Chapter 45 - Introduction to Facial Clefts

OVERVIEW

• Rehabilitation of cleft lip & palate encompasses all "appealing features" of plastic surgery:
  • Understanding etiopathogenesis/pathomechanics
  • Being part of a multidisciplinary team
  • Continued treatment through growth & development ("the 4th dimension")
  • Technical procedures involving both skeletal and soft tissues

HISTORY

A. The Age of Empiricism

• Ancient times: cleft lip/palate considered evidence of an evil spirit - affected children left to die in the wilderness
• Boo-Chai (1966): reported a successful cleft lip closure in ~390 AD in China (surgeon unnamed)
• Yperman (1295-1351) - Flemish surgeon; wrote the first fully documented description of cleft lip and its repair:
  • Used a triangular needle + twisted wax suture
  • Reinforced with a long needle + figure-of-eight thread passed through the lip
  • A similar technique was still used by Pancoast in 1844
• Franco (1556): wrote that cleft lips are sometimes associated with cleft of the jaw or palate
• 1561: wrote about cleft palate causing nasal speech; suggested obturators made of gold/silver or lead plate
• Paré (1564): described palatal occlusion using gold/silver plates; called the device an "obturateur"; also coined the term "bec-de-lièvre" ("harelip")
• Tagliacozzi (1597): used mattress sutures through all layers of the lip - a departure from needle closure/figure-of-eight technique
• Desault and Bichat (1798): introduced head bandage compression of premaxilla for bilateral cases
• Jacques Houllier: first to propose direct suture of palatal perforations (Gurlt, 1898)

Palate Surgery Milestones

• Graefe and Roux (1817 & 1819): closed soft palate cleft with interrupted twine sutures - Roux's patient had "dramatic change of voice" immediately
• Dieffenbach (1828):
  • Closed hard palate by separating palatal mucosa from bone
  • Recommended lateral relaxing osteotomies (but didn't use these until 1828)
  • Technique practiced into the 20th century
• Warren (1828): mentioned early closure of soft palate to narrow wide hard palate clefts
• Schweckendiek (1962): repopularized Warren's approach - subject of debate due to associated speech problems
• Langenbeck (1859 & 1861): introduced subperiosteal dissection to elevate periosteum with palatal mucosa, forming bilateral mucoperiosteal flaps - still used in some centers today
• Veau (1931): attacked Langenbeck's technique for failing to achieve palatal lengthening; in 1936 (Converse), converted Langenbeck's bipedicle flaps into single pedicle flaps based on descending palatine vessels
• Wardill (1937), Kilner (1937), Peet (1961): modified Veau's technique - resulted in a "push-back" technique widely used today
• Cronin modification (1957): simultaneous lengthening of nasal surface of velum
• Furlow (1986): advocated double Z-plasty type of cleft palate closure

Lip Surgery Milestones

• Mirault (1844): introduced modern crossflap technique of lip closure
• Blair and Brown (1930): popularized Mirault's technique in the 20th century
• Hagedorn (1884): rectangular flap to prevent linear contracture - led to Le Mesurier (1949)
• Z-plasty techniques: used to relieve linear scar contraction tendency
• Tennison (1952): low triangular flap technique
• Millard (1958): high Z-plasty rotation flap (Chapter 52)
• McComb (1986): reawakened interest in nasal deformity correction at time of primary lip repair

Orthodontics and Bone Grafting

• McNeil (1954) and Burston (1958): advocated orthodontics in the 1st year of life for proper arch relationships
  • Proposed early alignment would aid normal maxillary development
  • Appliance removal before puberty often caused recurrence of arch deformity
• Primary bone grafting failed to gain permanent support:
  • Bone grafts do not independently hold collapsing arches
  • Primary bone grafts hinder rather than promote maxillary growth (Kling, 1964)
  • Cause crossbite malocclusion and pseudoprognathism
  • Pruzansky (1964): condemned unscientific use of primary bone grafting
  • Most surgeons now recommend bone grafting of dentoalveolar clefts at time of permanent canine eruption (Chapter 55)

B. The Scientific Approach

• 19th century: design of surgical procedures began to be based on precise anatomic studies
• Pancoast (1844): divided insertion of palatal muscles to prevent suture line tension
• Fergusson (1844-1845): noted most palatal repairs disrupted - conducted anatomic studies leading to operation dividing:
  • Levator veli palatini muscles
  • Posterior tonsillar pillars
  • Sometimes anterior tonsillar pillars
  • (Incisions provided relaxation to prevent lateral pull)

C. Victor Veau - Father of Modern Cleft Lip & Palate Surgery

• Spent many hours studying embryologic specimens
• Key surgical insight: paucity of muscle fibers in the medial aspect of unilateral cleft and prolabial segment of bilateral cleft lip
  • "The median border of the cleft lip is sterile"
  • Surgical directive: "Demand nothing from inner muscles; utilize the maximum of lateral aspect muscles which are fertile; sacrifice all mucosa of inner aspect but preserve all mucosa of lateral aspect"
• Principal cause of mediocre bilateral cleft lip results: absence of muscle in the prolabial segment
  • "One can hope for contour and shape approaching normal only if the lip contains muscle"
  • "The muscular sterility of the prolabial segment" - landmark concept
• On bilateral cleft: used the pressure from lip repair to recess the premaxilla
  • Concept: the newborn operation creates conditions for normal development; the vomer will grow and increase nasal projection if not altered
• Vomer surgery: if the vomer is sectioned (as some did), the "axial beam supporting evolution of the face" is removed - compared to "eliminating the violins from a Beethoven symphony"

Veau's Embryological Contribution (his greatest)

• Began embryology work after age 60
• Followed Fleischmann's hypothesis: cleft lip is caused by the persistence of the subnarial epithelial membrane (not "absence of coalescence of facial processes" as classical theory held)
• Studied 140 embryos (5-25 mm) to map the primary palate formation
• Identified proof in 22 mm (unilateral cleft) and 23.3 mm (bilateral cleft) embryos
• Collaborated with Professor Hochstetter and Professor Politzer in Vienna/Fischel's lab
• Published with Politzer: "The Primary Palate: Formation, Anomalies" - "a work of pure embryology"

Embryologic Formation (Veau/Fleischmann):

• (A) Floor of the nose between the integument and the nasopalatine canal (primary palate) - a very precocious embryonic structure (5mm, 2nd week), appears when mesoderm invades epithelial wall (7mm), definitively constituted when bone differentiation begins from undifferentiated mesenchyme (11mm, 5th week)
• (B) Hard and soft palate - the secondary palate; constituted when palatine processes achieve fusion (30mm, 12th week)
• Malformation in secondary palate = congenital cleft of the palate (most often)
• 6 out of 10 malformations of the primary palate (true cleft lip) are associated with malformation of secondary palate

Fleischmann's Gist:

D. Other Important Scientific Contributions (cited):

• Fára (1968): anatomy of cleft lip & palate
• Kriens (1969): anatomy of cleft palate and velopharyngeal region
• Lubker (1968): physiology of velopharyngeal mechanism
• Warren & Devereux (1966): aerodynamics of velopharyngeal region
• Isshiki, Honjow & Morimoto (1968): acoustical analysis of speech and velopharyngeal incompetence
• Avery (1962): embryologic studies
• Stenström & Öberg (1960): anatomy of cleft lip-nose deformity
• Latham (1970): analysis of columella anatomy
• Johnston: studies of etiopathogenesis of clefting (Chapter 48)

KEY EXAM POINTS TO REMEMBER ⭐

1. Yperman = first documented description + repair of cleft lip (13th-14th century)
2. Paré = coined "harelip" ("bec-de-lièvre") + first obturator
3. Roux (1819) = first successful soft palate closure (immediate voice change noted)
4. Langenbeck = mucoperiosteal flaps for palate (still used)
5. Millard vs. Tennison = two competing lip repair techniques (Millard = rotation-advancement; Tennison = triangular)
6. Furlow (1986) = double Z-plasty for palate
7. Veau = father of modern surgery; "muscular sterility of the prolabial segment" concept; embryological pioneer
8. Fleischmann's theory = persistence of subnarial epithelial membrane (NOT failure of fusion)
9. Primary bone grafting = ABANDONED due to growth restriction and pseudoprognathism
10. Secondary bone grafting at time of permanent canine eruption = accepted standard (Chapter 55)
11. Multidisciplinary team approach essential, especially for complex craniofacial anomalies
12. "4th dimension" = continued treatment during growth and development