phenylephrine-hydrochloride and Blepharoptosis

Monocular elevation deficiency (MED) is a functional defect in upward eye movement caused by paralysis, restriction, or a combination of factors. The affected eye experiences limited movement in the upward, nasal, and temporal directions, often accompanied by downward deviation and ptosis. Previously known as double elevator palsy (DEP), MED can result from muscle paralysis responsible for upward movement, restriction of the inferior rectus muscle, or lesions in the supranuclear pathway. The Knapp procedure was once the most commonly used surgical approach for MED. However, in cases involving restriction of the inferior rectus muscle, a staged approach combining inferior rectus muscle weakening and subsequent Knapp procedure is necessary to avoid anterior segment ischemia. This has led to the development of alternative surgical techniques, such as single-rectus muscle transposition, partial tendon transposition, and muscle belly transposition. Differentiating MED subtypes based on the underlying paralysis or restriction etiology is crucial for determining the appropriate surgical plan. This review article summarizes the clinical features, classification, and available surgical options for MED, aiming to provide valuable insights for clinical research and treatment.. 单眼上转不足（MED）是由麻痹、限制或混合因素引起眼球上转功能缺陷，患眼向正上、鼻上和颞上方运动均受限，常伴下斜视和上睑下垂，既往又称为双上转肌麻痹（DEP）。除了负责上转的肌肉麻痹，下直肌限制和核上性病变也可参与其发病。Knapp术曾是最常用的MED术式。但当存在下直肌限制时，需首先减弱下直肌，再分期行Knapp术以避免眼前节缺血的发生，因而促生了其他的改良手术方式，如单条外直肌或内直肌转位术、部分肌腱转位术以及肌腹转位术等。根据麻痹或限制的病因进行MED的分型鉴别，对于制定合适的手术方案是非常必要的。本文对MED的临床特征、分型以及可供选择的手术方案进行综述，以期为临床研究和治疗提供参考。.

Topics: Blepharoptosis; Eye Movements; Humans; Nose; Paralysis

Topics: Abnormalities, Multiple; Blepharoptosis; Growth Disorders; Humans; Limb Deformities, Congenital; Nose; Syndrome

An increasing number of people are undergoing non-surgical aesthetic procedures, especially injections of botulinum toxin and dermal fillers. While toxin injections have lower rates of complications, profound and serious consequences can arise with the use of dermal fillers.. A 29-year-old woman presented to the eye casualty department with sudden visual loss, ptosis and ophthalmoplegia after having had non-surgical rhinoplasty in a beauty salon in West London. The filler was administered by a healthcare professional not registered with the General Medical Council (GMC) or similar governing body.. Despite prompt measures on arrival at our service, the symptoms of visual loss, ptosis and ophthalmoplegia persisted. Attempts from the patient and medical services to report the incident (to trading standards and the police) were to no avail.. This case highlights the poor treatment response to filler-related ophthalmic complications. It is also evident that in the United Kingdom, there appears to be poor regulation in the use of these products, a lack of clear guidelines for the management of their complications and finally no recourse for patients to challenge practitioners who lack medical registration and are not held accountable.

Topics: Adult; Arterial Occlusive Diseases; Blepharoptosis; Blindness; Dermal Fillers; Female; Humans; Nose; Ophthalmic Artery; Ophthalmoplegia; Rhinoplasty; Visual Acuity

Ptosis of the nasal tip is one of the most challenging aspects of rhinoplasty, and the tongue-in-groove (TIG) and the columellar strut (CST) techniques are the two most common procedures used to improve it. The purpose of this study was to introduce a new modification of TIG, to evaluate the long term results of the modified TIG, and to compare its effect on correction of the nasolabial angle with that of the CST. Eighty patients with droopy nasal tips (all female, aged <40 years) were divided into two groups of 40 each according to the technique used for correction (modified TIG or CST). The nasolabial angle was recorded preoperatively, immediately postoperatively, and after one year. The mean was 90.80° in the modified TIG group and 94.87° in the CST preoperatively, which did not differ significantly (p=0.056). Immediately postoperatively, it was significantly higher in the CST group (116.92° compared with 107.10°, p=0.01), but at the one-year follow-up, the droop was similar in both groups (-7.35° in the modified TIG, and -7.48° in the CST group, respectively, p=0.942). Both techniques significantly increased the nasolabial angle postoperatively (p<0.001 in both cases). This study showed that the modified TIG is a reliable and effective technique for reconstructing and correcting a droopy nasal tip, and its long-term efficacy is comparable with that of CST.

Topics: Adult; Blepharoptosis; Dental Implants; Female; Humans; Nasal Septum; Nose; Rhinoplasty; Treatment Outcome

Administration of local anesthetics is daily routine for most dental practitioners. Normally, the effect is achieved, and no adverse effects are seen. In this article, the authors describe the complications of immediate, intense and shooting pain, numbness, and marked pallor of the cheek, which occurred during infiltration of a local anesthetic in buccal vestibule infiltration. The patients moved suddenly because of pain and marked pallor of the cheek near the root of the nose and lower eyelid pallor was observed. The pain was very short and the injection was performed again after a few minutes. Two patients also reported an alteration of vision or paralysis of the extra-ocular muscles and drooping eyelid due to paralysis of the levator palpebrae superioris muscle and signs of numbness in the infraorbital area on the same side as the anesthesia. While 3 patients were also apprehensive and started to scare with heart palpitations, as they did not understand what was happening. Probably the anesthetic solutions were injected into an intravascular artery and passed from the extraosseous branch of posterior superior alveolar artery through to the infraorbital artery, which could produce the clinical signs observed in the present study. At the same time, the inoculation of anesthetic in the artery could be grounds for legal disputes for the dentist. In fact, in the absence of vascular disease, anomalies documented by the dentist, they would, however, respond to professional liability and be liable for damages caused to the patient. In conclusion, despite the fact that this condition requires no treatment, it could lead to the recognition of clinical signs in patient with injection of local anesthesia into the artery. At the same time, the inoculation of anesthetic in the artery could be grounds for legal disputes for the dentist.

Topics: Aged; Anesthesia, Dental; Anesthesia, Local; Anesthetics, Local; Blepharoptosis; Cheek; Dental Implantation; Dental Implants; Eyelids; Female; Humans; Injections; Male; Middle Aged; Nose; Oculomotor Muscles; Pain

Topics: Blepharoptosis; Dacryocystorhinostomy; Female; Humans; Intubation; Lacrimal Apparatus; Middle Aged; Nose; Postoperative Complications

Topics: Adult; Blepharoptosis; Emphysema; Humans; Male; Nose; Rhinitis, Allergic, Seasonal; Sneezing

We report on a young female patient with the clinical features of blepharophimosis-ptosis-epicanthus inversus syndrome (BPES, OMIM 110100) and a balanced chromosome translocation 46, XX, t(2;3)(q33;q23)dn.BPES is a rare autosomal dominant congenital disorder characterized by the eponymous oculo-facial features that are, in female patients, associated either with (type 1 BPES) or without (type 2 BPES) premature ovarian failure. Both types of BPES are caused by heterozygous mutations in the FOXL2 gene, which is located in chromosome band 3q23. Chromosome aberrations such as balanced rearrangements have only rarely been observed in BPES patients but can provide valuable information about regulatory regions of FOXL2. The translocation in this patient broadens our knowledge of pathogenic mechanisms in BPES and highlights the importance of conventional cytogenetic investigations in patients with negative results of FOXL2 mutation screening as a prerequisite for optimal management and genetic counseling.

Topics: Blepharophimosis; Blepharoptosis; Chromosome Breakage; Chromosomes, Human, Pair 2; Chromosomes, Human, Pair 3; DNA Mutational Analysis; Female; Forkhead Box Protein L2; Forkhead Transcription Factors; Humans; In Situ Hybridization, Fluorescence; Infant; Nose; Skin Abnormalities; Syndrome; Translocation, Genetic

A group of syndromes, consisting of Malpuech syndrome, Michels syndrome, Carnevale syndrome, OSA syndrome, and Mingarelli syndrome share the combination of symptoms of highly arched eyebrows, ptosis, and hypertelorism, and vary in other symptoms such as asymmetry of the skull, eyelid, and anterior chamber anomalies, clefting of lip and palate, umbilical anomalies, and growth and cognitive development. It has been suggested that they are in fact part of the same entity. Here, we describe a brother and sister with the same constellation of symptoms, and compare these with the various entities. We conclude that the present patients resemble most patients with Carnevale and Mingarelli syndrome, and the case reported by Guion-Almeida, and that these patients form together most probably the same entity. We suggest the name Carnevale syndrome as this author described this combination of symptoms for the first time. Malpuech and Michels syndromes are probably separate entities, although they may still be allelic. Pattern of inheritance of Carnevale syndrome is most likely autosomal recessive.

Topics: Abnormalities, Multiple; Blepharoptosis; Bone Diseases; Child, Preschool; Cleft Lip; Cleft Palate; Craniofacial Abnormalities; Craniosynostoses; Eyelids; Face; Female; Genes, Recessive; Humans; Hypertelorism; Male; Nose; Radiography; Skull; Syndrome; Torticollis; Urogenital Abnormalities

To map the gene responsible for Blepharophimosis Epicanthus Inversus and Ptosis syndrome(BPES) type I in a Chinese family.. Polymorphisms of 4 satellite markers of 3q were analyzed and linkage analysis was performed using linkage software (MLINK, Version 5.2).. No deletion was detected for the four markers. The maximum lod scores were: 0.77 (theta = 0.00) at D3S3045; 3.61 (theta = 0.00) at D3S1764; 0.11 (theta = 0.3) at D3S3053 and -0.03 (theta = 0.4) at D3S2436.. The gene responsible for BPES in the Chinese family was tightly linked to D3S1764.

Topics: Abnormalities, Multiple; Blepharophimosis; Blepharoptosis; Chromosome Mapping; Genetic Linkage; Humans; Nose; Polymorphism, Genetic

Two sisters are reported with up-slanting palpebral fissures, hypertelorism, ptosis, a broad, bifid nasal tip, a high-arched palate, mental retardation, abnormal EEG and hand malformations in one of the patients. The girls' parents originate from the same village. Although the findings resemble the recently defined neurofaciodigitorenal syndrome, some findings suggest that this is a newly recognized syndrome.

Topics: Abnormalities, Multiple; Blepharoptosis; Child; Child, Preschool; Electroencephalography; Family Health; Female; Humans; Hypertelorism; Intellectual Disability; Nose; Nuclear Family; Tooth Abnormalities

We present a family with six children of first cousin parents, in which three present with microcephaly, hypertelorism, down-slanting palpebral fissures, ptosis, a broad nasal tip, a short webbed neck, mental retardation and seizures. Two differential diagnosis, the Noonan and the Baraitser-Winter syndrome are discussed. The possibility of the description of a new MCA/MR syndrome is raised.

Topics: Abnormalities, Multiple; Astigmatism; Blepharoptosis; Brain; Child; Child, Preschool; Consanguinity; Face; Female; Genes, Recessive; Humans; Hypertelorism; Intellectual Disability; Male; Neck; Nose; Nystagmus, Pathologic; Pedigree; Seizures; Syndrome

Oculopharyngeal muscular dystrophy (OPMD) in the European population has been frequently diagnosed, but except for one black family, the occurrence in other ethnic groups is uncertain. We identified two unrelated OPMD Japanese families, including 34 affected individuals. Major clinical manifestations were bilateral ptosis and dysphagia starting after age 40. Histologic studies of limb muscles revealed mild myogenic changes, occasional rimmed vacuoles, and small angulated fibers. By contrast, cricopharyngeal muscle showed a marked loss of fibers and massive proliferation of connective tissue. Intranuclear tubulofilamentous inclusions (ITFI) of 8.5 nm outer diameter were observed in 2-5% of the nuclei in four different biopsied muscles. One patient with recurrent aspirations underwent successful cricopharyngeal myotomy. Aerodynamic examination was useful to evaluate velopharyngeal closure function. Our investigations revealed that OPMD is a geographically widespread disorder, and ITFI may be the specific morphologic hallmark.

Topics: Biopsy; Blepharoptosis; Family Health; Female; Humans; Japan; Male; Microscopy, Electron; Middle Aged; Muscle, Skeletal; Muscular Dystrophies; Nose; Oculomotor Muscles; Pedigree; Pharyngeal Muscles; Pharynx; Radiography

Topics: Abnormalities, Multiple; Adult; Blepharoptosis; Chromosome Deletion; Chromosomes, Human, Pair 11; Humans; Hypertelorism; Male; Micrognathism; Nose; Syndrome

Topics: Abnormalities, Multiple; Blepharoptosis; Child, Preschool; Coloboma; Humans; Infant; Infant, Newborn; Intellectual Disability; Karyotyping; Male; Nose; Pedigree; Phenotype

Maternal and paternal age and birth order of 14 sporadic cases of the autosomal dominant blepharophimosis-ptosis-epicanthus inversus-telecanthus (BPEI) phenotype were compared to similar statistics from control individuals. Correlation coefficients were determined for paternal age and incidence, maternal age and incidence, and birth order and incidence. The partial correlation coefficient of the father's age and incidence with maternal age and birth order held constant was -0.02 and that for the mother's age and incidence with paternal age and birth order held constant was 0.57. Maternal age seems to have a stronger influence than the paternal age in this group of BPEI patients.

Topics: Abnormalities, Multiple; Adult; Blepharoptosis; Eyelids; Female; Humans; Male; Maternal Age; Nose; Paternal Age; Phenotype; Skin Abnormalities; Syndrome

The importance of skeletal reduction of the interorbital distance in the treatment of patients with teleorbitism is now well recognized. In spite of this, results of surgery are not always as good as one would hope. For this there are two reasons: (1) reduction of the interorbital distance may be followed by deformities such as canthal drift, enophthalmus, pseudoptosis, and so forth; and (2) hypertelorism is frequently associated with a variety of other malformations that become more conspicuous after reduction of the interorbital distance. In this paper attention is focused on the mechanisms responsible for the appearance of new stigmata, on their prevention, and also on the treatment of the associated malformations.

Topics: Abnormalities, Multiple; Adult; Blepharoptosis; Child; Child, Preschool; Craniofacial Dysostosis; Eye Abnormalities; Facial Asymmetry; Female; Humans; Hypertelorism; Lacrimal Apparatus; Male; Nose; Oculomotor Muscles; Orbit; Rhinoplasty; Surgery, Plastic

Converse is using the term "interorbital space" for the ethmoid bone, especially its lateral wall. We added the frontal process of the upper jaw including spongy substance ("trajector"). Pathophysiology and clinical points of view of comminuted fractures are demonstrated.

Topics: Blepharoptosis; Craniofacial Dysostosis; Ethmoid Bone; Frontal Bone; Humans; Hypertelorism; Nose; Oculomotor Muscles; Orbit; Orbital Fractures; Skull Fractures

Topics: Blepharoptosis; Ethmoid Bone; Humans; Male; Maxillofacial Injuries; Nose; Skull Fractures; Surgery, Plastic

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

Topics: Abscess; Anti-Bacterial Agents; Blepharoptosis; Blindness; Cellulitis; Conjunctiva; Cornea; Diagnosis, Differential; Edema; Ethmoid Bone; Eye Diseases; Eyelids; Face; Hypesthesia; Lacrimal Apparatus; Neuralgia; Nose; Ophthalmic Nerve; Ophthalmoplegia; Optic Neuritis; Orbit; Papilledema; Periosteum; Prednisone; Pupil; Sinus Thrombosis, Intracranial; Sinusitis

Topics: Adolescent; Biopsy; Blepharoptosis; Blood Transfusion; Female; Humans; Maxillary Sinus; Neoplasm Metastasis; Neuroectodermal Tumors, Primitive, Peripheral; Nose; Nose Neoplasms; Palate; Radiography; Skull