Retreatment of a patient with Marfan syndrome and severe ... · Marfan syndrome is an inherited connective tissue disorder trans- ... her lips without mentalis strain (Fig 2). Intraoral

CASE REPORT

Retreatment of a patient with Marfan syndromeand severe root resorption

John E. Bilodeau

Springfield, Va

This case report describes the retreatment of a patient with Marfan syndrome whose earlier orthodontic andsurgical treatment had been unsuccessful. Marfan syndrome is an inherited connective tissue disorder trans-mitted as an autosomal dominant trait. The disorder results from molecular defects in the fibrillin gene that areresponsible for the impaired structural integrity of the skeletal, ocular, and cardiovascular systems. When shesought retreatment, the patient had an open bite, mandibular anterior crowding, severe root resorption, andtemporomandibular joint derangement with some resorption of the condyles. The second treatment, which in-cluded extractions and surgery, resulted in balanced and harmonious facial proportions, and a Class I occlu-sion with normal overjet and overbite. There was no further loss of condylar tissue, and the temporomandibularjoints were asymptomatic. More root resorption on the mandibular left canine and the left second premolarwas evident after the second treatment. (Am J Orthod Dentofacial Orthop 2010;137:123-34)

Ayoung woman needed orthodontic retreatment

for an open bite, crowded mandibular incisors,and temporomandibular joint (TMJ) derange-

ment with some flattening (resorption) of the condyles.She had a history of previous orthodontic and orthog-nathic surgical treatment. She has Marfan syndromeand extensive root resorption. Did the earlier treatmentcause the flattening of the condyles and root resorptionor was there a genetic predisposition, or both? Why didthe first treatment fail? Was retreatment worth the risk?

HISTORY AND ETIOLOGY

The patient was a white woman, aged 28 years 5months, with a history of orthodontic and orthognathicsurgical treatment that began at age 13 and lasted for5 years, culminating with orthognathic surgery at age18. Her medical history confirmed that she had Marfansyndrome, a genetic disorder. She had arachnodactylywith positive wrist (Walker) and thumb (Steinberg)signs (Fig 1). She was taking a beta-adrenergic blockerto control blood pressure in hopes of preventing aorticdissection, because she had evidence of aortic dilatationand mitral valve prolapse. This disorder weakens theconnective tissue of the aorta as it enters the heart.She had dural ectasis, hypermobility of her joints, oste-

Private practice, Springfield, Va.

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

ucts or companies described in this article.

Reprint requests to: John E. Bilodeau, 6116 Rolling Rd, Suite 201, Springfield,

VA 22152; e-mail, [email protected].

Submitted, February 2007; revised and accepted, May 2007.

0889-5406/$36.00

Copyright � 2010 by the American Association of Orthodontists.

doi:10.1016/j.ajodo.2007.05.029

oarthritis of her knees, and scoliosis. She had a Class IImalocclusion complicated by a 5-mm open bite, 6 mmof mandibular anterior crowding, and severe root re-sorption. She had a long lower anterior facial height.Her chief concerns were her ‘‘crooked teeth, openbite, and facial appearance.’’ The discomfort she wasexperiencing in her TMJs had been somewhat relievedwith splint therapy.

DIAGNOSIS

Facial photographs showed malar hypoplasia, retro-gnathia, down-slanting palpebral fissures and a longlower anterior facial height. She was unable to closeher lips without mentalis strain (Fig 2).

Intraoral photographs and dental casts (Fig 3)showed missing maxillary first premolars and a 5-mmopen bite from the second premolars anteriorly to thecentral incisors. The molars were in an Angle Class IIrelationship, and the canines were in a Class III relation-ship. The maxillary arch was constricted with a high-arched palate. The mandibular dental midline was2 mm left of the facial midline, and there was 6 mmof mandibular anterior crowding.

The panoramic radiograph (Fig 4) showed that allthird molars were missing as were the maxillary firstpremolars. There was extensive root resorption. Mostteeth showed pulpal obliteration. The condyles wereworn (resorbed) and flattened. Surprisingly, there wasminimal mobility of the teeth.

The cephalometric head film and tracing (Fig 5)showed an ANB angle of 3�. The SNA angle of 72� re-flected a retropositioned maxilla, and the SNB angle of69� confirmed mandibular deficiency. The FMA was

123

mailto:[email protected]

Fig 1. Signs of Marfan syndrome: A, arachnodactyly(spider fingers); B, positive Walker sign, with the distalphalanges of the first and fifth digits of 1 hand overlap-ping when placed around the opposite wrist; C, positiveSteinberg sign, with the thumb extending beyond the ul-nar border when completely opposed in the clenchedhand.

124 Bilodeau American Journal of Orthodontics and Dentofacial Orthopedics

January 2010

51�. The facial height index of Horn1 (posterior facialheight to anterior facial height) was .50, and it confirmeda skeletal open bite. The IMPA, a reflection of the rela-tionship of the mandibular incisor to the mandible, was96�. Because the FMA was high, 51�, the IMPA of 96�

confirmed a protrusive mandibular incisor position.

The Z-angle was 68�.2 The Wits measurement of0 mm was normal.3,4 The symphyseal area was gro-tesquely misshapen from the previous genioplasty. Theprevious surgical fixation devices were also evident.

When orthodontic retreatment is needed, it is impor-tant to review the prior diagnosis and treatment plan todetermine, if possible, why the treatment failed. Herprior treatment records were unavailable. However, af-ter studying her cephalometric and panoramic radio-graphs, it was apparent that orthodontic treatmentwith maxillary first premolar extractions with a maxil-lary surgical procedure and a genioplasty had beenperformed. It is reasonable to speculate that the primaryreason the treatment failed was that mandibular extrac-tions were not part of the initial treatment, and that thesurgical manipulations of the maxilla and the chin wereless than satisfactory.

TREATMENT OBJECTIVES

The following treatment objectives were deter-mined: (1) correct the open bite, (2) obtain a normal pro-file line to nose relationship and a normal Z-angle,3 (3)obtain normal canine and incisal guidance, (4) resolvethe crowding in both dental arches, (5) reduce the exces-sive lower anterior facial height, (6) reduce mentalisstrain, (7) eliminate TMJ dysfunction and discomfort, and(8) guard against further root and condylar resorption.

TREATMENT ALTERNATIVES

No treatment had to be considered an option,because of the Marfan syndrome, the failure of the firsttreatment, the amount of root resorption present, and thecondition of the condyles. Further root and condylarresorption were certainly possible.

The other alternative was extraction of the mandib-ular first premolars followed by an orthognathic surgicalprocedure to correct the vertical skeletal imbalance anda redo of the genioplasty to gain more of an esthetic pro-jection of her chin. This option would make it possibleto upright the mandibular incisors, reduce vertical facialheight, provide an esthetic change, and correct thedental malocclusion.

TREATMENT PLAN

After carefully considering the alternatives, theextraction and surgical option was chosen. The patientunderstood the risks of retreatment and was counseledthat teeth could be lost, and implant placement or splint-ing of her teeth would be necessary because of the com-plication of further root resorption. She was alsoinformed about the condition of her condyles and thatfurther loss of condylar tissue was possible. Despite

Fig 2. Pretreatment facial and intraoral photographs.

Fig 3. Pretreatment dental casts.

American Journal of Orthodontics and Dentofacial Orthopedics Bilodeau 125Volume 137, Number 1

the risks, she wanted to proceed; in fact, she was enthu-siastic about retreatment. She said, ‘‘anything would bebetter than what I have now, and I know I might losesome teeth.’’

When surgical intervention is part of the treatment,several analyses are used as guidelines to position thejaw bones both vertically and horizontally to providea pleasing and harmonious face.

Fig 4. Pretreatment panoramic radiograph showing rootresorption and flattening of the condyles.

Fig 5. Pretreatment cephalometric and headfilm tracing.


January 2010

McNamara’s nasion Frankfort perpendicular wasused as a guideline to determine the placement of themaxilla and the maxillary incisors in the horizontalplane.5 The maxilla should be positioned so that PointA closely approximates this ‘‘line,’’ and the maxillaryincisor is 5 mm 6 2 mm anterior to nasion Frankfortperpendicular. The maxillary incisor should be placedabout 110� to the palatal plane.

The skeletal position of the mandible can bechecked with the analysis of Delaire et al,6 which usesa line from the frontal and maxillary bone intersectionconnected to the posterior clinoid process and fromthis point to menton. This angular measurement shouldbe 85� to 90�. Also, with a line drawn perpendicular tothis line, a vertical assessment can be made by measur-ing the upper facial height from nasion to anterior nasalspine (ANS) and the lower facial height from ANS tomenton. The distance from ANS to nasion should be45% of the total facial height, and the distance fromANS to menton along this line should be 55% of the to-tal facial height. To determine the ideal facial height, .45can be divided into the nasion-ANS distance. This is thetotal ‘‘hard-tissue’’ height that is ideal for a patient. Thismeasurement gives guidelines about whether to open orclose a patient vertically.

In any surgical treatment plan, a soft-tissue evalua-tion is necessary. Variations in the soft tissues that coverthe face can produce misleading conclusions if diagno-sis and treatment planning are based on skeletal mea-surements alone. By using the soft-tissue analysis ofLegan and Burstone,7 the clinician can evaluate the hor-izontal and vertical soft tissues of the mandible. Thisanalysis is used as follows: (1) the SN line is recon-structed 7� upward from its original position, and (2)a perpendicular is drawn from soft-tissue glabella tothis line. Soft-tissue pogonion should closely approxi-mate this line. Vertical soft-tissue proportions can be

checked by drawing a line perpendicular from glabellato soft-tissue nasal point and from subnasale to menton.The ratio of this distance should be 1:1. Another helpfulsoft-tissue evaluation is Merrifield’s Z-angle2 and theinterrelationship of the profile line to the middle of thenose. The profile line should intersect the nose at the an-terior aspect of the nares, and the Z-angle, when mea-sured to the Frankfort horizontal, should be between72� and 78�.

Merrifield’s total space analysis was used to deter-mine space requirements.8,9 The McNamara analysis5

confirmed that Point A was posterior to the nasionFrankfort perpendicular, and the maxilla would needto be surgically moved forward.

A decision was made to extract the mandibular firstpremolars. This extraction pattern would resolve the

Fig 6. Presurgical dental casts.

Fig 7. Presurgical panoramic radiograph.


dental crowding and allow the mandibular incisors to beuprighted to increase overjet to gain maximumadvancement of the mandible.

Both the soft-tissue analysis of Legan and Burstone7

and the analysis of Delaire et al6 showed that verticalheight needed to be reduced. Because of the poor condi-tion of the condyles, further study and a search of theliterature was done.

Arnett et al10 found that medial or lateral torque ofthe mandibular condyle associated with sagittal osteot-omy resulting in medial or lateral condylar compres-sion creates the possibility for late (9-18 months)condylar resorption and Point B relapse. It was rea-soned that any surgical manipulation of the proximal(condyle) segment would have an unpredictable out-come and that a sagittal split should be avoided.Therefore, after all space closure, only a LeFort Iosteotomy and a genioplasty were to be performed toreduce vertical facial height by autorotation of themandible. This type of surgical intervention wouldbe noninvasive to the mandibular condyles and wouldprovide a pleasing profile line to nose relationship anda favorable Z-angle. The genioplasty would also haveto be redone.

TREATMENT PROGRESS

Because of mitral valve prolapse, and on the adviceof her physician, this patient was premedicated with 2 gof amoxicillin to prevent bacterial endocarditis beforeall appointments. The mandibular teeth only werebanded or bonded sequentially with the 10-2 systemof Merrifield.9,11 A .022-in standard nontorqued, non-angulated edgewise appliance was used. The maxillary

Fig 8. Presurgical cephalometric headfilm and tracing.

Fig 9. Computerized visual treatment objectives.


January 2010

teeth would remain without appliances until the man-

dibular incisors were uprighted and the mandibular

arch stabilized. It was reasoned that this approach

would protect the maxillary incisors from further root

resorption until they absolutely had to be aligned and

leveled. The patient was instructed to wear a high-

pull J-hook headgear directly against the mandibular

canine brackets to retract these teeth into the first pre-

molar extraction sites. After canine retraction, the man-

dibular anterior teeth were carefully and slowly

retracted with a .020 3 .025-in closing loop archwire.

The J-hook headgear was worn against the canine

brackets to support anterior retraction. After space clo-

sure in the mandibular arch, a .0213 .025-in stabilizing

archwire was placed.

At this juncture, the maxillary teeth were bandedand bonded. Reproximation of the maxillary anteriorteeth was necessary to create enough space to resolvethe maxillary crowding. Impressions were taken atevery appointment, and the dental casts were hand-articulated to assess the postoperative occlusion. Preop-erative records were taken to plan the orthognathicsurgical procedure (Figs 6-8).

PREOPERATIVE DIAGNOSIS

The presurgical cephalometric tracing (Fig 8)showed that the FMA remained at 51�. The facial heightindex of Horn1 remained the same at .50. The IMPAof 71� confirmed that the mandibular incisors had beenuprighted over basal bone. The Z-angle remained at 68�.

Point A and the maxillary incisors were 13 mmposterior to nasion Frankfort perpendicular.

The analysis of Delaire et al6 showed that themandible could come forward because the posteriorclinoid-FMA-menton angle was 80� and could bepositioned between 85� and 90�. The vertical analysisof Delaire et al showed an upper facial height of 57mm; therefore, the lower facial height should be 69mm. It was actually 88 mm or 19 mm more than whatit should be for a harmonious facial balance. Reducingthe vertical dimension with a LeFort maxillary impactionwould produce a large autorotation of the mandible (per-haps as much as 10-12 mm); this would cause forward po-sitioning of pogonion. Because mandibular surgery wasto be avoided, a large advancement of the maxilla wouldbe necessary to maintain a Class 1 molar relationship.

Fig 10. Posttreatment facial and intraoral photographs.

Fig 11. Posttreatment dental casts.


Fig 12. Posttreatment panoramic radiograph.


January 2010

The Legan-Burstone soft-tissue analysis confirmedthat the mandible needed to come forward, and verticalfacial height needed to be reduced 15 mm to achievea 1:1 ratio and a well-balanced facial profile. Dependingon the amount of autorotation achieved, the genioplastywould be redone to further project pogonion anteriorlyto more closely satisfy the soft-tissue projection of theanalysis, reposition the infrahyoid muscles, and achievea normal Z-angle.

A computerized visual treatment objective was cre-ated with the DFplus software (Dentofacial Planner,Toronto, Ontario, Canada) (Fig 9).

Fig 13. Posttreament cephalometric headfilm andsuperimposed tracings.

TREATMENT RESULTS

The posttreatment photographs (Fig 10) show thebalance and harmony of facial proportions that wasachieved with the orthodontic and surgical approach.The midline is in the center of the patient’s face. Shecan close her mouth without mentalis strain.

The posttreatment dental casts (Fig 11) show a Class Iocclusion with normal overjet and overbite. Theocclusion exhibits canine and incisal guidance. Theopen bite was corrected. The maxillary second molarsare still settling and will eventually come into occlusion.

The posttreatment panoramic radiograph (Fig 12)shows that the level of root length was maintained,except for the mandibular left canine and second premo-lar, which had decreases in root length. There was no fur-ther loss of condylar tissue. The TMJs wereasymptomatic. The mandibular incisors were uprightedover basal bone to an IMPA of 79�. Because of theLeFort impaction of the maxilla, the mandible wasautorotated 11 mm. This rotation allowed the FMA todecrease to 41�. The genioplasty projected pogonion far-ther anteriorly to approach the Legan-Burstone glabellaperpendicular. The Z-angle improved to a normal 75�.

All skeletal cephalometric measurements showedimprovement. With the analyses previously described,the McNamara analysis5 and that of Delaire et al6 illus-trate the postsurgical position of the teeth. The maxillaryincisor was positioned 4 mm closer to nasion Frankfortperpendicular at 110� to the palatal plane. The mandiblewas at 83� according to Delaire et al, and the verticalhard-tissue relationship was reduced by 10 mm. TheLegan-Burstone analysis7 showed that vertical soft-tissue glabella to soft-tissue subnasale and soft-tissuesubnasale to soft-tissue menton were in a 1:1 relation-ship. Soft-tissue pogonion was slightly behind soft-tissue glabella perpendicular. The composite cephalo-metric tracings (Fig 13) show mandibular incisor

Fig 14. Periapical radiographs show root resorption.


uprighting, maxillary anterior movement and impaction,mandibular autorotation and forward movement, andfacial profile improvement.

The periapical radiographs show the severe rootresorption (Fig 14).

Treatment time was 24 months. A .030-in mandibu-lar lingual retainer was bonded to each anterior tooth toproduce a splinted anterior segment. A removablemaxillary circumferential retainer was also placed.

DISCUSSION

There is no doubt that the retreatment of this patientwas clinically challenging and not without risk. Treat-ment was undertaken with much trepidation. Thisauthor had treated this patient’s adoptive mother withorthodontics and a mandibular advancement with a suc-cessful result several years earlier; this encouraged thepatient to seek retreatment. She had not consideredretreatment before because her first treatment wasdone at a dental school, and she accepted her first out-come as all that could be done. It was reasoned thateven if she lost teeth, prosthetic replacements wouldhave a better prognosis with the jaws in an optimalposition and the open bite corrected. As mentioned,the maxillary teeth were not banded until the mandibu-lar arch was stabilized to try to minimize further rootresorption. Patients with Marfan syndrome have anincreased risk of root resorption and pulpal necrosis

with orthodontic treatment.12 TMJ dysfunction and con-

dylar resorption can be important aspects of the disor-

der,13 as can obstructive sleep apnea and upper airway

resistance.14 Severe periodontitis has also been reported

by Straub.15

Marfan syndrome is an autosomal dominant hered-

itary connective-tissue disorder. The incidence is esti-

mated to be at least 1 case per 5000 to 10,000 people.

The syndrome is caused by gene coding for fibrillin-1,

an extracellular matrix glycol-protein. It was first

described by Dr Bernard Marfan in 1896 and was sub-sequently included among the hereditary disorders ofconnective tissues. The gene responsible for the muta-tion was identified in the region of chromosome15q21.1.16 This patient was adopted, and her familialhistory was unknown. Clinical features of the disordercan include tall stature, ectopia lentis, mitral valve pro-lapse, aortic-root dilation, aortic dissection, joint hyper-mobility, arachnodactyly, dural ectasis, highly archedpalate, dental crowding, down-slanting palpebralfissures, and retrognathia. This patient exhibited allthese features except aortic dissection and ectopialentis.

At the onset of treatment, some questions came tomind. Is the TMJ sensitive to changes in mechanicalloads? Can the amount of root resorption be controlled?What about retention and the need for future prosthetictreatment?


January 2010

Mongini17 showed condylar changes after occlusalequilibration. Peltola18 found radiographic changes inpatients treated with orthodontics when compared withcontrols. Arnett et al19,20 concluded that the TMJ isnot immutable and that changes in occlusion (lost teeth,orthodontic or orthognathic manipulations), excessiveparafunctional habits, and articular disc-condyle rela-tionships could contribute to remodeling of the articularstructures of the TMJ. They noted that 1 patient canexperience dysfunctional remodeling (ie, condylysis)whereas another subjected to a similar insult might adaptto the mechanical stress with functional remodeling.

Internal derangement can occur with21-25 and with-out23,24,26-28 remodeling. DeBont et al29 showed thatosteoarthrosis of the mandibular condyle can occur inthose with a normal articular disc-condyle relationship.

Furstman30 described the phenomenon that severeosteosclerotic changes of the mandibular condyle havebeen associated with the loss of occlusal stability. Gazitet al31 and Ehrlich et al32 found structural changes in theTMJ associated with unstable occlusion, including boneresorption and fibrocartilage calcification.

‘‘Posteriorization’’ of the mandibular condyle sec-ondary to occlusal changes might lead to postglenoidspine and posterior condylar resorption.10,27,33 Arnettet al,10 Arnett and Tamborillo,33 and Arnett34 observedcondylar resorption when the condyles were displacedposteriorly after orthognathic surgery. Wolford andCardenas35 described some characteristics that appearto make a patient most susceptible to idiopathic condy-lar resorption. These factors include (1) female sex(approximately 9:1 female to male ratio), (2) age rangeof 10 to 40 years with a strong predominance for teen-agers in their pubertal growth phase, (3) high occlusalplane angle and mandibular plane angle, and (4) ClassII skeletal pattern with or without open bite. They foundthat condylar resorption rarely occurs in patients withlow mandibular plane angle or those with a Class IIIskeletal relationship.

A number of systemic disease states can lead tocondylar resorption.36 These include rheumatoid orjuvenile rheumatoid arthritis, systemic lupus erythema-tosus, familial Mediterranean fever, Sjogren’s syn-drome, Marfan syndrome, psoriatic arthritis, andidiopathic condylysis. Arnett et al20 concluded that con-dylar resorption is multifactorial and based on the host’sadaptive capacity and mechanical stimuli. They stated,‘‘when predisposing host factors are not present, occlu-sal treatments normally result in functional remodeling.However, dysfunctional remodeling from low level me-chanical stress (orthodontics, orthognathic surgery,prosthetics) may occur subsequent to an inadequatehost adaptive capacity, coincidental internal derange-

ment of the joint, excessive parafunction, macrotrauma,or unstable occlusion. Dysfunctional remodeling pro-voked by the treatment of dentoskeletal deformities is,to some extent, dependent on the presence of these hid-den factors. However, it seems likely that excessivetreatment compression is capable of initiating substan-tial condylar resorption and resultant occlusal changeswithout contribution of other stimuli.’’ Arnett et al20

described a 3-fold treatment for condylar resorption:(1) control or eradicate the etiologic factors, (2) stabi-lize the unstable occlusion and the TMJs, and (3) correctthe resulting occlusal deformity.

Recommended treatment options for condylarresorption include (1) splint therapy to minimize jointloading, (2) arthroscopic lysis and lavage, (3) condylarreplacement with a costochondral graft if resorptionrecurs or cannot be controlled, and (4) maxillary surgeryto correct the occlusal deformity.19, 20,28,37,38

In this patient, maxillary surgery was chosen toreduce the load on the condyles. She was informedthat a costochondral graft was possible if the condylesresorbed completely. A case report showed that a patientwith virtually no condyles treated with the same regi-men of orthodontics and surgery experienced noadverse sequelae.39 The genioplasty was redone witha cortical osteotomy to suspend the mentalis muscle toachieve optimal facial balance and harmony.

External apical root resorption (EARR) is the loss ofroot structure in the apical region that can be seen onradiographs. It is an unpredictable phenomenon, andits etiology is unknown. Hartsfield et al40 found thatthe degree and severity of EARR are multifactorial,involving host and environmental factors. Geneticfactors account for at least 50% of the variation inEARR. Variation in the interleukin 1 beta gene in ortho-dontically treated patients accounts for 15% of thevariation in EARR. Those authors found historical andcontemporary evidence that the earliest event leadingto EARR is injury to the periodontal ligament (PDL)and supporting structures at the site of root compressionafter orthodontic force.40

Multinucleated cells called odontoclasts responsiblefor the resorption of the dental tissues’ cementum anddentin share many cytochemical and morphologic char-acteristics with osteoclasts that are responsible for boneresorption. Odontoclasts and osteoclast precursors orig-inate from hemopoietic cells in the bone marrow.41

Brezniak and Wasserstein41,42 reported that loss of api-cal root material is unpredictable, and, when it extendsinto the dentin, it is irreversible. Orthodontic force leadsto microtrauma of the PDL and activation of many cel-lular events associated with inflammation. Root resorp-tion begins adjacent to hyalinized zones and occurs


during and after elimination of hyaline (necrotic)tissues. In their review of the literature, Brezniak andWasserstein41,42 found that EARR is classified into3 types: surface resorption, involving small areasfollowed by spontaneous repair from intact parts of thePDL; inflammatory resorption when resorption hasreached the dentinal tubules; and replacement resorptionwhen bone replaces the resorbed tooth material andleads to ankylosis. Brudvik and Rygh43 found that rootresorption continued in the area where hyalinized tissuepersisted even after active force had ended. They hy-pothesized that the determinants of resorption and repairgenerally seem to be associated with the persistence andremoval of necrotic tissue and a process of repair startedfrom the periphery in the resorbed lacunae where thePDL had been reestablished, whereas ongoing resorp-tion was observed beneath existing hyalinized tissue.

EARR is the bane of orthodontists and a commonsequela associated with orthodontic treatment.Although EARR is a frequent iatrogenic outcome asso-ciated with orthodontics, Harris and Butler44 and Harriset al45 found that it can also occur without orthodontictreatment, presumably as a function of occlusal forces.DeShields,46 Sharpe et al,47 and Parker and Harris48

reported that the amount of tooth movement is posi-tively associated with the extent of EARR. McNab etal49 found extraction patterns can influence EARRbecause of the increased tooth movement required toclose extraction spaces. Sameshima and Sinclair50

found that patients whose 4 first premolars wereextracted had more EARR than those treated withoutextractions or extractions of only the maxillary first pre-molars. Tainthongchai et al51 found that the amount oftime spent in orthodontic treatment can be a factor inEARR. Lee et al,52 in a clinical study, showed that expo-sure of the roots to 2 sequential orthodontic procedures,1 in adolescence and the other during adulthood,actually decreased the extent of EARR.

This patient’s EARR at the beginning of treatmentwas probably caused by many factors, including theduration of the first treatment (5 years), perhaps the useof vertical elastics to control the open bite, and certainlythe genetic influence of Marfan syndrome. EARR did notappreciably increase during the second treatment exceptfor the mandibular left canine and first premolar. This canbe attributed to careful and slow leveling and retractionof the mandibular incisors and by not having applianceson the maxillary teeth until the mandibular arch was sta-bilized. No elastics were used except for those used bythe surgeon during the surgical care phase of treatment.

Several anecdotal reports have demonstrated thestability of teeth with severe root resorption.53-55

Parker53 showed that severely resorbed maxillary

incisors after orthodontic treatment were still function-ing well after 33 years. Roberts56 suggested that retain-ing teeth with fixed appliances should be done withcaution because occlusal trauma to the fixed teeth orsegments might cause further EARR. A fixed mandibu-lar retainer was bonded from canine to canine, and thepatient’s occlusion was adjusted to provide optimalfunction in all excursive movements. She will be fol-lowed in the long term in retention.

Certainly, the long-term prognosis of her dentition isguarded. Technology and research are constantly evolv-ing. If she retains her teeth for another 10 to 15 years, thetissue and bone support can remain viable for futureesthetic implant placement that will maintain soft-tissuecontours and papillae forms. The early detection andmedical management of Marfan syndrome has signifi-cantly increased her life expectancy. Was the risk worththe reward? The patient thinks it was.

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