phenylephrine-hydrochloride and Exophthalmos

Saethre-Chotzen syndrome is an autosomal acrocephalosyndactyly syndrome whose gene has been assigned to chromosome 7p (TWIST). A case of a 13-year-old girl with Saethre-Chotzen syndrome (ACS III) is described. The features of the syndrome include: turriplagiocephaly with a cranial circumference of 52 cm, facial asymmetry, low hairline, proptosis, antimongoloid slanting of palpebral fissures, nasal deviation with high bridge, angled ears, scoliosis and torticollis, clinodactyly of the fourth and fifth toes, large halluxes, and neurosensorial hypoacusia. For correction of the deformity, a cranioorbital remodeling was performed. The craniofacial approach with remodeling of the frontal bar and reduction of the turricephaly resulted in a satisfactory morphological and functional outcome, with complete three-dimensional reshaping and remodeling of the frontonasoorbital area.

Topics: Acrocephalosyndactylia; Adolescent; Chromosomes, Human, Pair 7; Craniosynostoses; Exophthalmos; Eyelids; Facial Asymmetry; Female; Frontal Bone; Humans; Nose; Orbit; Treatment Outcome

We describe a newborn girl with a lethal sclerosing bone dysplasia leading to prenatal skeletal alterations and microcephaly, proptosis, hypoplastic nose and midface, small jaw, cleft palate, hypertrophied gums, intracranial calcifications, and generalized osteosclerosis. There is a remarkable similarity between our patient and six previously reported infants subsequently categorized as having a distinct entity: Raine syndrome. Autosomal recessive inheritance is postulated based on parental consanguinity in several of the previous cases and in our patient.

Topics: Brain Diseases; Calcinosis; Exophthalmos; Facies; Female; Genes, Recessive; Humans; Infant, Newborn; Nose; Osteosclerosis; Radiography; Syndrome

We report a patient with Antley-Bixler syndrome and review 13 patients from the literature. The cardinal features of this condition include craniosynostosis, severe mid-face hypoplasia, proptosis, choanal atresia/stenosis, frontal bossing, dysplastic ears, depressed nasal bridge, radiohumeral synostosis, long-bone fractures and femoral bowing, urogenital abnormalities and a normal karyotype. Early death was identified in 54% of the reported cases, usually due to respiratory complications. The oldest patient at the time of follow up was 10 years of age. Intellectual performance has been variable (developmental testing of our patient at 30 months of age showed a range of developmental skills equivalent to 6 to 11 months of age). Chronic respiratory distress, especially if accompanied by periods of apnea, may be important in the causation of mental retardation. Some patients with the syndrome have normal intelligence, which suggests a normally developing brain, particularly if a craniectomy is performed to treat sutural synostosis and indicates that there may be secondary factors (e.g., apnea) playing a role in the mental retardation (as seen in our patient with a history of apnea) in patients with the Antley-Bixler syndrome. Since choanal atresia/stenosis which diminishes the airway passage is a cardinal feature of this syndrome, choanal stenting should be performed on those patients with this finding during infancy to decrease the airway obstruction. All patients followed beyond infancy were ambulatory, including our patient at 35 months of age, who will take steps with assistance. Although most cases are sporadic, there were reports of recurrence in siblings of both sexes in two families, suggesting an autosomal recessive mode of inheritance.

Topics: Abnormalities, Multiple; Adult; Anal Canal; Child, Preschool; Ear; Exophthalmos; Face; Female; Femoral Fractures; Femur; Humans; Male; Nose; Skull; Syndrome; Synostosis

Venous malformation is a common tumor of the orbit, presenting with visual impairment and proptosis. These lesions can be excised via an external or transcranial approach, but recent advances in minimally invasive surgery have led to increased popularity of endoscopic approach.. The authors report a 62-year-old female presenting with diplopia and proptosis, found to have a 1.7-cm venous malformation of the orbital apex. An endoscopic trans-nasal approach was undertaken for surgical resection.. The tumor was successfully removed in a single specimen, without injury to the nearby orbital anatomy. The patient experienced resolution of her presenting symptoms 3 weeks after the procedure, with no adverse effects. A review of the literature demonstrates increasing reports of similar successful cases.. A minimally invasive, endoscopic resection of orbital venous malformation is possible and provides a reasonably safe and effective approach. Further large-scale studies are needed to fully explore its outcomes relative to traditional approaches.

Topics: Arteriovenous Malformations; Diplopia; Endoscopy; Exophthalmos; Female; Humans; Middle Aged; Nose; Orbital Neoplasms

Osteoid osteoma was first reported in 1935 by Jaffe. It is a kind of benign bone disease with the limitation of well-defined lesions. It is rarely seen in the periorbital region. The only symptom of the patient was unilateral proptosis of right. The result of MRI examination was cystic masses in the edge of the right orbit. The tumor was complete removed by the nasal endoscopic approach during the surgery. Pathology result was reported as osteoid osteoma. There was no significant complications of diplopia, visual acuity decreased, enophthalmos, cerebrospinal fluid leakage and others.

Topics: Endoscopy; Exophthalmos; Face; Humans; Magnetic Resonance Imaging; Nasal Surgical Procedures; Nose; Osteoma, Osteoid; Skull Base; Skull Base Neoplasms

Although the endonasal approach is frequently used, and the inadvertent displacement of a bone fragment into the orbital cavity is possible, no reports have yet described the overcorrection of medial orbital wall fractures using the endonasal approach. The authors report 2 patients of the overcorrection of a medial orbital wall fracture using an endonasal approach.In the first patient, a 26-year-old Chinese-Korean woman experienced a fracture of the right medial orbital wall without entrapment of the medial rectus muscle. Eleven days after the trauma, endonasal reduction was performed. Postoperative computed tomography revealed overcorrection of the medial orbital wall and lateral displacement of the medial rectus muscle. On postoperative day 19, exophthalmos of the operated side was still observed (o.d. 20 mm/o.s. 17 mm). In the second patient, a 25-year-old Korean man experienced a fracture of the left medial orbital wall without entrapment of the medial rectus muscle. Postoperative computed tomography showed overcorrection of the medial orbital wall and a laterally displaced medial rectus muscle. On postoperative day 4, exophthalmos of the operated side was observed (o.d. 23 mm/o.s. 26 mm).For fractures of the medial wall, surgery should be performed according to the recommended indications. When the endonasal approach is used to treat medial orbital wall fractures, great care is required in reducing the herniated orbital tissue and inserting the sheet to avoid overcorrection.

Topics: Adult; Exophthalmos; Female; Humans; Male; Natural Orifice Endoscopic Surgery; Nose; Orbit; Orbital Fractures; Postoperative Period; Tomography, X-Ray Computed

The objective of this study was to review the various clinical features associated with Wegener's granulomatosis (WG) in the head and neck region and to discuss the difficulty of diagnosing patients with early stage WG.. Between January 1998 and August 2007, WG was diagnosed and treated in 16 patients at the Department of Otolaryngology, Hyogo College of Medicine. Clinical and operating records of these patients were analyzed retrospectively. Diagnosis was based on the Japanese criteria proposed by the Japanese Ministry of Health and Welfare in 1998.. Ten patients (62.5%) had a definite diagnosis of WG, and the other six patients (37.5%) had a probable diagnosis of WG. The period from the onset to diagnosis was between 1 month and 30 years. The generalized form of WG was observed in three patients (18.8%), and the limited form of WG was observed in the other 13 patients (81.2%). Nasal, aural, and ophthalmic symptoms were initially presented in 10, 3, and 3 patients, respectively. Cytoplasmic pattern antineutrophil cytoplasmic antibodies (cANCAs) and perinuclear pattern ANCA (pANCA) were positively detected in 68.8% (11/16) and 27.2% (3/11) of the patients, respectively. Five of 14 patients (35.7%) had pathologic features of WG in biopsy samples from the head and neck region. Three patients in whom a diagnosis of WG was difficult are presented, and immediate lessons of our experience were discussed.. This study emphasized the difficulty of diagnosing WG, particularly at an early stage and when limited to the head and neck region. The biggest challenge faced in diagnosing WG is that it requires a high index of suspicion. When WG was suspected, we should obtain an accurate medical history from patients and repeat serologic and histopathologic examinations.

Topics: Adolescent; Adrenal Cortex Hormones; Adult; Aged; Antibodies, Antineutrophil Cytoplasmic; Blood Sedimentation; C-Reactive Protein; Cerebrospinal Fluid Otorrhea; Cranial Nerve Diseases; Dacryocystitis; Diplopia; Earache; Epistaxis; Exophthalmos; Female; Follow-Up Studies; Granulomatosis with Polyangiitis; Hearing Loss; Humans; Immunosuppressive Agents; Male; Middle Aged; Nasal Obstruction; Nose; Retrospective Studies; Scleritis; Tinnitus; Visual Acuity

Transnasal endoscopic orbital decompression is emerging as a new minimally invasive technique that avoids the need for cutaneous or gingival incision. This surgical approach appears to be a safe and effective procedure, even for the treatment of ocular disease, and it can be performed under general or local anaesthesia. The authors present a case of severe monolateral non-Graves' axial exophthalmos, accompanied by severe anisometropic myopia, which was treated successfully with functional endoscopic sinus surgery. The minimally invasive surgical procedure resulted in a marked reduction of exophthalmos without any severe complications and with minimal discomfort for the patient, who was discharged from hospital on the fourth postoperative day.

Topics: Anisometropia; Endoscopy; Exophthalmos; Female; Humans; Magnetic Resonance Imaging; Middle Aged; Myopia; Nose

To present the results of endonasal endoscopic orbital decompression in patients with Graves' ophthalmopathy.. Endonasal endoscopic orbital decompression was performed in 32 orbits of 21 patients with Graves' ophthalmopathy. In 17 patients the surgery was performed because of active ophthalmopathy non-responsive to conservative treatment, and in 4 patients for esthetic reasons. Preoperative and postoperative examination included visual acuity, examination of the eyelids and cornea, ocular motility, cover testing, Hertel exophthalmometry, and applanation tonometry.. Visual acuity improved from preoperative 0.81+/-0.28 (mean +/- standard deviation) to postoperative 0.92+/-0.21 (p=0.0032, Student t-test). Retraction of upper and lower eyelids, as well as exposure keratitis, was reduced after operation (p<0.001). Mean proptosis reduction in all orbits was 4.6+/-1.7 mm (p<0.001). An average reduction of intraocular pressure was 3.4+/-3.0 mmHg (p<0.001). New-onset diplopia developed in 8 patients. Diplopia persisted in 9 out of 11 patients who had preoperative diplopia. Two patients experienced postoperative relief of diplopia. Ocular motility was subsequently corrected by eye muscle surgery in 13 eyes, whereas prisms were used in other 5 manifestly strabic eyes.. Endonasal endoscopic orbital decompression procedure improved visual acuity, decreased proptosis and intraocular pressure, and also had favorable cosmetic results in most patients. Post decompression diplopia and strabismus were successfully managed by either eye muscle surgery or application of prisms.

Topics: Adult; Aged; Croatia; Decompression, Surgical; Diagnostic Techniques, Ophthalmological; Endoscopy; Exophthalmos; Female; Graves Disease; Hospitals, University; Humans; Male; Middle Aged; Nose; Ophthalmologic Surgical Procedures; Orbit; Treatment Outcome

Topics: Accidents, Traffic; Adult; Exophthalmos; Eye Injuries; Female; Humans; Joint Dislocations; Nose; Optic Nerve Injuries; Orbit; Wounds, Nonpenetrating

To evaluate the safety and efficacy of transnasal orbital decompression for severe Graves' ophthalmopathy.. Retrospective noncomparative case series with extended clinical follow-up.. Seventy-eight consecutive subjects who were operated on for compressive optic neuropathy with loss of visual acuity or visual field defects after failure of medical and radiation therapy.. Strictly transnasal, endoscopic-controlled bilateral decompression of the medial and inferomedial wall of the orbit.. Preoperative and postoperative examination, including vision, Hertel exophthalmometry, ocular motility, visual fields, Goldmann perimetry, and notification of complications, intranasal signs of inflammation, and subjects' assessment of the procedure.. One hundred forty-five endonasal decompressions were performed on 78 subjects (63 women, 15 men, 52.2 +/- 10.3 years) during a 10-year period. Sixty five patients were bilaterally operated on; 15 required only unilateral decompression. Four of 78 needed repeat surgery. Visual acuity increased from a preoperative average of 0.50 +/- 0.27 (range, 0.01-1.25) to 0.75 +/- 0.21 (range, 0.01-1.25) postoperatively. An average reduction of proptosis of 3.94 +/- 2.73 mm (range, -1.0-11.0 mm) was achieved with a mean preoperative Hertel measurement of 22.19 +/- 3.13 mm (range, 15-34 mm). Ocular motility was corrected by recession of the medial rectus muscle in 58 of 78 cases. Twenty-six of these 58 cases were simultaneously operated on in the same surgical session immediately after the transnasal decompression, and the others after a period of 2 to 3 months.. The transnasal orbital decompression procedure improved vision, decreased proptosis in a range comparable to more invasive techniques, and had favorable cosmetic results without additional disfiguration by scars. Morbidity was far less than with other approaches. Postdecompression strabismus was successfully managed by recession of both medial orbital muscles in the same surgical session.

Topics: Adult; Aged; Aged, 80 and over; Decompression, Surgical; Exophthalmos; Eye Movements; Female; Follow-Up Studies; Graves Disease; Humans; Male; Middle Aged; Nose; Optic Nerve Diseases; Orbit; Retrospective Studies; Safety; Visual Acuity; Visual Field Tests; Visual Fields

A clinical study of the craniofacial features in Apert syndrome is based on our experience with 136 cases. Characteristics included hyperacrobrachycephaly, steep wide forehead, flat occiput, common craniofacial asymmetry, ocular hypertelorism and proptosis, downslanting palpebral fissures, divergent upgaze and esotropic downgaze, a tendency towards large ears, and marked depression of the nasal bridge. The nose is short and wide with a bulbous tip, and the anterior facial height is reduced. Common features during infancy included horizontal grooves above the supraorbital ridges that disappear with age, a break in the continuity of the eyebrows, and a trapezoidal-shaped mouth at rest. Radiographic aspects of Apert syndrome were also assessed. Tables are provided which compare the craniofacial features of Apert and Crouzon syndromes.

Topics: Acrocephalosyndactylia; Craniofacial Dysostosis; Ear, External; Esotropia; Exophthalmos; Eyelid Diseases; Face; Facial Asymmetry; Facial Bones; Forehead; Humans; Hypertelorism; Infant; Mouth Abnormalities; Nasal Bone; Nose; Occipital Bone; Orbit; Radiography; Skull; Strabismus

Exophthalmos from Graves' disease can result in visual disability and cosmetic deformity. Surgical treatment of this disorder is now possible through an intranasal endoscopic approach that allows removal of the medial orbital wall and floor without an external incision. Endoscopic orbital decompression was performed on 22 orbits in 14 patients for treatment of progressive exophthalmos. Local anesthesia was used in five cases. Sixteen procedures involved a concurrent lateral orbital decompression performed through an external approach. There were no intraoperative or postoperative complications. Visual acuity remained stable or improved in all cases. Proptosis was reduced an average of 3.2 +/- 1.1 mm (range 2 to 4.5 mm) by endoscopic decompression alone. When a lateral decompression was also performed, proptosis was reduced by an additional 2.4 mm, for an average improvement of 5.6 +/- 1.7 mm (range 2 to 8 mm). Endoscopic orbital decompression appears to be a safe technique for the treatment of exophthalmos that can be performed effectively with the patient under general or local anesthesia.

Topics: Adult; Aged; Aged, 80 and over; Curettage; Diplopia; Dissection; Endoscopy; Ethmoid Bone; Exophthalmos; Female; Follow-Up Studies; Graves Disease; Humans; Male; Maxilla; Middle Aged; Nose; Orbit; Osteotomy; Sphenoid Bone; Sphenoid Sinus; Visual Acuity

The chronologic changes in eyeball position in relation to bony landmarks were studied by CT scans. The results were applied to the preoperative evaluation in 16 craniofacial synostosis patients. Ten subjects from each age group were selected from more than 2000 subjects who underwent CT scanning of the skull without any brain and skull diseases. These age groups ranged from birth to 20 years of age. The following four distances were measured on the CT section incorporating lenses: (1) the distance from the dorsum sellae to the lenses, (2) the distance between the lateral orbital rim and the lenses, (3) the distance from the nasal root to the lenses, and (4) the width between the lenses. These distances also were measured in 16 craniofacial synostosis patients preoperatively, and the results were compared with those of the healthy individuals in the same age group. The distances of the lenses from the nasal root and from the lateral orbital rim were larger than in the healthy individuals in almost all craniofacial synostosis patients. However, the distance from the dorsum sellae to lenses was the same as that in healthy individuals on the whole.

Topics: Adolescent; Adult; Aging; Cephalometry; Child; Child, Preschool; Exophthalmos; Eye; Eye Abnormalities; Facial Bones; Humans; Infant; Lens, Crystalline; Nose; Orbit; Reference Values

Malignant endocrine exophthalmos is a complication seen in 1-2% of patients treated for thyrotoxicosis. A variety of surgical techniques have been used to prevent loss of vision due to compression of the optic nerve. In this study, nine patients with rapidly diminishing vision were operated upon using a lateral rhinotomy technique creating the largest possible space for expansion into the nose and decompressing the optic nerve far back in the orbit. In all patients the vision was improved 1 year after surgery and the proptosis was decreased.

Topics: Adult; Aged; Exophthalmos; Female; Humans; Male; Middle Aged; Nose; Orbit

Topics: Child; Diabetes Mellitus, Type 1; Exophthalmos; Female; Granulomatosis with Polyangiitis; Hematuria; Humans; Nose; Proteinuria

Topics: Blepharoptosis; Dura Mater; Emphysema; Exophthalmos; Eye Injuries; Facial Injuries; Humans; Interprofessional Relations; Nose; Orbit; Paranasal Sinuses; Skull Fractures; Vision Disorders

Topics: Abscess; Animals; Arteries; Brain; Brain Abscess; Cattle; Cattle Diseases; Corynebacterium Infections; Corynebacterium pyogenes; Exophthalmos; Lung; Male; Nose; Orbit

Topics: Exophthalmos; Humans; Nasal Polyps; Nasopharyngeal Neoplasms; Nose

Topics: Exophthalmos; Humans; Nose; Nose Diseases