scholarly journals CHARGE Syndrome Associated with Angle Closure despite High Myopia: A Case Report with Structural Suggestion

2020 ◽  
Vol 11 (1) ◽  
pp. 28-36
Author(s):  
Saki Dote ◽  
Shunsuke Nakakura ◽  
Hirotaka Tanabe ◽  
Etsuko Terao ◽  
Yuki Nagata ◽  
...  

CHARGE syndrome is associated with multiple malformations, and the main ocular abnormality is coloboma. We describe an unusual case of a 15-year-old girl with CHARGE syndrome having high intraocular pressure and narrow angle closure despite refractive high myopia (−9.0 dpt) in her left eye. Gonioscopy revealed peripheral anterior synechia (approximately 90°) in the superior quadrant of the left eye. Both eyes exhibited similar axial length (about 22 mm) and corneal curvature (about 8 mm). However, microcornea (9 mm), thicker central cornea and iris induced narrower anterior components in the left eye than in the right eye. Preventing the chance of acute primary angle closure attack, the patient underwent laser iridotomy in the left eye; however, long-term follow-up is needed. Additionally, we developed a hypothesis for the mechanism of unilateral angle closure despite high myopia by investigating the ocular structural parameters in detail.

2021 ◽  
pp. 263246362097804
Author(s):  
Vanita Arora ◽  
Pawan Suri

Anatomy and physiology are the basis of human body functioning and as we have progressed in management of various diseases, we have understood that physiological intervention is always better than an anatomical one. For more than 50 years, a standard approach to permanent cardiac pacing has been an anatomical placement of transvenous pacing lead at the right ventricular apex with a proven benefit of restoring the rhythm. However, the resultant ventricular dyssynchrony on the long-term follow-up in patients requiring more than 40% ventricular pacing led to untoward side effects in the form of heart failure and arrhythmias. To counter such adverse side effects, a need for physiological cardiac pacing wherein the electrical impulse be transmitted directly through the normal conduction system was sought. His bundle pacing (HBP) with an intriguing alternative of left bundle branch pacing (LBBP) is aimed at restoring such physiological activation of ventricles. HBP is safe, efficacious, and feasible; however, localization and placement of a pacing lead at the His bundle is challenging with existing transvenous systems due to its small anatomic size, surrounding fibrous tissue, long-learning curve, and the concern remains about lead dislodgement and progressive electrical block distal to the HBP lead. In this article, we aim to take the reader through the challenging journey of HBP with focus upon the hardware and technique, selective versus nonselective HBP, indications and potential disadvantages, and finally the future prospects.


2021 ◽  
Vol 21 (1) ◽  
Author(s):  
BoRan Mu ◽  
ZhiQiang Zhang ◽  
Chongdong Liu ◽  
Kunning Zhang ◽  
ShuHong Li ◽  
...  

Abstract Background Inguinal endometriosis (IEM) is a rare extra pelvic endometriosis. Here, we study the clinical characteristics, management strategies, and long-term gynecological outcomes of IEM patients at Beijing Chaoyang Hospital. Case presentation Three patients presented with a total of four lesions (one on the left side, one on the right side, and one bilaterally). The diameters of the four lesions were 2 cm, 2 cm, 3.5 cm and 1.5 cm, respectively. Two patients were admitted with inguinal hernias. Two patients were admitted with endometrioses—one with ovarian endometriosis and one with pelvic endometriosis. The hernia sac was repaired concomitantly via excision of the round ligament in two patients. One patient underwent a concomitant laparoscopy for gynecologic evaluations, including an ablation to the peritoneal endometriosis, and resection of the left uterosacral ligament endometriosis and pelvic adhesiolysis. All lesions were located on the extraperitoneal portion of the round ligament and were diagnosed histologically. No recurrence was observed in the inguinal region. All patients diagnosed with adenomyosis were treated with medication alone without any complaints. Conclusions Inguinal endometriosis can occur simultaneously with pelvic endometriosis. In most cases, a concomitant hernia sac appears together with groin endometriosis. Clinical management should be individualized and performed in tandem with general practitioners and obstetrics & gynecology experts. Pelvic disease, in particular, should be followed-up by a gynecologist.


2002 ◽  
Vol 12 (3) ◽  
pp. 224-228 ◽  
Author(s):  
Haifa Abdul Latiff ◽  
Mazeni Alwi ◽  
Hasri Samion ◽  
Geetha Kandhavel

This study reviewed the short-term outcome of transcatheter closure of the defects within the oval fossa using an Amplatzer® Septal Occluder. From January 1997 to December 2000, 210 patients with defects within the oval fossa underwent successful transcatheter closure. We reviewed a total of 190 patients with left-to-right shunts, assessing the patients for possible complications and the presence of residual shunts using transthoracic echocardiogram at 24 h, 1 month, 3 months and one year. Their median age was 10 years, with a range from 2 to 64 years, and their median weight was 23.9 kg, with a range from 8.9 to 79 kg. In 5 patients, a patent arterial duct was closed, and in 2 pulmonary balloon valvoplasty performed, at the same sitting. The median size of the Amplatzer® device used was 20 mm, with a range from 9 to 36 mm. The median times for the procedure and fluoroscopy were 95 min, with a range from 30 to 210 min, and 18.4 min, with a range from 5 to 144 min, respectively. Mean follow-up was 20.8 ± 12.4 months. Complete occlusion was obtained in 168 of 190 (88%) patients at 24 h, 128 of 133 (96.2%) at 3 months, and 103 of 104 (99%) at one year. Complications occurred in 4 (2.1%) patients. In one, the device became detached, in the second the device embolized into the right ventricular outflow tract, the lower end of the device straddled in the third, and the final patient had significant bleeding from the site of venupuncture. There were no major complications noted on follow-up. We conclude that transcatheter closure of defects within the oval fossa using the Amplatzer® Septal Occluder is safe and effective. Long-term follow-up is required, nonetheless, before it is recommended as a standard procedure.


Author(s):  
Claudia Raluca Mărginean ◽  
Patricia Maria Luminița Simu ◽  
Robert Aurelian Tiucă ◽  
Alexandru Mariean-Șchiopu ◽  
Iunius Paul Simu

"Pilocytic astrocytoma is the most frequent type of brain tumor diagnosed during childhood. It originates from midline structures and is associated with good prognosis, with an estimated survival rate higher than 95%. We presented the case of a male patient diagnosed at the age of 6 with pilocytic astrocytoma located in the right optic nerve with associated chiasm infiltration. Incomplete resection of the tumoral process was performed, without any additional therapy, as association of chemotherapy or radiotherapy is still controversial among specialists. The patient had an unpredictable severe evolution of the disease, with associated leptomeningeal dissemination and extreme worsening of neurological and endocrinologic status. Six years after diagnosis, despite complex medical efforts the patient died. This article raises awareness of leptomeningeal dissemination risk, a rare evolution in cases of pilocytic astrocytoma. Early diagnosis, complete tumoral resection, tumor location and careful long-term follow up are key factors for long-term survival. "


2018 ◽  
Author(s):  
Ye Zhang ◽  
Ravi Thomas ◽  
Cong Wang ◽  
Xiangyu Shi

Abstract Background: Spherophakia is a rare condition and compared to its occurrence with familial and systemic disorders, isolated spherophakia is even more uncommon. This rare case of isolated spherophakia will raise the alertness of physicians when dealing with a high myopia patient with shallow anterior chamber and relatively normal fundus. Case presentation: We report a case of a 17-year-old male who experienced painless decrease of vision in both eyes (OU) for 11 years, with progression of visual impairment and occasional ocular pain for one year. Examination revealed high myopia, increased intraocular pressures (IOP, 28 mmHg in the right eye (OD) and 33 mmHg in the left (OS)), shallow central anterior chambers, lenses of a spherical shape with superior subluxation, occludable angles without peripheral anterior synechiae, and healthy optic discs OU. A diagnosis of bilateral isolated spherophakia, lens subluxation, high myopia and secondary angle closure (AC) was made. Pars plana lensectomy with anterior vitrectomy and scleral suturing of an intraocular lens was performed. On postoperative follow-up at 19 days OD and 63 days OS, the visual acuity was 6/6 and the IOP was normal without any medication OU. Conclusions: A presentation with high myopia, shallow anterior chamber with a normal retina should alert the clinician to the possibility of spherophakia, as compared to high myopia caused by elongated axial length. Surgery should be considered in cases of spherophakia with AC where the IOP cannot be controlled by noninvasive means.


2020 ◽  
Vol 30 (3) ◽  
pp. 409-412
Author(s):  
Murat Surucu ◽  
İlkay Erdoğan ◽  
Birgül Varan ◽  
Murat Özkan ◽  
N. Kürşad Tokel ◽  
...  

AbstractObjective:Double-chambered right ventricle is characterised by division of the outlet portion of the right ventricle by hypertrophy of the septoparietal trabeculations into two parts. We aim to report our experiences regarding the presenting symptoms of double-chambered right ventricle, long-term prognosis, including the recurrence rate and incidence of arrhythmias after surgery.Methods:We retrospectively investigated 89 consecutive patients who were diagnosed to have double-chambered right ventricle and underwent a surgical intervention from 1995 to 2016. The data obtained by echocardiography, cardiac catheterisation, and surgical findings as well as post-operative follow-up, surgical approaches, post-operative morbidity, mortality, and cardiac events were evaluated.Results:Median age at the time of diagnosis was 2 months and mean age at the time of operation was 5.3 years. Concomitant cardiac anomalies were as follows: perimembranous ventricular septal defect (78 patients), atrial septal defect (9 patients), discrete subaortic membrane (32 patients), right aortic arch (3 patients), aortic valve prolapse and/or mild aortic regurgitation (14 patients), and left superior caval vein (2 patients). The mean follow-up period was 4.86 ± 4.6 years. In these patients, mean systolic pressure gradient in the right ventricle by echocardiography before, immediately, and long-term after surgical intervention was 66.3, 11.8, and 10.4 mmHg, respectively. There were no deaths during the long-term follow-up period. Surgical reinterventions were performed for residual ventricular septal defect (2), residual pulmonary stenosis (1), and severe tricuspid insufficiency (1).Conclusion:The surgical outcomes and prognosis of double-chambered right ventricle are favourable, recurrence and fatal arrhythmias are unlikely in long-term follow-up.


2019 ◽  
Vol 29 (1) ◽  
pp. 157-158
Author(s):  
Akira Furutachi ◽  
Kojiro Furukawa ◽  
Aiko Komatsu ◽  
Eijiro Nogami

Abstract Anomalous origin of the right coronary artery (RCA) from the pulmonary artery is a very rare congenital heart disease, and several reports have described long-term events after surgery. We report the case of a 46-year-old woman who underwent reimplantation of the RCA for anomalous origin of the right coronary artery from the pulmonary artery 16 years ago. An RCA aneurysm gradually developed and dilated over time, and we resected the aneurysm and also grafted the right gastroepiploic artery graft to the distal RCA. Careful long-term follow-up is required to avoid overlooking such a rare but life-threatening complication after surgical repair of anomalous origin of the right coronary artery from the pulmonary artery.


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