Fetoscopic laser coagulation for placental chorioangioma: a report of three cases and literature review / 中华围产医学杂志

Objective:To evaluate the outcome of laser coagulation under fetoscope for placental chorioangioma (CA).Methods:The clinical data of three pregnant women with giant CA treated by laser coagulation under fetoscope in Peking University Third Hospital from January 2018 to December 2020 were analyzed retrospectively. Relevant articles up to September 2022 were retrieved from Wanfang Database, China National Knowledge Infrastructure and PubMed, and the clinical data of all patients were retrospectively summarized. Indications and intervention effects of fetoscopic laser therapy were analyzed. Descriptive statistics was used to describe the data.Results:Thirteen patients were involved in this study including 10 cases retrieved from the databases. The average age of the pregnant women was (30.3±6.2) years old. There were 12 cases of single pregnancy and one case of twin pregnancy (monochorionic diamnionic twin pregnancy). Except for cases for which data were not available in the literatures, at the diagnosis of CA, the average gestational age was (19.9±4.5) weeks ( n=7) and the average maximum diameter of the mass was (6.1±4.1) cm ( n=6). The patients underwent fetoscopic laser therapy at an average gestational age of (25.0±2.0) weeks ( n=13) with the average maximum tumor diameter of (7.6±2.8) cm ( n=9). After treatment, the amniotic fluid volume of three cases decreased to normal. In one case, the amniotic fluid volume decreased but was still above the upper limit of the normal range. Moreover, the maximum tumor diameter decreased in four cases; the peak systolic velocity of the fetal middle cerebral artery decreased to normal in one case; fetal heart function became normal in two cases and fetal edema was relieved in one case. Among the three patients treated in our hospital, the blood supply of CA disappeared after treatment. Intrauterine fetal death occurred in two cases. The other 11 patients gave birth to live babies at the gestational age of (36.6±3.8) weeks with five through cesarean section (5/11), five through vaginal delivery (4/11) and two not reported. The birth weight of the neonates was (2 712±1 023) g and all of them survived. The gender of five neonates were reported and all were females, two of them were monochorionic diamnionic twins. No abnormality was found in the three neonates delivered in our hospital during a six-month follow-up. No abnormality was reported in the other neonates during ten days to six months of follow-up. Conclusions:Fetoscopic laser coagulation may help reduce the size of CA, decrease complications and improve pregnancy outcomes.

Ultrasonographic features of incarceration of the gravid uterus / 中华超声影像学杂志

Objective:To summarize the ultrasound characteristics of incarceration of gravid uterus (IGU) for improving the diagnostic accuracy of IGU.Methods:Three cases of IGU patients were diagnosed in Peking University Third Hospital from May 2018 to May 2020. CNKI, Wanfang Data, China Science and Technology Journal Database and PubMed were searched using the search terms "incarcerate uterus" or "uterine incarceration" and "gravid" through January 2000 to July 2020, 53 IGU cases were found. The ultrasound data and outcomes of the 56 IGU patients were retrospectively analyzed. The display rate of various ultrasonic features were counted. Relevant literatures were reviewed and the experience were summarized.Results:Of the 56 cases with IGU, 45 cases (80.4%) had positive results, of which 34 cases (60.7%) were found abnormal cervix(elongated anteriorly and superiorly displaced cervix or poorly visualized cervix), 27 cases (48.2%) were found retroversion of the gravid uterus, 12 cases (21.4%) were found that the fundus of the uterus lay deeply in the Douglas pouch, 4 cases (7.1%) were found anteriorly and superiorly displaced bladder. There was statistically significant difference between the displaying rates of abnormal cervix and retroversion of the gravid uterus(χ 2=5.452, P<0.05). Conclusions:Abnormal cervix was the most common feature of IGU by ultrasound. Correct identification of the cervix is helpful to improve the detection rate of IGU.

Fetoscopic cord laser therapy in management of monochorionic monoamniotic twin pregnancies / 中华围产医学杂志

Objective:To analyze fetoscopic cord laser therapy for management of monochorionic monoamniotic (MCMA) twin pregnancies.Methods:The clinical data of fetoscopic cord laser therapy, including cord occlusion, transection, and disentanglement in three pairs of MCMA twins from January 2020 to January 2021 in Peking University Third Hospital were summarized. Literature on cord occlusion and/or transection in MCMA twins were retrieved from Cochrane Library, PubMed, EMBASE, CBM, WanFang, and CNKI from the time at establishment to December 2020. The clinical conditions, surgical indications and methods, disease progression, and maternal and infant prognosis were analyzed.Results:Three cases of MCMA twins in this study period received fetoscopic cord laser therapy between 17-24 weeks, among which two cases gave birth at full-term without any maternal or infant complications, and one was terminated due to fetal malformation. Seven English articles including 29 MCMA twin pregnancies were retrieved. In addition to the three cases reported in this article, a total of 32 cases were analyzed. The indication of cord occlusion and/or transection included twin-reversed arterial perfusion sequence (21.9%, 7/32), fetal malformation (46.9%, 15/32), selective fetal growth restriction (sFGR) (21.9%, 7/32), twin-to-twin transfusion syndrome (TTTS) (3.1%, 1/32), TTTS combined with sFGR (3.1%, 1/32), single intrauterine death (3.1%, 1/32). Gestational age at surgery was between 14 +1 to 27 +3 weeks. No maternal complication due to the operation was reported. After exclusion of two cases who did not receive cord transection and one case was terminated due to fetal malformation, all the other 29 co-twins were born alive at the gestational age between 24 +3 to 40 weeks and birth weight between 800-3 800 g. Among the 29 live born babies, four died soon after birth with unclarified reasons in the literature and one was born with multiple malformations which were detected prenatally, and the other 24 neonates were healthy during the follow-up from 1 month to 9 years old. Conclusions:For MCMA twin pregnant women with umbilical cord entanglement or other indications for fetal reduction, cord occlusion, transection, and disentanglement using fetoscopic cord laser is safe and effective for protecting the surviving fetus.

A family of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy / 中华神经科杂志

Objective@#To investigate the clinical features, imaging features, pathological features and gene diagnosis of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL).@*Methods@#Clinical manifestations, signs and imaging characteristics of a female patient hospitalized in the First Affiliated Hospital of Zhengzhou University for more than 10 days due to headache were analyzed, and skin biopsy and HTRA1 and Notch3 gene detection were performed. The pedigree of the proband was investigated in detail, and HTRA1 gene test and related imaging examination were conducted in parallel. Due to the deceased parents of the patient, relevant genetic testing could not be conducted. A control group of 100 healthy people were analyzed.@*Results@#The clinical manifestations of proband were headache after insomnia, hearing loss in the right ear, easy to wake up and sweat at night. Brain MRI showed diffuse patchy long T1 and long T2 signals in bilateral fronto-parietal temporal occipital insula, internal and external capsule areas, bilateral basal ganglia areas, and bilateral thalamus. Fluid attenuated inversion recovery sequence showed high signals. Magnetic sensitive weighted imaging showed scattered patchy low signals in bilateral cerebral and cerebellar hemispheres, bilateral basal ganglia area, left thalamus and brain stem. The proband had consanguineous parents. A homozygous mutation C to T transition at position 589 (c.589C>T) was found in exon 3 of HTRA1 gene with the proband and both siblings. The heterozygous c.589C>T mutation appeared in another sister of the proband. Under the light microscope of skin biopsy, pigmentation in the basal layer of the skin could be seen, collagen fiber hyperplasia in the dermis was accompanied by a small amount of inflammatory cell infiltration, and no definite amyloidosis was found. No mutations were found in Notch3 gene. Because the patient′s parents were deceased, genetic testing was not possible. One hundred healthy controls had no such mutation.@*Conclusions@#The CARASIL family with HTRA1 gene c.589C>T homozygous mutation was reported, and the pathogenicity of the mutation was confirmed. HTRA1 genetic testing is recommended for diagnosis and differential diagnosis of CARASIL with family history or clinical suspicion.

A family of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy / 中华神经科杂志

Objective To investigate the clinical features, imaging features, pathological features and gene diagnosis of cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL). Methods Clinical manifestations, signs and imaging characteristics of a female patient hospitalized in the First Affiliated Hospital of Zhengzhou University for more than 10 days due to headache were analyzed, and skin biopsy and HTRA1 and Notch3 gene detection were performed. The pedigree of the proband was investigated in detail, and HTRA1 gene test and related imaging examination were conducted in parallel. Due to the deceased parents of the patient, relevant genetic testing could not be conducted. A control group of 100 healthy people were analyzed. Results The clinical manifestations of proband were headache after insomnia, hearing loss in the right ear, easy to wake up and sweat at night. Brain MRI showed diffuse patchy long T1 and long T2 signals in bilateral fronto?parietal temporal occipital insula, internal and external capsule areas, bilateral basal ganglia areas, and bilateral thalamus. Fluid attenuated inversion recovery sequence showed high signals. Magnetic sensitive weighted imaging showed scattered patchy low signals in bilateral cerebral and cerebellar hemispheres, bilateral basal ganglia area, left thalamus and brain stem. The proband had consanguineous parents. A homozygous mutation C to T transition at position 589 (c.589C>T) was found in exon 3 of HTRA1 gene with the proband and both siblings. The heterozygous c.589C>T mutation appeared in another sister of the proband. Under the light microscope of skin biopsy, pigmentation in the basal layer of the skin could be seen, collagen fiber hyperplasia in the dermis was accompanied by a small amount of inflammatory cell infiltration, and no definite amyloidosis was found. No mutations were found in Notch3 gene. Because the patient′s parents were deceased, genetic testing was not possible. One hundred healthy controls had no such mutation. Conclusions The CARASIL family with HTRA1 gene c.589C>T homozygous mutation was reported, and the pathogenicity of the mutation was confirmed. HTRA1 genetic testing is recommended for diagnosis and differential diagnosis of CARASIL with family history or clinical suspicion.

Microwave ablation for selective feticide in complicated monochorionic twin pregnancies / 中华围产医学杂志

Objective To evaluate the feasibility,safety and short-term fetal outcome of ultrasoundguided percutaneous microwave ablation for selective feticide in complicated monochorionic twin pregnancies.Methods Twenty-two patients who underwent microwave ablation for selective fetal reduction in Peking University Third Hospital between July 2015 and June 2016 were retrospectively studied.Surgical complications,fetal outcomes and neural development at age of 60 days were recorded.One-way ANOVA,Mann-Whitney U test,Chi-square or Fisher's exact test was used for statistical analysis.Results Indications of the 22 patients for selective feticide included five cases of twin pregnancy with one anomalous fetus,five cases of severe twintwin transfusion syndrome (TTTS),three cases of twin reserved arterial perfusion (TRAP) and nine cases of selective intrauterine growth restriction (sIUGR).All surgeries were successful under regional anesthesia.Four pregnancies (18.2％,4/22) ended in miscarriage following surgery,three of which were caused by intrauterine fetal death (13.6％,3/22) and one by premature rupture of membranes before 26 weeks of gestation (4.5％,1/22).Totally,18 babies were born alive at an average gestational age of (35.5 4.2) weeks (26～40 weeks+2) and with an average birth weight of (2 380.778) g (800～3 430 g),and four of them were preterm infants less than 32 weeks of gestation.Except for one extremely low birth weight infant,whose gestational age was 26 weeks at birth and birth weight was 800 g,died two days after birth,17 infants were discharged alive,giving an neonatal survival rate of 17/18.The total fetal survival rate was 77.3％ (17/22).Polyhydramnios,placenta location,and position of the fetus being aborted showed no significant effect on miscarriage or fetal survival rate (all P＞0.05).No severe maternal complications occurred.No brain injuries were detected by imaging examination in 17 infants who were followed up to 60 days of age.Conclusions Ultrasound-guided percutaneous microwave ablation can be used for selective feticide in complicated monochorionic twin pregnancies.Further studies are needed to evaluate its effects on long-term fetal outcome.

Research of placental vascular distribution and clinical outcome in monochorionic twins / 中华妇产科杂志

Objective To study the placental vascular distribution of monochorionic (MC) twins with twin-to-twin transfusion syndrome (TTTS) or hirth weight discordance.Methods Twenty-eight MC placentas were injected in Peking University Third Hospital between Feb.2010 and Feb.2011.The vascular distribution type (parallel,crossed,mixed and monoamniotic),the anastomosis of vessels and the placental sharing were recorded.The outcome of pregnancy and the placental characteristics of birth weight discordance (birth weight discordance ≥ 20％) in non-TTTS MC twins were analyzed.Results (1) The outcome of pregnancy:the miscarriage or gestational weeks of 28 MC twins were 20 to 38 weeks (median of 35 weeks).Six cases were TTTS,3 of which received fetoscopic laser occlusion of communicating vessels (FLOC).There were 48 live births,with an average birth weight of (2036 ± 623) g.(2) Type of placental vascular distribution:in the 28 MC placentas,number of parallel,crossed,mixed and monoamniotic type of placental vascular distribution were 4 (14％),14 (50％),6 (21％) and 4 (14％) cases,respectively.No parallel type was found in TTTS.There was no significant difference of vascular anastomosis or unequal placental sharing among the different placental vascular distribution types (P ＞ 0.05).(3) Characteristics of placental vascular distribution in birth weight discordance twins:there were 20 non-TTTS MC twin pregnancies,all of which got live births of both babies.Birth weight discordance equal to or more than 20％ was found in 6 pairs of newborns,while birth weight discordance less than 20％ was found in the rest 14 cases.Ratio of unequal placental sharing was significantly different between the two groups (P ＜ 0.01).There was no significant difference of umbilical cord insertion,placental vascular distribution and anastomosis in the two groups (P ＞0.01).Conclusions Vascular distribution type of MC twins might be related to TTTS.Unequal placental sharing is a risk factor of birth weight discordance in non-TTTS MC twins.