Clinical aspects of cleft palate repair

Clinical Aspects of Cleft Palate Repair

Ahmed Atef, Msc, MRCSSpecialist of plastic surgery

Mataria Teaching Hospital

Objective

• Incidence

• Surgical Anatomy

• Embryology

• Classification / Cleft Variants

• Clinical Effects

• Management

• Future

Cleft Palate / Cleft lip is the the most commoncraniofacial malformation

Second most common congenital defect

Isolated Cleft palate

• No racial variation

• 1:2000 live birth

• M:F = 1:2

• Left : Right : B/L = 6:3:1

Surgical Anatomy

The palate forms a dynamic boundary between the oral cavity and the nasal cavity. It is composed of the hard palate anteriorlyand the soft palate posteriorly.

Normal Palate

• Primary Palate

• Secondary Palate

Hard Palate

Soft Palate

Surgical Anatomy

Surgical Anatomy

The hard palate includes the palatal processes of the maxilla and the horizontal plate of the palatine bone with adherent mucoperiosteum (attached to bone by Sharpey’s fibres).

Surgical Anatomy

Three pairs of foramina mark the surface of the bony palate

• Incisive Foramen

• Greater Palatine Foramen

• Lesser Palatine Foramen

The soft palate is a dynamic structure that acts as a valve between the oropharynx and nasopharynx.

An intact and functioning soft palate is essential for normal speech and feeding.

Soft palate• Mucosa• Five paired muscles &

central aponeurosisTensor veli palatiniLevator veli palatiniPalatoglossusPalatopharyngrusUvualis

*Veli (Latin) means curtain

Surgical Anatomy

Tensor palatiOrigin: scaphoid fossa of the medial

pterygoid plate, the lateral part of the cartilaginous auditory tube then passes around the pterygoidhamulus as a tendon

Insertion: broad triangular tendon at the posterior aspect of the hard palate as part of the palatine aponeurosis

Action: tense the soft palate to form a platform that the other muscles may elevate or depress.

Surgical Anatomy

Levator palati

Origin: petrous bone and the medial part of the auditory tube

Insertion: middle third of upper surface of the soft palate at upper surface of the palatine aponeurosis as far as the midline

Surgical Anatomy

Levator palati

The paired muscles form

a ‘V’-shaped sling pulling

the soft palate upwards

and backwards to close

the nasopharynx.

Surgical Anatomy

Palatoglossus

Origin: Palatine aponeurosis

Insertion: Side of tongue

Action: Pulls root of tongue upward and backward, narrows transverse diameter of oropharynx

Surgical Anatomy

Palatopharyngeus

Origin: Palatine aponeurosis

Insertion: Posterior border of thyroid cartilage

Action: Elevates wall of pharynx, pulls palatopharyngeal folds medially

Surgical Anatomy

Musculus uvulae

Origin: Posterior border of hard palate

Insertion: Mucous membrane of uvula

Action: Elevates uvula

Surgical Anatomy

The soft palate is raised by the contraction of the levator palati.

At the same time, the upper fibers of the superior constrictor muscle pull the posterior pharyngeal wall forward.

The palatopharyngeus muscles contract to pull palatopharyngealarches medially, like side curtains.

Surgical Anatomy

By this means The intact palate can periodically, selectively, and completely isolate the nasopharynx from the oropharynx during Feeding & Speech

Surgical Anatomy

This harmony in muscular action is necessary for

Velopharyngeal Competence

Surgical Anatomy

Surgical Anatomy

Surgical Anatomy

Embryology

Development of the face begins in the fourth week in utero, when neural cells migrate and fuse with mesodermal elements to form the facial primordium.

It results from the fusion

– Two mandibularprocesses

– One frontonasal process

– Two maxillary processes

Embryology

The palate develops between the 5th and the 12th week

CRITICAL period of palatal development is between the 6th and the 9th week.

Soft palate development is completed at 12th week

Embryology

Primary palate : Median palatine process from the medial nasal prominences.

Secondary palate : Lateral palatine process from the maxillary prominence

Embryology

6th – 9th week: Initially, the palatine processes are oriented vertically on either side of the developing tongue.

The tongue is displaced inferiorly as the head grows and the neck straightens, the lateral palatine processes are elevated and grow medially to fuse with the septum

Embryology

Is Cleft a Deficiency?

Embryology

Interference with fusion results in Cleft

Three theories:

i) Failure of fusion of the lateral shelves

ii) Failure of mesodermal penetration of the shelves:

iii) Mechanical interference (the tongue) such as in Pierre Robbin Sequence

Embryology

Gato et al. 2002, expression of chondroitin sulfate proteoglycan is important in palatal shelf adhesion and is supposed to be regulated by TGF-b3

Gato A, Martinez ML, Tudela C, Alonso I, Moro JA, Formoso MA, Ferguson MWJ, Martinez-lvarez C (2002) TGF-b3-induced

chrondroitin sulphate proteoglycan mediates palatal shelf adhesion.

Bush et al. 2003; Herr et al. 2003, Expression of T box transcription factor Tbx22 is found in the inferior nasal septum and the palatal shelf before fusion.

Bush JO, Lan Y, Maltby KM, Jiang R (2002) Isolation and developmental expression analysis of Tbx22, the mouse homolog of the human x-linked cleft palate gene. Dev Dyn 225: 322-326

Herr A, Meunier D, Mller I, Rump A, Fundele R, Ropers H-H, Nuber UA (2003) Expression of mouse Tbx22 supports its role in palatogenesis and glossogenesis. Dev Dyn 226:579–586

Embryology

Classification

Veau Classification 1931

Veau Class I: isolated soft palate cleft

Veau Class II: isolated hard and soft palate

Veau Class III: unilateral CLAP

Veau Class IV: bilateral CLAP

Classification

Striped Y by Kernahan 1971 Millard modification

Cleft Variant

Cleft Variant

Cleft Variant

Cleft Variant

syndrome Treacher-Collins

Syndromatic Cleft

Pierre Robin syndrome

Syndromatic Cleft

Syndromatic Cleft

Van der Woude’s syndrome

Clinical effects

Patients with cleft deformities experience a multitude of problems including

• Feeding problems

• Speech difficulties

• Otologic issues

• Midface growth impairment.

Clinical effects

Feeding

The infant is usually not able to suck efficiently due to inability to achieve negative pressure.

Nasal regurgitation.

Feeding regimen: includes the use of squeeze bottles and holding in a nearly sitting position during feeding

Clinical effects

Speech

Patients are unable to produce high intra-oral pressure.

Normal velopharyngeal closure is crucial for production of intelligible speech; any abnormalities in this mechanism can result in hypernasality, nasal emissions, imprecise production of consonants.

Hearing

Serous otitis media.

Abnormality of LVP which aids the TVP to dilate ET.

Nasal regurgitation.

Treatment with myringotomy tubes is required pre- and post-cleft repair.

Management requires a multidisciplinary approach spanning multiple specialties

• Plastic surgery

• Speech pathology

• Otolaryngology

• Genetics

• Pediatrics

• Orthodontics

• Audiology

Goal

Restoring the morphologic form & function

Production of a competent velopharyngealsphincter

Principles

• Closure of the defect

• Correction of the abnormally inserted muscles

• Reconstruction of the palatine sling

• Tension free repair

• 2 layer repair of the hard palate & 3 layer repair of the soft palate

Von Langenbeck 1861 pioneered the first bipediclemucoperiosteal flaps and relaxing incisions for palate closure surgery in one stage.

Langenbeck v, B. Uranoplasty by means of raising mucoperiosteal flaps. Arch klin chir. 1861;2:205

Veau 1931, The vomer flap and suturing of velar muscles aiming at lengthening the palate

Wardill and Kilner 1937, “pushback” theory V-Y retro positioning of the palate increases the length further.

By connecting the lateral incisions to the incisions made for the nasal turn in flaps.

Wardill WEM. The technique of operation for cleft palate. Br J Surg. 1937;25: 117-130

A different approach was described by Furlow 1986 with the double-opposing z-plasty without relaxing incisions

Furlow LT, Jr. Cleft palate repair by double opposing Z-plasty. Plastic and reconstructive surgery. 1986;78:724-738

The Bardach 1991 two-flap palatoplasty uses two large full-thickness hard palate flaps that are mobilized and closed anteriorly and medially without pushback

Bardach, J. and P. Nosal: Geometry of the two-flap palatoplasty. (2nd). St. Louis, Mosby-Year Book, 1991

2002 ., & Sommerlad et al2000 Rohrich et al.,

Closure of the palate can be performed in two stages. This involvesclosing the soft palate early, between 3 and 6 months

of age, and delaying the repair of the hard palate.Sommerlad BC, Mehendale FV, Birch MJ, Sell D, Hattee C, Harland K. Palate rerepairrevisited. Cleft Palate Craniofac J. 2002;39:295-307.

To limit the effect of the hard palate repair on maxillary growth. It is suggested that the subperiosteal scarring impairs midfacial growth.

Preoperative considerations

• Age: 9-12 month

• Associated anomalies

• Routine Lab. Investigations

• Booking a unit of packed RBCs after G/XM

• Otologic and audiologic assessment

Operative preparations

i) RAE tube

ii) Dingman

iii) Shoulder roll

iv) Head Donut

v) Local anesthetic with

1:200,000 epi

vi) Position: supine, neck

Extended, reverse trendlenberg

vii) Throat pack

Operative preparations

Steps

i) Inject 1 :200 000 epinepherine into the palate.ii) Don't inject in areas sutures will be placediii) Wait 7 minutes for the epinephrine to take effectiv) Make incision along the medial side of the cleft v) Make releasing incision to get to bone on both sidesvi) Use freer to elevate mucoperiosteal flapvii) Dissect nasal mucosavii) Strip LVP muscle off abnormal insertion & create palatine

slingviii) Three layer repair

Steps

Vomerian flap

Postoperative care

• Keep your eye on the airway

• AB

• Analgesic

• Feeding: fluids, soft diet, no bottles for 3w

• Arm restraints

ComplicationsEarly:

Haemorrhage

Airway obstruction

Dehiscence

Fistula

Late:

Bifid uvula

VPI

Maxillary hypoplasia

Dental malalignment

Tissue engineering advancements over the last decade has provided a plethora of materials that may be suitable for the healing of craniofacial defects like the cleft palate.

Future directions with regards to the use of stem cells especially ASCs in craniofacial repair are discussed, including possible scaffold for reconstruction of palatal defect

Quiz

Embryogenesis of primary & secondary palate?

Muscles of soft palate?

Velopharyngeal mech?

Clinical effects?

Preoperative preparations?

Principles of repair?

Postoperative care?