domingo, 30 de noviembre de 2014

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.




lunes, 24 de noviembre de 2014

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]
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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]
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martes, 11 de noviembre de 2014

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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