Congenital Tarsal Kink Syndrome: Literature Review and Case Report

Background: The congenital tarsal kink syndrome is a rare form of congenital upper eyelid entropion associated with cardiovascular, musculoskeletal or central nervous system disorders. This syndrome must be recognized and surgically treated as a perinatal emergency to avoid associated complications—corneal ulcer, corneal leucoma, secondary amblyopia and decreased vision among children. Methods: A literature review was conducted to clarify the diagnosis particularities and the corrective surgery options of the congenital entropion on the upper eyelid. Results: Four relevant studies were found by researching the Web of Science and PubMed databases up to November 2021 for “congenital tarsal kink syndrome” and “congenital upper eyelid entropion”. Conclusions: In this paper, we present a case of congenital unilateral entropion of the upper left eyelid in the context of a tarsal kink syndrome in a one-month old infant, manifested by the absence of eyelashes on the upper eyelid of the left eye, hyperlacrimation and conjunctival hyperemia. Essential in managing the upper eyelid entropion is protecting the cornea. Furthermore, correcting a tarsal kink is eminently surgical, choosing between open or closed procedures. Herein, we address the difficulty in the timely diagnosis of this uncommon condition and make formal recommendations based on all reported cases.

Postoperative Ecchymosis in Upper Blepharoplasty: A Split Face Study of Tranexamic Acid Versus Etamsylate

Objective: To compare the efficacy of tranexamic acid (TXA) versus etamsylate (ETM) in reducing postoperative ecchymosis in upper blepharoplasty. Introduction: With an increase in demand for shortened recoveries after facial aesthetic surgery, various optional approaches have been sought out. In terms of ecchymosis, TXA and ETM have been most commonly used. Method: A prospective, intrapatient split face study was conducted from January 2020 to January 2021 in 40 patients who underwent upper blepharoplasty under local anesthesia. Two equal anesthetic solutions were prepared; Solution A contained TXA and solution B contained ETM. Solution A was injected in the right eyelid and solution B in the left eyelid. Postoperative ecchymosis was assessed by 2 blinded evaluators, the assisting surgeon (Observer 1) and an external surgeon (Observer 2) at 48 h and on the seventh day using a scale published by Sagiv et al. Results: Postoperative ecchymosis was found to be less at 48 h by both observers with TXA solution. Moreover, at the seventh day, no ecchymosis was found in 32.5% with TXA solution compared to 2.5% with ETM solution. Kappa analysis showed concordance between observers. Conclusion: In our study, TXA solution was a more effective therapy when compared to ETM solution for reducing ecchymosis. Larger case studies are required to prove the difference for validation.

INVASIVE FUNGAL VASCULOPATHY AND CENTRAL NERVOUS SYSTEM COMPLICATIONS

60 years female admitted with subacute onset of pain left side of face , swelling around left eye , drooping of left eyelid , loss of vision in left eye of 3 days duration . After admission in the hospital course she developed weakness of right upper and lower limb, congestion and proptosis of left eye. She was having diabetes for the past three years, poorly controlled. Random blood sugar was 450mg/dl and urine acetone was positive. During the hospital course she developed diabetic ketoacidosis for which she was treated. ENT and Ophthalmology opinion was obtained as left orbital apex syndrome.

Conjunctival Tarsal Actinic Keratosis Treated with Interferon Alfa-2b: A Rare Case Report and Literature Review

Conjunctival neoplasia is one of the most frequent tumors in the eye. Actinic keratosis (AK) or solar keratosis is a precancerous lesion that is included with other epithelial tumors. This alteration does not break the basal membrane. There is enough evidence of successful outcomes to consider interferon alfa-2b (IFN alfa-2b) as the first choice of treatment for this type of tumors. In addition, side effects are mild and uncommon. We report a case in an 83-year-old woman who was referred to evaluate a leukoplakia in the tarsal conjunctiva of the lower left eyelid that measured 1 cm in diameter. Pathological study revealed AK. After the INF alfa-2b treatment, we observed conjunctival hyperemia, noninfiltrated upper nasal de-epithelization, and inferior nasal bulla. AK with presentation in conjunctiva is rarely described and in tarsal conjunctiva is exceptional. It is the first case published with only tarsal conjunctiva affectation.

A case study on various disorders and defects present in congenital myasthenia syndrome

The Congenital Myasthenia Syndromes (CMS) are a different gathering of problems that have a hidden deformity in the transmission of signs from nerve cells to muscles. These problems are described by muscle shortcoming, which is declined upon effort. The time of beginning, seriousness of introducing indications and dissemination of muscle shortcoming can shift starting with one patient then onto the next. The synapse, acetylcholine, or ACh for short that goes about as a compound 'courier' with guidelines for the muscles to contract. A three years old child female patient was brought to our department with the complaints of drooping of the left eyelid after one week she developed drooping of right eye. With scientific and laboratory discoveries, she is identified by congenital myasthenia and treatment was started. Evidence from case notes, history, review and accept. Muscle fatigue habits included limb, trunk, bulbar, respiratory, nasal, extraocular muscles and patients reacted with anticholinesterase and 3,4-diaminopyridine. Quick channel syndrome compared with AChR in serious respiratory emergencies in infancy or early childhood. Two children's fatalities, also in care and family history of sibling deaths, highlight the need for effective genetic diagnosis.

Huge peripapillary staphyloma with craniofacial clefts: A case report

Purpose: We reported the occurrence of a congenital unilateral huge peripapillary staphyloma in association with craniofacial clefts for the first time. Case report: A 1-year-old boy presented with a large defect on his left eyelid, a wide oblique columella nasi and an atypical wedge-shaped extension of the unilateral anterior hairline. Magnetic resonance imaging (MRI) examinations revealed there were cracks on his nasal septum, palate, and superior alveolar midline. Moreover, we surprisingly uncovered a gourd-shaped eyeball with the compressed optic nerve on the right side, while the right eye seemed normal from appearance. Under anaesthesia, fundus examination of the right eye showed a 15 mm-deep excavation surrounding the optic disc with defective choroid and dysplastic optic papilla. We reconstructed the left eyelid of the patient to protect his cornea and would make other solutions according to the results of follow-up. Conclusion: Peripapillary staphyloma and craniofacial clefts are two dissimilar rare congenital anomalies. In this patient, we firstly observed the co-existence of the two defects, which may provide the experience to the diagnosis and treatment of peripapillary staphyloma and craniofacial clefts. This case also gives us the pathogenic inspiration for further studies of peripapillary staphyloma and craniofacial clefts.

Partial resolution of chronic unilateral sinonasal obstructive disease in a cat using a temporary polyvinylchloride stent

Case summary A 3-year-old male neutered domestic shorthair cat presented for further investigation of a swollen left eyelid and a hard, non-painful bony swelling over the left frontal sinus. Physical examination revealed no nasal discharge or airflow through the left nostril. A CT of the head revealed a left frontal sinus obstruction with expansile remodelling and osteolucency. Drainage of the frontal sinus obstruction was alleviated via placement of a temporary polyvinylchloride (PVC) stent that was left in place for 6 weeks. Purulent material removed from the frontal sinus returned a positive culture for Pseudomonas aeruginosa susceptible to marbofloxacin that was continued for 4 weeks, and for an additional 2 weeks after stent removal. A left-sided mucoid nasal discharge returned 3 months after removal of the stent. Repeat CT performed 20 months after surgery found the presence of a gas-filled frontal sinus and partial resolution in the amount of fluid within the mid and rostral nasal cavity. A repeat positive culture for P aeruginosa was also obtained. The exact cause of the chronic unilateral sinonasal obstruction remains unclear, but an underlying chronic rhinitis with secondary obstructive frontal sinusitis or sinus mucocoele with secondary nasal extension was suspected. Relevance and novel information Chronic rhinosinusitis in cats can be a challenging condition to treat and cure. This case illustrates the partial resolution of chronic unilateral sinonasal obstructive disease in a cat using a temporary PVC stent.

MON-235 Mycobacterium Fortuitum Infection Mimicking Sellar Chondrosarcoma in a Non-Immunosuppressed Patient: An Unusual Cause of Hypopituitarism and Oculomotor Nerve Palsy

A 32-year old male patient of central African origin presented with diplopia and left eyelid ptosis. He described a 2-month history of fatigue, weight loss (8 Kg), headaches, diffuse myalgia and night sweats. Clinical examination revealed cavernous sinus syndrome with left eyelid ptosis and weakness of eye adduction. Magnetic resonance imaging of the brain demonstrated a 36 mm mass centered on the left petroclival suture, infiltrating the sella and the pituitary gland, the ipsilateral orbital apex as well as the cavernous sinus bilaterally. The mass showed heterogenous enhancement after gadolinium injection, with elements of central necrosis and was associated with an extensive bone destruction. These radiologic features raised the hypothesis of chondrosarcoma. Chest computed tomography demonstrated multiple lung micro-nodules suspect of metastasis. Laboratory testing of the anterior pituitary function revealed low free-T4 (11 pmol/l, n = 12-22) with normal TSH (0.4 mUI/l, n=0.3-4.2), low total testosterone (1.5 ugr/l, n = 3.3-8.1) with normal LH and FSH and slight hyperprolactinemia (27 ugr/l, n = 4-15). IGF-1, 24-h urinary free cortisol, as well as morning serum cortisol and cortisol after 250 mcg ACTH stimulation test were normal. There was no evidence of diabetes insipidus. Levothyroxine was prescribed. Craniotomy was performed, for left optic nerve decompression and biopsy of the mass. Pathologic examination revealed granulomatous, giganto-cellular and necrotizing inflammation, but no evidence of malignancy. PCR for Mycobacterium tuberculosis complex was negative but Mycobacterium fortuitum was detected in sputum and also confirmed in cerebral biopsy latter. Other causes of granuloma were excluded (brucellosis, cat scratch disease, histoplasmosis, syphilis, coccidioidomycosis, tropical germs etc.). Different causes of immunosuppression (including HIV) were excluded. The patient was treated with amikacin, isoniazid and ciprofloxacin for several months and improved gradually. MRI performed one year later demonstrated significant decrease on the size of the sellar mass (more than 50% of its initial size). Central hypogonadism regressed spontaneously with decrease in tumor size, and normal testosterone levels were achieved at one-year follow-up (7 ugr/l, n = 3.3-8.1). Mycobacterium fortuitum infections of the sella turcica are poorly described in literature in non-immunosuppressed individuals. Although usually not pathogenic, histopathological examination, identification in the CNS lesion and the lungs and response to treatment are convincing evidence of a causal relationship. Differential diagnosis from malignant lesions is challenging and biopsy is necessary in order to establish the cause and offer adequate treatment.