phenylephrine-hydrochloride and Neurofibromatosis-1

This paper discusses clinical features and management aspects of an extremely rare entity, neurofibroma of the external nose.. Database searches were performed using PubMed, Embase and Google Scholar to identify previously published articles.. Twelve articles comprising 13 patients with neurofibroma of the external nose were included. The mean age of presentation was 31 years. Sixty-nine per cent of patients were diagnosed at final histopathology. External approach rhinoplasty was performed in 76.9 per cent of patients, while the intranasal approach was used in 15.3 per cent of patients. There was a 15.3 per cent association with neurofibromatosis type 1. Recurrence was noted in 23 per cent of patients.. It may be challenging to diagnose this entity clinically because of its rarity and striking features on histopathology. Neurofibroma of the external nose should be kept in mind as a differential diagnosis for any soft progressive swelling over the nose. Management requires complete excision, with cosmesis and restoration of functions.

Topics: Adult; Humans; Neurofibroma; Neurofibromatosis 1; Nose; Nose Neoplasms; Rhinoplasty

Although free flap transfer is commonly performed to reconstruct facial defects, the submandibular facial artery and vein have historically been considered as adequate recipient vessels for microsurgical reconstruction. If the vascular pedicles of the free flap are short, vein grafts are necessary. The purpose of this study was to determine the indications for and effectiveness of using the nasolabial facial vessels for midfacial reconstruction. A retrospective chart review of 6 patients undergoing microsurgical reconstruction for defects of the face revealed 6 free tissue transfers in which the nasolabial facial artery and vein were considered for use as recipient vessels. Flap success rates were evaluated. Six patients (5 men and 1 woman) underwent 6 free flap transfers. Five anterior helix free flaps were used for the reconstruction of defects in the lower third of the nose. Nasal defects were due to trauma in 4 patients and squamous cell carcinoma in 1 patient. In 1 neurofibromatosis type 1 case, a radial forearm flap was used for reconstruction of the left orbital defect. The facial artery and vein in the nasolabial fold were used as the recipient artery and vein in every case. The mean length of follow-up was 5.8 years. All flaps survived. All patients were satisfied with the degree of aesthetic improvement after surgery.Use of the facial artery and vein in the nasolabial fold for facial reconstruction is reliable and safe. The nasolabial facial artery and vein should be considered as primary recipient vessels in microsurgical reconstruction of the midface.

Topics: Adult; Esthetics; Face; Female; Humans; Male; Microsurgery; Middle Aged; Neurofibromatosis 1; Nose; Nose Neoplasms; Orbit; Plastic Surgery Procedures; Retrospective Studies; Rhinoplasty; Surgical Flaps; Treatment Outcome

This disease was first described by von Recklinghausen and Festscher and has been known as the von Recklinghausen's disease or neurofibromatosis (NF). Numerous articles have been published on this subject where majority of the authors have stressed their difficulty in achieving favourable surgical results. This disease is pathologically accepted to be of neuroectodermal origin with a positive family history in approximately 50% of the cases, autosomal dominant trait and is known to involve the periorbital regions, orbit (preoperative CT scan: upper left and right photographs), temporal region to a variable extent, mid-facial region to the mandibular region. The indicated treatment for this disease is surgery including cranio-maxillofacial surgery even though re-evaluation of the conventional methods of surgery should be considered. This particular case is an extremely difficult and challenging case for any reconstructive plastic surgeon and required extensive preoperative planning. The systematic multiple stage surgical approach for an extremely severe von Recklinghausen's disease, elephantiasis neurofibromatosa is presented and discussed in detail.

Topics: Anesthesia, General; Anesthesia, Local; Cheek; Eyebrows; Face; Facial Neoplasms; Female; Humans; Lip; Middle Aged; Neurofibroma, Plexiform; Neurofibromatosis 1; Nose; Orbit; Plastic Surgery Procedures; Preoperative Care; Tomography, X-Ray Computed; Treatment Outcome

Release of high molecular weight-neutrophil chemotactic activity from human tissues, cells and secretion was studied in vivo and in vitro. Lung, nasal turbinate, nasal polyps, skin of neurofibromatosis, basophils from chronic myeloid leukemia and cultured basophilic cells from cord blood released this mediator following calcium ionophore, antigen, anti-IgE or homogenization in vitro. Its release was also demonstrated in human nasal secretions from patients with allergic rhinitis following antigen challenge. Regarding mononuclear cells no release of this mediator was observed from normal donors or asthmatic patients having no active attack upon challenge with calcium ionophore, phytohemagglutinin or antigen. Homogenized duodenum released high molecular weight-neutrophil chemotactic activity but less activity in comparison with other tissues or cells mentioned above.

Topics: Asthma; Basophils; Chemotactic Factors; Chemotaxis, Leukocyte; Duodenum; Humans; Leukocytes, Mononuclear; Lung; Nasal Polyps; Nasal Provocation Tests; Neurofibromatosis 1; Neutrophils; Nose; Rhinitis, Allergic, Perennial

Topics: Child; Female; Humans; Neurofibromatosis 1; Nose; Skin Neoplasms; Spherocytosis, Hereditary

Topics: Eyelid Neoplasms; Humans; Medical Records; Neurilemmoma; Neurofibromatoses; Neurofibromatosis 1; Nose