CHOROIDAL NEVUS ASSOCIATED WITH VITELLIFORM DEPOSITION IN A PATIENT WITH AUTOSOMAL DOMINANT BEST DYSTROPHY.

BACKGROUND/PURPOSE: To describe the clinical, optical coherence tomography (OCT), fundus autofluorescence and ultrasound findings of a patient with a choroidal nevus actively exuding vitelliform material in the setting of autosomal dominant Best dystrophy (BD). METHODS: The patient's clinical course was followed over time with ophthalmic examinations and multimodal imaging. RESULTS: A 71-year-old male patient with BD was referred for evaluation of a choroidal nevus in the right eye. Dilated fundoscopic examination showed a small pigmented choroidal nevus in the temporal periphery. Over a 3-year period, the nevus developed progressive deposition of vitelliform material along its inferior border. Meanwhile, OCT and fundus photography showed only slight growth. Ultrasound showed no change in height; basal measurements were confounded by the increased vitelliform deposits. Genetic testing confirmed a heterozygous mutation in the BEST1 gene and electrophysiology was consistent with BD. CONCLUSIONS: Dysfunction of the retinal pigment epithelium associated with BD may cause novel presentations of other conditions such as choroidal nevi. The implication for malignant transformation of a choroidal nevus associated with vitelliform deposit accumulation in this context is unknown.

Sustained Control of Serpiginous Choroiditis with the Fluocinolone Acetonide 0.18 mg Intravitreal Implant.

Purpose: To describe an alternative treatment for a patient with serpiginous choroiditis (SC) who was not tolerant to systemic therapies. Methods: Case report of a patient with serpiginous choroiditis with their clinical course followed with ophthalmic examinations and multimodal imaging overtime. Patients and Results. A 57-year-old female with serpiginous choroiditis was treated for seven years with numerous therapies including systemic steroids, immunosuppressive agents, and repeated dexamethasone intravitreal implants. The patient was intolerant of systemic therapies and would flare if dexamethasone injections were performed less frequently than every 8 weeks, making a viable long-term treatment plan problematic. Following one injection of the fluocinolone acetonide 0.18 mg intravitreal implant, she has experienced sustained control for 20 months. Discussion and Conclusions. Real-world treatment of SC is complex as long-term control is necessary, and associated side effects of the therapies provided may limit sustained use. The fluocinolone acetonide implant lasts 36 months and may be an alternative long-term management option, especially in the setting of systemic medication intolerance for some patients with SC.

Newer Insights into Prostatic Utricle in Proximal Hypospadias.

Introduction: Prostatic utricles (PUs) are the remnants of Mullerian ducts found in the male posterior urethra. Enlarged symptomatic utricles are known to be associated with hypospadias. There is a dearth of literature defining an enlarged utricle and also its clinical significance. Aims: The aim of this study was to describe anatomical difference of PUs of cases with severe hypospadias and also to find their clinical significance if any. Materials and Methods: A prospective study was carried out, and all patients with proximal hypospadias were enrolled. Cystoscopy was performed in all cases. Symptomatology, size, and location of PU were recorded. PU of >0.5 cm was considered enlarged. Results: In the present series, a total of 70 cases were included over a period of 2 years. Enlarged utricle was found in 47/70 (67.14%) on cystoscopy. They were wide-mouthed and negotiated 9 Fr/11 Fr cystoscope with ease. Recurrent epididymo-orchitis, recurrent urinary tract infection, and obstructive features were the most common complications requiring intervention. Conclusions: PUs in boys with hypospadias are enlarged as well as wide-mouthed. Most of these remain asymptomatic, but few of them carry the potential of complications. Cystoscopy helps in direct visualization of utricular anatomy, so it should be a preferred investigating modality for investigating a PU.

Central Venous Catheter-Related Infectious Complications in Pediatric Surgical Patients: A Single-Center Experience.

The purpose of this study was to estimate the rate of central venous catheter (CVC) colonization and catheter-related bloodstream infections (CRBSIs) in pediatric surgical patients at our institute and to determine the various risk factors for their occurrence. The electronic medical records of 260 children undergoing surgery with simultaneous CVC insertion were retrospectively reviewed. Data on demographics, primary organ system involved, site of CVC, CVC dwell time, CVC colonization, CRBSI, and organisms isolated on culture were collated, categorized, and analyzed. The rate of CVC colonization and CRBSI was 32.8 per 1,000 catheter days (19.6%) and 17.4 per 1,000 catheter days (10.4%), respectively. Patients with CVC colonization and CRBSI had a significantly higher proportion of patients younger than 1 year of age ( p = 0.014). The CVC dwell time was significantly higher in both CVC colonization (7 [5-8] days) and CRBSI (6 [5-9] days) patients ( p = 0.005). The frequency of femoral catheterization was significantly higher in patients with CRBSI and CVC colonization ( p < 0.001). Coagulase negative staphylococcus was the commonest isolate in CVC infections. Age (adjusted odds ratio [OR] = 0.87; p = 0.009), CVC dwell time (adjusted OR = 1.28; p = 0.003), and femoral CVC (adjusted OR = 9.61; p < 0.001) were independent risk factors for CRBSI. Conclusion: This study reveals important observations regarding the infectious complications of CVC in pediatric surgical patients. The rates of CVC colonization and CRBSI in this study were found to be higher as compared with previously reported rates in Western literature. However, these findings are significant in view of paucity of existing literature in pediatric surgical patients. In our study, higher risk of CRBSI was associated with younger age, increasing CVC dwell time, and femoral venous catheterization. We recommend strict compliance with CVC insertion and maintenance practices and adherence to CVC care bundles to minimize these serious complications.

Catheter test: A reliable alternative to assess adequacy of bladder management in patients of PUV in developing countries.

INTRODUCTION: Following posterior urethral valve ablation, persisting bladder dysfunction can lead to lack of improvement or worsening of hydronephrosis and as well, to lack of improvement or even worsening renal function. On the other hand, managing bladder dysfunction offers the possibility of lessening both the degree of hydronephrosis and the level of serum creatinine. As we have no available urodynamics we feel impelled to find other means of diagnosing and managing abnormal bladder dynamics in these boys. We wanted to determine if a short course of continuous bladder drainage would be an effective means of lowering abnormal serum creatinine levels. Based on an assumption that most bladders following valve ablation, especially in those boys with a persistently elevated serum creatinine post valve ablation, have some form of dysfunction, we wanted to determine if a short course of continuous catheter drainage could improve these abnormal serum creatinine levels. METHODS: The study was conducted over a 3year period and included all children up to 3 years of age who had higher age-appropriate nadir serum creatinine, 3 months post valve ablation. Patients were followed up at 1 week, 2 month and 3-month post valve ablation. On third follow-up visit patients with an elevated serum creatinine level were subjected to continuous bladder drainage (catheter test) for 5 days and serum creatinine level was recorded at the end of 5th day. Enforced bladder management which included dose titration of anti-muscaranic drugs and clean intermittent catheterization (CIC) with or without nocturnal catheter drainage (NOC) was instituted for next 3 months. Serum creatinine was determined at the end of the 3 month period and compared with prior values. RESULTS: A total of 21 patients were included in the study. The mean age of the children at the time of valve ablation was 6.97 ± 4.38 months. There was a significant fall in serum creatinine from 0.64 ± 0.12 mg/dl pre 5 days of drainage to 0.48 ± 0.10 at the end of the 5 day period (p = 0.024) along with a significant rise in GFR (p = 0.001). Following 3 months of enforced bladder management, there was a very mild rise in creatinine to 0.51, a normal age adjusted value. DISCUSSION: The study demonstrates the importance of early diagnosis of bladder dysfunction in boys with an elevated serum creatinine levels post valve ablation. Elevated nadir serum creatinine 3 months post ablation seems to be a useful indicator of possible bladder dysfunction; lowering of serum creatinine following a 5 day trial of continuous bladder drainage confirms this diagnosis. However, the lack of availability of urodynamic testing leaves the specific type of bladder dysfunction undiagnosed. CONCLUSION: Lowering of serum creatinine following a 5-day trial of continuous bladder drainage can be used as a rough indicator of existing bladder dysfunction in boys with a history of PUV and should be used in countries without urodynamic equipment as an indicator for at least a 3 month trial of enforced bladder management.

Optimising working space for laparoscopic pyeloplasty in infants: Preliminary observations with the SGPGI Protocol.

AIMS: This study aimed to test the efficacy of SGPGI protocol to minimise bowel distension and optimise working space for laparoscopic pyeloplasty in infants. METHODOLOGY: All infants who underwent laparoscopic pyeloplasty for unilateral pelvi-ureteric junction obstruction (PUJO) between January 2017 and March 2020 were included in the study. The patient cohort was divided into two groups: Group A and B. Group A included patients who underwent routine pre-operative preparation. Group B included patients wherein the SGPGI protocol was used. The key features of the protocol were fasting for 8 h, enemas, inserting a nasogastric tube in the pre-operative period and decompressing the colon on the operation table. Demographic features, pre-operative, intraoperative and post-operative parameters were compared between the two groups. RESULTS: A total of 26 infants with unilateral PUJO underwent laparoscopic pyeloplasty during the study period. Group A included 12 patients and Group B included 14 patients. Both the groups were similar in age, weight and sex distribution. The median surgeon's rating score for suturing conditions was 2 for Group A and 5 for Group B patients (P > 0.05). The operating time was significantly longer in Group A (196 ± 21 min) as compared to Group B (114 ± 18 min) (P < 0.05). In Group A, intra-abdominal pressure (IAP) varied between 9 and 14 mmHg (median 12 mmHg), while in Group B, IAP varied between 6 and 9 mmHg (median 8 mmHg) (P < 0.05). In Group A, in 2/12 cases (16.7%), conversion to an open procedure was necessary because of inadequate working space owing to gross intestinal distension. Two patients in Group A also had intraoperative injuries to adjacent structures due to poor working space. CONCLUSIONS: Optimal working space is critical to the performance of advanced laparoscopic surgery like pyeloplasty in infants. SGPGI protocol significantly improves working space, which permits a faster and safer surgery with a lower intra-abdominal working pressure. This protocol is simple, safe and easy to replicate at most centres in our country.

Delayed referral for orchidopexy: Scrutinising the causes.

Orchidopexy for undescended testis is recommended at a younger age than heretofore; our study aimed to assess delays, and their causes, by retrospective analysis of data from a single tertiary care centre over one year (2015-2016). Almost 80% of children were brought after 1 year of age, mostly because of delayed referral by primary physicians (60%), or missed diagnosis by parents or primary physicians (20%). Misconception about the risk of surgery below 1 year was significant (15%). A timely referral is encouraged.

A Perplexing Case of Abdominal Pain That Led to the Diagnosis of Zollinger-Ellison Syndrome.

Zollinger-Ellison syndrome (ZES) is a rare clinical disorder, characterized by hypersecretion of gastric acid and multiple ulcers distal to the duodenal bulb. This occurs via the release of gastrin by neuroendocrine tumors known as gastrinomas. Patients with ZES present with nonspecific GI symptoms, which often leads to a delay in diagnosis. Our patient is a 55-year-old female with chronic abdominal pain, nausea, and diarrhea. She underwent EGD, EUS, MRCP, CT scans, and cholecystectomy, which did not reveal the cause of her symptoms. Repeat EGD showed a cratered ulcer in the second portion of the duodenum, suspicious for ZES. Serum gastrin was initially only moderately elevated while on PPI therapy, but chromogranin A was also elevated. Repeat gastrin level after stopping PPI therapy was 1639 pg/mL. Somatostatin receptor scintigraphy was obtained, which showed two small lesions in the gastrinoma triangle. She subsequently underwent a Whipple pancreaticoduodenectomy and pathology was positive for four microscopic foci of a neuroendocrine tumor. She reported improvement in her symptoms after surgery. This case highlights the need for increased awareness of ZES in patients with unexplained GI complaints and emphasizes the use of multiple modalities in the diagnosis of ZES.

A rare case of splenic lymphoma in a patient with polymyositis manifesting as gastric variceal bleeding.

We report an unusual case of upper gastrointestinal bleeding due to isolated gastric variceal bleeding in a patient with splenomegaly who was subsequently diagnosed with diffuse large B-cell lymphoma. The patient is a 47-year-old male with a history of polymyositis who presented to the emergency room with complaints of lightheadedness and melena for 2 days. On initial presentation, the patient had positive orthostatic vital signs. He was found to be anemic with presenting hemoglobin of 5.8 g/dl (compared with 13.4 g/dl 4 months prior to presentation). The patient was aggressively resuscitated with intravenous fluid and blood transfusions. An emergency esophagogastroduodenoscopy was performed which showed isolated gastric varices in the fundus of the stomach, with no active bleeding or high-risk stigmata. Abdominal computed tomography revealed focal splenic vein thrombosis and splenomegaly with ill-defined hypodensities. Portal and superior mesenteric veins were patent. Mild edema was seen surrounding the spleen and non-specific abdominal lymphadenopathy was also reported. A surgical consultation recommended an urgent splenectomy. Pathology of the removed spleen revealed diffuse large B-cell lymphoma. Positron emission tomography-computed tomography revealed lymphomatous disease in the thorax, abdomen, pelvis and bone marrow. The patient was subsequently started on chemotherapy.