[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.

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.

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.

Nasal glioma presenting with strabismus.

Congenital midline nasal masses are rare anomalies that occur in about 1 in 20,000 to 40,000 live births. Nasal gliomas are thought to be collections of heterotopic tissue of neurogenic origin, which have lost their intracranial connection. It rarely cause ocular distortion and deformity in the medial orbital wall. We describe here a case of a 12-month-old baby girl diagnosed as extranasal glioma at the lateral nasal wall and medial orbital wall presenting with strabismus and subsequently treated in our service and perform a literature review.

Congenital Melanocytic Nevus of the Nose Removed Using Dermabrasion, Hydroquinone, and Serial Excision.

We report a child with a congenital pigmented nevus of the nose involving the left ala, sidewall, soft triangle, and tip. Removal of the lesion was performed using dermabrasion, topical hydroquinone, and serial excision to optimize the aesthetic outcome. The patient was left with a linear scar and did not require reconstruction with a skin graft or flap.

Does nasal neuroglial heterotopia represent a risk for the newborn during delivery?

Neuroglial heterotopia is a rare developmental abnormality. Most frequently the diagnosis is established at birth or in early childhood by a typical clinical presentation. Neuroglial heterotopia can be intracranial or extracranial. A typical example of extracranial heterotopia is nasal glioma, which can be isolated or can communicate directly with the intracranium. The most sensitive investigation for the confirmation of its site is magnetic resonance imaging. Histological investigation is crucial in establishing the diagnosis. The authors present the case of postnatally assessed nasal glioma. They emphasize the importance of detailed prenatal investigation as most important in preventing birth trauma and consequent complications.

Huge nasopharyngeal teratoma with a cleft palate and bifid tongue in a patient with pierre robin syndrome.

PURPOSE: Nasopharyngeal teratoma is a very rare neoplasm that develops on the oronasal cavity as an expanding and cavity-filling lesion. We present a rare case of nasopharyngeal teratoma associated with a cleft palate and bifid tongue in a patient with Pierre Robin syndrome. METHODS: A preterm male neonate of 33 weeks and 6 days was referred to our department. A protruding solid mass with approximately 7.0 × 4.0-cm size arising from the vomer and combined cleft palate and tongue covered with pinkish skin with lanugo were observed. In addition, there was a bandlike tissue connecting vomer and sublingual area, which divided and restricted the anterior portion of the tongue. RESULTS: An early surgical intervention was decided because of problems with airways and nutrition caused by the mass. The mass was completely removed from the vomer. The histologic examination of the mass was consistent with mature teratoma. After his first operation, he was finally diagnosed with the Pierre Robin syndrome. After 4 months, a tongue reduction was conducted for macroglossia, and after 9 months, remnant teratoma excision and a palate repair were conducted using 2-flap palatoplasty. CONCLUSIONS: A nasopharyngeal teratoma is a rare case and induces a cleft palate and bifid tongue in a preterm infant, leading to severe airway and nutritional problems. A teratoma interferes with the fusion of embryonic tissues in the early developmental period, in particular, with the palatal fusion severely prevented if the teratoma is accompanied by a Pierre Robin syndrome such as in this case.

Surgical management of immature teratoma involving the oral cavity and orbit in a neonate.

Teratomas are congenital germ cell tumors composed of elements from 1 or more of the embryonic germ layers and contain tissues usually foreign to the anatomic site of origin. In the head and neck region, these lesions are rare, and 90% of head and neck teratomas present during the neonatal and infantile periods. Besides, in neonates, it carries serious risk for respiratory distress as well as feeding problems due to oral cavity and airway obstruction.Here, an unusual case of intraoral teratoma involving the orbit in a newborn who underwent immediate surgical excision successfully is described.

Melanoma arising in a child with a medium-sized congenital melanocytic nevus.

Pediatric melanomas are rare and some of them may arise on giant congenital melanocytic nevi. The risk of developing melanoma on a medium-sized nevus is not clear but is thought to be very rare. Proliferative cellular nodules which mimic malignant melanoma may pose significant diagnostic challenges. We report the case of a 9-year-old patient who developed a melanoma on a medium-sized congenital melanocytic nevus on the tip of the nose, requiring a complex surgery with excellent aesthetic results.

Los melanomas en pediatría son raros y algunos de ellos pueden surgir sobre nevos melanocíticos congénitos gigantes. El riesgo de desarrollar melanoma en un nevo de tamaño mediano no está claro, pero se cree que es muy raro. Los nódulos proliferativos celulares, que imitan al melanoma, pueden plantear importantes desafíos diagnósticos. Presentamos el caso de una paciente de 9 años que desarrolló un melanoma sobre un nevo melanocítico congénito de tamaño mediano en la punta de la nariz, que requirió un procedimiento quirúrgico complejo con excelentes resultados estéticos.

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.

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.

[Excision for congenital nasal dermoid and sinus cyst in children].

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. All intra-osseous patients and complicated superficial cases underwent surgical excision of NDSC and nasal reconstruction with the assistance of endoscope. Initial presentation, medical history, imaging workups, surgical approaches, complications, rates of recurrence and cosmetic outcomes were evaluated. Descriptive statistics was used for the results analysis. Results: Among 53 cases, the most common presentation included a nasal-glabella mass (n=21, 39.6%), a dorsal punctum (n=13, 24.5%) and a dorsal mass (n=9, 17.0%). The sites of NDSC included nasal glabella (n=22, 41.5%), nasal bridge (n=27, 50.9%) and nasal tip (n=4, 7.5%). Of all patients, 24 cases (45.3%) had superficial lesions, 19 cases (35.8%) had intraosseous extension into the frontonasal bones, 10 cases (18.9%) extended intracranially but remained extradural. Surgical approaches included transverse incision (n=22, 41.5%), minimal midline vertical incision (n=27, 50.9%) and external rhinoplasty (n=4, 7.5%). All NDSC were successfully excised and no nasal reconstruction needed. All cases were followed up from 9 to 151 months with a mean of 67.3 months. Five patients (9.4%) with recurrence were observed and were managed successfully with reoperation. During the follow-up, no nasal deformity was noted, and cosmetic outcome was favorable for all patients. Conclusion: Endoscope-assisted excision has the advantage of clear vision, small trama and low recurrence rate for children with NDSC.

Diagnosis and treatment of congenital nasal dermoid and sinus cysts in 11 infants: A consort compliant study.

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. All patients presented with a nasal root mass, 2 patients also had nasal tip fistula, and only 1 case had a history of preoperative infection. Preoperative enhanced computed tomography (CT) examination showed nasal surface lesion (type I) in 3, nasal intraosseous (type II) in 5, intracranial epidural (type III) in 2, and intracranial dural (type IV) in 1 patient. The main surgical methods included direct resection with a vertical midline incision (9 patients), vertical incision + transnasal endoscopic resection + skull base repair (1 patient), and transverse incision of the lower margin of the left eyebrow (1 patient). All wounds healed well without serious complications.Using the 4-type classification method in combination with the preoperative CT findings to analyze the extent of NDSC in infants is helpful for formulating the surgical plan. Using vertical incision approach alone or combined with nasal endoscopy for minimally invasive surgery can meet the needs of complete resection and reconstruction.Our results provide clinical data that can help establish standardized criteria for the diagnosis and treatment of NDSCs in infants.

Total forehead flap: hemicraniofacial giant congenital melanocytic nevus.

Being non-hair-bearing and relatively thin and having similar color and texture of the skin of the forehead provide an excellent characteristic not only for nasal reconstruction but also in other facial areas. A 28-year-old man presented in the Department of Plastic Surgery, Argerich Hospital, Buenos Aires, Argentina, with a giant congenital melanocytic nevus extended on complete left cheek, total nose, both upper and lower lids, ocular globe, and temporoparietal region. Tumor resection and reconstructive aspect were planned in 3 stages. (1) Excision of the tumor located in the cheek, nose, and both upper and lower left lids. The reconstruction of the cheek was made with a total forehead flap. The nose was resurfaced by means of a total skin graft. (2) resection of the temporoparietal nevus. (3) Treatment of the tumor involving the ocular structures. Total forehead flap was outlined, permitting carryout of skin of the total forehead area, and the blood supply was originated from the frontal and parietal branches of the temporal superficial artery and secondarily by anastomosis with the branches arising from both homolateral auricular and occipital vessels. Postoperative control 1 year after surgery showed an excellent aesthetic and functional result not only in the recipient area but also in the donor area.

Congenital and acquired lesions of the nasal septum: a practical guide for differential diagnosis.

Many different types of lesions may involve the nasal septum, and some can destroy it. Congenital nasal septal anomalies are rare and tend to have fairly typical imaging features, which, when considered alongside the imaging appearance of the normal anatomy, help determine the correct diagnosis in most instances. By contrast, many acquired lesions have nonspecific imaging features, and their diagnosis therefore must be based also on the patient's age and the histologic findings. Acquired nasal septal abnormalities may be caused by trauma, infection, toxicity, inflammation, or tumors. Traumatic lesions may result from surgery or from repetitive behaviors such as rhinotillexomania. Frequent use of decongestants and cocaine also may erode the nasal septum. Bacteria, mycobacteria, and fungi may cause infections of the nasal septum, particularly in immunocompromised patients. Inflammatory diseases that may affect the nasal septum include sarcoidosis, reparative granuloma, and Wegener granulomatosis. Last, the tumors that may arise in the nasal septum or may involve it secondarily include carcinomas, Pindborg tumor, sarcoma, angiofibroma, hemangioma, neuroendocrine tumor, and schwannoma.

Neuroglial heterotopia causing neonatal airway obstruction: presentation, management, and literature review.

Neuroglial heterotopias are rare congenital masses that are thought to represent encephaloceles that become sequestered on the extracranial side of the skull base. Although most often adjacent to bony skull base defects, they lack communication to the subarachnoid space. They contain mature neuroglial tissue and specialized central nervous system elements, such as a functioning choroid plexus. A case is presented of neonatal airway obstruction due to neuroglial heterotopia in the nasopharynx. The patient's clinical course and treatment are discussed, along with their radiology and histology. The relevant scientific literature is reviewed.

Choanal Atresia and Other Neonatal Nasal Anomalies.

Congenital nasal deformities can cause nasal obstruction with early respiratory distress. Choanal atresia is characterized by no communication between the nasal cavity and nasopharynx. Pyriform aperture stenosis involves more anterior nasal obstruction with limited intranasal space. Nasal masses such as encephaloceles, gliomas, and dermoids are thought to be related through a skull base defect in utero. Imaging with computed tomography and MRI are helpful in distinguishing lesions and identifying intracranial communication. Nasolacrimal duct cysts can get infected and cause obstruction. Nasal septal deviation can be corrected at bedside if detected early. Evaluation and treatment are discussed with all these entities.

A congenital teratoma with a cleft palate: report of a case.

Teratomas are benign tumors containing cells from ectodermal, mesodermal and endodermal layers. They occur in about 1 in every 4000 births and most commonly in the sacrococcygeal region, followed by the ovaries. Congenital epignathus teratomas are rare embryological neoplasms localised in the region of head and neck. An epignathus is found in approximately 1:35,000 to 1:200,000 live births. This accounts for 2-9% of all teratomas. Size and location of the neoplasm in the oronasopharynx is variable. Teratomas are partly undiagnosed at the time of birth. They may exist with an intracranial extension or as small polyps. Large epignathi can lead to difficult management during and after birth. The case of a newborn girl with a combination of an epignathus and a cleft palate is described. The epignathus presented as a huge mass extending out of the mouth of the infant girl. On the day of birth debulking of the extraoral portion of the tumor, followed by intraoral exstirpation, was performed. The results of the histologic examination indicated a congenital epignathus. Six months later a recurrence was found.