Relationship between macular intervortex vein anastomosis and optical coherence tomography biomarkers in chronic central serous chorioretinopathy.

BACKGROUNDS: To evaluate the optical coherence tomography (OCT) biomarkers of patients with central serous chorioretinopathy (CSC) according to the presence or absence of direct anatomical relation to intervortex vein anastomosis (IVA) on indocyanine green angiography. METHODS: We reviewed the records of 39 patients with chronic CSC. Patients were categorized in 2 groups: presence of IVA(Group A) or absence of IVA(Group B) in the macular region. Localization of IVA was categorized in 3 areas according to ETDRS grid:inner 1 mm circle (area-1), 1-3 mm middle circle (area-2) and 3-6 mm outer circle (area-3). RESULTS: There were 31 eyes in Group A,21 eyes in Group B. Mean age of the patients was 52.5 ± 11.3years in Group A,47.2 ± 11years in Group B(p<0.001).Mean initial visual acuity (VA) was 0.38±0.38LogMAR in Group A, 0.19±0.21LogMAR in Group B(p<0.001).Mean subfoveal choroidal thickness(SFCT) was 436.3 ± 134.3µ in Group A,480.2 ± 136.6µ in Group B(p<0.001).Localization of IVA in area-1 was correlated with inner choroidal attenuation (ICA) and leakage on IVA(p = 0.011,p = 0.02). Localization of IVA in area-3 was correlated with irregular lesions on RPE(p = 0.042).Smokestack configuration,intraretinal cysts and ICA were correlated with worse initial VA(p<0.001,p = 0.001 and p = 0.04).Shaggy subtype of photoreceptor disruption was associated with better initial VA(p = 0.003). CONCLUSIONS: We detected older age, worse initial VA and thinner SFCT in patients with chronic CSC and macular region IVA(m-IVA). Long term follow-up of patients with and without m-IVA may exhibit the difference in treatment outcomes and development of neovasculopathy.

Comparison of pregnancy outcomes and placental characteristics between selective fetal growth restriction with and without thick arterio-arterial anastomosis in monochorionic diamniotic twins.

BACKGROUND: Unequal placental territory in monochorionic diamniotic twins is a primary cause of selective fetal growth restriction (sFGR), and vascular anastomoses play important role in determining sFGR prognosis. This study investigated differences in placental characteristics and pregnancy outcomes in cases of sFGR with and without thick arterio-arterial anastomosis (AAA). METHODS: A total of 253 patients diagnosed with sFGR between April 2013 and April 2020 were retrospectively analyzed. An AAA greater than 2 mm in diameter was defined as a thick AAA. We compared placental characteristics and pregnancy outcomes between cases of sFGR with and without thick AAA. RESULTS: Prevalence of AAA, thick arterio-venous anastomosis (AVA), veno-venous anastomosis (VVA), and thick VVA were significantly higher in the thick AAA group relative to the non-thick AAA group (100.0 vs. 78.5%, P < 0.001; 44.3 vs. 15.4%, P < 0.001; 27.1 vs. 10.8%, P = 0.017, and 24.3 vs. 6.2%, P = 0.004, respectively). The total numbers of AVA and total anastomoses were significantly higher in thick AAA group relative to the non-thick AAA group (5 [1, 14] vs. 3 [1, 15, P = 0.016; and 6 [1, 15] vs. 5 [1, 16], P = 0.022, respectively). The total diameter of AAA, AVA, VVA, and all anastomoses in the thick AAA group was larger than in the non-thick AAA group (3.4 [2.0,7.1] vs. 1.4 [0.0, 3.3], P < 0.001; 6.3 [0.3, 12.0] vs. 2.5 [0.3, 17.8], P < 0.001; 4.2±1.8 vs. 1.9±1.2, P =0.004; and 10.7 [3.2,22.4] vs. 4.4 [0.5, 19.3], P < 0.001, respectively). Growth-restricted fetuses in the thick AAA group exhibited significantly increased birthweight relative to those in thenon-thick AAA group (1570 (530, 2460)g vs. 1230 (610, 2480)g, p = 0.002). CONCLUSIONS: In the placentas associated with sFGR, thick AAA can co-occur with thick AVA and VVA, and placental angiogenesis may differ significantly based upon whether or not thick AAA is present. The birth weights of growth-restricted fetuses in cases of sFGR with thick AAA are larger than in cases without thick AAA.

Study of the placental characteristics and time of onset of twin-to-twin transfusion syndrome.

INTRODUCTION: To investigate the correlation between placental superficial anastomoses, placental territory and the umbilical cord attachment site with the time of onset of twin-to-twin transfusion syndrome (TTTS), and to explore the influence of placental characteristics on the time of onset of TTTS. METHODS: A retrospective analysis was performed on 48 cases of TTTS managed conservatively at the Obstetrics Department of Peking University Third Hospital from April 2014 to April 2019. Placental superficial anastomoses, placental territory, the distance between the insertion points of the umbilical cord were measured after placental dye injection. Correlation analysis was conducted between placental characteristics and the time of onset of TTTS. RESULTS: (1) The incidence of AA anastomoses was 33.3% (16/48) with a mean total diameter of 2.3 ± 1.4 mm, that of AV anastomoses was 95.8% (46/48) with a mean total diameter of 1.2 ± 0.4 mm, and that of VV anastomoses 22.9% (11/48) with a mean total diameter of 2.3 ± 1.1 mm (2) The time of onset of TTTS was positively correlated with the umbilical insertion ratio (Spearman correlation coefficient = 0.404, P = 0.004), but not correlated with the total diameter of anastomoses in the three different types, or with placental territory discordance. DISCUSSION: The time of onset of TTTS was positively correlated with the umbilical insertion ratio, which suggest that the distance between umbilical cord insertion sites may affect the time of onset of TTTS. The smaller the distance between the umbilical cord insertion sites, the earlier the time of onset of TTTS is likely to be.

Intermittent absent and reversed umbilical artery flows in appropriately grown monochorionic diamniotic twins in relation to proximate cord insertion: A harmful combination?

OBJECTIVE: To compare the prevalence of intermittent absent or reversed end-diastolic flow (iAREDF) in the umbilical artery in appropriately grown monochorionic diamniotic (MCDA) pregnancies with and without proximate cord insertion (PCI), and to evaluate pregnancy outcome. METHODS: The prevalence of iAREDF in MCDA pregnancies with PCI (n = 11) was compared with a control group without PCI (n = 33). PCI was defined as a distance between the cord insertions below the fifth percentile. Placental sharing, number, and diameter of anastomoses were assessed by placental examination. Pregnancy outcome was evaluated. RESULTS: iAREDF was present in 7/11 PCI pregnancies, compared with 0/33 in the control group (P ≤ .01). All PCI pregnancies and 94% of controls had arterioarterial (AA)-anastomoses (P = .56), the diameter was larger in the PCI group, respectively 3.3 vs 2.1 mm (P = .03). Three cases with iAREDF had adverse outcome, two resulted in fetal death of which one with brain damage in the co-twin, another underwent early premature emergency section for fetal distress. CONCLUSION: iAREDF occurs in a large proportion of MCDA pregnancies with PCI and is related to the diameter of the AA anastomosis. We hypothesize that iAREDF in appropriately grown MCDA twin pregnancies reflects an unstable hemodynamic balance with an increased risk for fetal deterioration. Whether outcome in these pregnancies can be improved by altered management requires further investigation.

A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report / Doença renal policística autossômica recessiva, shunt esplenorrenal e fibrose hepática congênita em adolescente: relato de caso

ABSTRACT A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.

RESUMO Relato de caso de uma paciente adolescente de 16 anos de idade com diagnóstico prévio de doença renal policística autossômica recessiva (DRPAR), que apresentou quadro agudo de pneumonia bilateral e hemorragia digestiva alta por ruptura de varizes esofágicas, bem como ascite e edema de membros inferiores. Necessitou de estabilização clínica intensiva e tratamento endoscópico do sangramento digestivo. Após investigação dos diagnósticos diferenciais da hepatopatia crônica, diagnosticou-se shunt esplenorrenal espontâneo, e realizou-se biópsia hepática guiada por ecografia com diagnóstico de cirrose, espectro típico da DRPAR. Recebeu alta hospitalar assintomática e foi encaminhada para avaliação de transplante duplo.

A teenage patient with autosomal recessive polycystic kidney disease, a splenorenal shunt, and congenital hepatic fibrosis: a case report.

A 16-year-old female patient previously diagnosed with autosomal recessive polycystic kidney disease (ARPKD) presented with acute bilateral pneumonia, upper gastrointestinal bleeding caused by ruptured esophageal varices, ascites, and lower limb edema. She required intensive care and an endoscopic procedure to treat the gastrointestinal bleeding. The analysis of the differential diagnosis for chronic liver disease indicated she had a spontaneous splenorenal shunt. Ultrasound-guided biopsy revealed the patient had cirrhosis, as characteristically seen in individuals with ARPKD. She had no symptoms at discharge and was referred for review for a combined transplant.

Glomus coccygeum: a review.

With limited information about the coccygeal glomus found in classic textbooks, we deemed it necessary to review the subject. The illustrations presented in this article derive from four coccygeal glomera incidentally encountered during examination of pilonidal disease specimens. Familiarization with its microanatomical features may help to avoid inappropriate interpretation of this enigmatic structure.

Cerebroretinal microangiopathy with calcifications and cysts (CRMCC) or "Coats Plus": when peripheral retinal vasculature signals neurologic disease.

An 8-month-old girl presented with vitreous hemorrhage, peripheral retinal arteriovenous anastomosis, and hypoplasia of the thumb. Magnetic resonance imaging of the brain showed multiple hyperintensities, cysts, and calcifications in the white matter. DNA testing showed a mutation in one allele of the CTC gene, supporting the diagnosis of cerebroretinal microangiopathy with calcifications and cysts (CRMCC), or Coats plus disease, despite which designation this condition can present without subretinal exudate. In infants with peripheral retinal vascular disease, neuroimaging can identify the characteristic abnormalities of CRMCC.

[Macroscopic description of placental vascular anastomoses after dye injection for the comprehension of monochorionic pregnancy complications]. / Injection des anastomoses vasculaires pour la compréhension des complications propres aux grossesses monochoriales.

OBJECTIVES: The aim of the study is to compare placental monochorionic angioarchitecture complicated with twin-oligohydramnios-polyhydramnios sequence (TOPS), twin anemia polycythemia sequence (TAPS), twin reversed arterial perfusion (TRAP) and selective intra uterine growth restriction (sIUGR) to normal uneventful monochorionic placenta. METHODS: Between December 2012 and December 2015, monochorionic placenta has been studied at the multiple pregnancy care center of the Femme-Mère-Enfant Hospital in Lyon. Umbilical chords were catheterized and dye injected for macroscopic analysis of angioarchitecture at the anatomopathology department. Placentas treated with laser foetoscopic surgery were excluded. RESULTS: A total of 126 placentas were injected in the post-partum period. In total, 95% (119/126) of the placentas presented arteriovenous anastomoses (AVA). Median number of AVA was 7. The prevalence of at least one velamentous cord insertion was higher in TOPS and selective intrauterine growth restrictions P<0.01 and P<0.01 respectively, compared to uneventful pregnancies. Arterio-arterial anastomoses (AAA) were present in 82.7% (77/93) of uneventful placentas versus 33.3% of TOPS (P<0.01) and 28.5% of TAPS (P<0.01). The prevalence of veno-venous anastomoses was significantly higher in TOPS (P<0.01). All TAPS placentas showed marginal arteriovenous anastomoses. In TRAP placenta, the acardiac twin had no specific vascular territory. CONCLUSION: The study confirms literature findings on prevalence of vascular anastomoses in monochorial placentas, suggesting the protective role of AAA in TOPS and TAPS. The role of VVA is yet hard to determinate. Macroscopic observations of monochorionic placentas are valuable and essential keys for understanding, managing and treating anastomotic syndromes.

Hemodynamic Parameters and Early Intimal Thickening in Branching Blood Vessels.

Intimal thickening due to atherosclerotic lesions or intimal hyperplasia in medium to large blood vessels is a major contributor to heart disease, the leading cause of death in the Western World. Balloon angioplasty with stenting, bypass surgery, and endarterectomy (with or without patch reconstruction) are some of the techniques currently applied to occluded blood vessels. On the basis of the preponderance of clinical evidence that disturbed flow patterns play a key role in the onset and progression of atherosclerosis and intimal hyperplasia, it is of interest to analyze suitable hemodynamic wall parameters that indicate susceptible sites of intimal thickening and/or favorable conditions for thrombi formation. These parameters, based on the wall shear stress, wall pressure, or particle deposition, are applied to interpret experimental/clinical observations of intimal thickening. Utilizing the parameters as "indicator" functions, internal branching blood vessel geometries are analyzed and possibly altered for different purposes: early detection of possibly highly stenosed vessel segments, prediction of future disease progression, and vessel redesign to potentially improve long-term patency rates. At the present time, the focus is on the identification of susceptible sites in branching blood vessels and their subsequent redesign, employing hemodynamic wall parameters. Specifically, the time-averaged wall shear stress (WSS), its spatial gradient (WSSG), the oscillatory shear index (OSI), and the wall shear stress angle gradient (WSSAG) are compared with experimental data for an aortoceliac junction. Then, the OSI, wall particle density (WPD), and WSSAG are segmentally averaged for different carotid artery bifurcations and compared with clinical data of intimal thickening. The third branching blood vessel under consideration is the graft-to-vein anastomosis of a vascular access graft Suggested redesigns reduce several hemodynamic parameters (i.e., the WSSG, WSSAG, and normal pressure gradient [NPG]), thereby reducing the likelihood of restenosis, especially near the critical toe region.

Diabetes impairs arterio-venous specification in engineered vascular tissues in a perivascular cell recruitment-dependent manner.

Cell-based tissue engineering is a potential treatment alternative for organ replacement. However, the lack of a robust vasculature, especially in the context of diseases such as diabetes, is a major hindrance to its success. Despite extensive research on the effects of diabetes in angiogenic sprouting, its effects on vessel arterio-venous (AV) specification have not been addressed. Using an engineered tissue that yields functional vessels with characteristic AV identities, we demonstrate that type 1 diabetes negatively affects vessel AV specification and perivascular cell (PVC) coverage. Blockage of PVC recruitment in normoglycemia does not affect blood flow parameters, but recapitulates the vascular immaturity found in diabetes, suggesting a role for PVCs in AV specification. The downregulation of Jagged1 and Notch3, key modulators of endothelial-perivascular interaction, observed in diabetes support this assertion. Co-culture assays indicate that PVCs induce arterial identity specification by inducing EphrinB2 and downregulating EphB4. This is antagonized by high glucose or blockage of endothelial Jagged1. Engineered tissues composed of microvessels from diabetic mice display normal PVC coverage and Jagged1/Notch3 gene expression when implanted into non-diabetic hosts. These indicate a lack of legacy effect and support the use of a more aggressive treatment of diabetes in patients undergoing revascularization therapies.

A South Indian cadaveric study on obturator neurovascular bundle with a special emphasis on high prevalence of 'venous corona mortis'.

Surgical procedures in the pelvic region are very challenging because of the complex anatomy of this region. "Corona mortis" is a term used to describe retro-pubic anastomosis between the obturator and external iliac vessels. It is considered as a key structure as significant haemorrhage may occur if the vessels are cut accidentally during pelvic surgeries. Earlier studies have documented a high frequency of venous anastomosis compared to its arterial counterpart. The objective of our study was to document the prevalence of venous corona mortis in South Indian human adult cadaveric pelvises. We conducted this study on 73 cadaveric pelvic halves. Out of the 73 hemi pelvises, 36 were normal without any variations of the obturator vessels while 37 hemi pelvises (51%) showed the presence of abnormal obturator vessels which proves to be a very high incidence in terms of variations. Out of the 37 hemi pelvises, 25 (68%) showed the presence of 2 obturator veins, out of which 1 was normal and the other was an abnormal obturator vein. 8 hemi pelvises (22%) had only abnormal obturator vein. Most of the abnormal obturator veins drained into the external iliac vein, while two veins drained into inferior epigastric veins. Venous corona mortis is said to be frequently encountered during surgery and is considered to be as important as arterial corona mortis in its clinical implications. Individual evaluation of this risky anatomical structure should be done prior to any surgical interventions.

Internal carotid artery stenosis with persistent primitive hypoglossal artery treated with carotid artery stenting: A case report and literature review.

Persistent primitive hypoglossal artery (PPHA) is a persistent carotid-basilar anastomosis. It rarely remains at birth. It occasionally may be a risk for ischemia and embolic infarction to the posterior cerebral circulation, especially in patients with carotid stenosis proximal to the origin of persistent primitive arteries. We describe a case of a 60-year-old woman with asymptomatic internal carotid artery (ICA) stenosis and ipsilateral PPHA successfully treated by carotid artery stenting (CAS). A few cases of CAS for ICA stenosis with PPHA have been reported, but the strategy and methods in each case were different because of its unique anatomy and hemodynamics. It is essential to prevent distal embolisms and preserve blood flow at the territory of both the ICA and PPHA. The protection method should be selected carefully. We review the literature and discuss appropriate treatment strategies.

Monochorionic twins with unequal placental sharing: why can the outcome still be favorable?

OBJECTIVE: In monochorionic diamniotic (MCDA) twin pregnancies, unequal placental sharing does not always lead to adverse outcomes. The aim of this study is to investigate how unequal placental sharing may be compensated by placental anatomical changes. METHODS: Between January 2012 and July 2013, a total of 60 uncomplicated MCDA pregnancies ending in live birth of healthy twins were studied. Placentas were divided into two groups; an equally shared placenta group (placenta territory discordance ≤ 25%, N = 40) and an unequally shared placenta group (placenta territory discordance >25%, N = 20). Angioarchitecture, cord insertion type and the distance between two cord insertions were compared. RESULTS: Vascular anastomoses were seen in all 60 placentas, and 58 placentas (96.7%) had arterioarterial anastomoses (AA). The overall diameter of the AA was larger in the unequally shared placenta group as compared to the equally shared placentas (0.27 ± 0.12 cm versus 0.19 ± 0.1 cm, p < 0.05). The distance between the cord insertions was shorter in the unequally shared group (14.5 ± 6.0 cm versus 18.3 ± 6.5 cm, p < 0.05). CONCLUSION: The absence of adverse outcomes in unequally shared placenta group can be explained by the presence of large AA and shorter distance between cord insertions, protecting the twin with the smaller placental part against growth restriction and other pathology.