Developmental Anomalies of The Face Dr Ryan Fernandes

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Developmental Anomalies of The Face Dr Ryan Fernandes Assistant Professor Department Of General Surgery

Developmental Anomalies of The Face Dr Ryan Fernandes Assistant Professor Department Of General Surgery

Learning objectives • To accurately know the development of face. • To enumerate the

Learning objectives • To accurately know the development of face. • To enumerate the developmental anomalies of the face. • To know the clinical features and management of Cleft lip and Cleft palate

 • The face is formed by 5 processes which surround an opening -

• The face is formed by 5 processes which surround an opening - the stomatodaeum 1. Frontonasal – single process 2. Maxillary – one on each side 3. Mandibular – one on each side

FRONTONASAL PROCESS • Two nasal pits, each surrounded by a lateral and medial nasal

FRONTONASAL PROCESS • Two nasal pits, each surrounded by a lateral and medial nasal process. • The two medial processes fuse to form the intermaxillary segment • It consists a. Philtrum of the upper lip b. An upper jaw component which carries the four incisor teeth c. The triangular primary palate(pre maxilla) d. The nasal septum which develops as a downgrowth from fused medial nasal processes

 • The lateral nasal process forms the side of the nose. It takes

• The lateral nasal process forms the side of the nose. It takes no part in the formation of the upper lip

THE MAXILLARY PROCESS • The maxillary process fuse with the lateral nasal process at

THE MAXILLARY PROCESS • The maxillary process fuse with the lateral nasal process at the nasolacrimal groove and with the medial nasal processes to form the lateral part of the upper lip • The two lateral palatine processes fuse in the midline to form the secondary palate – develop from inner aspect of maxillary process • The palate is formed by the fusion of secondary palate to primary palate • The incisive foramen marks the junction of the two components of the palate.

THE MANDIBULAR PROCESS • The mandibular processes give rise to the lower lip and

THE MANDIBULAR PROCESS • The mandibular processes give rise to the lower lip and jaw. • When the epithealial structures have fused, a mesodermal mass flows in between the two layers of the fused epithelium to provide supporting structures of the face.

Classification – Cleft Lip and Cleft Palate I. Cleft lip alone II. Cleft of

Classification – Cleft Lip and Cleft Palate I. Cleft lip alone II. Cleft of primary palate III. Cleft of secondary palate IV. Cleft of both primary & secondary palates V. Cleft lip & cleft palate together

I. Cleft lip alone • Unilateral • Bilateral • Median

I. Cleft lip alone • Unilateral • Bilateral • Median

II. Cleft of primary palate only This lies in front of the incisive foramen.

II. Cleft of primary palate only This lies in front of the incisive foramen. a. Complete – absence of pre maxilla b. Incomplete – rudimentary pre maxilla • Unilateral • Bilateral • Median

III. Cleft of secondary palate only This is the cleft which lies behind the

III. Cleft of secondary palate only This is the cleft which lies behind the incisive foramen a. Complete – nasal septum & vomer are separated from palatine process b. Incomplete c. Submucous It can be - cleft with soft palate involvement - cleft without soft palate involvement

CLEFT LIP • Central – rare. Seen in upper lip between 2 medial nasal

CLEFT LIP • Central – rare. Seen in upper lip between 2 medial nasal processes (Hare Lip) • Lateral -maxillary & median nasal processes, commonest, can be unilateral or bilateral • Incomplete cleft lip does not extend into the nose • Complete cleft lip extends into the nasal floor • Simple cleft lip is only the cleft in the lip • Compound cleft lip is cleft lip with cleft of the alveolus

Anatomy Of Cleft Lip • Occurs due to disruption of muscles of the upper

Anatomy Of Cleft Lip • Occurs due to disruption of muscles of the upper lip & nasolabial region. • The facial muscles are divided into 3 muscular rings of Delaire – A. Nasolabial muscle ring B. Bilabial muscle ring C. Labiomental muscle ring

Unilateral Cleft Lip • Nasolabial & bilabial muscle rings are disrupted on one side.

Unilateral Cleft Lip • Nasolabial & bilabial muscle rings are disrupted on one side. • Results in asymmetrical deformity involving the external nasal cartilages, nasal septum & anterior maxilla. • These deformities influence the mucocutaneous tissues causing a displacement of nasal skin onto the lip as well as changes to the vermillion & lip mucosa

Bilateral Cleft Lip • Here the deformity is more profound but symmetrical. • The

Bilateral Cleft Lip • Here the deformity is more profound but symmetrical. • The two superior muscular rings are disrupted on both sides producing a flaring of the nose, a protrusive pre maxilla & an area of skin in front of the pre maxilla devoid of muscle known as prolabium.

Cleft Palate. Embryology • Embryologically, the primary palate consists of all anatomical structures anterior

Cleft Palate. Embryology • Embryologically, the primary palate consists of all anatomical structures anterior to the incisive foramen, namely the alveolus and upper lip. • The secondary palate is defined as the remainder of the palate behind the incisive foramen, divided into the hard palate and, more posteriorly, the soft palate.

Hard palate • The normal hard palate can be divided into three anatomical and

Hard palate • The normal hard palate can be divided into three anatomical and physiological zones 1. The central palatal fibromucosa is very thin and lies directly below the floor of nose. 2. The maxillary fibromucosa is thick and contains the greater palatine neurovascular bundle. 3. The gingival fibromucosa lies more lateral and adjacent to the teeth.

Soft palate • The muscle fibres of the soft palate are orientated transversely with

Soft palate • The muscle fibres of the soft palate are orientated transversely with no significant attachment to the hard palate. • In a cleft of the soft palate the muscle fibres are orientated in an anteroposterior direction, inserting into the posterior edge of the hard palate

CLEFT PALATE • It is due to failure of fusion of two palatine processes.

CLEFT PALATE • It is due to failure of fusion of two palatine processes. • Defect in fusion of lines between pre-maxilla & palatine processes of maxilla one on each side. • When premaxilla & both palatine processes do not fuse, it leads into complete cleft palate(Type I cleft palate)

 • Incomplete fusion of these 3 components can cause incomplete cleft palate beginning

• Incomplete fusion of these 3 components can cause incomplete cleft palate beginning from uvula towards posteriorly at various lengths • So it could be – ØType II a – bifid uvula ØType II b – bifid soft palate ØType II c – bifid soft palate & posterior part of hard palate.

Clinical Features • Small maxilla with crowded teeth, absent/poorly developed upper lateral incisors. •

Clinical Features • Small maxilla with crowded teeth, absent/poorly developed upper lateral incisors. • Bacterial contamination of upper respiratory tract with recurrent infection. • Chronic otits media with deafness. • Swallowing difficulties & speech problems. • Cosmetic problems.

Problems in cleft disorders • Difficulty in sucking & swallowing • Speech is defective

Problems in cleft disorders • Difficulty in sucking & swallowing • Speech is defective especially in cleft palate mainly to phonate B, D, K, P, T & G. • Altered dentition or supernumerary teeth

Primary Management Antenatal diagnosis • An antenatal diagnosis of cleft lip, whether unilateral or

Primary Management Antenatal diagnosis • An antenatal diagnosis of cleft lip, whether unilateral or bilateral, is possible by ultrasound scan after 18 weeks of gestation. • Isolated cleft palate cannot be diagnosed by antenatal scan. • When an antenatal diagnosis is confirmed - referral to a cleft surgeon

Antenatal (Contd) • Photographs of cleft lip shown to parents ‘before and after’. •

Antenatal (Contd) • Photographs of cleft lip shown to parents ‘before and after’. • Introduction to a parent support group and meeting parents of a child with a similar cleft who has undergone surgery

Feeding • Most babies born with cleft lip and palate feed well and thrive,

Feeding • Most babies born with cleft lip and palate feed well and thrive, provided that appropriate advice is given and support is available. • Some mothers are successful in breastfeeding, particularly when the cleft is incomplete and confined to the lip. • Good feeding patterns can be established with soft bottles and modified teats.

Feeding(Contd) • Simple measures, such as enlarging the hole in the teat. • Feeding

Feeding(Contd) • Simple measures, such as enlarging the hole in the teat. • Feeding plates, constructed from a dental impression of the upper jaw, are rarely necessary to improve feeding.

Airway • Hypoxic episodes during sleep and feeding. • Intermittent airway obstruction is more

Airway • Hypoxic episodes during sleep and feeding. • Intermittent airway obstruction is more frequent and is managed by nursing the baby prone. • Persistent airway compromise - ‘Retained nasopharyngeal intubation’ to maintain the airway. • Surgical adhesion of the tongue to the lower lip (labioglossopexy) in the first few days after birth is an alternative

Delaire timing of cleft surgery • Unilateral/bilateral cleft lip alone, in one stage operation

Delaire timing of cleft surgery • Unilateral/bilateral cleft lip alone, in one stage operation done in 4 to 6 months. • Cleft palate alone involving only soft palate, in one stage surgery is done in 6 months. • For cleft palate alone involving both soft & hard palates soft palate in 6 months, hard palates in 18 months. • In combined cleft lip & palate, unilateral or bilateral in 2 stages – cleft lip & soft palate in 6 months, hard palate in 18 months

Treatment for Cleft Lip Millard criteria used to undertake surgery for cleft lip (Rule

Treatment for Cleft Lip Millard criteria used to undertake surgery for cleft lip (Rule of 10) • 10 kg in weight • 10 weeks old • 10 gm% haemoglobin

 • Millard cleft repair by rotating the nasolabial flaps • Management of associated

• Millard cleft repair by rotating the nasolabial flaps • Management of associated primary or secondary cleft palate deformity • Proper post operative management like control of infection, training for sucking, swallowing & speech

Cleft lip surgery • Skin incisions are developed to restore displaced tissues, including skin

Cleft lip surgery • Skin incisions are developed to restore displaced tissues, including skin and cartilage, to their normal position, while gaining access to the facial, nasal and lip musculature. • Muscular continuity is achieved by subperiosteal undermining over the anterior maxilla. • Nasolabial muscles are anchored to the premaxilla with non-resorbable sutures.

Oblique muscles of orbicularis oris are sutured to the base of the anterior nasal

Oblique muscles of orbicularis oris are sutured to the base of the anterior nasal spine and cartilaginous nasal septum. Closure of the cleft lip is completed by suturing the horizontal fibres of orbicularis oris to achieve a functioning oral sphincter.

Cleft palate surgery • Cleft palate closure can be achieved by one- or twostage

Cleft palate surgery • Cleft palate closure can be achieved by one- or twostage palatoplasty. • The surgical principle is mobilisation and reconstruction of the aberrant soft palate musculature together with closure of the residual hard palate cleft by minimal dissection and subsequent scar formation

Principles Of Palatoplasty • Timing is between 14 18 months. • Mucoperiosteum flap is

Principles Of Palatoplasty • Timing is between 14 18 months. • Mucoperiosteum flap is raised. • Palatal defect is closed using 3 layers – nasal, muscle or oral layers.

 • Early repair causes retarted maxillary growth- due to trauma to the growth

• Early repair causes retarted maxillary growth- due to trauma to the growth centre & periosteum of the maxilla during surgery if done early. • Late repair causes speech defect.

Secondary Management • Regular examination of ear, nose and throat • Hearing support is

Secondary Management • Regular examination of ear, nose and throat • Hearing support is given using hearing aids. • Speech problems occur due to velopharyngeal incompetence, articulation problems also can occur- speech therapy is given. • It is corrected by pharyngoplasty, veloplasty, speech devices.

Hearing • Sensorineural hearing loss – hearing aid • Secretory otitis media – prophylactic

Hearing • Sensorineural hearing loss – hearing aid • Secretory otitis media – prophylactic myringotomy - grommet insertion • Regular audiological assessment during childhood

Speech • Velopharyngeal incompetence – increased nasal airflow producing a nasal quality of speech

Speech • Velopharyngeal incompetence – increased nasal airflow producing a nasal quality of speech associated with poor function of soft palate • Articulation problem – videofluoroscopy study, nasal airflow. • Speech problem – Speech and language therapy.

Dental • Delayed tooth development • Delayed eruption of teeth • Hypodontia or hyperdontia

Dental • Delayed tooth development • Delayed eruption of teeth • Hypodontia or hyperdontia occurring in the region of cleft alveolus • Dietary advice, fluoride supplements and fissure sealants

Orthodontic Management • Mixed dentition(8 to 10 years) – to expand maxillary arches as

Orthodontic Management • Mixed dentition(8 to 10 years) – to expand maxillary arches as a prelude to alveolar bone graft • Permanent dentition(14 – 18 years) – to align dentition and provide a normal functioning occlusion. Also involves surgical correction of malpositioned/retrusive maxillary osteotomy.

SUMMARY • Cleft lip and cleft palate are the most common congenital anomalies of

SUMMARY • Cleft lip and cleft palate are the most common congenital anomalies of the head and neck. • Occur 1 in every 750 live births. • Anomalies occur due to failure of separate mesodermal masses to flow between various processes which are already joined by two layers of epithelial cells. • Millard’s surgery is the most common surgery performed for cleft lip.

References • 1. • 2. • 3. • 4. Lee Mcgregor’s Synopsis of Surgical

References • 1. • 2. • 3. • 4. Lee Mcgregor’s Synopsis of Surgical Anatomy Human Embryology – Inderbir Singh Bailey and Love Textbook of Surgery SRB’s Manual of Surgery

THANK YOU

THANK YOU