Congenital Intraoral Dermoid and Epidermoid Cyst with Orocutaneous Fistula Presenting with Life-threatening Airway Obstruction in an Infant.

Dermoid cysts and epidermoid cysts in the floor of the mouth are rare in the pediatric age group. In this case report, we have discussed the presentation, management, and literature review of a 6-month-old female child presenting with both dermoid and epidermoid cysts in the floor of the mouth with an orocutaneous fistula.

[Removal of a dermoid cyst of the floor of the mouth in a patient with severe hemophilia A]. / Udalenie dermoidnoi kisty dna rta u bol'nogo tyazheloi formoi gemofilii A.

The article presents a case of a surgical treatment of removing a dermoid cyst of the floor of the oral cavity in a patient with severe hemophilia A. A detailed analysis was carried out of the surgical operation, postoperative management, coagulation factor replacement therapy and accompanying therapy, as well as the features of anesthesia, which allowed a surgical intervention without any hemorrhagic and infectious complications.

[Our experience in the treatment of congenital nasal median heterotopias in children and an overview of various treatment tactics]. / Nash opyt lecheniya vrozhdennykh nazal'nykh sredinnykh geterotopii u detei i obzor taktik lecheniya.

Dermoid nasal cysts (congenital nasal median heterotopias) are a rare congenital pathology in children. OBJECTIVE: Yo consider the clinical picture, methods of radiation diagnosis and to study the surgical results of a dermoid cyst of the nose according to the literature. MATERIALS AND METHODS: A retrospective review of medical histories with the diagnosis of "Dermoid cyst of the back of the nose and nasal cavity, epidermal cyst of the back of the nose, glioma, encephalocele" was conducted from 2017 to 2022 in the Pediatric Otorhinolaryngological Department of the National Medical Research Center for Otorhinolaryngology of the Federal Medical-Biological Agency of Russia. The case histories were analyzed by the nature of the lesion, the imaging techniques performed, the course of the operation and the results obtained. MATERIAL AND METHODS: A total of 16 medical histories were analyzed, the average age was 4.5 years (range 10 months - 15 years), over the past 5 years with a diagnosis of "Dermoid cyst of the nasal dorsum and nasal cavity, glioma, epidermal cyst of the nasal dorsum, encephalocele". All patients underwent magnetic resonance imaging (MRI) in the preoperative period, 14 patients also underwent computed tomography (CT). RESULT: Of these, 7 had a confirmed dermoid cyst with a fistula, 3 patients without a fistula, 3 patients had glioma, and 1 had encephalocele, 2 patients had an epidermoid cyst. A fistulous opening of the dermoid cyst of the nasal dorsum and nasal cavity was observed in the upper third of the nasal dorsum in 3 children, in the middle third in 2 patients and in the lower third in 2 children. The article presents a scheme for the characteristics of the lesion and the tactics of surgical treatment in comparison with the data of foreign authors. Intraoperatively, intracranial spread occurred in 6 patients. Various surgical approaches for intracranial proliferation and a corresponding literature review are also presented. Catamnestic follow-up ranged from 1 to 5 years (on average, 3.5 years), no relapses or postoperative complications were noted. CONCLUSION: Nasal median heterotopias are a rare congenital anomaly. Preoperative preparation should include CT and MRI to assess the lesion and exclude intracranial spread. The surgical approach depended directly on the localization of heterotopia and its spread. All patients had a good cosmetic result after the surgical treatment performed by us according to the author's method.

Midline cutaneous anomalies of the craniospinal axis.

Patients with midline cutaneous anomalies of the craniospinal axis can be indicative of underlying embryonic defects, such as neural tube defects. Lack of familiarity with these midline aberrant skin findings may lead to misdiagnosis and delayed treatment. In this review, midline cutaneous anomalies of the craniospinal axis including aplasia cutis congenita, cranial and spinal dysraphism, and other developmental anomalies are explored in detail with emphasis on cutaneous clues to the diagnosis and appropriate workup.

Ultrasonographic Spectrum of Cutaneous Cysts With Stratified Squamous Epithelium in Pediatric Dermatology: Pictorial Essay.

High-resolution ultrasound (HRUS) is an important diagnostic method in dermatology, especially in pediatric population. The most common type of cutaneous cysts in children corresponds to cysts with stratified squamous epithelium (CSSE). The objective is to present the different ultrasonographic appearance of histologically proven CSSE in a retrospective review. Epidermoid, milium, trichilemmal, dermoid, and pilonidal cyst and steatocystomas are included. Utility of HRUS in diagnosis of cutaneous lesions is well established. It is important to know-and stay updated-about the wide spectrum of ultrasonographic appearance of CSSE in order to avoid misleading diagnoses.

Dermoid cyst of the infratemporal fossa: a case report of surgical resection by infratemporal fossa type B approach.

OBJECTIVE: This is a case report of a dermoid cyst located in the infratemporal fossa and its surgical removal using infratemporal fossa type B approach. CASE REPORT: A 15-year-old male was referred from a local clinic after an incidental finding of a mass lesion in the skull base area on computed tomography (CT). Pre-operative magnetic resonance imaging showed a large cystic mass lesion, expanding to the foramen ovale with fat component in the right infratemporal fossa region. The lesion was completely excised using an infratemporal fossa type B approach. CONCLUSION: An extremely rare case of dermoid cysts of the infratemporal fossa was managed with infratemporal fossa type B approach without severe complication.

Management of a rare case of intraventricular ruptured dermoid cyst and chemical meningitis.

INTRODUCTION: Intraventricular dermoid cyst are very rare benign tumour. Due to benign nature, it may go un-noticed for years and might present with sudden rupture. Ruptured cyst can cause chemical meningitis, hydrocephalus, seizures etc. Due to lack of data, there are no defined guidelines about its management. We are reporting an interesting case of ruptured intraventricular dermoid cyst and chemical meningitis, who was managed successfully and will review the literature. CASE REPORT: A 48 years male patient was brought with complaint of sudden mental deterioration, irritability, headache, vomiting for 3 days. He had history of seizures for 1 year. On examination, his higher mental functions were deteriorated, had neck rigidity and left lower limb weakness. CT/MRI brain confirmed diagnosis of right frontal horn ruptured dermoid cyst with scattered fat droplets, hydrocephalus and ventriculitis. Excision of cyst was done via right trans-sulcal (superior frontal sulcus) approach. Patient recovered well. DISCUSSION: Intraventricular dermoid cyst are a rare benign lesion. Due to potential of rupture, it must be surgically treated. Steroids should be used to treat chemical meningitis. Various individual case reports have shown good outcome after surgical management. CONCLUSION: We recommend early surgery, thorough ventricular wash with ringer lactate, post-operative extraventricular drain and steroid cover to manage ruptured cyst and chemical meningitis.

Split cord malformation associated with congenital dermoid cyst and myeloschisis - case-based literature review on possible embryonic derivation and implications.

Split cord malformation co-existing with either congenital dermoid or myeloschisis has been previously reported. Theories exist which explain the underlying embryopathy behind the occurrence of each of these anomalies in conjunction with split cord malformation. However, the occurrence of all three anomalies in the same patient, to the best of our knowledge, has not yet been reported in the literature. We report two cases on the co-existence of congenital dermoid cyst with both myeloschisis and split cord malformation and review the literature on previous postulations of mechanisms that could possibly explain the simultaneous occurrence of all three distinct anomalies. They involve all three primary germ layers and therefore share a common embryonic origin from the epiblast. We try to explain their co-existence based on the theory of notochordal splitting and endomesechymal tract formation consequent on endodermal-ectodermal adhesion as postulated by Beardmore and Wigglesworth and discuss on implications in terms of treatment paradigms. The unusual combination of all three anomalies in both patients probably highlights a need for further research on their pathogenesis despite these previous theories. These cases demonstrate the fact that currently accepted embryological explanations of most pathologies especially outliers still fall short with much remaining to be understood.

Lawsonella clevelandensis causing spinal subdural empyema.

Lawsonella clevelandensis is a recently described species and genus of bacterium in the Corynebacterineae suborder which is Gram-stain positive, partially acid-fast and anaerobic. Very few cases of human infection due to this organism are described and here we seek to add to the limited medical literature. We report the case of a 2-year-old girl who presented with an infected spinal dermoid cyst secondary to Lawsonella clevelandensis which required surgical drainage and a long course of antibiotics. We encountered diagnostic and therapeutic difficulties because this is a fastidious organism which was difficult to culture and ultimately required molecular detection and identification. To the best of our knowledge, this is only the seventh reported case of Lawsonella clevelandensis causing human infection worldwide and the first in the UK. This is the first reported case of Lawsonella clevelandensis infection in a child and the second reported case of this organism causing spinal infection.

The evidence mismatch in pediatric surgical practice.

PURPOSE: Outpatient pediatric surgical practice often involves conditions of limited morbidity but significant parental concern. We explore existing evidence-based management recommendations and the mismatch with practice patterns for four common outpatient pediatric surgical conditions. METHODS: Using the Cochrane Rapid Review Group recommendations and librarian oversight, we conducted a rapid review of four outpatient surgical conditions: dermoid cysts, epigastric hernias, hydroceles, and umbilical hernias. We extracted patient demographics, intervention details, outcome measures and evaluated justifications presented for chosen management options. A metric of evidence volume (patient/publication ratio) was generated and compared between diagnoses. RESULTS: Out of 831 articles published since 1990, we identified 49 cohort studies (10-dermoid cyst, 6-epigastric hernia, 25-hydrocele, and 8-umbilical hernia). The 49 publications included 34,172 patients treated across 18 countries. The evidence volume for each outpatient condition demonstrates < 1 cohort/condition/year. The evidence mismatch rate varied between 33 and 75%; many existing recommendations are not evidence-based, sometimes conflicting and frequently misrepresentative of clinical practice. CONCLUSIONS: Published literature concerning common outpatient pediatric surgical conditions is sparse and demonstrates wide variations in practice. All individual practice choices were justified using either risk of complications or patient preference. Most early intervention practices were based on weak or outdated studies and "common wisdom" rather than genuine evidence. LEVEL OF EVIDENCE: III.

Imaging in gynecological disease (24): clinical and ultrasound characteristics of ovarian mature cystic teratomas.

OBJECTIVE: To describe the clinical and ultrasound features of ovarian mature cystic teratomas (MCTs). METHODS: This was a retrospective study. From the International Ovarian Tumor Analysis (IOTA) database, we identified patients with a histologically confirmed diagnosis of MCT who had undergone transvaginal ultrasound examination between 1999 and 2016 (IOTA phases 1, 2, 3 and 5) in one of five centers. Ultrasound was performed by an experienced examiner who used the standardized IOTA examination technique and terminology. In addition to extracting data from the IOTA database, available two-dimensional grayscale and color or power Doppler images were reviewed retrospectively to identify typical ultrasound features of MCT described previously and detect possible new features using pattern recognition. All images were reviewed by two independent examiners and further discussed with two ultrasound experts to reach consensus. RESULTS: Included in the study were 454 patients with histologically confirmed MCT. Median age was 33 (range, 8-90) years and 66 (14.5%) patients were postmenopausal. Most MCTs were described by the original ultrasound examiner as unilocular (262/454 (57.7%)) or multilocular (70/454 (15.4%)) cysts with mixed echogenicity of cystic fluid (368/454 (81.1%)), acoustic shadowing (328/454 (72.2%)) and no or little vascularization on color Doppler (color score 1, 240/454 (52.9%); color score 2, 123/454 (27.1%)). The median largest lesion diameter was 66 (range, 15-310) mm. A correct preoperative diagnosis of MCT was suggested by the original ultrasound examiner in 372/454 (81.9%) cases. On retrospective review of ultrasound images of 334 MCTs that had quality sufficient for assessment, 'dots and/or lines' and/or 'echogenic white ball' (typical features according to the literature) were present in 271/334 (81.1%) masses. We identified four new ultrasound features characteristic of MCT: 'cotton wool tufts', 'mushroom cap sign', 'completely hyperechogenic lesion' and 'starry sky sign'. At least one classical or novel ultrasound feature was present in 315/334 (94.3%) MCTs. Twenty-nine (8.7%) MCTs manifested vascularized solid tissue, of which seven exhibited no typical features. CONCLUSION: We provide a comprehensive overview of conventional and newly described ultrasound features of MCTs. Only a small proportion of MCTs did not manifest any of the typical features. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Epidemiology and clinical characteristics of congenital choristomas in the ocular adnexa of pediatric patients.

PURPOSE: To investigate the epidemiology and clinical characteristics of infants presenting with conjunctival, palpebral, or orbital congenital choristomas (dermolipomas, epidermoids, and dermoid cysts) and children undergoing surgery for congenital choristomas in the ocular adnexa. METHODS: We reviewed the medical files of congenital choristomas in children seen in The Capital Region of Denmark during a 5-year period (2014-2018). Children (< 18 years) were divided into two groups: those referred < 1 year of age (Group I) and those undergoing surgery to remove the lesion (Group II). Group I was used to calculate a population-based incidence of congenital choristomas by comparing our data to birth statistics from the Danish Medical Birth Registry. RESULTS: A total of 97 children were included, 43 in Group I and 70 in Group II (including 16 patients from Group I). The total incidence of congenital choristomas was 1 in 2537 live born children. Most lesions were palpebral choristomas (27/43, 63%) located in the superotemporal region (17/27, 63%), followed by the superonasal region (7/27, 26%). The main reasons for surgical removal of a congenital choristoma were growth (28/70, 40%) or cosmesis (25/70, 36%). CONCLUSION: The total incidence of congenital choristomas in the ocular adnexa of infants < 1 year of age, including both conjunctival and palpebral congenital choristomas, is about 1 in 2537 live born children in The Capital Region of Denmark. Hence, congenital choristomas are common masses found in the ocular adnexa.

Sebaceous adenoma occurring within an intracranial dermoid cyst.

Among intracranial cystic lesions, dermoid cysts and epidermoid cysts are relatively common benign tumors. In a small number of these tumors, it is known that squamous cell carcinomas arise in the lining epithelium of the cysts. Among tumors derived from the appendage, only one case of hidradenoma within a dermoid cyst and no cases of sebaceous tumor have been reported previously. In the present case, a protruding lesion was present in the cystic wall, and it was composed of two cell types: sebaceous cells (sebocytes) and basaloid/germinated cells, being characteristic of this tumor. It is essential to distinguish it from other sebaceous lesions such as hyperplasia, sebaceoma, sebaceous carcinoma, and basal cell carcinoma with sebaceous differentiation derived from the epidermis. The critical distinguishing points in making a differential diagnosis among these lesions are the ratio of the two cell types and the presence or absence of other components such as hair sacs, invasion or cellular atypia. Immunohistochemical examination revealed that the tumor cells were positive for the epithelial markers, such as cytokeratin (CK)14, p63, p40, high-molecular CK, and adipophilin; these findings are peculiar to sebaceous adenoma. Although there have been several similar case reports of sebaceous tumors associated with dermmoid cysts in the ovaries, most of the intracranial lesions were squamous cell carcinomas that developed within the cysts, and there has been no precedent showing an association with a sebaceous tumor. The present report describes the first case of sebaceous adenoma that occurred in an intracranial dermoid cyst.

Is pre-operative imaging of external angular dermoid cysts in children necessary?

AIM: To investigate the frequency and modality of pre-operative imaging in children with an external angular dermoid cyst and whether this influenced subsequent management. METHODS: A retrospective study was undertaken to assess the presentation, investigation, treatment and outcomes in children aged less than 16 years with an external angular dermoid cyst. All children who underwent external angular dermoid cyst excision between January 2008 and April 2021 at a regional children's hospital were analysed. RESULTS: Sixty-one patients (32 boys) were identified. Fifty-four were managed by paediatric surgeons and seven by ophthalmic surgeons. Pre-operative imaging was performed in 19 (31%) patients, including plain radiographs (3), ultrasound scan (14), computerised tomography scan (1), and magnetic resonance imaging scan (2). None of these investigations showed evidence of an intracranial or orbital extension of the cyst or changed management. All cysts were excised under day-case general anaesthesia. There was one minor postoperative complication and two recurrences. CONCLUSIONS: External angular dermoid cysts rarely communicate through the bone with the orbit or anterior cranial fossa. Routine pre-operative imaging is unnecessary unless there are clinical features such as an atypical location (e.g. temporal), eye signs (e.g. strabismus, proptosis and globe displacement) or a draining sinus.

Isolated lesions of the suprasternal notch in pediatric patients.

BACKGROUND: Palpable masses of the head and neck are a common indication for imaging in the pediatric population. Midline lesions of the infrahyoid neck, particularly lesions isolated to the suprasternal notch, are not well studied. OBJECTIVE: To delineate the histopathological and imaging spectrum of masses that occur within and isolated to the suprasternal notch. MATERIALS AND METHODS: A retrospective study was performed to identify patients with an isolated lesion of the suprasternal notch that had available pathological diagnoses. Available imaging was reviewed and characterized by fellowship-trained pediatric radiologists and compared by descriptive statistics to the final pathological diagnoses. RESULTS: Eighteen masses isolated to the suprasternal notch with available pathological diagnoses were identified. Of these, congenital epithelial inclusion cysts were diagnosed in 14 patients (77.8%) with dermoid cysts comprising 11 of those (61.1%) and epidermoid cysts accounting for 3 (16.7%). The most common imaging appearance was a cystic or pseudosolid appearance without vascularity. CONCLUSIONS: Isolated resected lesions of the suprasternal notch in pediatric patients are most frequently dermoid/epidermoid cysts, with a differential diagnosis including other less common entities.

A Pediatric Case of Cauda Equina Dermoid Cyst Resected by Minimally Invasive Unilateral Hemilaminectomy.

A 3-year-old boy had difficulty sitting up and walking for several months. Magnetic resonance imaging (MRI) revealed an intradural tumor at the L3-4 level. The tumor was successfully resected by unilateral hemilaminectomy and diagnosed as dermoid cyst. The patient had an uneventful postoperative course without pain, and MRI found no recurrence after surgery. A small bone defect remained that might be favorably reconstructed with autologous and artificial bone. Hemilaminectomy allowed us to resect the cauda equina dermoid cyst with minimal invasiveness. Pediatric patients require follow-up as they are more likely to experience spinal deformity or instability after surgery.

Orbital and periorbital dermoid cysts: a retrospective analysis of 270 lesions.

PURPOSE: To report the clinical characteristics and intraoperative findings of periorbital and orbital dermoid cysts and their relationship to location and rupture. METHODS: Retrospective review of 270 cases with orbital or periorbital dermoid cysts that presented over a period of 11 years. Patients were included if diagnosis of dermoid cyst was made by histopathologic analysis. Clinical characteristics and operative outcomes were recorded and analyzed with Chi-squared analyses or univariate regression. Multivariate binary logistic regression was performed to assess predictors of location and rupture. RESULTS: Dermoids frequently occurred unilaterally and were more frequently described as mobile (61.5%), followed by fixed (30.8%), and partially fixed (7.7%). Dermoid rigidity was most commonly described as firm (75.5%), followed by cystic, soft, and rubbery. The most common dermoid location was superotemporal (60.4%). On Chi-squared analysis, superotemporal lesions were less likely to have orbital extension than non-superotemporal lesions (OR 0.28, 95% CI: 0.11-0.70, p = .01), less likely to undergo CT (OR 0.16, 95% CI: 0.06-0.41, p < .01) or MRI (OR 0.23, 95% CI: 0.13-0.41, p < .01), more likely to be described as mobile (OR 2.91, 95% CI: 1.32-6.43, p = .01), and less likely to rupture intraoperatively (OR 0.28, 95% CI: 0.11-0.73, p = .01). No variables were associated with rupture in multivariate analysis. CONCLUSION: Superotemporal dermoid cysts are common in the pediatric population, less likely to have orbital extension, undergo imaging, and have intraoperative rupture when compared to other locations in the orbit.

Rare Location of a Dermoid Cyst in the Parotid Gland: A Case Report.

Dermoid cyst of the parotid gland is a lesion composed of benign tissues of ectodermal and mesodermal origin. Although a dermoid cyst can be encountered across nearly all sites of the body, its location in the head and neck area is quite uncommon and even more unusual inside the parotid gland. We present a case of a patient with gradually enlarging tumour in her right parotid gland who underwent surgical removal of the tumour histologically corresponding to a dermoid cyst.

[Congenital dermoid cyst of the middle ear with invasion into the external auditory canal in a 1 year 10 month old child]. / Vrozhdennaya dermoidnaya kista srednego ukha s prorastaniem v naruzhnyi slukhovoi prokhod u rebenka 1 goda 10 mesyatsev.

The article presents a casuistic clinical observation of the treatment of a child aged 1 year 10 months with a dermoid cyst of the middle ear. The diagnosis was established on the basis of computed tomography and histological examination of the mass. Surgical treatment was performed in the volume of a separate attic-anthromastoidotomy with removal of the formation and one-stage type III tympanoplasty. In the catamnesis, the child's condition is satisfactory, the otoscopic picture shows no signs of inflammation.

Ovarian Dermoid Cysts Associated with Paraneoplastic Syndrome N-methyl-D-aspartic Acid Receptor Antibodies Encephalitis.

STUDY OBJECTIVE: To describe the incidence of ovarian dermoid cysts associated with paraneoplastic encephalitis syndrome due to N-methyl-D-aspartic acid (NMDA) receptor antibodies among women undergoing surgical resection of dermoid cysts. DESIGN: Retrospective cohort study. SETTING: University-affiliated department of obstetrics and gynecology. PATIENTS: All patients with pathology-proven ovarian dermoid cysts who underwent surgical resection in our department between January 2008 and December 2019. Their demographic, clinical, and surgical characteristics are described, with emphasis on cases diagnosed with anti-NMDA receptor encephalitis. INTERVENTIONS: Ovarian dermoid cyst resection by cystectomy or salpingo-oophorectomy. MEASUREMENTS AND MAIN RESULTS: A total of 233 patients were operated on for ovarian dermoid cysts, comprising 2 cases diagnosed with anti-NMDA receptor encephalitis (0.85%). Among the women without NMDA receptor encephalitis, the mean age was 33.3 ± 14.9 years, 84.0% were of reproductive age, 5.2% were premenarchal, and 10.8% were menopausal. The mean diameter of the dermoid cyst in this group was 77.3 ± 33.3-mm. The 2 patients diagnosed with anti-NMDA receptor encephalitis were 21 years old and 42 years old. The diameters of their dermoid cysts were 15-mm and 80-mm, respectively. The patients with anti-NMDA receptor encephalitis were managed with laparoscopic resection, plasmapheresis, intravenous immunoglobulins, and corticosteroids; 1 patient also received immunosuppressive treatment. Both recovered without significant neurologic sequela. CONCLUSION: Paraneoplastic syndrome due to NMDA receptor antibodies is a rare complication of dermoid cysts. This complication may occur in younger or older women, as well as in small- or large-diameter cysts. Thus, a high index of suspicion is required to correctly diagnose and treat women presenting with neurologic symptoms in the presence of dermoid cysts.