-
European Review For Medical and... Nov 2023Proteus syndrome (PS) is an extremely rare disorder with ocular manifestations. In this study, we aimed to describe the ophthalmic characteristics and the clinical...
BACKGROUND
Proteus syndrome (PS) is an extremely rare disorder with ocular manifestations. In this study, we aimed to describe the ophthalmic characteristics and the clinical course of an unusual PS patient to acquire a comprehensive and intensive understanding of ocular PS and highlight the importance of collaborative treatment by ophthalmologists.
CASE PRESENTATION
A case of PS with atypical ocular features and syndromes was observed in a Chinese female. Her proptosis and vision impairment were relieved after Endoscope-Navigation system (ENS)-aided optic canal decompression. A 1.5-year follow-up showed that the treatment was temporarily effective, but the disease continued to develop. A review of the literature was conducted: forty-eight patients met the inclusion criteria. Although ocular manifestations play important roles in PS diagnosis, only a limited number of cases have been reported to have ocular abnormalities. And to date, almost none of these reports have described the treatment in detail. Therefore, PS patients with ocular manifestations were reviewed.
CONCLUSIONS
PS is a complex disorder with variable characteristics and progressive imbalances. In this paper, the clinical symptoms, molecular characteristics, and differential diagnosis of PS are introduced. More importantly, the ocular manifestations, treatment, and prognosis of PS cases to date are summarized and discussed. This study aimed to acquire a comprehensive and intensive understanding of ocular PS and to reveal the importance of collaborative treatment by ophthalmologists.
Topics: Humans; Female; Proteus Syndrome; Eye
PubMed: 37975355
DOI: 10.26355/eurrev_202311_34306 -
Oral Radiology Jan 2021Proteus syndrome (PS) is an extremely rare disorder with asymmetric and disproportionate bone overgrowth. Craniofacial abnormalities in PS are less frequent than... (Review)
Review
OBJECTIVES
Proteus syndrome (PS) is an extremely rare disorder with asymmetric and disproportionate bone overgrowth. Craniofacial abnormalities in PS are less frequent than skeletal abnormalities. Although there are recognized oral and maxillofacial manifestations of PS, few case reports describing these manifestations are available. Thus, the objective of this systematic review and case report is to describe oral and maxillofacial manifestations of PS and to report a PS case.
METHODS
A 31-year-old male presented with restricted mouth opening and pain during mastication. A panoramic radiograph and an occlusal radiograph were obtained. Reports with relevant keywords were assessed. Data were summarized and demonstrated using a critical appraisal checklist for case reports.
RESULTS
The panoramic radiograph demonstrated unilateral overgrowth of the mandible, impacted teeth, and deciduous prolonged retention. Thirteen PS case reports were identified.
CONCLUSIONS
Proteus syndrome oral and maxillofacial manifestations may include dental agenesis, impacted teeth, malocclusion, asymmetric dental growth and maturation, frontal line displacement, asymmetric tongue enlargement, mandibular hemihypertrophy and asymmetry, presence of exostoses/hyperostosis, degenerative changes in the temporomandibular joint, alterations of maxillary and mandibular vertical and/or horizontal growth, and enlargement of mandibular canal and foramen. The PROSPERO systematic review registration number is CRD42019140942.
Topics: Adult; Humans; Male; Mandible; Maxilla; Proteus Syndrome; Radiography, Panoramic; Tooth, Impacted
PubMed: 31734933
DOI: 10.1007/s11282-019-00416-y -
Clinical Rheumatology Jul 2019Piriformis pyomyositis is a rare form of purulent skeletal myositis. As previous studies concerning piriformis pyomyositis had lower level of evidence and no systematic...
Piriformis pyomyositis is a rare form of purulent skeletal myositis. As previous studies concerning piriformis pyomyositis had lower level of evidence and no systematic review has been published yet, we performed a systematic search to review and describe causes, symptoms, red flags, and available treatment options for piriformis pyomyositis. Using PubMed and PubMed Central databases, we found 21 articles describing 23 cases of piriformis pyomyositis. Based on the retrieved information, alongside acute sciatica like buttock and/or hip pain, high-grade fever, aggressive deep seated gluteal pain, neurological deficit of sciatic nerve distribution, positive straight leg raising test, and raised inflammatory biomarkers (erythrocyte sedimentation rate, ESR, C-reactive protein, CRP) provide clues for diagnosis of piriformis pyomyositis. Some cases were very ill but no death was documented. Staphylococcus aureus was the most common pathogen, but Group A as well as Group β Streptococcus, Salmonella typhi, Proteus mirabilis, Brucella melitensis, and Escherichia coli were also involved in the disorder. To treat the piriformis pyomyositis, broad-spectrum antibiotics were found to be useful; however, sometimes, antibiotic switching was warranted based on blood and tissue aspirate reports. Drainage and/or surgical exploration of the affected piriformis muscle were required in cases where antibiotics appeared ineffective. Piriformis pyomyositis is a rara avis and performing of prospective studies will hardly be feasible.
Topics: Anti-Bacterial Agents; Bacterial Infections; Humans; Low Back Pain; Piriformis Muscle Syndrome; Pyomyositis; Sciatica; Staphylococcal Infections
PubMed: 31049761
DOI: 10.1007/s10067-019-04552-y -
Journal of Obstetrics and Gynaecology :... Aug 2020We performed a comprehensive systematic review of acute pyelonephritis in pregnancy using PubMed, SCOPUS, ClinicalTrials.gov, and Ovid from inception to April 2018....
We performed a comprehensive systematic review of acute pyelonephritis in pregnancy using PubMed, SCOPUS, ClinicalTrials.gov, and Ovid from inception to April 2018. About 7796 references were screened for inclusion, and 52 references from 1908 to 2017 were included. One hundred seven cases of acute pyelonephritis in pregnant women were reviewed. Gestational age at diagnosis was reported as 2 (2%), 43 (40%), and 51 (52%) during the first, second, and third trimesters, respectively. Maternal complications included sepsis (49%), acute respiratory distress syndrome (47%), anaemia (33%), acute kidney injury (10%), renal abscess (6%), and death (6%). 25 preterm deliveries (23%), 6 intrauterine foetal demises (6%), 4 spontaneous abortions (4%), and 8 neonatal intensive care unit admissions (7%) were reported. Microorganisms cultured included (51%), (8%), (5%), (5%), (4%), and (3%). Early diagnosis and management led to fewer complications.Impact statement Acute pyelonephritis during pregnancy can lead to adverse pregnancy outcomes and in this article, we highlight the most common outcomes previously reported. Previous studies have reported maternal adverse outcomes and only very few stressed on fetal/neonatal outcomes. The results add that not only is maternal morbidity/mortality is increased, but also increases adverse outcomes for the fetus/neonate, such as preterm delivery and fetal/neonatal demise. The implications from this article serve to increase a medical providers knowledge on how to appropriately counsel pregnant women with acute pyelonephritis. In addition, future research can aim to understand why pregnant women are more prone to morbidity and mortality compared to nonpregnant women.
Topics: Acute Disease; Adult; Female; Fetal Death; Gestational Age; Humans; Infant, Newborn; Maternal Mortality; Pregnancy; Pregnancy Complications, Infectious; Pregnancy Outcome; Pyelonephritis
PubMed: 32648533
DOI: 10.1080/01443615.2019.1647524