Cephalometric skeletal evaluation of patients with Incontinentia Pigmenti

Abstract

a b s t r a c tPurpose: The aim of this study was to evaluate the skeletal characteristics of patients with the rare genetic disease of Incontinentia Pigmenti, by lateral cephalometric analysis on the antero-posterior plane and by frontal cephalometric analysis on the horizontal plane.Methods: Lateral skeletal cephalometric analyses were performed according to Steiner for evaluation of antero-posterior direction, and frontal skeletal cephalometric analyses according to Ricketts for evaluation of horizontal direction in 9 patients with IP. Left and right facial widths at the level of the zygomatic arch were also evaluated. The Student t-test was used for paired to a 5% level of significance data.Results: The lateral skeletal cephalometric findings were not statistically significant, but the Class II was the most frequent finding (44.4%), followed by Class III (33.3%) and Class I (22.2%). The right maxillo-mandibular width was significantly lower than normal values, and the right facial width was significantly higher than the left, at the level of the zygomatic arch. Conclusions:Patients with IP showed more skeletal discrepancies of Class II and III than Class I malocclusion, and had significant horizontal facial skeletal asymmetries. This should alert health professionals to route these patients for orthodontic assessment and possible therapeutic interventions. However, larger samples are needed to better elucidate if these cephalometric findings can be specifically related to IP.

Multidisciplinary consensus recommendations from a European network for the diagnosis and practical management of patients with incontinentia pigmenti

“…[22][23][24][25][26] Other clinical manifestations Mammary gland complications are relatively common and constitute a minor diagnostic criterion (Table 1). Bone anomalies 27 as well as lethal forms of pulmonary hypertension have been described 28,29 often in isolated cases.…”

Oral Rehabilitation as Part of a Multidisciplinary Treatment in a Case Study of Pigmentary Incontinence

“…Although the frequency with which PI patients present agenesis, hypodontia, and even anodontia, and skeletal class III with loss of vertical dimension has been reported in the literature [ 10 , 11 ], our patient had no missing teeth and presented a skeletal class II with an increase in the vertical dimension (ENA-Xi-Pm > 47°) and convex profile [ 10 , 11 , 12 , 13 ].…”

“…In a coronal section, cranial asymmetry and cerebral parenchyma atrophy were observed, with marked grooves in the temporal and parietal lobes and an increase in the size of the cerebral ventricles (Figure 5). Although the frequency with which PI patients present agenesis, hypodontia, and even anodontia, and skeletal class III with loss of vertical dimension has been reported in Although the frequency with which PI patients present agenesis, hypodontia, and even anodontia, and skeletal class III with loss of vertical dimension has been reported in the literature [10,11], our patient had no missing teeth and presented a skeletal class II with an increase in the vertical dimension (ENA-Xi-Pm > 47 • ) and convex profile [10][11][12][13].…”

“…the literature [10,11], our patient had no missing teeth and presented a skeletal class II with an increase in the vertical dimension (ENA-Xi-Pm > 47°) and convex profile [10][11][12][13].…”

Orthodontic Treatment for Bloch-Sulzberger Syndrome in Patient with Cleft Lip and Palate

“…With regard to maxillofacial morphology, Maahs et al 5) reported Class II malocclusion as the most common (44.4%), followed by Class III (33.3%), and Class I (22.2%) following cephalographic evaluation of 9 patients with Bloch-Sulzberger syndrome. However, several other reports describe development of anterior crossbite as a result of poor maxillary growth 3,9,10,16) .…”