Perineal groove is a well-defined clinical entity that belongs to a broader group of anorectal malformations. It is characterized by a non-epithelialized mucous membrane that appears as an erythematous sulcus in the perineal midline, extending from the posterior vaginal fourchette to the anterior anal orifice. The defect is gradually cicatrized, unless there are complications like infection, defecation disorders, trauma, and bleeding. The differential diagnosis includes several other conditions like trauma, infection, irritant dermatitis, lichen sclerosis, and ulcerated hemangioma. Since it is a rare malformation, it is often misdiagnosed and its presence often elicits unnecessary diagnostic workup and intervention. In this respect, neonatologists, dermatologists, or pediatric surgeons may under- or overestimate it. We report four cases of perineal groove out of 2250 live births at a Greek Maternity Hospital between September 2016 and April 2019. The “high” incidence of perineal groove cases in our Department allowed us to familiarize with this rare defect and minimize our clinical interventions.
Primary hemostasis, similar to other systems in the adjusting and transitioning neonate, undergoes developmental adaptations in the first days of life. Although platelets of neonates do not differ quantitatively compared with those of adults, they functionally present with major differences, thus supporting the theory of a “hypofunctional” phenotype that is counterbalanced by high hematocrit and more potent von Willebrand factor multimers. No clinical effect of bleeding tendency has hence been established so far for healthy term neonates. However, discrepancies in functionality have been noted, associated with gestational age, with more pronounced platelet hyporesponsiveness in preterm neonates. Multiple methods of in vitro platelet function evaluation such as PFA-100/200, platelet aggregometry, flow cytometry, and cone and platelet analyzer have been used for assessment of neonatal primary hemostasis. Several pregnancies are characterized as “high-risk” when risk factors preexist in maternal history or evolve during pregnancy. These pregnancies require specialized observation as they may have unpredictable outcome. High-risk pregnancies include clinical entities such as preeclampsia, pregnancy-induced smoking during pregnancy, gestational diabetes mellitus (GDM), autoimmune diseases, and other maternal hematological conditions. In some cases, like systemic lupus erythematosus, antiphospholipid antibody syndrome, and maternal immunologically based thrombocytopenia, neonatal thrombocytopenia is regarded as a prominent hemostasis defect, while in others, like pregnancy-induced hypertension and preeclampsia, both quantitative and qualitative disorders of neonatal platelets have been reported. In other pathologies, like GDM, neonatal primary hemostasis remains vastly unexplored, which raises the need for further investigation. The extent to which primary hemostasis is affected in neonates of high-risk pregnancies is the main objective of this narrative review.
BackgroundPlatelet function of fetal growth restricted (FGR) neonates remains a field of debate. Platelet function analyzer (PFA-100) offers a quantitative in vitro assessment of primary, platelet-related hemostasis. Our aim was to examine platelet function using PFA-100 in FGR neonates and associate our results with perinatal parameters.MethodsPFA-100 was applied on 74 FGR neonates, 48 full-term (>37 weeks' gestation) and 26 preterm neonates (<37 weeks). The control group consisted of 118 healthy neonates. Two closure times (CTs) with COL/EPI and COL/ADP cartridges were determined on cord blood samples for each subject. Statistical analysis was performed by SAS 9.4. The statistical significance level was set at 0.05 and all tests were two-tailed.ResultsCOL/EPI CTs were prolonged in FGR (median 132 s, IQR 95–181 s) compared with control neonates (median 112.5 s, IQR 93–145 s), p = 0.04. Median COL/EPI CT for term and preterm FGR neonates was 126 s (IQR 90–157 s) and 137 s (IQR 104–203), respectively (p = 0.001), and COL/ADP CT was 70 s (IQR 62–80 s) for term and 75 s (IQR 68–82 s) for preterm FGR neonates (p = 0.08). Among FGR neonates, COL/EPI CT was related with delivery time (with preterm neonates exhibiting prolonged COL/EPI CTs), p = 0.05. No correlation was proved between both CTs and hematological parameters in FGR neonates.ConclusionFGR neonates showed impaired platelet function via PFA-100, with preterm FGR neonates confronting the greatest risk. Prolonged COL/EPI CTs in FGR neonates seemed to be independent of hematological parameters and could warn for closer evaluation during the first days of their lives.
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