Beware frontotemporal dermoids - they may have intracranial extension: a case of a middle cranial fossa cyst

Congenital orbitofacial dermoid cysts are epithelial lined structures of ectodermal origin that may be associated with sinus tracts and variable deep extension. Midline lesions may extend intracranially, while lateral lesions are thought never to do so. Consequently only midline lesions are usually imaged prior to surgery. We describe a lateral dermoid sinus communicating with an intracerebral dermoid cyst within the middle cranial fossa in a 3 year-old with recurrent periorbital cellulitis and intermittent discharge from a small pimple in the temporal region. This case demonstrates that some lateral "dermoids" need to be treated with more caution if there are unusual features such as dimpling, discharge of material or recurrent wider infection, and preoperative imaging should be carried out to exclude intracranial extension.     

The management of midline transcranial nasal dermoid sinus cysts.

The most common congenital midline nasal masses are nasal dermoid sinus cysts (NDSC) [Hughes GB, Sharpino G, Hunt W, Tucker HM. Management of the congenital midline nasal mass--a review. Head Neck Surg 1980;2:222-33.]. Their clinical importance hinges on their potential to communicate with the central nervous system. Preoperative diagnosis of an intracranial extension allows for referral to a craniofacial team with the appropriate skills and experience for a transcranial approach. All patients with a NDSC require imaging with high resolution multiplanar MRI scans and complimentary fine cut CT scan to reveal the anatomical extent of the tract and its relationship to the anterior cranial fossa. A single-stage craniofacial approach to resection of midline NDSC extending to the anterior cranial base is effective with minimal morbidity [Yavuzer R, Bier U, Jackson IT. Be careful: it might be a nasal dermoid cyst. Plast Reconstr Surg 1999;103:2082-3; Denoyelle F, Ducroz V, Roger G, Garabedian EN. Nasal dermoid sinus cysts in children. Laryngoscope 1997;107:795-800; Rohrich RJ, Lowe JB, Schwartz MR. The role of open rhinoplasty in the management of nasal dermoid cysts. Plast Reconstr Surg 1999;104:2163-70; Rahbar R, Shah P, Mulliken JB, et al. The presentation and management of nasal dermoid-a 30-year experience. Arch Otolaryngol Head Neck Surg 2003;129:464-71; Posnick JC, Bortoluzzi P, Armstrong DC, Drake JM. Intracranial nasal dermoid sinus cysts: computed tomographic scan findings and surgical results. Plast Reconstr Surg 1994;93:745-54 [discussion 755-56]; Bartlett SP, Lin KY, Grossman R, Kratowitz J. The surgical management of orbitofacial dermoids in the pediatric patient. Plast Reconstr Surg 1993;91:1208-15.]. The cyst and tract are accessed through a combination of a nasal and transcranial approach. This allows visualisation and dissection of the tract with only a small incision on the nasal dorsum to include the cutaneous punctum when present. Transnasal endoscopic techniques have been advocated where the dermoid is located within the nasal cavity and there is little or no cutaneous involvement [Weiss DD, Robson CD, Mulliken JB. Transnasal endoscopic excision of midline nasal dermoid from the anterior cranial base. Plast Reconstr Surg 1998;101:2119-23.]. We present a review of five cases referred to our unit between 1999 and 2004 with a diagnosis of a midline nasal dermoid sinus cyst and radiological evidence of intracranial communication. All cases had a communication with the anterior cranial fossa diagnosed preoperatively and were treated surgically with a craniofacial approach. An intracranial extension was identified at operation in each case and this was confirmed on histopathology. The only significant complication resulted from an early postoperative infection, requiring re-operation. There were no recurrences and acceptable aesthetic outcomes have been observed in all cases.     

Intracranial extension of a nasal dermoid sinus cyst in A 56-year-old man

Background. 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. Methods. One case of a nasal dermoid cyst with intracranial involvement in a 56-year-old man is reported herein. Results. 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. Conclusion. 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. © 1994 John Wiley & Sons, Inc.     

Dermal sinus and dermoid cyst revealed by abscess formation in posterior fossa. Report of 2 pediatric cases and review of the litterature

Cranial dermal sinus, usually associated with dermoid cyst, is the persistance of an abnormal embryonal communication between the skin and the central nervous system. It may be the source of intracranial infection, most often a meningitis and rarely an abscess formation. Two cases of little girls (18 months and 2 years) having dermal sinus with dermoid cyst revealed by cerebellar abscess formations are reported. In the first case there were multiple cerebellar abscesses with hydrocephalus leading to a raised intracranial pressure. In the second case there was an abscess formation adjacent to the dermoid cyst. CT scan showed cysts and abscesses but MRI, achieved in the second case, was useful in demonstrating the sinus tract as well as the associated cyst and abscess. The two patients underwent a posterior fossa surgery with antibiotic therapy. In the first case abscess drainage and ventricular external drainage were necessary before sinus and cyst excision. Two months after surgery the two patients were neurologically intact and developping well. Surgery with total excision of dermal sinus and dermoid cyst, even sometimes difficult, must be preferred to the simple abscess drainage and antibiotic therapy.     

Pediatric dermoid cysts of the head and neck

OBJECTIVE: To review the characteristics and determine treatment outcomes of pediatric dermoid cysts. STUDY DESIGN AND SETTING: Retrospective review of the presentation, diagnosis, treatment, and outcomes of all pediatric dermoid cysts of the head and neck examined between 1980 and 2002 at Mayo Clinic. RESULTS: Forty-nine patients (59% girls) had a dermoid cyst of the head and neck. The median age at diagnosis was 22 months. The most common presenting sign was a palpable mass, noted in 100% of patients. During evaluation, approximately 25 patients (51%) had imaging studies. The most common location of the cysts was periorbital (61%), followed by the neck (18%; including 1 submental cyst). Various surgical approaches were chosen. In 2 patients (4%), the dermoid cyst had an intracranial extension. Only 1 patient experienced recurrence. The median pathologic diameter of the cysts was 1.2 cm. CONCLUSIONS: Dermoid cysts are unusual neoplasms that often present in childhood, with the orbit being the area most commonly affected in the head and neck region. Imaging studies help rule out an intracranial or intraorbital extension. With complete excision, recurrence is unusual. Significance Our review will assist both primary care physicians and subspecialists in diagnosing and treating dermoid cysts.     

Dermoid cyst of the lateral ventricle associated with ethmoidal dermal sinus. Report of a case

We report a case of a ventricular dermoid cyst associated with a dermal sinus connected with the ethmoidal cells in a patient who developed rapid symptoms of raised intracranial pressure. Computed tomography showed a cystic mass in the right lateral ventricle with a hydrolipidic image in the left frontal horn of the ventricle and associated hydrocephalus. Magnetic resonance imaging showed a heterogeneous T1 hyperintense mass with a fistulous tract communicating with the ethmoid cells. A cerebrospinal fluid ventriculo-peritoneal shunt was initially established, which required further revision. A right sided transventricular approach was undertaken in a second stage, allowing resection of a dermoid cyst. Obliteration of the dermal sinus tract was obtained using pericranial duraplasty. Clinical and imaging features are discussed. The need for total resection including the tumor capsule and occlusion of the fistula are emphasized if recurrence and infection are to be prevented.     

Obstructive neonatal respiratory distress: infected pyriform sinus cyst

Infected lateral cervical cysts in newborn are rare. We present the case of a baby born at 41 weeks of gestation. At day 3, persistent cyanosis was noted, and a mass appeared in the left cervical region next to the sternocleidomastoid muscle. No cutaneous sinus was visible. Ultrasound imaging showed no sign of blood flow within the mass and no septae. The mass extended down to the aortic arch and pushed the trachea to the right. A cervical lymphangioma was first suspected. Puncture of the mass evacuated 80 mL of pus, and a drain was put in place. Opacification through the drain showed a tract originating from the left pyriform fossa. Preoperative laryngoscopy and catheterization of the fistula tract confirmed the diagnosis. The cyst was totally excised up to the sinus with the assistance of a guidewire inserted orally through a rigid laryngoscope. This is a rare case of an infected pyriform sinus cyst in the neonatal period.     

Nasofrontal dermoid sinus cyst: report of two cases

OBJECTIVE AND IMPORTANCE: Nasofrontal dermoid sinus cysts are rare. The embryological origin, presentation, treatment, and genetic associations of two cases of these cysts are discussed. Emphasis is placed on physical findings and the importance of addressing both the intracranial and extracranial components. CLINICAL PRESENTATION: The first patient, a 33-year-old woman, sought care for chemical meningitis. As a child, she was differentiated from her identical twin sister by a dimple on the tip of her nose. The second patient, a 34-year-old man, sought care for new-onset seizures. Since birth, he had a dimple on the tip of his nose. As a child, he had undergone resection of a nasal cyst. Imaging studies in both patients indicated a midline anterior cranial base mass within the falx and a defect in the crista galli. INTERVENTION: Both patients underwent biorbitofrontal nasal craniotomy. A bifrontal craniotomy was performed first, then removal of the orbitonasal ridge. The dermoid and involved falx were resected. The sinus tract was followed through the crista galli and resected up to the osteocartilaginous junction in the nose. The remainder of the tract was resected via a small incision through the nares. The dura was closed primarily by mobilizing the dura along the sides of the crista galli. After surgery, both patients still possessed their sense of smell. CONCLUSION: Nasofrontal dermoid sinus cysts have a unique embryological origin. A midline basal frontal dermoid associated with a dimple on the nasal surface with or without protruding hair and sebaceous discharge is the pathognomonic presentation. It is important to address both the intracranial and extracranial component surgically. Although concomitant anomalies and familial clustering have been described, most cases are spontaneous occurrences.     

Nasal dermoid sinus cysts: a retrospective review and discussion of investigation and management

Nasal dermoid sinus cysts are uncommon congenital anomalies presenting either as cysts or sinuses. They are frequently associated with extension into the intracranial space, requiring craniotomy for adequate resection. At the Royal Children's Hospital in Melbourne, Australia, we have managed 25 patients with nasal dermoid sinus cysts over 8 years and present details of clinical features, preoperative assessment, and surgical management. Six patients presented with infection, including 1 with osteomyelitis. Four of our patients had intracranial extension of their lesions, and all were treated successfully with tailored investigation and appropriate surgical procedures. Insights into diagnosis, investigation, and surgery are offered to facilitate the management of these challenging lesions.     

Intracranial complications of midline nasal dermoid cysts

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.     

Congenital dermoid cysts and fistulae of the nose. Apropos of 19 cases]

Nineteen patients with nasal dermal sinuses were operated over a period of twelve years. 12 patients presented with a fistula and 8 with a cyst. One of them presented with a double sinus ostia. In six patients, intracranial extension was confirmed through a surgical approach. Clinical examination and CT scans determined the choice of surgical approach. All sinuses were removed by two surgical procedures: direct incisions, either vertical midline or horizontal glabellar, and nasofrontal osteotomy via a coronal approach. The main theories of dermoid development are explained and embryogenesis is compared to the operative findings.     

Intra-axial CNS dermoid cyst

Intracranial dermoid cysts are rare tumors. They constitute 0.3% of intracranial tumors. These are commonly seen in the midline and sylvian area. Intraaxial lesions are extremely rare. We report the case of a 35-years-old female with a large intraaxial dermoid cyst, which was reported as oligodendroglioma on imaging studies done preoperatively, but was confirmed to be a dermoid cyst intra-operatively and on histopathological examination; thus highlighting a diagnostic dilemma. Patient did well post operatively and there is no recurrence in the one year follow-up. To conclude, dermoid cysts are rare benign tumors, and intraaxial lesions are still rarer. Complete surgical excision may become difficult due to adherence to nerves and vessels.     

Nasal dermoid sinus cysts and the role of open rhinoplasty.

All suspected congenital abnormalities of the nose require further evaluation. The nasal dermoid sinus cyst (NDSC) is one of the many midline nasal masses that often pose diagnostic and treatment dilemmas for the plastic and reconstructive surgeon. NDSCs are distinct from other facial dermoids in their potential for involving deeper contiguous structures, and intracranial extension. Accurate diagnosis and effective treatment are essential to avoid craniofacial skeletal deformation, cyst rupture, and infection that could cause cutaneous, ocular, or intracranial complications. A comprehensive discussion of the embryogenesis, pathogenesis, diagnosis, and surgical management of the NDSC is presented to delineate the role of open rhinoplasty in optimizing the management of this congenital nasal deformity.     

Persistent third branchial apparatus

Neck sinuses, cysts or fistulae arising from the third branchial apparatus, have seldom been reported. Between 1979 and 1989, 16 cases were diagnosed as persistent third branchial apparatus based on a fistula open or in proximity to the pyriform sinus. There were 8 boys and 8 girls whose ages ranged from newborn to 13 years. Esophagogram was performed in 6; 4 showed a fistula tract leading to the pyriform sinus. Others were demonstrated at surgery. The main presentations were suppurative thyroiditis (5), lateral neck fistula (5), cyst (3), mass (1), abscess (1), and esophageal stricture (1). This was interpreted as a spectrum of disease that in the newborn may present as a cyst, and later in childhood as a fistula in the lower neck or recurrent suppurative thyroiditis if the fistula ends in the thyroid gland. It is imperative to search for this internal communication to the pyriform sinus to make a correct diagnosis and to avoid development of esophagocutaneous fistula postoperatively. With more awareness of this disease entity, the noted incidence should increase.     

The clinical relevance of certain observations on the histology of the thyroglossal tract

A study of all patients operated on with a clinical diagnosis of midline neck cyst at the Children's Hospital of Eastern Ontario confirms our impression that certain important facts regarding the microanatomy of thyroglossal duct cyst and its associated tract are the subject of a number of misconceptions entrenched both in surgeon's minds and in some standard reference texts. Our own observations and a review of the literature dating back to Sistrunk's own original contribution in 1920 lead us to emphasize the following: (1) thyroglossal duct cysts seldom have an intact lining; (2) the thyroglossal duct is frequently multiple and arborizes; (3) its course is always anterior to the hyoid bone; (4) it is seldom discernible to the naked eye and it is futile to attempt to dissect it out at operation. By the same token, a full Sistrunk procedure must be done even if the tract is not seen; and (5) deep cervical dermoid cysts may mimic thyroglossal duct cyst when firmly fixed to the hyoid bone, but the presence of sebaceous material in the cyst will identify it as a dermoid because thyroglossal duct cysts do not undergo keratinizing squamous metaplasia.     

Nasal dermoïd cyst with intracranial extension: which approach?

Dermoïd cyst is the most common midline congenital nasal tumor. Intracranial extension is rare but possible, must be suspected and confirmed by a cerebral magnetic resonance imagining (MRI). Only total surgical removal via a combined intracranial/extracranial approach appears to provide a complete resolution and effective protection against late recurrence. We report a case of a 2 years old patient who was operated for a nasal congenital cyst extending to the nasal septum. Anatomopathology showed a dermoïd cyst. Five years later, he presented local recurrence of the dermoïd cyst with intracranial extension through a bifid crista galli. We conclude that to avoid recurrence, the removal of the nasal cyst and sinus tract must be followed to its dural attachment. A transfacial approach can be associated with frontal craniotomy, which can provide adequate exposure for complete removal of the intracranial component of the cyst and sinus tract. A literature review was performed.     

Indocyanine Green Fluorescence Endoscopy at Endonasal Transsphenoidal Surgery for an Intracavernous Sinus Dermoid Cyst: Case Report

The complete resection of intracavernous sinus dermoid cysts is very difficult due to tumor tissue adherence to important anatomical structures such as the internal carotid artery (ICA), cavernous sinus, and cranial nerves. As residual dermoid cyst tissue sometimes induces symptoms and repeat surgery may be required after cyst recurrence, minimal invasiveness is an important consideration when selecting the surgical approach to the lesion. We addressed a recurrent intracavernous sinus dermoid cyst by the endoscopic endonasal transsphenoidal approach assisted by neuronavigation and indocyanine green (ICG) endoscopy to confirm the ICA and patency of the cavernous sinus. The ICG endoscope detected the fluorescence signal from the ICA and cavernous sinus; its intensity changed with the passage of time. The ICG endoscope was very useful for real-time imaging, and its high spatial resolution facilitated the detection of the ICA and the patent cavernous sinus. We found it to be of great value for successful endonasal transsphenoidal surgery.     

Ruptured intracranial dermoid cysts

BACKGROUND

Intracranial dermoid cysts are rare congenital neoplasms that are believed to arise from ectopic cell rests incorporated in the closing neural tube. The rupture of an intracranial dermoid cyst is a relatively rare event that typically occurs spontaneously. In the past it was believed that rupture is always fatal, a hypothesis that is not supported by more recently reported cases. The symptoms associated with rupture vary from no symptoms to sudden death.

METHODS

The present paper analyzes published cases of ruptured intracranial dermoid cysts in terms of their age profile and their clinical presentation and describes an additional case.

RESULTS

Analysis of published cases revealed headache (14 out of 44 patients; 31.8%) and seizures (13 out of 44 patients; 29.5%), to be the most common signs of rupture followed by, often temporary, sensory or motor hemisyndrome (7 out of 44 patients; 15.9%), and chemical meningitis (3 out of 44 patients; 6.9%).

CONCLUSION

Headache occurred primarily in younger patients (mean age 23.5 ± 9.3 years), whereas seizures primarily occurred in older patients (mean age 42.8 ± 11.3 years). The patients with sensory or motor hemisyndrome associated with rupture of an intracranial dermoid cyst showed a more homogeneous age distribution (mean age 38.4 ± 23.5 years).     

Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus: case report

BACKGROUND: Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION: A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.     

Clinical and embryological approaches to nasal dermoid sinus cysts

Nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. Thirty children with nasal midline masses and/or sinus ostia were surgically treated in the pediatric ENT and cervicofacial surgery department of Trousseau's children's hospital (Paris-France) between 1995 and 2002. All our patients underwent radiological evaluation including CT scan (including axial and coronal planes) and/or MRI (in all three planes) in search of intracranial extension. Thirteen of our patients presented with a midline cyst only, ten had nasal pit only, and seven had combined anomalies. Preoperative radiology and surgery showed an intracranial extension in 3 patients, which exhibited contact of the cyst with the dura. Recurrence being expected if any dermal tissues were left in place, "one-time-excision management" was the rule. CT and MR imaging features were reviewed. The external rhinoplasty procedure resulted in a wide surgical approach, low recurrence and good esthetic results. The embryological and anatomical origins of NDSCs are reviewed. Development of NDSC during embryological development implicates two necessary and sufficient conditions: competence of the ectodermal and mesectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions, and a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. We propose to distinguish NDSCs of anterior topography, located at the anterior skull base level from the basal NDSCs, located at the middle skull base level. We reviewed the various localizations of NDSCs, revisiting a forgotten embryological theory, which unify the various clinical localizations of anterior NDSCs.