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Introduction: The nasolacrimal canal is an important structure. It morphologically varies throughout its length. The narrowest part of the canal is the key factor in the development of the primary acquired nasolacrimal duct obstruction. This study was aimed to assess anteroposterior nasolacrimal canal diameter and study its variation by comparing the data with the gender of the patients. Methods: A descriptive cross-sectional study was conducted among the patients coming to the Chitwan Medical College, Nepal from January 2021 till June 2021. In total 200 eligible CT scans of patients were included. CT scan was performed using Somatom Definition Edge CT scan machine. The data were entered into Microsoft excel, later transferred into Statistical Package of Social Science (SPSS) version 16 and analysed using descriptive statistics. Results: There were 200 participants in total in this study in which majority were males 126 (63.3%) male and 72 (36.4%) female participants. The mean age of the participants was 40.81±18.06 years. The measurement on the right side of both genders was more than the left side. In total the measurement on the right side (Mean ± SD (mm)) was 4.18±0.092 mm. Conclusions: This study concludes the diameter of nasolacrimal canal differ by gender and the sides of individual. It was found to be more in females and on the right side of Nepalese.

Ameloblastic fibro-odontoma (AFO) is a rare benign mixed odontogenic tumor that affects children and young adults. AFO occurs mainly intraosseous. Extraosseous AFO is extremely rare. We report 2 cases of rare peripheral ameloblastic fibro-odontoma in 2- and 12-year-old female patients. Microscopic examination revealed a benign proliferation of odontogenic epithelium associated with a dentinoid material distributed within a cell-rich mesenchymal stroma resembling dental papilla. Simple surgical excision of the lesion is usually curative. There was no recurrence after a short period of follow-up. Clinicians should be cognizant of this rare entity, which can be considered in a differential diagnosis of gingival growths that are noted in early childhood.

Neuroendocrine carcinomas (NEC) are a group of malignant neoplasms usually located in the lungs or gastrointestinal tract. Fewer cases are located in the head and neck, and in these rare presentations, the lingual tonsil, larynx, and major salivary glands are the most frequently affected sites. NECs exhibit similar characteristics regardless of where they arise. However, because these neoplasms are rare, a clear understanding of their aetiopathogenesis has yet to be described, and options for treatment have varied and are not unified. A rare NEC of the floor of the mouth is reported here; it appears that this location has not been reported previously. The diagnosis was established through histopathological analysis, and the patient underwent systemic treatment. He had a partial response to treatment in the first 3 months, but died 6 months after the initial diagnosis. This highly uncommon tumour can pose a significant diagnostic challenge for clinicians and pathologists alike and can result in diagnostic delay.

The association of pheochromocytoma in patients with neurofibromatosis type I has rarely been reported in low-income countries, especially on the African continent. A 43-year-old woman with neurofibromatosis type I was diagnosed with a right adrenal pheochromocytoma in Pretoria, South Africa. To our knowledge, this report is the first case to be published of a patient with neurofibromatosis type I diagnosed with a pheochromocytoma in Pretoria, and one of three cases on the African continent. The rarity may be due to the two associated conditions being under-reported, undiagnosed, misdiagnosed or possibly the association is rare on the African continent. The clinician dealing with these two conditions should be aware of the association.

Summary Aim Orbital subperiosteal hematoma (OSPH) is a rare entity following blunt trauma that can significantly affect the vision of a growing child. The purpose of this study was to describe the spectrum of the clinical presentations along with the imaging findings and treatment outcome of traumatic OSPH. Case Series This is a retrospective case series of six patients below 16 years of age, diagnosed with OSPH following trauma. Electronic medical records were reviewed for details of clinical features, imaging findings, management details and outcomes. Discussion The median duration of the presentation was 7 days (range 6–50 days). Proptosis and dystopia remained the most common presenting features. Significant vision loss was noted in four patients at presentation. A computed tomography (CT) scan revealed the superior quadrant of the orbit to be involved most frequently. Five patients were treated surgically and one conservatively. Significant vision loss was noted in one patient despite of initial surgical management. Conclusion Although rare, OSPH should be considered as a differential in children presenting with proptosis and dystopia following blunt trauma. Younger children are particularly at higher risk for developing permanent visual loss and should be treated promptly by draining the OSPH.

Background/Objective Vanishing bone disease (VBD) is a rare entity, characterized by massive osteolysis and lymphovascular proliferation. Our objective was to report the case of a 22-year-old man who presented with VBD of the ribs and the challenges involved with its management in this location. Case Report A 22-year-old man presented with left-sided chest and back pain. An x-ray revealed that the fourth to sixth ribs on the left side of the chest were missing. The erythrocyte sedimentation rate was normal (5 mm/h; normal value, Discussion/Conclusion VBD may present diagnostic and therapeutic challenges; the abovementioned patient was diagnosed with VBD after excluding secondary causes of osteolysis. Although a high index of suspicion is required to diagnose VBD, it also mandates close monitoring and follow-up.

The term Sneddon's syndrome (SS) has been used since 1965 to describe a vasculopathy characterized by a combination of cerebrovascular disease with livedo racemosa. SS may be classified as antiphospholipid+ (aPL+) or antiphospholipid- (aPL-). Little is known about aPL- SS; in this review we describe the epidemiology and pathogenesis of aPL- SS, as well as the clinical and histologic features. We discuss recent findings in terms of neurologic and cardiac involvement. Moreover, differential diagnoses of conditions that may present with both livedo racemosa and stroke are discussed. Finally, we discuss real-life practical issues such as the initial investigations to be performed, long-term follow-up, and therapeutic management of aPL- SS patients.

Anal adenocarcinoma is a rare clinical entity for which the optimal management is not defined.This study aimed to describe the multidisciplinary management and outcomes of patients with anal adenocarcinoma.This is a retrospective cohort study.This study was conducted at a quaternary cancer center.Men and women with anal adenocarcinoma treated between 1995 and 2016 were selected.Fifty-two patients were treated with either chemoradiotherapy or trimodality therapy including radiation therapy, chemotherapy, and surgical resection.Local failure, regional failure, and distant metastasis rates were estimated using the cumulative incidence method. The Kaplan-Meier method was used to estimate progression-free survival and overall survival. The multivariable Cox proportional hazards model was used to evaluate the clinical predictors of outcome.There was a higher 5-year rate of local failure in patients treated with chemoradiotherapy compared with trimodality therapy (53% vs 10%; p0.01). The 5-year incidence of distant metastases was 29% (trimodality therapy) versus 30% (chemoradiotherapy; p = 0.9); adjuvant chemotherapy did not reduce the incidence of distant metastases (p = 0.8). Five-year overall survival was 73% (trimodality therapy) versus 49.4% (chemoradiotherapy; p = 0.1). On multivariable analysis, factors associated with worse overall survival were treatment with chemoradiotherapy, cT3-4 category disease, and node-positive disease.This study is limited by its small sample size and retrospective nature.Although treatment may continue to be tailored to individual patients, better outcomes with a trimodality therapy approach were observed. See Video Abstract at http://links.lww.com/DCR/B708.ADENOCARCINOMA ANAL: UNA ENTIDAD POCO FRECUENTE EN NECESIDAD DE UN MANEJO MULTIDISCIPLINARIO.El adenocarcinoma anal es una entidad clínica poco frecuente por lo que aún no se define el manejo óptimo.Describir el manejo multidisciplinario y los resultados de los pacientes con adenocarcinoma anal.Estudio de cohorte retrospectivo.Centro de cáncer cuaternario.Hombres y mujeres con adenocarcinoma anal tratados entre 1995 y 2016.Cincuenta y dos pacientes fueron tratados con quimiorradioterapia o terapia trimodal que incluyó: radioterapia, quimioterapia y resección quirúrgica.Se estimaron las tasas de falla local, falla regional y metástasis a distancia mediante el método de incidencia acumulada. Se utilizó el método de Kaplan-Meier para estimar la supervivencia libre de progresión y la supervivencia global. Los riesgos proporcionales de multivariable Cox se utilizaron para evaluar los predictores clínicos de los resultados.Hubo una mayor tasa de falla local a cinco años en pacientes tratados con quimiorradioterapia en comparación con terapia trimodal (53% vs 10%; p0,01). La incidencia a cinco años de metástasis a distancia fue del 29% (terapia trimodal) versus 30% (quimiorradioterapia) (p = 0,9); la quimioterapia adyuvante no redujo la incidencia de metástasis a distancia (p = 0,8). La supervivencia global a cinco años fue del 73% (terapia trimodal) versus 49,4% (quimiorradioterapia); p = 0,1. En el análisis multivariable, los factores asociados con una peor supervivencia general fueron el tratamiento con quimiorradioterapia, enfermedad de categoría cT3-4 y enfermedad con ganglios positivos.Este estudio está limitado por su pequeño tamaño de muestra y su naturaleza retrospectiva.Aunque el tratamiento puede seguir adaptándose a pacientes individuales, se observaron mejores resultados con un enfoque TTM. Conslute Video Resumen en http://links.lww.com/DCR/B708. (Traducción- Dr. Francisco M. Abarca-Rendon).

Ameloblastoma is a locally aggressive, benign epithelial odontogenic neoplasm currently classified to include conventional, unicystic, and extraosseous/peripheral subtypes. Giant cells have been reported in various malignancies but rarely in odontogenic neoplasms. To date, only a single case of unicystic ameloblastoma with stromal giant cells has been reported in the literature. We report two new cases with histologic features compatible with unicystic ameloblastoma with stromal giant cells. CD68 immunohistochemical staining of the giant cells supported a histiocytic origin. Further research is needed to better understand the origin and nature of these giant cells.

Xanthogranulomatous cholecystitis (CXG) is a rare entity of cholecystitis, characterized by the presence of xanthogranulomas within the gallbladder wall, that could be misdiagnosed as a vesicular carcinoma. We report a case of 66-year-old man with xanthogranulomatous cholecystitis associated with an incidental finding of a fusion of the planes of the liver which is a rare anatomic variant. Imaging especially ultrasounds, CT scan, and MRI play a key role in the characterization of those anomaly, thus avoiding a non-suitable surgical procedure.

Actinomycotic mycetoma is a disease of the tropical region and usually presents as a chronic, suppurative and deforming granulomatous infection. We present an unusual case of actinomycotic mycetoma of the abdominal wall that was found to infiltrate into the bowel. A 51 year-old man presented with pain and swelling in the left flank of 2-year duration. Even after comprehensive preoperative evaluation with advanced radiological imaging, biochemistry and pathology, the diagnosis could not be arrived at. Histopathological examination of the excised specimen after the surgery guided to the diagnosis of actinomycotic mycetoma, which entirely changed the management in the postoperative period. We propose that mycetoma should be kept as a possible differential diagnosis for anterior abdominal wall swelling in the indicated clinical setting and the investigations be done keeping the same in mind. Otherwise, a lot of valuable time may be lost allowing the disease to progress further.

La localización extranodal gastrointestinal del linfoma de Hodgkin comprende el 5% de todos los linfomas. Dentro de este grupo, el linfoma anal primario representa menos del 0,05%, siendo por tanto una entidad extremadamente rara. Por otro lado, los tumores neuroendocrinos son un grupo heterogéneo de neoplasias relativamente poco frecuentes, pero de localización fundamentalmente digestiva. La asociación entre un linfoma de Hodgking de localización anal y un tumor neuroendocrino intestinal no ha sido descrita previamente en la literatura, pero no es en absoluto raro que los tumores neuroendocrinos puedan coexistir con otro tipo de neoplasias. Los autores presentan el caso infrecuente de presentación de linfoma Hodgkin de localización anal asociado a un tumor neuroendocrino intestinal en una paciente mujer de 74 años, describiéndose la clínica, resultados de pruebas complementarias y tratamiento recibido.

Hepatocellular carcinoma (HCC) is the most common primary malignancy of the liver. It most commonly metastasizes haematogenously to the lungs and bones, less commonly via lymphatics to lymph nodes. However, metastasis to the parapharyngeal space has yet to be reported. This is the first clinical report of the treatment of parapharyngeal metastasis from HCC. The case of a 46-year-old man who was found to have a parapharyngeal soft tissue mass during routine follow-up 12 years post deceased-donor liver transplantation for hepatitis B-related HCC is reported here. This was investigated and diagnosed to be metastatic HCC. He underwent excision of the parapharyngeal metastasis, followed by adjuvant radiotherapy. Parapharyngeal metastasis is a rare occurrence in HCC. It is important to be cognizant of the possibility of disease spread to this location in order to ensure early detection and treatment. Surgical excision with adjuvant radiotherapy should be considered to achieve disease control.

Intravascular leiomyomatosis (IVL) is a rare entity defined by benign smooth uterine muscle cells that typically originate from the uterus with the potential to spread into veins possibly up to the heart. The diagnosis for patients presenting with cardiac symptoms may be difficult and imaging often interpreted as thrombus or atrial myxoma.

Left-sided portal hypertension is a rare entity with considerable splenogastric collateralization. We report a case of esophagectomy in the setting of left-sided portal hypertension with resulting treatment of both the esophageal cancer and the portal hypertension.

Isolated fallopian tube torsion is an extremely rare occurrence in a young female. The lady concerned presented with acute abdominal pain and the ovaries were normal on the scan with dilated fallopian tubes. On laparoscopy, it was revealed that she was suffering from fallopian tube torsion and laparoscopic salpingectomy was performed. The patient recovered well postoperatively.

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a rare neoplasm arising from spindle cells and most commonly arising from pleura. Spinal SFT/HPC is a rare entity; hence, it is not on the top of the differential diagnosis list when a clinician faces a spinal lesion. In the review of the literature, there exist less than 50 case reports of intradural extramedullary SFT/HPC. Here, we present a 54-year-old female patient who underwent subtotal surgical excision of an intradural extramedullary spinal mass pathologically reported to be SFT/HPC and had symptomatic recurrence in the 3rd year of follow-up. Surgical intervention was unachievable and the patient was given 45 Gy to the surgical cavity followed by a 5.4 Gy boost to visible tumor with external radiotherapy. Patient reported significant relief of her symptoms. We aim to contribute to the formation of a treatment algorithm for this rare entity.

Spindle cell oncocytoma (SCO) of the pituitary is a rare tumor of the adenohypophysis occurring in the sellar/suprasellar region. This tumor has been recognized as a distinct entity by the WHO Classification of CNS tumor in 2007. Spindle cell oncocytoma of the pituitary gland accounts for 0.1–0.4% of all sellar region tumors and is predominantly seen in the older adult population. This rare entity simulates clinical and radiological features of pituitary adenoma and is often misdiagnosed. Though WHO grade 1, the tumor can recur and have invasive properties. Herein, we report a 61-year-old woman with panhypopituitarism and temporal field cut, clinically and radiologically diagnosed as pituitary macroadenoma, while the histomorphological and immunohistochemistry features helped in arriving at a diagnosis of Spindle Cell Oncocytoma. The clinicopathological, histomorphological, immunohistochemical, and molecular properties of the tumor are further discussed. Keywords: Spindle cell oncocytoma, Adenohypophysis, Pituitary, Macroadenoma, Panhypopituitarism.

Serous borderline tumor is a rare entity of peritoneum which can lead to diagnostic dilemma due to its resemblance with reactive lesions of peritoneum and ovarian borderline and malignant neoplasms. Complete clinical details, histopathological evaluation and immunohistochemistry aid in arriving at correct diagnosis. Recognizing this entity is important as it carries good prognosis. Keywords: Mesothelial hyperplasia, Primary peritoneal carcinoma, Serous borderline tumor.

Urachal carcinoma is a very rare tumor, commonly found in the urachal remnant connecting the bladder dome to the umbilicus. Diagnosis is often challenging due to the location of the tumor and its late presentation. We hereby report the case of a 49-year-old female where the diagnosis of urachal carcinoma was made and a robotic partial cystectomy associated with en bloc resection of the umbilicus was performed. We aim to present the clinical aspects, presentation, and diagnosis of this rare entity along with a review of the literature.

Chronic acral hyperkeratotic dermatosis includes several conditions such as lichen simplex chronicus (LSC), hypertrophic lichen planus (HLP), psoriasis vulgaris (Ps), acral acanthosis nigricans, acquired zinc deficiency, and necrolytic acral erythema (NAE). LSC, Ps, and HLP respond to conventional treatments such as topical corticosteroids, immuno-modulators such as tacrolimus, and oral methotrexate. Zinc-responsive acral hyperkeratosis is a novel entity that resembles the above mentioned diagnoses clinically but fails to respond to the above treatment options. NAE is a rare condition, commonly associated with hepatitis C virus infection and manifest similar clinical features of zinc-responsive acral hyperkeratosis, but differs histopathologically. Both conditions show a good response to oral zinc supplementation. As there is a paucity of literature on zinc-responsive acral hyperkeratosis, we are highlighting the case.

Tricuspid annular PVCs constitute 8% of idiopathic PVCs and 5% of RV PVCs. Although a rare entity to encounter in routine clinical practice, it can be a prime culprit behind major arrhythmic burden in few individuals. Electroanatomic approach with sub tricuspid retrograde catheter technique can target those annular PVCs and decrease the arrhythmia burden to normal or near normal range. Although procedural approach for parahisian PVCs requires a close look to avoid injury to native conduction system, TA PVCs are a real challenge due to inherent catheter instability and contact issue in sub tricuspid retrograde approach.

Infectious endocarditis is an infrequent pathology but with a very significant morbidity and mortality context. The MostCommon responsible germs are streptococci and staphylococci.Neisseria is a strain of bacteria characterized by several subgroups such as gram-negative diplococci which are present in the respiratory tract and which are generally harmless and other gram-negative bacteria such as Neisseria gonorrhoeae which are known to be pathogens involved in several pathologies.We report a case of infectious endocarditis in 37-year-old patient with mitral valve replacement who presented a prolonged febrile syndrome with discovery on transesophageal echocardiography of vegetation and blood cultures of an SP Neisseria.

Background: Moyamoya disease (MMD) and peripheral pulmonary artery stenosis (PPAS) are relatively rare and demonstrate steno-occlusive vascular lesions in different organs. Genetic studies identified RNF213 polymorphism c.14576G>A (rs112735431) as a susceptibility variant for East Asian MMD. RNF213 polymorphism c.14576G>A is further associated with various vascular lesions of other organs. In this study, we aimed to clarify the incidence and clinical manifestations of PPAS in MMD patients and analyze the correlation between RNF213 genotype and PPAS. Methods: This retrospective case-control study investigated the association between RNF213 polymorphism and PPAS in 306 MMD/quasi-MMD patients, reviewing the medical charts and imaging records of consecutive patients with MMD admitted from January 2015 to December 2020. Results: PPAS was observed in 3 MMD/quasi-MMD patients (0.98%, 3/306). RNF213 polymorphism c.14576G>A was determined for all 306 MMD/quasi-MMD patients. The incidence of PPAS in RNF213-wildtype, RNF213-heterozygote, and RNF213-homozygote MMD/quasi-MMD patients was 0% (0/101), 0.5% (1/200), and 40% (2/5), respectively. The association between PPAS and homozygote polymorphism of RNF213 c.14576G>A was statistically significant in MMD/quasi-MMD patients (p = 0.0018). In all cases, pulmonary artery hypertension due to PPAS was evident during their childhood and young adolescent stages. Surgical indications for MMD were discouraged in 1 case due to her severe cardiopulmonary dysfunction. Conclusions: The homozygote variant of RNF213 polymorphism c.14576G>A can be a potential predisposing factor for PPAS in MMD/quasi-MMD patients. Despite the relatively rare entity, PPAS should be noted to determine surgical indications for MMD/quasi-MMD patients.

Ectopia is defined as the presence of a normal-appearing tissue in an abnormal location, where it is not normally found. In the literature, it is also referred to as heterotopia and is described as choristoma when it forms a mass. Lung tissue ectopia is a rare anomaly and is characterized by presence of mature lung tissue comprising of well-formed bronchi, bronchioles and alveoli at an abnormal location. Occurrence of ectopic lung tissue in the skin is an extremely rare entity and the diagnosis is mostly established by histopathologic examination. This report documents a rare occurrence of cutaneous ectopic lung tissue at two locations (neck and back). Cutaneous ectopia at multiple sites has not been described in the literature till date. This article is protected by copyright. All rights reserved.

Tuberculous otitis media is a rare entity which requires high index of suspicion in an endemic country like India. A 38-year-old female with no prior history of tuberculosis or any predisposing factor presented to us with fever and altered sensorium. She was subsequently diagnosed to have disseminated tuberculosis with multiple cerebral tuberculomas. She complained of ear discharge which on further evaluation was established as smear positive tuberculous otitis media. This rare coexistence is seldom reported in literature.

Solitary extramedullary plasmacytoma is a rare entity. The diagnosis is established by clinical evaluation, detailed radiological workup and histopathology with immunohistochemistry markers. There is no clear standard of care for a solitary laryngeal plasmacytoma. A case of solitary laryngeal plasmacytoma is being reported here with the rationale for the chosen treatment modality.

Mastoid lymph node inflammation is a rare entity. Pathological conditions in the vicinity of the mastoid processes can be challenging for maxillofacial head-neck surgeons to address. We report a case of suppurative mastoid lymphadenitis in an 18-year-old Caucasian male. To our knowledge, there are no publications that highlight the clinical, ultrasonographic, intra-, and postoperative data for any pathologic process that presented as mastoiditis.

Spontaneous rupture of the ureter is a rare entity most commonly a result of ureteral stone disease. Despite the overall low prevalence, the diagnosis should be considered so appropriate imaging and management can be performed without delay. Although treatment has shifted towards more minimally invasive intervention and conservative management, open surgical intervention should be considered in certain situations to mitigate the risk of long-term complications. Here we present a unique case of spontaneous ureteral rupture complicated by recurrent infections and fistulous sinus tract formation.