“PALATOSCHISIS”

CLEFTS?????Distortions of the face and cranium with deficiencies or excesses of tissues that cleave anatomic planes in a linear fashion.

(PEDIATRIC PLASTIC SURGERY, MATHES)

Incidence CLP- Most common craniofacial anomaly - Second

most common Congenital anomaly*.*(2nd to club foot) CLP- 3.7/1000 live births CPO- 0.62/1000 live births SMC- 1 in 1200 to 2000 live births CL/P-45% CP- 40% CL-15% Males- CL/P Females- CPO Racial differences- CPO-Not significant

CLP- Asians> Blacks Syndromes

- CLP occurs as part of >100 syndromes

- 50% CP and 30 % CL/P have associated anomalies.

Etiology Genetic

Environmental

smoking, steroids, anticonvulsants, alcohol, folate deficiency

Excess retinoic acid Maternal Age Syndromic clefts- CPO- common (50%) CL- rare

Sticklers SyndromeVelocardiofacial SyndromeGoldenhar SyndromeOrofacial Digital SyndromeVan Der Woudes SyndromeTreacher Collins Syndrome

Embryology of Cleft Palate 8-11 weeks

EMBRYOGENESIS AT 7TH-8TH WEEK

• FAILURE OF FUSION- Dursy (1869) & His (1892)(Fusion of fingerlike facial processes)

• MESODERMAL PENETRATION THEORY- Warbrick(1938) Stark & Ehrmann(1958)

(Bilaminar ectodermal sheets> mesenchymal & neural crest cell migration)

• NEUROMERIC THEORY- Carstens MH (2000, 2002)(-Neural tube conceived as a series of developmental zones within CNS-Neuromeric zones- 6 prosomeres (Shh, Wnt, En),

2 mesomeres, 12 rhombomeres (HOX)

-Unique barcode (genetic code) for each neuromeric zone)Adv- Permits mapping of face into devptal zones

Anatomic & Clinical obs of Tessier is compatible

Theories

Patten

Secondary palate

except uvula Lip & Uvula

Causes of cleft palate Hypoplasia oxycephaly Abnormal directional growth of the mesenchymal

processes Failure of fusion Breakdown of the fused mesenchymal process Threshold

Prenatal Diagnosis by USG 13 to 16 wks. of IUL – a good thing

The sensitivity is highest when is associated with other structural anomalies.

Cleft palate with an intact lip is the most difficult orofacial malformation to diagnose prenatally.

Detected in only 13 of 198 cases in one large series.

Three-dimensional ultrasound, can provide a clear image of the malformation.

Types of Palatal Clefts Unilateral Bilateral

Narrow Wide

Complete Partial Sub-

mucosal

CLASSIFICATIONS

VEAU, 1931

KARNAHAN & STARK,1958 modified by ELSAHY, 1973 & MILLARD, 1977

1 4

7

8

9

FRIEDMANS MODIFICATION Too cumbersome

LASHAL- Paraphrase given by Kreins, 1989 Anatomic site, side and type of cleft

Complete- UPPER CASE Partial- lower case Microform- *

American Cleft Palate Association, 1962

TESSIERS CLASSIFICATION OF CRANIOFACIAL CLEFTS

ANATOMY

ANATOMY THE LEVATOR SLING - ELEVATES THE SOFT

PALATE (BACKWARD AND UPWARD DIRECTION)

THE PALATOPHARYNGEAL SLINGS- APPROXIMATES THE POSTERIOR FAUCIAL PILLARS AND NARROW THE PHARYNGEAL APERTURE

TWO SLINGS ACT TOGETHER BY CONTRACTION

EACH GROUP FORMS A “V” INTO AND THE WHOLE MECHANISM PARTAKES OF THE FEATURES OF “X”.

IN 1969 KRIENS SUMMARIZED THE PATHOLOGY OF THE CLEFT PALATE ANOMALY AS THE BASIS FOR PLANNING RECONSTRUCTION:

THE FORWARD AND TRANSVERSE DISPLACEMENT OF MUSCULAR INSERTIONS BUT THE ORIGINS ARE NORMAL.

THE SYNERGISTICALLY INTERWOVEN ANTERIOR PORTIONS OF THE LEVATOR VELI PALATI AND OF THE LONGITUDINAL PORTION OF THE PALATOPHARYNGEAL MUSCLE (VEAU’S CLEFTMUSCLE).

THE PALATOSALPINGEAL FASCIA FORMING FUNCTIONAL BOUNDARY BETWEEN THE CONSTRICTOR PHARYNGEAL AND PALATOPHARYNGEAL MUSCLES (LEVATOR SPACE OF CLEFT PALATE).

THE PLANE OF LOOSE CONNECTIVE TISSUE BETWEEN THE PALATOPHARYNGEAL SPHINCTER AND THE PALATOSALPINGEAL MUSCLE (PLANE OF BLUNT DISSECTION IN THE VELUM PROPER).

THE ALTERED INSERTION OF THE HYPOPLASTIC HORIZONTAL PALATINE TENDON OF THE TENSOR VELI PALATI (NO APONEUROSIS IN THE CLEFT VELUM)

Sub- mucous Cleft (CALNAN) Midline clear zone ( discontinuous Levator veli

palatini) Palpable notch (Muscle diastasis) Bifid uvula Diagnosis- Speech evaluation & Endoscopy

MANAGEMENT OF CLEFT PALATE- PROBLEM LIST Oro- nasal communication Feeding difficulties Dental problems- Malocclusion Growth Speech Hearing Recurrent ear infections

Airway obstruction may present in children with a cleft palate, especially those with mandibular hypoplasia (ie, a Pierre Robin sequence).

Otitis > 50% children with CLP Restriction of growth is seen in 25-35%

cases- Maxillary Hypoplasia VPI- Speech difficulties Malocclusion

FEEDING DIFFICULTIES

OVERCOMING FEEDING DIFFICULTIES

Weight gain is important. 2 to 3 ounces of milk per pound per

24 hours Feeding sessions < 35 minutes The Mead-Johnson Cleft Palate Nurser

is the most commonly used bottle and nipple for babies with clefts. 

 Ross Cleft Palate Nurser  Haberman Feeder, manufactured by

Medela

GOALS of treatment Intact primary and secondary palate Normal speech, language, and

hearing Nasal airway patency Class I occlusion with normal

masticator function Good dental and periodontal health Normal psychosocial development

ASSESSMENT OF THE NEONATE

Cleft Palate Team

Coordinator Plastic surgeon Otolaryngologist and

Audiologist Speech Pathologist Maxillofacial Surgeon Dentist and

Orthodontist Genetic councellor

Neonatal Maxillary Orthopaedics C. Kerr McNeil, neonatal maxillary orthopedics still

remains a controversial part of the comprehensive care for cleft lip and palate.

Important aspect of preoperative treatment: control and correction of the lateral segments in

unilateral and bilateral conditions the effect of bone stimulation plays an important

role in this correction.

Forces which are within the limits of biological tolerance are said to stimulate bone & can be regarded as “intensified normal forces”.

Appliances•Active-Extra- Oral Strapping•Semi-active- the McNeil and Burston type of appliances•Passive - Zurich approach as proposed by Hotz & Gnoinski

Early primary alveolar Bone grafts- OUTDATED

Claimed Advantages

1. Establish normal tongue posture, aiding speech development

2. Aid in feeding 3. Help the surgeon in uniting the lip and

closing the floor of the nose 4. Be a psychological help to the parents 5. Stimulate palatal bone growth 6. Reduce middle ear infections 7. Prevent the collapse of palatal segments,

thereby reducing the need for future complex orthodontic treatment

Rigid External Distractor

McCarthy in 1990’sSevere maxillary hypoplasiaCan be started as early as 5

years of age.

PRIMARY SURGICAL TREATMENT GOALS

Separation of Oral & Nasal Cavities. Anatomic approximation of

displaced muscles. Appropriate Speech outcome. Non- restriction of Maxillary growth.

Early palate closure

Pre- op Health Status GA investigations Syndromes(Cardiac, Renal, spine Abnormalities) Audiological Evaluation

Intra- op RAE endotracheal tube Dingman’s gag LA/ Saline-adrenaline infil. Neurovascular bundle- Greater palatine vessels

and nerve Incision Circumferential freeing Nasal closure Muscle Oral Closure

*Junction of HP & Sp – most common location for fistulas

History of Cleft Palate Sugery

Roux (early 19th century) (Sub- mucosal cleft surgery)

Carl Ferdinand Von Graefe (1820)

Johann Friedrich Diffenbach (1840)(Lateral relaxing incisions & Ether anaesthesia)

Bernhard Von Langenbeck(Mucoperiosteal Plane)

Surgical Treatment Timing of surgical closure depends on the width and not

the length of the cleft space. No single technique used is universal Furlow procedure is the most common technique for cleft

palate closure. Surgical techniques :

von Langenbeck Schweckendiek 2-flap 3-flap (V-to-Y) double reverse z-plasty (Furlow) palatoplasties

Although most of the repairs do not involve repairing the muscular sling, doing so allows better palatal and eustachian tube functions.

von Langenbeck Technique(1861) Every CP repair performed

today incorporates its principles

Mucoperiosteal flaps Flaps are advanced

medially to close the palatal cleft.

Advantages:

•Simple

•Less dissection

Disadvantages:•Does not increase the length of the palate•Anterior fistulas Note

it is impossible to obtain a two-layer closureof the alveolar portion of the cleft

Newer Methods: Intra velar veloplasty

Veau- Wardill- Kilner Technique (1937)

Dorrance- VPI- # of hamulus- change of muscle vector & comb with von Langenbeck tech will increase soft palate length.

In 1937, Kilner and Wardill independently described the V-Y repositioning technique.

This technique is primarily used for repair of incomplete clefts or clefts of the secondary palate.

Osteotomy & repositioning of greater palatine foramen.

Advantage:Maintainence of blood supplyUseful in Wider cleftsAdds lengthDisadvantage:Fistula

Furlow’s Double reverse z-plasty In 1986, Furlow described a technique to lengthen

the velum and to create a functioning levator muscle sling.

This method is difficult to perform in wide clefts but a good method when the cleft is narrow or if a submucous cleft exists.

The technique involves opposing z-plasties of the mucosa and the musculature of the soft palate.

The goal is to separate the nonfunctioning attachments to the posterior border of the hard palate and to displace the mucosa and the musculature posteriorly.

Advantages:•Nasal z- plasty placed more laterally•Good speech outcome•Less fistulaDisadvantages:•Non- anatomic technique.

Two- Flap Palatoplasty Bardach (1987) Arch of cleft will

provide the length needed for closure.

Combines intra- velar veloplasty

Intra- velar Veloplasty

Vomer Flaps

• Veau advocated approximation of Levator muscle sling.• Braithwaite was first to perform extensive muscle dissection• Cutting – division of tensor veli palatine tendon and

repositioning the muscle.• Cutting & Sommerland- “re repair” of levator muscle when

primary palatoplasty results in VPI

• Superior based flaps• Inferior based flaps- original

Schweckendiek Technique

2 staged approachEarlier soft palate later hard

palateSoft palate + lip= 4 to 6 monthsHard Palate= 4 -5 yearsEarlier hard palate closure is

proposed- 18- 24 months

Alveolar Bone Grafting Alveolar bone grafting is an integral part of repairing clefts

that involve the anterior maxilla. Establishing a bony union can help to prevent maxillary

segmental collapse, to close oronasal fistulas, and to encourage eruption of teeth.

Bone grafting in patients younger than 2 years is considered primary, and secondary grafting occurs afterward.

Graft material can be obtained from the hip, the ribs, the extremities, or the outer table of the skull.

The surgical procedure involves raising mucosal pedicles on either side of the maxillary defect.

Primary bone grafting- OUTDATED Secondary bone grafting- before the eruption of the canine.

Pierre Robin Sequence Micrognathia Glossoptosis Cleft palate 17% non- syndromic 34% Stickler; 11% VCF Positioning > tongue-

lip adhesion, floor of mouth release, tracheostomy or mandibular distraction osteogenesis

Velocardial Facial Syndrome

“Velum” – palate 1978 - cleft palate,

cardiac, facial appearance, learning disability

22q11 deletion 5-8% of CP children Possible motor

weakness, immune deficiency

Medially displaced carotids

Velopharyngeal Incompetence

Age 5-7

Speech pathologist – therapy, nasality measurements

Videofluoroscopy

Nasoendoscopy

Cinefluoroscopy

Posterior Pharyngeal Flap(Rosenthal) Restores the delicate balance b/w hypernasal resonance and airway obstruction. Hogan- size of flap & lateral port control based on pressure- flow studies of Warren. Shprintzen et al- tailor made pharyngeal flap based on the lateral wall motion. (Narrow, moderate, wide) Superior or inferior based. Transverse- Kapetansky- theoretically nerve preservation- not popular. Mitnick- VCF Syndrome- anomalies of vertebral & carotid arteries, close to C1. Risks – bleeding, dehiscence, infection, nasal obstruction.

Sphincter Pharyngoplasty Wilfred Hynes- Initially, salpingopharyngeus

muscle sutured transversely with mucosa.

Later SPP, with palatopharyngeus and sup. Constrictor.

Ortichoea

Jackson & Silverton

Complications of Surgery Intra- operative

Hemorrhage Flap Tearing Improper suturing

Post- Operative OSAS Airway obstruction Uncontrolled hemorrhage Fistula VPI

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