CASE REPORT
Orthodontic treatment considerations for a patient with erythropoietic protoporphyria Richard G. Standerwick,a Edwin H. K. Yen,b and Benjamin Pliskac Vancouver, British Columbia, Canada Erythropoietic protoporphyria (EPP) is an inherited blood disorder in which formation of the heme group of hemoglobin is defective. Specifically, a deficiency of the enzyme ferrochelatase leads to the accumulation of protoporphyrin, resulting in often painful photosensitivity of the skin and tissues. The prevalence of EPP is estimated at 1:75,000 to 1:200,000. Photosensitivity is exhibited upon exposure to light with specific wavelengths through the creation of reactive oxygen products (oxidants), activation of the complement system, and mast cell degranulation. The aim of this article is to report the orthodontic treatment of an 11-year-old boy with EPP, a Class III skeletal relationship, and an anterior crossbite. Orthodontic treatment established normal overbite and overjet. Short-term periodontal and dental tissue responses to treatment were noted. Extra care was needed when collecting photographic and radiographic records for this patient and during some treatment procedures to avoid causing a photosensitive reaction of the skin or oral mucosa. (Am J Orthod Dentofacial Orthop 2013;144:899908)
P
orphyrias are a group of metabolic disorders resulting from defective porphyrin and heme synthesis. They are classified as erythropoietic or hepatic, depending on which organ site expresses the defect in heme synthesis; by the clinical manifestations of cutaneous photosensitivity; or by neurovisceral symptoms.1-4 Erythropoietic protoporphyria (EPP) was first described by Kosenow and Treibs (as cited in Lecha5) and further by Magnus et al6 and is a subset of the erythropoietic porphyrias with a prevalence between 1:75,000 and 1:200,000.2,3,5,7,8 EPP is an inherited disorder of the heme metabolic pathway characterized by accumulation of protoporphyrin in blood, erythrocytes, and tissues, resulting in cutaneous manifestations of photosensitivity.3,7,9,10 The accumulation of protoporphyrin is a result of a deficiency in the ferrochelatase enzyme.2,3 Protoporphyrin accumulates in red blood cells and their precursors and eventually diffuses out of the red blood From the Department of Orthodontics, Faculty of Dentistry, University of British Columbia, Vancouver, British Columbia, Canada. a Associate clinical professor; private practice, Langley, British Columbia, Canada. b Professor and dean emeritus. c Assistant professor. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported. Address correspondence to: Richard G. Standerwick, 20159 88th Ave, Suite E207, Langley, BC, Canada V1M0A4; e-mail, richard@transformationorthodontics. com. Submitted, January 2013; revised and accepted, February 2013. 0889-5406/$36.00 Copyright Ó 2013 by the American Association of Orthodontists. http://dx.doi.org/10.1016/j.ajodo.2013.02.032
cells into the plasma as the red blood cells mature.8 Excessive protoporphyrin accumulates in the vascular and perivascular areas and absorbs light energy at 320 to 595 nm on the exposed areas of skin.3,5,7 Peak absorption is in the range of 400 to 410 nm (the Soret band).7 The energy absorption causes the formation of reactive oxygen products, the activation of the complement system, direct and indirect degranulation of mast cells, and chemotaxis of polymorphonuclear leucocytes.3,5,7,11 EPP typically first presents symptoms in early infancy after sun exposure; it is characterized by cutaneous manifestations of acute painful photosensitivity leading to erythema and edema. The severity of the disorder varies significantly, from extreme stinging, itching, and burning sensations to petechiae and, uncommonly, blistering.2-4,8,12 Affected children might cry and scream for hours, often throughout the night, without an obvious reason.7 The pain has been described as like “a lighted match being held against your skin”; a 6-year-old child, after crying for a full weekend, asked his mother to “please cut my hands off and let me go to sleep.”7 Recurrent episodes of unexplained hand and facial swelling can warrant further investigation.13 It is often diagnostic that the affected person attempts to relieve the pain by applying cold water to the skin.7,13 Because there is no cure for EPP, treatment is focused on prevention and symptom management. Exposure to light within the noxious range should be avoided, with protective clothing and sunscreens to limit photosensitivity. Even sunlight through window glass can precipitate 899
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photosensitivity.8 Other therapies to relieve photosensitivity have been tried, but most lack studies that definitively show clinical relevance7; however, cholestyramine therapy might be helpful to increase fecal excretion of protoporphyrin.8 Because of the lack of sunlight exposure and therefore decreased endogenous vitamin D production, supplementation of vitamin D (1,25 dihydroxycholecalciferol) is required to prevent deficiency and to maintain normal bone metabolism and homeostasis.10,14,15 Protoporphyrin blood concentrations and liver function are monitored with regular blood testing, and appearance is assessed for jaundice.3,5,7 Protoporphyrin is not water soluble; therefore, excretion is facilitated by its solubilization in the liver followed by biliary secretion.7,16 When the protoporphyrin solubilizing capacity of the liver is exceeded, the result can be hepatic failure, which occurs in approximately 10% of affected persons3,5,7; this can be fatal without prompt liver transplantation.7,17 During clinical procedures, operating and room lighting must be filtered to avoid inducing severe burns.18 Dental curing lights emit light wavelengths of approximately 480 nm and should also be considered a potential danger to EPP patients. This case report describes the nonextraction orthodontic treatment and management of a patient with EPP. DIAGNOSIS AND ETIOLOGY
The patient, a boy, age 11 years 10 months, sought an orthodontic consultation for his anterior crossbite. His medical history included EPP, allergy to penicillin, and concerns about medications because of the hepatic and kidney associations with EPP. He took vitamin D daily. No liver issues were diagnosed before or during treatment. His notable facial attributes included a concave facial profile, a canted mandibular lip on smiling with otherwise normal facial symmetry and facial thirds, a functional shift of the mandible (1 mm anteriorly and 2-3 mm superiorly) (Fig 1). Notable dental attributes included a super Class I molar relationship, a partial anterior crossbite, a 1.8-mm overjet, a 3-mm overbite, adequate mandibular and inadequate maxillary arch lengths, and good dental hygiene (Fig 2). The cephalometric radiographs were analyzed using software (version 11.5; Dolphin Imaging and Management Solutions, Chatsworth, Calif). The adductor sesamoid was present on the hand-wrist radiograph, and the patient appeared to display a cervical vertebral maturation stage 2; therefore, it was likely that pubertal growth was imminent or already attained (Figs 3-5).19-23 The skeletal analysis involved components from the Indiana University School of Dentistry, American Board
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of Orthodontics, Bjork, Bjork 2, Michigan Surgery, and Litt Biodynamic analyses (version 11.5; Dolphin Imaging and Mangement Solutions). This variable analysis was used because of the short anterior cranial base, the altered position of nasion, and the decreased angulation of the S-N line because any cephalometric measurement associated with nasion was most likely skewed. The skeletal attributes included a hypodiverent tendency, normal lower face height, decreased ANB and A-B/OP (Wits), and increased SNA and SNB. The relative rather than literal values of SNA and SNB were considered because of the altered position of nasion and the decreases in sella-nasion-basion and SN-articulare angulation. A decreased SN-Ba angulation tends to translate into increased values for SNA and SNB.24-26 The maxillary position determined by condylion to anterior nasal spine displayed maxillary retrusion, whereas condylion to pogonion had a normal mandibular position. The maxillary incisors exhibited a decreased inclination relative to the palatal plane and an increased inclination relative to the Frankfort horizontal. The mandibular incisors showed retroclination and increased protrusion relative to the A-point–pogonion line (Table 1). No clicking or pain was reported in the right or left temporomandibular joint. The maximum opening was slightly restricted at 39 mm for a soft end stop, and maximum opening was 49 mm. Lateral excursive and protrusive movements were normal (8-10 mm); the mandible deviated to the left on closure. Tooth and condylar morphologies on the panoramic radiograph showed no overt pathology. TREATMENT OBJECTIVES
The maxillary objectives were to maintain the maxilla in the anteroposterior, vertical, and tranverse dimensions. The mandibular objectives were to decrease the anteroposterior protrusion, increase the vertical dimension possibly reducing anteroposterior protrusion, and maintain the transverse dimension. The maxillary molar position was to be maintained in the anteroposterior, vertical, and transverse dimensions. The maxillary incisor objectives included increases in incisor protrusion and inclination, and maintenance of vertical position. Mandibular molar and incisor anteroposterior and vertical positions were to be maintained. Maxillary molar, mandibular molar, and mandibular canine widths were to be maintained. The treatment objectives included alignment of the dental arches and establishment of a Class I molar relationship and normal overbite and overjet. Facial convexity was expected to increase with elimination of the mandibular functional shift and correction of the anterior crossbite.
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Fig 1. Pretreatment photographs, age 12 year 7 months. Patients with EPP must limit their exposure to light to prevent painful photosensitivity; therefore, records were collected with flashless photography.
Fig 2. Pretreatment dental casts.
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Fig 3. Hand-wrist radiograph at age 12 years 7 months. The radiographs were collected sequentially, 1 to 2 months apart. The least vascular structures were examined first to assess the patient's response.
Fig 5. Cephalometric radiograph at age 12 years 10 months.
that pseudo-Class III patients display maxillary retrusion caused by functional inhibition of growth; therefore, catch-up growth or restoration of the normal growth pattern can be expected after the establishment of normal overbite and overjet.27-30 TREATMENT PROGRESS
Fig 4. Panoramic radiograph at age 12 years 9 months.
TREATMENT ALTERNATIVES
The treatment alternatives included no treatment, surgical treatment, extraction of permanent teeth, or a nonextraction approach. The proclination of the maxillary incisors, increased A-point to pogonion, and Class III tendency might have favored an extraction or surgical approach depending on the weight given to selected factors. Also considered were tissue reactions relative to extraction, space closure, and orthodontic treatment in general, since they were unknown for this genetic condition. A nonextraction treatment plan was chosen based on the clinical presentation of the mandible relative to the maxilla in centric relation and the maxillary incisor inclination relative to the palatal plane. It has been shown
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Informed consent was obtained from the patient and his parents before treatment and to allow consultation with the patient's physicians. The primary care physician at British Columbia Children's Hospital and the consulting physician for the Canadian Porphyria Foundation31 were consulted. The consensus was that radiographic exposure should be fine. A hand-wrist radiograph and panoramic and lateral cephalometric radiographs were exposed sequentially a month apart to allow observation of any resulting symptoms (exposure of the least vascular structures first). The maxillary and mandibular arches were indirectly bonded with the Sondhi indirect bonding kit (3M Unitek, Monrovia, Calif) and allowed to chemically cure. The orthodontic appliance was a 0.022-in slot bracket with MBT prescription (3M Unitek). A modified Gelb mandibular appliance was used to disarticulate the teeth (2-mm Biocryl 021-027; Great Lakes Orthodontics, Tonawanda, NY). Wire sequencing was 0.014in nickel-titanium, 0.018-in copper-nickel-titanium, 0.019 3 0.025-in copper-nickel-titanium, 0.019 3 0.025-in beta-titanium, and 0.019 3 0.025-in stainless steel (G&H Wire, Franklin, Ind). At some point after the
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Table 1. Cephalometric measurements Skeletal measurements N-A-Pg ( ) N-A jj HP (mm) N-B jj HP (mm) N-Pg jj HP (mm) A-B jj HP (mm) A-B jj OP (mm) FH-SN ( ) SNA ( ) SNB ( ) ANB ( ) OP-HP ( ) PP-HP ( ) MP-HP ( ) Palatal-maxillary occlusal plane ( ) Mandibular occlusal-mandibular plane ( ) Chin angle (Id-Pg-MP) ( ) Y-axis (S-Gn-FH) ( ) Gonial/jaw angle (Ar-Go-Me) ( ) Chin angle (Id-Pg-MP) ( ) Anterior cranial base (SN) (mm) Posterior cranial base (S-Ar) (mm) Ramus height (Ar-Go) (mm) Length of mandibular base (Go-Pg) (mm) Maxillary unit length (Co-ANS) (mm) Mandibular unit length (Co-Pog) (mm) Harvold (CoPog)-(CoANS) ( ) Maxillary skeletal (A-Na perp) (mm) Mandibular skeletal (Pg-Na perp) (mm) MP-SN ( ) Upper face height (N-ANS) (mm) Lower face height (ANS-Me) (mm) Upper face height (N-ANS/[N-ANS1ANS-Me]) (%) Lower face height (ANS-Me/[N-ANS1ANS-Me]) (%) Ba-S-N ( ) SN-Ar ( ) Dental measurements 1/(HP) ( ) /1(HP) ( ) 1/-(UL) (mm) IMPA/1 (MP) ( ) Interincisal angle (U1-L1) ( ) /1(A-Pg) (mm) /1(N-Pg) (mm) U1-SN ( ) U1-ANS (mm) U6-PP (UPDH) (mm) L1-MP (LADH) (mm) U1-Me (mm) L6-MP (LPDH) (mm) Pr-N-A ( ) U1-palatal plane ( ) U1-NA (mm) GoGn-chin line ( ) L1-GoGn ( ) L1-NB (mm) Pg-NB (mm) Overjet (mm) Overbite (mm)
Pretreatment
Posttreatment
Mean
SD
Dev Normal
8 3.6 13.4 13.8 9.8 5.5 2.1 87.1 91.7 4.6 6.6 13.3 14.3 9.9 24.6 74.8 50.1 131.2 74.8 68.6 38.4 50.4 75.3 83.1 113.4 30.3 0.7 5.4 21.3 45.4 61.7 42.4 57.6 124.7 120.1
9.5 4.5 16.8 18.4 12.2 6.4 2 88.2 93.8 5.7 8.7 13 14.2 6.1 24.5 73.9 49.5 130.5 73.9 69.6 39 53.8 82.7 82.8 116.8 34.1 0.1 9.3 21.2 46.9 65.5 41.7 58.3 124.1 119.5
5.5 2 8.5 9 6.5 0.5 6 82 80 2 9 0.5 25 10 20 70 60 124.4 70 73.9 33.5 45.3 73 90 113 20 0 4 33 NA NA 43 57 130 124
4.5 3.5 4.5 5 2.5 2 4 3 4 1.5 2.5 3 5 4 5 5 3 6.7 5 3 4 4.5 3 5 8 3 3.1 5.3 6 NA NA 100 100 100 5
3 1.6 4.9 4.6 6.5 2.5 1 1.7 2.9 4.4 6.2 4.6 2.1 0 0.9 1 3.3 1 1 1.7 1.2 1.1 0.8 1.4 0.1 3.4 0.2 1.8 1.9 NA NA 0 0 0.1 0.8
119.6 78.6 2.4 87.1 139 5.4 3.2 112.6 26 21 39.5 5.7 27.4 3.9 106.3 6.1 78.1 90.3 2.3 1.2 1.9 3.6
133.8 80 0.2 85.8 126.2 6.2 3.5 126.8 26.6 22.3 42 5.6 30.7 7 120.8 11.4 76.4 88.3 2.6 1.3 1.7 1.8
110 60 2.5 90 130 2.5 3.5 102.6 30 20 40 35 30 2 110 4 70 94 4 NA 2.5 2.5
6.5 4.5 1 5 6 2 2.5 5.5 5 3 3 3 3 1 5 2.7 5 7 1.8 NA 2.5 2
1.5 4.1 0.1 0.6 1.5 1.4 0.1 1.8 0.8 0.3 0.2 9.8 0.9 1.9 0.7 0.8 1.6 0.5 0.9 NA 1.8 0.5
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Table 1. Continued Soft-tissue measurements Facial convexity (G0 -Sn-Po0 ) ( ) UL protrusion (UL-SnPg0 ) (mm) LL protrusion (LL-SnPg0 ) (mm) UL to E-plane (mm) LL to E-plane (mm) ILG (HP) (mm)
Pretreatment
Posttreatment
Mean
SD
Dev Normal
1.5 2.9 5.7 5.2 0.2 1.2
4.1 2.5 3.5 6.2 2.5 0.7
13.5 3.5 2.8 3 2.5 NA
5.5 1.8 1.7 2 2.5 NA
2.7 0.3 1.7 1.1 1.1 NA
Relative rather than literal values of SNA and SNB were considered because of the altered position of N and the decreases in SN-Ba and SN-Ar angulations. Pretreatment measurements, age 12 years 7 months; final measurements, age 14 years 0 months. Dev Normal, Standard deviation from normal; HP, horizontal plane/Frankfort horizontal; OP, occlusal plane; FH, Frankfort horizontal; PP, palatal plane; MP, mandibular plane; UPDH, upper posterior dental height; LADH, lower anterior dental height; LPDH, lower posterior dental height; UL, upper lip; LL, lower lip; ILG, interlabial gap; NA, not applicable.
Fig 6. Posttreatment photographs at age 14 years 0 months.
bonding appointment, the modified Gelb fractured and was not reported until the patient returned with multiple debonded brackets. The loose brackets were rebonded with glass ionomer cement (Fuji Ortho; GC Corporation, Tokyo, Japan). The self-curing time of the cement was 10 minutes. Bite buildups were placed on the mandibular permanent first molars with the glass ionomer cement to eliminate compliance issues. A panoramic radiograph was taken 4 months after the initial bonding to evaluate periodontal structures and root position. During leveling with the 0.019 3 0.025-in beta-titanium wire, the glass ionomer buildups were removed to allow eruption of the posterior teeth; immediately, an acrylic appliance with an anterior biteplate was fabricated. Class III 3/16-in, 6-oz
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elastics were added from the maxillary permanent first molars to the mandibular canines. Compliance with the biteplate appliance and elastics was low. Throughout treatment, there were also many rebondings of brackets because of debonds between the bracket and cement. Impressions were made for study models, and the buildups were removed. Elastics were changed to Class III 1/4-in, 3.5 oz elastics from the maxillary first molars to Kobayashi hooks on the mandibular lateral incisors. Vertical elastics (3/16-in, 3.5 oz) were worn from the maxillary first and second premolars to the mandibular first and second premolars. At debond, temporary Invisacryl C (021-056; Great Lakes Orthodontics) retainers were delivered, and final
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Fig 7. Posttreatment dental casts.
Fig 8. Posttreatment panoramic radiograph at age 14 years 0 months. Pulp stones were evident on the initial panoramic radiograph and appeared to increase in size and radiopacity during treatment. Glass ionomer buildups are evident on the final panoramic radiograph but were subsequently removed.
records were obtained. Maxillary and mandibular Hawley retainers were inserted after 1 month (becaue of the Christmas holidays rather than by intention). The retainer was evaluated at 1 week, 1 month, 3 months, and 6 months, and scheduled yearly or as needed. TREATMENT RESULTS
There were no reported adverse symptoms from the radiographic exposure. Tooth movement appeared to be normal, and no gross abnormal signs of external apical root resorption were evident. In general, the
periodontal tissues appeared to tolerate the orthodontic manipulation well (Figs 6-9). The maxilla grew anteriorly; however, the relative position of SNA was maintained in the anteroposterior dimension. The maxilla was basically maintained vertically, with a slight forward or counterclockwise rotation, and the transverse dimension was maintained. The mandible displayed increases in protrusion (SNB and Co-Pg) and the vertical dimension, most likely due to growth, whereas the relative vertical dimension was maintained. The mandible displayed approximately 2 of forward and counterclockwise rotation. The final position of the mandibular condyles on the lateral cephalogram might have been affected by head position in the cephalostat (pterygoid fissures are not superimposed well on the final cephalogram) or might have displayed an increase in growth asymmetry. The maxillary molar position displayed protraction and extrusion and was maintained in the transverse dimension. The maxillary incisors displayed increases in protrusion and inclination, and the vertical dimension was maintained. The mandibular molar and incisor positions were maintained in the anteroposterior dimension, and they displayed increases in the vertical dimension and extrusion. Maxillary molar, mandibular molar, and canine widths were maintained. After active orthodontic treatment, dental alignment, a Class I molar relationship, and normal overbite and overjet were established (Figs 10 and 11, Table 1).
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Fig 9. Posttreatment cephalometric radiograph at age 14 years 0 months. The final position of the mandibular condyles on the lateral cephalogram might have been affected by head position in the cephalostat (pterygoid fissures are not superimposed well on the final cephalogram) or might be caused by an increase in growth asymmetry.
Fig 10. Craniofacial superimpositions: 12 years 10 months (black) and 14 years 0 months (red). The maxilla grew anteriorly, but the relative position of SNA was maintained in the anteroposterior dimension. The maxilla was basically maintained vertically and displayed a slight forward or counterclockwise rotation. The transverse dimension was maintained. The mandible displayed increases in protrusion (SNB and Co-Pg) and the vertical dimension, likely due to growth, whereas the vertical transverse dimension was maintained.
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Fig 11. Regional superimpositions: 12 years 10 months (black) and 14 years 0 months (red). The maxilla displayed a slight forward or counterclockwise rotation, and the mandible displayed approximately 2 of forward or counterclockwise rotation. The maxillary molar position displayed protraction and extrusion and was maintained in the transverse dimension. The maxillary incisors displayed increases in protrusion and inclination, and the vertical dimension was maintained. The mandibular molar and incisor positions were maintained in the anteroposterior dimension, with an increase in the vertical dimension with extrusion. DISCUSSION
To our knowledge, there have been no published articles discussing orthodontic treatment of a patient with EPP. Whether protoporphyrin would accumulate in the bone or tissues and how that might affect tissue reactions were unknown. However, further postorthodontic consultation seems to indicate that the
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protoporphyrin would remain in the blood and vasculature and not negatively affect the associated tissues.12,32 Clinical management of a patient with EPP centers on the reduction of light exposure and the prevention of the painful photosensitivity associated with the disease. To that end, alterations to typical clinical procedures included flashless photography and a chemical rather than a light-cured adhesive; the office lights were turned off as much possible, and the windows near the treatment chair were covered to block the daylight. Interestingly, the fluorescent lights at the parent's office have been changed to a type emitting a less noxious wavelength, and the patient's school has ultraviolet restrictive window coverings. The patient's parents were hesitant to have radiographs taken, but there was a need to assess the skeletal, dental, and periodontal structures initially and during treatment. Upon consultation with the patient's physicians at British Columbia Children's Hospital and the physicians at the Canadian Porphyria Foundation,31 it was agreed that the wavelength of x-radiation (0.1-10 nm) seemed sufficiently distant from the ultraviolet and visible light spectrum encompassing the protoporphyrin absorption range (320-595 nm) to be safe. Radiographic exposure did not elicit an adverse reaction. Photosensitivity with EPP is selectively produced by specific wavelengths.12 As can be observed in the patient's clinical photographs, the limits of photography without flash are evident, and the use of a filter to absorb the specific wavelengths with future photography is under consideration. Light filters have been reported in the literature for use during hepatic transplantation surgery for a patient with EPP and tend to be yellow filters omitting the wavelengths below 470 nm; this offers a good balance between protection and altered color perception.33 In that case report, the specific filters were CLS-200-X and TA-81 from Madico (Woburn, Mass), Spergel #22 Deep Amber from Rosco (Stamford, Conn), and 61011 from Reflective SA (Cretil, France), all obtained from Ganta Trading (Oslo, Norway).33 However, the recommendations might vary depending on region, and these protective measures have been not accepted universally for all surgical procedures, such as laparoscopy and endoscopy.33 In the literature, we found no reported pathognomonic radiographic features of EPP at the time of consultation.12 Also, we found no reported bleeding concerns with the EPP patient or bleeding risks at the time of consultation.12 Pulp stones were evident on the initial panoramic radiograph and appeared to increase in size and radiopacity during treatment. Glass ionomer buildups were evident on the final panoramic radiograph but were subsequently removed. The frontal sinus was enlarged initially and appeared to increase in size on the final radiograph.
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The patient appeared to display a cervical vertebral maturation stage 4 by the end of treatment; this might indicate the completion of peak height velocity growth unless he is a true skeletal Class III.34,35 The difficulty of a patient with EPP to access outdoor activities might predispose him or her to an increased body mass index and forward or counterclockwise mandibular rotation.36-39 Treatment planning is made more difficult by the addition of this factor, especially when the patient displays a Class III tendency. However, our patient has a similar skeletal pattern to his parents, who are unaffected by EPP. Moreover, craniofacial growth could also be affected by thickening of the facial tissues or scarring that might occur with time over areas of skin exposed to sunlight.38,40-44 To further explore this issue, additional research is needed. CONCLUSIONS
1.
2.
The nonextraction orthodontic treatment of a patient with EPP appears to have a normal shortterm tissue response. Dental radiographic exposure did not result in a pathologic response.
REFERENCES 1. Lecha M, Herrero C, Ozalla D. Diagnosis and treatment of the hepatic porphyrias. Dermatol Ther 2003;16:65-72. 2. Gross U, Hoffmann GF, Doss MO. Erythropoietic and hepatic porphyrias. J Inherit Metab Dis 2000;23:641-61. 3. Lecha M, Puy H, Deybach JC. Erythropoietic protoporphyria. Orphanet J Rare Dis 2009;4:19. 4. Poh-Fitzpatrick MB. Clinical features of the porphyrias. Clin Dermatol 1998;16:251-64. 5. Lecha M. Erythropoietic protoporphyria. Photodermatol Photoimmunol Photomed 2003;19:142-6. 6. Magnus IA, Jarrett A, Prankerd TA, Rimington C. Erythropoietic protoporphyria. A new porphyria syndrome with solar urticaria due to protoporphyrinaemia. Lancet 1961;2:448-51. 7. Todd DJ. Erythropoietic protoporphyria. Br J Dermatol 1994;131: 751-66. 8. Rose L, Kaye D. Internal medicine for dentistry. 2nd ed. St. Louis: Mosby; 1990. 9. Murphy GM, Hawk JL. Erythropoietic protoporphyria advances today, with a special tribute to the late Professor Ian Magnus. Br J Dermatol 2006;155:501-3. 10. Wahlin S, Floderus Y, Stal P, Harper P. Erythropoietic protoporphyria in Sweden: demographic, clinical, biochemical and genetic characteristics. J Intern Med 2011;269:278-88. 11. Jensen NF, Fiddler DS, Striepe V. Anesthetic considerations in porphyrias. Anesth Analg 1995;80:591-9. 12. Lecha M. Personal communication; September 26, 2012. 13. Michaels BD, Del Rosso JQ, Mobini N, Michaels JR. Erythropoietic protoporphyria: a case report and literature review. J Clin Aesthet Dermatol 2010;3:44-8.
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14. Roberts W. Bone physiology, metabolism, and biomechanics in orthodontic practice. In: Graber TM, Vanarsdall RL, Vig KWL, editors. Orthodontics: current principles and techniques. St Louis: Elsevier Mosby; 2005. p. 221-92. 15. Roberts W, Huja S, Roberts J. Bone modeling: biomechanics, molecular mechanisms, and clinical perspectives. Semin Orthod 2004;10:123-61. 16. Casanova-Gonzalez MJ, Trapero-Marugan M, Jones EA, MorenoOtero R. Liver disease and erythropoietic protoporphyria: a concise review. World J Gastroenterol 2010;16:4526-31. 17. Cripps DJ, Goldfarb SS. Erythropoietic protoporphyria: hepatic cirrhosis. Br J Dermatol 1978;98:349-54. 18. Kooijman MM, Brand HS. Oral aspects of porphyria. Int Dent J 2005;55:61-6. 19. Bjork A, Helm S. Prediction of the age of maximum pubertal growth in body height. Angle Orthod 1967;37:134-43. 20. Chapman SM. Ossification of the adductor sesamoid and the adolescent growth spurt. Angle Orthod 1972;42:236-44. 21. Gu Y, McNamara JA. Mandibular growth changes and cervical vertebral maturation. a cephalometric implant study. Angle Orthod 2007;77:947-53. 22. O'Reilly MT, Yanniello GJ. Mandibular growth changes and maturation of cervical vertebrae—a longitudinal cephalometric study. Angle Orthod 1988;58:179-84. 23. Baccetti T, Franchi L, McNamara JA Jr. An improved version of the cervical vertebral maturation (CVM) method for the assessment of mandibular growth. Angle Orthod 2002;72:316-23. 24. Jarvinen S. Relation of the SNA angle to the saddle angle. Am J Orthod 1980;78:670-3. 25. Jarvinen S. Saddle angle and maxillary prognathism: a radiological analysis of the association between the NSAr and SNA angles. Br J Orthod 1984;11:209-13. 26. Jarvinen S. Relation of the SNA angle to the NSAr angle in excellent occlusion and in malocclusion. Am J Orthod 1982;81:245-8. 27. Anderson I, Rabie AB, Wong RW. Early treatment of pseudo-class III malocclusion: a 10-year follow-up study. J Clin Orthod 2009; 43:692-8. 28. H€agg U, Tse A, Bendeus M, Rabie AB. A follow-up study of early treatment of pseudo Class III malocclusion. Angle Orthod 2004;74:465-72. 29. Rabie AB, Gu Y. Diagnostic criteria for pseudo-Class III malocclusion. Am J Orthod Dentofacial Orthop 2000;117:1-9.
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30. Rabie AB, Gu Y. Management of pseudo Class III malocclusion in southern Chinese children. Braz Dent J 1999;186: 183-7. 31. Canadian Porphyria Foundation. Neepawa, Manitoba, Canada. Available at: www.cpf-inc.ca; accessed October 13, 2010. 32. Lim HW, Poh-Fitzpatrick MB, Gigli I. Activation of the complement system in patients with porphyrias after irradiation in vivo. J Clin Invest 1984;74:1961-5. 33. Wahlin S, Srikanthan N, Hamre B, Harper P, Brun A. Protection from phototoxic injury during surgery and endoscopy in erythropoietic protoporphyria. Liver Transpl 2008;14:1340-6. 34. Baccetti T, Franchi L, McNamara JA. Growth in the untreated Class III subject. Semin Orthod 2007;13:130-42. 35. Mitani H, Sato K, Sugawara J. Growth of mandibular prognathism after pubertal growth peak. Am J Orthod Dentofacial Orthop 1993; 104:330-6. 36. Ferrario VF, Dellavia C, Tartaglia GM, Turci M, Sforza C. Soft tissue facial morphology in obese adolescents: a three-dimensional noninvasive assessment. Angle Orthod 2004;74:37-42. 37. Ohrn K, Al-Kahlili B, Huggare J, Forsberg CM, Marcus C, Dahllof G. Craniofacial morphology in obese adolescents. Acta Odontol Scand 2002;60:193-7. 38. Standerwick RG, Roberts WE. The aponeurotic tension model of craniofacial growth in man. Open Dent J 2009;3:100-13. 39. Sadeghianrizi A, Forsberg CM, Marcus C, Dahllof G. Craniofacial development in obese adolescents. Eur J Orthod 2005;27: 550-5. 40. Suurmond D, van Steveninck J, Went LN. Some clinical and fundamental aspects of erythropoietic protoporphyria. Br J Dermatol 1970;82:323-8. 41. Nahlieli O, Kelly JP, Baruchin AM, Ben-Meir P, Shapira Y. Oromaxillofacial skeletal deformities resulting from burn scar contractures of the face and neck. Burns 1995;21:65-9. 42. Kung TA, Gosain AK. Pediatric facial burns. J Craniofac Surg 2008; 19:951-9. 43. Houston WJ. Mandibular growth rotations—their mechanisms and importance. Eur J Orthod 1988;10:369-73. 44. Solow B, Kreiborg S. Soft-tissue stretching: a possible control factor in craniofacial morphogenesis. Scand J Dent Res 1977;85: 505-7.
American Journal of Orthodontics and Dentofacial Orthopedics
Orthodontic treatment considerations for a patient with erythropoietic protoporphyria Richard G. Standerwick,a Edwin H. K. Yen,b and Benjamin Pliskac Vancouver, British Columbia, Canada Erythropoietic protoporphyria (EPP) is an inherited blood disorder in which formation of the heme group of hemoglobin is defective. Specifically, a deficiency of the enzyme ferrochelatase leads to the accumulation of protoporphyrin, resulting in often painful photosensitivity of the skin and tissues. The prevalence of EPP is estimated at 1:75,000 to 1:200,000. Photosensitivity is exhibited upon exposure to light with specific wavelengths through the creation of reactive oxygen products (oxidants), activation of the complement system, and mast cell degranulation. The aim of this article is to report the orthodontic treatment of an 11-year-old boy with EPP, a Class III skeletal relationship, and an anterior crossbite. Orthodontic treatment established normal overbite and overjet. Short-term periodontal and dental tissue responses to treatment were noted. Extra care was needed when collecting photographic and radiographic records for this patient and during some treatment procedures to avoid causing a photosensitive reaction of the skin or oral mucosa. (Am J Orthod Dentofacial Orthop 2013;144:899908)
P
orphyrias are a group of metabolic disorders resulting from defective porphyrin and heme synthesis. They are classified as erythropoietic or hepatic, depending on which organ site expresses the defect in heme synthesis; by the clinical manifestations of cutaneous photosensitivity; or by neurovisceral symptoms.1-4 Erythropoietic protoporphyria (EPP) was first described by Kosenow and Treibs (as cited in Lecha5) and further by Magnus et al6 and is a subset of the erythropoietic porphyrias with a prevalence between 1:75,000 and 1:200,000.2,3,5,7,8 EPP is an inherited disorder of the heme metabolic pathway characterized by accumulation of protoporphyrin in blood, erythrocytes, and tissues, resulting in cutaneous manifestations of photosensitivity.3,7,9,10 The accumulation of protoporphyrin is a result of a deficiency in the ferrochelatase enzyme.2,3 Protoporphyrin accumulates in red blood cells and their precursors and eventually diffuses out of the red blood From the Department of Orthodontics, Faculty of Dentistry, University of British Columbia, Vancouver, British Columbia, Canada. a Associate clinical professor; private practice, Langley, British Columbia, Canada. b Professor and dean emeritus. c Assistant professor. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported. Address correspondence to: Richard G. Standerwick, 20159 88th Ave, Suite E207, Langley, BC, Canada V1M0A4; e-mail, richard@transformationorthodontics. com. Submitted, January 2013; revised and accepted, February 2013. 0889-5406/$36.00 Copyright Ó 2013 by the American Association of Orthodontists. http://dx.doi.org/10.1016/j.ajodo.2013.02.032
cells into the plasma as the red blood cells mature.8 Excessive protoporphyrin accumulates in the vascular and perivascular areas and absorbs light energy at 320 to 595 nm on the exposed areas of skin.3,5,7 Peak absorption is in the range of 400 to 410 nm (the Soret band).7 The energy absorption causes the formation of reactive oxygen products, the activation of the complement system, direct and indirect degranulation of mast cells, and chemotaxis of polymorphonuclear leucocytes.3,5,7,11 EPP typically first presents symptoms in early infancy after sun exposure; it is characterized by cutaneous manifestations of acute painful photosensitivity leading to erythema and edema. The severity of the disorder varies significantly, from extreme stinging, itching, and burning sensations to petechiae and, uncommonly, blistering.2-4,8,12 Affected children might cry and scream for hours, often throughout the night, without an obvious reason.7 The pain has been described as like “a lighted match being held against your skin”; a 6-year-old child, after crying for a full weekend, asked his mother to “please cut my hands off and let me go to sleep.”7 Recurrent episodes of unexplained hand and facial swelling can warrant further investigation.13 It is often diagnostic that the affected person attempts to relieve the pain by applying cold water to the skin.7,13 Because there is no cure for EPP, treatment is focused on prevention and symptom management. Exposure to light within the noxious range should be avoided, with protective clothing and sunscreens to limit photosensitivity. Even sunlight through window glass can precipitate 899
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photosensitivity.8 Other therapies to relieve photosensitivity have been tried, but most lack studies that definitively show clinical relevance7; however, cholestyramine therapy might be helpful to increase fecal excretion of protoporphyrin.8 Because of the lack of sunlight exposure and therefore decreased endogenous vitamin D production, supplementation of vitamin D (1,25 dihydroxycholecalciferol) is required to prevent deficiency and to maintain normal bone metabolism and homeostasis.10,14,15 Protoporphyrin blood concentrations and liver function are monitored with regular blood testing, and appearance is assessed for jaundice.3,5,7 Protoporphyrin is not water soluble; therefore, excretion is facilitated by its solubilization in the liver followed by biliary secretion.7,16 When the protoporphyrin solubilizing capacity of the liver is exceeded, the result can be hepatic failure, which occurs in approximately 10% of affected persons3,5,7; this can be fatal without prompt liver transplantation.7,17 During clinical procedures, operating and room lighting must be filtered to avoid inducing severe burns.18 Dental curing lights emit light wavelengths of approximately 480 nm and should also be considered a potential danger to EPP patients. This case report describes the nonextraction orthodontic treatment and management of a patient with EPP. DIAGNOSIS AND ETIOLOGY
The patient, a boy, age 11 years 10 months, sought an orthodontic consultation for his anterior crossbite. His medical history included EPP, allergy to penicillin, and concerns about medications because of the hepatic and kidney associations with EPP. He took vitamin D daily. No liver issues were diagnosed before or during treatment. His notable facial attributes included a concave facial profile, a canted mandibular lip on smiling with otherwise normal facial symmetry and facial thirds, a functional shift of the mandible (1 mm anteriorly and 2-3 mm superiorly) (Fig 1). Notable dental attributes included a super Class I molar relationship, a partial anterior crossbite, a 1.8-mm overjet, a 3-mm overbite, adequate mandibular and inadequate maxillary arch lengths, and good dental hygiene (Fig 2). The cephalometric radiographs were analyzed using software (version 11.5; Dolphin Imaging and Management Solutions, Chatsworth, Calif). The adductor sesamoid was present on the hand-wrist radiograph, and the patient appeared to display a cervical vertebral maturation stage 2; therefore, it was likely that pubertal growth was imminent or already attained (Figs 3-5).19-23 The skeletal analysis involved components from the Indiana University School of Dentistry, American Board
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of Orthodontics, Bjork, Bjork 2, Michigan Surgery, and Litt Biodynamic analyses (version 11.5; Dolphin Imaging and Mangement Solutions). This variable analysis was used because of the short anterior cranial base, the altered position of nasion, and the decreased angulation of the S-N line because any cephalometric measurement associated with nasion was most likely skewed. The skeletal attributes included a hypodiverent tendency, normal lower face height, decreased ANB and A-B/OP (Wits), and increased SNA and SNB. The relative rather than literal values of SNA and SNB were considered because of the altered position of nasion and the decreases in sella-nasion-basion and SN-articulare angulation. A decreased SN-Ba angulation tends to translate into increased values for SNA and SNB.24-26 The maxillary position determined by condylion to anterior nasal spine displayed maxillary retrusion, whereas condylion to pogonion had a normal mandibular position. The maxillary incisors exhibited a decreased inclination relative to the palatal plane and an increased inclination relative to the Frankfort horizontal. The mandibular incisors showed retroclination and increased protrusion relative to the A-point–pogonion line (Table 1). No clicking or pain was reported in the right or left temporomandibular joint. The maximum opening was slightly restricted at 39 mm for a soft end stop, and maximum opening was 49 mm. Lateral excursive and protrusive movements were normal (8-10 mm); the mandible deviated to the left on closure. Tooth and condylar morphologies on the panoramic radiograph showed no overt pathology. TREATMENT OBJECTIVES
The maxillary objectives were to maintain the maxilla in the anteroposterior, vertical, and tranverse dimensions. The mandibular objectives were to decrease the anteroposterior protrusion, increase the vertical dimension possibly reducing anteroposterior protrusion, and maintain the transverse dimension. The maxillary molar position was to be maintained in the anteroposterior, vertical, and transverse dimensions. The maxillary incisor objectives included increases in incisor protrusion and inclination, and maintenance of vertical position. Mandibular molar and incisor anteroposterior and vertical positions were to be maintained. Maxillary molar, mandibular molar, and mandibular canine widths were to be maintained. The treatment objectives included alignment of the dental arches and establishment of a Class I molar relationship and normal overbite and overjet. Facial convexity was expected to increase with elimination of the mandibular functional shift and correction of the anterior crossbite.
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Fig 1. Pretreatment photographs, age 12 year 7 months. Patients with EPP must limit their exposure to light to prevent painful photosensitivity; therefore, records were collected with flashless photography.
Fig 2. Pretreatment dental casts.
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Fig 3. Hand-wrist radiograph at age 12 years 7 months. The radiographs were collected sequentially, 1 to 2 months apart. The least vascular structures were examined first to assess the patient's response.
Fig 5. Cephalometric radiograph at age 12 years 10 months.
that pseudo-Class III patients display maxillary retrusion caused by functional inhibition of growth; therefore, catch-up growth or restoration of the normal growth pattern can be expected after the establishment of normal overbite and overjet.27-30 TREATMENT PROGRESS
Fig 4. Panoramic radiograph at age 12 years 9 months.
TREATMENT ALTERNATIVES
The treatment alternatives included no treatment, surgical treatment, extraction of permanent teeth, or a nonextraction approach. The proclination of the maxillary incisors, increased A-point to pogonion, and Class III tendency might have favored an extraction or surgical approach depending on the weight given to selected factors. Also considered were tissue reactions relative to extraction, space closure, and orthodontic treatment in general, since they were unknown for this genetic condition. A nonextraction treatment plan was chosen based on the clinical presentation of the mandible relative to the maxilla in centric relation and the maxillary incisor inclination relative to the palatal plane. It has been shown
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Informed consent was obtained from the patient and his parents before treatment and to allow consultation with the patient's physicians. The primary care physician at British Columbia Children's Hospital and the consulting physician for the Canadian Porphyria Foundation31 were consulted. The consensus was that radiographic exposure should be fine. A hand-wrist radiograph and panoramic and lateral cephalometric radiographs were exposed sequentially a month apart to allow observation of any resulting symptoms (exposure of the least vascular structures first). The maxillary and mandibular arches were indirectly bonded with the Sondhi indirect bonding kit (3M Unitek, Monrovia, Calif) and allowed to chemically cure. The orthodontic appliance was a 0.022-in slot bracket with MBT prescription (3M Unitek). A modified Gelb mandibular appliance was used to disarticulate the teeth (2-mm Biocryl 021-027; Great Lakes Orthodontics, Tonawanda, NY). Wire sequencing was 0.014in nickel-titanium, 0.018-in copper-nickel-titanium, 0.019 3 0.025-in copper-nickel-titanium, 0.019 3 0.025-in beta-titanium, and 0.019 3 0.025-in stainless steel (G&H Wire, Franklin, Ind). At some point after the
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Table 1. Cephalometric measurements Skeletal measurements N-A-Pg ( ) N-A jj HP (mm) N-B jj HP (mm) N-Pg jj HP (mm) A-B jj HP (mm) A-B jj OP (mm) FH-SN ( ) SNA ( ) SNB ( ) ANB ( ) OP-HP ( ) PP-HP ( ) MP-HP ( ) Palatal-maxillary occlusal plane ( ) Mandibular occlusal-mandibular plane ( ) Chin angle (Id-Pg-MP) ( ) Y-axis (S-Gn-FH) ( ) Gonial/jaw angle (Ar-Go-Me) ( ) Chin angle (Id-Pg-MP) ( ) Anterior cranial base (SN) (mm) Posterior cranial base (S-Ar) (mm) Ramus height (Ar-Go) (mm) Length of mandibular base (Go-Pg) (mm) Maxillary unit length (Co-ANS) (mm) Mandibular unit length (Co-Pog) (mm) Harvold (CoPog)-(CoANS) ( ) Maxillary skeletal (A-Na perp) (mm) Mandibular skeletal (Pg-Na perp) (mm) MP-SN ( ) Upper face height (N-ANS) (mm) Lower face height (ANS-Me) (mm) Upper face height (N-ANS/[N-ANS1ANS-Me]) (%) Lower face height (ANS-Me/[N-ANS1ANS-Me]) (%) Ba-S-N ( ) SN-Ar ( ) Dental measurements 1/(HP) ( ) /1(HP) ( ) 1/-(UL) (mm) IMPA/1 (MP) ( ) Interincisal angle (U1-L1) ( ) /1(A-Pg) (mm) /1(N-Pg) (mm) U1-SN ( ) U1-ANS (mm) U6-PP (UPDH) (mm) L1-MP (LADH) (mm) U1-Me (mm) L6-MP (LPDH) (mm) Pr-N-A ( ) U1-palatal plane ( ) U1-NA (mm) GoGn-chin line ( ) L1-GoGn ( ) L1-NB (mm) Pg-NB (mm) Overjet (mm) Overbite (mm)
Pretreatment
Posttreatment
Mean
SD
Dev Normal
8 3.6 13.4 13.8 9.8 5.5 2.1 87.1 91.7 4.6 6.6 13.3 14.3 9.9 24.6 74.8 50.1 131.2 74.8 68.6 38.4 50.4 75.3 83.1 113.4 30.3 0.7 5.4 21.3 45.4 61.7 42.4 57.6 124.7 120.1
9.5 4.5 16.8 18.4 12.2 6.4 2 88.2 93.8 5.7 8.7 13 14.2 6.1 24.5 73.9 49.5 130.5 73.9 69.6 39 53.8 82.7 82.8 116.8 34.1 0.1 9.3 21.2 46.9 65.5 41.7 58.3 124.1 119.5
5.5 2 8.5 9 6.5 0.5 6 82 80 2 9 0.5 25 10 20 70 60 124.4 70 73.9 33.5 45.3 73 90 113 20 0 4 33 NA NA 43 57 130 124
4.5 3.5 4.5 5 2.5 2 4 3 4 1.5 2.5 3 5 4 5 5 3 6.7 5 3 4 4.5 3 5 8 3 3.1 5.3 6 NA NA 100 100 100 5
3 1.6 4.9 4.6 6.5 2.5 1 1.7 2.9 4.4 6.2 4.6 2.1 0 0.9 1 3.3 1 1 1.7 1.2 1.1 0.8 1.4 0.1 3.4 0.2 1.8 1.9 NA NA 0 0 0.1 0.8
119.6 78.6 2.4 87.1 139 5.4 3.2 112.6 26 21 39.5 5.7 27.4 3.9 106.3 6.1 78.1 90.3 2.3 1.2 1.9 3.6
133.8 80 0.2 85.8 126.2 6.2 3.5 126.8 26.6 22.3 42 5.6 30.7 7 120.8 11.4 76.4 88.3 2.6 1.3 1.7 1.8
110 60 2.5 90 130 2.5 3.5 102.6 30 20 40 35 30 2 110 4 70 94 4 NA 2.5 2.5
6.5 4.5 1 5 6 2 2.5 5.5 5 3 3 3 3 1 5 2.7 5 7 1.8 NA 2.5 2
1.5 4.1 0.1 0.6 1.5 1.4 0.1 1.8 0.8 0.3 0.2 9.8 0.9 1.9 0.7 0.8 1.6 0.5 0.9 NA 1.8 0.5
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Table 1. Continued Soft-tissue measurements Facial convexity (G0 -Sn-Po0 ) ( ) UL protrusion (UL-SnPg0 ) (mm) LL protrusion (LL-SnPg0 ) (mm) UL to E-plane (mm) LL to E-plane (mm) ILG (HP) (mm)
Pretreatment
Posttreatment
Mean
SD
Dev Normal
1.5 2.9 5.7 5.2 0.2 1.2
4.1 2.5 3.5 6.2 2.5 0.7
13.5 3.5 2.8 3 2.5 NA
5.5 1.8 1.7 2 2.5 NA
2.7 0.3 1.7 1.1 1.1 NA
Relative rather than literal values of SNA and SNB were considered because of the altered position of N and the decreases in SN-Ba and SN-Ar angulations. Pretreatment measurements, age 12 years 7 months; final measurements, age 14 years 0 months. Dev Normal, Standard deviation from normal; HP, horizontal plane/Frankfort horizontal; OP, occlusal plane; FH, Frankfort horizontal; PP, palatal plane; MP, mandibular plane; UPDH, upper posterior dental height; LADH, lower anterior dental height; LPDH, lower posterior dental height; UL, upper lip; LL, lower lip; ILG, interlabial gap; NA, not applicable.
Fig 6. Posttreatment photographs at age 14 years 0 months.
bonding appointment, the modified Gelb fractured and was not reported until the patient returned with multiple debonded brackets. The loose brackets were rebonded with glass ionomer cement (Fuji Ortho; GC Corporation, Tokyo, Japan). The self-curing time of the cement was 10 minutes. Bite buildups were placed on the mandibular permanent first molars with the glass ionomer cement to eliminate compliance issues. A panoramic radiograph was taken 4 months after the initial bonding to evaluate periodontal structures and root position. During leveling with the 0.019 3 0.025-in beta-titanium wire, the glass ionomer buildups were removed to allow eruption of the posterior teeth; immediately, an acrylic appliance with an anterior biteplate was fabricated. Class III 3/16-in, 6-oz
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elastics were added from the maxillary permanent first molars to the mandibular canines. Compliance with the biteplate appliance and elastics was low. Throughout treatment, there were also many rebondings of brackets because of debonds between the bracket and cement. Impressions were made for study models, and the buildups were removed. Elastics were changed to Class III 1/4-in, 3.5 oz elastics from the maxillary first molars to Kobayashi hooks on the mandibular lateral incisors. Vertical elastics (3/16-in, 3.5 oz) were worn from the maxillary first and second premolars to the mandibular first and second premolars. At debond, temporary Invisacryl C (021-056; Great Lakes Orthodontics) retainers were delivered, and final
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Fig 7. Posttreatment dental casts.
Fig 8. Posttreatment panoramic radiograph at age 14 years 0 months. Pulp stones were evident on the initial panoramic radiograph and appeared to increase in size and radiopacity during treatment. Glass ionomer buildups are evident on the final panoramic radiograph but were subsequently removed.
records were obtained. Maxillary and mandibular Hawley retainers were inserted after 1 month (becaue of the Christmas holidays rather than by intention). The retainer was evaluated at 1 week, 1 month, 3 months, and 6 months, and scheduled yearly or as needed. TREATMENT RESULTS
There were no reported adverse symptoms from the radiographic exposure. Tooth movement appeared to be normal, and no gross abnormal signs of external apical root resorption were evident. In general, the
periodontal tissues appeared to tolerate the orthodontic manipulation well (Figs 6-9). The maxilla grew anteriorly; however, the relative position of SNA was maintained in the anteroposterior dimension. The maxilla was basically maintained vertically, with a slight forward or counterclockwise rotation, and the transverse dimension was maintained. The mandible displayed increases in protrusion (SNB and Co-Pg) and the vertical dimension, most likely due to growth, whereas the relative vertical dimension was maintained. The mandible displayed approximately 2 of forward and counterclockwise rotation. The final position of the mandibular condyles on the lateral cephalogram might have been affected by head position in the cephalostat (pterygoid fissures are not superimposed well on the final cephalogram) or might have displayed an increase in growth asymmetry. The maxillary molar position displayed protraction and extrusion and was maintained in the transverse dimension. The maxillary incisors displayed increases in protrusion and inclination, and the vertical dimension was maintained. The mandibular molar and incisor positions were maintained in the anteroposterior dimension, and they displayed increases in the vertical dimension and extrusion. Maxillary molar, mandibular molar, and canine widths were maintained. After active orthodontic treatment, dental alignment, a Class I molar relationship, and normal overbite and overjet were established (Figs 10 and 11, Table 1).
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Fig 9. Posttreatment cephalometric radiograph at age 14 years 0 months. The final position of the mandibular condyles on the lateral cephalogram might have been affected by head position in the cephalostat (pterygoid fissures are not superimposed well on the final cephalogram) or might be caused by an increase in growth asymmetry.
Fig 10. Craniofacial superimpositions: 12 years 10 months (black) and 14 years 0 months (red). The maxilla grew anteriorly, but the relative position of SNA was maintained in the anteroposterior dimension. The maxilla was basically maintained vertically and displayed a slight forward or counterclockwise rotation. The transverse dimension was maintained. The mandible displayed increases in protrusion (SNB and Co-Pg) and the vertical dimension, likely due to growth, whereas the vertical transverse dimension was maintained.
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Fig 11. Regional superimpositions: 12 years 10 months (black) and 14 years 0 months (red). The maxilla displayed a slight forward or counterclockwise rotation, and the mandible displayed approximately 2 of forward or counterclockwise rotation. The maxillary molar position displayed protraction and extrusion and was maintained in the transverse dimension. The maxillary incisors displayed increases in protrusion and inclination, and the vertical dimension was maintained. The mandibular molar and incisor positions were maintained in the anteroposterior dimension, with an increase in the vertical dimension with extrusion. DISCUSSION
To our knowledge, there have been no published articles discussing orthodontic treatment of a patient with EPP. Whether protoporphyrin would accumulate in the bone or tissues and how that might affect tissue reactions were unknown. However, further postorthodontic consultation seems to indicate that the
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protoporphyrin would remain in the blood and vasculature and not negatively affect the associated tissues.12,32 Clinical management of a patient with EPP centers on the reduction of light exposure and the prevention of the painful photosensitivity associated with the disease. To that end, alterations to typical clinical procedures included flashless photography and a chemical rather than a light-cured adhesive; the office lights were turned off as much possible, and the windows near the treatment chair were covered to block the daylight. Interestingly, the fluorescent lights at the parent's office have been changed to a type emitting a less noxious wavelength, and the patient's school has ultraviolet restrictive window coverings. The patient's parents were hesitant to have radiographs taken, but there was a need to assess the skeletal, dental, and periodontal structures initially and during treatment. Upon consultation with the patient's physicians at British Columbia Children's Hospital and the physicians at the Canadian Porphyria Foundation,31 it was agreed that the wavelength of x-radiation (0.1-10 nm) seemed sufficiently distant from the ultraviolet and visible light spectrum encompassing the protoporphyrin absorption range (320-595 nm) to be safe. Radiographic exposure did not elicit an adverse reaction. Photosensitivity with EPP is selectively produced by specific wavelengths.12 As can be observed in the patient's clinical photographs, the limits of photography without flash are evident, and the use of a filter to absorb the specific wavelengths with future photography is under consideration. Light filters have been reported in the literature for use during hepatic transplantation surgery for a patient with EPP and tend to be yellow filters omitting the wavelengths below 470 nm; this offers a good balance between protection and altered color perception.33 In that case report, the specific filters were CLS-200-X and TA-81 from Madico (Woburn, Mass), Spergel #22 Deep Amber from Rosco (Stamford, Conn), and 61011 from Reflective SA (Cretil, France), all obtained from Ganta Trading (Oslo, Norway).33 However, the recommendations might vary depending on region, and these protective measures have been not accepted universally for all surgical procedures, such as laparoscopy and endoscopy.33 In the literature, we found no reported pathognomonic radiographic features of EPP at the time of consultation.12 Also, we found no reported bleeding concerns with the EPP patient or bleeding risks at the time of consultation.12 Pulp stones were evident on the initial panoramic radiograph and appeared to increase in size and radiopacity during treatment. Glass ionomer buildups were evident on the final panoramic radiograph but were subsequently removed. The frontal sinus was enlarged initially and appeared to increase in size on the final radiograph.
907
The patient appeared to display a cervical vertebral maturation stage 4 by the end of treatment; this might indicate the completion of peak height velocity growth unless he is a true skeletal Class III.34,35 The difficulty of a patient with EPP to access outdoor activities might predispose him or her to an increased body mass index and forward or counterclockwise mandibular rotation.36-39 Treatment planning is made more difficult by the addition of this factor, especially when the patient displays a Class III tendency. However, our patient has a similar skeletal pattern to his parents, who are unaffected by EPP. Moreover, craniofacial growth could also be affected by thickening of the facial tissues or scarring that might occur with time over areas of skin exposed to sunlight.38,40-44 To further explore this issue, additional research is needed. CONCLUSIONS
1.
2.
The nonextraction orthodontic treatment of a patient with EPP appears to have a normal shortterm tissue response. Dental radiographic exposure did not result in a pathologic response.
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