phenylephrine-hydrochloride and Lipoma

Pai syndrome is a rare congenital disorder first described in 1987. The main clinical features of the syndrome include median cleft of the upper lip, intra-cranial lipoma, and cutaneous polyps. Only four cases have been described previously. This is the fifth who is a twin of Arabian descent to be reported. Full description of the clinico-pathological features and a review of the relevant medical literature is presented. To the best of our knowledge, this is the first case of Pai syndrome in a twin in the English literature.

Topics: Biopsy, Needle; Brain Neoplasms; Cleft Lip; Female; Follow-Up Studies; Humans; Infant, Newborn; Lipoma; Magnetic Resonance Imaging; Nose; Polyps; Skin Neoplasms; Syndrome

Seven patients with a corpus callosum lipoma associated with a frontal cranial defect with or without a superficial lipoma or a fronto-nasal dysplasia, were encountered over a period of 12 years. This group of patients is reported as these associated lesions may provide a lead to the cause of lipomas of the corpus callosum as well as of the different lesions in the fronto-facial region. From our experience and that of others, the frontal lesion may be an extracranial lipoma, a frontal bone defect, with or without an external lipoma, a lipomeningocele connecting the extracranial lipoma through a frontal defect with the corpus callosum lipoma, a frontal encephalocele. Fronto-nasal dysplasia associated with a corpus callosum lipoma, seems to form an independent group although some of these patients may have other extracranial lesions as well. It is suggested by the authors that as the primitive mesenchyme gives rise to the meninx primitiva as well as to the fronto-facial skeleton a disturbance of the neural crest may give rise to these combined lesions. Further analysis of similar cases may indicate the timing of these events, and perhaps reveal a common causative factor.

Topics: Adult; Agenesis of Corpus Callosum; Brain Neoplasms; Child; Corpus Callosum; Encephalocele; Female; Frontal Bone; Humans; Infant; Lipoma; Magnetic Resonance Imaging; Male; Nose; Tomography, X-Ray Computed

The author presents the first reported case of a nasal angiolipoma presenting as "Pinocchio" nasal tip deformity. The two types of angiolipomas (infiltrating and non-infiltrating) are discussed as are two surgical approaches to this deformity. The author feels that the external rhinoplasty technique is an excellent way to approach this problem, especially in patients who are suffering severe emotional stress due to peer pressure.

Topics: Child; Diagnosis, Differential; Esthetics; Hemangioma; Humans; Lipoma; Male; Nose; Nose Neoplasms; Surgery, Plastic

Topics: Humans; Lipoma; Neoplasm Recurrence, Local; Nose

Topics: Female; Humans; Lipoma; Middle Aged; Nose; Rhinoplasty

Topics: Adult; Facial Asymmetry; Female; Humans; Lipoma; Nose; Nose Neoplasms

A hitherto unreported case is presented wherein a 2year old child had a 'cranial lipomeningocoel' or a 'nasal lipo-encephalocoel'. The child presented with a growing mass in the base of the nose. Investigations revealed that the nasal mass was a lipoma that was an extension of intracranial lipoma. The intracranial component extended up to corpus callosum. Resection of the extracranial extension and basal reconstruction resulted in cosmetic recovery.

Topics: Child, Preschool; Corpus Callosum; Female; Humans; Lipoma; Nose

Intramuscular lipomas are rare, benign, mesenchymal tumors occurring deep in the fascia, typically involving large muscle groups in adults. We report a case of an intramuscular lipoma occurring as a subcutaneous midline nasal mass in a 3-month-old infant. The differential diagnosis of a midline mass on the glabella of an infant is important and should include developmental anomalies such as nasal glioma, nasal dermoid cyst, and encephalocele, so neuroimaging is an essential first step in evaluating these lesions to exclude intracranial extension.

Topics: Biopsy, Needle; Dermoid Cyst; Diagnosis, Differential; Encephalocele; Follow-Up Studies; Glioma; Humans; Immunohistochemistry; Infant; Lipoma; Male; Neuroimaging; Nose; Risk Assessment; Skin Neoplasms; Subcutaneous Tissue; Tomography, X-Ray Computed; Treatment Outcome

To investigate the clinical and pathological features of solitary pterygoid benign lesions, as well as the treatment and outcome of endoscopic surgery.. We retrospectively analyzed clinical data of 4 patients with pterygoid benign lesions in our department. High resolution CT and enhanced MRI were performed before the operations, then endoscopic surgeries were carried out under the circumstance of general anesthesia. After a follow-up in 12 months to 48 months, nasal endoscopy and MRI examination were performed. Therefore we are able to understand the situation of operations and postoperative recurrences, and to inquire about the changes of symptoms and the relief of symptoms before and after surgery.. From the Pathological diagnosis, it showed 2 cases of cystic lesions, 1 case of spindle cell lipoma, 1 case of inflammatory lesion. Postoperative follow up showed scar formation, smooth surface, no recurrence, and no new symptom. Nasal obstruction is relieved after the surgery, and no changes in the sympotoms of headache were observed.. The combination of high resolution CT with enhanced MRI isimportant for ascertaining the location, extent and nature of the pterygoid lesions. Endoscopic surgery is a minimally invasive, safe, and effective method for the treatment of solitary pterygoid benign lesions.

Topics: Cysts; Endoscopy; Headache; Humans; Lipoma; Magnetic Resonance Imaging; Nasal Obstruction; Nasal Surgical Procedures; Neoplasm Recurrence, Local; Nose; Postoperative Period; Retrospective Studies; Tomography, X-Ray Computed

A 19-year-old woman was referred for nasal breathing and aesthetic concerns regarding her nose. A computed tomography scan revealed a massive osseous shield anterior to the piriform aperture. Furthermore, there was a submucosal median alveolar cleft, and the posterior arch of C1 was missing. The magnetic resonance imaging brain scan revealed a curvilinear lipoma of corpus callosum. The ectopic nasal bone was removed by open rhinoplast,y and nasal function and aesthetics were restored. The described features defy conventional clinical diagnosis and severity classifications and present a diagnostic conundrum somewhere between a mild form of frontonasal dysplasia, oculoauriculofrontonasal syndrome, and Pai syndrome.

Topics: Abnormalities, Multiple; Adolescent; Cervical Vertebrae; Cleft Palate; Corpus Callosum; Diagnosis, Differential; Female; Humans; Lipoma; Magnetic Resonance Imaging; Nose; Phenotype; Tomography, X-Ray Computed

We report on two unrelated Brazilian boys with craniofacial anomalies that involve the frontonasal process and the first branchial arch associated with pericallosal lipoma. To our knowledge this condition seems to have been reported only once previously, but may represent a new condition within the group of the frontonasal dysgenesis. Clinical and imaging data, phenotypic evolution, and differential diagnosis are discussed.

Topics: Adult; Branchial Region; Craniofacial Abnormalities; Female; Frontal Bone; Humans; Infant, Newborn; Lipoma; Male; Nose; Young Adult

A 17-year-old previously unreported patient with Pai syndrome is described. The boy had median cleft of upper lip, a polypoid skin mass over the columella, a minimal cleft of the upper central incisors, frontal alopecia of the anterior hairline, and bifid nose. Magnetic resonance imaging showed pericallosal lipoma. No mental retardation was present, and a chromosomal study showed normal male 46, XY karyotype.

Topics: Adolescent; Alopecia; Brain Neoplasms; Cleft Lip; Corpus Callosum; Humans; Lipoma; Magnetic Resonance Imaging; Male; Nasal Polyps; Nose; Syndrome

Topics: Abnormalities, Multiple; Brain Neoplasms; Child; Cleft Lip; Cleft Palate; Corpus Callosum; Humans; Hypertelorism; Lipoma; Male; Nose; Tomography, X-Ray Computed

We report 3 patients with isolated cleft or coloboma of the nose--an extremely rare occurrence. This entity belongs to no. 1 of Tessier's classification of craniofacial clefts. Excessive separation of the medial and lateral nasal processes of the frontonasal process may be the genesis of this condition. Management should address two points: the cleft and the associated malformations of the cartilaginous nasal framework.

Topics: Adult; Child; Child, Preschool; Female; Follow-Up Studies; Humans; Infant; Lipoma; Male; Nose; Reoperation; Rhinoplasty; Soft Tissue Neoplasms; Surgical Flaps

An autosomal dominant dysplasia-malformation syndrome affecting seven individuals in one family is reported. The components of the syndrome include congenital nasopalpebral lipoma, telecanthus, and bilateral colobomas of upper and lower lids without midface hypoplasia. It appears to be the second recorded example resulting from an autosomal dominant gene fully penetrant in both sexes.

Topics: Child, Preschool; Coloboma; Eyelids; Humans; Lipoma; Male; Nose; Pedigree; Syndrome

A longitudinal study was performed of eight patients with fronto-nasal dysplasia. The follow-up exceeded 15 years in some patients. The eight cases showed lipoma of the corpus callosum. The only clinical alterations observed were cosmetic ones. No neurological abnormality was found, although their intellectual level was just within normal limits, the average IQ being 95, ranging from 82-103. No relationship between the IQ and the type of facies or the size of the lipoma was found. All the children presented psychological alterations, especially misanthropy and shyness. The lipoma was diagnosed by computerised tomography. Two otherwise unidentifiable small lipomas were found with this technique. Pneumoence-phalography and carotid arteriography, which had been performed on nearly all the patients before CT scanning, had demonstrated some alterations. These anomalies slightly deformed the anterior portion of the lateral ventricles and anterior cerebral arteries, but a lipoma in the corpus callosum had not been considered. Only in one case was hypogenesis of the corpus callosum clearly demonstrated. The presence of an extra-osseous lipoma on the forehead and of a vertical bony bar in the intracranial midline in contact with the frontal bone are definite signs of the presence of a lipoma in the corpus callosum. The lipoma is in anterior contact with the vertical bony bar. The neurological alterations presented by the patients in this series are minor compared with those described by other authors writing of children with lipoma of the corpus callosum without FND. Because of mild clinical alteration in these children we have not considered removing the lipoma.

Topics: Brain Neoplasms; Child; Child, Preschool; Corpus Callosum; Female; Frontal Sinus; Humans; Infant; Infant, Newborn; Lipoma; Male; Nose; Radiography

Topics: Humans; Lipoma; Neoplasms; Nose; Nose Neoplasms