtaining a pertinent history and in performing an adequate physical
examination. We then learn to correlate signs and symptoms with
pathophysiology in order to guide us in arriving at the most likely diagnosis from
among several differentials. We then become acquainted with ancillary
diagnostic examinations that may be requested to support our presumptive
diagnosis, as well as with principles of management for the most common
disorders affecting facial expression. We hope you enjoy the activities and
exercises in this module as we enjoyed designing them.

TABLE OF CONTENTS

TITLE PAGE E-1

INTRODUCTION AND TABLE OF CONTENTS E-2
ALGORITHM E-3
Lesson 1: Mechanical Disorders of Facial Expression E-5
Congenital E-5
Infectious/Inflammatory E-9
Traumatic/Toxic E- 23
Neoplastic E- 29
Degenerative/Systemic E- 31
Lesson 2: Neural/Electrical Disorders of Facial Expression E- 32
Congenital/Developmental E-32
Inflammatory/Infectious E- 33
Traumatic/Toxic E-38
Neoplastic E-39

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 Facial Movement
Disorder
ALGORITHM

History and
P.E.

Mechanical Neural/ Electrical

Congenital/ Infectious/ Traumatic/ Neoplastic Degenerative

Developmental Inflammatory Toxic

Cleft Lip and Hansen’s Fractures Facial Scleroderma

Palate Disease Hemangiomas

Craniofacial Neurofibromatosis
Clefts

Facies Basal and

Squamous Cell
Cancer

Other
disfiguring
cutaneous
lesions

Congenital/ Infectious/ Traumatic/ Neoplastic Degenerative

Developmental Inflammatory Toxic

Mobius Bell’s palsy Otitis Media Parotid Tumor

Syndrome

Herpes Zoster Iatrogenic Temporal

Bone
Carcinoma

Trigeminal Facial Nerve

E-3 Neuralgia Schwannoma
 LESSON ONE
Mechanical Disorders of Facial Expression

OBJECTIVES: At the end of this lesson, the student should be able to:

1. obtain a systematic and comprehensive clinical history and perform an

adequate physical examination on a patient with a mechanical disorder
of facial expression

2. discuss the pathophysiology, signs and symptoms of common

mechanical disorders of facial expression

3. list the most likely differential diagnosis and arrive at a single most
likely (parsimonious) cause of disease

4. describe ancillary diagnostic procedures and discuss the principles of

therapy for common mechanical disorders of facial expression

A. Congenital/Developmental

1. Cleft Lip and Palate

Oral-facial clefts are birth defects in which the tissues of the mouth or lip
don't form properly during fetal development. In the United States, clefts occur
in 1 in 700 to 1,000 births, making it the fourth most common major birth
defect. Clefts occur more often in children of Asian, Latino, or Native
American descent (Perrotin et al., 2001). According to the Philippine Birth
Defects Registry Project (1999-2001), it is the 3rd most common birth defect
in our country occurring in 5.6:10000 live births.

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To facilitate your understanding of this session, let us define some terms:

1. Malformation

A morphologic defect of an organ, part of an organ, or a larger area of the

body resulting from an intrinsically abnormal developmental process. Origin:
L. Malus = evil, formation = a forming. Ex. Polydactyly, cleft lip, renal
agenesis. Malformations initiated earlier during organogenesis are more
comlex. The later the defect is initiated, the simpler the malformation.

2. Deformation

An abnormal form or position of a part of the body caused by nondisruptive

mechanical forces. Ex. Clubfoot, congenital hip dislocation, congenital
postural scoliosis. Deformations arise most frequently in late fetal life from
lack of fetal movement and from intrauterine molding by mechanical forces.

3. Disruption

A morphologic defect of an organ, part of an organ, or a larger region of the

body resulting from a breakdown of, or interference with, an originally normal
developmental process. Ex. Amniotic band amputation, asymmetric
encephaloceles, and bizarre facial clefts
(Cohen, 2000)

Embryology

In facial morphogenesis, neural crest cells migrate into the facial region,
where they form the skeletal and connective tissue and all dental tissues except
the enamel. Vascular endothelium and muscle are of mesodermal origin (Cohen,
2000).

The upper lip is derived from medial nasal and maxillary processes.
Failure of merging between the medial nasal and maxillary processes at the fifth
week of embryonic development, on one or both sides, results in clefting. Cleft lip
usually occurs at the junction between the central and lateral parts of the upper
lip on either side. The cleft may affect only the upper lip, or it may extend more
deeply into the maxilla and the primary palate. (Cleft of the primary palate
includes cleft lip and alveolus.) If the fusion of palatal shelves is impaired also,
the cleft lip is accompanied by cleft palate.

Cleft palate is a partial or total lack of fusion of palatal shelves. It can

occur in a number of ways:

• Defective growth of palatal shelves

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 • Failure of the shelves to attain a horizontal position
• Lack of contact between shelves
• Rupture after fusion of shelves

The secondary palate develops from the right and left palatal processes.
Fusion of palatal shelves begins at the 8th week of the fetal period and continues
usually until the 12th week. One hypothesis is that a threshold exists beyond
which delayed movement of palatal shelves does not allow closure to take place,
and this results in a cleft palate.

Activity 1:
Arrange the following illustrations chronologically and match with their
corresponding age of gestation (in weeks) and embryological event/s.

1 2

3 4

A) 7th wk AOG - Tongue moves downward, shelves rotate upward toward

each other and fuse in midline. Fusing of palatal shelves continues
backward and includes the soft palate and uvula.
B) 5th wk AOG - Palatal shelves fuse, medial nasal and palatal shelf of the
lateral maxillary process directed downward at first. There is free
communication between nasal and oral cavity.

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 C) 6th wk AOG - Formation of one fused nasal process and two maxillary
processes: upper lip, upper alveolus, lower alveolus, & primary palate.
D) 4th wk AOG - Maxillary swellings enlarge through proliferation of
ectomesenchyme of 1st pharyngeal arch. Start of palatogenesis.

Cleft lip can be easily diagnosed by performing ultrasound in the second

trimester of pregnancy when the position of the fetal face is located correctly.
Usually, it is not possible to diagnose a cleft palate by an ultrasound; however,
an experienced physician or technician may catch an atypical movement of the
fetal tongue in a lateral view. In the case of a large cleft palate, the tongue moves
up into an open space (cleft) in the roof of the oral cavity. Recently, 3D imaging
has been introduced to prenatal ultrasound diagnostics of cleft anomalies and
seems to be very promising for recognizing a cleft palate in a fetus.

Did you know ???

In a randomized, controlled, double-blind, multicenter trial sponsored by the
British Medical Research Council (MRC) showed a 72% decrease in the
recurrence of neural tube defects when women ingested 4 mg/d of folic acid prior
to conception and during 12 weeks thereafter (MRC Vitamin Study Research
Group, 1991; Wald, 1993). However, it was for cleft lip and palate (CL/P)
anomalies that the first attempts were made to use prophylactic multivitamin
therapy, including folic acid, to prevent recurrences in humans (Douglas, 1958;
Conway, 1958; Peer, 1958). In a large population-based case control study of
fetuses and live-born infants during 1987-1989 in California, Shaw (1995)
reported that periconceptional use of multivitamins, which usually contain 0.4 mg
or more of folic acid, reduced the occurrence of CL/P by approximately 27-50%.

Having a baby with cleft lip and palate can be a devastating experience for
parents. Hence our approach should be sensitive to their feelings as we obtain
the clinical history and perform a physical examination. It should be showed that
our questions are not meant to “fault-find” as the cleft is most probably not due to
any fault or wrong doing on the mother’s or father’s part.

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 Sample questionnaire for patients with cleft lip and palate deformity:

Name: Age/Sex:
Address: Birthday:
Source of Information:

History Taking:

1. Any family member with congenital maxillofacial defect?

a. Specify family member –
b. Specify congenital anomaly –

2. During mother’s pregnancy, any history of…

a. Drug intake? What trimester? Specify.
b. Maternal illness? What trimester? Specify disease entity &/or
signs and symptoms.
c. Smoking/drinking alcoholic beverages?
d. Multivitamin intake?
e. Trauma?
f. Exposure to x-rays/radioactivity?

Physical examination:
1. General inspection of face
2. Otoscopy/Pneumatic otoscopy - look for any sign of effusion, dullness
and retraction/bulging of tympanic membrane
3. Inspect nasal cavity using otoscope (if pedia), nasal speculum (if adult)
4. Inspect oral cavity – classify cleft lip and palate deformity according to
the Thallwitz classification (*see below); note dentition

Thallwitz Classification:
Divide key areas of the face (lip,
alveolus, hard & soft palate) into thirds.
Affectation of 1/3 of the lip would classify
it as L1. If 2/3 of the lip is affected, it
would be classified as L2, and so on.
The whole classification is writted as
L_A_H_S_H_A_L_ . Note that laterality
is not indicated. This is because the first
L AH is for the right side of the patient
and the HAL is for the left side of the
patient. In order for you to remember
this, just think of it as if you were
examining the patient right in front of
you.

“Computergestützte Dokumentation von Patienten mit Lippen-Kiefer-Gaumensplaten”; Aus der Klinik fü Mund-,
Kiefer- und Gesichtschirurgie/Plastische, Operationen des Universitätsklinkums Charitė; May 1997

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Activity 2:
The following are patients that we have encountered in our institution. Classify
the following pictures according to the Thallwitz Classification.

A A

B C

ANSWERS:
Source: PGH Dept of ORL

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How do you feed a CLAP infant??

The more worrisome matter in the care of an infant who has cleft lip and
palate is his feeding. Below are some of the tips that you can give to a
mother/parent in the care and feeding of a cleft lip and palate child:

™ The mother should apply pressure to the areola with her fingers to help
the engorged nipple protrude. She should hold the infant in a semi-upright,
straddle, or football position. She should support the breast by holding it
between her thumb and middle finger, making sure that the infant's lower
lip is turned out and the tongue is under the nipple.

™ In a case of an isolated CL, the infant typically does not experience

feeding problems beyond learning how to "latch on" to the nipple at the
beginning of the feeding. Infants with CP must squeeze the milk out of the
nipple by compressing the nipple between the tongue and whatever
portion of the palate that remains.

™ For infants with bilateral CLP, breastfeeding is not possible. The mother
can use a breast pump, then, she can feed the baby with a bottle.

™ When bottle feeding, take note of the following:

o A soft nipple is generally better than a hard nipple (some can be
softened by boiling).
o Use a crosscut nipple to prevent choking. Any nipple can be
crosscut manually using a single-edged razor blade. The crosscut
is on the tongue side.
o The bottle should be squeezed and released, not continually
squeezed.
o The nipple is angled to a side of the mouth, away from the cleft.

™ More upright or seated positions prevent the milk from leaking to the nose
and causing the infant to choke.

™ Advise the mother to stop feeding and allow the infant to cough or sneeze
for a few seconds when nasal regurgitation occurs. A palatal obturator
may be used and is encouraged to be used in cases of palatal clefts not
only to prevent regurgitation and to support high-flow feeding.

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 The following is the algorithm followed in our institution in the management of cleft lip and
palate patients. Note that continuum is not necessarily followed but rather depends upon age of
initial consult.

0-6 months
A. Initial evaluation/documentation
B. Fabrication of intra-oral appliance with monthly
modification – feeding instruction
C. Otologic Evaluation/Examination
D. Vocal play and initial parent counseling on speech
E. General Pediatric evaluation and immunizations

6-12 months
A. CLAP surgery – single stage modified Millard with alarplasty and two flap
palatoplasty with optional alveolar bone grafting, with possible VT tube insertion
B. Start of intensive speech therapy after single stage CLAP repair
C. Continued general pediatric follow-up and nutrition evaluation
D. Dental evaluation and care during primary tooth eruption

1-5 years old

A. Secondary problem assessment and correction
a. Correction of velopharyngeal insufficiency (pharyngoplasty)
b. Minor lip revision
c. Closure of fistula
B. Psychiatric Evaluation re: Body Image Issues
a. General Pediatric follow-ups
b. Continued Speech therapy (articulation and phonetic classes; consonant
pronunciation

8-10 years old

A. Alveolar bone grafting
B. Continue orthodontic follow-up
C. Speech therapy – higher level and language and speech

16-18 years old

A. Orthognathic Surgery

19 years old

A. Aesthetic Surgery
Clinical Practice Guidelines,
PGH Dept of ORL 2003

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Activity 3:
Enumerated below are some of the sequences and syndromes associated with
cleft lip and palate. Fill in the missing letters to complete their names.

1. M_bi_s Sequence – rare congenital disorder characterized

by unilateral or bilateral peripheral abducens and facial nerve
palsies. It is often accompanied by other cranial nerve
involvement, limb malformations and hypoplasia of the
pectoralis major (Poland anomaly). This sequence may also be
accompanied by craniofacial and orofacial deformities such as
microglossia, microstomia, cleft lip and palate, bifid uvula,
mandibular micrognathia, and hypodontia. Mental retardation
and autism have occasionally been reported.

Child with eyes closed.

eMedicine: Congenital
Facial Paralysis

2. _ti_k_er syndrome – a multisystem disorder

characterized by progressive arthro-ophthalmopathy. This
syndrome originally described by Stickler et al. consists of
myopia, cataracts, retinal detachment, cleft palate, flattening
of the midface and spondyloepiphyseal dysplasia. This
syndrome is caused by a collagen defect with most of the
tissues affected becoming rich in collagen type 2.
www.tracheostomy.com/
trachkids/kids10.htm

3. _i_rr_-R_b_n Sequence - characterized by a triad of

mandibular micro-retrognathia, glossoptosis, cleft of the
secondary palate and upper airway obstruction. 80% of cases
are associated with a syndrome, commonly Stickler syndrome.

www.mkg-berlin.de/.../
pierre_robin_gross.jpg

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 5. _ct_odactyly-e__odermal D_s_lasia Clefting –
a rare, complex autosomal dominant syndrome
with variable expression commonly manifested by
ectodermal dysplasia (eg. Anomalous hair, teeth,
sweat glands, nails and nasolacrimal ducts),
ectrodactyly (split hand/foot or “lobster claw”
deformity), cleft lip with or without cleft palate and
urogenital anomalies.

6. _ro-F_c_al-Di_i_al Syndrome – a group of disorders

with several heterogenous manifestations including lobulated
cleft tongue, multiple oral frenulae, cleft palate, polydactyly,
brachydactyly, syndactyly and other less common
anomalies.

Did you know???

A syndrome is a condition that is recognized by multiple factors that are present
and related to the same genetic condition. It is a group of anomalies which
contain multiple malformations and/or sequences.

A sequence is a pattern of morphology (or problems) that are seen to have

come from a single factor. It occurs when a single developmental defect results
in a chain of secondary defects, which may, in turn, lead to tertiary defects. The
result is a variably expressed group of defects, all of which can be traced back to
the original event.

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2. Craniofacial Clefts

Craniofacial clefts are major clefts affecting the face, the cranium, or both. They
occur without any pattern of heredity. They are much rarer than simple cleft
lip/palate, although their true incidence is difficult to calculate since the milder
forms are likely to go unrecognized.

The severity of craniofacial clefts varies considerably, ranging from a barely

perceptible notch on the lip or on the nose or a scar-like structure on the cheek,
to a dramatic separation of all layers of facial structures. They may appear with
numerous syndromes and are classified on the Tessier severity scale of 1-14
(see below).

What is a Tessier cleft?

Clefts may involve the mouth, cheeks, eyes, ears and forehead and may
continue into the hairline. These craniofacial clefts are often referred to as
Tessier clefts. They are numbered from 0-14 to indicate the location and extent
of the cleft using the mouth, nose and eye sockets as landmarks, with the midline
designated 0. These more extensive conditions may also be described
anatomically, such as “oro-occular cleft” and “fronto-nasal dysplasia.”

Who is Tessier?
Dr. Paul Tessier (pronounced Tess ee ay) published his definitive text on
craniofacial clefts in 1976. His work brought descriptive order to complex facial
clefts: a way “to identify the consistent, anatomic pathways of soft tissue and
skeletal clefts.” (David, 1989) Dr. Tessier also demonstrated that the area
around the eyes could be safely reconstructed, using an intracranial approach.
Previously, the orbits were thought to be too delicate to withstand surgery.

Synopsis of Dr. Tessier’s ideas:

• A cleft is diagnosed when there is any interruption of either soft tissue

(hair line, eyebrows, eyelids, nostrils, lips or ears) or skeleton. Clefts
occur in well-defined places and along definite axes. They may be
evaluated by their relationship to two main functional systems: the mouth
and the eyes.

• A soft tissue cleft corresponds with a skeletal cleft and conversely.

(Later writers indicate that this is not always true).

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• Bone and soft tissue are rarely involved to the same extent. From the
midline to the infra orbital foramen, soft tissues defects are more frequent
(or more destructive) than those of the skeleton. From the infra orbital
foramen to the temporal bone, skeletal defects are more severe than soft
tissue clefts, except for ear deformities.

• Clefts occur in well-defined places. Description of clefts based on

skeleton (rather than soft tissue) is easier because of the constancy of
most skeletal points.

• Clefts are not seen along the course of a main vessel. Clefts in the
skeleton do not imply absence of principal vessels or nerves in the area.

• Clefts are situated along definite axes. Clefts of the upper lip, eyebrow,
and frontal bone are often along the same axis as that of clefts of the
cheek and lip.

• The cleft lip condition is encountered in clefts number 1, 2 and 3**

**Tessier clefts of the lip are, in fact, anatomically distinct from classical
cleft lip.

• Clefts may be evaluated by their relationship with two main functional

systems: either the lip and upper jaw, or the eyelids and orbital cavity.
The orbit is described as two hemispheres: clefts running north through
the upper lid are cranial; clefts running south through the lower lid are
facial.

Figure showing the direction of facial clefts as designed by Dr. Paul Tessier

http://www.cleftline.org/som/0301/Tessier%20Info.pdf

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The areas of the face affected depend on the particular cleft. For example cleft
#0 causes a widening of the middle of the nose, whereas clefts #1 and 2 affect
the side of the nose. Cleft #3 separates the side of the nose from the cheek and
may be associated with absence of the eye on that side. Cleft #4 may also be
associated with absence of the eye is located a little more to the side. Cleft #5 is
rare and affects the upper jaw and lip. Clefts #6,7 and 8 affect the area of the
zygomatic -cheek bone- and cause deformities of the side of the face and corner
of the mouth as well as of the lower eyelid. Clefts #11-14 affect the forehead and
may even cause bulging of the brain through a defect in the forehead.

Activity 4.
Identify the following Tessier cleft:

www.cleftline.org/som/0301/som0301Smith.htm

Complex surgical procedures along the guidelines of craniofacial surgery are

planned and carried out by a multitude of specialists. Treatment generally starts
in infancy and may extend into adolescence. Careful planning will reduce the
number of necessary operations and will assure the best possible results.

3.. Facial hemangioma

Hemangiomas are collections of dilated small blood vessels that occur either
superficially in the skin (capillary hemangioma); or, deeper in other organs of the
body (cavernous). Hemangiomas are benign endothelial cell neoplasms that are
commonly absent at birth but have rapid growth during infancy and with
spontaneous remission, later in life. Hemangiomas may be difficult to distinguish
from vascular malformations. Vascular malformations, such as lymphangiomas
and arteriovenous malformations, however are present immediately birth, grow
very slowly and persist throughout adult life (Robbins, et al., 1999).

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 Hemangiomas may be found anywhere on the body. However, they are most
disturbing to parents when they are found on the infant's face. The natural course
of the disease may be varied depending on the anatomic part involved. Orbital
hemangiomas for instance could present as gradual blurring of vision in a child
(De Angelis, et al., 2001). Someimes, the size and location of hemangiomas may
interfere with vital functions such as feeding, talking or breathing.

On physical examination, capillary hemangiomas are seen as bright red to

blue lesions that can be slightly elevated or can level with the surface of the skin
and usually have an intact epithelium. Cavernous hemangiomas are also red-
blue lesions that are distinguished by the formation of large, dilated vascular
channels. They are usually soft and spongy masses, 1-2 cm in diameter,
although giant forms may occur.

Diagnosis of hemangiomas could involve the use of CT scans, which reveal a

poorly circumscribed mass that has no bony erosion and that easily enhances
with intravenous contrast. Other imaging modalities such as MRI can also be
used to visualize the extent of the tumor. Earlier diagnostic practices included
the aspiration of non-clotting blood. This practice was based on the fact that large
soft-tissue hemangiomas trap platelets inside causing a consumptive
coagulopathy. These can manifest as petechiae, easy bruisability or overt
bleeding. This specific type of hemangioma is called the Kasabach-Meritt
syndrome. If a patient presents with history, physical examination findings, and
imaging study findings consistent with hemangioma, no laboratory studies are
necessary (http://www.emedicine.com/orthoped/topic499.htm).

Principles of treatment of hemangiomas begin with close observation, as

these lesions tend to involute. Corticosteroid therapy has also been use. The use
of interferon alpha 2a is currently one of the newer forms of treatment modality
for hemangioma patients.

These are some examples of facial hemangiomas occurring in children

http://www.craniofacial.net/before_after/tumor_before_after/Hemangioma.asp

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B) Infectious/Inflammatory

Leprosy/Hansen’s Disease

CAUSE
Mycobacterium leprae or leprosy bacili

MODE OF TRANSMISSION

Airborne: inhalation of droplet/spray from coughing and

sneezing of untreated leprosy patient
http://www.doh.gov.ph/advisory
SIGNS AND SYMPTOMS /leprosy.htm

• long standing skin lesions that do not disappear with ordinary treatment
• loss of feeling/numbness on the skin
• loss of sweating and hair growth over the skin lesions
• thickened and/or painful nerves in the neck, forearm, near elbow joint
and the back of knees

Types of leprosy (http://www.icm.tn.gov.in/leprosy6.htm)

Leprosy type Features
Tuberculoid • Can be either one large red patch with well-defined raised borders or a
large hypopigmented asymmetrical spot
• Lesions become dry and hairless
• Loss of sensation may occur at site of some lesions
• Tender, thickened nerves with subsequent loss of function are common
• Spontaneous resolution may occur in a few years or it may progress to
borderline or rarely lepromatous types
Borderline • Similar to tuberculoid type except that lesions are smaller and more
tuberculoid numerous
• Disease may stay in this stage or convert back to tuberculoid form, or
progress
Borderline • Numerous, red, irregularly shaped plaques
borderline
• Sensory loss is moderate
• Disease may stay in this stage, improve or worsen
Borderline • Numerous lesions of all kinds, plaques, macules, papules and nodules.
lepromatous Lesions looking like inverted saucers are common
• Hair growth and sensation are usually not impaired over the lesions
Lepromatous • Early nerve involvement may go unnoticed
• Numerous lesions of all kinds, plaques, macules, papules and nodules
• Early symptoms include nasal stuffiness, discharge and bleeding, and
swelling of the legs and ankles

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 • Left untreated, the following problems may occur:
• Skin thickens over forehead (leonine facies), eyebrows and
eyelashes are lost, nose becomes misshapen or collapses, ear
lobes thicken, upper incisor teeth fall out
• Eye involvement causing photophobia (light sensitivity),
glaucoma and blindness
• Skin on legs thickens and forms ulcers when nodules break
down
• Testicles shrivel causing sterility and enlarged breasts (males)
• Internal organ infection causing enlarged liver and lymph nodes
• Voice becomes hoarse due to involvement of the larynx
• Slow scarring of peripheral nerves resulting in nerve thickening
and sensory loss. Fingers and toes become deformed due to
painless repeated trauma.

HOW IS THE DIAGNOSIS MADE?

Leprosy has very characteristic clinical features but the diagnosis must be
confirmed because of the need for prolonged treatment with antibiotics. A skin
biopsy may show characteristic granulomas (mixed inflammatory cell infiltrate in
the deeper layers of the skin, the dermis) and involvement of the nerves. Special
staining of the tissue may show acid fast bacilli, the number visible depending on
the type of leprosy.

The bacteria (see figure below) may also be found in lepromatous leprosy on
smears taken from skin slits made in the ear lobes, but the smears will be
negative in the tuberculoid or borderline forms of the disease.

Ziehl-Neelsen stain for the smear preparation reveals acid-fast bacilli (arrows) in and
outside macrophages. (www.leprastichting.nl/ infolep/pagina.asp?pagk & www.fit-for-travel.de/.../
Krankheiten/lepra.htm )

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TREATMENT

Multi-Drug Therapy (MDT)

Management of leprosy is aimed at stopping infection and minimising potential

physical deformities. Antibiotics used first-line to eliminate organisms include
dapsone, rifampicin and clofazimine. Varying regimens are used depending on
the type of leprosy and the severity of infection. This may be a combination of
two or three antibiotics given over varying lengths of time (up to years). Other
antibiotics include minocycline, ofloxacin and clarithromycin.

Oral corticosteroids and thalidomide are helpful in preventing nerve damage by

reducing swelling. Long courses are necessary to decrease severity of
deformities and disabilities.

Surgery may sometimes be used to drain abscesses to restore nerve function,

reconstruct collapsed nose, or to improve function or appearance of affected
areas.

PREVENTION AND CONTROL

• treat all leprosy cases to prevent spread of infection

• young children should avoid direct contact with untreated patients
• practice personal hygiene
• maintain body resistance by healthful living
o good nutrition
o enough rest and exercises
o clean environment

ON-GOING LOCAL STUDIES

http://www.ritm.gov.ph/program/lepro.htm

1. Risk Factors in the Development of Leprosy among Household Contacts

in Cebu, in collaboration with the Cebu Provincial Health Office, funded by
the Culion Foundation, Inc.
2. Post Genome Research in the Laboratory Diagnosis of Leprosy in
collaboration with the Department of Microbiology, Yonsei University,
funded by RITM institutional funds
3. Chemoprophylaxis of Persistently ELISA Positive Household Contacts
based on the Risk Factor Study in Cebu in collaboration with the Cebu
Provincial Health Office, funded by the Culion Foundation, Inc.
4. Ofloxacin Containing-Combined Regimen in the Treatment of Multi-
bacillary Leprosy in collaboration with the Jose Reyes Memorial Medical
Center, funded by the UNDP/WORLD BANK/WHO Special Program for
Research and Training in Tropical Diseases (TDR).

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Did you know???

In 1873, and Dr. Armauer Hansen of Norway had

astounding news for the world: leprosy was caused by
a bacterium (Mycobacterium leprae). Until then, the
disease was thought to be from a curse or sinful ways.

clendening.kumc.edu/ dc/pc/hansen.jpg

C. Traumatic Facial Injuries

Traumatic facial injuries can cause facial movement disorders with injury to
the skin, underlying soft tissues and craniomaxillofacial bones. Immediately after
the injury, the process of healing immediately begins and the following are the
stages and processes of skin, soft tissue and bone repair.

1. Wound Healing, Bone Healing and Fractures

Wound Healing

The process by which tissue repair takes place is termed wound healing
and is comprised of a continuous sequence of inflammation and repair, in which
epithelial, endothelial, inflammatory cells, platelets and fibroblasts briefly come
together outside their normal domains, interact to restore a semblance of their
usual discipline and having done so resume their normal function.

The process of wound repair differs little from one kind of tissue to another
and is generally independent of the form of injury. Although the different
elements of the wound healing process occur in a continuous, integrated
manner, it is convenient to divide the overall process into three overlapping
phases and several natural components for descriptive purposes.

E-21
 www. Orthoteers.co.uk

Activity 5:
Arrange the following processes chronologically, A being the first step in wound
healing.

____ 1. fibroblasts make collagen to fill in the wound

_____2. constriction of blood vessels to control bleeding

_____3. new blood vessels form

_____4. white blood cells clean the wound of debris and bacteria

_____5. new collagen forms

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Inflammatory Stage

This stage occurs during the first few days. The wounded area attempts to
restore its normal state by constricting blood vessels to control bleeding.
Platelets and thromboplastin make a clot. Inflammation also occurs and is a
visible indicator of the immune response. White blood cells clean the wound of
debris and bacteria.

Proliferative Stage

After the inflammatory stage, the proliferative stage lasts about 3 weeks (or
longer, depending on the severity of the wound). Granulation occurs, which
means that special cells called fibroblasts make collagen to fill in the wound. New
blood vessels form. The wound gradually contracts and is covered by a layer of
skin.

Maturation and Remodeling Stage

This stage may last up to 2 years. New collagen forms, changing the
shape of the wound and increasing strength of tissue in the area. Scar tissue,
however, is only about 80% as strong as the original tissue. The body's ability to
heal during this stage is diminished in the elderly.

Diagram 2. Illustrates the phases of wound healing and the cells involved in each
phase. www.orthoteers.co.uk

E-23
WOUND CARE

There are four basic steps to follow in caring for any wound. Perhaps the most
important factor in wound healing is compliance: in other words, caring for the
wound consistently and correctly.

1. Debride and cleanse

Debridement means the removal of dead tissue. It can be accomplished in

an autolytic manner, the wound itself is encouraged to do this task by the use of
dressings or by use of biochemical enzymes by using wet-to-dry dressings (in
which a wet dressing is allowed to dry, trapping material in it, and is then
carefully removed); or mechanical implements such as scalpel or scissors to
remove dead tissue from more serious wounds.

Cleansing refers to the removal of any foreign debris from the wound and any
bacteria. It is usually accomplished by irrigating the wound with clean fluid.

Many previously accepted wound-cleansing solutions have been found to be

toxic to fibroblasts and lymphocytes. These solutions include povidone-iodine,
acetic acid, iodophor, hydrogen peroxide, and Dakin's solution (sodium
hypochlorite). Normal saline solution effectively removes contaminants and has
the same salt concentration as the fluid in cells, so it does not damage cells by
pulling water out of them. Normal saline is also inexpensive and readily available.

2. Maintain a moist environment

During wound healing, cells and fluid are slowly exuded. The exudate
provides an environment that stimulates healing because it contains white blood
cells, growth factors, and other special enzymes and hormones. A moist
environment preserves this exudate, speeding wound healing and promoting skin
growth. It also prevents dressings from adhering to the wound and damaging the
fragile tissue when the dressing is removed. A moist environment can easily be
maintained using gauze moistened with normal saline solution or with a Vaseline-
based ointment. Normal saline solution will support autolytic debridement, absorb
discharge, and trap bacteria.

3. Prevent further injury

In order to prevent further injury, the initial cause of the wound must be
determined and addressed and the area must be protected from additional
trauma.

E-24
4. Provide Materials for healing

Proteins, made up of amino acids, are necessary for all phases of wound
healing, including angiogenesis, fibroblast proliferation, collagen synthesis, and
scar remodeling. Proteins also support the immune system, helping to prevent
infection. Fats and carbohydrates are also needed to supply the extra energy
used in healing and to prevent proteins from being used for energy. Water is
necessary to replace losses through vomiting, bleeding, wound discharge, and
fever. Vitamins and minerals also play key roles in the healing process, as will be
discussed later.

BONE HEALING

Activity 6:
Fill in the box summarizing the stages and components involved in bone healing.
Choose from the choices below.

a. 2nd day- 2 weeks b. macrophages, PMNs, mast cells c. osteoclasts

d. removal of tissue debris e. time of injury-24-72hours f. formation of cartilage

g. nitric oxide h. repopulation of medullary canal i. middle of repair-7 years

Inflammatory Reparative Stage Remodelling

Stage Stage
Duration
Components/
Cells Involved
Events

E-25
Inflammatory phase

The inflammatory phase occurs at the time of injury up to 24-72 hours. The
injured tissues and platelets release vasoactive mediators, growth factors and
other cytokines. These cytokines influence cell migration, proliferation,
differentiation and matrix synthesis. During this phase the growth factors recruit
fibroblasts, mesenchymal cells & osteoprogenitor cells to the fracture site. The
macrophages, PMNs & mast cells arrive at the fracture site at the 48 hour to
begin the process of removing the tissue debris.

Important cytokines in bone healing:

BMP Osteoinductive, induces metaplasia of mesenchymal cells into

osteoblasts

Target cell for BMP is the undifferentiated perivascular

mesenchymal cell
TGF-β Induces mesenchymal cells to produce type II collagen and
proteoglycans Induces osteoblasts to produce collagen

PDGF Attracts inflammatory cells to the fracture site

FGF Stimulates fibroblast proliferation
IGF-II Stimulates type I collagen production, cartilage matrix synthesis
and cellular proliferation
IL-1 Attracts inflammatory cells to the fracture site
IL-6 Attracts inflammatory cells to the fracture site

Reparative phase

This phase begins on the second day to 2 weeks. The vasoactive substances
(Nitric Oxide & Endothelial Stimulating Angiogenesis Factor) cause
neovascularisation & local vasodilation. During this phase the undifferentiated
mesenchymal cells migrate to the fracture site and have the ability to form cells
which in turn form cartilage, bone or fibrous tissue. The fracture haematoma is
organised and fibroblasts and chondroblasts appear between the bone ends and
cartilage is formed (Type II collagen). Bear in mind that the amount of callus
formed is inversely proportional to the amount of immobilisation of the fracture. In
fractures that are fixed with rigid compression plates there can be primary bone
healing with little or no visible callus formation.

E-26
 Remodeling:

Remodeling, the last phase of bone healing starts at the middle of repair
phase up to 7 years. Fracture healing is complete when there is repopulation of
the medullary canal. For cortical bone, remodeling occurs by invasion of an
osteoclast which is then followed by osteoblasts which lay down new lamellar
bone (osteon). As for the cancellous bone, it occurs on the surface of the
trabeculae which causes the trabeculae to become thicker.

Phases of Bone
Healing

1. Inflammatory
2. Reparative
3. Remodeling
4. Healed bone

Did you know???

Wolff’s Law which states that form follows function is applied in remodeling of
bone. It is dependent on the mechanical forces applied to it.

E-27
Activity 7:
Write on the corresponding column the systemic or local factors affecting bone
healing.

a. Nerve function b. NSAIDS c. degree of bone loss d. vascular

injury e. age f. degree of local trauma g. hormones h.
nutrition i. type of bone fractured j. degree of immobilization k. infection

Systemic Local

Fractures

A break in a bone is called a fracture. There's more than one way to

break or fracture a bone. A break can be anything from a hairline fracture (a thin
break in the bone) to the bone being broken into two or more pieces.

The bones are tough but even tough stuff can break. Like a wooden
pencil, bones bend under some strain like too forceful bending and once the
pressure is too sudden or too much, they might snap. You can break a bone by
falling off a skateboard or if your sister's bike falls over on you in just the right
way.

E-28
 Activity 8:
Matching Type: Match the type of the fracture with the description of the
fracture.

A B

1. when bone has broken into two pieces A. open fracture

2. when bone cracks on one side only B. comminuted

3. when bone broken in one place C. greenstick

4. when bone broken into more than two pieces D. complete

5. when bone is sticking through skin E. single

Pertinent questions to ask in a patient with possible facial fractures

1. When was the date and time of injury?

2. Where was the injury sustained?
3. What was the mechanism of injury?
4. Was loss of consciousness?
5. Was there any functional loss or change (sight, hearing, smell, mouth
opening)
6. Was there a change in the bite or the way the teeth are occluded?

Physical Examination in a patient with possible facial fractures

The facial skeleton can be divided into thirds. The upper third begins at the
hairline and ends at the eyebrows. The middle third of the face extends from
below the supraorbital rims to the incisal edges of the upper teeth. This region
consists of the nasal structures, the cheekbones, the maxilla, and the upper teeth
and their supporting structures (ie, dentoalveolar processes). A circumferential
rim of bone composed of the frontal, zygomatic, and maxillary bones protects the
eyes. The lower third of the face consists of the mandible, the lower teeth, and
their supporting structures (dentoalveolar processes). We will use this division in
the examination of the patient to make it easier and more systematic.

E-29
 I. Inspection
A. Upper third
1. Is it symmetrical?
2. Are there any depressions, lacerations, swelling?
B. Middle third
1. Are there any depressions, lacerations, swelling, hematoma?
2. Nasal or ear discharge? Is it clear or bloody?
3. Is it symmetrical?
4. Diplopia? Ptosis? Hemorrhage?
5. Nasal obstruction?
C. Lower third
1. Is it symmetrical?
2. Swelling, lacerations, bleeding?

II. Palpation

Palpation of the facial skeleton aids in the detection of fractures. With

general pressure over the bony prominences of the skull and face, the examiner
attempts to elicit pain, crepitus, instability, or the presence of a step-off.

A. Maxilla
The maxilla is examined by placing the palm of one hand on the
patient's forehead with the other hand, grasping the anterior maxillary teeth
and firmly attempting to move the maxilla in all directions.
B. Mandible
The examiner places his or her thumbs intraorally along the lingual
aspect of the mandible and applies outward pressure in an effort to elicit
instability, pain, or both. If pain is elicited during this mandibular stress test,
then a fracture must be excluded. Check also intraorally for tooth mobility.
C. Ocular Trauma
If highly considering the possibility of a globe laceration or rupture
palpation is strictly contraindicated to prevent leakage of the vitreous
substance. The globe should be protected with an eye patch and referred
immediately.

Diagnostic Examinations in Facial Fractures

A basic facial series consists of three or four films: a Waters view, postero-
antero view with cephalad angulation, a Caldwell view , a lateral view, and
occasionally a submentovertex view. If a nasal fracture is suspected, then a soft
tissue lateral view of the nasal bone can be done. Of these views, the most
consistently helpful view in facial trauma is the Waters view. It tends to show all

E-30
of the major facial structures at least as well and often better than other
radiographic views of the face (Dolan, 1984). In these views you have to look for
soft tissue swelling, symmetry, sharpness which are important in diagnosing
fractures.

Treatment

Airway management, hemostasis, and systematic evaluation for associated

injuries, particularly of the head and neck, are the first issues to resolve when a
patient is injured. These issues take precedence over an examination to rule out
possible facial fractures.

The goals for management of all facial fractures are the following:

1. to make an accurate diagnosis

2. to obtain precise anatomic reduction of the fracture
3. to stabilize the fracture to achieve facial contour, symmetry, and
primary healing of the bones; and
4. to reestablish the pretraumatic dental occlusion.

Mandibular Fracture

To illustrate the approach to facial fractures let us consider the mandibular

fracture. The mandible is another commonly fractured bone in the head, and
most of these fractures are obvious on clinical exam. Clinical findings include
facial distortion, malocclusion of the teeth, or abnormal mobility of portions of the
mandible or teeth.

Activity 9:
Case 1: R.F. a 23 year old male fastfood delivery driver accidentally crashed into
a parked ambulance to avoid a pedestrian while swerving along Pedro Gil Street.
He hit his jaw on the pavement when he was thrown from the motorcycle,and
was rushed to the PGH-ER.

1. If you were the attending physician what would you do first?

a. ask the history of the patient

b. examine the patient for other injuries
c. secure/assess airway
d. reassure the patient

E-31
2. What in the history is/are pertinent to ask the patient?

a. was there a loss of consciousness?

b. was there any change in mouth opening?

c. when was the date and time of injury?

d. all of the above

3. What physical examination findings will point to a mandibular fracture?

a. swelling

b. inability to open mouth

c. tooth mobility

d. all of the above

4. Considering a mandibular fracture, which diagnostic examination/s would you

order?

a. Skull AP/L

b. Panoramic

c. Mandible APO

d. b and c only

e. all of the above

E-32
Activity 10:
The illustration demonstrates the frequency of fractures by anatomical area.
Label the different parts of the mandible to determine the area most and least
frequently fractured.

Illustration demonstrating FREQUENCY OF FRACTURES BY ANATOMICAL

AREA
1
2

4
5

7
6
8

When double fractures occur, they are usually on contralateral sides of the
symphysis. Common combinations include the angle plus the contralateral body
or condyle. Triple fractures occasionally occur, and the most common type is
fracture of both condyles plus the symphysis.

D) Neoplastic

There are several skin malignancies that can cause facial dysmorphisms.
These tumors are characteristically aggressive, invasive and most oftentimes,
with poor prognosis. In the Philippines, most of our poor countrymen do not have
a the privilege of regular medical check-ups. Most Filipinos afflicted with these
kinds of illness will, expectedly come to you towards the later stage of their
disease, sometimes to the point that they will literally have no faces to show you.

1. Squamous Cell Carcinoma

Squamous cell carcinoma is the most common tumor found on sun

exposed areas in older people such as the head and the neck (Robbins, et al.,
1999). Other people who are in risk of this invasive tumor are those that are

E-33
exposed to industrial carcinogens, arsenics, ionizing radiation, tobacco and betel
nut chewing (which is common in our provinces). These tumors, when they do
not invade through the basement membrane appear as sharply defined, red,
scaling plaques. Further discussion on squamous cell carcinoma will be
discussed in the larynx/voice self-instrucional learning module.

2. Basal Cell Carcinoma

Basal cell carcinoma are slow growing tumors that seldom metastasize.
Just like in squamous cell carcinoma, these tumors are very invasive and have
the propensity to occur in sun-exposed areas of the body such as the face,
specially in lightly pigmented people.

Clinically, these tumors present as pearly papules that contain prominent

subepidermal blood vessels called telangiectasias. This tumor of the skin
usually appears as a small, fleshy bump on the face, neck ears or scalp.
Occasionally, these tumors may appear on the torso, usually as flat, pink
patches.

Did You Know???

… that Basal Cell Carcinoma was commonly called “rodent ulcers”.
This is because advance lesions can ulcerate and extensive local invasion
of the bone and facial sinuses may occur after several years of neglect or
even in unusually aggressive tumors, just like the common household rat
who bores through and eat up almost anything in its way!

Basal cell carcinoma can be diagnosed by incision/section biopsy. On

histologic examination, the tumor cells resemble those in the normal basal cell
layer of the epidermis. They do not occur on mucosal surfaces but arise from the
epidermis or follicular epithelium. There are two patterns seen: 1) multifocal
growths which originate from the epidermis and extend over several square
centimeters or more of skin surface; 2) nodular lesions which grow downward
deeply into the epidermis as cords and islands of basophilic cells embedded in a
mucinous matrix and often surrounded by several lymphocytes as well as
fibroblasts.

E-34
 A PGH patient with basal cell carcinoma

Did You Know???

For several years, since the early part of the 20th century, the treatment of basal
cell CA was mainly the surgical removal of the cancer and surrounding tissue, which
most of the time produced disfiguring results. That was until the Moh’s micrographic
surgery (MMS) was developed.

MMS is a form of surgery for skin cancer developed by Frederick Moh in the
early 1940s. His technique included the removal of the part of the skin that involved
the cancer. The skin is removed layer by layer, with the surgeon examining each
layer intra-operatively as it is removed, to determine if the cancer is still evident. If
cancer cells exist in the sample of removed skin, another layer of skin is removed.

Since Moh’s micrographic surgery involved the evaluation of each layer of skin,
and removes each layer only when needed, it reduces the severity of disfigurement
when compared to the conventional surgical excision which removes all of the tumor
and its surrounding margins.

A recent study published (Smeets, et al., 2004), concluded that MMS may be
superior to conventional surgical excision for the treatment of basal cell carcinomas,
particularly larger cancers, as surgery can produce aesthetic defects. However, the
overall recurrence rates were almost identical between the two treatments, leaving no
conclusive benefits of one type of treatment over the other in terms of treatment
efficacy.

E-35
 Did You Know???
In diagnosisng certain malignancies/tumors, biopsies can be very vital diagnostic
tools.There are several types of biopsies, each one being appropriate in different circumstances.
Doctors may also vary on which biopsy method they prefer. Regardless of the method, biopsies
all involve taking a sample of tissue from the a mass and looking at it under a microscope to see
if it is cancer. Below are the most common types of biopsies.

1. Fine-needle Aspiration Biopsy (FNAB) - This biopsy can be performed with local
anesthetic or no anesthetic (a patient can always request anesthetic) and involves
inserting a small needle into the mass. It can be done in the doctor's office. This biopsy
serves two functions. If the mass is fluid-filled (cystic), performing this biopsy will
remove the fluid, making a clear diagnosis of a cyst and eliminating the need for
surgery. If the mass is solid, however, the needle will collect some of the cells from the
mass, which the pathologist can then look at to determine if the patient has cancer.

2. Core-cutting Needle Biopsy -This similar to FNAB, but instead of collecting cells as an
aspirate, a larger needle takes out a small piece of tissue, called a core. This biopsy can
also be done in the doctor's office.

3. Excisional Biopsy - Excisional Biopsies involve removing the whole mass. If the mass is
found to be benign (not cancerous), this will probably be all the treatment the patient will
need. If the mass is cancerous, but the biopsy removed sufficient non-cancerous tissue
around the mass, the biopsy serves as a lumpectomy. This biopsy is usually done in the
operating room as an outpatient surgical procedure with local anesthesia or local
anesthesia plus sedation.

4. Incisional Biopsy - This biopsy procedure involves removing part, but not all, of a mass.
Today, fine needle aspiration and core biopsy have eliminated most of the need for
incisional biopsy. This biopsy is also an outpatient surgical procedure.

5. Punch biopsy
A core of skin (dermis, epidermis, and fat) is removed with a special biopsy instrument.
The hole is typically closed with a suture and heals with little scarring. This type of
biopsy is typically used to sample skin rashes and small masses.

E) Degenerative/Systemic
1. Scleroderma
Scleroderma (also known as systemic sclerosis) is a rare, but progressive
disease that leads to hardening and stratching of the skin and connective tissues
due to excessive fibrosis throughout the body. Its course usually begins with a
few dry patches of skin on the hands or face that begin getting thicker and
harder.
The skin is most commonly affected although the kidneys, GI tract, muscles,
lung and heart are also frequently involved (Robbins, et al. 1999). A great
number of patients with scleroderma present with diffuse sclerotic atrophy of the
skin which initially begins in the fingers and distal regions of the upper extremities
and extends proximally to involve the upper arms, shoulders, neck and face.

E-36
 LESSON TWO
NEURAL/ELECTRICAL DISORDERS OF FACIAL EXPRESSION

Objectives: At the end of this lesson, the student should be able to:
1. obtain a systematic and comprehensive clinical history and perform
an adequate physical examination on a patient with a neural or
electrical disorder of facial expression
2. discuss the pathophysiology, signs and symptoms of common
neural or electrical disorders of facial expression
3. list the most likely differential diagnosis and arrive at a single most
likely (parsimonious) cause of disease
4. describe ancillary diagnostic procedures and discuss the principles
of therapy for common neural or electrical disorders of facial
expression

Activity 11:
Aside from congenital musculo-skeletal facial defects, another important factor
affecting facial expression is facial nerve status. The facial nerve is a mixed
nerve containing both sensory and motor components. It carries nerve impulses
to the muscles of the face, tear glands, salivary glands, and stapedius muscle. It
also transmits taste from the anterior 2/3 of the tongue. Below is a diagram of
the facial nerve. Fill in the blanks corresponding to the numbered segments of
the illustration below:

E-37
 1

http://www.neuro.wustl.edu/neuromuscular/pathol/diagrams/viianat.htm

Since the function of the facial nerve is so complex, many symptoms may
occur when the fibers of the facial nerve are disrupted. A disorder of the facial
nerve may result in twitching, weakness, in dryness of the eye or the mouth, or in
disturbance of taste. Below are some of the more common and apparent
manifestations of a facial nerve disorder:

E-38
Facial nerve paralysis: Signs

• Facial asymmetry
• Eyebrow droop
• Loss of forehead & nasolabial folds
• Drooping of corner of mouth
• Uncontrolled tearing
• Inability to close eye
www.emedicine.com -
• Lips cannot be held tightly together Dynamic Reanimation for
• Difficulty keeping food in mouth, drooling Facial Paralysis Article by
• Facial muscle atrophy (Late) Steven M Parnes, MD_files

The low power objective field view above shows a cross-section of a nerve bundle
showing the epineureum (not labeled) enveloping the nerve bundle and the perineureum
enveloping the nerve fascicles.
http://www.cytochemistry.net/microanatomy/nerve/peripheral_nerve_histology.htm

E-39
How is facial paralysis evaluated?
HISTORY TAKING

Evaluation begins with thorough history to help determine the cause. Below are
some of the details that should be asked regarding the patient’s facial paralysis:

1. Duration
2. Presence of the following right before, during or after onset of facial
paralysis:
a. Headache, vertigo/dizziness, change in sensorium, other
neurologic signs/symptoms
b. Recent head trauma
c. Ear discharge, tinnitus, ear fullness, hearing loss
d. Fever, non-specific signs/symptoms like colds & cough
e. Lumps or masses in post or pre-auricular area

PHYSICAL EXAMINATION:

A physical examination will help to determine in localizing facial nerve damage.

In general, a peripheral lesion of CN VII will affect the upper and lower face while
a central lesion will only affect the lower face.

Multiple classifications of facial nerve injury are found in the literature; the most
frequently used is the House-Brackmann scale, as shown below.

House-Brackman Facial Nerve Grading System

Reference: House JW, Brackman DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985:93,146-147.

Grade Definition

I Normal symmetrical function in all areas

II Slight weakness noticeable only on close inspection

Complete eye closure with minimal effort
Slight asymmetry of smile with maximal effort
Synkinesis barely noticeable, contracture, or spasm absent

E-40
 III Obvious weakness, but not disfiguring
May not be able to lift eyebrow
Complete eye closure and strong but asymmetrical mouth
movement with maximal effort
Obvious, but not disfiguring synkinesis, mass movement or
spasm

IV Obvious disfiguring weakness

Inability to lift brow
Incomplete eye closure and asymmetry of mouth with maximal
effort
Severe synkinesis, mass movement, spasm

V Motion barely perceptible

Incomplete eye closure, slight movement corner mouth
Synkinesis, contracture, and spasm usually absent

VI No movement, loss of tone, no synkinesis, contracture, or

spasm

Aside from observation of gross facial movement, the following should be

done:

1. Otoscopy/Pnuematic otoscopy
2. Oral cavity visualization and palpation
3. Palpation of peri-auricular area
4. Tuning fork tests/Pure tone audiometry & speech testing

LOCALIZING/PROVOCATIVE TESTS

A group of tests checking tear production (Schrimer test), saliva production

(Lashley cups, Schneyer’s device), taste sensation and stapedius muscle
movement (stapedius reflex) can help to determine if only a small branch of the
facial nerve is damaged. This is known as topographic localization.

E-41
ANCILLARY PROCEDURES

1. Computed tomography/Magnetic resonance imaging may be used to

image temporal bone fractures, tumors compressing the nerve, or frank
nerve disruption.
2. Electromyography techniques may be used to detect certain types of
neuromuscular disease.
3. Nerve excitability test (NET) uses electrical impulses to compare the
normal facial nerve on one side of the face with the abnormal one on the
other.
4. Electroneurobility testing (ENoG) goes further than NET, by giving
actual numbers to help with the comparison.
5. Pure tone audiometry and speech testing may be used to detect facial
nerve injury to the middle ear; hyperacusis develops on the affected side
in the absence of facial nerve input to the stapedius muscle.
6. A new method of measuring facial motion, automated face analysis
(AFA), originally developed for evaluating emotion through facial
expressions, uses "computer vision" approaches to quantify facial motion.

Disorders of the facial nerve, including paralysis, develop from a variety of

causes. Most are idiopathic, which means that the cause remains unknown.
Abnormal movement or paralysis of the face can result from infection, injury,
tumors, or it may even be iatrogenic. Read the following cases presented below
and see if you can arrive at a correct diagnosis based on your knowledge of the
anatomy and physiology of the facial nerve.

CASE 1:

A 20-year old male comes to the out-patient department with a chief complaint of
progressive facial asymmetry of 5 months duration. Upon further questioning, he
denies history of any head injury or any other co-morbid illness except for 9 yrs
duration of on & off occasionally foul-smelling ear discharge on the left. Physical
examination is essentially normal except for a perforated tympanic membrane on
the left ear with minimal mucopurulent discharge. Neurologic exam revealed the
following: shallow nasolabial fold on the left, inability to close left eye on
maximum exertion, and decreased hearing on the left. What is your initial
impression? House-Brackmann score?

Answer: Acute or chronic middle ear infections occasionally cause a

weakness of the face due to swelling or direct pressure on the nerve. In acute
infections, the weakness usually subsides as the infection is controlled and the
swelling around the nerve subsides. Facial nerve weakness occurring in
chronically infected ears is usually due to pressure from a cholesteatoma (skin-
lined cyst). Mastoid surgery is performed to eradicate the infection and relieve

E-42
nerve pressure. In some cases of huge cholesteatoma formation, the facial nerve
may be extensively damaged so as to cause permanent and complete facial
nerve paralysis. This patient presents with facial nerve paralysis grade VI.

CASE 2:

A 43-year old female came to the clinic due to a right infra-auricular mass.
Physical examination showed the mass to be 4x4cm in size, firm, fixed, non-
tender. Coincidentally, you also noted shallow nasolabial fold on the ipsilateral
side. Patient however can fully close his eyes with minimum effort and is able to
raise both eyebrows. What is your diagnosis? What segment of the facial nerve
do you think is affected? House-Brackmann score?

Answer:

Parotid masses occur most commonly in the lower pole,

or tail, and in the superficial lobe of the gland. The most
common presentation is that of an asymptomatic pre- or
infra-auricular mass. Pain or facial nerve paralysis is
less frequent and more likely is due to malignancy.
The buccal branch innervates the levator labii superioris
alaeque nasi responsible for elevating the medial
nasolabial fold and nasal ala. This patient presents with
Grade II facial nerve paralysis.

Now that we have learned some basic knowledge regarding the facial nerve,
we can now further classify nueral/electrical disorders of facial expression into
the following:

A) Congenital/Developmental

1. Mobius Sequence

Mobius sequence is a rare birth defect caused by the absence or

underdevelopment of the 6th and 7th cranial nerves, which control eye
movements and facial expression. The first symptom, present at birth, is an
inability to suck. Other symptoms can include: feeding, swallowing, and choking
problems; excessive drooling; crossed eyes; lack of facial expression; inability to
smile; eye sensitivity; motor delays; high or cleft palate; hearing problems; and
speech difficulties. Small or absent brain stem nuclei that control the cranial
nerves, as well as decreased numbers of muscle fibers, have been reported.
Deformities of the tongue, jaw, and limbs, such as clubfoot and missing or

E-43
webbed fingers, may also occur. As children get older, lack of facial expression
and inability to smile become the dominant visible symptoms. The prognosis for
otherwise normal development is excellent in most cases.

B) Inflammatory/Infectious

1. Bell’s palsy

Bell’s palsy is one of the most common neurologic disorders affecting the
cranial nerves. It is an abrupt, isolated, unilateral, peripheral facial paralysis
without detectable causes. This syndrome of idiopathic facial paralysis was first
described by Sir Charles Bell, yet much controversy still surrounds its etiology
and management. Bell’s palsy is certainly the most common cause of facial
paralysis worldwide.

However, not all facial nerve palsies can be diagnosed as Bell’s palsy. Sir
Terence Cawthorne’s (1969) statement that “all that palsies is not Bell’s” can
indeed hold much ground as several studies have shown. Lapena and Chiong
(1997) reviewed 5 cases of facial nerve neuromas that also present similarly with
Bell’s palsy. They observed that “Most patients with facial nerve neuromas are
treated (medically managed) for months or years until properly diagnosed, with
few surgeons acquiring significant experience in managing these lesions.
Therefore, a good clinician should rule out other causes of facial asymmetry and
not use Bell’s palsy as a wastebasket diagnosis.

Have you seen a patient with Bell’s palsy? These patients would most
commonly be seen initially in the emergency room. The cosmetic appearance of
a distorted face and the abrupt functional impairment are the driving forces that
prompt emergency evaluation. Patients often fear they have had a stroke and
that their distorted facial appearance is permanent.

Pathophysiology

The actual pathophysiology is unknown;. One of the more common and

popular theories points to the inflammation of the facial nerve. During this
process, the nerve increases in diameter and becomes compressed as it courses
through the temporal bone.

Clinical History

Patients with Bell’s Palsy would begin their history with the following
symptoms: pain behind the ears, numbness in the affected side of the face,
recent upper respiratory infection (URI) and/or viral syndrome, drooling and
sometimes alltered taste, altered hearing, and tearing from eyes.

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Physical Examination Findings

The classic definition of Bell palsy describes mononeuric involvement of the

facial nerve. This means that on physical examination you can note of the
shallow nasolabial fold on one side of the face and sometimes , the inability to
close the ipsilateral eyelids. Remember that weakness and/or paralysis from
involvement of the facial nerve manifests as weakness of the entire face (upper
and lower) on the affected side. Focus attention on the voluntary movement of
the upper part of the face on the affected side.

This is an example of a patient with Bell’s Palsy

The approach to management of Bell’s palsy can vary from one doctor to the
other. To most physicians, it is initiated by administering steroids which could
help lessen the inflammation, if indeed it is the main cause of the paralysis. Anti-
virals are also employed if there is a strong indication of a viral etiology of the
palsy.

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 Charles Bell was a pioneer in
the study of the human
nervous system. He
discovered that nerves are
actually bundles of fibers,
each with its own origin. He
showed that some nerve
fibers have sensory functions
and others have motor
functions. Bell's palsy, a
paralysis or weakness of
muscles controlled by the
facial nerve, is named after
him. The illustration on the
title page of this module is
one of his illustrations of the
facial nerve anatomy.

2. Herpes Zoster

What Herpes Zoster Oticus?

Herpes zoster oticus is a viral infection of the inner, middle, and external
ear. HZ oticus manifests as severe ear pain and associated cutaneous vesicular
eruption, usually of the external canal and pinna.The infection is called Ramsay
Hunt syndrome when associated with facial nerve paralysis.Herpes zoster
oticus is caused by the reactivation of latent Varicella zoster virus, which has
remained dormant within sensory ganglia (commonly the geniculate ganglion) of
the facial nerve.

A latent viral infection is an infection of a virus that has remained dormant and
inactive in a certain anatomical part of the body. In Herpes Zoster Oticus, the
varicella zoster virus , or the virus that commonly is known to cause
“chickenpox” during childhood becomes dormant and after several years may
become the latent viral infection that will again reactivate to cause the
disease.

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 Typically, patients present with severe otalgia. Complaints include the
following:

- Painful, burning blisters in and around the ear, on the face, in the
mouth, and/or on the tongue.
- Vertigo
- Hearing loss
- Eye pain

A person affected by Ramsay Hunt Syndrome

In taking the history and PE of a patient with such symptoms…

It is important to ellicit the history of previous varicella zoster virus

infection, more commonly known as chicken pox. Most people would
remember if they had a previous chiken pox infection, as their “marks” would
show (maculopapular dyspigmentations secondary to Varicella infection).
.
When asked, patients may recall a distant history, perhaps in childhood,
of chickenpox (varicella). Have you ever had chickenpox? If yes, then it is
not imposible that you may also get infected with the herpes zoster virus!

So now, how can one differentiate a central lesion of the facial nerve
from that of a peripheral lesion such as that of Herpes Zoster Oticus?

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 Extra Challenge:
How could you differentiate a central lesion of the facial nerve from a peripheral lesion
such as that of Herpes Zoster Oticus?

Activity 12:

Explain the associated findings in Herpes Zoster Oticus

1.Dysgeusia (alteration in taste)

2.Inability to fully close the ipsilateral eye, which may lead to the
occasional presentation of drying and irritation of the cornea.

How do we treat Herpes Zoster Oticus?

The treatment of herpes zoster oticus is mainly supportive, including the use
of warm compresses, analgesics, and systemic antibiotics for secondary
bacterial infection. Again corticosteroids come into the picture for prompt control
of inflammation while anti-virals are used if a viral etiology is being considered.

3. Otitis Media

Ear infections, such as chronic otitis media can also compromise facial nerve
function. This is because a substantial part of the facial nerve passes through
the middle ear. Therefore, infections of the middle ear, manifested as chronic ear

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discharge (mostly since shildhood) could also be accompanied by facial nerve
paralysis.

C) Traumatic/Toxic

Trauma, including iatrogenic transaction or tractions, to the facial nerve can

occur along its course, specifically in surgeries such as that of the parotid gland
and of the middle ear and temporal bone.

Surgery to the parotid gland may compromise facial nerve function by

accidental, and unwanted transection. Principles of surgery of the parotid gland
primary centers on isolation of the facial nerve as it transects the superficial and
deep lobe of the parotid gland. Only when the facial nerve is identified, should
the surgeon begin excision of the parotid gland.

Surgeries involving the middle ear and temporal bone can also compromise
facial nerve function as the facial nerve courses along the middle ear area.
Again, it is thus very vital to identify the nerve during surgery, in order not to
damage it.

D) Neoplastic
1. Parotid Tumor and other malignancies

Parotid glands, which comprise part of what is considered the major

salivary glands saliva, are located infrauricularly. They are divided into a deep
and a superficial lobe by the facial nerve. There is however no strict anatomic
division of the parotid gland and part of the gland anterios to the branches of
facial nerve in it is considered superficial and that found posterior to the nerve
is deep. The glands are not attached to the bones of the upper or lower jaw.

Most parotid tumors (80%) are benign. The remaining 20% are malignant.
The most common benign tumor of the parotid gland is called pleomorphic
adenoma or bengin mixed tumor while an adenoid cystic carcinoma is a
common malignant tumor of the parotid gland. Factors that increase your risk
of a cancerous parotid tumor include: Radiation exposure to the jaw or neck
and tobacco use.

The most common tests to determine the nature of a parotid tumor include
a CT scan and MRI which can identify tumors, nodes and other soft-tissue
abnormalities adjacent to or within the parotid gland. FNA (fine needle
aspiration biopsy), may also be helpful. During this procedure, a small amount
of fluid is withdrawn from the parotid to see if malignant cells are present.

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 The facial nerve may be compressed by a large tumor, or be in close
proximity to a tumor. In these cases, the facial nerve will inevitably be
damaged (to a variable degree) if the nerve is peeled from the capsule of the
tumor. In general, the function of the damaged facial nerve will recover in
three months if the nerve itself is not disrupted. During this period, treatment,
including injection of Vitamin B1, B12, and the functional training of facial
muscles, may serve to accelerate the recovery of facial nerve weakness.

Treatment of Parotid Tumors

The surgical procedure of superficial parotidectomy is commonly used in

the treatment of superficial parotid tumors. It is essential that, where possible,
the facial nerve should be preserved, so its identification and careful
dissection is of paramount importance. Mechanical damage to the facial
nerve should be avoided in most cases if surgical excision is to be done.
Radiation therapy, if the tumor is malignant

An Illustration of the Parotid Gland

Temporal bone CA

Carcinoma of the temporal bone is rare, accounting for less than 0.2% of
all tumors of the head and neck. This includes cancers arising from skin of the
pinna that spread to the temporal bone; primary tumors of the external auditory
canal (EAC), middle ear, mastoid, or petrous apex; and metastatic lesions to the
temporal bone.

These tumors can spread and enlarge to compress the facial nerve structure
and affect its function. Tumor seeding might also use the course of the facial

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Facial nerve schwanoma/neurolimomma

About 5% of cases of facial nerve paralysis are caused by tumors, such as

those previously mentioned. Factors which should increase the clinician’s
suspicion of a possible tumor include: a slowly developing paresis over more
than three weeks, facial twitching, additional cranial nerve deficits, recurrent
ipsilateral involvement, associated adenopathy, or a palpable neck or parotid
mass. In these cases, MRI and CT should be obtained.

One of the more common benign tumors causing facial nerve paralysis is
a facial nerve schwanomma. A study by Lapena and Chiong (1997) reviewed a
case series of 5 patients from the Philippine General Hospital who all presented
with unilateral facial asymmetry and who all had a histologic diagnosis of facial
nerve neurilemomma. This study highlights the fact that although considered as
very common Bell’s palsy is not the sole etiology in patient’s presenting with
facial asymmetry.

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10. Smeets NWJ, Krekels GAM, Ostertag JU, et al. Surgical excision vs. Moh’s
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13. www.cleftline.org/som/0301/Tessier%20Info.pdf

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