Critical Element of Care

1 THROUGH 4 MONTHS:

 Monitor feeding and growth 

• Repair cleft lip 

• Begin/continue pre-surgical orthopedics if indicated

5 THROUGH 15 MONTHS:

• Monitor feeding, growth and development 

• Consider ear tubes/assess hearing 

• Repair cleft palate

16 THROUGH 24 MONTHS:

• Monitor ear tubes and hearing 

• Assess speech-language and development 

• Monitor development 

2 THROUGH 5 YEARS:

Assess speech for VPI; consider interventions 

• Monitor ear tubes and hearing 

• Revise lip/nose before school if needed 

• Assess child’s development, including language and psychological adjustment 

6 THROUGH 11 YEARS:

• Assess speech for VPI; consider interventions 

• Orthodontic interventions and alveolar bone grafting 

• Monitor school performance and psychological adjustment 

• Involve child in medical decision-making process 

12 THROUGH 21 YEARS:

• Jaw surgery, rhinoplasty if needed 

• Final orthodontics 

• Genetic counseling 

• Assess overall psychological adjustment 

• Review school issues/vocational plans

References:

American Association of Oral and Maxillofacial 

Surgeons. (1995). Standards of Care for Cleft Lip 

and Palate. Berkowitz, S. (1994). The Cleft Palate 

Story. Chicago: Quintessence Publishing Co., Inc. 

American Cleft Palate-Craniofacial Association. (1993) Parameters for the evaluation and treatment 

of patients with cleft lip/palate or other craniofacial anomalies. Cleft Palate-Craniofacial Journal, 30, 

(Suppl. 1). 

American Cleft Palate-Craniofacial Association. (2010). Standards for Approval of Cleft Palate and 

Craniofacial Teams-Commission on Approval of Teams. www.acpa-cpf.org/Standards/Standards_

2010.pdf

American Cleft Palate-Craniofacial Association. (1996). Team Standards Self-Assessment Instrument. Berkowitz, S. (1994). The Cleft Palate Story. Chicago: Quintessence Publishing Co., Inc. 

Gorlin, R.G. (2001). Syndromes of the Head and Neck. [4th edition.] New York: Oxford University Press. Journal of Ultrasound Medicine, 10, 577. (1991) Losee, J & Kirschner, R. (2008). Comprehensive Cleft Care. McGraw-Hill Professional. 

Moller K.T. & Starr, C.D. (Eds.). (1993). Cleft Palate:Interdisciplinary Issues and Treatment. Austin, TX: Pro-ed. 

Office of Maternal and Child Health. U.S. Department of Health and Human Services. (1987). Surgeon General’s Report: Children with Special Health Care Needs. 

Shaw, William C., et al. (1992). A six-center international study of treatment outcome in patients with clefts of the lip and palate: parts 1-5. Cleft Palate-Craniofacial Journal, 29, 393-418. Sphrintzen, R.J. & Bardach, J. (1995). Cleft Palate Speech Management: A Multidisciplinary Approach. St. Louis, MO: Mosby. 

U.S. Department of Health and Human Services. Oral Health in America: A Report of the Surgeon General.

Cleft Lip and Palate Outline

Introduction

• Approximately 15 types of orofacial clefting with cleft lip and palate being the
most common
• Most common congenital malformations of the head and neck
• 1 in 700 live births (1/1000 in the US)
• In the US, approximately 7500 babies are born with orofacial clefts per year
with an average lifetime medical cost of $100,000 (750 million per year)
• Associated problems include otological disease, speech and language
problems, dental deformities, and psychosocial issues
• Best managed with a multidisciplinary approach (medical and surgical)

Prevalence
• Ethnic groups(CL+/-P)
– Highest rate
• Native American and Asians ( 2/1000 live births)
– Intermediate rate
• European descendants (1/1000 live births)
– Lowest rate
• African populations (1/2500 live births)
• No difference between ethnic groups for cleft palate only (1/2000 live birth)
• Gender
– 2:1 – M:F ratio – cleft lip +/- palate
– 1:2 – M:F ratio – cleft palate only (late closure of palatine shelves)

Causes
• Folklore explanations
– Aztecs – eclipses occurred because a bite had been taken out of the
moon
– Prevented with an obsidian knife above the pregnant abdomen
– Modern Mexico – prevented with keys and safety pins

Early Chinese

– Eating rabbit – “hare lip”

– Bad karma or wrongdoings

• Philippines

– Force to the fetal face

• Familial or “In the blood”

Familial

• 2 unaffected parents with 1 child 

affected

– Risk for future children:

• 4.4% for CL+/- palate

• 2.5% for CP only

• 1 parent affected

– Risk for future children

• 3.2% for CL+/- palate

• 6.8% for CP only

• 1 parent affected with 1 child affected

– Risk for future children

• 15.8% for CL+/- palate

• 14.9% for CP only

Etiology

• Majority of orofacial clefts are 

nonsyndromic

– 70% of CL +/- palate

– 50% of CP only

• Nonsyndromic clefts

– multifactorial

– Clusters in families but not mendelian

– Palate development complex process 

with several proteins, growth factors, and 

transcription factors involved

• IRF-6, TGF –B2, TGF-alpha

– Any disturbance in the process can result 

in clefting

Syndromic clefts:

– Associated with over 300 

syndromes

• Van der Woude syndrome – the most 

common

– Autosomal dominant

– Lower lip pits

– Teratogen exposure

• Ethanol, thalidomide, phenytoin

– Environmental factors

• Amniotic banding, maternal 

diabetes, maternal folate deficiency

Anatomy

• Orbicularis oris

• Vermillion (wet/dry border)

• Cupid’s bow

– Along the upper vermillion 

cutaneous border (white 

roll), two midline 

elevations form the bow

• Philtrum

– Philtral columns and 

dimple

• Tubercle

Primary Palate

• Medial nasal prominences 

fuse to form the primary 

palate

• Consists of maxillary 

alveolar arch with 4 

incisors and the hard 

palate anterior to the 

incisive foramen

• Primary palate forms 

before the secondary 

palate begins formation

Secondary Palate

• During the 6th week

– Shelf-like outgrowths from the bilateral maxillary processes, grow vertically 

down on both sides of the tongue 

• During the 7th week

– The tongue moves inferiorly and the palatal shelves migrate to a horizontal 

position above the tongue

• Palatal fusion occurs in an anterior to posterior direction and completes 

with uvular fusion (1 week later in females)

Cleft Lip and Palate Formation

• Disruptions at any stage of the developmental process can result in 

clefts

– Timing

– Positioning 

• Pierre Robin Sequence – micrognathia

– Wide U shaped cleft palate

– Failure of fusion of the palatal shelves result in clefts of the secondary 

palate

Unilateral Cleft Lip

• Incomplete

– Muscle fibers of the orbicularis oris are often 

intact but hypoplastic

– Varying degrees of clefting

• Complete

– Orbicularis oris inserts at the columella medially 

and ala laterally on the cleft side

– Columella is displaced to the normal side

– Nasal ala on the side of the cleft is displaced 

laterally, inferiorly, and posteriorly

– Nasal tip is deflected towards the noncleft side

• Alveolus may or may not be involved

Bilateral Cleft Lip

• Orbicularis oris attaches at the lateral cleft margins 

bilaterally at the nasal ala

• Premaxilla protrusion

• Symmetrical nasal deformities

– Laterally displaced ala – widely flared

– Extremely short columella

References

Arosarena, Oneida A. "Cleft lip and palate." Otolaryngologic Clinics of North America 40.1 (2007): 27-60.

2. Cummings, Charles W., and Paul W. Flint. Cummings Otolaryngology Head & Neck Surgery. Philadelphia: Mosby Elsevier, 2010. Print.

3. Dixon, Michael J., et al. "Cleft lip and palate: understanding genetic and environmental influences." Nature Reviews Genetics 12.3 

(2011): 167-178.

4. Dyleski, R. A., D. M. Crockett, and R. W. Seibert. "Cleft lip and palate: evaluation and treatment of the primary deformity." Head and 

neck surgery—otolaryngology, 4th edn. Lippincott Williams & Wilkins, Philadelphia (2006): 1317-1335.

5. Grayson, Barry H., and Deirdre Maull. "Nasoalveolar Molding for Infants Born with Clefts of the Lip, Alveolus, and Palate." Seminars in 

Plastic Surgery. Vol. 19. No. 4. Thieme Medical Publishers, 2005.

6. Liau, James Y., A. Michael Sadove, and John A. van Aalst. "An evidence-based approach to cleft palate repair." Plastic and 

reconstructive surgery 126.6 (2010): 2216-2221.

7. Marazita, Mary L. "The evolution of human genetic studies of cleft lip and cleft palate." Annual Review of Genomics and Human 

Genetics 13 (2012): 263-283.

8. Moore, Keith L., and A. M. R. Agur. Essential Clinical Anatomy. Philadelpia, PA: Lippincott Williams & Wilkins, US, 2006. Print.

9. Robin, Nathaniel H., et al. "The multidisciplinary evaluation and management of cleft lip and palate." Southern medical journal 99.10 

(2006): 1111-1120.

10. Szabo, C., et al. "Treatment of persistent middle ear effusion in cleft palate patients." International journal of pediatric 

otorhinolaryngology 74.8 (2010): 874-877.

11. van Aalst, John A., Kamal Kumar Kolappa, and Michael Sadove. "MOC-PS (SM) CME Article: Nonsyndromic Cleft Palate." Plastic and 

reconstructive surgery 121.1S (2008): 1-14.

Presurgical Nasoalveolar Molding in Infants with Cleft Lip and Palate

Presurgical infant orthopedics has been employed since the 1950s as an adjunctive neonatal therapy for the correction of cleft lip and palate. In this paper, we present a paradigm shift from the traditional methods of presurgical infant orthopedics. Some of the problems that the traditional approach falled to address include the deformity of the nasal cartilages in unilateral as well as bilateral clefts of the lip and palate and the deficiency of columella tissue in infants with bilateral clefts. The nasoalveolar molding (NAM) technique we describe uses acrylic nasal stents attached to the vestibular shield of an oral molding plate to mold the nasal alar cartilages into normal form and position during the neonatal period. This technique takes advantage of the malleability of immature cartilage and its ability to maintain a permanent correction of its form. In addition, we demonstrate the ability to nonsurgically construct the columella through the application of tissue expansion principles. This construction is performed by gradual elongation of the nasal stents and the application of tissue-expanding elastic forces that are applied to the prolabium. Use of the NAM technique has eliminated surgical columella reconstruction and the resultant scar tissue from the standard of care in this cleft palate center.



A heavy-bodied silicone impression material is used to take the initial impression as soon after birth as possible, when the cartilage is plastic and moldable. In case of an airway emergency, the surgeon is always present to help with the impression. The infant is held upside down by the surgeon, and the impression tray is inserted into the oral cavity. The tray is seated until impression material is observed just beginning to extrude past its posterior border. The infant is kept in the inverted position to keep the tongue forward and to allow fluids to drain out of the oral cavity. Once the impression material is set, the tray is removed, and the mouth is examined for residual impression material that may be left behind. A cast or model of the alveolar anatomy is made by filling the impression with a dense plaster material (dental stone). The molding plate is fabricated on the dental stone model. It is made of hard clear acrylic and is lined with a thin layer of soft denture material. Care is taken to reduce the border of the plate in the area of the labial frenum attachments and other areas that may be likely to ulcerate. Parents are instructed to keep the plate in full time and to take it out for cleaning as needed, at least once a day. Initially, it may take longer to feed the infant with the plate in place, but the child quickly adjusts and parents report that the infant soon will not eat without it. The appliance is secured extraorally to the cheeks and bilaterally by surgical tapes, which have an orthodontic elastic band at one end. The elastics loop over a retention arm extending from the anterior flange of the plate. The retention arm is positioned approximately 40 degrees down from the horizontal to achieve proper activation and to prevent unseating of the appliance from the palate. The tapes are changed once a day.

The benefits of NAM are numerous. In the short term, the tissues are well aligned before primary lip and nose repair, which enables the surgeon to achieve a better and more predictable outcome with less scar tissue formation. In the long term, studies indicate that the change in nasal shape is stable with less scar tissue and better lip and nasal form. This improvement reduces the number of surgical revisions for excessive scar tissue, oronasal fistulas, and nasal and labial deformities. With the alveolar segments in a better position and increased bone bridges across the cleft, the adult teeth have a better chance of erupting in a good position with adequate periodontal support.

Santiago et al found that 60% of patients who underwent NAM and GPP did not require secondary bone grafting. Sato et al found that, in the remaining 40% who did need a bone graft, there was more bone remaining in the graft site than in patients who did not previously undergo GPP. This was explained by the presence of bone bridges in the graft site resulting from the primary GPP. Henkel and Gundlach found that, in 68% to 73% of patients in whom a Millard-type GPP was performed, a secondary bone graft was not required. Fewer surgeries result in substantial cost savings for families




and insurance companies.Another important benefit of NAM is the opportunity for the parents to take part actively in the habilitation of their child.




NAM has evolved over the past decade into its present form through contributions made by practicing clinicians and parents. This method of treatment requires attention to detail with appliance adjustments that are at times less than a millimeter in dimension.




Clinical skills in NAM develop over time. Efficiency in treating patients increases as these clinical skills improve, and these skills may be advanced by the training of a dental assistant or laboratory technician to make adjustments to the molding plate under direct supervision of the practicing clinician. Since the initiation of NAM and the associated surgical technique, there has been a significant difference in the outcome of primary surgical cleft repair.

REFERENCES

McComb H. Primary correction of unilateral cleft lip nasal deformity: a 10-year review. Plast Reconstr Surg. 1985;75:791–799. [PubMed]
Latham R. Development and structure of the premaxillary deformity in bilateral cleft lip and palate. Br J Plast Surg. 1973;26:1–11. [PubMed]
Millard D. Cleft Craft: The Evolution of Its Surgery. Bilateral and Rare Deformities. Boston: Little, Brown; 1977.
Berkowitz S. A comparison of treatment results in complete bilateral cleft lip and palate using a conservative approach versus Millard-Latham PSOT procedure. Semin Orthod. 1996;2:169–184.[PubMed]
McNeil C. Orthodontic procedures in the treatment of congenital cleft palate. Dental Record.1950;70:126–132. [PubMed]
Georgiade N, Latham R. Maxillary arch alignment in the bilateral cleft lip and palate infant, using pinned coaxial screw appliance. Plast Reconstr Surg. 1975;56:52–60. [PubMed]
Hotz M, Perko M, Gnoinski W. Early orthopaedic stabilization of the praemaxilla in complete bilateral cleft lip and palate in combination with the Celesnik lip repair. Scand J Plast Reconstr Surg Hand Surg. 1987;21:45–51. [PubMed]
Grayson B, Cutting C, Wood R. Preoperative columella lengthening in bilateral cleft lip and palate. Plast Reconstr Surg. 1993;92:1422–1423. [PubMed]
Matsuo K, Hirose T, Tomono T, et al. Nonsurgical correction of congenital auricular deformities in the early neonate: a preliminary report. Plast Reconstr Surg. 1984;73:38–51. [PubMed]
Matsuo K, Hirose T. Nonsurgical correction of cleft lip nasal deformity in the early neonate. Ann Acad Med Singapore. 1988;17:358–365. [PubMed]
Matsuo K, Hirose T, Otagiri T, Norose N. Repair of cleft lip with nonsurgical correction of nasal deformity in the early neonatal period. Plast Reconstr Surg. 1989;83:25–31. [PubMed]
Matsuo K, Hirose T. Preoperative non-surgical overcorrection of cleft lip nasal deformity. Br J Plast Surg. 1991;44:5–11. [PubMed]
Grayson B, Santiago P. In: Aston SJ, Beasley RW, Thorne CH, editor. Grabb and Smith's Plastic Surgery. 5th ed. Philadelphia: Lippincott-Raven; 1997. Presurgical orthopedics for cleft lip and palate. pp. 237–244.
Grayson B, Santiago P, Brecht L, Cutting C. Presurgical nasoalveolar molding in infants with cleft lip and palate. Cleft Palate Craniofac J. 1999;36:486–498. [PubMed]
Grayson B, Cutting C. Presurgical nasoalveolar orthopedic molding in primary correction of the nose, lip, and alveolus of infants born with unilateral and bilateral clefts. Cleft Palate Craniofac J.2001;38:193–198. [PubMed]
Cutting C B, Bardach J, Pang R. A comparative study of the skin envelope of the unilateral cleft lip nose subsequent to rotation-advancement and triangular flap lip repairs. Plast Reconstr Surg.1989;84:409–417. discussion 418–419. [PubMed]
Cutting C, Grayson B. The prolabial unwinding flap method for one-stage repair of bilateral cleft lip, nose, and alveolus. Plast Reconstr Surg. 1993;91:37–47. [PubMed]
Cutting C. In: Rees T, La Trenta G, editor. Aesthetic Plastic Surgery. Philadelphia: Saunders; 1994. Cleft nasal reconstruction. pp. 497–532.
Cutting C, Grayson B, Brecht L, Santiago P, Wood R, Kwon S. Presurgical columellar elongation and primary retrograde nasal reconstruction in one-stage bilateral cleft lip and nose repair. Plast Reconstr Surg. 1998;101:630–639. [PubMed]
Millard D, Jr, Latham R. Improved primary surgical and dental treatment of clefts. Plast Reconstr Surg. 1990;86:856–871. [PubMed]
Santiago P, Grayson B, Cutting C, Gianoutsos M, Brecht L, Kwon S. Reduced need for alveolar bone grafting by presurgical orthopedics and primary gingivoperiosteoplasty. Cleft Palate Craniofac J. 1998;35:77–80. [PubMed]
Wood R, Grayson B, Cutting C. Gingivoperiosteoplasty and midfacial growth. Cleft Palate Craniofac J. 1997;34:17–20. [PubMed]
Lee C, Grayson B, Cutting C. Unilateral cleft lip and palate patients following gingivoperiosteoplasty. San Diego: American Association of Orthodontics and Dentofacial Orthopedics; 1999. In: Program and Abstracts of the Annual Session of the American Association of Orthodontics and Dentofacial Orthopedics.
Maull D, Grayson B, Cutting C, et al. Long-term effects of nasoalveolar molding on three-dimensional nasal shape in unilateral clefts. Cleft Palate Craniofac J. 1999;36:391–397. [PubMed]
Lee C, Grayson B, Cutting C. The need for surgical columella lengthening and nasal width revision before the age of bone grafting in patients with bilateral cleft lip following presurgical nasal molding and columella lengthening. Scottsdale: American Cleft Palate–Craniofacial Association; 1999. In: Program and Abstracts of the 56th Annual Session of the American Cleft Palate–Craniofacial Association.
Sato Y, Grayson B, Barillas I, Cutting C. The effect of gingivoperiosteoplasty on the outcome of secondary alveolar bone graft. Seattle: American Cleft Palate–Craniofacial Association; 2002. In: Program and Abstracts of the 59th Annual Session of the American Cleft Palate–Craniofacial Association. p. 51.
Henkel K, Gundlach K. Millard gingivoperiosteoplasty: an alternative to osteoplasty of alveolar clefts. Mund Kiefer Gesichtschir. 2002;6:261–265. [PubMed]
Pfeifer T, Grayson B, Cutting C. Nasoalveolar molding and gingivoperiosteoplasty versus alveolar bone graft: an outcome analysis of costs in the treatment of unilateral cleft alveolus. Cleft Palate Craniofac J. 2002;39:26–29. [PubMed]

Vertical Maxillary Growth After 2 Different Surgical Protocols in Unilateral Cleft Lip and Palate Patients

The aim of the mention article was to compare vertical maxillofacial growth in patients born with unilateral cleft lip and palate (UCLP) who were treated using two different surgical protocols.

Cleft lip and palate (CLP) is one of the most common congenital anomalies. Treatment protocols for management of children with CLP differ markedly between cleft teams (Mossey et al., 2009). Between 1965 and 1974, the protocol used by the cleft team in Gothenburg, Sweden, included hard palate closure using a cranially based vomer flap followed by a Wardill-Kilner (W-K) push-back palatoplasty. This technique led to poor midfacial growth and occlusion (Friede and Johanson, 1977). Based on follow-up studies, the protocol was changed in 1975 by introducing a delayed hard palate closure technique (DHPC). This method included closure of the soft palate with a posteriorly based vomer flap in the first year of life. Closure of the hard palate was delayed until the stage of mixed dentition. This DHPC technique showed significantly better long-term midfacial growth and occlusion (Friede et al., 1980, 2012; Friede, 1998), with favorable speech development (Lohmander-Agerskov, 1998; Lohmander et al., 2012).

Graber (1949) pioneered the research on factors influencing maxillary development in CLP patients and stated that cleft surgery had a detrimental effect on maxillary growth (Graber, 1949). Restricted maxillary growth has been a constant finding in studies evaluating CLP patients treated according to different surgical protocols (Ross, 1987; Semb and Shaw, 1998; Khanna et al., 2012). Most previous work has been focused on craniofacial growth in the sagittal and transverse dimensions (Mars et al., 1992; Molsted et al., 1992; Lisson et al., 1999).

The vertical maxillary growth restriction has been shown to be a common finding in operated cleft patients, and it has also been shown to vary between different surgical techniques and their timing (Ross, 1987). Moreover, the growth restriction has been found to differ between anterior or posterior maxillary dimensions and to change the maxillary inclination angle (Swennen et al., 2002). Reduced anterior vertical maxillary growth can be observed clinically as reduced overbite (Ross, 1987; Lisson et al., 2005), and reduced posterior vertical maxillary growth has been suggested to affect speech (Stellzig-Eisenhauer, 2001).


The effect of surgery on vertical maxillary growth in patients with CLP is less well understood, and further investigation of the effect of different surgical protocols on these dimensions is still needed. The aim of the present study was to compare how vertical maxillary growth is affected by W-K with cranially based vomer flap and by the Gothenburg DHPC with posteriorly based vomer flap.

The study was conducted on lateral cephalometric radiographs taken at 10 years of age from 92 consecutive Caucasian patients born with unilateral cleft lip and palate (UCLP). The patients underwent surgical treatment at the Department of Plastic Surgery, Sahlgrenska University Hospital, Gothenburg, Sweden.

Exclusion criteria were: secondary palatal surgical procedure, syndromic clefts, craniofacial or systemic anomalies, and presence of Simonart's band of more than 0.5 mm. Fistula closure was not regarded as an exclusion criterion.

The patients were divided into two groups according to the surgical protocol used.

The W-K group consisted of 46 consecutive patients born between 1965 and 1974

This study revealed significantly higher values for anterior upper facial height, anterior maxillary height, and overbite in the Gothenburg DHPC group than in the W-K group However, while the values for anterior upper facial height seemed to approach noncleft reference values in the DHPC group more than in the W-K group, the maxillary inclination showed the opposite tendency (Thilander et al., 2005).

The findings of a more normal anterior upper facial height, anterior maxillary height, and overbite in the DHPC than in the W-K group are in accordance with previous studies using the DHPC protocol, which showed favorable maxillary growth for all dimensions investigated compared to the W-K protocol (Friede and Johanson, 1977; Friede et al., 1980, 2012; Bakri et al., 2012).

The normal growth of the maxillary complex has been extensively studied; regarding vertical dimension, the maxilla is relocated downward through appositional growth in the hard palate and the alveolar process. The bony surfaces of the maxilla are selectively resorptive or depository to maintain the general shape of the midface during growth (Bjork and Skieller, 1974). Normal midfacial growth also involves displacement of the maxilla forward and downward in relation to the vomer. Through studies of CLP patients, the displacement has been documented to occur in the vomeropremaxillary suture and mainly during the first year of life (Friede, 1977,1978).

The vertical dimension of the maxilla is close to normal in unoperated cleft patients, indicating that surgery is the factor mainly responsible for growth restriction (Lambrecht et al., 2000). Thus, in the W-K technique, extensively denuded palatal bone results in scar tissue that negatively affects the maxillary growth in all dimensions (Ross, 1970; Ishikawa et al., 1998). The cranially based vomer flap in this technique is suggested to result in bone formation across the cleft, in addition to disturbing the vomeropremaxillary suture, resulting in increased restriction of maxillary growth (Prydso et al., 1974; Friede and Johanson, 1977; Friede and Lilja, 1994). However, the DHPC technique in the present study included early soft palate closure, and the remaining cleft in the hard palate has been shown to narrow markedly until the hard palate closure, instead reducing cicatrization from hard palate repair (Owman-Moll et al., 1998; Friede and Enemark, 2001). Moreover, whatever technique is used, operating on the cleft palate at later age has also been shown to reduce the restriction of the maxillary growth (Bardach et al., 1984; Xu et al., 2012). The DHPC techniques showed cephalometric values that were lower than in noncleft individuals, indicating that restricted vertical growth still occurs (Thilander et al., 2005). This is in agreement with the statement of Ross (1970) that repair of the cleft palate by any surgical technique will result in inhibition of the growth of the maxillary complex.






In conclusion, the present study that the Gothenburg DHPC protocol in patients with complete UCLP results in more normal anterior maxillary vertical growth and overbite, and therefore increased maxillary inclination at 10 years of age.


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Lilja J, Mars M, Elander A, Enocson L, Hagberg C, Worrell E, Batra P, Friede H. Analysis of dental arch relationships in Swedish unilateral cleft lip and palate subjects: 20-year longitudinal consecutive series treated with delayed hard palate closure. Cleft Palate Craniofac J. 2006;43:606–611. [Abstract] [Medline]


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New neonatal classification of unilateral cleft lip and palate

Objectives : To bring a neonatal classification system of unilateral cleft lip and palate and to correlate this classification with the distribution of the permanent lateral incisor and maxillary growth. Design : Retrospective with longitudinal follow-up. Setting : Tertiary. Patients : A total of 112 individuals with treated unilateral cleft lip and palate and 30 controls. Main Outcome Measures : Unilateral cleft lip and palate neonatal casts were classified anatomically in four categories, in which Class 1 corresponds to a maxillary arch with a narrow alveolar cleft; Class 2 corresponds to a balanced form; Class 3 corresponds to a wide cleft and short maxilla; and Class 4 corresponds to a wide cleft and long maxilla. The classification was correlated with the distribution of the permanent lateral incisor. Maxillary growth was evaluated using a cephalometric analysis after the age of 10 years. Results : Clinical classification of unilateral cleft lip and palate found 10 cases of Class 1 (8.9%), 34 cases of Class 2 (30.4%), 46 cases of Class 3 (41.1%), and 22 cases of Class 4 (19.6%). The permanent lateral incisor was most often present in narrower clefts (Classes 1 and 2); whereas, large clefts (Classes 3 and 4) were relatively more frequently associated with an agenesis of the permanent lateral incisor (P =.019). Maxillary growth impairment was most severe in Class 3, with a mean sella-nasion-A point angle at 71.9° ± 4.6° (P <.001). Conclusions : Using the cleft width, arch form, and shape of the nasal septum, unilateral cleft lip and palate can be classified into four different classes at birth, which can all give information about permanent lateral incisor agenesis and maxillary growth. Referencias Jean-Charles Doucet, Christian Delestan, Pedro Montoya, Lucia Matei, Michèle Bigorre, Christian Herlin, Caroline Baümler, Jean-Pierre Daures, and Guillaume Captier (2014) New Neonatal Classification of Unilateral Cleft Lip and Palate Part 2: To Predict Permanent Lateral Incisor Agenesis and Maxillary Growth. The Cleft Palate-Craniofacial Journal: September 2014, Vol. 51, No. 5, pp. 533-539.

Botulinum Toxin to Improve Results in Cleft Lip Repair

A scar represents dermal fibrous replacement tissue and results from a wound that has healed by resolution rather than regeneration. Undesirable scars, such as hypertrophic or keloid scars, occur most frequently over the anterior chest, shoulders, scapular area, lower abdomen, and suprapubic region.6 The skin of the upper lip is also at risk of hypertrophic scars because of the repetitive movements of the underlying orbicularis oris muscle during speech, eating and drinking, and facial expression that transmit tension forces that distract and widen the healing wound. The incidence of hypertrophic upper lip scars ranges between 12 and 27 percent in the mixed population but increases when controlled for ethnicity to 32.2 percent in Hispanics and 36.3 percent in Asians.1,7,8 In addition, primary cheiloplasty scars are more conspicuous because they do not align with the relaxed skin tension lines of the face.

A patient’s cheiloplasty scar is a lifelong marker that he or she was born with a cleft lip. The position of a cheiloplasty scar makes it difficult to conceal, and its conspicuity can inflict significant psychological impact on its bearer. According to a structured semiquantitative survey of 97 elective/aesthetic surgical patients, 91 percent would value even a small improvement in the quality of their scar.9 Patients were dissatisfied with surgical scars irrespective of their sex, age, ethnicity, or geographic location, and wished their scars were less noticeable. With current cheiloplasty techniques, the anatomy of the lip can be restored adequately.10 As a result of charitable funding from the Noordhoff Craniofacial Foundation, almost all patients presenting to our center with cleft lip are able to undergo cheiloplasty at approximately 3 months of age. Our own survey of patients treated at our center revealed that more than 90 percent would regard even a small improvement in their cheiloplasty scar to be worthwhile (unpublished data). This is despite our use of several established strategies to attempt to optimize cheiloplasty scars in our center.

First, we repair the orbicularis oris muscle so that it bears the majority of the tension of the wound. This causes the overlying skin to become slightly redundant (and therefore tension-free), which allows enough skin for philtral column reconstruction on the cleft side. Second, we strictly use wound taping to minimize tension across the lip. These tapes span across the upper lip from cheek to cheek and are placed purposefully so that the nasolabial folds are deepened (and thus the skin is redundant) and the upper lip protrudes. Adhesive tape has been used by plastic surgeons for decades, with or without sutures, to reduce wound tension to prevent hypertrophic scar formation.11–13Third, we strictly use silicone gel sheeting over the scar when the baby is asleep. Topical silicone preparations have been recommended in the management of hypertrophic and keloid scars and are known to reduce the recurrence of hypertrophy following scar revision surgery in patients at higher risk of hypertrophic scarring.14,15 The benefit of topical silicone is less clear in patients who lack a history of abnormal scarring.15 Although there remains a lack of consensus as to whether topical silicone gel sheets should be applied routinely for upper lip cheiloplasty scars, this has remained routine practice at our center for many years.10 The compliance with lip taping and silicone sheets is very high in our population. The parents are well supported in this regard both by each other (many, if not most, of the parents of our children who have been operated on are routinely in contact on social media groups to encourage compliance) and by our Noordhoff Craniofacial Foundation social workers. The use of taping and topical silicone and the method of lip repair were all constant in this study; the only variable, which was blinded, was whether the vial for injection contained botulinum toxin or vehicle alone.

The contraction of the muscles of facial expression cause increased skin tension and the accentuation of dynamic rhytides that are often considered cosmetically undesirable. Carruthers et al. first realized that patients treated with botulinum toxin chemodenervation for blepharospasm experienced concurrent improvements in their dynamic glabella rhytides, sparking its widespread use in aesthetic practices ever since. Similarly, Gassner et al. injected botulinum toxin around frontal wounds to chemoimmobilize underlying musculature and therefore reduce wound tension during scar formation, with resultant improvements in cosmetic outcomes in an animal study.16Several human studies have since demonstrated that the injection of botulinum toxin can improve facial scars.17–20 Tollefson et al. first published the use of botulinum toxin to immobilize the upper lip for cheiloplasty in three cleft infants aged 3 to 6 months.2 However, it was unknown whether the botulinum toxin was responsible for the satisfactory aesthetic results that they reported. Galárraga injected the upper lips of five children undergoing cheiloplasty intraoperatively.3Electromyographic tracings proved that significant reductions in orbicularis oris muscular activity were resulting from the botulinum toxin treatment. Again, although the author hypothesized that this chemoimmobilization might benefit scarring, no direct evidence for the benefit of botulinum toxin was provided.

We investigated botulinum toxin injection as an additional potential intervention that might further improve our cheiloplasty scars over and above our established protocolized techniques. To our knowledge, this current study represents the first objective and subjective evaluation of scars following botulinum toxin injection into the upper lip during cheiloplasty. Although there was no significant difference in Vancouver Scar Scales between the experimental and control groups, photographic visual analogue scale and photographic measurements both revealed consistently better appearing and narrower scars as a result of botulinum toxin treatment compared with vehicle controls


No complications (i.e., infection, bleeding, wound dehiscence, oral incontinence, or feeding dysfunction) were found. Pascual-Pascual and Pascual-Castroviejo studied the safety of botulinum toxin type A in children younger than 2 years. The dosage used for obstetric brachial plexus palsy and cerebral palsy of 6.55 units/kg, which is much higher than the dosage required for chemodenervation of the orbicularis oris muscle, was considered safe.21 Moreover, neither of the previous studies that used botulinum toxin type A injection into the orbicularis oris muscle reported complications such as feeding difficulties.

CONCLUSIONS


These results indicate that our established method of upper lip repair and mechanical immobilization by taping is controlling well against wound tension in infants caused by orbicularis oris but that botulinum toxin has an additional beneficial effect on scar width that, although small, is subjectively noticeable. According to the Vancouver Scar Scale, however, botulinum toxin provided no additional benefits for scar pigmentation, vascularity, pliability, or height of cheiloplasty scars.

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8. Onizuka T, Ichinose M, Hosaka Y, Usui Y, Jinnai T. The contour lines of the upper lip and a revised method of cleft lip repair. Ann Plast Surg. 1991;27:238–252

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16. Gassner HG, Sherris DA, Otley CC.. Treatment of facial wounds with botulinum toxin A improves cosmetic outcome in primates. Plast Reconstr Surg. 2000;105:1948–1953; discussion 1954–1955

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Cleft Lip & Cleft Palate

Cleft lip, cleft palate, and combinations of the two are the most common congenital anomalies of the head and neck. The incidence of facial clefts has been reported to be 1 in every 650–750 live births, making this deformity second only to clubfoot in frequency as a reported birth defect.

The cleft may involve the floor of the nostril and lip on one or both sides and may extend through the alveolus, the hard palate, and the entire soft palate. A useful classification based on embryologic and anatomic aspects divides the structures into the primary and the secondary palate. The dividing point between the primary palate anteriorly and the secondary palate posteriorly is the incisive foramen. Clefts can thus be classified as partial or complete clefts of the primary or secondary palate (or both) in various combinations. The most common clefts are left unilateral complete clefts of the primary and secondary palate and partial midline clefts of the secondary palate, involving the soft palate and part of the hard palate.

Most infants with cleft palate present some feeding difficulties, and breast-feeding may be impossible. As a rule, enlarging the openings in an artificial nipple or using a syringe with a soft rubber feeding tube will solve difficulties in sucking. Feeding in the upright position helps prevent oronasal reflux or aspiration. Severe feeding and breathing problems and recurrent aspiration are seen in Pierre Robin sequence, in which the palatal cleft is associated with a receding lower jaw and posterior and cephalic displacement of the tongue, obstructing the naso-oropharyngeal airway. This is a medical emergency and is a cause of sudden infant death syndrome (SIDS). Nonsurgical treatment includes pulling the tongue forward with an instrument and laying the baby prone with a towel under the chest to let the mandible and tongue drop forward. Insertion of a small (No. 8) nasogastric tube into the pharynx may temporarily prevent respiratory distress and may be used to supplement the baby’s feedings. Placement of an acrylic obturator or appliance has proved quite successful in alleviating the breathing difficulties by bringing the tongue down and permitting a better nasal airway. Several surgical procedures that bring the tongue and mandible forward have been described but should be employed only when conservative measures have been tried without success. Recently, the use of distraction of the mandible has shown some beneficial effects. However, it should be done with great caution in the neonate.

Treatment

Surgical repair of cleft lip is not considered an emergency. The optimal time for operation can be described as the widely accepted “rule of 10.” This includes body weight of 10 lb (4.5 kg) or more and a hemoglobin of 10 g/dL or more. This is usually at some time after the 10th week of life. In most cases, closure of the lip will mold distortions of the cleft alveolus into a satisfactory contour. In occasional cases in which there is marked distortion of the alveolus, such as in severe bilateral clefts with marked protrusion of the premaxilla, preliminary maxillary orthodontic treatment may be indicated. This may involve the use of carefully crafted appliances or simple constant pressure by use of an elastic band.

General endotracheal anesthesia via an orally placed endotracheal tube is the anesthetic technique of choice. A variety of techniques for repair of unilateral clefts have evolved over many years. Earlier procedures ignored anatomic landmarks and resulted in a characteristic “repaired harelip” look. The Millard rotation advancement operation that is now commonly used for repair employs an incision in the medial side of the cleft to allow the Cupid’s bow of the lip to be rotated down to a normal position. The resulting gap in the medial side of the cleft is filled by advancing a flap from the lateral side. This principle can be varied in placement of the incisions and results in most cases in a symmetric lip with normally placed landmarks. Bilateral clefts, because of greater deficiency of tissue, present more challenging technical problems. Maximum preservation of available tissue is the underlying principle, and most surgeons prefer approximation of the central and lateral lip elements in a straight line closure, rolling up the vermilion border of the lip (Manchester repair).

Secondary revisions are frequently necessary in the older child with a repaired cleft lip. A constant associated deformity in patients with cleft lip is distortion of the soft tissue and cartilage structures of the ala and dome of the nose. These patients often present with deficiency of growth of the structures of the mid face. This has been attributed to intrinsic growth disturbances and to external pressures from the lip and palate repairs. Some correction of these deformities, especially of the nose, can be done at the initial lip operation. More definitive correction is done after the cartilage and bone growth is more complete. These may include scar revisions and rearrangement of the cartilage structure of the nose. Recent approaches involve degloving of the nasal skin envelope with complete exposure of the abnormal cartilage framework. These are then rearranged in proper position with or without additional grafts. Maxillary osteotomies (Le Fort I with advancement) will substantially correct the midfacial depression. A tight upper lip due to severe tissue deficiency can be corrected by a two-stage transfer of a lower lip flap known as an Abbe flap.

In utero repair of cleft lip deformities has recently become a topic of discussion. In utero repair affords the potential to provide a scarless repair and correct the primary deformity. Furthermore, scarless fetal lip and palate repairs may prevent the ripple effect of postnatal scarring with its resultant secondary dentoalveolar and midface growth deformities. While these suggestions make in utero repair attractive, the risk of fetal loss remains high. Preterm labor is a major complication and one that is directly related to the large hysterotomy required for fetal exposure. Due to the great risks associated with it, intrauterine fetal surgery is still largely reserved for severe malformations that cannot be helped significantly by postnatal intervention.

Palatal clefts may involve the alveolus, the bony hard palate, or the soft palate, singly or in any combination. Clefts of the hard palate and alveolus may be either unilateral or bilateral, whereas the soft palate cleft is always midline, extending back through the uvula. The width of the cleft varies greatly, making the amount of tissue available for repair also variable. The bony palate, with its mucoperiosteal lining, forms the roof of the anterior mouth and the floor of the nose. The posteriorly attached soft palate is composed of five paired muscles of speech and swallowing.

Surgical closure of the cleft to allow for normal speech is the treatment of choice. The timetable for closure depends on the size of the cleft and any other associated problems. However, the defect should be closed before the child undertakes serious speech, usually before age 2. Closure at 6 months usually is performed without difficulty and also aids in the child’s feeding. If the soft palate seems to be long enough, simple approximation of the freshened edges of the cleft after freeing of the tissues through lateral relaxing incisions may suffice. If the soft palate is too short, a pushback type of operation is required. In this procedure, the short soft palate is retrodisplaced closer to the posterior pharyngeal wall utilizing the mucoperiosteal flaps based on the posterior palatine artery.

Satisfactory speech following surgical repair of cleft palate is achieved in 70–90% of cases. Significant speech defects usually require secondary operations when the child is older. The most widely used technique is the pharyngeal flap operation, in which the palatopharyngeal space is reduced by attaching a flap of posterior pharyngeal muscle and mucosa to the soft palate. This permits voluntary closure of the velopharyngeal complex and thus avoids hypernasal speech. Various other kinds of pharyngoplasties have been useful in selected cases.

Estes JM et al: Endoscopic creation and repair of fetal cleft lip. Plast Reconstr Surg 1992;90:743.  [PubMed: 1410025] 

Lorenz HP, Longaker MT: In utero surgery for cleft lip/palate: minimizing the “ripple effect” of scarring. J Craniofac Surg 2003;14:504.