[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.

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.

[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.

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.

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.

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.

Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

Median philtrum sinus: a rare presentation and review of management options.

Nasal dermoids are rare congenital abnormalities, and there is only one previously reported case of a philtrum sinus tract extending to the skull base. A 2-month-old boy was presented with an incidental finding of a median philtrum sinus with no infective features and no other abnormal clinical findings. MRI demonstrated a sinus tract extending from the philtrum to the crista galli. On multidisciplinary review, a conservative approach was taken due to the asymptomatic nature of the patient. Given the rarity of nasal dermoids, diagnosis requires precise clinical examination and MRI to identify the extent of the tract. Management options require a multidisciplinary case-specific approach and include non-surgical as well as surgical approaches.

Sparing a Craniotomy: The Role of Intraoperative Methylene Blue in Management of Midline Dermoid Cysts.

BACKGROUND: Midline nasal dermoid cysts are rare congenital anomalies that extend intracranially in approximately 10 percent of cases. Cysts with intracranial extension require a craniotomy to avoid long-term complications, including meningitis, abscesses, and cavernous sinus thrombosis. Current guidelines recommend preoperative imaging with either magnetic resonance imaging or computed tomography to determine appropriate management. METHODS: Patients who underwent excision of a midline nasal dermoid cyst between January 1995 and September 2016 were identified using Current Procedural Terminology codes. In cases with equivocal imaging findings or uncertain stalk extent during surgical dissection, methylene blue was used intraoperatively. Demographics, preoperative imaging findings, intraoperative dye findings, surgical approach, and complications were collected. RESULTS: A total of 66 midline dermoid cyst excisions were identified; 17 (25.8 percent) had intracranial extension requiring craniotomy. Preoperative imaging showed a subcutaneous cyst in 41 (62.1 percent), intraosseous tracking in three (4.5 percent), and intracranial extension in 15 (22.7 percent). Twelve patients (18.2 percent) had preoperative imaging that was inconsistent with intraoperative findings. Methylene blue was used in 17 cases and indigo carmine was used in one case. Intraoperative dye findings changed management in five cases, and in three cases a craniotomy was avoided without evidence of cyst recurrence. CONCLUSIONS: This report is the largest published series of midline dermoid cysts with intracranial extension. In almost 20 percent of cases, preoperative imaging was not consistent with intraoperative findings. Given disparate radiographic and intraoperative findings, methylene blue is a valuable tool that can facilitate appropriate, morbidity-sparing management of midline dermoid cysts. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Modern Management of Nasal Hemangiomas.

IMPORTANCE: Current treatment approaches for infantile hemangiomas of the nose include observation, pharmacologic agents, surgery, and/or laser therapy. Because of the known functional, social, and cosmetic effect of nasal deformities, obtaining the best possible result is critical. Optimal timing, type, duration, and extent of therapy remain unclear. OBSERVATIONS: Results of a review of 86 patients (64 females and 22 males; mean age, 4.8 months [range, 2 days-23 years]) with infantile hemangiomas of the nose treated from January 1, 1999, to December 31, 2015, and a review of the literature are presented to gain insight into the preferred approach to the treatment of these lesions. Patients underwent single-modality and multimodality treatment with pulsed-dye laser (n = 73), oral corticosteroids (n = 11), intralesional corticosteroids (n = 2), propranolol hydrochloride (n = 30), and surgery (n = 50). The treatment decision algorithms and outcomes based on tumor phase and infantile hemangioma subtype are reviewed in detail. Nine articles met the criteria to be included in the literature review. Literature from the era before the approval of propranolol advocates for early use of oral or intralesional corticosteroids followed by surgery or pulsed-dye laser in cases of unacceptable outcomes. Literature from the era after the approval of propranolol supports early initiation of oral ß-blockers until proliferation ceases or until additional intervention is necessary. CONCLUSIONS AND RELEVANCE: Despite a lack of higher levels of evidence, there exists a general consensus between the literature and clinical experience advocating for early multimodality treatment to achieve the best result possible by the time the children reach certain sociodevelopmental milestones.

Nasal glial heterotopia or congenital hemangioma? A case report.

Nasal glial heterotopia (NGH) is a rare benign tumor of the median line. We describe the case of a child presenting a lateral nasal mass. The characteristics of the prenatal ultrasound and the postnatal clinical examination argued in favor of a congenital hemangioma (CH). The MRI performed at 6 weeks of life suggested glial heterotopia. This diagnosis was confirmed by the pathological analysis. Congenital hemangiomas and nasal glial heterotopies have similar clinical presentations. Prenatal ultrasound diagnosis between NGH and CH is difficult. Fetal MRI is not yet highly specific for these two lesions, but it can eliminate an intracerebral connection in cases of NGH. Postnatal exams are more specific. Flow on the Doppler exam is rapid for CH and slow for NGH. On MRI, these two lesions appear as a hypersignal on T2-weighted sequences, but less intense for NGH than for CH. Distinguishing between NGH and CH can be difficult. This does not have a direct incidence on treatment because it is surgical in both cases.

Cleft palate caused by congenital teratoma.

A cleft palate results from incomplete fusion of the lateral palatine processes, the median nasal septum and the median palatine process. This case report describes a rare case of congenital teratoma originating from the nasal septum that may have interfered with the fusion of the palatal shelves during embryonic development, resulting in a cleft palate. An infant girl was born at 40 weeks of gestation weighing 3020 g with a complete cleft palate associated with a large central nasopharyngeal tumour. Computed tomography (CT) of the head showed a well defined mass of mixed density. The tumour was attached to the nasal septum in direct contact with the cleft palate. A biopsy confirmed the teratoma. Tumour resection was performed at 5 months, soft palate reconstruction at 7 months and hard palate closure at 14 months. There was no sign of local recurrence 1 year later. Most teratomas are benign and the prognosis is usually good. However, recurrence is not rare if germ cell carcinomatous foci are present within the teratoma. For these reasons, we advocate the use of a two-stage procedure in which closure of the cleft palate is postponed until histological examination confirms complete excision of the teratoma.

[Congenital median dermoid fistula of nasal dorsum: one case report].

Congenital median dermoid is an uncommon disease. Surgery is the main curative treatment. To review a clinical case and to summarise the characteristics and treatment experience of this disease, referring to the related literature, it is expected that we can provide more clinical thought and therapeutic method for congenital median dermoid fistula of nasal dorsum.

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.

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.

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.

[Congenital nonpulsatile midline nasal masses].

These tumours are rare, benign abnormalities including dermoids, gliomas and encephaloceles that result from aberrant embryologic development. They can cause severe deformity of the midface and nasal structures and may have an intracranial extension that requires neurosurgical consultation. Thus preoperative manipulations, i.e. biopsies, are contraindicated as it can lead to cerebrospinal fluid leak and meningitis. The treatment is surgical excision and should be performed early. Neuroimaging is essential in the evaluation of specific type, presence of intracranial extension and presurgical planning.

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.