Double-bellied inferior rectus muscles with aplasia of medial rectus muscles.

This report describes a rare occurrence of double-bellied inferior rectus muscle with aplasia of the medial rectus muscle in both eyes of a young woman seeking cosmetic correction for her strabismus. The patient presented with a large-angle exotropia and a -4 limitation of adduction in each eye. On attempted adduction, there was minimal downshoot and upper eyelid retraction. Magnetic resonance imaging of the orbit revealed hypoplastic medial rectus muscles; however, intraoperatively the muscle was not found at the insertion or when traced back as far as possible. The inferior rectus muscles were noted to have double bellies converging close to each other at the insertion. The patient underwent a bilateral lateral rectus recession with nasal transposition of the inferior rectus muscles and was cosmetically well aligned, with a residual exotropia of 4Δ at 3 months' follow-up. This unusual anatomical variation may be the result of deviation of muscle paths.

Modified Nishida's procedure in a case of medial rectus hypoplasia.

Introduction: We report a case of large angle exotropia in a child with limitation of adduction in the left eye with a radiological finding of hypoplastic medial rectus. Methods: A 3- year- old male child presented with left eye large angle exotropia, left face turn, -4 adduction limitation and severe amblyopia. Orbital imaging revealed hypoplasia of the medial rectus and intraoperatively a thin medial rectus was noted. The surgical procedure planned was lateral rectus recession combined with Modified Nishida's technique in the left eye. In this technique the superior and inferior recti were transposed medially by inserting non-absorbable sutures in the sclera posteriorly, closer to the upper and lower borders of the medial rectus muscle. Result: There was improvement in adduction of left eye and reduction of original deviation following maximal lateral rectus recession and a modified Nishida's approach. The early and optimal correction of exotropia also improved the compliance to patching with subsequent gain in visual acuity of the amblyopic eye. Conclusion: Modified Nishida's technique has the advantage of no muscle splitting and no tenotomy, remains a less invasive surgical procedure to correct large deviations. The modification of placing the bellies closer to medial rectus augments the effect and further improves adduction in cases with severe limitation of adduction. This technique can thus be considered as a possible surgical approach in young children with large angle exotropia due to hypoplastic medial rectus.

Supernumerary Extraocular Muscle: A Rare Cause of Atypical Restrictive Strabismus.

Background and objectives: Supernumerary extraocular muscle (SEOM) is extremely rare. The purpose of this paper was to review the clinical characteristics and surgical outcomes of SEOM patients with atypical restrictive strabismus. Materials and Methods: A retrospective review was conducted on the data from 12 SEOM cases. Pre- and post-operative measurements consisted of visual acuity, cycloplegic refraction, ocular alignment, ocular motility, binocular vision, and imaging. Management strategies included either conservative or surgical treatments. Results: Of the 12 cases reviewed (seven females, five males), the mean ± SD age was 14.3 ± 10.6 years (range: 4-38 years). The right eye was affected in six cases, the left in five, and both eyes in one case. The major clinical manifestations included restrictive ocular motility (12 cases), with seven cases in no less than three directions; varying degrees of horizontal or vertical strabismus; ipsilateral amblyopia (10 cases); and unequal palpebral aperture (10 cases). Imaging results revealed muscular bands originating from the annulus of Zinn and insertion into the globe or other recti, as well as anomalous muscular bands connecting two or more recti, sometimes with optic nerve involvement. Three patients received conservative treatment, while rectus recession with or without resection (seven patients) or rectus disinsertion plus globe fixation (two patients) were performed in those receiving surgical treatments. A surgical success was achieved in four cases. Conclusions: For restrictive strabismus, imaging plays an important role in the diagnosis of SEOM. When the SEOM is difficult to resect, a personalized surgical strategy may be required to achieve a good ocular alignment.

Nasal insertion of the superior oblique tendon presenting as Brown syndrome.

BACKGROUND: Anomalous ocular muscle insertions are a rare cause of ocular motility disturbances. METHODS: We report the clinical presentation and the intraoperative findings of two cases with an abnormally nasally inserted superior oblique tendons presenting with a Brown syndrome-like clinical picture. RESULTS: Case no 1 was a 5-year-old girl presenting with a chin up position. There was bilateral limitation of elevation in adduction, -4 on the right side and -3 on the left side with +1 downshoot on adduction on either side Patient was orthotropic in down-gaze with small V-pattern exotropia. Case no 2 was a 4-year-old boy presenting with an esotropia of 35Δ that was partially corrected with his spectacles to 20Δ. Ductions showed -4 defective elevation in adduction of the right eye. Surgical exploration in both cases revealed abnormal nasal insertion of the superior oblique tendons. The line of insertion had a convexity facing superonasally. The posterior fibers were inserted 7-8 mm posterior and just nasal to the nasal border of the superior rectus insertion, while the anterior fibers were shorter and inserted 5 mm nasal and 4 mm posterior to the nasal edge of superior rectus insertion. In both cases, there was an improvement in the elevation on adduction after superior oblique lengthening. CONCLUSIONS: Abnormal nasal insertion of the superior oblique muscle enhances the depressor effect of the muscle and can create a Brown-like picture.

Congenital anomalies of inferior rectus and its surgical outcomes.

Congenital inferior rectus hypoplasia and aplasia is a rare abnormality of the extraocular muscles, and it is the second most common after superior oblique muscle aplasia. It has been reported either in isolation or with coexisting ocular or systemic associations. We describe here cases of inferior rectus hypoplasia/aplasia, their clinical features, and surgical approach to achieve satisfactory outcomes. We retrospectively reviewed medical records from January 2009 to December 2020 of patients with vertical strabismus due to inferior rectus hypoplasia/aplasia at a tertiary eye care center. Those who underwent surgical intervention with an adequate follow up postoperatively were included in the study. Three patients who presented with congenital hypertropia and diagnosed of inferior rectus aplasia/hypoplasia and who underwent strabismus surgery were identified. Diagnosis was based on clinical examination and orbital imaging. Coexisting horizontal deviation was present in two patients. None of the patients had any other ocular, cranial, or systemic anomalies. All patients had isolated inferior aplasia/hypoplasia with normal other extraocular muscles on orbital imaging. Intraoperatively, ipsilateral superior rectus was tight in all patients. All underwent ipsilateral superior rectus recession, whereas two patients with large vertical strabismus required additional inferior oblique anteronasal transposition in the same eye. Satisfactory outcomes were achieved in all three patients without the need for resurgery. Moderate to large vertical A pattern strabismus and limitation of infraduction are common clinical findings. Orbital imaging helps not only in diagnosis but also in surgical planning. Weakening of antagonist superior rectus with anteronasal transposition of inferior oblique not only gives good surgical outcomes but also avoids potential complications related to vertical transposition of either horizontal rectus muscle.

Anomalous insertion of the superior oblique tendon onto the superior rectus muscle causing congenital superior oblique palsy.

Congenital superior oblique (SO) palsy is often associated with anomalies of its tendon, increased tendon laxity being the most common. Rarely, the tendon lies in an abnormal location nasal to the superior rectus (SR) muscle, either attaching to the sclera or to Tenon's capsule. We describe a case of a child who presented with abnormal head posture and exotropia. The orthoptic evaluation revealed a left hypertropia and V-pattern exotropia. The motility pattern and the Parks three-step test were suggestive of left-sided SO palsy. Intraoperatively, the left SO tendon was very lax and was absent from its usual insertion. Further exploration revealed it to be inserted entirely to the nasal border of the SR muscle, with no scleral attachment. This tendon was advanced to its normal attachment to the sclera, and 11 mm inferior oblique recession was also performed. Postoperative alignment was satisfactory.

Extraocular muscle hypoplasia associated with Axenfeld-Rieger syndrome.

We describe a four-year-old girl with bilateral severe iris hypoplasia and secondary ocular hypertension. Genetic testing revealed a de novo deletion in the FOXC1 gene, establishing the diagnosis of Axenfeld-Rieger syndrome (ARS). The girl developed a gradually increasing exotropia, up to 95 prism diopters by the age of 3 years wherefore strabismus surgery was performed. Intra-operatively, only very rudimentary developed medial and lateral rectus muscles were found. This is the first observation of pronounced hypoplasia of both medial and lateral rectus muscles associated with ARS.

Lagoftalmo na hanseníase: experiência clínica em centro de referência amazonense / Lagophthalmos in leprosy: clinical experience in an Amazon reference center

RESUMO Objetivo: O presente trabalho teve por objetivo caracterizar o perfil epidemiológico e clínico de pacientes com lagoftalmo associado à hanseníase, atendidos no ambulatório de oftalmologia da Fundação Alfredo da Matta, Manaus, Amazonas. Métodos: Trata-se de estudo retrospectivo realizado por meio da análise dos prontuários clínicos dos pacientes incluídos no estudo. Sexo, idade, forma clínica, grau de incapacidade no diagnóstico e desfecho foram obtidos dos prontuários. Início, tipo de comprometimento (unilateral ou bilateral), grau de intensidade do lagoftalmo e alterações oculares associadas também foram compilados. Resultados: Foram incluídos 65 pacientes; 66,1% eram do sexo masculino e 53,8% tinham idade superior a 60 anos. Em relação à classificação operacional da hanseníase, a maioria dos pacientes (81,5%) era multibacilar: 33,8% na forma de hanseníase borderline e 47% virchowiana. 36,9% casos apresentavam sequelas oculares associadas ao lagoftalmo: opacidade corneana, epífora, ceratopatia em faixa, e neovascularização corneana.41,6% evoluíram para a cegueira. O lagoftalmo foi conduzido de forma clínica em 23 pacientes e a abordagem cirúrgica foi indicada em 42. Em relação ao tratamento cirúrgico consistiu principalmente no implante de peso de ouro e na cantoplastia de Tessier. Discussão: O lagoftalmo nessa casuística acometeu mais homens idosos, esteve relacionado à forma multibacilar, com hanseníase do tipo virchowiano como relatado na literatura. O diagnóstico de lagoftalmo foi tardio na maioria dos casos, explicando o grande número de sequelas incluindo a cegueira. Conclusão: O presente estudo reforça a necessidade de acompanhamento oftalmológico precoce para que as potenciais e graves sequelas associadas a essa condição sejam evitadas.

ABSTRACT Objective: The present study aimed to characterize the epidemiological and clinical profile of patients with lagophthalmos associated with leprosy, seen at the ophthalmology outpatient clinic of Fundação Alfredo da Matta, Manaus, Amazonas. Methods: This is a retrospective study carried out by analyzing the medical records of the patients included in the study. Sex, age, clinical form, degree of disability in diagnosis and outcome were obtained from medical records. Onset, type of impairment (unilateral or bilateral), degree of intensity of lagophthalmos and associated eye changes were also compiled. Results: 65 patients were included; 66.1% were male and 53.8% were older than 60 years. Regarding the operational classification of leprosy, most patients (81.5%) were multibacillary: 33.8% in the form of borderline leprosy and 47% virchowian. 36.9% of cases had ocular sequelae associated with lagophthalmos: corneal opacity, epiphora, band keratopathy, and corneal neovascularization.41.6% progressed to blindness. Lagophthalmos was performed clinically in 23 patients and the surgical approach was indicated in 42. Regarding surgical treatment, it consisted mainly of gold weight implantation and Tessier's canthoplasty. Discussion: Lagophthalmos in this sample affected more elderly men, was related to the multibacillary form, with leprosy-like leprosy as reported in the literature. The diagnosis of lagophthalmos was delayed in most cases, explaining the large number of sequelae including blindness. Conclusion: The present study reinforces the need for early eye care so that the potential and serious sequelae associated with this condition are avoided.

Intraoperative absent bilateral medial recti in syndromic craniosynostosis.

Patients with syndromic craniosynostosis are usually associated with the complexity of the malformation complex. We describe here detailed oculo-motility disorder and a remarkable finding of hypoplastic bilateral media recti on imaging and its intraoperative absence in patients with phenotypic features resembling Shprintzen-Goldberg syndrome (SGS). SGS is a rare congenital disorder with craniosynostosis affecting multiple systems including mentation and having a considerable overlap of its phenotypic features with Marfan syndrome. Large A-pattern exotropia found in these patients may be related to the craniofacial features and their bearing on extraocular muscle development and function. In this paper, we aimed to sensitise ophthalmologists and strabismologists concerning the necessity to recognise syndromic associations of patients with craniosynostosis presenting with a large squint, be aware of the intraoperative surprises and consider the challenges in its management.

Examination of the Annular Tendon (Annulus of Zinn) as a Common Origin of the Extraocular Rectus Muscles: 2. Embryological Basis of Extraocular Muscles Anomalies.

Purpose: Many reports have described anomalous connections of the superior rectus (SR) with other extraocular rectus muscles, in which additional heads of the other three rectus muscles likely provided the connections. We examined how these connections are established during fetal development. Methods: We analyzed paraffin-embedded horizontal sections from 25 late-stage fetuses. Horizontal sections are best suited for understanding the mediolateral relationships of muscle origins. Results: We confirmed a common tendinous origin of the lateral rectus (LR), inferior rectus (IR) and medial rectus (MR) muscles that was separated from the SR origin. Notably, eight fetuses (32%) had tendinous or muscular connections between the SR and other rectus muscles that had one of four morphologies: (a) a thin tendon from the SR to the common tendon of the three rectus muscles (2 fetuses), (b) a thin tendon to the LR (one fetus), (c) a thin tendon to the inferior rectus muscle origin (two fetuses), and (d) SR muscle fibers arising from an additional head of the LR (three fetuses). Conclusions: The SR seemed to issue a thin tendon that passed along the inferior or lateral side of the oculomotor nerve. Conversely, the LR and inferior rectus muscle were likely to carry a supernumerary bundle that reached the SR. The accessory head of the medial rectus muscle showed a stable morphology in that it seemed to also provide an anomalous double head. However, the presence of an accessory head in the LR was rare. In contrast with our previously published diagram of the orbital apex, the accessory head of the medial rectus muscle passed along the lateral side of the superior oblique.

Outcomes of Early Correction of Congenital Myogenic Ptosis Using Transconjunctival Levator Plication.

INTRODUCTION: Early correction of congenital ptosis may be indicated due to a risk of amblyopia or because of an abnormal head tilt. One of the main problems, of planning ptosis surgery in very young children, is the inability to measure the levator function. AIM: The aim of the article was to analyze the early correction of congenital myogenic ptosis. METHODS: This was a retrospective, interventional, case series study, conducted on 12 eyes of 12 patients with unilateral, mild to moderate, congenital myogenic ptosis. Surgical correction of ptosis was performed by transconjunctival levator muscle plication. Pre- and postoperative measurements of the upper lid margin to central corneal reflex (MRD1) and upper lid skin crease height (UEC) were obtained, as well as the presence or absence of a reaction to topically applied phenylephrine 2.5% solution. RESULTS: The mean age of the patients was 29.83 months (range 14-45 months). A negative phenylephrine test was noted in only 3 (25%) of cases. Equalization of upper lid height was achieved in 6 (50%), and a hypocorrection of up to 1 mm was noted in 4 (33%) of patients. There was only 1 hypercorrection of 1 mm, noted in the first postoperative month. In one case of hypocorrection of 2 mm, the height of the lid dropped between the 1 and 3 months follow up. Subsequent revision surgery was performed, with a good outcome. With regard to the upper lid skin crease height (UEC), the mean preoperative difference in relation to the contralateral (non-operated) lid, was 2.16 mm, whereas the average postoperative or final difference was 0.41 mm. CONCLUSION: Correction of myogenic ptosis in small children, using transconjunctival levator plication, in whom levator function cannot be measured, may have a satisfactory postoperative outcome.

Minimal invasive vertical muscle transposition for the treatment of large angle exotropia due to congenital medial rectus hypoplasia: Case Report and Literature Review.

Reports of isolated anomalies of the medial rectus (MR) muscle in literature are sparse. It has been identified as a subtype of congenital cranial dysinnervation disorder that affects the normal development of brainstem motor neurons. Herein, we report a 37-year-old male presented with large-angle exotropia since the birth of right eye with palpebral fissure widening. On examination of ocular movements, there was -6 limitation of adduction. There was no limitation in other ocular movements. In the preoperative CT scan, all extraocular muscles were present. He underwent surgery in right eye. Intraoperatively in the site of medial rectus, we found an empty sheath without muscle fibers indicating medial rectus hypoplasia. The width of muscle insertion was normal. Surgery consisted of lateral rectus muscle recession 10 mm in hang-back method and vertical muscle transposition procedure, by a modification of Nishida technique, in which the vector of superior and inferior recti was transposed medially by inserting non-absorbable sutures at nasal margins of muscles secured to sclera 8 mm posterior to medial rectus site without tenotomy or splitting. The deviation was decreased to less than 10 PD exotropia in primary position. The adduction was improved from -6 to -4. The palpebral fissure asymmetry was also corrected. Here, we also reviewed clinical features of all cases of medial rectus hypoplasia/aplasia in the literature and discussed surgical approaches. For vertical rectus transposition and horizontal muscle weakening, this technique has the advantages of being simpler and less traumatic to ocular tissues and unlike the traditional transposition procedures, there is no need for tenotomy and splitting.

Double-bellied medial rectus muscle in a patient with Down syndrome and congenital esotropia.

A 13-year-old male with Down syndrome, pseudophakic secondary to congenital cataract presented with esotropia. During bilateral medial rectus recession, a unilateral two-bellied right medial rectus was identified and recessed successfully with complete resolution of the deviation. Clinicians facing a two-bellied medial rectus can consider continuing with their surgical plan.

Craniosynostosis and oculomotor disorders.

Simple, complex or syndromic craniosynostosis may be responsible for ocular and especially oculomotor pathologies. Among simple craniosynostosis, anterior plagiocephaly is the most frequently associated with oculomotor disorders. Oculomotor disorders encountered in craniosynostosis are specific to this pathology. They may be related to orbital deformities or oculomotor muscle malformations. Early craniofacial surgery reduces the onset and severity of these oculomotor disorders which is very important for ophtalmological patient care. Indeed, these oculomotor disorders are difficult to treat for the ophthalmologist with most of the time several surgeries needed, and lead to amblyopia if neglected.

Persistent visual disturbances after concussion.

BACKGROUND: Ocular dysfunction, including eye movement defects, has been documented in up to 69% of patients with concussion. However, standard sports-related concussion assessment protocols do not typically include any clinical examination of the ocular system. OBJECTIVE: The aim of this article is to inform general practitioners (GPs) about ocular defects associated with concussion, identify test procedures and highlight the important role of GPs within the concussion paradigm. DISCUSSION: Ocular dysfunction that commonly occurs with concussion includes abnormalities of accommodation, convergence, saccades and smooth pursuits. This may cause blurred vision, double vision, ocular pain and difficulty with close work. Symptoms can severely affect daily work, school or play activities. Patients complaining of extended ocular symptoms following concussion should be referred to an ophthalmologist for a complete ocular assessment.

Double-bellied superior rectus muscle.

Congenital variations of extraocular muscles are rare. We report a double-bellied superior rectus muscle, observed in an adult male cadaver aged 70 years. The superior rectus muscle had two equal-sized bellies, which took separate origins from the common tendinous ring and united to form a common belly 1 cm before the insertion. Due to the duplication, the muscle extended laterally beyond the levator palpebrae superioris. Both its bellies were supplied by oculomotor nerve. To the best of our knowledge, this is the first report on doubling of the belly of the superior rectus muscle.