Congenital pediatric nasal dermoid on the lateral part of the nasal root ? a series of two cases

Traitement de la stérilité et mélanome : question non résolue en 2016

The management of midline transcranial nasal dermoid sinus cysts

Introduction
 Congenital midline nasal masses include nasal dermoids, gliomas, encephaloceles. Although rare, these disorders are clinically important because of their potential for connection to the central nervous system. Preoperative knowledge of an intracranial connection is a necessity to allow for neurosurgical consultation and possible planning for craniotomy. This study discusses the clinical presentation of congenital midline nasal mass and the role of imaging modalities like CT scan and MRI in diagnosis and the surgical management.
 Materials and Methods
 This prospective study is carried from March 2014 to March 2016, during which 4 cases presented to the Otorhinolaryngology department. Pre-operative evaluation of the patients included endoscopic evaluation along with haematological investigations, CT Scan and MRI. The masses were removed with nasal endoscopic sinus surgery or by external approaches and neurosurgical intervention.
 Result
 The age of the patients ranged from 3 years to 25 years. Three of them were male and one female. There was one case of nasoethmoidal encephalocele and the other three were dermoids (intranasal dermoid cyst, nasal dermoid cyst and nasal dermoid sinus cyst).
 Conclusion
 Congenital midline nasal masses are rare. These disorders are clinically important because of their intracranial connection which require proper evaluation with radiological imaging like CT scan and/or MRI before FNAC and any surgical intervention.

Nasal dermoid sinus cysts are rare congenital lesions that result from aberrant embryonal development. Cases of nasal dermoids associated with intracranial extension have been reported in the past, but have almost always occurred in children. One case of a nasal dermoid cyst with intracranial involvement in a 56-year-old man is reported herein. A 56-year-old man presented with a draining sinus in the right medial canthal area and a nasal pit. Imaging studies suggested a nasal dermoid cyst with intracranial extension. He subsequently underwent a craniofacial removal of the lesion with a satisfactory postoperative course. That nasal dermoid cysts can remain dormant until the age of 56 suggests that the incidence of this lesion with intracranial extension may be higher than previously reported. A one-stage combined otolaryngologic/neurosurgical procedure is advocated for those lesions that are extra- and intracranial.

Nasal dermoid sinus cyst is a rare congenital midline anomaly. The embryological origin is different from the dermoid cysts seen in other parts of the face. Nasal dermoid sinus cysts constitute 1% to 3% of all dermoid cysts and 11% to 12% of head and neck dermoid cysts. While most lesions are detected in the first 3 years, in some cases they may not be noticed until later years. Delay in diagnosis increases recurrent infections, airway obstruction and intracranial complications. The authors present a 34-year-old patient with a broad nasal dermoid cyst who had previously undergone external rhinoplasty for a nasal dermoid cyst but relapsed after 5 months.

ObjectiveDermoid cyst (DC) is a congenital cyst with the potential to have intracranial extension (ICE). This study aims to evaluate the imaging yield and surgical outcomes of nasal, medial brow, forehead, and scalp DCs.DesignRetrospective review of craniofacial DCs treated at our institution between 1992 and 2024.ResultsA total of 117 patients (57 females) were included. The median age at cyst detection and removal were 4.8 months (IQR 3.6-9.6) and 1.8 years (IQR 0.9-5.3), respectively. In 42 patients, parents have noticed the presence of the cyst immediately after birth. Cyst wall rupture during surgical removal was reported in 15.4%. The median follow-up time was 1.3 months (IQR 0.5-12.2). Three patients experienced recurrence. No postoperative complication was reported. The regions with the highest prevalence of ICE were the forehead, frontotemporal scalp, and nasal region. The lateral frontal/temporal scalp had a 33.3% rate of ICE. Midline forehead/scalp lesions demonstrated a higher risk of ICE compared to their lateral equivalents (54.5% vs 17.5%, P = .03). The sensitivity and specificity of magnetic resonance imaging (MRI) were 100% and 95.7%, while for computed tomography (CT scans) were 72.7% and 96.5%. The Area Under the Curve for MRI was 0.978, and for CT was 0.846. The sensitivity and specificity of ultrasound were 50% and 100%.ConclusionsMidline forehead/scalp DCs are more prone to extend intracranially than lateral DCs. MRI had a higher sensitivity and specificity than CT scans in detecting ICE. Routine screening imaging should be considered in midline forehead/scalp, lateral frontal/temporal, and nasal DCs.

Background: Nasal dermoid cysts are common tumors in children. Due to anomalies in embryologic development of the nasal complex, sometimes an intracranial extension exists. When these cysts become infected they can lead to meningitis, brain abscess and death. Methods: We report the case of a 1.5-year-old girl admitted to the paediatric intensive care unit after infection of a nasal dermoid cyst. Results: The infant had a spiking fever and epileptic seizures. She was stabilized, intubated and a CT scan showed a subcutaneous mass with an adjacent zone of encephalitis and brain abscess formation. Neurosurgical interventions were necessary to lower intracranial pressure and control infectious spread. After a hospital stay of 69 days the child could be discharged. Due to her young age, irreversible brain damage is expected. Conclusion: Nasal midline dermoid cysts are considered benign swellings. When an intracranial extension exists, infection can lead to deleterious complications. It is important for health care practitioners to be aware of this imminent risk. Suspicion of a nasal midline dermoid cyst should prompt a careful clinical work-up with an ultrasound followed by CT or MRI imaging. The treatment is complete excision to avoid disastrous complications and recurrences.

A Dalmatian was presented with a subcutaneous swelling in the dorsal midline between the eyes. No opening in the skin was identified at the dorsal nasal planum and there was no discharge. Positive contrast sinography showed contrast material filling a cyst that extended to the frontal bone. At surgery, the cyst had a tubular shape and was embedded in a fibrous dermal tissue strand running into the bony nasal septum. The nasal dermoid sinus cyst was surgically removed by limited dorsal rhinotomy, followed by excision of the remaining strand from a bony recess in the lamina perpendicularis ethmoidalis. It is proposed that this nasal dermoid cyst that extends through the frontal bone with no sinus tract is classified as a type V subtype c.

The use of a subcutaneous fat hinge flap for reconstruction in an adult patient with a midline nasal dermoid sinus cyst

Endonasal endoscopic approach for intracranial nasal dermoid sinus cysts in children

Nasal tip dermoid cysts that are present in pediatric patients can be complicated by mass effect causing compression and distortion of underlying cartilaginous structures. The purpose of this report is to describe a single surgeon's technique and results of nasal tip dermoid cyst excision in pediatric patients through an open rhinoplasty approach followed by tip reconstruction and immediate fat grafting. A series of 3 pediatric patients presented with 1 to 1.5 cm nasal tip dermoid cysts that were causing distortion of the lower lateral cartilages. All 3 underwent excision of the cyst through an open rhinoplasty approach. The nasal tip was reconstructed with interdomal sutures and immediate fat grafting. Fat grafts were harvested from the abdominal wall and implanted in the nasal tip. The grafts filled the resulting dead space and were secured with the fibrin glue. The soft tissue was redraped, and the skin was closed. The final postoperative result, 2 years after surgical intervention, included a well-proportioned and appropriately shaped nasal tip without obvious incisional scars in all patients. The skin overlying the previously excised dermoid cyst was supple and healthy. Excision of nasal tip dermoid cysts in pediatric patients can be approached through an open rhinoplasty approach, tip reconstruction, and immediate fat grafting. This approach can provide assistance in correcting contour deformities and may help preserve an at-risk soft-tissue envelope.

Rationale:Nasal dermoid cysts are rare congenital lesions and may present as a midline nasal swelling. Nasal dermoid cysts usually present at birth and are commonly diagnosed by 3 years of age. An incidentally detected nasal dermoid cyst in an adult patient is extremely rare.Patient concern:We report an unusual case of an adult patient with an incidentally diagnosed dermoid cyst of the nasal septum, which presented as a longstanding discharging pit in the nasal columella since childhood.Diagnosis:A preoperative diagnosis of dermoid cyst of the nasal septum was made.Intervention:We performed the external rhinoplasty incision, including a small elliptical incision for removal of the columella pit.Outcome:There has been no evidence of recurrence during the 2-year follow-up.Lessons:An incidentally detected nasal dermoid cyst in an adult patient is extremely rare. The most important point for the preoperative diagnosis of nasal dermoid cysts is to identify the presence or absence of intracranial extension. The treatment of choice for nasal dermoid cysts is complete surgical excision with clear margins.

Operative options for extracranial nasal dermoid cysts: A meta-analysis

Management of midline nasal dermoid lesions in children by external rhinoplasty

Up to 10% of midline nasal dermoid cysts have intracranial extension. Previous techniques of excision include frontal and frontonasal craniotomies via a coronal approach, combined with a direct cutaneous excision of the dermoid cyst. While the coronal incision allows for wide visualization, it carries significant risks of transfusion, blood loss, and scarring. The authors present an alternative technique in which access is gained through a midline extension of the dermoid cyst excision that provides direct access for a keyhole frontal craniotomy. The authors utilize a nasal bone osteotomy, pericranial flap, and keyhole-type craniotomy performed through a nasal midline incision for the treatment of nasal dermoid cysts with intracranial extension. They performed a retrospective chart review of all patients with nasal dermoid cysts treated at the Ann & Robert H. Lurie Children's Hospital of Chicago from 2009 to 2017. Patient demographic data, operative data, and in- and outpatient complication data were collected. In 10 patients with cyst extension near or into the intracranial cavity (7 with true intracranial extension), the nasal osteotomy technique was performed. The mean blood loss was 13 ml, with a 0% transfusion rate. The mean length of inpatient stay was 1 day. A durotomy was made and repaired as part of the dermoid cyst dissection in 3 patients. One patient underwent intraoperative placement of a lumbar drain. The mean operative time was 228 minutes. There were no intraoperative or postoperative complications, including the need for a reoperation. No patients had any long-term complications, and no patients have had dermoid cyst recurrence. The appearance of the scar was acceptable in all cases. The midline approach to nasal dermoid cysts with intracranial extension is safe and results in limited blood loss, short operative times, and short lengths of inpatient hospital stay. This is a viable technique for the treatment of this challenging pathology.