Relato de caso clínico de infecção odontogênica resultando em complicação cervicofacial / Reporte de caso clínico de infección odontogênica com resultado de complicación cervicofacial / Clinical case reportof odontogenic infectionresulting cervicofacial complication

Introdução: A saúde bucal é um aspecto que não deve ser subestimado pelos pacientes, principalmente se considerar que as infecções odontogênicas podem levar a quadros graves, incluindo complicações cervicotorácicas, como Mediastinite e cervicofaciais, como Angina de Ludwig. Para tanto, é imprescindível que os profissionais da odontologia saibam reconhecer os principais sinais e sintomas dessas infecções, sua evolução, conhecer as complicações associadas e qual o manejo adequado. Objetivo: Assim, é objetivo deste trabalho, relatar, discutir um caso clínico de uma infecção odontogênica grave que acarretou em complicação cervical, com trajeto em direção ao mediastino, necessitando manejo multidisciplinar, e explorar os principais aspectos desse quadro e a conduta necessária, que exige, no mínimo, intervenção cirúrgica, antibioticoterapia e manutenção das vias aéreas. Relato de caso: O caso trata de um paciente com infecção odontogênica, iniciada como uma pericoronarite do dente 38 semieruptado, que evoluiu para a área cervical, demandando imediata drenagem nesta região pois encaminhava-se para uma mediastinite. Após a drenagem cervical e antibioticoterapia e, assim que houve redução do trismo, foi removido o dente 38, evoluindo para a cura.Conclusões:As infecções odontogênicas, principalmente as que acometem os espaços fasciais e cervicais profundos, são potencialmente graves e devem ter suas principais manifestações clínicas entre os domínios de conhecimento dos profissionais Bucomaxilofaciais, pois necessitam de diagnóstico preciso, manejo rápido e tratamento adequado e precoce, considerando a velocidade com que podem evoluir (AU).

Introduction: Oral healthis an aspect that should not be underestimated by patients, especially considering that dental infections can lead to serious symptoms, including cervicothoracic complications, such as Mediastinitis and cervicofacial complications, such as Ludwig's Angina. Therefore, it is essential that dental professionals know how to recognize the main signs and symptoms of these infections, their evolution, know the associated complications and appropriate management.Objective: Thus, this work aims to report and discuss a clinical case of a serious odontogenic infection that resulted in a cervical complication, with a path towards the mediastinum, requiring multidisciplinary management, and to explore the main aspects of this condition and the necessary conduct, which requires, at least, surgical intervention, antibiotic therapy and airway maintenance.Case report: The case concerns a patient with odontogenic infection, which began as pericoronitis of semi-erupted tooth 38, which progressed to the cervical area, requiring immediate drainage in this region as it was heading towards mediastinitis. After cervical drainage and antibiotic therapy and, as soon as the trismus was reduced, tooth 38 was removed, progressing towards healing.Conclusions: Odontogenic infections, especially those that affect the fascial and deep cervical spaces, are potentially serious and should have their main clinical manifestations among the domains of knowledge ofOral and Maxillofacial professionals, as they require accurate diagnosis, rapid management and adequate and early treatment, considering the speed at which they can evolve (AU).

Introducción: La salud bucal es un aspecto que los pacientes no deben subestimar, especialmente considerando que las infecciones odontógenas pueden derivar en afecciones graves, incluidas complicaciones cervicotorácicas, como la mediastinitis, y complicaciones cervicofaciales, como la angina de Ludwig.Para ello, es fundamental que los profesionales odontológicos sepan reconocer las principales señalesy síntomas de estas infecciones, su evolución, conocer las complicaciones asociadas y el manejo adecuado.Objetivo: Así,el objetivo de este trabajo es reportar y discutir un caso clínico de infección odontogénica grave que resultó en una complicación cervical, con trayecto hacia el mediastino, que requirió manejo multidisciplinario, y explorar los principales aspectos de esta condicióny las medidas necesarias, que requiere, como mínimo, intervención quirúrgica, terapia con antibióticos y mantenimiento de las vías respiratorias.Reporte de caso: El caso se trata de un paciente con una infección odontogénica, que comenzó como pericoronaritis del diente 38 semi-erupcionado, la cual progresó hacia la zona cervical, requiriendo drenaje inmediato en esta región ya que se encaminaba para una mediastinitis.Después del drenaje cervical y la terapia antibiótica y, una vez reducido el trismo, se extrajo el diente 38, evolucijjonando hacia la cura.Conclusiones: Las infecciones odontogénicas, especialmente aquellas que afectan los espacios fasciales y cervicales profundos, son potencialmente graves y deben tener sus principales manifestaciones clínicas entre los dominios del conocimiento de los profesionales Orales y Maxilofaciales, pues requieren de un diagnóstico certero, un manejo rápido y un tratamiento adecuado y temprano, considerando la velocidad a la que pueden evolucionar (AU).

Absceso periamigdalino bilateral. Reporte de un caso y revisión de las controversias en el diagnóstico y tratamiento / Bilateral peritonsillar abscess. A case report and review of controversies surrounding diagnosis and management

La presentación bilateral del absceso periamigdalino es poco frecuente. Su abordaje es controversial y se discute si realizar amigdalectomía en caliente versus diferida. Se presenta el caso de un paciente de sexo masculino, de 14 años, con odinofagia, trismo y fiebre. Presentaba hipertrofia amigdalina bilateral, pilares abombados y edema de paladar blando. Tomografía computada: hipertrofia amigdalina bilateral, con realce poscontraste, ambas con colección, edema con moderada estenosis faríngea. Se decidió internación para tratamiento endovenoso y amigdalectomía con drenaje bilateral. Resolución completa del cuadro con alta a las 48 horas. Ante la presencia de un absceso periamigdalino, debe considerarse la posibilidad de un absceso contralateral oculto. Debe ser diagnosticado y tratado adecuadamente para prevenir complicaciones. La amigdalectomía en caliente podría ser un tratamiento seguro y debería ser considerado en pacientes que serán sometidos a anestesia para drenaje. La decisión final debe ser determinada para cada caso en particular.

The bilateral presentation of peritonsillar abscess is uncommon. Its management is controversial and it has been argued whether a quinsy tonsillectomy or an interval tonsillectomy should be performed. Here we describe the case of a 14-year-old boy with sore throat, trismus, and fever. He had bilateral tonsillar hypertrophy, convex arches, and soft palate edema. Computed tomography: bilateral tonsillar hypertrophy, with post-contrast enhancement, both with collection, edema with moderate pharyngeal stenosis. The patient was hospitalized for intravenous therapy and tonsillectomy with bilateral drainage resulting in a complete resolution of his condition and discharge at 48 hours. In the presence of a peritonsillar abscess, an unsuspected contralateral abscess should be considered. It should be diagnosed and managed adequately to prevent complications. Quinsy tonsillectomy could be safe and should be considered in patients who will undergo anesthesia for abscess drainage. The final decision should be made for each patient on an individual basis.

Craniorachischisis in a 33-week-old female fetus: A case report

@#We report the case of a 33-week-old female fetus born with craniorachischisis to a gravida 5, para 4 (3104) mother with no previous history of conceiving a child with a neural tube defect. Craniorachischisis is characterized by anencephaly and an open defect extending from the brain to the spine and is the most severe and fatal type of neural tube defect. Although the cause of neural tube defects is hypothesized to be multifactorial and is usually sporadic, the risk is increased in neonates born to mothers with a family history or a previous pregnancy with neural tube defect, both of which are not present in the index case. This case is unique in that only during the fifth pregnancy did the couple conceive a child with a neural tube defect, emphasizing that folic acid supplementation, the sole preventive measure proven to decrease the risk of neural tube defects, remains to be important in the periconceptual period for all women of childbearing age.

A case report of the first Filipino infant diagnosed with cystic fibrosis through the Philippine Newborn Screening Program

@#Cystic Fibrosis (CF) is a rare condition among Asians and has not been reported in the Philippines as of this time. The inclusion of this disease in the Philippines’ Expanded Newborn Screening Program (ENBS) has provided this Filipino family the opportunity of early detection and appropriate management of this condition that could ensure the survival of the proband and his other surviving siblings. Here we present a case of a 24-month-old male who had a positive Expanded Newborn Screening (ENBS) test for cystic fibrosis and eventually underwent further tests to confirm a homozygous deletion of exons 1 - 2 of the CFTR gene. He subsequently had recurrent pneumonia but is being managed by a team consisting of a pulmonologist, gastroenterologist, and a metabolic dietitian. The proband had an older sibling whose Newborn Screening (NBS) test was normal and who eventually expired from recurrent bouts of pneumonia. This sibling was never managed as a case of cystic fibrosis. Implications on the diagnosis and management of CF in the local setting is also discussed. The importance of an appropriate CF panel customized to the local population should be reiterated and carrier testing should be encouraged to help with proper family counseling for future pregnancies for the family involved.

Descending aortic intramural hematoma with pulmonary embolism: A case report / 中国胸心血管外科临床杂志

@#Aortic intramural hematoma and pulmonary embolism are two rapidly progressive and life-threatening diseases. A 65-year-old male patient with descending aortic intramural hematoma and pulmonary embolism underwent pulmonary embolectomy and descending aortic stent-graft placement, with good postoperative results.

Autologous ilium graft combination with titanium plate for sternal reconstruction: A case report / 中国胸心血管外科临床杂志

@#The sternum is the pivotal component of the thoracic cavity. It is connected with the clavicle and ribs on the upper part and both sides respectively, and plays an important role in protecting the stability of the chest wall. Sternal resection usually results in a large segmental chest wall defect that causes the chest wall to float and requires sternal reconstruction. This paper reports a 62 years male patient with thymic squamous cell carcinoma with sternal metastasis, who underwent thymotomy, sternal tumor resection and autologous lilum graft combined with sternal reconstruction by titanium plate after relevant examination was completed and surgical contraindications were eliminated. The patient was followed up for 6 months, the respiratory and motor functions were normal and the thoracic appearance was good.

Echo-guiding percutaneous aortic stent implantation for coarctation of the aortic: A case report / 中国胸心血管外科临床杂志

@#Currently, transcatheter intervention has emerged as a first-line treatment for coarctation of the aortic. Due to the radiation exposure associated with catheter interventional therapy, there are numerous restrictions, which harms both patients and medical personnel and is dependent on sizable radiation apparatus. Here, we report for the first time a case of echo-guiding percutaneous aortic stent implantation for a 27 years female patient of reproductive age. After discharge, the patient's aortic coarctation pressure decreased to 18 mm Hg, and the surgical results were satisfactory.

Renal cell carcinoma, clear cell type with secondary polycythemia in a young Filipino male: A case report

Introduction@#Clear Cell Renal Cell Carcinoma, a renal cortical tumor characterized by malignant epithelial cells with clear cytoplasm and compact alveolar or acinar growth pattern interspersed with intricate arborizing vasculature.1 This is rare in people less than 45 years old. Though it has varied clinical manifestations, its classical triad: abdominal mass, hematuria, and groin pain only present in four to 17% of cases.2 We therefore present a case of renal cell carcinoma occurring in an unusual age group who presented with vague gastrointestinal symptoms and polycythemia which accounts only less than 5% of cases.3@*Case Presentation@#This is a case of a 28-year-old Filipino male who presented with epigastric pain with abdominal fullness and anorexia who later complained of frequent vomiting after solid and liquid intake. CBC revealed polycythemia. Gastroscopy with biopsy showed esophagitis Los Angeles classification Grade A and duodenal mass obstructing 95% of the lumen. Computed tomographic scan of whole abdomen revealed large renal mass, right of 15.9x9.35x11.34cm extending superiorly at the antropyloric region causing gastric luminal narrowing down to first and second segments of duodenum with a 4.2cm enlarged lymph node in aortocaval area. Magnetic resonance imaging revealed a huge complex right renal mass of 12x12x10cm in size extending beyond Gerota’s fascia with 8x5.2x6.2cm lymph node compressing the vena cava. Right radical nephrectomy was done for both supportive management to relieve the obstruction and for histologic diagnosis which revealed clear cell renal cell carcinoma. JAK2 gene mutation test was done to determine the cause of polycythemia and phlebotomy was performed to address the problem.@*Conclusion@#This case presents with vague gastrointestinal symptoms which is atypical of renal cell carcinoma, hence highlights the importance of properly investigating its cause. Furthermore, a multidisciplinary approach involving different subspecialties plays a significant role in the diagnosis and management in this patient.

Cardiocerebral infarction presenting as Opalski Syndrome: A case report

Introduction@#Opalski Syndrome is a rare type of stroke variant presenting with signs of lateral medullary syndrome plus ipsilateral hemiparesis. A confirmed myocardial infarction simultaneously occurring with this acute ischemic stroke syndrome makes this an even more challenging case of Cardiocerebral infarction.@*Case@#The patient is a 48-year-old female, a known diabetic and asthmatic, and was seen due to a 3-day history of sudden onset of non-rotatory dizziness associated with diplopia, non-projectile vomiting, numbness of the left side of the face and lingual dysarthria. She was initially admitted in a primary hospital wherein she developed chest pain, dyspnea and diaphoresis. She was transferred and was received with findings of miosis of the left eye, rotational nystagmus, left dysmetria, decreased pain and temperature sensation on the right extremities, left central facial palsy, tongue deviation to the left side, left hemiparesis and upward Babinski on the left. We localize this lesion on the left lateral medullary area with involvement of the caudal left corticospinal tract. Hence, a clinical impression of Opalski Syndrome was made. This was confirmed with cranial MRI findings of T2/FLAIR hyperintense focus involving the left lateral aspect of the medulla. The patient’s Troponin I was also elevated and she was managed as a case of acute coronary syndrome - NSTEMI. Hence, a diagnosis of Type III Cardiocerebral infarction was made. Medical intervention was started with dual antiplatelet therapy and anticoagulation with noted clinical improvement. @*Conclusion@#This case report highlights the diagnosis of Opalski Syndrome in a patient also presenting with Cardiocerebral infarction. There should be prompt recognition of the following disease entities to have an effective treatment, avoid cardiac and neurologic sequelae, and achieve an overall favorable prognosis.

Rare metastatic primary intra-abdominal follicular dendritic cell sarcoma poorly responsive to chemotherapy: A case report

Background@#Follicular dendritic cell sarcoma (FDCS) accounts for about 0.4% of soft tissue sarcomas. Approximately onethird of cases occur in extranodal sites and about 28% of extranodal FDCS may metastasize. Intra-abdominal occurrence is rare and there is limited published data to guide oncologists on how to best treat this malignancy.@*Case Presentation@#This is a case of a 33-year-old female who came in due to incidental finding of a left supraclavicular mass with 2-year history of early satiety. Neck node biopsy revealed a poorly differentiated malignant tumor with positive staining for CD21, CD23, vimentin and S100 consistent with FDCS. PET-CT revealed an intensely FDG-avid large mass in the left upper abdomen with signs of necrosis and mass effect. The patient was given three different chemotherapy regimens that included (1) gemcitabine/docetaxel, (2) single agent doxorubicin and (3) ifosfamide/etoposide, but she progressed on all these. Off-label use of bendamustine was then offered and after just the first cycle, the patient reportedly regained strength and was able to get up from wheelchair with noted interval decrease in size of the cervical mass. Unfortunately, the patient deteriorated and succumbed to infection and multiple pulmonary embolisms.@*Conclusion@#Intra-abdominal FDCS is a rare malignancy with heterogenous outcomes with no uniform treatment strategy at present. Molecular tumor board discussion and multi-disciplinary approach in extranodal FDCS is important in the diagnosis and management. Patients with multiple poor prognostic factors are at risk for tumor recurrence, metastasis, and death.

Class III camouflage orthodontic treatment in adult patient with anterior crossbite and abnormal functional shift: A case report

@#Skeletal Class III malocclusion is a complex malformation with a prevalence of 81.6% in Airlangga University Dental Hospital, distributed in patients aged 15-45 years old. Camouflage treatment of skeletal Class III malocclusion improves prognosis with a mild-to-moderate shift. This study aimed to discuss orthodontic camouflage as an option for adult patients with Class III malocclusion, emphasizing its indications, implications, and expected results. This report presents the case of a 17-year-old male patient with poor facial aesthetics associated with protruded chin, abnormal functional shift, and temporomandibular joint pain. The facial profile was concave with lower anterior multiple diastemas, mandibular lip protrusion, mandibular displacement, and anterior crossbite. This case was treated by camouflage therapy using straight wire appliance system combined with elastic class III. After 24 months of treatment during the pandemic, the mandibular displacement and the crossbite were corrected, the teeth were arched, the anterior crossbite fixed, and the profile became convex. Camouflage orthodontic treatment can be an effective management option to achieve functional occlusion, stability, and a satisfactory aesthetic impression in adult patients with mild to moderate skeletal Class III deformities, anterior cross bite, and functional shift.

Synovial sarcoma of the Hypopharynx in a Filipino female: A case report

@#Synovial sarcoma of the hypopharynx is an uncommon malignancy, with less than 100 cases reported in available journals. We report a case of a 22-year-old female presenting with dysphagia and enlarging hypopharyngeal mass, clinically diagnosed as hypopharyngeal malignancy, right, at least stage III. Histopathologic examination including immunohistochemistry study with TLE1 and SS18 Fluorescence In Situ Hybridization (FISH) confirm the diagnosis of synovial sarcoma. This is the first reported case of synovial sarcoma of the hypopharynx in the Philippines confirmed by SS18 FISH. Due to the size of the mass, chemoradiotherapy followed by surgery is the current plan of management for this patient.

Presumed monozygotic twin kidney transplantation with a thin basement membrane nephropathy donor: A case report

@#Identical or Monozygotic twin kidney transplant usually possess an excellent immunological match and provide the opportunity to minimize or even avoid immunosuppression toxicity. However, there are concerns regarding disease recurrence among end stage kidney disease (ESKD) patients with an unknown etiology. Together with the risk of inherent, familial disease affecting donors and recipients alike, more invasive tests such as a pretransplant biopsy are being considered to ascertain renal prognosis. A 30-year-old female, known case of CKD Stage 5D from an unknown etiology, with secondary hyperparathyroidism and heart failure, presented at our OPD for kidney transplantation. Her donor is her identical twin who is asymptomatic and denies comorbidities. The recipient discloses a previous history of blood transfusion. Immunological workup revealed the following: matched blood type, zero HLA mismatch, negative T-cell tissue crossmatch but with a positive Class I HLA antigen screen. Antibody specificity revealed the presence of donor specific antibodies (DSA). After workup completion, the patient underwent a right kidney transplant with a preimplantation wedge biopsy on the donor kidney. Immediate graft function was noted post operatively. The wedge biopsy revealed a thinned glomerular basement membrane, consistent with Thin Basement Membrane Nephropathy (TBMN). The patient was started on immunosuppression and prophylaxis during the duration of the post operative period without any complications. Five months post-transplant, both the recipient and donor maintain an adequate renal function without any signs of allograft rejection. In this case report, we have demonstrated that TBMN may serve as a viable donor for a presumed monozygous twin kidney transplantation. When a live donor with TBMN is being considered, a thorough work-up and identification of high-risk features are essential to exclude other progressive renal diseases during the pretransplant evaluation.

Minimal change disease associated with SARS-CoV-2 (COVID-19) infection among adult Filipinos: A report of two cases and review of related literature

@#A 40-year-old Filipino female with a history of right total mastectomy for a low-grade phyllodes tumor was admitted due to stillbirth. Her laboratory results revealed an incidental finding of a positive COVID-19 RT-PCR swab, serum creatinine 1.04 mg/dL, urine RBC 1/HPF, and a 24-hour urine protein of 9.22 grams with hypoalbuminemia and dyslipidemia. Serologic workup was noted to be negative. A kidney biopsy was performed which demonstrated unremarkable light microscopy (LM) and immunofluorescence (IF) with widespread podocyte-foot process effacement, consistent with minimal change disease. She was started on prednisone (1 mg/kg/day) and achieved complete remission after six weeks. A 61-year-old Filipino male with a history of Type 2 Diabetes Mellitus, Hypertension, Dyslipidemia, and mild COVID-19 infection four months prior, now presented with diarrhea. On admission, his COVID-19 RT-PCR swab revealed a reinfection. Workup demonstrated a serum creatinine 3.39 mg/dL, urine RBC 2/HPF, and urine ACR 2.6 g/g. Serologic tests were negative. He was diagnosed with Nephrotic Syndrome and underwent kidney biopsy. Findings showed an unremarkable LM and IF with widespread podocyte-foot process effacement, consistent with minimal change disease. He was started on prednisone (1 mg/kg/day) and achieved complete remission after eight weeks. SARS-CoV-2 (COVID-19) may present with a variety of kidney involvement which includes glomerulopathies such as MCD. An accurate diagnosis using the patient’s clinical presentation, renal histopathology, and adjunct laboratory examinations, is essential to direct effective management and good outcomes.

A case report of inferior oblique reattachment after Inferomedial Orbital Wall Reconstruction from Total Maxillectom

@#The detachment and reattachment of inferior oblique (IO) have been done by ophthalmologists in inferomedial orbital wall fracture repair to avoid inducing a new diplopia in patients post-operatively. However, doing them in orbital wall reconstruction in patient who underwent maxillectomy for a malignancy has not been described yet. We describe a case where a disinserted inferior oblique was reattached after titanium mesh implantation to prevent diplopia after the surgery. This is the case of a 40-year-old male diagnosed with recurrent bilateral nasomaxillary ameloblastoma who underwent total maxillectomy of the right with removal of the inferomedial orbital wall, and detachment and reattachment of inferior oblique with no resulting inferior oblique palsy and diplopia from hypotropia or incyclotorsion. This is the first report, to our knowledge, to describe reattachment of IO in its approximate insertion during reconstruction of the inferomedial orbital wall after total maxillectomy from a malignancy with no resulting diplopia.

A case report of a Filipino boy with childhood cataract and clinically diagnosed Roberts Syndrome

@#Childhood cataract is a common cause of visual impairment. Familial types are uncommon among Filipinos. Furthermore, it is not common to have one that follows an autosomal dominant pattern of inheritance but with associated syndromic presentation like Roberts syndrome which is an autosomal recessive disorder. This is a case of a 9-year-old Filipino boy with cataract in the left eye associated with low-set ears, facial asymmetry, underdeveloped nasal ala, cleft lip and palate, macroglossia, micrognathia, short right shin, and absent feet. Patient was clinically diagnosed with Roberts syndrome. We present a clinically diagnosed Roberts syndrome (RS), the first reported RS in a Filipino in local and international literature to our knowledge with an autosomal dominant childhood cataract. Genetic testing can assist in the confirmation of this case.

Nodular melanoma in a 53-year-old male with glioblastoma multiforme: A rare case report

@#Although melanoma only accounts for 1% of skin cancers, it is responsible for most skin cancer deaths. Glioblastoma multiforme, a high-grade astrocytoma, is the most aggressive and devastating primary brain tumor. These two diseases remain to be the biggest therapeutic challenge in both specialties of dermatology and neuro-oncology. A 53-year-old Filipino male who presented with a 2-year history of generalized dark brown and black patches on the body developed weakness and numbness of the left extremities. Biopsy and immunohistochemical staining of the skin revealed nodular melanoma with adjacent regressing melanoma. Biopsy of the intracranial mass showed glioblastoma multiforme. One month after the partial excision of the intracranial mass, the patient expired due to brain herniation. Nodular melanoma and glioblastoma multiforme may occur concomitantly in a patient. A review of the literature suggests a shared genetic predisposition. Its existence carries a poor prognosis and requires early detection to start aggressive treatment.

Management of advanced thymoma presenting with myasthenia gravis in a resource-limited setting: A case report

@#Thymomas are rare tumours which generally account for only 0.2 – 1.5% of mediastinal tumours in adults. Around 40% of patients present with systemic symptoms such as motor weakness due to myasthenia gravis (MG), pure red cell aplasia, and hypogammaglobulinemia. Based on recent guidelines, management of advanced thymoma uses a multimodal approach, which is thymectomy followed by radiotherapy, but not all health care centers have radiotherapy facilities. A 52-year-old woman presented with nasal voice and had difficulty swallowing food. Patient was diagnosed with myasthenia gravis (MG). CT scan with contrast of the thorax showed a heterogenous solid mass in anterior mediastinum. Histopathological examination showed thymoma type B2. Thymectomy followed by seven cycles of platinum-based chemotherapy were done on the patient. Evaluation afterward showed complete remission of thymoma. The patient’s motor weakness improved after the chemotherapy. Post-chemotherapy period was uneventful at six months on follow-up visit. The dosage of acetylcholinesterase inhibitor drug is reduced periodically due to improvement in motor weakness. The case emphasizes how to manage an advanced thymoma with MG with limited therapeutic options, and the importance of multidisciplinary management involving oncologists, surgeons, and neurologists.

Evaluation of Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum

ABSTRACT Purpose: To assess Meibomian gland dysfunction using meibography in patients with xeroderma pigmentosum and correlate with ocular surface changes. Methods: This cross-sectional study evaluated patients with xeroderma pigmentosum. All patients underwent a comprehensive and standardized interview. The best-corrected visual acuity of each eye was determined. Detailed ophthalmic examination was conducted, including biomicroscopy examination of the ocular surface, Schirmer test type I, and meibography, and fundus examination was also performed when possible. Meibomian gland dysfunction was assessed by non-contact meibography using Oculus Keratograph® 5M (OCULUS Inc., Arlington, WA, USA). Saliva samples were collected using the Oragene DNA Self-collection kit (DNA Genotek Inc., Ottawa, Canada), and DNA was extracted as recommended by the manufacturer. Factors associated with abnormal meiboscores were assessed using generalized estimating equation models. Results: A total of 42 participants were enrolled, and 27 patients underwent meibography. The meiboscore was abnormal in the upper eyelid in 8 (29.6%) patients and in the lower eyelid in 17 (62.9%). The likelihood of having abnormal meiboscores in the lower eyelid was 16.3 times greater than that in the upper eyelid. In the final multivariate model, age (p=0.001), mutation profile (p=0.006), and presence of ocular surface malignant tumor (OSMT) (p=0.014) remained significant for abnormal meiboscores. For a 1-year increase in age, the likelihood of abnormal meiboscores increased by 12%. Eyes with OSMT were 58.8 times more likely to have abnormal meiboscores than eyes without ocular surface malignant tumor. Conclusion: In the final model, age, xeroderma pigmentosum profile, previous cancer, and clinical alterations on the eyelid correlated with a meiboscore of ≥2. Meibomian gland dysfunction was common in patients with xeroderma pigmentosum, mainly in the lower eyelid. The severity of Meibomian gland dysfunction increases with age and is associated with severe eyelid changes.

Partial regression of peripapillary myelinated nerve fibers after non-arteritic anterior ischemic optic neuropathy / Regressão parcial das fibras nervosas peripapilares mielinizadas após neuropatia óptica isquêmica anterior não arterítica

ABSTRACT A 71-year-old woman presented a non-arteritic anterior ischemic optic neuropathy in an optic nerve with previously registered superonasal peripapillary myelinated nerve fibers. Her past medical history was significant for controlled systemic hypertension, hyperlipidemia, and diabetes mellitus. The physiologic cup was absent in both optic discs. Non-arteritic anterior ischemic optic neuropathy mainly affected the temporal and inferior sectors of the peripapillary retinal nerve fiber layer, as could be demonstrated by retinal nerve fiber layer optical coherence tomography and optic disc optical coherence tomography angiography. Unlike other published reports, just a slight regression of the myelinated nerve fibers was observed after 1 year of follow-up. This occurred because ischemia mainly affected the temporal and inferior peripapillary sectors, whereas myelinated nerve fibers were superonasal to the optic disc.

RESUMO Uma mulher de 71 anos de idade apresentou neuropatia óptica isquêmica anterior não arterítica no nervo óptico com fibras nervosas peripapilares mielinizadas previamente registradas. Seu histórico médico foi significativo para hipertensão arterial sistêmica controlada, hiperlipidemia e diabetes mellitus. Em ambos os discos ópticos, a tacícula fisiológica esteve ausente. A neuropatia óptica isquêmica anterior não arterítica afetou principalmente os setores temporal e inferior da camada de fibras nervosas da retina peripapilar, como demonstrado pela tomografia de coerência óptica da camada de fibras nervosas da retina e pela angiotomografia de coerência óptica do disco óptico. Ao contrário de outros relatórios publicados, apenas uma ligeira regressão das fibras nervosas mielinizadas foi observada após um ano de acompanhamento. Isto pode ser explicado pelo fato da isquemia ter afetado principalmente os setores temporal e inferior peripapilares, enquanto as fibras nervosas de mielina eram nasal superior ao disco óptico.