keyword
https://read.qxmd.com/read/34294225/congenital-pyriform-aperture-stenosis-ten-years-experience
#1
Cinthia Giselle Pérez, Paula Gabaldon Masse, Alejandro Cocciaglia, Hugo Rodríguez
INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is an uncommon malformation and a rare cause of respiratory distress in neonates that may require early surgical intervention. The aim of this study is to report our experience in patients operated for CNPAS and present characteristics of this population. MATERIAL AND METHODS: A retrospective, analytical cohort study was conducted of patients who underwent surgical treatment for CNPAS in the Hospital de Pediatría Garrahan from May 2008 to May 2018...
July 2021: Acta otorrinolaringologica española
https://read.qxmd.com/read/33957404/seromucinous-hamartoma-of-ethmoid-sinus-in-pediatric-patient-case-report
#2
Ghassan Alokby, Rawan S Alayed, Jude B Al Fayez
INTRODUCTION AND IMPORTANCE: Seromucinous hamartoma is a rare benign glandular proliferation arising from the respiratory epithelium, which was originally described by Baillie and Batsakis in 1974. Since this time, case reports started to be published on SH, as a middle aged and elderly disease, here we report a case of a pediatric patient who found to have SH. PRESENTATION OF THE CASE: 2-year-old girl, brought by her parent with a complain of a mass at the right medial canthal area for one year...
April 27, 2021: International Journal of Surgery Case Reports
https://read.qxmd.com/read/33912866/congenital-mucocele-of-the-nasal-dorsum-a-case-report
#3
Seçil Bahar Dal, Ömer Faruk Ünal
Congenital nasal dorsum cysts are very rare lesions. Its differential diagnosis lies between gliomas, dermoid cysts and encephaloceles. We present a case of solitary congenital external nasal cyst with no intranasal fistulous tract connection in a newborn. Histopathologic analysis of the mass demonstrated findings consistent with an external mucocele. Total excision with external open approach provided the cure with good cosmetic outcome. This is the first report presenting an external mucocele in a newborn in the literature...
March 2021: Turkish Archives of Otorhinolaryngology
https://read.qxmd.com/read/33889472/nasal-embryonal-rhabdomyosarcoma-in-the-pediatric-population-literature-review-and-report-of-midline-presentation
#4
Jordan H Larson, Rachel Rutledge, Laura Hunnell, Daniel K Choi, Robert G Kellogg, Sanjay Naran
Background: Congenital midline nasal masses are rare anomalies and are typically benign nasal dermoid sinus cysts (NDSCs). Rhabdomyosarcomas (RMSs) are even less common, and only a fraction affect sites like the external nose, nasal cavity, nasopharynx, and paranasal sinuses. We review the clinical presentation and treatment of nasal, nasopharyngeal, and paranasal RMSs and report the first documented midline presentation. Methods: We queried PubMed for articles with titles containing the terms rhabdomyosarcoma or sarcoma botryoides and nose, nasal, paranasal, sinonasal, nasopharynx, or nasopharyngeal...
April 2021: Plastic and Reconstructive Surgery. Global Open
https://read.qxmd.com/read/33876893/nasal-glial-heterotopia-a-rare-interdisciplinary-surgical-challenge-in-newborns
#5
Paolo Gasparella, Georg Singer, Stephan Spendel, Eszter Nagy, Hans Georg Eder, Philipp Klaritsch, Holger Till, Emir Q Haxhija
Nasal Glioma (NG) represents a rare congenital abnormality of the neonate, which can be associated with skull defects or even a direct communication to the central nervous system. MRI serves valuable information for differentiation from encephalocele, dermoid cyst and congenital hemangioma. Complete resection remains the treatment of choice. We present two cases of NG, which were both suspected during prenatal ultrasound and MRI. In the first case, postnatal MRI showed a transcranial continuity. Mass excision was performed and the defect was covered by a glabellar flap allowing a good cosmetic result...
April 20, 2021: La Pediatria Medica e Chirurgica: Medical and Surgical Pediatrics
https://read.qxmd.com/read/33824791/red-blood-cell-exchange-in-a-patient-with-extramedullary-hematopoiesis-and-cor-pulmonale-secondary-to-beta-thalassemia
#6
Pallavi Kopparthy, Amar H Kelkar, Kunal Aggarwal, Samantha De Filippis, Brad Fletcher
RATIONALE: Beta thalassemia is a congenital defect in the production of the beta globin chain. Patients with beta thalassemia major will have higher levels of hemoglobin F (HbF), which is suboptimal in releasing oxygen to tissue. Herein, we describe the use of red blood cell (RBC) exchange transfusion, a therapy typically used in sickle cell patients, in the management of a patient with beta thalassemia with extensive extramedullary hematopoiesis and elevated levels of HbF. Patient concerns: A 34-year-old male of mixed African American and Southeast Asian descent with a known history of beta thalassemia major presented with progressive dyspnea on exertion with marked fatigue...
March 1, 2021: Curēus
https://read.qxmd.com/read/33795610/developmental-cysts-of-the-orbit-with-mixed-conjunctival-and-cutaneous-epithelial-phenotypes
#7
Kaveh Vahdani, Caroline Thaung, Eugene Tay, Geoffrey E Rose
AIM: To describe the characteristics of patients presenting with congenital orbital cysts containing epithelia of both cutaneous phenotype-that is, epidermis with or without keratin, and at least one dermal structure (sweat gland or pilosebaceous unit)-and conjunctival phenotype (with goblet cells); these cysts with mixed lining are termed "dermo-conjunctival" cysts. PATIENTS AND METHODS: Review of clinical records for patients having dermo-conjunctival cysts that were treated between 1997 and 2017; patients with cysts of solely conjunctival or solely cutaneous origin were omitted...
March 31, 2021: Ophthalmic Plastic and Reconstructive Surgery
https://read.qxmd.com/read/33778431/intranasal-glial-heterotopia-in-an-infant-boy
#8
Wei Zhang, Lixing Tang, Wentong Ge
Introduction: Nasal glial heterotopia is a rare congenital developmental disorder characterized by meningeal epithelium and/or glial components. Case presentation: A 2-month-old boy presented for treatment of a congenital mass in the right nasal cavity near the pharynx. The preoperative diagnosis was congenital intranasal neoplasm. Nasal endoscopic resection of the nasopharyngeal mass was performed under general anesthesia. Histological findings in the resected tissue supported a diagnosis of intranasal glial heterotopia...
March 2021: Pediatric Investigation
https://read.qxmd.com/read/33706014/congenital-bony-nasal-cavity-stenosis-a-review-of-current-trends-in-diagnosis-and-treatment
#9
REVIEW
Francesca Galluzzi, Werner Garavello, Gianluca Dalfino, Paolo Castelnuovo, Mario Turri-Zanoni
Congenital bony nasal cavity stenosis is caused by alterations of the normal embryological development of the nasal cavity. Depending on the site of the obstruction, the most important types of stenosis are: choanal atresia and stenosis, congenital nasal pyriform aperture stenosis, congenital midnasal stenosis, arhinia and nasal septum deviation. Although they are uncommon, they could be potentially life-threatening conditions that require early diagnosis and proper treatment. In case of neonatal nasal obstruction, appropriate differential diagnosis with other causes, such as rhinitis and sinonasal masses, are performed by nasal endoscopy and radiological exams...
May 2021: International Journal of Pediatric Otorhinolaryngology
https://read.qxmd.com/read/33670814/congenital-suborbital-undifferentiated-sarcoma-in-a-crossbred-calf
#10
Joana G P Jacinto, Marilena Bolcato, Arcangelo Gentile, Cinzia Benazzi, Luisa Vera Muscatello
Undifferentiated sarcomas are rare conditions that represent a group of unclassified sarcomas. The purpose of this study is to describe the clinical and pathological features of a calf showing a congenital infiltrating suborbital mass suggestive of undifferentiated sarcoma. The animal was referred because of respiratory distress and the presence of a right suborbital mass since birth. At ultrasonography, the mass displayed an irregular shape with multiple cavities. Radiographs revealed a diffuse, poorly defined mass with different densities overlying the bony structures of the skull...
February 18, 2021: Animals: An Open Access Journal From MDPI
https://read.qxmd.com/read/33541005/-progress-of-the-diagnosis-and-treatment-of-congenital-nasal-dermoid-sinus-cysts
#11
REVIEW
Yuqin Zheng, Bin Ye, Jingrong Lv, Mingliang Xiang
<b/> Congenital midline nasal masses are rare. Nasal dermoid sinus cysts(NDSC) are the most common type of the congenital midline nasal masses in childhood. Clinical manifestations are midline nasal cysts, fistula and intracranial attachments. Nasal encephalocele and glioma should be included in the differential diagnosis. Radiologic images are instructive. NDSC are easily misdiagnosed, leading to recurrence and surgical trauma affecting the face. Early appropriate surgical excision is recommended...
February 2021: Journal of Clinical Otorhinolaryngology, Head, and Neck Surgery
https://read.qxmd.com/read/33412306/fetal-diaphragmatic-eventration-a-case-report
#12
B Sallout, D Alshebli, L Sallout, B Al Baqawi, M S Faden
BACKGROUND: Congenital diaphragmatic eventration (CDE) is a rare congenital malformation that is well described in the pediatric literature. In contrast to congenital diaphragmatic hernia (CDH), there is no physical defect in the diaphragm with CDE. Prenatal differentiation of the two pathologies represents a diagnostic and prognostic challenge. CASE: A 26-year-old nulliparous woman was evaluated for a fetal thoracic mass. At 22 weeks, detailed morphology ultrasound revealed a multi-cystic structure in the left side of the thorax...
January 4, 2021: Journal of Obstetrics and Gynaecology Canada: JOGC
https://read.qxmd.com/read/33388986/neonatal-nasal-obstruction
#13
REVIEW
Andrés Alvo, Galia Villarroel, Cecilia Sedano
PURPOSE: Congenital nasal obstruction can be a significant cause of respiratory distress in the newborn, given that they are considered to be obligate nasal breathers. Several different causes have been described, which can be broadly classified as anatomical/malformative, non-tumoral masses and cysts, benign and malignant neoplasia, inflammatory/infectious, traumatic/iatrogenic, and miscellaneous. The purpose of this review is to provide updated and useful clinical information for teams involved in neonatal care, especially in a hospital setting...
January 3, 2021: European Archives of Oto-rhino-laryngology
https://read.qxmd.com/read/33209002/a-rare-case-of-nasal-glial-heterotopia-in-an-infant
#14
Karishma Tahlan, Nadeem Tanveer, Harresh Kumar, Himanshi Diwan
Glial heterotopias are the displacement of neuroglial tissue in extracranial sites. Nasal glial heterotopias can be of three types-extranasal, intranasal and mixed. Root of the nose is the most common location. These are rare anomalies with an incidence of 1 case in 20,000-40,000 live births. Here we report the case of a 6-month-old infant with a congenital mass located at the root of the nose. Non-contrast computed tomography studies showed no evidence of intracranial communication of the lesion. The mass was excised, and on histopathological examination, it showed glial tissue with astrocytes in a fibrillary background and fibroconnective tissue...
July 2020: Journal of Cutaneous and Aesthetic Surgery
https://read.qxmd.com/read/32916632/nasal-meningoencephalocele-a-retrospective-study-of-clinicopathological-features-and-diagnosis-of-16-patients
#15
Li Xue, Dong Gehong, Wu Ying, Tao Jianhua, Zhang Hong, Liu Honggang
OBJECTIVE: Nasal meningoencephalocele (encephalocele or cephalocele) is a rare condition with congenital, traumatic, or spontaneous origins. We investigated the clinicopathological characteristics of nasal encephaloceles to improve pathologists' and clinicians' understanding of this disease. METHODS: Sixteen patients with nasal encephaloceles were enrolled in this retrospective study investigating the condition's clinical and morphological features. RESULTS: Patients' average age was 37...
August 13, 2020: Annals of Diagnostic Pathology
https://read.qxmd.com/read/32861456/congenital-pyriform-aperture-stenosis-ten-years-experience
#16
Cinthia Giselle Pérez, Paula Gabaldon Masse, Alejandro Cocciaglia, Hugo Rodríguez
INTRODUCTION: Congenital nasal pyriform aperture stenosis (CNPAS) is an uncommon malformation and a rare cause of respiratory distress in neonates that may require early surgical intervention. The aim of this study is to report our experience in patients operated for CNPAS and present characteristics of this population. MATERIAL AND METHODS: A retrospective, analytical cohort study was conducted of patients who underwent surgical treatment for CNPAS in the Hospital de Pediatría Garrahan from May 2008 to May 2018...
August 27, 2020: Acta Otorrinolaringológica Española
https://read.qxmd.com/read/32702883/intranasal-glial-heterotopia-in-a-male-infant-a-case-report
#17
Wei Zhang, Lixing Tang, Pengpeng Wang, Wentong Ge, Xin Ni
RATIONALE: Nasal glial heterotopia is a rare type of neoplasm consisting of meningothelial and/or neuroglial elements. PATIENT CONCERNS: A 2-month-old male was evaluated for treatment of a congenital mass in the right nasal cavity near the pharynx. DIAGNOSES: The patient was preoperatively diagnosed with a congenital intranasal neoplasm. INTERVENTIONS: Nasal endoscopic resection of the nasopharyngeal mass was performed under general anesthesia...
July 17, 2020: Medicine (Baltimore)
https://read.qxmd.com/read/32481248/diagnosis-and-treatment-of-congenital-nasal-dermoid-and-sinus-cysts-in-11-infants-a-consort-compliant-study
#18
Kun Ni, Xiaoyan Li, Limin Zhao, Jiali Wu, Xiaojun Liu, Haibo Shi
There have been few studies on congenital nasal dermoid and sinus cysts (NDSCs) in infants.This study was performed to obtain clinical data for the diagnosis and treatment of NDSCs in infants.We performed a retrospective analysis of 11 infants admitted with NDSCs between 2014 and 2019. Patient demographics, lesion site, preoperative radiological findings, surgical technique, intraoperative findings, and postoperative sequelae were analyzed.In total, 11 infants (average age, 19 months; lowest age, 10 months) were included in this study...
May 22, 2020: Medicine (Baltimore)
https://read.qxmd.com/read/32268691/-excision-for-congenital-nasal-dermoid-and-sinus-cyst-in-children
#19
X J Yang, J Zhang, L X Tang, P P Wang, J H Sun, Y N Wang, W T Ge
Objective: To explore the surgical effect and experience of endoscope-assisted excision for congenital nasal dermoid and sinus cyst (NDSC) in children. Methods: Fifty-three patients with congenital NDSC treated in Beijing Children's Hospital from January 2007 to December 2018 were retrospectively reviewed, including 30 boys and 23 girls, with the age ranging from 9 to 145 months (mean age 35.6 months). The ultra-low-dose CT scan and MRI of the paranasal sinuses were performed for all patients. Excisions of NDSC under general anesthesia were performed for all patients, and surgical approaches were dependent on location and extent of the lesions according to radiographic workups...
March 7, 2020: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://read.qxmd.com/read/32003094/clinical-characteristics-and-genetic-analysis-of-cystic-fibrosis-transmembrane-conductance-reseptor-related-disease
#20
Ayse Ayzit Kilinc, Gulizar Alishbayli, Hasan Emir Taner, Fugen Cullu Cokugras, Haluk Cokugras
BACKGROUND: Cystic fibrosis transmembrane conductance receptor (CFTR)-related disease is diagnosed in patients affected by CFTR dysfunction but who do not fully meet the CF diagnostic criteria. Only 2% of all CF patients have CFTR-related disease. We define the demographic characteristics of such patients, perform mutational analyses, and describe the clinical findings. METHODS: Twenty-four patients were followed-up in terms of CFTR-related disease. Patients with CF symptoms but who did not completely fulfil the CF diagnostic criteria were enrolled...
January 30, 2020: Pediatrics International: Official Journal of the Japan Pediatric Society
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