Figure 3 - uploaded by Abdulaziz A Bakathir
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-Unenhanced computerized tomographic scan of the head; bone window a-c) axial at the level of orbits and d) axial at the level of maxilla and mandible. The selected images of facial bones reveal marked thickening of the maxilla and cranial bones with a " ground glass " appearance (arrow).
Source publication
This case report describes a 21-year-old female patient with a complex medical condition of end-stage chronic renal failure and secondary hyperparathyroidism presenting with a history of gradual enlargement of the facial bones over a period of one year. The facial enlargement primarily involves the maxilla causing a bizarre facial and dental deform...
Citations
... These facial enlargements cause a bizarre facial deformity and dental malocclusion. [14][15][16][17] Primary HPT also rarely presents in the mandible as a large exophytic mass or painless swelling, [18] and also as a giant-cell epulis as an initial feature of primary HPT. [19] Primary HPT patients were more likely to have tori. ...
Parathyroid hormone (PTH) plays a key role in the regulation of calcium homeostasis. It is secreted by a pair of parathyroid glands located behind the thyroid gland. Primary hyperparathyroidism is the disorder which is seen in 0.2%–0.3% of the population. It is the third most common endocrine disorder after Diabetes Mellitus and Thyroid disorders. There are several systemic manifestations of the disease including skeletal, Renal, abdominal, neurological ones. The following case report discusses some of the classic oral manifestations of the disease and signifies the role of an Oral physician in the diagnosis of underlying systemic disorder.
... Rai et al. [14] also found a higher circulating level of parathyroid hormone in patients with primary hyperparathyroidism in additional to frequent occurrences of loss of lamina dura, ground-glass finding, and a lessening in the mandibular cortical width in these patients. Furthermore, uremia-related changes in facial bone structures have been stated in literature [15][16][17]. For example, Bakathir et al. [15] described the progressive enlargement of facial bones of a 21-year-old female patient with uremia whose facial enlargement involved the maxilla and caused facial and dental deformities. ...
... Furthermore, uremia-related changes in facial bone structures have been stated in literature [15][16][17]. For example, Bakathir et al. [15] described the progressive enlargement of facial bones of a 21-year-old female patient with uremia whose facial enlargement involved the maxilla and caused facial and dental deformities. Lopes et al. [16] reported two uremic females with facial disfigurement affecting the maxilla and the mandible. ...
Reports on the prevalence of torus mandibularis among dialysis patients have been limited and inconclusive. A wide variety of oral manifestations has been found in patients with hyperparathyroidism. Furthermore, uremia-related changes in facial bone structures have been described in the literature. This prospective observational study examined 322 hemodialysis patients treated at the Chang Gung Memorial Hospital from 1 August to 31 December 2016. Two subgroups were identified: patients with torus mandibularis (n = 25) and those without (n = 297). Clinical oral examinations including inspection and palpation were employed. Our study found that most mandibular tori were symmetric (84.0%), nodular (96.0%), less than 2 cm in size (96.0%), and located in the premolar area (92.0%). Poor oral hygiene was observed among these patients, with 49.7% and 24.5% scoring 3 and 4, respectively, on the Quigley–Hein plaque index. More than half (55.0%) of patients lost their first molars. Multivariate logistic regression analysis revealed that blood phosphate level (odds ratio = 1.494, p = 0.029) and younger age (odds ratio = 0.954, p = 0.009) correlated significantly with torus mandibularis. The prevalence of torus mandibularis in patients receiving hemodialysis in this study was 7.8%. Younger age and a higher blood phosphate level were predictors for torus mandibularis in these patients.
... Uremia-related changes in facial bone structures have been reported in literature [15][16][17]. Bakathir et al. [15] described the progressive enlargement of facial bones of a 21-year-old female uremic patient whose facial enlargement involved the maxilla and caused facial and dental deformities. Lopes et al. [16] presented two female uremic patients with facial disfigurement affecting the maxilla and the mandible. ...
... Uremia-related changes in facial bone structures have been reported in literature [15][16][17]. Bakathir et al. [15] described the progressive enlargement of facial bones of a 21-year-old female uremic patient whose facial enlargement involved the maxilla and caused facial and dental deformities. Lopes et al. [16] presented two female uremic patients with facial disfigurement affecting the maxilla and the mandible. ...
Introduction
This study attempted to survey the oral findings of hemodialysis patients and analyze the prevalence and predictors for torus palatinus (TP) in this patient population.
Methods
A total of 322 hemodialysis patients were recruited. Patients were organized into two groups, based on the presence (n=93) or absence (n=229) of TP. Demographic, laboratory, and dialysis-related data were obtained for analysis.
Results
The prevalence of TP was 28.9% in this study. Patients with TP were younger in age [57.8±10.0 (37.4-86.9) versus 62.4±12.3 (25.0-87.8) years old; P=0.001] and predominantly female (60.2% versus 38.0%; P<0.001), compared to patients without TP. All TPs (100.0%) were symmetrical and located along the midpalatal suture. Most TPs were flat-shaped (55.9%) and near premolars (78.5%). The blood tests revealed higher blood concentrations of phosphate (5.4±1.1 versus 4.9±1.1 mg/dL; P=0.001) and lower blood concentrations of bicarbonate (20.9±2.4 versus 22.0±2.3 mmol/L; P<0.001) in patients with TP. Multivariate regression modeling showed that younger age [odds ratio (OR) 0.968; 95% confidence interval (CI) 0.939–0.982; P<0.001], female gender (OR 2.305; 95% CI 1.374–3.867; P=0.002), higher blood concentration of phosphate (OR 1.411; 95% CI 1.110–1.794; P=0.005), and lower blood concentration of bicarbonate (OR 0.868; 95% CI 0.791–0.994; P=0.040) were significant predictors for TP.
Conclusion
The prevalence of TP is 28.9%, and the majority of patients suffering TP are female. Younger age, female gender, elevated blood concentration of phosphate, and lower blood concentration of bicarbonate are predictors for TP.
... mg/dL), ionised calcium level was 1.09 mmol/L (normal range: 1.09-1.29 mmol/L), phosphorus level was 7.3 mg/dL (normal range: The term ULO is derived from the appearance of the facial deformity due to bony overgrowth around the oral cavity which resembles the face of a lion. Radiographically, ULO manifests as hypertrophy and hyperostosis of the facial bones and calvaria, especially the maxilla and mandible, and diffuse mixed sclerotic and lytic changes best illustrated by non-contrast CT. [2][3][4][5] The reason as to why the facial bones and calvaria are selectively affected in ULO cases is not clear. In the current case, the affected patient had all of the typical clinical, laboratory and radiographical features of ULO. ...
... If left untreated, patients with ULO may develop severe disfigurement, dysphagia, speech impairment, respiratory distress and blindness. [2][3][4] The management of ULO cases should focus on controlling secondary or tertiary hyperparathyroidism, either with medication or surgically with a parathyroidectomy. 3 Unfortunately, surgical resection was not feasible in the current case and the patient did not adhere to prescribed medical therapy. ...
... [2][3][4] The management of ULO cases should focus on controlling secondary or tertiary hyperparathyroidism, either with medication or surgically with a parathyroidectomy. 3 Unfortunately, surgical resection was not feasible in the current case and the patient did not adhere to prescribed medical therapy. As such, there was no evidence of clinical improvement at a one-month follow-up appointment. ...
... Oral physicians should be aware that renal osteodystrophy can affect 90% of patients undergoing dialysis [31]. Earlier detection and improved therapeutic control have decreased the severity of cases, but prolonged dialysis regimens were still recently reported in 70% of individuals after 3 years of dialysis [32]. Of patients with CKD, 1.5% may present with brown tumors [33], and in a study by Cecchetti, of 115 patients with CKD, 10 patients had brown tumors, 5 of which occurred in the craniofacial bones [8]. ...
... Bone expansion can occur even without the presence of brown tumors [36]. These bony expansions can cause speaking difficulties [37,38], mastication difficulties [20,39], airway obstruction [32,35,[40][41][42][43][44][45], and malocclusion [46]. Two cases of condylar resorption also caused occlusal difficulties [36,39]. ...
Objectives:
We sought to identify oral symptoms found in hyperparathyroidism and compare their rate of occurrence, as well as potential variations in sequelae between primary, secondary, and tertiary hyperparathyroidism.
Materials and methods:
Database searches were performed through EMBASE and PubMed, with a continual handsearch for relevant articles. PRISMA guidelines were followed.
Results:
Two hundred five articles including 245 patients were analyzed with data extraction. The average age was 34.02 years old (age range 1-83), with 91 male and 154 female patients (1:1.7 M/F ratio). Patients presented with symptoms including facial asymmetry or swelling (167/214 cases; 78.0%), oral pain (30/214; 14.0%), systemic symptoms (25/214; 11.7%), referrals or incidental findings (16/214; 7.5%), and neuropathy (6/214; 2.8%) independently and in combination together. Bony pathology occurred most often in the mandible (100/245 cases; 40.8%), while 72 cases were in the maxilla (29.4%) and 73 cases in both jaw bones (29.8%).
Conclusions:
Our data collection identifies a wide variation in the presentation of hyperparathyroidism. In order to be more certain of oral maladies from hyperparathyroidism, studies with large patient populations need to be conducted at healthcare centers to clarify the oral outcomes of hyperparathyroidism.
Clinical relevance:
What was thought to be a characteristic finding of HPT, mandibular radiolucency occurred in only a minor portion of cases. Furthermore, the pathognomonic sign of HPT on radiograph, loss of lamina dura, was only the third most common presentation. Bone pathology was most commonly reported in literature, but should not be assumed the only oral sequelae of hyperparathyroidism.
... There are several studies that demonstrate changes in the growth of the mandible or maxillae that can be compatible with hyperplasia or hypoplasia. 13 However, no syndrome or disorder of the sphenoid bone itself has been reported that might alter only its pterygoid process. 14,15 Only unilateral fibrous dysplasia of the sphenoid bone has been reported to cause a full enlargement of the affected side. ...
This study was performed to evaluate the relationship between pterygoid plate asymmetry and temporomandibular joint disorders.
Cone-beam computed tomography (CBCT) images of 60 patients with temporomandibular disorders (TMD) involving pain were analyzed and compared with images of 60 age- and gender-matched controls. Three observers performed linear measurements of the lateral pterygoid plates.
Statistically significant differences were found between measurements of the lateral pterygoid plates on the site that had pain and the contralateral site (p<0.05). The average length of the lateral pterygoid plates (LPPs) in patients with TMD was 17.01±3.64 mm on the right side and 16.21±3.51 mm on the left side, and in patients without TMD, it was 11.86±1.97 mm on the right side and 11.98±1.85 mm on the left side. Statistically significant differences in the LPP length, measured on CBCT, were found between patients with and without TMD (p<0.05). The inter-examiner reliability obtained in this study was very high for all the examiners (0.99, 95% confidence interval: 0.98-0.99).
Within the limits of the present study, CBCT lateral pterygoid plate measurements at the side with TMD were found to be significantly different from those on the side without TMD. More research is needed to explore potential etiological correlations and implications for treatment.
... The lesions from OFC and BT are not painful and often produce symptoms, depending on the location of facial asymmetry, oral mass, nasal obstruction, sinusitis, progressive visual disturbances, proptosis, chewing diffi- culties, hearing abnormalities, and airway obstruction in the pediatric population. [32][33][34][35] These symptoms are typi- cally referred for treatment to specialties of maxillofacial surgery, oral surgery, reconstructive and plastic surgery, ENT, ophthalmology, and dentistry. These specialties expressed interest in the disease and reported case studies in the literature. ...
Osteitis fibrosa cystica (OFC) is the most frequent type of osseous change in renal osteodystrophy affecting the majority of dialysis patients. Brown tumors are a severe form of OFC. The involvement of the craniofacial skeleton causing facial disfigurement in patients on dialysis appears to be limited to case reports. After searching PubMed, we performed a systematic review of 127 cases with a severe form of OFC resulting in a facial disfigurement to understand possible determinants for this condition. We found that since the first published case in 1974, and after a peak in 1996, there appears to be an increase in published reported cases. Only 27.6% of these cases were published in nephrology journals. The most common region for reported cases was North America. Mean age of these patients was 31.2 years with a mean dialysis duration of 7 years. Almost 67% were women, and almost all were on hemodialysis. The disease tended to most commonly localize to the maxilla (73.2%) and mandible (57.5%). As part of the treatment, 59% of patients had a parathyroidectomy. More than one-third (35.4%) had symptomatic improvement at follow-up. Mean follow-up was 1.6 years. Clinicians should be aware of this clinical presentation of a severe form of OFC and/or brown tumors. Timely diagnosis and intervention may help to prevent or decrease destructive bone changes and reduce negative psychological consequences of facial disfigurement.
© 2015 International Society for Hemodialysis.
... These facial enlargements cause a bizarre facial deformity and dental malocclusion. [14][15][16][17] Primary HPT also rarely presents in the mandible as a large exophytic mass or painless swelling, [18] and also as a giant-cell epulis as an initial feature of primary HPT. [19] Primary HPT patients were more likely to have tori. ...
Parathyroid hormone plays an important role in the metabolism of calcium and phosphorus, so, influence the mineralization of bone and teeth. Parathyroid disorder may lead to hyper or hyposecretion of hormone, which results in various oral manifestations. Common oral manifestations in patients with hyperparathyroidism (HPT) are brown tumor, loss of bone density, soft tissue calcification, and dental abnormalities. In hypoparathyroidism, the dental abnormalities are a delay or cessation of dental growth and development, paresthesia of the tongue or lips and alteration of the facial muscles. Dentist can easily diagnose the parathyroid disorders owing to their particular oral manifestations and radiographic finding. Dental management of patients with HPT involves a higher risk of bone fracture, whereas in hypoparathyroidism the caries control is the main concern. It is the important that the dentist be aware of the risks and difficulties that may arise during the dental management of these patients.
... 3 Our two cases had very unusual and strikingly similar presentations, not clearly fitting the classic description of either pure tertiary hyper- parathyroidism or Paget's disease. While the coexistence of these two disorders has been reported just recently 4 and remains a possible explanation, these lesions were most compatible with prior reports of disfiguring leontiasis ossea 3,5 or Sagliker syndrome 2,6 in this patient population. On physical appearance, they looked very much alike and different from their own past photographs. ...
We are reporting on a series of two patients with end-stage renal disease on hemodialysis, presented for surgical parathyroidectomy secondary refractory hyperparathyroidism. Both patients had failed maximized medical managements, including higher-than-usual doses of the calcimimetic cinacalcet (270 and 180 mg/day, respectively). On physical exam, both patients had marked symmetrical craniofacial hypertrophy with coarse distortion of facial features, similar in appearance to past reports of Sagliker syndrome. On X-ray and computed tomographic exam, they had peculiar areas of bone absorption on the skull, imitating the radiologic appearance of multiple myeloma. Bone biopsy of the maxilla, however, did not show the expected brown tumor, but rather described only fibrosis and reactive bone formations. This phenotype developed while being on cinacalcet, progressed despite escalation of therapy, and improved only after parathyroidectomy. Both patients developed massive "hungry bone syndrome" after parathyroidectomy necessitating prolonged IV calcium infusion. This pattern of severe facial distortion likely represented an adverse consequence of severe tertiary hyperparathyroidism, along with supraphysiologic dose of cinacalcet administration and 25-hydroxy vitamin D deficiency in sensitive individuals. The genetic base of this observation remained unexplained.
