Sight-threatening progressive corneo-scleral involvement in porphyria cutanea tarda

2021 ◽  
Vol 14 (10) ◽  
pp. e245160
Author(s):  
Sonali Prasad ◽  
Vidhata Vidhata ◽  
Subhash Prasad

Porphyria cutanea tarda is the most common type of porphyria. It is associated with a deficiency of uroporphyrinogen decarboxylase enzyme responsible for heme synthesis. Clinical manifestations are predominantly dermatological and very rarely present with ocular involvement. Although scleral thinning in the interpalpebral area is a well-documented entity, sight-threatening corneal involvement is rarely described. We, herein report a case of a 58-year-old man who presented with ocular surface dryness, photophobia and mild redness. Slit-lamp biomicroscopy revealed corneo-scleral thinning in both eyes. The diagnosis was confirmed with a urine porphyrin test, serum iron and serum ferritin levels. We started him on conservative management after which he was lost to follow-up. He presented again after 6 years with total corneal opacification and progressive loss of vision in the right eye.

2016 ◽  
Vol 2016 ◽  
pp. 1-6 ◽  
Author(s):  
Cuilan Li ◽  
Shiyan Tang ◽  
Xingcheng Gao ◽  
Wanping Lin ◽  
Dong Han ◽  
...  

Background. Diverticulum, one of the long-term sequelae of cesarean section, can cause abnormal uterine bleeding and increase the risk of uterine scar rupture. In this study, we aimed to evaluate the efficacy of combined laparoscopic and hysteroscopic repair, a newly occurring method, treating post-cesarean section uterine scar diverticulum.Methods. Data relating to 40 patients with post-cesarean section uterine diverticulum who underwent combined laparoscopic and hysteroscopic repair were retrospectively analyzed. Preoperative clinical manifestations, size of uterine defects, thickness of the lower uterine segment (LUS), and duration of menstruation were compared with follow-up findings at 1, 3, and 6 months after surgery.Results. The average preoperative length and width of uterine diverticula and thickness of the lower uterine segment were recorded and analyzed. The average durations of menstruations at 1, 3, and 6 months after surgery were significantly shorter than the preoperative one (p<0.05), respectively. At 6 months after surgery, the overall success improvement rate of surgery was 90% (36/40). Three patients (3/40 = 7.5%) developed partial improvement, and 1/40 (2.5%) was lost to follow-up.Conclusions. Our findings showed that combined treatment with laparoscopy and hysteroscopy was an effective method for the repair of post-cesarean section uterine diverticulum.


2018 ◽  
Vol 9 (1) ◽  
pp. 232-237 ◽  
Author(s):  
Salim Z. Asaad ◽  
Nazimul Hussain

A 21-year-old female presented with decreased vision in the right eye. Best corrected visual acuity was 6/60 in the right eye and 6/6 in the left eye. Funduscopy of the right eye revealed a subfoveal nodule with surrounding exudates and temporal peripheral retinal vessel telangiectasia with exudation. The patient underwent retinal laser in the areas of telangiectatic vessels and capillary non-perfusion as seen on fundus fluorescein angiography. It was combined with an intravitreal injection of bevacizumab which was repeated twice at monthly intervals. The macular lesion and peripheral vessels along with subretinal exudates showed resolution during the course of treatment. However, the patient was lost to follow-up and returned 5 months later when examination revealed increased macular fibrosis. In the literature, the reported presentation of subfoveal nodule in Coats disease is during the first decade. It progresses to macular fibrosis within a few months. This case illustrates that subfoveal nodule before macular fibrosis in Coats disease may present later, even up to the third decade.


2009 ◽  
Vol 11 (10) ◽  
pp. 878-880 ◽  
Author(s):  
Michal O. Hess

A 4-month-old castrated male domestic shorthair cat was presented for a right hind leg lameness of 1-week duration. Physical examination revealed a painful swelling over the right distal femur. A radiograph of the right stifle revealed an extensive lytic lesion involving the distal metaphysis of the right femur. Cytological examination of a needle aspirate was consistent with a deep fungal infection. A pigmented dimorphic fungus was isolated in pure culture from a representative needle aspirate from the lesion. The patient improved substantially after 5 weeks of high-dose itraconazole therapy, although it was subsequently lost to follow-up.


2021 ◽  
Author(s):  
Xiaoxu Wang ◽  
Wei Liu ◽  
Lejian He ◽  
Min Chen ◽  
Jianbo Shao ◽  
...  

Abstract Purpose Summarized the clinical characteristics and diagnosis and treatment process of three cases of nodular fasciitis of ear, to provide a basis for clinical diagnosis and treatment. Methods Reviewed the clinical manifestations, images, pathology, treatment and postoperative follow up results of three cases of pediatric nodular fasciitis in the Department of Otorhinolaryngology, Head and Neck Surgery, Beijing Children's Hospital, Capital Medical University from 2018 to 2020. Results The average age at diagnosis were 24 months, with two girls and a boy. Two lesions were found in the left ear and one in the right ear. All cases had a history of biopsy before surgery. Two of three cases showed a sign of rapid growth after biopsy and three of which were ineffective in anti-inflammatory treatment. FISH test for USP6 were performed in two of the three cases with positive results. Three lesions show a hypointensity or isointensity on T1-weighted MRI and a heterogeneous hyperintensity on T2-weighted MRI. ‘‘Fascial tail’’ sign was found on image of all three cases. All lesions underwent surgical resection. Follow-up showed no recurrence and had an intact ear appearance. Conclusion The early misdiagnosis rate of nodular fasciitis of the ear is high. Combine clinical features with imaging findings may improve the accuracy of preoperative diagnosis. Besides the appearance of pathology, USP6 gene test is also an important tool in the diagnosis. The final diagnosis should be based on comprehensive assessment. Complete surgical resection can prevent recurrence.


2014 ◽  
Vol 13 (3) ◽  
pp. 185-187
Author(s):  
Luis Claudio de Velleca e Lima ◽  
Fernando Gritsch Sanchis

OBJECTIVE: To quantify the mobility of the lower cervical spine after seven years of total cervical disc replacement at two levels. METHOD: This clinical study was designed randomly and prospectively at the spine surgery center at the Hospital Nossa Senhora das Graças, in Canoas, RS-Brazil and at the Hospital Don João Becker, in Gravataí, RS-Brazil. Seventeen patients were included in the study that was designed to compare the data obtained from annual and sequential manner until the end of seven years. A comparison was made with the prior range of motion (ROM) of each patient. All patients were diagnosed with not tractable symptomatic cervical degenerative disc disease with two adjacent levels between C-3 and C-7. RESULTS: A total of patients underwent TDR in two levels and at the end of seven years, only one patient was lost to follow-up. The pre and postoperative ROM was the same in the first three years however after the fourth year there was a gradual decline with a loss of 12% of preoperative ROM in flexion, 21% in extension and 23% in the right and left lateral bending at the end of seven years. CONCLUSIONS: The clinical outcome of this study is evidence level IV in evaluating the ROM for Moby-C(r) for TDR in two adjacent levels at the lower cervical spine. These results show that the ROM is maintained during the first three years, gradually declining after that.


PEDIATRICS ◽  
1979 ◽  
Vol 63 (1) ◽  
pp. 130-134
Author(s):  
Fergus M.B. Moylan ◽  
Daniel C. Shannon

The chest roentgenograms of 142 neonates who survived mechanical ventilation for respiratory distress syndrome (N = 99) and prolonged apnea (N = 43) were reviewed. Thirty-seven infants had bronchopulmonary dysplasia (BPD) and 17 of these developed lobar hyperinflation of the right lower lobe and collapse of the right upper lobe. Regional lung function was measured with a xenon 133 technique in three of these infants and in five other patients who either died or were lost to follow-up. All had BPD with right lower lobe overinflation. Ventilation was less in the lower regions than the upper regions bilaterally ( P &lt;.001), indicating that the hyperinfiation of the lower lobes was not compensatory for upper lobe collapse but was due to emphysema. Mean regional perfusion was equal in the upper and lower regions of the chest. This preferential distribution of lobar emphysema and ipsilateral atelectasis in BPD tended to present and regress simultaneously, but in many infants it lasted as long as eight weeks. Only one infant with persistent atelectasis developed pneumonia. The best mode of therapy appears to be supportive.


2001 ◽  
Vol 125 (12) ◽  
pp. 1562-1565
Author(s):  
Camilo Adem ◽  
Marie-Christine Aubry ◽  
Henry D. Tazelaar ◽  
Jeffrey L. Myers

Abstract Context.—Diffuse pulmonary hemorrhage is an uncommon presenting manifestation of angiosarcoma. Objective.—To review the clinical, radiologic, and pathologic findings of patients with metastatic angiosarcoma who presented with diffuse pulmonary hemorrhage. Design.—Patients fulfilling inclusion criteria were identified from the consultation files. Clinical and radiologic data were obtained from referring pathologists. Histologic slides were reviewed in all patients. Results.—Our patients included 6 men and 1 woman, aged 31 to 73 years; 4 patients were younger than 40 years. Six patients presented with hemoptysis, and all had diffuse abnormalities on radiographic studies. Clinical considerations prior to biopsy included pulmonary hemorrhage syndrome (n = 2), acute respiratory failure (n = 1), and infection (n = 1). Metastatic disease was included in the differential diagnosis in only 1 patient. None had a diagnosis of malignancy prior to lung biopsy. All biopsies showed hemorrhage associated with atypical epithelioid and spindle cells, forming anastomosing vascular channels, distributed along and within lymphatics and arteries. Neoplastic cells were immunoreactive for factor VIII–related protein or CD31 in all cases for which special stains were available. Three patients with complete follow-up died of their disease. Primary sites were discovered in all 3 patients who underwent autopsy examination. Two tumors arose in the heart and 1 in the pelvic soft tissues. One additional patient had a likely primary site identified in the right atrium by cardiac ultrasound and was subsequently lost to follow-up. Conclusion.—Angiosarcoma should be included in the differential diagnosis of diffuse pulmonary hemorrhage, especially in young adults.


Author(s):  
Maria de Lourdes de Abreu FERRARI ◽  
Eduardo Garcia VILELA ◽  
Luciana Costa FARIA ◽  
Claudia Alves COUTO ◽  
Célio Jefferson SALGADO ◽  
...  

Whipple's disease (WD) is a rare systemic disease of infectious etiology which involves the small intestine but can virtually affect any organ. We present here five cases (four males and one female) ranging in age from 20 to 59 years. All patients had intestinal involvement associated or not with clinical manifestations linked to this organ. Vegetation in the tricuspid valve was observed in one patient, suggesting endocarditis caused by Tropheryma whippelii, with disappearance of the echocardiographic alterations after treatment. In one of the male patients the initial clinical manifestation was serologically negative spondylitis, with no diarrhea occurring at any time during follow-up. Ocular involvement associated with intestinal malabsorption and significant weight loss were observed in one case. In the other two cases, diarrhea was the major clinical manifestation. All patients were diagnosed by histological examination of the jejunal mucosa and, when indicated, of extraintestinal tissues by light and electron microscopy. After antibiotic treatment, full remission of symptoms occurred in all cases. A control examination of the intestinal mucosa performed after twelve months of treatment with sulfamethoxazole-trimethoprim revealed the disappearance of T. whippelii in four patients. The remaining patient was lost to follow-up.


2020 ◽  
Vol 11 (2) ◽  
pp. 177-180
Author(s):  
Bader AlQahtani ◽  
Ahmad Abdelaal ◽  
Ehab Karamallah

Purpose: The aim of this report is to describe an unusual case of cataractous lens extrusion through a diseased cornea in the absence of any history of trauma or surgery. Patient and Methods: An elderly female with a history of blepharitis, corneal vascularization, and scarring presented with foreign body sensation, mild ocular pain, and a brunescent cataractous lens extruding through an area of corneal melt of the right eye after being lost to follow-up. The patient was operated urgently and then referred to a specialized eye hospital for further management. Conclusion: In the setting of social neglect and poor compliance to treatment and appointments, consequences of common ophthalmic conditions can lead to serious vision and organ-threatening complications. These might require prompt medical and surgical attention.


BMC Neurology ◽  
2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Hongfeng Wen ◽  
Di Jin ◽  
Lina Cai ◽  
Tao Wu ◽  
Haichao Liu

Abstract Background Brucellosis is a common zoonotic disease that may have a variety of clinical manifestations when it affects the nervous system. Ischemic stroke is a rare clinical symptom, but if it is not diagnosed and treated early, it may cause more severe consequences. Case presentation We report a 38-year-old man presenting with hearing impairment for four years and sudden weakness of the right limb for two years, recurrent aphasia, and gradual weakness of bilateral lower limbs for nine months. He had bilateral positive Babinski’s sign. Cerebrospinal fluid (CSF) showed raised protein and pleocytosis. Magnetic resonance imaging (MRI) showed ischemic infarcts in the pons and extensive enhancement of spinal meninges combined with spinal cord atrophy and ischemia. The tests revealed Brucella Rose Bengal positive in serum and CSF. Brucella culture in CSF was also positive. Next-generation sequencing (NGS) of CSF revealed positive for Brucella with 105 species were detected. He showed significant improvement with antibiotics at five months follow-up. Conclusions Neurobrucellosis may mimic stroke and transverse myelitis like syndromes. NB is a treatable infectious condition and should always be considered in the differentials, especially if there are risk factors, as in our case.


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