Indirect cylopexy in cases with cyclodialysis using flanged prolene cylopexy technique.

This study aims to describe a new technique of indirect cyclopexy in the treatment of cyclodialysis. Cases who had cyclodialysis for more than 3 h of extent and were resistant to medical treatment underwent flanged prolene cyclopexy (FPC) technique. Five cases were included in this study. Mean duration between the initial surgery and FPC was 4.6 ± 4.3 months. Hypotony maculopathy was present in all cases. All cases underwent FPC in combination with pars plana vitrectomy and silicone oil injection. Circumferential FPC was applied in four cases, while focal FPC was performed in one case. Postoperatively, no complications occurred, visual acuity increased in four cases, and cyclodialysis closed in all cases. In the follow-up, silicone oil was removed in three cases, but silicone oil could not be removed in two cases with ongoing hypotony. Preliminary results showed that FPC is a promising technique in the surgical treatment of cyclodialysis.

RESULTS OF TWO CASES WITH DISTINCT COMPLICATIONS DUE TO INTRAVITREAL BEVACIZUMAB IN RETINOPATHY OF PREMATURITY.

PURPOSE: To present the surgical outcome and long-term follow-up of 2 cases who underwent intravitreal bevacizumab injection for retinopathy of prematurity (ROP) and subsequently developed full thickness macular hole and endophthalmitis. METHODS: Case report. PATIENTS: The first case is a premature girl who was delivered 650 g at the 27th week of postmenstrual age (PMA) because of preeclampsia and received intravitreal 0.16 mg bevacizumab bilaterally at the 38th PMA with the diagnosis of ROP. She was referred with endophthalmitis 10 days after injection in the left eye. Endophthalmitis resolved with pars plana vitrectomy that was performed the following day. The second case is a premature girl who was delivered at the 30th week of PMA weighing 1,230 g and received intravitreal 0.16 mg bevacizumab injections bilaterally at the 39th PMA with a diagnosis of ROP. Retinopathy of prematurity regressed in the follow-up; however, full thickness macular hole was noted in the right eye on the first week of the injection. Pars plana vitrectomy, internal limiting membrane peeling, and gas tamponade were performed at the 41st PMA week. Full thickness macular hole persisted despite pars plana vitrectomy whereas glaucoma emerged during the follow-up. CONCLUSION: Development of endophthalmitis and macular hole may occur after intravitreal injections in cases with ROP.

NONPERFORATING INTRASCLERAL STABBING FOR SUTURELESS 23G SCLEROTOMY CLOSURE.

PURPOSE: The need for suturing leaking sclerotomies have not been eliminated completely in transconjunctival sutureless vitrectomy (TSV). This study aims to describe a novel technique for 23-gauge (G) sclerotomy closure in TSV surgery and discuss its effectiveness. MATERIALS AND METHODS: Two hundred and thirty cases of 180 patients who underwent 23G TSV with various diagnoses were included to the study. Cases with connective tissue diseases, thin sclera, and significant conjunctival and scleral scarring were excluded. Nonperforating intrascleral stabbing (NIS) was performed to leaking 23G sclerotomies (n = 650) and 27G chandeliers (n = 84). Demographics, rate of sclerotomy closure with NIS procedure, the need for suturing, and complications of the procedure were recorded. RESULTS: The overall success of NIS was found to be 91.0% in sclerotomies. Although 9.1% of sclerotomies required sutures, 592 of the 650 sclerotomies could be closed with NIS procedure ( P < 0.001). Nonperforating intrascleral stabbing procedure helped close 98.8% of leaking 27G chandeliers. An average of 1.28 ± 0.52 NIS attempts were needed for successful sclerotomy closure. Closure of sclerotomies with the NIS technique were somewhat related to the location of the sclerotomy, history of TSV and NIS, duration of TSV, and type of endotamponade. Hypotonia and choroidal detachment were seen in one case. No additional interventions were needed to normalize intraocular pressure in any case. Subconjunctival hemorrhage happened to be the leading complication of the procedure. CONCLUSION: NIS procedure seems as a practical, reproducible, cost-effective, and uncomplicated approach, which significantly reduces the need to suture 23G sclerotomies. Further studies are required.

Anti-Tumor Necrosis Factor Treatment in the Management of Pediatric Noninfectious Uveitis: Infliximab Versus Adalimumab.

Purpose: Management of uveitis displays a particular challenge in childhood. This study aims to compare the efficacy and safety of infliximab (IFX) and adalimumab (ADA) in pediatric noninfectious uveitis that were refractory to conventional immunosuppresives. Methods: This retrospective single-center study included 33 patients who were treated with anti-tumor necrosis factor (TNF) agents (16 with IFX and 17 with ADA). Patients had diverse etiologies, including juvenile idiopathic arthritis, idiopathic uveitis, and Behçet's disease. Demographic characteristics, systemic diagnosis, findings of the ophthalmological examination, control of ocular inflammation, response to treatment, and the rate of clinical remission were studied. Results: Fourteen (87.5%) patients receiving IFX and 10 (58.8%) patients receiving ADA achieved response to treatment during the follow-up (P = 0.118). The agents were discontinued with complete clinical remission in 6 (37.5%) patients receiving IFX and in 2 (11.8%) patients receiving ADA (P = 0.118). Baseline visual acuities and parameters of inflammation improved significantly in both groups after anti-TNF therapy. Conclusion: Both IFX and ADA are safe and effective for pediatric noninfectious uveitis.

Challenges in the management of an ignored cause of hyperammonemic encephalopathy: pyruvate carboxylase deficiency.

Pyruvate carboxylase (PC) deficiency is a rare autosomal recessive disease and provides clinics in three essential phenotypes. Type B PC deficiency is characterized by lactic acidosis and hyperammonemia. We report a Turkish patient who was diagnosed with type B PC deficiency. Despite the application of anaplerotic treatment with biotin, citrate and arginine-aspartate, continuous veno-venous hemodialysis (CVVHD) treatments were applied due to the failure to keep hyperammonemia and lactic acidosis under control. Ammonia values increasing to 860 µmol/L were observed. A homozygous novel variant was detected in PC gene analyses containing a 12-base pair deletion on exon 8. Although the mutation found was not reported previously, it was accepted as a pathogenic variant due to its presence in a functional region of the protein. In type B PC deficiency, although a high level of ammonia is expected, it rarely exceeds 200 µmol/L. As far as we know, the present case has the highest ammonia values in the literature. This paper has been shared to highlight to keep PC deficiency in mind regarding the differential diagnosis of hyperammonemia, particularly in the presence of lactic acidosis, and to serve as a model for the use of different modalities in the management process of PC deficiency.

Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant.

INTRODUCTION: Abernethy malformation is a very rare condition in which mesenteric venous blood is drained into systemic circulation by an extra hepatic portosystemic shunt. Here we present a case of a female in infantile period who is a liver transplant candidate with biliary atresia and ventricular septal defect (VSD) accompanying Abernethy malformation type 2. CASE REPORT: The patient who underwent Kasai operation at postnatal day 35 was identified as a liver transplant candidate in the postnatal month 6 due to growth retardation in the infantile period, insufficient weight gain and liver failure (portal hypertension and massive gastrointestinal bleeding). CONCLUSION: Abernethy malformation is divided into two groups as type 1 and type 2; type 2 shunts, which are less common, but more common in males, are not accompanied by other congenital anomalies. Due to the female gender, having biliary atresia and VSD, the development of liver failure at an early stage despite undergoing Kasai operation at the appropriate time, our case differs from the literature. In conclusion, this case is presented in order to indicate the proper and effective use of imaging methods in neonatal cholestasis cases.How to cite this article: Ergon EY, Ermis N, Colak R, Polat B, Ozdemir S, Yildiz M, Kulali F, Ecevit C, Ergun O, Calkavur S. Abernethy Malformation Type 2 and Biliary Atresia Coexistence: A Rare Cause of Infantile Liver Transplant. Euroasian J Hepatogastroenterol, 2018;8(2):163-166.

The role of serial measurements of serum insulin-like growth factor 1 levels in the development of retinopathy of prematurity.

AIM: To determine the role of serum insulin-like growth factor-1 levels in the development of retinopathy of prematurity, which is a major cause of childhood blindness worldwide. MATERIAL AND METHODS: We prospectively studied newborn infants born at a postmenstrual age of <32 weeks and birth weights <1 500 gr, between January 1st, 2015, and December 31st, 2015. A total of 40 infants were enrolled in the study. The retinal examination time was determined in accordance with the American Academy of Pediatrics recommendations for retinopathy of prematurity screening and follow-up. Retinopathy of prematurity was classified according to the international classification of retinopathy of prematurity. Serum Insulin like growth factor 1 levels were measured serially in blood samples on the 1st, 3rd, 7th, 21st, and 28th day. RESULTS: Among the 40 infants, 11 (27.5%) constituted the retinopathy of prematurity group and 29 comprised the non-retinopathy of prematurity group. In the retinopathy of prematurity group, the mean gestational age and birth weight was significantly lower. The demographic features of the study cohort were similar. The duration of mechanical ventilation was significantly greater in the retinopathy of prematurity group compared with the non-retinopathy of prematurity group (p=0.036). In terms of neonatal morbidities such as respiratory distress syndrome, intraventricular hemorrhage, bronchopulmonary dysplasia, patent ductus arteriosus, and necrotizing enterocolitis, no differences were detected between the groups. The mean serum insulin-like growth factor-1 levels in retinopathy of prematurity group were significantly lower than those in the non-retinopathy of prematurity group at each time point (1st, 3rd, 7th, 21st, and 28th day of postnatal life) (p=0.001). CONCLUSIONS: This study demonstrated the low serum insulin-like growth factor-1 levels was associated with retinopathy of prematurity development.

Spectral domain optical coherence tomography findings of patients under treatment for pediatric acute lymphoblastic leukemia.

PURPOSE: To investigate the use of spectral domain optical coherence tomography (SD-OCT) findings in pediatric acute lymphoblastic leukemia (ALL) patients. METHODS: Children that were diagnosed with precursor B-cell ALL and classified as belonging to the medium-risk group for relapse were selected for this study. Individuals who were in continuous remission and on maintenance therapy were included in the study group. Cases that had central nervous system involvement were excluded. Age-matched, otherwise healthy children were selected for the control group. Each study participant underwent a comprehensive eye examination and SD-OCT evaluation. Thickness measurements were made within the retinal nerve fiber layer (RNFL), central macula, posterior polar, and peripapillary choroid. RESULTS: A total of 112 eyes of 56 children were included: 54 eyes in the study group and 58 in the control group. Compared to the control group, subfoveal and temporal choroidal thicknesses of the posterior pole were significantly thinner in the study group (P < 0.005). Similarly, peripapillary choroidal thicknesses were significantly thinner in most sectors of the study group (P < 0.005). There were no major differences between groups in terms of central macular thicknesses and overall RNFL thicknesses. CONCLUSIONS: Evidence of choroidal attenuation was found in this subgroup of pediatric ALL patients. Further studies are warranted to clarify the utility of SD-OCT in detecting subclinical ocular involvement and monitoring treatment response and risk of relapse in patients with pediatric leukemia.

Comparison of Retinal Nerve Fiber Layer Thickness in Terms of Birth Weight in Prematurely Born Children.

PURPOSE: To study the potential effects of both prematurity and the sub-groups of low birth weight on thickness of RNFL. METHODS: Prospective case series of 26 preterm school-aged children with low birth weight whose retinal nerve fiber layer analyses with RTVue-100 Fourier-domain optic coherence tomography were performed in 2013 at the Department of Ophthalmology, Erzurum Region Education and Training Hospital. RESULTS: The mean retinal nerve fiber layer thicknesses were 100.6 ± 13.3 microns in extremely low birth weight, 103.9 ± 8.4 microns in very low birth weight, and 104.1 ± 10.8 microns in low birth weight groups. There was no significant difference in RNFL among the groups. CONCLUSIONS: No significant relationship was found between birth weights and retinal nerve fiber layer thickness of preterm children who were appropriate for gestational age.

An Important Cause of Blindness in Children: Open Globe Injuries.

Objective. Our aim was to present and evaluate the predictive factors of visual impairment and blindness according to WHO criteria in pediatric open globe injuries. Methods. The medical records of 94 patients younger than 18 years who underwent primary repair surgery were reviewed retrospectively. The initial and final visual acuity, anterior and posterior segment findings, and zone of injury were noted. The patients were classified as blindness in one eye or visual impairment in one eye. Results. Of 412 patients who presented with open globe injury, 94 (23%) were under 18 years old. Fifty-four (16 females, 38 males) children were included. The mean age of the children was 7.1 ± 4.1 years. According to WHO criteria, 19 of 54 patients (35%) had unilateral blindness and 8 had unilateral visual impairment (15%). There was no significant relationship between final visual acuity and gender and injured eye. In visually impaired and blind patients, presence of preoperative hyphema, retinal detachment, and zone 2 and zone 3 injuries was significantly higher. Conclusion. Presence of hyphema and zone 2 and zone 3 injuries and retinal detachment may end up with visual impairment and/or blindness in children.

Investigation of TNF-alpha gene (G308A) and GSTP1 gene (Ilel05Val) polymorphisms in Turkish patients with retinopathy of prematurity.

BACKGROUND/AIM: Retinopathy of prematurity (ROP) is one of the most frequent causes of blindness in newborn babies. Currently, its etiology is not fully understood.-In this study we aimed to investigate the correlation between a patient group with ROP and a control group in terms of the tumor necrosis factor-alpha (TNF-alpha) (G308A) gene and glutathione-S-transferase P1 (GSTP1) (Ilel05Val) gene polymorphism. MATERIALS AND METHODS: Sixty-two patients diagnosed with ROP and 58 control subjects were included in this study. For TNF-alpha (G308A) gene and GSTP1 (Ilel05Val) gene polymorphisms, the polymerase chain reaction-restriction fragment length polymorphism method was used. In statistical analysis the significance level was determined as P < 0.05. RESULTS: When the patient and control groups were compared in terms of TNF-alpha (G308A) gene and GSTP1 (Ilel05Val) gene polymorphisms, no statistically significant difference was found (P > 0.05). CONCLUSION: In our study, no correlation was identified between TNF-alpha (G308A) gene and GSTP1 (Ilel05Val) gene polymorphisms and susceptibility for development of ROP. Further studies are required with more cases of ROP patients and other gene polymorphisms that could be related.

The importance of microalbuminuria in predicting patient outcome in a PICU.

OBJECTIVE: To evaluate the prognostic significance of microalbuminuria in critically ill children. DESIGN: Prospective study. SETTING: PICU of a teaching hospital. PATIENTS: Admitted critically ill children. INTERVENTIONS: The urine albumin-creatinine ratio was measured at admission and at 24 hours. Pediatric Risk of Mortality, Pediatric Index of Mortality II, Pediatric Logistic Organ Dysfunction, and Inotrope Score were calculated. MEASUREMENTS AND MAIN RESULTS: In total, 102 patients (median age, 19 mo) were included in the study, among whom were 30 mortalities. Microalbuminuria was identified in 62 patients (64%). The patients were classified into three groups: patients with sepsis, patients with noninfectious systemic inflammatory response syndrome, and patients without systemic inflammatory response syndrome. The highest clinical scores, albumin-creatinine ratio levels, mortality rate, and duration of mechanical ventilation were found in the sepsis group, and the lowest values were seen in patients without systemic inflammatory response syndrome (p < 0.05). Significant correlations were observed between the albumin-creatinine ratio levels and the clinical scores (p < 0.05). The receiver operating characteristics curve analysis showed that the areas under the curves were 0.818 and 0.781, respectively, for albumin-creatinine ratio measured at admission and at 24 hours to identify PICU mortality. At a cutoff value of 34.2 mg/g, albumin-creatinine ratio measured at admission may be able to discriminate between patients a with sensitivity of 63.3%, specificity of 93.3%, positive predictive value of 95%, and negative predictive value of 56%. CONCLUSIONS: Microalbuminuria is a simple, inexpensive, and useful tool for predicting mortality and morbidity in critically ill children in the PICU.

Sequential anti-cytomegalovirus response monitoring may allow prediction of cytomegalovirus reactivation after allogeneic stem cell transplantation.

BACKGROUND: Reconstitution of cytomegalovirus-specific CD3(+)CD8(+) T cells (CMV-CTLs) after allogeneic hematopoietic stem cell transplantation (HSCT) is necessary to bring cytomegalovirus (CMV) reactivation under control. However, the parameters determining protective CMV-CTL reconstitution remain unclear to date. DESIGN AND METHODS: In a prospective tri-center study, CMV-CTL reconstitution was analyzed in the peripheral blood from 278 patients during the year following HSCT using 7 commercially available tetrameric HLA-CMV epitope complexes. All patients included could be monitored with at least CMV-specific tetramer. RESULTS: CMV-CTL reconstitution was detected in 198 patients (71%) after allogeneic HSCT. Most importantly, reconstitution with 1 CMV-CTL per µl blood between day +50 and day +75 post-HSCT discriminated between patients with and without CMV reactivation in the R+/D+ patient group, independent of the CMV-epitope recognized. In addition, CMV-CTLs expanded more daramtaically in patients experiencing only one CMV-reactivation than those without or those with multiple CMV reactivations. Monitoring using at least 2 tetramers was possible in 63% (n = 176) of the patients. The combinations of particular HLA molecules influenced the numbers of CMV-CTLs detected. The highest CMV-CTL count obtained for an individual tetramer also changed over time in 11% of these patients (n = 19) resulting in higher levels of HLA-B*0801 (IE-1) recognizing CMV-CTLs in 14 patients. CONCLUSIONS: Our results indicate that 1 CMV-CTL per µl blood between day +50 to +75 marks the beginning of an immune response against CMV in the R+/D+ group. Detection of CMV-CTL expansion thereafter indicates successful resolution of the CMV reactivation. Thus, sequential monitoring of CMV-CTL reconstitution can be used to predict patients at risk for recurrent CMV reactivation.

Herpes zoster ophthalmicus and lateral rectus palsy in an elderly patient.

Acquired palsy of the lateral rectus presents with horizontal diplopia and has a broad differential. Herpes zoster ophthalmicus- (HZO) related cranial nerve palsy is a transient and self-limiting condition. Systemic antiviral treatment is administered in order to prevent sight-threatening complications. In suspected cases, zosteriform rash should be questioned. One should keep in mind that acquired esotropia in the elderly may sometimes present following HZO.

Maternal preeclampsia is associated with an increased risk of retinopathy of prematurity.

OBJECTIVE: To determine the effect of maternal preeclampsia on development and severity of retinopathy of prematurity (ROP) in preterm infants. METHODS: This prospective study consisted of two groups: the study group, which is composed of preterm infants (≤32 weeks) born to a mother with preeclampsia, and the comparison group, which is composed of preterm infants (≤32 gestational age) born to normotensive mothers. We used the International Classification of Retinopathy of Prematurity Revisited for classifying ROP. The first eye examination was performed at postnatal age of 4 weeks. RESULTS: A total of 385 infants were included in the study. ROP was diagnosed in 109 infants (28%). The incidence of ROP in infants born to preeclamptic mothers (40.5%) was significantly higher compared with those born to normotensive mothers (22.4%) (P<0.05). The number of infants with stage 1, 2, and 3 ROP was significantly higher in infants born to preeclamptic mothers compared with the control group (P<0.05). In multiple logistic regression model, preeclampsia was found to predict ROP (odds ratio 1.78, 95% confidence interval 0.66-1.90). CONCLUSION: Maternal preeclampsia was found to be associated with increased ROP development risk in premature infants. ROP was also more severe in infants born to pre-eclamptic mothers. The role of maternal preeclampsia in the occurrence and severity of ROP remains to be elucidated.

Induction of bilateral ligneous conjunctivitis with the use of a prosthetic eye.

The purpose of this article to report a case of ligneous conjunctivitis in an anophthalmic socket, in respect of a 20-year-old woman. The subject woman had a history of left enucleation surgery presented with bilateral palpebral ligneous conjunctivitis and ligneous gingivitis. The hematologic study revealed a severe plasma plasminogen deficiency. The eyelid lesions were successfully treated with surgical excision, topical heparin and corticosteroid eyedrops. However, the ligneous lesions recurred bilaterally after she was fitted with a prosthetic eye and were refractory to intensive topical treatment with heparin and cyclosporin A eye drops. This case shows that the use of a prosthetic eye may induce ligneous conjunctivitis in an anophthalmic socket and normal eye which is refractory to topical treatment.