Rubeoza tęczówki, ciężka niewydolność oddechowa i retinopatia wcześniaków – opis przypadku

The aim: Case study reports for the first time about development of massive iris neovascular complication in course of retinopathy of prematurity related to systemic and ocular ischemic syndrome due to tracheostomy-requiring extremely severe premature respiratory failure. Material and method: Premature female, 950 grams birth weight, born from 17-year-old gravida 1, at 28 weeks’ gestation by cesarean section due to premature placental abruption with threatening hemorrhages, with 1 to 5 Apgar score. The baby developed severe respiratory failure which required tracheostomy, advanced bronchopulmonary dysplasia treated with steroids (BPD) and respiratory distress syndrome (RDS) with failure to extubate together with secondary ocular ischemia. All the mentioned with multifactorial organs complications (NEC, leucopenia, anemia, pneumonia, periventricular leucomalacia, electrolyte abnormalities and metabolic acidosis) resulted in massive peripupillary iris neovascularization (NVI) in both eyes coexisting with retinopathy of prematurity (ROP) in 38 weeks’ PMA infant. Ultrasonography-B, slit-lamp and indirect fundus examinations with photography were used to document focusing ocular diagnosis. The previous retinopathy of prematurity screening examinations performed at standard intervals of time starting from four weeks of life, that is 32 weeks’ PMA continuing every two weeks did not present typical lesions seen in retinopathy, however in the second zone of retina slightly marked “plus sign” was visible. Ophthalmological examination at 38 weeks’ PMA disclosed massive, round capillary dilatation of the minor arterial circle, spreading to the iris periphery form pupillary margin, and narrow pupils which did not fully react to mydriasis. The intraocular pressure was normal. Ultrasound examination revealed major thickening and echo increase of peripheral retina with its partial detachment. Result: Diode-laser pan-retinal photocoagulation and cryoapplication was performed leading to successful regression of neovascular anterior segment (rubeosis was no longer visible by slit-lamp examination) as well as inhibition of retinopathy of prematurity progression. Conclusion: In described preterm infant, vasoactive molecules released by the ischemic retina may have induced vasodilation of iridal vessels, therefore increasing the perfusion of segments of the minor arterial circle, rendering them clinically visible by examination. It has been observed that hypoxia besides other inflammatory risk factors may have significant influence not only on the development of severe anterior-neovascular complications but also on rapid progression of advanced ROP stages with omission of typical clinical stages of ROP. Simultaneously retinal laser-panphotocoagulation with cryotheraphy seems to be the beneficial method for treatment of these complications. The therapy is effective and destroys the cells that produce Vascular Endothelial Growth Factor (VEGF) which is known to be the most important key factor in the progression of ROP.Cel pracy: Pierwsze doniesienie, na podstawie opisu przypadku, o rozwoju masywnej rubeozy tęczówki w przebiegu retinopatii wcześniaków, powikłanej układowym i ocznym zespołem niedokrwienia wymagającym tracheostomii z powodu skrajnie ciężkiej niewydolności oddechowej u wcześniaka. Materiał i Metody: Wcześniak, płci żeńskiej, urodzony z masą ciała 950 gram, w 28-ym tygodniu życia płodowego przez cięcie cesarskie z powodu przedwczesnego oddzielenia łożyska z zagrażającym krwawieniem, od 17-letniej ciężarnej, ze skalą Agar od 1-5. U dziecka rozwinęła się ciężka niewydolność spowodowana zespołem zaburzeń oddychania (ZZO) z zaawansowaną dysplazją oskrzelowo-płucną wymagająca tracheostomii i włączenia steroidoterapii. Zaburzeniom tym towarzyszyły objawy wtórnego niedokrwienia gałki ocznej. Wszystkie wymienione powikłania wraz z wielonarządowymi takimi jak (martwicze zapalenie jelit, leukopenia, anemia, zapalenie płuc, leukomalacje okołokomorowe, nieprawidłowości elektrolitowe i kwasica metaboliczna) skutkowały masywną okołoźreniczną neowaskularyzacją w obu oczach (NVI) z towarzyszącą retinopatią wcześniaczą (ROP) w 38 tygodniu PMA. W badaniach: ultrasonograficznym B, w lampie szczelinowej, w badaniu pośrednim dna oka oraz w wykonanej fotografii dna oka udokumentowano rozpoznanie okulistyczne. Poprzednie badania przesiewowe w kierunku retinopatii wcześniaczej przeprowadzone w standardowych odstępach czasu – co 2 tygodnie, rozpoczynając od czwartego tygodnia życia, czyli 32 tygodniu PMA, nie wykazały wówczas typowych zmian obserwowanych w retinopatii, natomiast ujawniły słabo zaznaczony objaw plus w drugiej strefie siatkówki. Ciśnienie wewnątrzgałkowe było w granicach normy. W dalszym przebiegu choroby, badanie USG wykazało znaczące pogrubienie oraz hiperechogeniczność obwodowej siatkówki z częściowym jej odwarstwieniem. Wyniki: W leczeniu zastosowano panfotokoagulację siatkówki laserem-diodowym wraz z jednoczasową krioaplikacją poprzeztwardówkową, uzyskując wycofanie neowaskularyzacji (rubeoza tęczówki niewidoczna w badaniu lampą szczelinową). Wnioski: W przypadku opisywanego wcześniaka, wazoaktywne cząsteczki uwolnione przez niedokrwioną siatkówkę mogły wywołać poszerzenie naczyń tęczówki, tym samym, zwiększyć przepływ w kole tętniczym mniejszym co uwidoczniono w badaniu. Zaobserwowano, że niedotlenienie, obok innych zapalnych czynników ryzyka może mieć istotny wpływ nie tylko na rozwój ciężkiej neowaskularyzacji przedniego odcinka oka, ale również na szybką progresję zaawansowanych stadiów ROP z pominięciem typowych dla przebiegu klinicznego stopni tego schorzenia. Panfotokoagulacja siatkówki wydaje się być korzystną metodą leczenia obserwowanych powikłań. Terapia jest skuteczna i niszczy komórki produkujące czynnik wzrostu śródbłonka naczyniowego (VEGF), który uważany jest za kluczowy w rozwoju ROP

Clinical Outcomes after Uncomplicated Cataract Surgery with Implantation of the Tecnis Toric Intraocular Lens

Purpose. To evaluate the clinical outcomes after uncomplicated cataract surgery with implantation of an aspheric toric intraocular lens (IOL) during a 6-month follow-up. Methods. Prospective study including 27 consecutive eyes of 18 patients (mean age: 66.1±11.4 years) with a visually significant cataract and corneal astigmatism ≥ 0.75 D and undergoing uncomplicated cataract surgery with implantation of the Tecnis ZCT toric IOL (Abbott Medical Optics). Visual, refractive, and keratometric outcomes as well as IOL rotation were evaluated during a 6-month follow-up. At the end of the follow-up, patient satisfaction and perception of optical/visual disturbances were also evaluated using a subjective questionnaire. Results. At 6 months after surgery, mean LogMAR uncorrected (UDVA) and corrected distance visual acuity (CDVA) were 0.19±0.12 and 0.14±0.10, respectively. Postoperative UDVA of 20/40 or better was achieved in 92.6% of eyes. Mean refractive cylinder decreased significantly from −3.73±1.96 to −1.42±0.88 D (p<0.001), while keratometric cylinder did not change significantly (p=0.44). Mean absolute IOL rotation was 1.1±2.4°, with values of more than 5° in only 2 eyes (6.9%). Mean patient satisfaction score was 9.70±0.46, using a scale from 0 (not at all satisfied) to 10 (very satisfied). No postoperative optical/visual disturbances were reported. Conclusion. Cataract surgery with implantation of the Tecnis toric IOL is an effective method of refractive correction in eyes with corneal astigmatism due to the good IOL positional stability, providing high levels of patient’s satisfaction

The assessment of reversibility in airway obstruction

In the medical literature there have been described some methods to determine bronchodilator response. They are different because of the required percentage increase in FEV1 and /or FVC and the way of expressing results -as the percent of predicted or initial value. This study on 38 subjects evaluated that there is better bronchodilator responses when the results are presented as the percentage of the initial value compared with the predicted value. The intensivity of impovement in FEV1 depends on the baseline airway obstruction. There are more meaningfull responses in subjects with &#8217;poor&#8217; initial value when using the criteria of change in FEV1 as a percentage of initial value. About 10% of positive responses are misunderstood as &#8216;poor&#8217; when it was considered improvement only in FEV1 (they are positive with reference only to FVC). Pneumonol. Alergol. Pol. 2004, 72, 499:50

Large deletion causing von Hippel-Lindau disease and hereditary breast cancer syndrome

Patients with intragenic mutations of the VHL gene have a typical disease presentation. However in cases of large VHL gene deletions which involve other genes in the proximity of the VHL gene a presentation of the disease can be different. To investigate whether large VHL deletions that remove the FANCD2 gene have an effect on the disease phenotype, we studied a family with a 50 kb large deletion encompassing these two genes. Four patients in this family were affected by VHL-related lesions. However one carrier of the deletion also had bilateral ductal breast cancer at age 46 and 49. Both tumors were of ~2 cm in diameter. On one side lymph nodes were affected. One tumor was ER- and PR-negative (HER2 s unknown) and the second was ER- and PR-positive, and HER2-negative. Our study suggests that a deletion of FANCD2 gene, an important gene in the DNA repair pathway, may be associated with an increased risk of breast cancer, but further studies are needed in this regard

Ocular manifestations in a newborn from a pregnancy complicated by an antiphospholipid syndrome – a case report

The paper presents a case of ophthalmologic manifestations, episcleritis and retinal branch vein thrombosis, in a neonate born to a mother with antiphospholipid syndrome (APS) in the course of systemic lupus erythematosus. Female neonate (birth weight 1150 g, Apgar scores 6, 7 and 7) was born with respiratory distress syndrome, moderate anemia and grades I and II intraventricular hemorrhage. Ophthalmic examination revealed an enormous pre-retinal hemorrhage with accompanying thrombotic changes typical of retinal vein in the fundus of the left eye. Episcleritis was found in the anterior segment of the eye and later confirmed by ultrasound. Laboratory tests showed increased levels of maternal antibodies aCL IgG, antyβ2GP1 IgG and antyβ2GP1 IgM. No ANA, LA and SS-A/SS-B antibodies were detected. Increased concentrations of aCL IgG and a lengthening APTT were observed in newborn blood at first but the parameters normalized by 6 months of age. Our observations allowed us to conclude that early examination of neonates born to mothers with antiphospholipid syndrome is essential as it allows fast identification of pathological retinal changes by means of assessing the presence of aPL antibodies

Epithelioid haemangioendothelioma of the lungs

Authors present a case of EH as an accidental finding in 40 year old woman appeared as the presented small, solid nodules in both lungs on a routine chest radiograph and HRCT scans. There were no signs in clinical examination, no abnormalities were presented in the spirometric and respiratory tests, neither in the endoscopic digestive tract examinations nor in CT scans of abdomen. The histological examination of open lung biopsy revealed the morphology of epithelioid haemangioendothelioma. During one year follow up no clinical nor radiological progressions have been observed