Bilateral Transient Dilated and Fixed Pupils After Microvascular Decompression: Rare Clinical Experience.

Microvascular decompression (MVD) has a satisfactory safety, and it is the only surgical treatment for neurovascular compression diseases, such as hemifacial spasm, trigeminal neuralgia, and glossopharyngeal neuralgia, from the perspective of etiology. Bilateral dilated and fixed pupils have long been regarded as a sign of life threatening, which is common in patients with cerebral herniation due to cranial hypertension. However, transient dilated pupils after MVD have not been previously reported. Here, we presented 2 patients with bilateral transient dilated and fixed pupils after MVD and discussed the possible etiologies through the literature review. Physical examination of both patients showed bilateral pupils were normal and without a medical history of pupil dilation. They underwent MVD under general anesthesia and used propofol and sevoflurane. In both cases, the vertebral artery was displaced, and Teflon pads were inserted between the vertebral artery and the brain stem. Postoperation, we found transient bilateral mydriasis without light reflection in both patients. The emergency head computed tomography revealed no obvious signs of hemorrhage and cerebral herniation. About 1 hour later, this phenomenon disappeared. Therefore, the authors think if MVD is successfully carried out, bilateral transient mydriasis may not necessarily indicate brain stem hemorrhage, cerebral herniation, and other emergency conditions, which can be recovered within a short time. The causes could be related to stimulation of the sympathetic pathway in the brain stem during MVD and side effects of anesthetics.

Pourfour du Petit Syndrome: A Rare Association With Cluster Headache.

Pourfour du Petit syndrome is an uncommon cause of eyelid retraction, associated with unilateral mydriasis and hemifacial hyperhidrosis. This syndrome is caused by hyperactivity of the ipsilateral oculosympathetic pathway and needs to be recognized because it has an opposite clinical presentation but the same topographic and diagnostic value as Horner syndrome. The authors report a rare case of Pourfour du Petit syndrome associated with cluster headache and discuss pathophysiological hypotheses, clinical presentation, complementary exams, pharmacologic testing, treatment options, and prognosis. Early detection of these symptoms may lead to swift diagnosis and treatment.

An uncommon case of intermittent Pourfour du Petit Syndrome associated with acute angle-closure glaucoma successfully treated by laser iridotomy.

PURPOSE: To describe a case of acute angle-closure glaucoma secondary to intermittent mydriasis related to Pourfour du Petit Syndrome caused by tracheal deviation. MATERIALS AND METHODS: A 70-year-old Caucasian woman visited the Emergency Room of the University Eye Clinic complaining of blurring of vision and difficulty to move superior eyelid in her right eye. Examination revealed reactive mydriasis, and upper lid retraction on the right side. The rest of the ophthalmological examination was normal, and a cranial computed tomography (CT) did not identify any abnormalities. A cervical CT showed the presence of an accentuated lateral right convex deviation of the trachea, attributable to a fibrothorax. A right Pourfour du Petit syndrome was suspected. Although the mydriasis had in the meantime vanished, the patient was admitted to the Neurological Clinic. RESULTS: Five days later she suffered acute pain in her right eye. Ophthalmological examination of the right eye revealed conjunctival hyperemia, marked corneal edema, reduced depth of anterior chamber, permanent mydriasis. As assessed by Goldmann applanation tonometry, intraocular pressure (IOP) was 48 mm Hg. Fundus examination was normal in both eyes. Gonioscopy revealed angle closure in all quadrants. Slit lamp examination of the contralateral eye was normal; IOP was 10 mm Hg. After hypotensive medical therapy, iridotomy with YAG laser was performed. Thereafter, IOP stabilized at 12 mm Hg. CONCLUSIONS: This is the first report in the literature of a case of acute angle-closure glaucoma secondary to mydriasis related to Pourfour du Petit Syndrome caused by tracheal deviation.

A case of recurrent facial pain associated with a Pourfour du Petit syndrome: a new entity?

We report the case of a 38-year-old woman with a history of migraine who experienced an association of recurrent unilateral facial pain and Pourfour du Petit syndrome. The episodes occurred for between a few seconds and up to 3 minutes up to 6 times a day mimicking short-lasting unilateral neuralgiform headaches with cranial autonomic symptoms. No lesional cause was found and the use of topiramate led to a nearly complete disappearance of the episodes. This new entity raises the question of a novel autonomic dysfunction in short-lasting unilateral neuralgiform headaches with cranial autonomic symptoms or an unexpected presentation of migraine.

Congenital mydriasis associated with megacystis microcolon intestinal hypoperistalsis syndrome.

We report a case of congenital mydriasis in a neonate with megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). Pilocarpine testing and gastrointestinal pathology in our patient suggest that the mydriasis is due to an underlying smooth muscle myopathy of the iris sphincter muscle. These findings may have important implications regarding the pathogenesis of MMIHS.

Pharmacological management of intra-operative miosis during cataract surgery.

Cataract surgery requires a well-dilated and stable pupil for a good outcome. Unexpected pupillary constriction during surgery increases the risk of complication. This problem is more pronounced in children. There are now pharmacological interventions that help tackle this unforeseen happening. Our review discusses the simple and quick options available to a cataract surgeon when faced with this dilemma. As cataract surgical techniques continue to improvise and get faster, an adequate pupil size is of paramount importance. Various topical and intra-cameral drugs are used in combination to achieve mydriasis. Despite good pre-operative dilation, the pupil can be quite unpredictable during surgery. Intra-operative miosis limits the field of surgery and increases the risk of complications. For example, if the pupil size decreases from 7 mm to 6 mm, this 1 mm change in pupil diameter will lead to a decrease of 10.2 mm2 in the area of surgical field. Making a good capsulorhexis with a small pupil can be a challenge, even for an experienced surgeon. Repeated touching of the iris increases the risk of fibrinous complications. Removal of cataract and the cortical matter becomes increasingly difficult. Intra-ocular lens implantation in the bag also requires adequate dilation. When dealing with challenging cases like lens subluxation, pseudo-exfoliation, and zonular dehiscence, a small pupil further increases the risk and adversely affects the surgical outcome. Hence, achieving and maintaining adequate mydriasis throughout surgery is essential. This review highlights the risk factors for small pupils during surgery and current management strategies.

Pourfour du Petit syndrome associated with a cervical vertebral anomaly.

Pourfour du Petit syndrome is a rare dysautonomic disorder characterized by mydriasis, eyelid retraction, and hyperhidrosis and is caused by irritative stimulation of the sympathetic cervical chain. The authors describe a 45-year-old woman with iris heterochromia, who presented with episodes of ipsilateral mydriasis and hyperhidrosis and was found to have a cervical vertebral anomaly, probably present since birth, as the cause of Pourfour du Petit syndrome.

Risk factors for intraocular pressure elevation during the early period post cataract surgery.

PURPOSE: To assess the risk factors for intraocular pressure (IOP) elevation during the early period post cataract surgery. STUDY DESIGN: Retrospective study. METHODS: This study involved 1587 eyes that underwent cataract surgery at the Baptist Eye Institute, Kyoto, Japan between April 2020 and May 2021. In all subjects, risk factors for early postoperative IOP elevation (i.e., an increase of IOP of 10 mmHg or more at 1-day postoperative compared with that at baseline, or a postoperative IOP of 28 mmHg or more) were analyzed by multivariate logistic regression analysis. RESULTS: Of the 1587 treated eyes in this study, 100 (6.3%) experienced early-postoperative IOP elevation. Of those 100 eyes, 78.0% were men, 27.0% had an axial length (AL) of ≥ 26.5 mm, 23.0% had a history of glaucoma treatment, 11.0% had poor mydriasis and 10.0% had intraoperative floppy iris syndrome (IFIS). Multivariate analysis findings revealed that male [odds ratio (OR) 4.36; 95% confidence interval (CI) 2.63-7.23; P < 0.001], AL of ≥ 26.5 mm (3.11; 1.83-5.30; P < 0.001), a history of glaucoma treatment (2.83; 1.63-4.91; P < 0.001), poorly mydriasis (2.63; 1.16-6.01; P = 0.02), IFIS (4.37; 1.78-10.74; P = 0.001) and baseline high IOP (1.09; 1.01-1.18; P = 0.03) were associated with increased IOP during the early period post cataract surgery. CONCLUSIONS: The findings in this study reveal that male sex, high myopia, a history of glaucoma treatment, poor mydriasis, IFIS and baseline high IOP are risk factors for IOP elevation during the early period post cataract surgery.

Anisocoria and mydriasis after scalp nerve block: a case report.

Strategies for the assessment of abnormal neurological findings during general anesthesia are limited. However, pupil abnormalities may represent serious neurological complications. We herein present a case of new-onset anisocoria and mydriasis that developed after scalp nerve block. The patient's signs were possibly related to increased intracranial pressure with resulting brain shift that ultimately affected the oculomotor nerves. A 45-year-old man was scheduled for left cerebellar tumor resection and ventricular drainage surgery; however, anisocoria and left pupillary mydriasis were observed after induction of general anesthesia and performance of scalp nerve block. After reducing the intracranial pressure, the right pupil showed constriction (1 mm) but the left pupil was dilated (5 mm). The pupils were of similar size postoperatively. Although pupillary dilation during general anesthesia has been previously described, this is the first case in which the mydriasis was considered to have been caused by brain shift due to increased intracranial pressure after scalp nerve block. Thus, we propose this phenomenon as a new possible cause of pupillary changes. Actively monitoring this presentation intraoperatively could enable early detection of and intervention for complications, therefore improving the prognosis.

Genetic and genomic analysis of classic aniridia in Saudi Arabia.

PURPOSE: To determine the genetic and genomic alterations underlying classic aniridia in Saudi Arabia, a region with social preference for consanguineous marriage. METHODS: Prospective study of consecutive patients referred to a pediatric ophthalmologist in Saudi Arabia (2005-2009). All patients had paired box gene 6 (PAX6) analysis (sequencing and multiplex ligation-dependent probe amplification analysis if sequencing was normal). If PAX6 analysis was negative, the following were performed: candidate gene sequencing (forkhead box C1 [FOXC1], paired-like homeodomain transcription factor 2 [PITX2], cytochrome P450, family 1, subfamily B [CYP1B1], paired-like homeodomain transcription factor 3 [PITX3], and v-maf avian musculoaponeurotic fibrosarcoma oncogene homolog [MAF]) and molecular karyotyping by array competitive genomic hybridization (250K single nucleotide polymorphism (SNP) arrays). RESULTS: All 12 probands (4 months-25 years of age; four boys and eight girls) had lens opacity and foveal hypoplasia in addition to no grossly visible iris. Four cases were familial. All cases were products of consanguineous unions except for three, one of which was endogamous. Heterozygous PAX6 mutations (including two novel mutations) were detectable in all but two cases, both of which were sporadic. In one of these two cases, the phenotype segregated with homozygosity for a previously-reported pathogenic missense FOXC1 variant (p.P297S) when homozygosity for chromosome 11q24.2 deletion (chr11:125,001,547-125,215,177 [rs114259885; rs112291840]) was also present. In the other, no genetic or genomic abnormalities were found. CONCLUSIONS: The classic aniridia phenotype in Saudi Arabia is typically caused by heterozygous PAX6 mutations, even in the setting of enhanced homozygosity from recent shared parental ancestry. For PAX6-negative cases, interaction between missense variation in an anterior segment developmental gene and copy number variation elsewhere in the genome may be a potential mechanism for the phenotype.

Ciliary ganglioplegic migraine: migraine-related prolonged mydriasis.

BACKGROUND: Transient, unilateral mydriasis has been reported in migraine patients, and this has been regarded as a possible co-morbidity between migraine and Adie's tonic pupil. Mydriasis that outlasts the duration of migraine attacks is rare. METHODS: Through an eight-year period we have studied all patients referred to our neurological clinic because of migraine attack with mydriasis. All the patients underwent thorough neurologic and ophthalmologic examinations including MRI of the brain and testing of the pupil response to ocular instillation of dilute pilocarpine (0.125%). RESULTS: Seven women and two men, with a mean age of 33.8 ± 12.3 years (range: 19-52) were included. The patients presented during one hemicranial migraine attack with an ipsilateral mydriasis that persisted for a mean of three months, while migraine headaches remained with their typical episodic course. In all the patients a cholinergic supersensitivity in the symptomatic pupil was demonstrated, thus pointing to a dysfunction of the ipsilateral ganglionic parasympathetic fibers. CONCLUSIONS: Synchronous co-localization of the features suggests a pathogenic link between the pupil dysfunction and migraine, rather than a simultaneous coexistence of two independent disorders. Likely explanations include a latent Adie's pupil that could have been triggered during a particular migraine attack; a ciliar ganglionic lesion/dysfunction produced by the migrainous process; an ophthalmoplegic migraine with selective parasympathycoparesis; or an episodic ciliar ganglionitis with migrainous features. Ciliary ganglioplegic migraine is proposed as a nominal term pointing to the possible anatomic source of the migrainous-related pupil dysfunction; the pathogenesis remains unknown.

Pigment dispersion syndrome and pigmentary glaucoma--a major review.

Pigment dispersion syndrome (PDS) is an interesting condition that can lead to secondary open angle glaucoma. Pigmentary glaucoma is primarily a disease of young people, myopes and men. PDS is characterized by the presence of Krukenberg spindles, iris trans-illumination defects, trabecular meshwork pigmentation and backward bowing of the iris. Posterior bowing of the iris causes rubbing of the pigmented iris epithelium against lens structures, liberation of pigment and trabecular meshwork changes that result in reduced aqueous outflow with the risk of glaucoma. Peripheral laser iridotomy can reverse backward bowing of the iris and may prevent progression of pigmentary glaucoma.

Benign Episodic Unilateral Mydriasis in a Flight Nurse.

BACKGROUND: Benign episodic unilateral mydriasis is one cause of anisocoria. This phenomenon is thought to be related to an imbalance between the sympathetic and parasympathetic nervous systems. There is a documented association with migraines, but asymptomatic cases have also been reported. A challenge with all cases is the level of investigation required to exclude more sinister causes of nervous system dysfunction. In a dynamic flight environment, additional considerations need to be made, such as varying light levels and use of night vision devices. CASE REPORT: A 27-yr-old woman on deployment to Afghanistan as a flight nurse presented to the role one clinic with right-sided mydriasis. The patient denied headache or any history of migraines. A dilated right pupil that was reactive to light was found on exam. Symptoms and exam findings resolved shortly after initial presentation. We consulted an ophthalmologist who requested patient transfer for review. He made a diagnosis of benign episodic unilateral mydriasis. DISCUSSION: There are a variety of causes for anisocoria. A thorough history and examination are required to avoid unnecessary investigations that may not be locally available in the more austere deployed military settings. From an operational perspective, the decision needs to be made regarding the maintenance of flight status. Consideration needs to be given to patient care capability when treating a flight nurse. In cases of rapid resolution such as this, removal from operational status is not reasonable should a clinician be confident of the diagnosis.Schiemer A. Benign episodic unilateral mydriasis in a flight nurse. Aerosp Med Hum Perform. 2017; 88(5):500-502.

Recurrent Painful Ophthalmoplegic Neuropathy with Residual Mydriasis in an Adult: Should it Be Classified as Ophthalmoplegic Migraine?

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare condition that manifests as headache and ophthalmoplegia. It typically occurs in children. Although migraine or neuropathy have been suggested as etiologies, the precise etiology remains unclear. In the International Classification of Headache Disorders 3rd edition-beta version (ICHD3ß) (code 13.9), RPON was categorized into painful cranial neuropathies and other facial pains. We encountered a 48-year-old woman who had diplopia and right ptosis. The administration of prednisolone led to the immediate improvement of her oculomotor palsy, but residual mydriasis remained. Based on this case, the pathophysiology of RPON may involve temporary nerve inflammation with migraine. Repeated and severe migraine attacks may cause irreversible nerve damage. Thus, medication for migraine prophylaxis might be needed to prevent RPON.

A case of Horner syndrome with intermittent mydriasis in a patient with hypoplasia of the internal carotid artery.

We report a rare case of hypoplasia of the right internal carotid artery (ICA) with ipsilateral congenital Horner syndrome. The etiology and pathogenesis of hypoplasia of the ICA is not well understood. Multiple types of collateral flow have been reported to develop to maintain blood supply to the ipsilateral cerebral hemisphere. Although collateral flow may allow these patients to remain asymptomatic, we postulate that the enlarged posterior communicating artery (PcomA) in our patient caused mass effect on the cisternal segment of cranial nerve III causing intermittent mydriasis apart from Horner syndrome.

Unilateral hyperhydrosis in Pourfour du Petit syndrome.

Upper limp hyperhydrosis is an idiopathic disease with bilateral involvement. However, Pourfour du Petit syndrome, the opposite of Horner syndrome, may result in unilateral upper limb hyperhydrosis. It occurs following hyperactivity of the sympathetic cervical chain as a consequence of irritation secondary to trauma. We report herein two cases with Pourfour du Petit syndrome showing unilateral upper limb hyperhydrosis. The patients presented with right-sided mydriasis and ipsilateral hemifacial hyperhydrosis. The onset of disease was followed by a trauma in both patients. They underwent upper thoracic sympathectomy with favorable outcome. A history of an antecedent trauma in patients with unilateral upper limb hyperhydrosis and anisocoria may imply a possible diagnosis of Pourfour du Petit syndrome.

Paroxysmal autonomic alterations mimicking epilepsy: a case report.

A 22-year-old male patient presented with paroxysmal hyperhidrosis, mydriasis, hypertension, and tachycardia. Cranial and cervical MRI revealed focal atrophy in the high order zone of the central autonomic network and syringomyelia. His physical and neurological examinations were unremarkable. Physiological testing included EEG, SPECT, serum/urine tests and autonomic testing. A poor response was achieved with the medical and interventional procedures employed. As the central autonomic network is an integral component of the internal regulation system of the brain, any lesion, no matter where in the network, may lead to paroxysmal autonomic alterations mimicking epilepsy (Published with videosequences).