orthodontic treatment for a patient with congenital cutis laxa

CASE REPORT

Orthodontic treatment for a patient withcongenital cutis laxa

Roberto Carrilloa and P. Emile Rossouwb

Monterrey, Mexico, and Dallas, Tex

This case report was shown at the student case display, sponsored by the College of Diplomates of theAmerican Board of Orthodontics at the 2006 AAO Annual Session. (Am J Orthod Dentofacial Orthop2009;136:282-9)

SUMMARY OF TREATMENT

Case report category: Transverse discrepancyPatient’s date of birth: 8/6/1991Age: 12.0 yearsPretreatment records: 8/18/2003 (Figs 1-4, Table)Treatment Initiated: 10/13/2003Treatment completed: 7/12/2005Posttreatment records: 7/25/2005 (Figs 5-9,Table)Active treatment duration: 21 months

HISTORY AND ETIOLOGY

Medical: This 12-year-old patient’s chief complaintwas ‘‘I want to get my underbite fixed.’’ The patient hasan echocardiograph once a year and has a history of con-genital heart problems such as congestive heart failureand aortic valve defects, esophageal and kidney reflux,and allergic reactions to many medications. He wastreated for asthma with steroids in the past. The patientmust be premedicated before each dental procedurewhen there is a risk of gingival bleeding. The patienthas been diagnosed with cutis laxa, characterized bythe following: (1) premature aging appearance with gen-eralized elastolysis, and inelastic, loose, and hangingskin; (2) extremely rare disorder of connective tissues;(3) can be acquired or congenital (autosomal dominant,autosomal recessive, and X-linked recessive forms); (4)cardiopulmonary abnormalities are common and deter-

a Private practice; professor, Department of Orthodontics, UANL Dental School,

Monterrey, Mexico.b Professor and chairman, Department of Orthodontics, Baylor College of

Dentistry, Texas A&M Health Science Center, Dallas, Tex.

The authors report no commercial, proprietary, or financial interest in the

products or companies described in this article.

Reprint requests to: Roberto Carrillo, Ave. Gomez Morin #309 Col. Valle de Santa

Engracia, San Pedro Garza Garcia, NL, Mexico, 66268; e-mail, dr.rcarrillo@

gmail.com.

Submitted, January 2007; revised and accepted, April 2007.

0889-5406/$36.00

Copyright � 2009 by the American Association of Orthodontists.

doi:10.1016/j.ajodo.2007.04.049

282

mine the prognosis of life expectancy; (5) reported tobe associated with retardation of growth and develop-ment, midface hypoplasia, and prognathism; and (6)plastic surgery is recommended for patients with thissyndrome.1-5

Dental: There were generalized areas of decalcifi-cation. The etiology of the malocclusion could bea combination of the congenital characteristics of thedisease in addition to the impacted maxillary caninespreventing the anterior portion of the maxillary alveolarbone from developing. The constricted and underdevel-oped maxilla contributed to crowding, anterior and pos-terior crossbites, and lack of support to the upper lip.The forward mandibular slide of 2 mm was a responseto the anterior end-on occlusion during maximumintercuspation.

DIAGNOSIS

Facial: Leptoprosopic with ovoid facial outline.Orthognathic profile with malar deficiency, long lowerfacial third, obtuse nasolabial angle, and shallow mento-labial fold. Lack of tonicity of all facial muscles.

Skeletal: Hand-wrist radiograph analysis suggestedthat the patient had not reached peak height velocity. Inmaximum intercuspation, Class III skeletal relationshipwith a high mandibular plane angle and long lowerfacial third. Maxillary hypoplasia in the transverse andsagittal dimensions was also present (Wits, Harvoldmaxillomandibular differential, and ANB; see Table).

Dental: The patient had a 2-mm anterior mandibu-lar shift from centric relation to maximum intercuspa-tion, and blocked-out maxillary canines and uneruptedsecond permanent molars. In maximum intercuspation,bilateral Angle Class I molar relationship, anteriorcrossbite on maxillary 2-2 and posterior crossbite onmaxillary right second premolar and first molar. Nega-tive overjet of –2 mm and overbite of 3 mm. Maxillaryand mandibular dental midlines were 1 mm to the rightof the soft-tissue midline. Retroclined and retruded

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American Journal of Orthodontics and Dentofacial Orthopedics Carrillo and Rossouw 283Volume 136, Number 2

Fig 1. Pretreatment photographs.

maxillary incisors and retroclined mandibular incisors.Decalcified lesions in all erupted teeth of both arches.

SPECIFIC OBJECTIVES OF TREATMENT

Maxilla

Transverse: Increase.A-P: Increase projection.Vertical: Increase.

Mandible

Transverse: Maintain.A-P: Decrease projection.Vertical: Minimize increase of mandibular plane.

Maxillary dentition

Transverse: Increase intermolar width.

A-P: Protrude and procline maxillary incisors andmaintain molar position.Vertical: Extrude.

Mandibular dentition

Transverse: Maintain intercanine width.A-P: Maintain position.Vertical: Maintain.

Facial esthetics and function

Improve profile by enhancing upper lip support andimproving the nasolabial angle. Eliminate the anteriormandibular shift from centric relation to maximumintercuspation.

284 Carrillo and Rossouw American Journal of Orthodontics and Dentofacial Orthopedics

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Fig 2. Pretreatment study models.

Fig 4. Pretreatment cephalometric tracing.
Fig 3. Pretreatment radiographs.


Table. Cephalometric summary

Area Measurement A 8/18/2003 B 7/12/2005 Difference A–B

Maxilla to cranial base SNA 76� 77� 1�

FH-NA 87� 88� 1�

A-Na Perp (mm) –4 –2 2

Mandible to cranial base SNB 77� 75� –2�

SN-GoGn 45� 47� 2�

FMA 37� 38� 1�

FH-NPg 87� 87� 0�

Maxillomandibular ANB –1� 2� 3�

Wits (mm) –8 –5 3

Harvold mx-md differential 39 36 –3

Vertical height N-Me (mm) 128 136 8

ANS-Me (mm) 75 79 4

UFH/LFH (%) 42/58 43/57 1/–1

Maxillary dentition U1-NA (mm) 4 8 4

U1-SN 95� 105� 10�

Intermolar width (mm) 34 38 4

Mandibular dentition L1-NB (mm) 6 8 2

L1-MP 81� 80� –1�

Intercanine width (mm) 26 26 0

Soft tissue Esthetic plane to:

Lower lip (mm) –1 –1 0

Upper lip (mm) –6 –3 3

FH-N0Pg0 87� 87� 0�

TREATMENT PLAN

Because of the genetic disorder, before startingorthodontic treatment, it was decided to obtain medicalauthorization from the patient’s physician and then referhim for a checkup and dental cleaning. To improvefacial esthetics and the maxillary deficiency, the patientwas offered a nonextraction treatment along with ortho-pedic correction as a first attempt to correct the mal-occlusion. The patient required antibiotic premedicationbefore any appointment with a risk of bleeding becauseof his history of cardiac disease. The treatment plan in-cluded to fit bands in the maxillary molars and cementa rapid palatal expander with attachments for facemask.Activate the expander for 3 weeks and wear thefacemask for 14 to 16 hours per day for 8 to 12 months.To benefit facial esthetics, attempt to avoid extractionsand resolve the crowding by the space created withmaxillary expansion, and by protruding and procliningthe maxillary incisors to increase arch length. Assessthe space for the maxillary canines and incorporatethem into the arch after they have erupted. Incorporateall second molars into the treatment as they erupt intoocclusion. Obtain an Angle Class I molar/canine rela-tionship on both sides and resolve mandibular anteriorslide improving overjet and overbite. Wear interarchelastics during treatment as needed. Detail the occlusionand debond. Retain with maxillary wraparound Hawleyand a mandibular 3-3 bonded to each tooth.

APPLIANCES AND TREATMENT PROGRESS

A rapid maxillary expander was cemented withbands on the first molars and extension hooks mesialto the first premolars as attachments for the facemask.He was instructed to activate the expander twice a day(0.5 mm per day) for a total expansion time of 3 weeks.The facemask was delivered after 14 days of expansion,and the patient was instructed to wear it for not less than14 to 16 hours per day. The facemask was activated witha pair of rubber bands (1 per side) exerting a force of 14oz each. The maxillary central and lateral incisors werebonded with fixed edgewise appliances (0.018 3 0.025in, SPEED, System Orthodontics, Cambridge, Ontario,Canada) after 4 months of treatment; they were consol-idated as an anterior segment. The mandibular arch wasbonded with the same fixed appliances 7 months aftertreatment started. After 9 months of facemask wear, itwas stopped, the maxillary expander was removed,and the maxillary posterior teeth were bonded with fixedappliances. In addition to the expansion space created,space for the maxillary canines was achieved by wear-ing Class II elastics (3/16 in) from L6 to U4 and byplacing a stainless steel open-coil spring between U4and U2, in addition to 1-mm increments in archwirelength to advance and procline the maxillary anteriorsegment. After 9 months of treatment, a lateral cephalo-gram was taken to assess progress, maxillary incisorangulation, and A-P position. The maxillary canines


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Fig 5. Posttreatment photographs.

erupted after 12 months of treatment, and they werebonded and incorporated into the arch by using anoverlay 0.014-in nickel-titanium archwire throughthe 0.016-in auxiliary slot in the appliances. Afterall teeth were incorporated into the arches, a combi-nation of bilateral light Class III elastics from U6 toL3 (3/16 in), lateral triangular elastics from L4 to U3to L3 (3/16 in), and anterior box elastics U2 to U2 toL2 to L2 (1/4 in) were used throughout treatment toassist with coordination of the arches and consolida-tion of the dentition. The mandibular wire was sec-tioned L3-L3, and finishing elastics (3/4 in) wereworn for 3 weeks before debonding. The fixed appli-ances were removed after 21 months of active treat-ment, and the retention phase began with a maxillarywraparound Hawley and a mandibular fixed 3-3retainer.

RESULTS ACHIEVED

Maxilla

Transverse: Intermolar width increased by 4 mm.A-P: SNA and FH-NA angles increased by 1�.Vertical: Downward tipping of the palatal plane.

Mandible

Transverse: Unchanged.A-P: SNB angle decreased by 2�.Vertical: FMA increased by 1� and SN-GoGnincreased by 2�.

Maxillary dentition

Transverse: Intermolar width was increased by4 mm, and the crossbite was resolved.


Fig 6. Pretreatment study models.

Fig 7. Posttreatment radiographs.
Fig 8. Posttreatment cephalometric tracing.


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Fig 9. Cephalometric superimpositions.

A-P: The maxillary incisors were protruded (4 mmin relation to NA) and proclined (10� in relation toSN), and the maxillary molar position was maintained.Vertical: All maxillary dentition was extruded.

Mandibular dentition

Transverse: Intercanine width was maintained.A-P: Incisors’ crown torque was decreased 1� inrelation to the mandibular plane.Vertical: Incisors and molars were extruded.

FACIAL ESTHETICS AND FUNCTION

Protruding and proclining the maxillary incisors im-proved the nasolabial angle and upper lip support; thesebenefited the patient’s profile and facial balance. Theanterior mandibular shift was eliminated by correctingthe occlusal relationships and the backward rotation ofthe mandible; these also helped to achieve a positiveoverjet and complement the profile and facial esthetics.

ALTERNATIVE TREATMENTS

If the treatment goals with the nonextraction approachwere not achieved, the patient and his mother were ad-vised during the initial consultation of 2 other treatmentplans: extraction of permanent teeth and maxillofacialsurgery, which would be evaluated at the appropriate

time during treatment. The patient was also advisedof the possibility of surgical exposure of the maxillarycanines if they did not erupt after the space was created.

RETENTION

Maxillary wraparound Hawley retainer and mandib-ular permanent bonded canine-to-canine retainer.

FINAL EVALUATION OF TREATMENT

The patient had significant growth during treatment,and the improvements in facial esthetics were mainlydue to the change of upper lip support, improvementof the nasolabial angle, and mandibular backward rota-tion. The treatment outcome improved the patient’ssmile by increasing incisor display; this resulted ingreater self-esteem. The occlusion was corrected byelimination of the functional shift, finishing the treat-ment with coincident maximum intercuspation and cen-tric relation, positive overjet, and good anteriorguidance. The backward rotation of the mandible en-hanced the correction of the anterior crossbite as a resultof the facemask treatment. Adequate space to incorpo-rate the maxillary canines into the arch was obtainedvia the palatal expansion, protrusion and proclinationof the maxillary incisors, and preventing mesial driftof the maxillary posterior teeth. The mandibular


incisors were extruded, but they maintained theirA-P position; by extrusion of the maxillary incisors,an adequate overbite was obtained. The occlusion ofthe second molars, although acceptable, could havebeen improved during treatment. The posttreatment ra-diographic analysis showed all third molars uneruptedafter treatment. In addition, the root position of the max-illary right lateral incisor root could have been im-proved, but an acceptable clinical result was obtained.Retention is by a permanent bonded retainer from ca-nine to canine and a maxillary wraparound Hawley re-tainer. The patient was instructed on how to floss themandibular anterior area and to wear the maxillary re-tainer 24 hours a day for 8 weeks and then at night only.

REFERENCES

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2. Steiner CE, Cintra ML, Marques-de-Faria AP. Cutis laxa with

growth and developmental delay, wrinkly skin syndrome and gero-

dermia osteodysplastica: a report of two unrelated patients and a lit-

erature review. Genet Mol Biol 2005;28:181-90.

3. de Schepper S, Loeys B, de Paepe A, Lambert J, Naeyaert JM. Cutis

laxa of the autosomal recessive type in a consanguineous family.

Eur J Dermatol 2003;13:529-33.

4. George S, Jacob M, Pulimood S, Chandi SM. Cutis laxa. Clin Exp

Dermatol 1998;23:211-3.

5. Ogur G, Yuksel-Apak M, Demiryont M. Syndrome of congenital

cutis laxa with ligamentous laxity and delayed development:

report of a brother and sister from Turkey. Am J Med Genet

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orthodontic treatment for a patient with congenital cutis laxa

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