[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.

Dermoid Cyst of Nasal Tip and Nasal Septum.

ABSTRACT: Dermoid cyst is a congenital and benign disease with most occur on the head and neck. It is rarely that occur on the nasal tip and nasal septum at same time and rarely repair of using nasal septum mucosa. The authors treated a child with dermoid cyst in the nasal tip and septum. Only the dermoid cyst at the tip of the nose caused the change of appearance. Dermoid cyst of nasal septum did not cause any clinical symptoms. She underwent excision of the dermoid cyst at the tip of the nose and endoscopic surgery for the dermoid cyst in the nasal septum and used the nasal septum mucosa for repair at the same time. After 6 months of recovery, the appearance of the nasal tip recovered well without obvious scar, the nasal septum area recovered well, and the local stoma was unobstructed without recurrence. The authors found that this kind of nasal septal cyst with no clinical symptoms can achieve good therapeutic effect through endoscopic surgery and repair of using nasal septum mucosa, with less damage, rapid recovery, and good prognosis.

Pediatric congenital buttock sinus tract:10-year experience in a single institution.

PURPOSE: To review our experience with pediatric congenital buttock sinus tract, and to conclude the clinical characteristics and management of the disease. METHODS: Twenty-two pediatric patients diagnosed with congenital buttock sinus tract were included. Medical records were reviewed, and the patients were followed up. Continuous variables were presented by median and range. Categorical variables were presented as frequencies and percentages. RESULTS: Among the 22 patients, there were 8 boys (36.4%) and 14 girls (63.6%). The median first onset age was 42 months, and the range was 5 months to 12 years old. Admission age was 69.5 months, with a range from 14 months to 12 years old. Overall prior treatment time was 11 months, ranging from 3 months to 11 years. Twenty-one patients had definite congenital dimples since birth, and later manifested with infection through the dimple. All patients came to the doctor with complaint of the infection. The number of invasive procedures ranged from 0 to 5, with an average of 2. Radiology could exactly display the morphology and show the termination as a retrorectal cyst. The surgical procedure was adopted trans-fistula tract, and the pathological results showed a dermoid cyst in 11 patients and an epidermoid cyst in 10 patients. During the follow-up period of 34.5 months (range, 2 months to 8 years), 19 patients were uneventful and 3 patients suffered recurrence. Two of them underwent a second operation and had no recurrence ever since. The third patient did not receive a second operation, and the refractory infection was still present. CONCLUSIONS: Pediatric congenital buttock sinus tract is rare and has a female predominance in the morbidity. Patients have a distinctive congenital dimple on the buttock with recurrent infection, and there usually exists a congenital sinus tract from the dimple to the retrorectal space. Total excision is the only method for the cure. The nature of the disease is a retrorectal developing dermoid cyst or epidermoid cyst.

Management of congenital midline nasofrontal dermoid cysts in two identical twins: Case report.

Midline dermoid cysts are uncommon lesions that can lead to severe complications when an intracranial extension exists. We report the cases of two twin sisters referred to surgery for removal of the masses and the intracranial extension. They represent an additional example of familial nasal dermoid cysts, providing further support for a genetic basis for the disorder.

Intraorbital Dermoid Cyst With Zygomaticofrontal Suture Erosion in an Adult.

BACKGROUND: Dermoid cysts are squamous epithelium-lined sacs that result from abnormal migration of ectodermal cells. They are typically superficial and present in children. This study reviews a rare patient with a congenital dermoid cyst occurring as a superolateral intraorbital and extraconal mas. METHOD: Clinical report and review of the literature. RESULTS: A 27-year-old male presented with progressive left hypoglobus, proptosis, and diplopia. On computed tomography, a 2.0 × 2.1 × 1.1 cm well circumscribed mass centered on, and extended into, the frontozygomatic suture with smooth remodeling of the frontal bone. Magnetic resonance imaging showed heterogeneous T1 and T2 hyperintensity with hypointensity on fat suppression. A dermoid cyst originating from the frontozygomatic suture was suspected. Excision was performed through a left orbitocranial approach with a lateral orbitotomy through an upper eyelid crease incision. The mass occurred in the superior-lateral portion of the left orbit and originated from the intraorbital surface of the zygomaticofrontal suture line with significant bony remodeling without osseous transgression. The mass was removed completely including the lateral portion of the zygomaticofrontal suture. Pathologic examination was consistent with a dermoid cyst. Postoperative examination revealed resolution of his presenting headaches and diplopia. CONCLUSION: Congenital dermoid cysts occurring within the craniofacial region occur most commonly in the lateral orbit involving the superficial surface of the frontozygomatic suture. This patient represents the rare occurrence of a dermoid cyst originating from the deep surface of the frontozygomatic suture, resulting in delayed presentation and orbital symptoms of hypoglobus and diplopia. Definitive treatment remains complete surgical excision.

Split cord malformation type 1 with two hemicord lesions.

Split cord malformations are rare entities which may present in an occult manner or in association with other lesions or congenital anomalies. Rarely, these cases may have associated hemicord lesions. We report an unusual case, the first of its kind, a type 1 split cord malformation with two pathologically different lesions (lipoma and dermoid) on one hemicord.

Unusual Clinical Presentation and Association of Cranial Dermoid Tumor and Tethered Cord Syndrome.

Tethered cord syndrome (TCS), a neurological disorder characterized by the lower settlement of the conus medullaris, is a congenital spinal disease which is caused by split cord syndrome, meningomyelocele, and spinal tumors. Cranial dermoid tumor (CDT) is a congenital benign tumor which is generally located on the midline of the cranium. Even though TCS is highly associated with spinal dermoid tumor, the relationship of CDT and TCS is unusual. We pre-sent a case with an unusual symptom of CDT, motion-dependent pain, and an uncommon togetherness with TCS.

Nasopharyngeal Dermoids and Cleft Palate.

Nasopharyngeal dermoids associated with cleft palate present as intraoral protruding masses. Only 5 cases of nasopharyngeal dermoids associated with cleft palate have been reported in the literature. We are reporting 4 such cases encountered by us in the last 10 years in our series of 900 cleft palate surgeries. Imaging studies were done to know the extension of dermoid and to look for any associated congenital intracranial anomalies. We observed that nasopharyngeal dermoids are usually nonmalignant and can be easily managed by complete local excision followed by palatal closure after 6 months.

Phenotypic characterization of epibulbar dermoids.

The purpose of this study was to characterize the phenotypic presentation, clinical course, and outcomes of epibulbar dermoids (EpDs) which are the most common congenital eye tumor in children. Sixty-eight dermoids were identified in 58 eyes of 48 patients of Seattle Children's Hospital between 1981 and 2014 via electronic medical record search. Patients were organized into: "EpD-Only" [patients without other congenital anomalies (n = 13)], "EpD-CFM" [patients with a craniofacial microsomia (CFM) diagnosis (n = 25)], and "EpD-Other" [patients with other congenital anomalies (n = 10)]. All EpD in the EpD-Only group were unilateral and singular, while the EpD-CFM group had six cases with multiple unilateral EpD and five cases with bilateral EpD. In the EpD-Only group, 69 % of EpD were left sided, whereas in the EpD-CFM group, there was no side predisposition. Among both groups, the majority of EpD were limbal or lipodermoids in the inferotemporal quadrant of the eye. Surgery was more common and at a younger age in the EpD-CFM group than the EpD-Only group (56 vs. 38 %, 5.2 vs. 7.0 years). Follow-up surgeries occurred only in the EpD-CFM group (21 %). EpDs were most commonly associated with preauricular tags, congenital heart defects, genitourinary, and nervous system anomalies. Whereas the location and type of EpDs did not significantly differ between the groups, the phenotype in the EpD-Only group appears to be less complex. This may indicate an important difference between EpDs in isolation and those within CFM. Additional studies will further characterize these phenotypes and outcomes.

Floor-of-mouth dermoid cysts: report of 3 variants and a suggested change in terminology.

PURPOSE: Dermoid cyst is a frequently used descriptive term, but its definition changes according to the clinician (eg, dermatologists, neurologists, gynecologists, oral and maxillofacial surgeons, and plastic surgeons). It is sometimes used synonymously with teratoma. In oral and maxillofacial surgery, it is applied to describe congenital floor-of-mouth cysts of 3 histologic types: epidermoid, dermoid, and teratoid. This terminology is confusing and has led to some ambiguity in the literature. The purpose of this report is to document 3 cases illustrating the utility of a more specific term, congenital germline fusion cyst of the floor of the mouth. PATIENTS AND METHODS: Patients who presented with floor-of-mouth swelling were evaluated by history, clinical examination, and imaging studies (magnetic resonance imaging and computed tomography). RESULTS: Three cases (1 epidermoid variant, 1 dermoid variant, and 1 teratoid variant) are documented to illustrate the new terminology. CONCLUSION: Congenital germline fusion cyst is more reflective of the embryologic origins of the lesion than dermoid cyst and is inclusive of all 3 histologic variants.

Presternal dermoid cyst mimicking lymphatic malformation: a case report and review of the literature.

We describe an 11-month-old boy with an unusually large presternal mass present since birth. The large size, fluctuant properties, transillumination, compressibility, and imaging of this lesion were characteristic of a lymphatic malformation. Although four treatments with sclerotherapy markedly reduced its size, it was not until definitive treatment with surgical excision and the final pathology report that we arrived at the ultimate diagnosis of dermoid cyst. Dermoid cysts, although appearing along embryologic lines of closure, are rarely presternal. They are usually small, thick walled, and filled with sebaceous or keratinous fluid, which typically allows for clinical diagnosis, and show characteristic features on magnetic resonance imaging (MRI) and ultrasound. However, this case illustrates that dermoid cysts can appear in somewhat atypical locations, and imaging is not always diagnostic, so dermoid cyst should remain a part of the differential diagnosis for any lesion presenting midsternally, regardless of the size and imaging characteristics.

A nasal dermal sinus cyst involving the nasal septum.

Nasal dermoid sinus cysts (NDSCs) are rare neoplasms in the median line of the nasofrontal area. Unlike other dermoid cysts, a NDSC can manifest as a cyst, sinus, or fistula, and may extend intracranially. They usually occur as a mass in the median nasofrontal area and may have a pit. As an extensive tract can appear in some cases, careful preoperative evaluation and complete excision are required. A 15-year-old boy presented with a fistula, present from birth, in the nasal soft triangle. This report describes a case of NDSC occurring in the nasal septum and a fistula opening in the soft triangle, which is very rare, without recurrence after 4 years.

Midline dermoid cyst resulting in frontal bone erosion.

A dermoid cyst is a thin-walled benign tumor formed by the entrapment of ectodermal tissue during embryologic development, resulting in the inclusion of epithelium and adnexal elements within the tumor. Dermoids are not unique to a single anatomic location but are often isolated to the skin and subcutaneous tissue. They may occur intracranially or intra-abdominally, oftentimes associated with the ovary. If presenting as a midline mass of the skull, preoperative imaging with computed tomography and/or magnetic resonance imaging is necessary to evaluate for possible intracranial extension, given the altered embryologic development behind the formation of these cysts. Differential diagnosis of a midline frontonasal mass includes epidermoid or dermoid cyst, encephalocele, glioma, and sinus pericranii. The management of suspected dermoid cysts includes complete surgical excision, which may require a combined intracranial and extracranial approach. We present a 2-year-old boy who presented to our institution with a congenital midline scalp mass separate from the anterior fontanelle with complete underlying bony erosion to the sagittal sinus demonstrated on preoperative imaging, who required early surgical excision and reconstruction of the bony defect.

Congenital cystic eye: from prenatal diagnosis to therapeutic management and surgical treatment.

Only 28 cases of congenital cystic eye have been reported in the literature. The main issue in such cases is differential diagnosis between this malformation and different cystic malformations and masses of the orbital cavity and eyeball, the most common of which is microphthalmia with cyst. Both malformations arise from incomplete closure of the fetal optic vesicle in different stages of embryonic development. We present a case of congenital cystic eye, associated with coloboma and corneal dermoid of the fellow eye and with left brachiocephaly, discussing differential diagnosis with microphthalmia with cyst and illustrating the treatment we planned and performed. The patient first underwent a surgical excision of the left corneal dermoid, then a resection of the right orbital cyst. The last step was to perform a craniotomy and cranial vault remodeling. All the operations were planned and performed using a team approach. The team comprised an ophthalmologist, a plastic surgeon, and a neurosurgeon, and the result was a successful outcome.

Concomitant Congenital Intraoral Dermoid Cyst and Heterotopic Gastrointestinal Cyst.

Sublingual dermoid cysts and oral heterotopic gastrointestinal cysts are well-documented causes of sublingual swellings in infants. However, the simultaneous occurrence of both cysts is an extremely rare phenomenon, with only two previous cases reported in literature. We present a case of 1-month-old neonate with a cystic ventral tongue mass that on histologic examination showed a dermoid cyst with co-existing heterotopic gastric-type epithelium in the wall. Additional histochemical stains revealed focal areas of colonic metaplasia within the heterotopic gastric epithelium. CDX2 immunohistochemical staining confirmed derivation from colonic epithelium.

Surgical correction of midline nasal dermoid sinus cysts.

Nasal dermoid sinus cysts (NDSCs) are rare congenital anomalies affecting approximately 1 in 30,000 live births. Nasal dermoid sinus cysts are unsightly, prone to infection, and, importantly, may communicate with the central nervous system. Treatment is complete surgical excision. This study retrospectively evaluated management of a large single-center cohort of intracranial NDSCs.Nineteen patients with NDSC were identified from all patients presenting to the Leeds craniofacial service between June 2000 and August 2008. Patient demographics, clinical presentation, preoperative investigations, and surgical procedures undertaken were analyzed.Mean age at presentation and surgery were 6.3 and 7.6 years, respectively. Fifty-three percent were males. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed in 15 and 17 patients, respectively. One patient (5.3%) required local excision only. Eighteen (96.7%) underwent a bicoronal approach, and 13 (68.4%) of these required a craniotomy. The dura was opened in 7 (36.8%) patients. Neither CT nor MRI predicted the presence or absence of intracranial extension in all patients. Positive and negative predictive values for intracranial extension were 85.7% and 50% for CT and were 100% and 50.0% for MRI. Mean follow-up of 4.1 years shows no deep recurrences and 5 (26.3%) were superficial nasal recurrences only.A multidisciplinary approach can achieve good results with infrequent intracranial recurrence. We used a bicoronal approach to facilitate craniotomy when required intraoperatively because imaging is unable to diagnose intracranial extension with sufficient accuracy.

Congenital midline nasal mass: cases series and review of the literature.

Encephalocele, glioma and dermoid cyst are the most common midline nasal masses. Given their potential for intracranial extension, prompt treatment is necessary to prevent complications. Herein, we present two cases of midline nasal masses. A comparison was made to delineate the differences between their clinical courses, treatments and outcomes. Case 1 was a baby girl with respiratory distress beginning at birth. Nasal glioma without definite intracranial extension was present. The mass was completely excised with the aid of a video-assisted endoscope without complications. At follow-up two years after surgery, no recurrence was noted. Case 2 was a two-year-old boy with a midline nasal dermoid cyst. Extirpation of the lesion through a vertical-dorsal approach was performed. He was discharged three days after surgery with a satisfactory aesthetic result.

Intralingual dermoid cyst in an infant presenting swallowing and sleeping difficulties.

Dermoid cysts of the oral cavity are extremely rare. The most common site is the floor of the mouth whereas intralingual location is the most unusual. They may be congenital or acquired and according to their histological appearance they are distinguished in "true" dermoid, epidermoid or teratoid cysts. We present the clinical and radiographic findings of a large congenital intralingual "true" dermoid cyst in a 10-month-old boy. The large size of the lesion and the subsequent enlargement of the tongue caused difficulties in swallowing and sleeping, symptoms which subsided after the surgical treatment. The uncommon location, the large size and the very young age of the patient were the noteworthy parameters.