Key Points
Question
Can 3-dimensional (3D) stereophotogrammetry be used to detect disease progression in patients with craniofacial morphea (CM)?
Findings
In this cohort study of 27 patients with CM, participants underwent clinical and 3D stereophotogrammetry assessments at 2- to 12-month intervals. Over the course of 48 months, 10 patients demonstrated clinical progression of CM, which was corroborated by 3D stereophotogrammetry assessment by an expert and nonexpert with substantial interrater reliability.
Meaning
These findings suggest that noninvasive 3D stereophotogrammetry may serve as a valuable adjunctive tool for detecting disease progression in CM over time.
This cohort study assesses whether tracking 3-dimensional stereophotogrammetry–generated facial heat maps over time could illustrate clinically meaningful disease progression in patients with craniofacial morphea.
Abstract
Importance
Objectively determining disease progression in craniofacial morphea (CM) is challenging, as clinical findings of disease activity are often lacking.
Objective
To evaluate the utility of 3-dimensional (3D) stereophotogrammetry in detecting disease progression in CM over time.
Design, Setting, and Participants
This prospective cohort study included 27 pediatric and adult patients with CM from 2 hospitals in Boston (Boston Children’s Hospital and Brigham & Women’s Hospital) consecutively enrolled from April 1, 2019, to March 1, 2023. Review of 3D stereophotogrammetry images and data analysis occurred from March 1 to April 1, 2023.
Main Outcomes and Measures
Clinical and 3D stereophotogrammetry assessments were performed at 2- to 12-month intervals, depending on the clinical context. The 3D stereophotogrammetry images were then qualitatively rated as demonstrating no progression or definitive progression by an expert (board-certified plastic craniofacial surgeon) and nonexpert (board-certified dermatologist) in 3D stereophotogrammetry. In addition, κ coefficients were calculated for interrater reliability.
Results
Of 27 patients with CM (19 female; median age, 14 [range, 5-40] years) and 3D stereophotogrammetry images obtained from a minimum of 2 time points (median, 4 [range, 2-10] images) spaced a median of 3 (range, 2-12) months apart, 10 experienced progression of their disease based on clinical assessments performed during the study period. In all cases in which clinical progression was favored, blinded qualitative assessment of 3D stereophotogrammetry images also favored progression with substantial interrater reliability (κ = 0.80 [95% CI, 0.61-0.99]). Furthermore, review of 3D stereophotogrammetry detected occult progression of asymmetry not noted on clinical examination in 3 additional patients.
Conclusions and Relevance
In this prospective cohort study, blinded assessment of sequential 3D stereophotogrammetry images in patients with CM not only corroborated clinical assessment of disease progression but also detected occult progression of facial asymmetry not appreciable on clinical examination alone. Therefore, 3D stereophotogrammetry may serve as a useful adjunct to clinical examination of patients with CM over time. Future investigations are warranted to validate 3D stereophotogrammetry as an outcome measure in CM.
Introduction
Craniofacial morphea (CM) is an autoimmune disorder characterized by inflammation and subsequent atrophy of the skin and underlying soft tissue. While predominantly manifesting in the pediatric population as progressive hemifacial atrophy and/or en coup de sabre, adult-onset CM can also occur.1 In both instances, systemic immunosuppression is critical to abrogate disease activity and avoid significant cosmetic and functional sequelae. Even with appropriate treatment, risk for disease recurrence persists, with rates of 28% to 44% reported in the literature.2,3 Unfortunately, determining disease progression in CM remains challenging, as clinical findings of disease activity are often lacking. Existing tools include 2-dimensional (2D) photography, clinical documentation, and clinician and/or patient recall, all of which are limited in their ability to detect subtle evolving facial atrophy. A critical need for more sensitive and accurate ways to monitor patients with CM longitudinally persists.4
Three-dimensional (3D) stereophotogrammetry is a noninvasive, radiation-free imaging modality that has emerged as a potential tool for detecting and quantifying asymmetry in CM.4,5 Although well-known in the plastic surgery community as a validated means by which to evaluate the success of reconstructive procedures in the setting of facial trauma and other craniofacial disorders, the adoption of 3D stereophotogrammetry by the dermatology community has lagged behind.6 A recent pilot study4 demonstrated the ability of 3D stereophotogrammetry to detect pathologic asymmetry in patients with CM, including occult asymmetry not appreciable on clinical examination results alone. While the pilot study did not follow up patients longitudinally to detect disease progression or recurrence, we hypothesized that tracking 3D stereophotogrammetry–generated facial heat maps over time could illustrate clinically meaningful disease progression in patients with CM.
Methods
We performed a prospective study of 27 consecutive patients seen at Boston Children’s Hospital (BCH) and Brigham & Women’s Hospital outpatient rheumatology-dermatology clinics, Boston, Massachusetts, from April 1, 2019, to March 1, 2023. This study was approved by the BCH Institutional Review Board. All participants were referred to BCH for serial 2D photography and 3D stereophotogrammetry and provided written informed consent or assent. This study followed the Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) reporting guideline.
Three-dimensional facial photographs were taken using a 5-camera 3D imaging system (VECTRA M3, version 6.2.3; Canfield Scientific, Inc). Using a flip-registration procedure, the unaffected half of a patient’s face was reflected onto the affected half, enabling volumetric differences to display as a topographical heat map on a 4.5- to 8.0-mm gradient scale (Figure 1 and eMethods in Supplement 1).7 Patients met inclusion criteria with a diagnosis of CM based on expert opinion and/or biopsy for whom 3D stereophotogrammetry images were available from at least 2 points no more than 12 months apart. Electronic health records were reviewed from March 1 to April 1, 2023, for demographic characteristics, clinical features, treatment data, and documentation of disease recurrence or progression (Table 1). A board-certified dermatologist (K.S.S) and a board-certified craniofacial plastic surgeon (I.M.G.), both blinded to the clinical outcomes of patients, independently reviewed sequentially taken 3D stereophotogrammetry images to assess for progressive facial asymmetry. Statistical significance was assessed with κ coefficients calculated for interrater reliability in R, version 4.2.0, using the psych package, version 2.3.3 (R Program for Statistical Computing).
Figure 1. Flip-Registration Procedure Used to Create Topographical Heat Maps Highlighting Volumetric Differences and Asymmetry in Patients With Craniofacial Morphea (CM).
A flip-registration analysis was performed at each individual time point using built-in VECTRA face analysis software (VECTRA, version 6.2.3; Canfield Scientific Inc). In this representative patient with the en coup de sabre variant of CM, the entire area of the face was first selected (A). The face was then aligned symmetrically according to the user-defined midline of the face (B), which was identified by drawing a singular straight line from the midline of the nose down to the chin and up to the top of the forehead. A mirrored image of the unaffected side of the face was then reflected (x = 0) and overlayed on the affected side of the face (C). Distances between the affected and unaffected sides of the face were calculated using the VECTRA software to create topographical heat maps in a frontal view (D), 45° view (E), and lateral view (F).
Table 1. Clinical and Demographic Characteristics and Treatment History of Patients With CM Monitored Longitudinally With 3D Stereophotogrammetry.
| Variable | Data (N = 27)a |
|---|---|
| Age, median (range), yb | 14 (5-40) |
| Pediatric | 22 (81.5) |
| Adult | 5 (18.5) |
| Sex | |
| Female | 19 (70.4) |
| Male | 8 (29.6) |
| Race, ethnicity, and/or ancestryc | |
| American Indian or Alaska Native | 0 |
| Asian | 4 (14.8) |
| Black or African American | 1 (3.7) |
| Hispanic or Latino | 3 (11.1) |
| Pacific Islander or Native Hawaiian | 0 |
| White | 19 (70.4) |
| CM subtype | |
| Linear morphea (en coup de sabre) | 10 (37.0) |
| Progressive hemifacial atrophy (Parry Romberg) | 13 (48.1) |
| Both | 4 (14.8) |
| Disease activity at time of baseline 3D stereophotogrammetryd | |
| Active | 14 (51.9) |
| Initial episode | 8 (29.6) |
| Disease flare and/or recurrence | 6 (22.2) |
| Inactive | 13 (48.1) |
| Treatment at time of baseline 3D stereophotogrammetry | |
| Methotrexate only | 6 (22.2) |
| Mycophenolate mofetil only | 3 (11.1) |
| Systemic corticosteroids and methotrexatee | 5 (18.5) |
| Systemic corticosteroids and mycophenolate mofetile | 1 (3.7) |
| Methotrexate and mycophenolate mofetil | 1 (3.7) |
| Topical corticosteroids or immunomodulatory agents | 0 |
| Nonef | 11 (40.7) |
Abbreviations: CM, craniofacial morphea; 3D, 3-dimensional.
Unless otherwise indicated, data are expressed as No. (%) of patients. Percentages have been rounded and may not total 100.
Indicates at the time of baseline 3D stereophotogrammetry.
Classified by parent or self-reporting as documented in the electronic medical record.
Determined by clinical assessment. Categories are not mutually exclusive.
Includes intravenous or oral methylprednisolone.
Of those patients not receiving therapy at the time of baseline 3D imaging, 6 had previously completed a standardized treatment course of systemic corticosteroids and methotrexate or mycophenolate mofetil using Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans,8 and 4 obtained baseline 3D imaging on the day of CM diagnosis prior to the initiation of treatment.
Results
A total of 27 patients (19 female and 8 male; median age, 14 [range, 5-40] years) with CM and 3D stereophotogrammetry images from a median of 4 (range, 2-10) separate time points spaced a median of 3 (range, 2-12) months apart were included (Table 2 and Table 3). Race, ethnicity, and/or ancestry for subgroup analysis were classified by the parent or by self-reporting in the electronic medical record. A total of 4 patients (14.8%) were Asian; 1 (3.7%) was Black or African American; 3 (11.1%) were Hispanic or Latino; and 19 (70.4%) were White. Fourteen patients (51.9%) were considered to have clinically active disease at baseline 3D imaging, of whom 8 (57.1%) represented new-onset disease in the previous 6 months. Eleven patients were not receiving therapy at baseline 3D imaging, of whom 6 had completed a standardized treatment course of systemic corticosteroids and methotrexate or mycophenolate mofetil using Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans,8 and 4 obtained baseline 3D imaging on the day of CM diagnosis.
Table 2. Clinical Characteristics and Treatment History of Patients With CM and Progressive Facial Asymmetry Noted on 3D Stereophotogrammetry.
| Patient No. | Clinical characteristic | Treatment | ||||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Age at disease onset, y | Age at baseline 3D imaging, y | CM subtype | Clinically active disease at time of baseline 3D imaging | Medications at time of baseline 3D imaging | No. of 3D imaging time points | Duration of monitoring via 3D imaging, mo | Clinically suspected disease progression during study period | 3D Imaging suspected disease progression | If clinically suspected disease activity, initial episode or disease recurrence | History | Modulation in therapy based on clinically suspected disease progression | |||
| 1 | 10-15 | 16-20 | ECDS | No | None | 9 | 33 | Yes | Yes | Recurrence | S/p IV SCS (weekly pulses for 6 wk) plus methotrexate (for 3 y); no therapy for 3 y at time of clinical progression | IV SCS (3 pulses) plus mycophenolate mofetil started | ||
| 2 | 1-4 | 11-15 | ECDS | No | None | 10 | 35 | Yes | Yes | Recurrence | S/p oral SCS (for 3 mo) plus mycophenolate mofetil (for 8 y); no therapy for 3 mo at time of clinical progression | Mycophenolate mofetil resumed | ||
| 3 | 1-4 | 11-15 | ECDS and HFA | Yes | Methotrexate and mycophenolate mofetil | 3 | 5 | Yes | Yes | Recurrence | Methotrexate plus mycophenolate mofetil (for 6 y); methotrexate tapered at time of clinical progression | Methotrexate dose increased | ||
| 4 | 5-10 | 11-15 | ECDS and HFA | Yes | IV SCS and methotrexate | 2 | 6 | Yes | Yes | Initial episode | IV SCS plus methotrexate at time of clinical progression | No; patient within 3 mo of starting IV SCS and methotrexate; progression of asymmetry believed to be secondary to antecedent disease activity rather than therapeutic failure | ||
| 5 | 5-10 | 21-25 | HFA | Yes | None | 3 | 35 | Yes | Yes | Recurrence | S/p methotrexate (for 13 mo, nonadherent); no therapy for 26 mo at time of clinical progression | Mycophenolate mofetil started | ||
| 6 | 11-15 | 16-20 | HFA | No | None | 2 | 10 | Yes | Yes | Recurrence | S/p methotrexate (for 6 y); no therapy for 6 mo at time of clinical progression | Mycophenolate mofetil started | ||
| 7 | 35-40 | 35-40 | HFA | Yes | Mycophenolate mofetil | 6 | 23 | Yes | Yes | Recurrence | S/p IV SCS (weekly pulses for 12 wk) plus mycophenolate mofetil (for 23 mo); mycophenolate mofetil at time of clinical progression | Mycophenolate mofetil discontinued, switched to upadacitinib | ||
| 8 | 5-10 | 5-10 | ECDS | Yes | IV SCS and methotrexate | 6 | 18 | Yes | Yes | Initial episode | IV SCS plus methotrexate at time of clinical progression | No; patient within 3 mo of starting IV SCS and methotrexate; progression of asymmetry believed to be secondary to antecedent disease activity rather than therapeutic failure | ||
| 9 | 5-10 | 5-10 | HFA | Yes | None | 3 | 8 | Yes | Yes | Initial episode | None; baseline 3D imaging obtained at initial presentation | IV SCS (12 pulses) plus methotrexate started | ||
| 10 | 16-20 | 16-20 | HFA | No | Methotrexate | 2 | 6 | Yes | Yes | Recurrence | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 20 mo); methotrexate at time of clinical progression | Switched from methotrexate to mycophenolate mofetil | ||
| 11 | 1-4 | 5-10 | ECDS | Yes | IV SCS and methotrexate | 4 | 23 | No | Yes | NA | IV SCS (weekly pulses for 12 wk) plus methotrexate (for 2 y); methotrexate at time of baseline 3D imaging | Although progression was not suspected based on the patient’s clinical examination results, subsequent brain MRI demonstrated progressive CNS activity for which adjunctive mycophenolate mofetil was started | ||
| 12 | 11-15 | 16-20 | HFA | No | None | 2 | 7 | No | Yes | NA | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 3.5 y); no methotrexate for 1 y at time of baseline 3D imaging | No; clinical progression not favored | ||
| 13 | 11-15 | 16-20 | ECDS | No | Methotrexate | 5 | 22 | No | Yes | NA | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 4 y); methotrexate at time of baseline 3D imaging | No; clinical progression not favored | ||
Abbreviations: 3D, 3-dimensional; CM, craniofacial morphea; CNS, central nervous system; ECDS, en coup de sabre (linear morphea); HFA, hemifacial atrophy (Parry-Romberg syndrome); IV, intravenous; MRI, magnetic resonance imaging; NA, not applicable; SCS, systemic corticosteroids; S/p, status post.
Table 3. Clinical Characteristics and Treatment History of Patients With CM and Without Clinical or 3D Stereophotogrammetric Evidence of Disease Progression During Study Period.
| Patient No. | Clinical characteristic | Treatment history | ||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| Age at disease onset, y | Age at baseline 3D imaging, y | Craniofacial morphea subtype | Clinically active disease at time of baseline 3D imaging | Medications at time of baseline 3D imaging | No. of 3D imaging time points | Duration of monitoring via 3D imaging, mo | Clinically suspected disease progression during study period | 3D Imaging suspected disease progression | ||
| 14 | 5-10 | 11-15 | ECDS | No | Methotrexate | 5 | 36 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 5 y); methotrexate tapered at time of baseline 3D imaging |
| 15 | 11-15 | 16-20 | HFA | No | None | 2 | 9 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 3 y); none for 3 y at time of baseline 3D imaging |
| 16 | 11-15 | 16-20 | HFA | No | Mycophenolate mofetil | 3 | 8 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 4 y); had disease flare in the setting of subsequent methotrexate taper, so IV SCS (weekly pulses for 8 wk) and started mycophenolate mofetil; mycophenolate mofetil (for 6 mo) at time of baseline 3D imaging |
| 17 | 1-4 | 5-10 | ECDS and HFA | No | Mycophenolate mofetil | 6 | 24 | No | No | S/p IV SCS (weekly pulses for 12 wk); initial methotrexate trial but developed transaminitis and transitioned to mycophenolate mofetil; mycophenolate mofetil (for 2 y) at time of baseline 3D imaging |
| 18 | 16-20 | 16-20 | HFA | No | Methotrexate | 2 | 12 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 2 y); methotrexate at time of baseline 3D imaging |
| 19 | 5-10 | 11-15 | HFA | Yes | Methotrexate | 6 | 22 | No | No | Started methotrexate at time of baseline 3D imaging due to clinical concern for disease recurrence |
| 20 | 5-10 | 5-10 | ECDS | Yes | Oral SCS and methotrexate | 7 | 24 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate; methotrexate (for 4 mo) at time of baseline 3D imaging |
| 21 | 11-15 | 11-15 | ECDS and HFA | Yes | None | 3 | 9 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate (for 5 y); experienced disease flare in the setting of attempting to taper methotrexate, so resumed at standard dosing at time of baseline 3D imaging |
| 22 | 11-15 | 16-20 | HFA | No | Methotrexate | 2 | 5 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate; methotrexate (for 9 mo) at time of baseline 3D imaging |
| 23 | 11-15 | 11-15 | HFA | Yes | Methotrexate | 3 | 6 | No | No | S/p IV SCS (weekly pulses for 12 wk) plus methotrexate; methotrexate (for 3 mo) at time of baseline 3D imaging |
| 24 | 5-10 | 5-10 | ECDS | Yes | None | 3 | 8 | No | No | Started IV SCS and methotrexate at time of baseline 3D imaging for new-onset ECDS |
| 25 | 5-10 | 5-10 | ECDS | Yes | None | 2 | 6 | No | No | Started IV SCS and methotrexate at time of baseline 3D imaging for new-onset ECDS |
| 26 | 5-10 | 5-10 | ECDS | Yes | None | 2 | 6 | No | No | Started on IV SCS and methotrexate at time of baseline 3D imaging for new-onset ECDS |
| 27 | 1-4 | 5-10 | HFA | No | IV SCS and methotrexate | 2 | 6 | No | No | Previously treated for new-onset disease with methotrexate (for 18 mo), then no systemic therapy for 4.5 y; 2 mo prior to baseline 3D imaging, patient clinically believed to have disease recurrence, so started IV SCS (weekly pulses for 12 wk) and methotrexate |
Abbreviations: 3D, 3-dimensional; CM, craniofacial morphea; ECDS, en coup de sabre (linear morphea); HFA, hemifacial atrophy (Parry-Romberg syndrome); IV, intravenous; SCS, systemic corticosteroids; S/p, status post.
Over the course of the study period (April 1, 2019, to March 1, 2023), 10 patients (37.0%) were clinically thought to have disease progression at 14 discrete time points by 2 international experts in CM (F.D. and R.A.V.). Clinical activity was defined by the presence of new or progressive involvement on the head of erythema, induration, white-yellow or waxy appearance, shiny white wrinkling, and/or progressive atrophy. Five of these patients (50.0%) experienced worsening facial asymmetry, despite adherence to first-line therapy for CM, 4 (40.0%) experienced recurrence of previously quiescent disease, and 1 (10.0%) experienced continued disease activity in the setting of medication nonadherence (Figure 2 and Table 2). Modulation in therapy was recommended in 8 patients (80.0%). The remaining 2 patients (20.0%) experienced worsening asymmetry within 3 months of starting first-line treatment for CM, and therefore, progression of facial asymmetry was attributed to antecedent disease activity rather than therapeutic failure; notably, both patients experienced disease stabilization as assessed by clinical and 3D stereophotogrammetry by 6 months after treatment initiation.
Figure 2. Representative 2-Dimensional (2D) and 3-Dimensional (3D) Images of Progression in Craniofacial Morphea.
A man in his 40s with a history of adult-onset hemifacial atrophy. Frontal 2D (A-D) and 3D (E-H) photographs are shown at 4 time points (three-quarter views are available in eFigure 2 in Supplement 1). In the 3D images, yellow indicates minor and red indicates greater volume deficiency on the heat map side compared with the contralateral side. Atrophy and contour change on the right cheek, extending from the medial tear through to the medial cheek with flattening of the nasolabial fold, were noted on baseline imaging (A and E) before the initiation of treatment with pulse-dose corticosteroids and mycophenolate mofetil. Follow-up imaging at 9 (B and F), 18 (C and G), and 23 (D and H) months are shown (follow-up imaging from 3 and 6 months are available in eFigure 2 in Supplement 1). Disease progression was noted on the right medial cheek both in 2D (C and D) and 3D (G and H) imaging at 18– and 23–month follow-up. Given that the patient had already received first-line treatment with pulsed intravenous methylprednisolone and nearly 2 years of mycophenolate mofetil, the decision was made to transition from mycophenolate mofetil to oral Janus kinase inhibitor therapy to mitigate further disease activity.
Notably, in all cases in which clinical progression was favored, blinded qualitative assessment of 3D imaging by both an expert in 3D stereophotogrammetry (I.M.G., a board-certified plastic surgeon with clinical expertise in craniofacial disorders) and a nonexpert (K.S.S., a board-certified dermatologist) favored progression with substantial interrater reliability (κ = 0.80 [95% CI, 0.61-0.99])9 (Table 2). Additionally, in those patients without clinically suspected progression, not only was substantial concordance observed in independent review of 3D images (κ = 0.75 [95% CI, 0.58-0.91]), but occult progression of facial asymmetry was suspected in 3 patients, indicating that 3D stereophotogrammetry may be more sensitive in detecting subtle tissue loss not appreciable on clinical examination results alone (eFigure 1 in Supplement 1). Finally, concordance rates for the independent evaluation of 3D imaging for Asian, Black or African American, and Hispanic or Latino patients in the study cohort (n = 8) were calculated and revealed near perfect interrater reliability (κ = 0.83 [95% CI, 0.61-0.99]).
Discussion
In this prospective cohort study, we aimed to investigate the feasibility of using 3D stereophotogrammetry as an adjunctive tool to detect disease progression in CM over time. We found that 3D stereophotogrammetry not only corroborated clinical impressions of disease progression but also identified occult worsening of facial asymmetry not appreciable on clinical examination results alone. Moreover, assessment of 3D images demonstrated substantial to near-perfect interrater reliability among an expert and nonexpert in 3D stereophotogrammetry in different subgroup analyses, including in patients with and without clinical evidence of disease progression as well as those patients with Fitzpatrick skin types IV to VI, highlighting the accessibility of this tool to clinicians with varying levels of training and expertise in craniofacial disorders in different patient populations.
Based on our findings, 3D stereophotogrammety has the potential to fill a critical and unmet need for an easy-to-use and reliable tool for monitoring disease progression in patients with CM over time. To date, validated clinical tools for detecting disease activity in CM remain limited. The Localized Scleroderma Assessment Tool (LoSCAT)10 continues to be the most widely used clinical instrument for assessing disease activity and damage in morphea. While the LoSCAT has served as a crucial tool for expanding our understanding of morphea, it classifies dermal and subcutaneous tissue atrophy as representative features of tissue damage rather than disease activity.10 While atrophy can certainly be a sequelae of antecedent disease activity in other subtypes of morphea, in CM specifically, progressive atrophy can also be a sign of ongoing disease activity, highlighting a shortcoming of using LoSCAT to monitor CM over time.11 The more recently proposed Morphea Activity Measure tool12 attempts to address this limitation of the LoSCAT by including progressive atrophy in the past 6 months as a marker of disease activity in linear morphea. However, in the validation of this instrument, reliance on patient impressions proved suboptimal, and 3D change was difficult to assess based on clinical photographs alone, again highlighting the need for a more reliable imaging modality to quantify changes in facial soft tissue in patients with CM.12 While magnetic resonance imaging13 and ultrasonography14 have been piloted in assessing progression in CM, significant limitations of these radiographic methods persist, including their expense, inefficiency, and lack of widespread availability. In addition, young children require sedation to acquire magnetic resonance imaging of the brain and/or face, making serial examinations impractical and potentially unsafe.
By contrast, 3D stereophotogrammetry is a rapid, noninvasive, and radiation-free imaging modality that is readily available for in-office use. The ability to examine a topographical heat map highlighting volumetric differences and progression of facial atrophy in a clinical evaluation affords clinicians the opportunity to make more informed decisions regarding treatment. Moreover, this tool may be of particular utility in those patients with Fitzpatrick skin types IV to VI, where signs of disease activity including violaceous erythema or sclerosis can be even more difficult to discern, if present at all. Given that unrecognized and untreated deep-tissue extension of CM can lead to the accrual of permanent damage as well as long-standing ophthalmologic, neurological, and cosmetic sequelae, the recognition and aggressive treatment of ongoing disease activity in CM cannot be overemphasized.
However, one of the challenges of relying on changes in facial asymmetry as a marker of disease activity in CM is that progressive volumetric differences may be attributed to alternative causes. Prospective cohort studies in generalized morphea and linear morphea of the extremities15 have shown that disease activity tends to improve within 1 year of starting standard-of-care treatment. By contrast, stabilization of disease damage can take longer, with progression of dermal and subcutaneous atrophy sometimes noted years after disease activity has been exhausted. While similar prospective studies of the disease course in patients with CM are lacking, it is possible that the progressive volumetric changes noted on 3D stereophotogrammetry are sometimes emblematic of antecedent disease activity with continued volume loss from prior inflammation. In addition, in children and young adults, it remains challenging to assess whether progressive asymmetry derives from asymmetrical growth of the affected vs unaffected face over time, even following effective treatment. To limit this potential confounder, we restricted the time between sequentially obtained 3D stereophotogrammetry images to 12 months.
Limitations
Limitations of this study include small sample size and the lack of a criterion standard for assessing CM. For example, clinical determination of disease progression relied on the assessment of 2 international experts in CM. Additionally, the flip-registration procedure of 3D stereophotogrammetry may preclude its use in patients presenting with midline or symmetric facial defects (both of which are rare in CM).
Conclusions
Despite challenges and limitations, this cohort study of sequentially obtained 3D stereophotogrammetry images not only captured disease progression in patients with clinically suspected disease activity but also identified occult progression in patients for whom prior disease stabilization had been achieved. Three-dimensional stereophotogrammetry may thereby serve as a valuable adjunctive tool in monitoring patients with CM over time. Further work is necessary to validate this measure in a larger cohort and to guide its incorporation into medical decision-making for patients with CM.
eMethods. Imaging
eFigure 1. Representative Images of Occult Progression of Craniofacial Morphea Captured by 3D Stereophotogrammetry
eFigure 2. Additional Time Points and Three-Quarter Views of Patient in Figure 2 with Progression of Craniofacial Morphea
Data Sharing Statement
References
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Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
eMethods. Imaging
eFigure 1. Representative Images of Occult Progression of Craniofacial Morphea Captured by 3D Stereophotogrammetry
eFigure 2. Additional Time Points and Three-Quarter Views of Patient in Figure 2 with Progression of Craniofacial Morphea
Data Sharing Statement
