Extraocular Muscle Pathology in Aperts Syndrome

Author(s):  
Bruce R. Pachter ◽  
Shelia Margolis ◽  
Jacob Davidowitz ◽  
Goodwin M. Breinin

Aperts disease or Acrocephalosyndactyly described by Apert1 consists of oxycephaly (tower skull), exophthalmos, strabismus, antimongloid fissures, syndactyly (fusion of fingers, 2-4). This syndrome frequently involves ocular motility disturbances.: the latter have been suggested to result from mechanical limitations of the globe which are secondary to the bony malformation of the orbit2. Up until this time the question of a structural abnormality existing in the extraocular muscles themselves has not been investigated. The present light and electron microscopic study examined a segment of the inferior oblique muscle taken from a patient with Aperts syndrome.The right inferior oblique muscle from a patient with Aperts syndrome was completely disinserted and an 8mm segment was taken from its belly portion. The specimen was fixed initially in 1% paraformaldehyde-1% glutaraldehyde in phosphate buffer for 4 hrs, then transferred to 4% glutaraldehyde overnight. The muscle segment was postfixed in 1% osmium tetroxide, dehydrated in graded alcohols, embedded whole in Epon 812, and serially sectioned at 15 microns.

Author(s):  
Charlotte L. Ownby ◽  
Robert A. Kainer ◽  
Anthony T. Tu

One of the significant changes induced by the injection of rattlesnake (Crotalidae) venom is hemorrhage. Since crotaline antivenin does not prevent such local tissue damage, a more effective treatment of snakebite is needed. To aid in the development of such a treatment the pathogenesis of venom-induced hemorrhae was investigated.Swiss-Webster white mice were injected intramuscularly with Western diamondback rattlesnake (Crotalus atrox) venom. Two minutes after the injection, muscle tissue was obtained by bioosy from the thigh and fixed in 6% glutaraldehyde in Milloniq's phosphate buffer (DH 7.4, 2 hrs., 4°C). After post-fixation in 2% osmium tetroxide in Milloniq's phosphate buffer (pH 7.4, 1hr., 4°C) the tissue was dehydrated routinely in ethanol and embedded in Epon 812. The thin sections were stained with uranyl acetate in methanol and lead citrate then observed with either a Zeiss EM 9A or an Hitachi HS-8 electron microscope.


2020 ◽  
pp. 112067212095758
Author(s):  
Massimiliano Serafino ◽  
Andrea Lembo ◽  
Matteo Scaramuzzi ◽  
Andrea Dellavalle ◽  
Paolo Nucci

Pulled-in-two syndrome (PITS) is a serious intraoperative complication of strabismus surgery in which an extraocular muscle manipulated during the procedure is ruptured and potentially lost. Usually, there is a systemic or local condition that determines muscle weakness when put under tension. If the proximal portion of the broken muscle can be found, it can be reattached to the ocular globe or remaining muscle. If this is not possible, there are multiple varying approaches. We present three cases of PITS of the inferior rectus muscle, treated with good results with anterior and nasal transposition of the inferior oblique muscle. We propose this surgery as another potential technique if the muscle can not be retrieved.


2021 ◽  
Vol 62 (9) ◽  
pp. 1287-1291
Author(s):  
Seo Yoon Heo ◽  
Haeng-Jin Lee ◽  
Myoung Ja Chung ◽  
Min Ahn

Purpose: We report a case of phakomatous choristoma presenting as an orbital tumor with involvement of the inferior oblique muscle. Case summary: A 2-month-old male infant presented to our clinic with a right orbital mass that had been present since birth. Magnetic resonance imaging demonstrated a homogenous enhanced well-defined mass located in the inferomedial portion of the right orbit without bone erosion. By transconjunctival orbitotomy, the orbital tumor invading the inferior oblique muscle was identified and resected. Histopathology showed a thick basement membrane surrounding pseudoglandular structures embedded in a collagenous stroma, psammomatous calcific foci in the stroma, and eosinophilic material in the lumen. Immunohistochemical analysis showed positive staining for S-100 and cytokeratin. On histopathological evaluation, the tumor was diagnosed as phakomatous choristoma. Conclusions: To our knowledge, this is the first report in South Korea of phakomatous choristoma of the orbit with involvement of the inferior oblique muscle. Although rare, phakomatous choristoma should be included in the differential diagnosis of tumors occurring on the inferomedial side of the orbit.


Author(s):  
G.V. Muraveva ◽  
◽  
A.A. Vydrina ◽  

Purpose - to evaluate the effectiveness of a joint method for the treatment of severe amblyopia, combined with uncomfortable esotropia, including chemodenervation of the extraocular muscle and subsequent pleoptic treatment using the «Ambliokor» medical complex. Material and methods. The patient is 3 years old. Diagnosis: unfriendly non-paralytic esohypertropia with a prevalence of OD; amblyopia, severe OD, moderate OS, mixed astigmatism OU. The treatment was carried out in 2 stages. At the first stage in order to eliminate the dysbinocular factor of amblyopia, chemodenervation of the internal rectus muscle of the right eye with the preparation of botulinum toxin type A «Botox» was performed by the intraoperative technique. After 1 month, at the second stage, a complex pleoptic treatment was carried out. Results. After chemodenervation, 1 month later, orthotropy in the primary gaze position without glasses and with glasses was achieved. The vertical deviation significantly decreased to 5 degrees in the position of adduction of the eyeballs. The right lead was restored fully. The forced turn of the head was eliminated. After the third course of complex pleoptic treatment, visual acuity with correction was 0.7 on the right and 0.8 on the left. Restoration of central visual fixation in the right eye was noted. The simultaneous nature of vision was revealed. The deviation angle of the eyes in the primary gaze position without glasses and with glasses to the nose did not exceed +7 degrees according to Hirshberg. Hypertropia persisted in adduction up to 10 degrees according to Hirschberg (hyperfunction of the inferior oblique muscle) in both eyes. «V»-syndrome persisted. The patient is planned to surgically weaken the hyperfunction of the inferior oblique muscle of both eyes, followed by the continuation of a complex of pleopto-orthoptic treatment. Conclusion. The existing extensive base of various methods of influencing the functions of the amblyopic eye allows for successful treatment of even severe amblyopia. Key words: amblyopia, unfriendly esotropia, chemodenervation, pleoptic treatment.


2021 ◽  
Author(s):  
Lijuan Huang ◽  
Ningdong Li

Abstract Background: For the majority of the patients, surgical treatment of primary infantile exotropia (PIE) with large exodeviation angels of more than 50 prism diopters (PD) is not satisfactory. We evaluate the effectiveness of augmented lateral rectus (LR) muscle recession in treatment of congenital exotropia with large deviation angles. Methods: A retrospective analysis was performed for 25 patients with congenital exotropia who had at least 60 prism diopters (PD). Augmented LR recession was performed using a hemi hang-back technique. The ocular alignment, ocular motility and binocular vision were evaluated pre- and postoperatively. Results: There were 14 male and 11 female affected individuals with congenital exotropia included in this study, with a mean age of (3.92±2.02) years (range, 1 - 7 years). The average exodeviation angle was (68.0±8.78) PD (range, 60 - 80 PD) preoperatively. All patients underwent bilateral LR recession, of whom 3 patients underwent bilateral inferior oblique muscle transposition in the same operation. The mean surgical dosage was (11.08±1.19) mm for each lateral rectus muclse. All patients did not have abduction deficiency after a large amount of lateral rectus recession. 21 of 25 patients (84.0%) acquired orthophoria at the primary gaze position at the final visit. 13 patients obtained binocular vision. Conclusions: Augmented bilateral LR recession is an effective and safe surgical procedure for treatment of congenital exotropia with large deviation angles.


Author(s):  
F. Murata ◽  
K. Yoshida ◽  
S. Ohno ◽  
T. Nagata

As a series of histochemical studies on the mucosubstances of the granulocytes in this laboratory, the results obtained by electron microscopic radioautography and X-ray microanalysis have been reported.The femur bone marrows of adult rabbits weighing 1. 0-1. 5kg were diced into small pieces and incubated for 10, 30 and 60 minutes in Eagle's MEM supplemented with 10 % bovine serum, containing radioactive carrier free sulfuric acid at a concentration of lmCi/ml. They were fixed doubly in glutaraldehyde buffered with 0. 1 M phosphate buffer (pH 7. 2) and osmium tetroxide buffered with 0. 1 M phosphate buffer(pH 7. 2), dehydrated through a graded series of ethanol and acetone and embedded in Epon. Ultrathin sections were cut with an LKB ultrotome and radioautographed with Sakura NR-H2 emulsion by wire loop method, exposed for 127-203 days, developed with phenidone developer after gold latensification, stained with lead citrate and observed in Hitachi HS-9 electron microscope.


Author(s):  
Ronald H. Bradley ◽  
R. S. Berk ◽  
L. D. Hazlett

The nude mouse is a hairless mutant (homozygous for the mutation nude, nu/nu), which is born lacking a thymus and possesses a severe defect in cellular immunity. Spontaneous unilateral cataractous lesions were noted (during ocular examination using a stereomicroscope at 40X) in 14 of a series of 60 animals (20%). This transmission and scanning microscopic study characterizes the morphology of this cataract and contrasts these data with normal nude mouse lens.All animals were sacrificed by an ether overdose. Eyes were enucleated and immersed in a mixed fixative (1% osmium tetroxide and 6% glutaraldehyde in Sorenson's phosphate buffer pH 7.4 at 0-4°C) for 3 hours, dehydrated in graded ethanols and embedded in Epon-Araldite for transmission microscopy. Specimens for scanning electron microscopy were fixed similarly, dehydrated in graded ethanols, then to graded changes of Freon 113 and ethanol to 100% Freon 113 and critically point dried in a Bomar critical point dryer using Freon 13 as the transition fluid.


Author(s):  
W. G. Banfield ◽  
G. Kasnic ◽  
J. H. Blackwell

An ultrastructural study of the intestinal epithelium of mice infected with the agent of epizootic diarrhea of infant mice (EDIM virus) was first performed by Adams and Kraft. We have extended their observations and have found developmental forms of the virus and associated structures not reported by them.Three-day-old NLM strain mice were infected with EDIM virus and killed 48 to 168 hours later. Specimens of bowel were fixed in glutaraldehyde, post fixed in osmium tetroxide and embedded in epon. Sections were stained with uranyl magnesium acetate followed by lead citrate and examined in an updated RCA EMU-3F electron microscope.The cells containing virus particles (infected) are at the tips of the villi and occur throughout the intestine from duodenum through colon. All developmental forms of the virus are present from 48 to 168 hours after infection. Figure 1 is of cells without virus particles and figure 2 is of an infected cell. The nucleus and cytoplasm of the infected cells appear clearer than the cells without virus particles.


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