Adult nasal dermoid sinus cyst.

Midline congenital nasal lesions are rarely encountered in adults. We present the case of a 31-year-old man with a nasal dermoid sinus cyst who presented with a nasal dorsal abscess. We review the embryology of nasal dermoid sinus cysts, and we discuss their presentation, evaluation, and management in adults.

Congenital nasal malformations.

The nose is a prominent feature of the human face. Congenital malformations of the nose, whether functional or anatomic, affect the physiologic and psychologic wellness of children who have these anomalies. Congenital nasal abnormalities may be overt or subtle and can occasionally cause life-threatening emergencies at birth. A discussion of nasal embryology and development provides the basis for the discussion of some of the important congenital abnormalities seen in clinical practice. The final portion of the article is devoted to several of the more common syndromes in which nasal abnormalities are encountered.

Prenatal diagnosis of a nasal glioma in the mid trimester.

We report a case of fetal nasal glioma diagnosed at 21 weeks of gestation. The glioma appeared as a moderately hypoechoic mass arising from the junction between the medial aspect of the left orbit and the lateral aspect of the nose, and showing no internal vascularization on color and power Doppler ultrasonography. Fetal magnetic resonance imaging (MRI) excluded the possibility of an encephalocele by ruling out underlying bone defects. After an uneventful pregnancy, the nasal glioma was resected without complications at 4 months of age. The differential diagnosis of fetal paranasal facial masses is discussed.

Craniofacial dermoids: an embryological theory unifying nasal dermoid sinus cysts.

OBJECTIVE: The nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. The objective of this study was to review and reanalyze embryological hypotheses concerning NDSCs and to propose an embryological theory unifying the various anatomical characteristics of these lesions. The first case of frontal localization of a NDSC extending within the diploetic bone in a 9-month-old boy, presenting as a median frontal fistula with recurrent frontal swelling, 6 months after a mild frontal trauma is presented. RESULTS: Complete surgical removal was performed, and there was no evidence of either persistent or recurrent disease 2 years after his surgery. The embryological and anatomical origins of NDSCs are reviewed. This article reexamines and discusses major embryological theories on NDSC pathogenesis and proposes to refute the "prenasal space" theory of Grunwald and rehabilitate a forgotten embryological hypothesis, which unifies the main various clinical presentations of NDSCs.

[Aetiology of Angiofibromas: Reflection on their Specific Vascular Component]. / Die vaskuläre Komponente: Gedanken zur Entstehung des Angiofibroms.

BACKGROUND: Even though numerous theories have speculated either on the vascular or fibrous tumour component as tissue of origin, aetiology of angiofibroma still remain unclear. Histological investigations recently led Beham and coworkers to the assumption that angiofibromas have to be considered as vascular malformations. METHODS: After giving a literature review of the various theories on tumour origin the proposal to consider angiofibromas as vascular malformations is discussed on an embryological base. Taking typical clinical features of the tumour and knowledge of vasculogenesis into account a new explanation for origin of the vascular tumour component is presented. RESULTS: The vascular component of angiofibromas can be explained embryologically due to incomplete regression of the first branchial arch artery (vascular atavism). This vessel arises regularly between embryological day 22 and 24 and recedes during regular development completely until delivery via temporary formation of a vascular plexus. In the late stages of embryological development remnants of the plexus are found at the area of the sphenopalatine foramen, the typical site of angiofibroma origin. Incomplete regression of the vascular plexus of the former first branchial arch artery may form the vascular component of an angiofibroma arising due to growth stimulation at the time of adolescents. CONCLUSIONS: Incomplete regression of the first branchial arch artery presenting an atavism is suited to explain the vascular tumour component of angiofibromas considering main tumour characteristics (origin in the posterior nasal cavity close to the sphenopalatine foramen, main blood supply from the maxillary artery with possible feeders arising from the internal carotid artery). Our embryological contributions support to define angiofibromas as vascular malformations.

[New aspects in the genesis of inverted papillomas]. / Neue Aspekte zur Genese des Invertierten Papilloms.

In the last 15 years papers dealing with the inverting papilloma (IP) of the nose and the paranasal sinuses mainly described the clinical appearance and behaviour and/or discussed treatment modalities. Little is known about the aetiology of the IP. Comparisons between the histological appearance of the IP and the different stages of the embryonic development of Schneider's membrane, i.e. the lamina mucosa nasi derived from the ectoderm, show strikingly similar growth patterns. The main task of Schneider's membrane is to actively form the inner nose and the paranasal sinuses during development of the foetus. Hence, it employs the system of "asymmetric dichotomy" when growing into the embryonic tissue of the viscerocranium. As the IP originates exclusively in the area equivalent to Schneider's membrane and moreover presents some unique features which are histologically and morphologically identical with this membrane, the author believes that the embryonic determination to build up the sinuses and the meatus of the nose is responsible for the histological appearance of the inverting papilloma.

Management of the congenital midline nasal mass: a review.

The nasal encephalocele, the glioma, and the dermoid are the most common of the congenital midline nasal masses. Due to similar embryologic development, each of these lesions may be associated with bony cranial defects and intracranial abnormalities, as well as CSF leakage and the potential for fatal meningitis if not handled properly. Properative manipulation should be avoided. Radiologic studies are instructive only if they are positive. If intracranial attachments are identified radiologically or suspected clinically, neurosurgical consultation should be obtained, and intracranial exploration and resection should be carried out as the initial procedure. Extra-cranial resection of the remaining mass may be performed immediately after intracranial resection, may be postponed, or may become unnecessary.

Dermoid cysts of the nose: review of 21 cases.

Twenty-one cases of histologically confirmed dermoid cysts of the nose are reviewed. The sites involved were the dorsum (18), septum (5), glabella (2), nasal tip (2), and columella (1), with several patients having multiple sites of involvement. Bony or cartilaginous destruction occurred in 14 patients (67%). A sinus tract was evident in ten patients (48%). Complete excision of these lesions is necessary for prevention of recurrence. The differential diagnosis includes hemangiomas, gliomas, encephaloceles, epidermoid cysts, neurofibromas, and lipomas.