Risk of Intracranial Extension of Craniofacial Dermoid Cysts.

BACKGROUND: Dermoid cysts are benign lesions lined by keratinizing squamous epithelium that also contain epidermal adnexa (hair follicles, hair shafts, sebaceous glands, and both apocrine and eccrine sweat glands) and mesodermal derivatives (smooth muscle fibers, vascular stroma, nerves, and collagen fibers). Craniofacial dermoid cysts represent approximately 7 percent of all dermoids and have an incidence ranging between 0.03 and 0.14 percent. METHODS: The authors conducted a single-center, consecutive, nonrandomized comparative case series over a 20-year period of all patients treated surgically for craniofacial dermoid at the Royal Children's Hospital in Melbourne, Australia. Six hundred forty-seven patients had craniofacial dermoids and adequate information to be included in the study. The authors also conducted a thorough review of the literature using the MEDLINE and Embase databases. RESULTS: Six hundred forty-seven patients amounted to 655 lesions in our case series. The age at surgery ranged from 2 months to 18 years, with an average age of 25.65 months. The depth of the lesions was stratified using a classification system, and the risk of intracranial extension was assessed using these data. Midline nasal lesions are established as high risk by other studies, but frontal, temporal, and occipital lesions were found to be as risky if not more risky for intracranial extension. CONCLUSIONS: Several classification systems for craniofacial dermoid cysts have used both broader anatomical locations and physical characteristics to group these lesions and identify those warranting preoperative imaging. The authors propose a system using more specific classification of anatomical location to assist in the prompt identification of high-risk lesions and facilitate sound preoperative planning. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, V.

Hoyuelo cutáneo occipital en una recién nacida / Occipital skin dimple in a new-born girl

El seno dérmico es un defecto congénito de cierre del tubo neural que se produce entre la tercera y la cuarta semana de gestación. Es una variedad de disrafismo que permite una potencial comunicación entre la piel y el sistema nervioso central. Se localiza preferentemente en línea media posterior, desde el occipucio hasta la región lumbosacra; la localización occipital es la segunda más frecuente. La clínica es muy variable, desde pacientes asintomáticos hasta el desarrollo de meningitis o abscesos por comunicación con el sistema nervioso central como primera manifestación clínica. La resonancia magnética es la prueba de imagen diagnóstica más adecuada, ya que permite determinar la extensión de las lesiones y descartar lesiones a otros niveles. Se recomienda exéresis quirúrgica profiláctica total en todos los casos. Presentamos un caso de una recién nacida con un hoyuelo cutáneo en la región occipital visualizado en la primera exploración física

The dermal sinus is a congenital neural tube closure defect that occurs between the 3rd -4th week of gestation. It is a variety of dysraphism that allows a potential communication between the skin and the central nervous system. It often is located in the posterior midline, from occipital to lumbosacral region, the occipital location is the second most frequent. The clinic is very variable, from asymptomatic patients to development of meningitis or abscesses due to a communication with the central nervous system as the first clinical manifestation. Magnetic resonance imaging is the most appropriate diagnostic imaging test, it allows to determine the lenght of the fistulous path and rule out injuries at other levels. Total prophylactic surgical excision is recommended in all cases. We present a case of a newborn with a skin dimple in occipital region visualized in the first physical examination

Orbital Dermoid Cyst: Classification and its Impact on Surgical Management.

PURPOSE: Analysis of different clinical types of orbital dermoid cyst, and studying the impact of preoperative classification on the proposed surgical procedure. METHODS: A retrospective study of orbital dermoid cyst cases. The clinical and imaging data were reviewed. The different types of dermoid were recorded as superficial or deep, lateral or medial, exophytic or endophytic. The deep dermoids were classified according to their site and continuity of the cyst wall. The operative approach, intraoperative, and/or postoperative complications were recorded. RESULTS: The study included 153 cases of orbital dermoid. The superficial lesions represented 68.6%, of which 74.3% were lateral and 61.0% were endophytic. Intraoperative cyst perforation was recorded in 56.3% of exophytic cases approached through crease incision, and recurrence occurred in 6.3%. Defective cyst wall was found in 31.25% of deep orbital dermoid. Surgery was complicated by cyst rupture when excision was attempted, and recurrence was recorded in one case. Exposure, intended evacuation, and dissection of the cyst wall were better surgical approaches for these cases without reported complications. CONCLUSION: Preoperative classification of orbital dermoid had an important effect on the surgical procedure. Endophytic superficial lesions were approached through crease incision without complications, while exophytic lesions were better approached through infrabrow incision. Deep orbital lesions with defective wall were better approached through exposure, intended evacuation, dissection, and excision of the remaining cyst wall.

Classification of cysts with follicular germinative differentiation.

BACKGROUND: Cysts are very common in the routine of dermatopathology but follicular germinative (trichoblastic) differentiation in cysts is seen rarely. The presence of follicular germinative differentiation in a cyst alerts to consider the possibility of a basal cell carcinoma (BCC) arising in a cyst. METHODS: Five cystic lesions with zones of follicular germinative differentiation were collected. Hematoxylin and eosin sections were reassessed for architecture, types of follicular differentiation and stromal characteristics; immunohistochemical studies with Ber-EP4 were analyzed. Articles about follicular germinative differentiation in cystic lesions were reviewed. RESULTS: Cystic lesions with follicular germinative differentiation have been described in the literature under various names including trichoblastic infundibular cyst, cystic trichoblastoma, cystic panfolliculoma (CPF), dermoid cyst with basaloid proliferations, folliculosebaceous cystic hamartoma and BCC occurring in infundibular cysts. The lesions presented by us could be classified as three cystic trichoblastomas, one CPF and one cystic hamartoma with follicular germinative differentiation. CONCLUSIONS: Histopathologically, cystic trichoblastomas can be separated from CPFs. Some lesions defy classification and may be regarded as cystic follicular hamartomas. The presence of follicular papillae and bulb-like structures, advanced follicular differentiation like that of inner and outer root sheath exclude the differential diagnosis of BCC arising in a cyst.

Nasal dermoids in children: a proposal for a new classification based on 103 cases at Great Ormond Street Hospital.

OBJECTIVES: Nasal dermoids are rare developmental anomalies seen in children. This study reports the largest case series of 103 patients seen in a quaternary specialist unit over a 10-year period. We report the surgical and radiological findings and propose a new classification system, which clearly describes the extent of the lesions, thus allowing better surgical planning. METHODS: A retrospective review of case notes was conducted. Data collection included demographics, initial presentation, site of lesion, pre-operative CT and MRI imaging, surgical procedure, intraoperative findings (including depth of lesion), complications and recurrence. Surgical findings were correlated with radiological findings. RESULTS: A total of 103 patients were included in the study. The mean age at presentation was 29 months. 89% of children presented with a naso-glabellar or columellar lesion and 11% had a medial canthal lesion. All the patients underwent preoperative imaging and were treated with surgical excision. 58 children had superficial lesions, 45 had subcutaneous tracts extending to varying depths. Of these, 38 had intraosseous extension into the frontonasal bones, eight extended intracranially but remained extradural and two had intradural extension. There was good correlation between radiological and surgical findings. The superficial lesions were locally excised. The lesions with intraosseous tracts were removed via open rhinoplasty and the frontonasal bones drilled for access. Intracranial extension was approached either via a bicoronal flap and frontal craniotomy or the less invasive anterior small window craniotomy. CONCLUSIONS: This report describes the largest published cases series of nasal dermoids. The cases demonstrate the presenting features and the variable extent of the lesions. The new proposed classification; superficial, intraosseous, intracranial extradural and intracranial intradural, allows precise surgical planning. In the presence of intracranial extension, the low morbidity technique of using a brow incision and small window anterior craniotomy avoids the more invasive and commonly used bicoronal flap and frontal craniotomy.

A clinico-pathological evaluation of 194 patients with ovarian teratoma: 7-year experience in a single center.

OBJECTIVES: To evaluate patients diagnosed with teratoma over a period of seven years with regard to their general characteristics and treatment methods. MATERIAL AND METHODS: A total of 194 patients histologically diagnosed with ovarian teratoma (mature or immature) in the years 2005-2012 at the Ege University Gynecology and Obstetrics Department were evaluated. RESULTS: Average patient age was 34.3 +/- 2.16 years. Of these, 169 (86.3%) were premenopausal and 27 (13.7%) postmenopausal; average cyst diameter, measured during the surgery was 6.9 +/- 0.63 cm. While the teratoma had been an asymptomatic finding in 148 (75.5%) patients, 48 (24.5%) were symptomatic. In 107 patients (54.5%) a laparotomy and in 89 (45.5%) a laparoscopy were performed. The presence of torsion was observed during surgery in 9 (4.5%) patients. The 49 (55%) patients who underwent laparoscopic cystectomy experienced a rupture during the intervention, with no cases of clinical chemical peritonitis following the surgery CONCLUSION: Caution must be exercised with regard to possible cyst rupture in elderly patients and those with large dermoids; an laparoscopic approach may be reserved for younger patients. In case a rupture occurs during the operation, abundant peritoneal lavage is indicated; in order to limit risks during the controlled excision of the cyst an Endobag should be used if possible.

Floor-of-mouth dermoid cysts: report of 3 variants and a suggested change in terminology.

PURPOSE: Dermoid cyst is a frequently used descriptive term, but its definition changes according to the clinician (eg, dermatologists, neurologists, gynecologists, oral and maxillofacial surgeons, and plastic surgeons). It is sometimes used synonymously with teratoma. In oral and maxillofacial surgery, it is applied to describe congenital floor-of-mouth cysts of 3 histologic types: epidermoid, dermoid, and teratoid. This terminology is confusing and has led to some ambiguity in the literature. The purpose of this report is to document 3 cases illustrating the utility of a more specific term, congenital germline fusion cyst of the floor of the mouth. PATIENTS AND METHODS: Patients who presented with floor-of-mouth swelling were evaluated by history, clinical examination, and imaging studies (magnetic resonance imaging and computed tomography). RESULTS: Three cases (1 epidermoid variant, 1 dermoid variant, and 1 teratoid variant) are documented to illustrate the new terminology. CONCLUSION: Congenital germline fusion cyst is more reflective of the embryologic origins of the lesion than dermoid cyst and is inclusive of all 3 histologic variants.

Bilateral parieto-occipital dermoid sinuses in a Rottweiler.

Bilateral dermoid sinuses were identified on the parieto-occipital region of a Rottweiler. Diagnosis was confirmed by histological examination after successful complete surgical resection. The dermoid sinuses were independent with separate tracts. This unusual parasagittal location can be explained by craniofacial development: dermoid sinuses on the head could occur along the lines of embryological fusion and not only in the sagittal plane. A hypothesis of an origin at the level of the suture between the parietal and interparietal bones is possible in this case.

[Management of ovarian cysts]. / Prise en charge des kystes ovariens.

Ovarian cysts occur frequently in women of reproductive age. These are usually functional cysts which resolve spontaneously and whose evolution can be followed with ultrasound. Non-functional cysts have diverse histologic origins. The most common are serous and mucinous cystadenomas which arise from the epithelial wall of the ovary, endometriomas which arise in the setting of pelvic endometriosis, and dermoid cysts which arise from the germinal cells of the ovary. Endovaginal ultrasound with Doppler enhancement is the best imaging technique to establish the nature of cysts and to distinguish cysts suspicious for malignancy which require more invasive investigation. Pelvic laparoscopy is the surgical approach of choice for the treatment of non-functional benign ovarian cysts. Conservative treatment to shell out the cyst and preserve functional ovarian tissue should be reserved for women desirous of future pregnancies. The risk of ovarian cancer remains a major preoccupation of the surgeon. Where malignancy is suspected, laparoscopy is contraindicated and a median laparotomy is appropriate for radical extirpative surgery. This article describes the diagnostic techniques which allow a laparoscopic approach to presumably benign cysts and discusses surgical techniques specifically adapted to their different histologic nature of ovarian cysts.

Cutaneous cysts of the head and neck.

PURPOSE: This article reviews the types of cutaneous cysts in patients referred to the Facial Lesion Clinic at John Peter Smith Hospital in Fort Worth, TX, and proposes effective treatment modalities based on lesion and patient variables. Cyst variables included proper identification, size of the lesion, and acute or chronic processes. Patient considerations included age, skin type, and location. Medical and social histories were not noted. PATIENTS AND METHODS: Eighty-two patients who had 1 or more cysts removed over the 5-year period from July 15, 1998 to July 14, 2003 were reviewed for age, gender, histologic diagnosis, anatomic location of the lesion, and complications. RESULTS: Patients with epidermal inclusion cysts (79%), followed by pilar cysts (9%), hidrocystomas and dermoid cysts (3% each), and multiple other diagnoses (less than 2%) were treated. Neither complications nor recurrent infections were reported during the 5-year interval. There were no recurrent cyst formations noted by return appointment. CONCLUSION: Cystic lesions of the head and neck may be treated effectively as long as they are correctly identified and treated in a specific manner.

Dermoid cyst of the testis: a study of five postpubertal cases, including a pilomatrixoma-like variant, with evidence supporting its separate classification from mature testicular teratoma.

It is controversial if the rare dermoid cyst of the testis should be classified as a variant of mature teratoma or separately. The spectrum of findings is also ill defined, as is the relationship of dermoid cyst to intratubular germ cell neoplasia of the unclassified type (IGCNU). This study therefore reports the findings in five testicular dermoid cysts that occurred in five patients, 17-42 years of age, who presented with testicular masses. Four lesions consisted of a keratin-filled cyst with a thickened wall, whereas one had islands of "shadow" squamous epithelial cells with superimposed calcification and ossification (pilomatrixoma-like variant). Hair was identified grossly in two cases. On microscopic examination, four tumors had hair follicles with sebaceous glands showing a typical, cutaneous-type orientation to an epidermal surface, although no hair shafts were present in two. In addition, the fibrous wall contained smooth muscle bundles (all tumors) and eccrine or apocrine sweat glands (4 tumors). In some cases there were also glands lined by ciliated epithelium (4 tumors, including the pilomatrixoma-like variant), intestinal mucosa (1 tumor), and bone (2 tumors). There was no cytologic atypia or apparent mitotic activity, and no case had IGCNU in the seminiferous tubules. All patients were clinical stage I and were treated by orchiectomy without adjuvant therapy. All were well on follow-up from 1.5 to 9.5 years later. This study supports that dermoid cyst may have noncutaneous teratomatous elements and that an important criterion for its diagnosis is the absence of IGCNU. It also supports that it should be categorized separately from mature testicular teratoma because of the malignant nature of the latter in postpubertal patients. These observations suggest that there are at least two pathways for testicular teratomas in postpubertal patients: the more common being through IGCNU by differentiation from an invasive malignant germ cell tumor and the less common one, taken by dermoid cyst, by direct transformation from a nonmalignant germ cell.

True lateral dermoid cyst of the floor of the mouth.

Congenital dermoid cysts of the floor of the mouth are relatively rare but when they occur, they do so inevitably in the midline. We present a case of a true lateral dermoid cyst of this region without any intra-oral extension. We discuss the anatomical and histological classification of dermoid cysts within the floor of the mouth.

Orbital dermoid cysts: clinicopathologic correlations, classification, and management. The 1997 Josephine E. Schueler Lecture.

The dermoid cyst is one of the most common space-occupying orbital lesions, but there has been no large series that details the clinical, pathologic, and therapeutic aspects of this condition. Furthermore, despite the various presentations and types of dermoid cysts, there is no clinically applicable classification of these lesions. We performed a retrospective histopathologic study of 197 consecutive orbital and periorbital dermoid cysts excised at Wills Eye Hospital during a 32-year period, correlated the histopathologic findings with the recorded clinical and radiographic observations, and devised a clinically applicable classification of orbital dermoid cysts. The mean age at excision was 17 years (range, 5 months to 85 years), and the most frequent clinical finding was a painless subcutaneous mass, present in 89% of cases. In 72% of patients, the mass was located in the superotemporal orbital region and produced no abnormalities of vision or intraocular pressure. Computed tomography and magnetic resonance imaging, performed mainly in the more recent cases, disclosed a characteristic round to ovoid cystic lesion with a well-defined lining. Histopathologically, 165 of the cysts (84%) were lined by keratinizing, stratified squamous epithelium. Ten (5%) were lined by nonkeratinizing epithelium resembling conjunctival epithelium. Hair shafts were present in the wall or lumen of the cyst in 195 (99%) of specimens, sebaceous glands in 148 (75%), and sweat glands in 39 (20%); inflammation was present in 75 cases (38%). Based on these personal observations and a review of the literature, a classification of orbital dermoid cysts is proposed that we believe will provide guidance for the ophthalmologist in the management of these lesions.

Occult spinal dysraphism--the common combination of lesions and the clinical manifestations in 50 patients.

Fifty patients (26 males, 24 females) aged from birth to 51 years with occult spinal dysraphism were identified. All had Magnetic Resonance (MR) scans carried out. The MR scans were examined to determine the vertebral level of the conus and to see if one or more of the following were present; lipoma, syrinx, dermoid, diastematomyelia, and meningocele. In 43 patients the conus lay below the level of L3, 23 had a lipoma, 23 a meningocele, 20 an open central canal in the spinal cord or a syrinx, 15 a diastematomyelia and 4 a dermoid. The commonest combination of lesions was a long cord, a syrinx or an open central canal in the spinal cord and a lipoma. The diastematomyelias were always associated with a long cord and had the highest incidence of vertebral body anomalies (60%). The most frequent recorded signs were deformities of the feet, short legs, wasting of the calf muscles, weakness of the legs, back pain and bladder dysfunction. Combinations of these occurred with all of the lesions although some had none. Whilst deterioration was seen in about half of the patients, acute deterioration was uncommon and was associated most frequently with a dermoid or an expanded syrinx.

Pharyngeal dermoids ("hairy polyps") as accessory auricles.

The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory "ears" (auricles) without the growth potential of a teratoma.

Dermoid cysts: 16-year survey.

A total of 145 patients with orbital dermoid cysts examined at the Orbital Clinic, Institute of Ophthalmology, School of Medicine, University of Naples "Federico II" over a period of 16 years were reviewed. The orbital cysts were classified as exophytic and endophytic, according to their site of attachment in relation to the orbital rims. This classification can explain the different natural history of these lesions. The exophytic cysts growing externally are discovered in childhood, whereas the endophytic ones are discovered later in life when they produce bone damage, with or without invasion of the adjacent structures.

[Cranio-cerebral dermoid and epidermoid cysts. Classification and pathogenesis]. / Les kystes dermoïdes et épidermoïdes crânio-encéphaliques. Classification et pathogénie.

The pathogenesis of cranial dermoids and epidermoids is still controversial, owing to the multiple etiologies and locations of these lesions. We reviewed 25 cases, classified as follows: extradural lesion of the calvarium; of the occipital squama; dysraphic occipital lesions; and strictly intradural lesions. In the latter group, all lesions but one were in a paramedian, prenevraxial situation, and could be classified according to their situation relative to the tentorium cerebelli. In our series, strictly intradural dermoids are more often in a rostral situation, and epidermoids in a more caudal situation. Dermoids appear earlier in life than epidermoids, suggesting a more rapid growth due to eccrine secretion. For each group of lesions, the pathogenic hypotheses are studied. Inclusion of epidermal nests at different levels might result from traumatism, dysraphism, or developmental trouble in the lamination of the different layers of the meninges. Most intradural lesions trent to be related to the formation of Rathke's pouch and closure of the anterior neuropore.