Atlas Oral Maxillofacial Surg Clin N Am 10 (2002) 8599

Dermoid cysts, gliomas, and encephaloceles: evaluation and treatment

Jerey C. Posnick, DMD, MD, FRCS(C), FACSa,c, and Bernard J. Costello, DMD, MDb,c,*
a Departments of Plastic Surgery, Otorhinolaryngology/Head and Neck Surgery, Oral and Maxillofacial Surgery, and Pediatrics, Georgetown University School of Medicine, Washington, DC, USA b Departments of Otorhinolaryngology, Head and Neck Surgery, and Oral and Maxillofacial Surgery, Georgetown University School of Medicine, Washington, DC, USA c Posnick Center for Facial Plastic Surgery, Chevy Chase, MD, USA

A spectrum of congenital frontonasal malformations exists, with encephaloceles at one end, dermoid sinus cysts at the other, and gliomas in between. The encephalocele is a herniation of brain and meninges, and the glioma is composed of glial tissue (i.e., nonnervous cellular elements of nervous tissue), which is separate from the brain. In contrast to these neuroectodermal malformations, the dermoid cyst is a somatic ectodermal anomaly in the region. Dierentiating between encephaloceles and gliomas may be dicult. The glioma is rm and not compressible, whereas the encephalocele is not as rm and is compressible to some extent. Pulsation and increase in size on straining may be noted in encephaloceles. The diagnosis is conrmed by a CT scan or MR image. Persistence of ectodermal tissue anywhere anterior to the foramen cecum within the prenasal space, extending to the nasal tip, produces a dermoid cyst or sinus. Glabellar cysts or sinuses can be explained by persistence of the same tissue anterior to the region of the nasofrontal fontanelle. Failure of obliteration of the dural process, which normally exits through the foramen cecum in early embryonic life, sets the stage for development of dura-lined cysts, including dumbbellshaped lesions with elements above and below the foramen. Embryology The most widely accepted theory of the embryogenesis of nasally located encephaloceles, gliomas, and dermoid cyst sinus tracts was proposed by Grunwald in 1910 and later elaborated by Pratt. It states that during early embryologic development, the nose consists of three layers: ectoderm, mesoderm, and a deeper layer of cartilage that is part of the cartilaginous capsule. Later, at 50 to 60 days gestation in human beings, the nasal and frontal bones develop by intramembranous ossication of the mesoderm, but they remain separated by a space called the fonticulus nasofrontalis. Between the nasal and frontal bones and the deeper cartilaginous capsule, a space extends from the base of the skull to the nasal tip. A small projection of dura extends into this space (with or without arachnoid and neural tissue). The dura projection may extend (1) anteriorly through the fonticulus nasofrontalis or (2) inferiorly through the developing frontal bones into the prenasal space. These diverticula come into contact with the skin and may adhere to it. Normally, the dural diverticula regress with time. The nasal process of the frontal bones separates the skin from the dura, and the projection of dura becomes encircled

* Corresponding author. 100 Anderson Street, Apt. 435, Pittsburgh, PA 15212. E-mail address: drbjcostello@hotmail.com (B.J. Costello) 1061-3315/02/$ - see front matter 2002, Elsevier Science (USA). All rights reserved. PII: S 1 0 6 1 - 3 3 1 5 ( 0 1 ) 0 0 0 0 4 - X

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by an opening in the frontal bones called the foramen cecum. The foramen cecum eventually fuses with the fonticulus nasofrontalis in the area of the future cribriform plate and in so doing obliterates neuroectodermal connections. Incomplete closure at the glabella gives rise to spreading of the nasofrontal suture or to a canal through the bone. Incomplete closure of the midline anterior skull base leads to a wide foramen cecum with a distorted or bid crista galli. Depending on the patency of the diverticulum and its contents, the resulting lesion can be a dermal cyst or sinus tract, a glioma, or an encephalocele at the glabella or within the prenasal space.

Nasal dermoid sinus cysts Midline nasal dermoid sinus cysts are rare congenital lesions that result from faulty embryologic development. They are distinct from other facial dermoids in their potential to exist as a cyst, sinus, or stula, which also can involve deeper contiguous structures, and in their potential for intracranial extension. Failure to recognize and treat this condition promptly may lead to primary or recurrent (persistent) infections and result in meningitis or brain abscess. The nasal dermoid sinus cyst constitutes 1% to 3% of all dermoids of the body and 3% to 12% of dermoid cysts found in the head and neck (Fig. 1). Most cysts that occur arise sporadically; however, reports of familial occurrence have been documented. Associated congenital anomalies are

Fig. 1. A 3.5-year-old boy who was born with a cystic mass over the dorsum of the nose that was indicated by clinical and radiologic examination was determined to be a midline nasal dermoid sinus cyst extending intracranially underwent excision through a direct nasal and coronal (scalp) incision with immediate reconstruction. (A) Frontal view before surgery. (B) Frontal view 3 years after excision and reconstruction. (C) Axial CT scans demonstrating intracranial extension. (D) Coronal CT scans demonstrating intracranial extension. (E) Close-up frontal view before surgery. (F) Direct vertical nasal incision following the nasal dermoid sinus cysts down to bone (note multiple cysts). (G) Intraoperative view through a coronal (scalp) incision and after bifrontal craniotomy. The dermoid nasal sinus cyst tract is followed toward the dura. (H) The intracranial cyst sinus tract extension required excision of involved dura. With the mass excised and the dura repaired, an intraosseous dead space remains in the glabellar region of the frontal bones. (I) The intraosseous dead space is closed with autogenous cranial bone shavings. The frontal bone was initially xed with direct wires, but the replaced bone tended to collapse. Miniplates were used to secure the bone in the anatomically correct position. (From Posnick, J.C., Bortoluzzi, P., Armstrong, D.C., et al., Intracranial nasal dermoid sinus cyst: computed tomographic scan ndings and surgical results. Plast Reconstr Surg 1994; 93:745; with permission.)

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Fig. 1 (continued )

believed to be infrequent, although Wardinsky et al reported congenital anomalies in 41% of their patients with nasal dermoid sinus cysts. On histologic examination, the dermoid sinus cyst contains ectodermal and mesodermal derivatives. It is composed of a stratied squamous epithelial lining with specialized adnexal tissues, which may include hair follicles, pilosebaceous glands, and smooth muscle. The presence of adnexal dermal components distinguishes dermoid cysts from simple epidermoid cysts, and unlike teratomas, they do not contain an endodermal component. Intracranial extension of nasal dermoid sinus cysts is most frequently extraaxial, attached to the dura, or conned within the leaves of the anterior falx (Fig. 1). Intraaxial extension into brain parenchyma has been reported. Earlier reports suggest that intracranial extension is a rare occurrence of just 1% or 2% of the dermoid cysts that presenting in the nasal region. The more recent literature, however, indicates a much higher prevalence of intracranial extension: Sessions reported a prevalence of 30%; Wardinsky et al, 45%; Bradley, 10%; Clark et al, 26%; Naidich et al, 30%; Pensler et al, 19%; Paller et al, 25%; and Posnick et al, 36%. These studies may indicate a high percentage of intracranial extension that results from a referral bias to tertiary medical centers. Computed tomographic scans have proved accurate in assessing intracranial extension of nasal dermoid sinus cysts. Positive CT scan ndings include subcutaneous midline cysts, bid

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Fig. 1 (continued )

crista galli, and enlarged foramen cecum. MR imaging also can provide a clearer image of the soft tissues and is of value in dening soft tissue masses and following the location of the sinus tract. Early recognition, accurate diagnosis, and prompt treatment of nasal dermoid sinus cysts found to have intracranial extension are important. When nasal dermoid sinus cysts are untreated, possible complications include bacterial meningitis, chemical meningitis, and brain abscess. To gain exposure for the removal of nasal dermoid sinus cysts, the authors favor a direct vertical midline incision. If an intracranial extension is postulated, total excision of the lesion requires a combined intracranial-extracranial approach through an additional coronal (scalp) incision. Posnick et al documented the safety of carrying out a single-stage procedure when intracranial extension is present.

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Fig. 1 (continued )

In a previously published study, the presence of an intracranial extension of a nasal dermoid sinus cyst was documented by Posnick et al in 36% (5 of 14) of (consecutive) patients with nasal dermoid cysts. Although the authors believe the referral pattern may be biased toward the more complicated problems, this rate concurs with the high incidence reported by others. The authors patients varied in age from 4 to 48 months (mean, 25 months) at the time of referral. Most patients (three of ve) with intracranial extension had lesions that were asymptomatic masses (one had a chronic discharge and one recurrent bleeding). None had associated congenital anomalies, unlike the patients in the study of Wardinsky et al, who reported a 41% incidence of congenital anomalies. In accordance with the literature, most (four of ve) of the authors patients lesions were located on the nasal dorsum. Contrary to the literature that describes the sinus tract proceeding beneath the nasal bones and through the nasal septum into the skull base, in three of the ve patients, the tract penetrated the craniofacial skeleton at the level of the nasal bones. The point of penetration through the nasofrontal suture occurred in one patient who had multiple cysts in the nasofrontal area and nasal dorsum. This nding contrasts with the contention by Bartlett et al that a mass at or above the nasofrontal suture never penetrates the deeper contiguous structure of involvement. An alternative embryologic explanation can be provided to explain this occurrence: neuroectodermal derivatives may project through a patent fonticulus frontonasalis rather than through the prenasal space.

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Fig. 2. An 8-year-old girl raised in the Philippines was born with a large nasoethmoidal encephalocele that resulted in severe distortions of the upper and midface structures (left greater than right). She had not previously undergone treatment, and the mass aected vision, breathing, physical activities, and self-esteem. She was referred to the authors and underwent a combined neurosurgical and craniofacial approach, including plication of the encephalocele and immediate skeletal and soft-tissue reconstruction. The procedure was carried out through coronal (scalp) and direct vertical nasal incisions and required multiple osteotomies, autogenous cranial grafting, and plate and screw xation. (A) Worms-eye view before surgery. (B) Three-dimensional CT scan (frontal view) of craniofacial region before surgery. (C) Three-dimensional CT scan of craniofacial region indicating extent of encephalocele and secondary distortions of the skeleton. (D) Three-dimensional CT scan of dura outline indicating extent of encephalocele. (E) Prole view before surgery. (F) Midsagittal MR image indicating CNS soft tissues and extent of encephalocele. (G) Intraoperative birds-eye view indicating extent of vertical excision over dorsum of nose with dissection of encephalocele down to bone. (H) Intraoperative view through coronal incision after bifrontal and nasoethmoidal osteotomies to expose and then plicate encephalocele. (I) Full-thickness autogenous cranial bone graft crafted to replace medial orbital rims. (J) View of reconstructed orbitonasal regions. (K) Frontal view before surgery. (L) Frontal view 8 weeks after reconstruction just before the patients return to the Philippines. Vertical elongation of central midface and nasal soft tissues remains.

Gliomas Gliomas are composed of glial tissue that consists of non-nervous cellular elements of nervous cells. Gliomas are not familial but are seen more frequently in men than in women (ratio, 3:2). Pensler et al reviewed a series of ten patients with gliomas who were treated over a 6-year time frame at a major pediatric referral center. Two of four of the intranasal lesions exhibited intracranial extension. Six of ten of the gliomas were located extranasally. Extranasal gliomas are usually located on the dorsum or just lateral to the bridge of the nose. They are noncompressible lesions that do not transilluminate, and they appear as smooth, rm masses. The overlying skin may be discolored or telangiectatic. One of six extracranial gliomas in this study group was found to have intracranial extension. The intranasal gliomas are also noncompressible and appear reddish, rm, and polyploid in character. Nasal obstruction and secondary septal distortions with partial loss of olfaction may occur and depend on the size of the glioma. CT scanning or MR imaging or both should be carried out with contrast material to help clarify intracranial extension. Dierentiating between angiobromas and other tumors and encephaloceles or dermoids is important to ensure appropriate treatment.

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Fig. 2 (continued )

The treatment of a glioma, like that of a dermoid sinus cyst, is surgical excision. It is recommended that the lesions located intranasally be excised early in childhood when feasible to limit the complications of infection and obstructive nasal breathing. Patients with cranial bone erosion require an intracranial approach with immediate reconstruction that uses bone taken from the cranium. Extranasal gliomas are excised through direct incisions.

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Fig. 2 (continued )

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Fig. 3. A boy born with a large nasofrontal encephalocele underwent three surgical procedures at another institution in an attempt to plicate the encephalocele and reconstruct the fronto-orbitocranial base defects. Severe orbital hypertelorism, orbital dystopia, and skull and cranial base defects remained, and the nasolacrimal apparatus functioned poorly. He arrived for craniofacial reconstruction, which was carried out through a previous coronal (scalp) incision and required multiple orbital, nasal, and frontal osteotomies, autogenous cranial bone grafting, and plate and screw xation. (A) Frontal view after initial procedures by another surgeon indicates residual orbital hypertelorism and dystopia. (B) Two- and three-dimensional CT scans indicating extent of orbital dystopia/hypertelorism and skull defects. (C) Right oblique view before surgery. (D) Right oblique view after reconstruction. (E) Left oblique view before surgery. (F) Left oblique view after reconstruction. (G) Worms-eye view before surgery. (H) Worms-eye view after reconstruction. (I) Intraoperative close-up view of cranio-orbital region before surgery. (J) Intraoperative close-up view of cranio-orbital region after reconstruction. (K) Birds-eye view of anterior cranial vault before surgery. (L) Birds-eye view of anterior cranial vault after reconstruction. (M) Comparison of three-dimensional CT scans of craniofacial region before and after reconstruction. (N) Comparison of coronal CT slices through the midorbits before and after reconstruction.

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Fig. 3 (continued )

Encephaloceles Encephaloceles may occur as isolated malformations or together with other anomalies as part of various syndromes or associations. Encephaloceles can be classied as occipital, parietal, or anterior (Figs. 24). Further subdivision of anterior encephaloceles may be made into (1) visible

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Fig. 3 (continued )

(sincipital) and (2) not directly visible (basal). Approximately 75% of all encephaloceles occur in the occipital region. Parietal encephaloceles account for approximately 10% to 14% of all encephaloceles. Frontal encephaloceles are much less common than occipital encephaloceles in whites, with the ratio being 1:6.

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Fig. 3 (continued )

Frontonasal encephaloceles, encountered relatively rarely in occidental countries (1 in 35,000 live births), are seen commonly in southeast Asia (1 in 5000 live births). Charoonsmith, in Bangkok, has reported on a series of 310 individuals who presented with an encephalocele. He categorizes frontoethmoidal encephalomeningoceles into three subgroups: 1. Nasofrontal encephalocele: The osseous defect is located in the bregmatic region, between the two orbits, in the midline, at the root of the nose. The medial orbital walls are displaced laterally. 2. Nasoethmoidal encephalocele: The osseous defect is between the frontal and ethmoid bones. The herniated brain passes between the nasal bones and nasal cartilages with displacement of the medial wall of the orbits. 3. Nasoorbital encephalocele: The frontal and nasal bones are normal. The herniated brain passes through a defect in the medial orbital wall. Nasofrontal and/or nasoethmoidal encephaloceles result in a variably increased distance between the frontal bones and the nasal bones with vertical elongation of the nose. The extent of associated orbital hypertelorism varies. Not all hypertelorism necessarily requires correction. Osteotomies of the orbits to correct hypertelorism may be necessary if the displacement is particularly severe, however. There is no one reconstructive approach to these lesions. The design of the osteotomies varies based on the dysmorphology seen. Some patients with severe displacement of the orbital, ethmoid, nasal, and maxillary regions may benet from a facial bipartition procedure. These patients may require immediate reconstruction of the medial orbital walls and nasal complex using autogenous bone grafts taken from the cranium. Other patients may require only reshaping and reconstruction of the upper orbital rims and frontal bones. When the encephalocele is associated with hydrocephalus, early ventriculoperitoneal shunting is essential. The initial neurosurgical management of the encephalocele should, in general,

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go forward early in infancy. In most cases, repair of the osseous defect with autogenous cranial graft is preferred at the time of neurosurgical plication of the encephalocele because it reinforces the closure and limits the incidence of cerebrospinal uid leakage and need for early reoperation to manage this complication. It has been the authors experience that the displacement of craniofacial skeletal structures by the encephalocele does not progress over time unless untreated hydrocephalus expands the encephalocele, resulting in progressive skeletal destruction. Denitive reconstruction of the anterior cranial vault, orbits, and nasal bones is best postponed until 4 to 7 years of age, if at all possible. By waiting until the child has attained a more adult level of upper face skeletal maturity, the reconstruction is more denitive. Psychosocial considerations also support the time frame of 4 to 7 years of age for the nal cranioorbital procedure. When the procedure is carried out at this age, the child may progress through grade school with a real chance for a satisfactory body image and self-esteem. The functioning nasolacrimal apparatus is preserved and protected if possible, but it otherwise requires specic reconstruction. Strabismus and amblyopia are best evaluated by a pediatric neuroophthalmologist early in the childs life. Eye patching and surgical correction may be necessary but must be evaluated and treated before age 9 to 11 years to gain the maximum benet. The need for additional augmentation over the decient nasal bones and orthognathic ( jaw straightening) surgery in the teenage years should be anticipated for some patients. Waiting for the upper and midfacial growth to be more complete allows for a more denitive reconstruction of the nasal complex if required. When necessary, the elective orthognathic surgery is

Fig. 4. A newborn with a large nasofrontal encephalocele underwent a combined neurosurgical and craniofacial approach with encephalocele plication and soft-tissue reconstruction through a direct vertical nasal incision at 1 month of age. At 2 months of age, he returned to the operating room for ventriculoperitoneal shunting and autogenous cranial bone grafting to the frontonasal defect. He has maintained normal vision and has met his developmental milestones. (A) View of child soon after birth. (B) Close-up prole view of child before surgery. (C) Prole view at 8 months of age, after reconstruction. (D) Frontal view at 8 months of age, after reconstruction. Note hypertrophic scarring, which will require soft-tissue revision.

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Fig. 4 (continued )

carried out in conjunction with orthodontic treatment planned for completion at the time of early skeletal maturity (approximately 1315 years in girls and 1517 years in boys). Facial scar revision can be performed at any time after maturity of the scar tissue but is best delayed until most growth has been completed in the region of concern.

Summary The fronto-orbitonasal malformations of encephaloceles, dermoid sinus cysts, and gliomas represent a continuum of neuroectodermal anomalies. The dierentiation between them and other similar-appearing lesions is essential for eective management to proceed. Obtaining a reliable history, completing a careful physical examination, and obtaining accurate radiographic documentation represent the rst steps. Establishing the timing, staging, and specic surgical techniques for management of a fronto-orbitonasal encephalocele remains as much an art as a science.

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Suggested reading Nasal dermoid sinus cysts

Bartlett SP, Lin KY, Grossman R, Katowitz J. The surgical management of the orbitofacial dermoids in the pediatric patient. Plast Reconstr Surg 1993;91:120815. Clark WD, Bailey BJ, Stiernberg CM. Nasal dermoid with intracranial involvement. Otolaryngol Head Neck Surg 1985;93:1024. Crawford JK, Webster JP. Congenital dermoid cysts of the nose. Plast Reconstr Surg 1952; 9:235. Naidich TP, Bauer BS, McLone DG, et al. Nasal dermoid sinuses and cysts. Acta Radiol 1986;369(suppl):3224. Nevares RL, Mulliken JB, Robb RM. Ocular dermoids. Plast Reconstr Surg 1988;82:95964. Paller AS, Pensler JM, Tomita T. Nasal midline masses in infants and children: dermoids, encephaloceles, and gliomas. Arch Dermatol 1991;127:3626. Pensler JM, Bauer BS, Naidich TP. Craniofacial dermoids. Plast Reconstr Surg 1988; 82:9538. Posnick JC, Bortoluzzi P, Armstrong DC. Nasal dermoid sinus cysts: an unusual presentation, computed tomographic scan ndings, and surgical results. Ann Plast Surg 1994;32:51923. Posnick JC, Bortoluzzi P, Armstrong DC, Drake JM. Intracranial nasal dermoid sinus cysts: computed tomographic scan ndings and surgical results. Plast Reconstr Surg 1994; 93:7556. Sessions RB. Nasal dermal sinuses: new concepts and explanations. Laryngoscope 1982; 92(Suppl 29):1. Wardinsky TD, Pagon RA, Kropp RJ, et al. Nasal dermoid sinus cysts: association with intracranial extension and multiple malformations. Cleft Palate Craniofac J 1991;28:8795.

Glioma
Pensler JM, Ivescu AS, Ciletti SJ, et al. Craniofacial gliomas. Plast Reconstr Surg 1996;98:2730. Smith KR, Schwartz HG, Luse SA, et al. Nasal gliomas: a report of ve cases with electron microscopy of one. J Neurosurg 1963;20:968. Walker EA, Resler DR. Nasal glioma. Laryngoscope 1963;73:93.

Encephalocele
Charoonsmith T. Review of 310 patients with frontoethmoidal encephalomeningocele with reference to plastic reconstruction.: Transactions of the 8th International Congress of Plastic Surgery, Montreal, 1983. P. 314317. Charoonsmith T, Suwanwela C. Frontoethmoidal encephalomeningocele with special reference to plastic reconstruction. Clin Plast Surg 1974;1:2747. Cohen MM, Lemire RJ. Syndromes with cephaloceles. Teratology 1982;25:161172. David DJ: Cephaloceles: classication, pathology, and management. A review. J Craniofac Surg 1993; 4:192202. Gorlin RJ, Cohen MM, Levin LS: Syndromes of the head and neck. 3rd ed. New York: Oxford University Press, 1990 Jackson IT, Tanner NSB, Hide TAH. Frontonasal encephalocele: long nose hypertelorism. Ann Plast Surg 1983;2: 490500. MacFarlane R, Ritka JT, Armstrong D, Phillips J, Posnick JC, et al: Encephaloceles of the anterior cranial fossa. Pediatric Neurosurgery 1995: 23: pp 148158. Posnick JC: Dermoids, Gliomas and Encephaloceles: Evaluation and Treatment. Craniofacial and Maxillofacial Surgery in Children and Young Adults (Posnick JC, ed). W.B. Saunders Co., Philadelphia, 2000, Chap 25: pp 531563. Suwanwela C. Geographic distribution of frontoethmoidal encephalomeningocele. Br J Prev Soc Med 1972; 26:1938. Suwanwela C, Sukabote C, Suwanwela N. Frontoethmoidal encephalomeningocele. Surgery 1971; 69:61725. Suwanwela C, Suwanwela N. A morphological classication of sincipital encephalomeningoceles. J Neurosurg 1972; 36:20111.