[A case of subglottic cysts in monochorionic monoamniotic twins]. / Klinicheskoe nablyudenie razvitiya podskladkovykh kist u monokhorial'nykh monoamnioticheskikh bliznetsov.

The first observation in the world literature of the development of subglottic cysts in the larynx in monochorionic monoamniotic twins is presented. The girls were born prematurely at 34 weeks of gestation, from the first day of life for 7 and 8 days, respectively, were transferred to mechanical ventilation. At the fourth month of life, symptoms of laryngeal stenosis appeared and began to gradually progress, conservative therapy had no effect. The diagnosis of subglottic cysts was established on the basis of fibrolaryngoscopy; after endolaryngeal surgery, breathing returned to normal. This case demonstrates the importance of timely endoscopic examination of the respiratory tract in children with stridor.

[Clinical characteristics and outcomes of monochorionic monoamniotic twin pregnancy].

Objective: To investigate the clinical characteristics and outcomes of monochorionic monoamniotic (MCMA) twin pregnancy. Methods: The clinical data of 60 MCMA twin pregnant women who were terminated in Peking University Third Hospital from January 2011 to December 2019 were collected, and the general clinical data, prenatal examination and pregnancy outcomes were analyzed retrospectively. Results: The age of 60 MCMA twin pregnant women was (31.0±4.1) years old, among which 44 cases were primiparas (73%, 44/60) and 16 cases were multiparas (27%, 16/60). Fifty-eight cases were diagnosed as MCMA twin pregnancy prenatally and were confirmed after delivery. Median ultrasonic diagnosis of gestational age was 12 weeks (range: 8-30 weeks). In the 60 MCMA twin pregnancies, 6 cases were conjoined twins, 5 cases were complicated with twin reversed arterial perfusion sequence (TRAPS), and 10 cases were diagnosed as other fetal malformation by prenatal ultrasound examination. Among the 60 MCMA twin pregnant women, 19 cases had spontaneous abortion or induced abortion due to fetal malformation, fetal death or other reasons within 28 weeks of pregnancy, 41 cases entered the perinatal period, a total of 70 newborns survived. The main cause of perinatal fetal or neonatal death was fetal dysplasia. Conclusions: There is a high incidence of fetal abnormality and perinatal mortality in MCMA twin pregnancy. Accurate early diagnosis, enhanced management and monitoring during pregnancy, and individualized treatment are the keys to improve MCMA twin pregnancy outcomes.

Prevalence, antenatal management and perinatal outcome of monochorionic monoamniotic twin pregnancy: a collaborative multicenter study in England, 2000-2013.

OBJECTIVES: To determine the prevalence of monochorionic monoamniotic (MCMA) twin pregnancy and to describe perinatal outcome and clinical management of these pregnancies. METHODS: In this multicenter cohort study, the prevalence of MCMA twinning was estimated using population-based data on MCMA twin pregnancies, collected between 2000 and 2013 from 11 Northern Survey of Twin and Multiple Pregnancy (NorSTAMP) maternity units. Pregnancy outcome at < 24 weeks' gestation, antenatal parameters and perinatal outcome (from ≥ 24 weeks to the first 28 days of age) were analyzed using combined data on pregnancies confirmed to be MCMA from NorSTAMP and the Southwest Thames Region of London Obstetric Research Collaborative (STORK) multiple pregnancy cohort for 2000-2013. RESULTS: The estimated total prevalence of MCMA twin pregnancies in the North of England region was 8.2 per 1000 twin pregnancies (59/7170), and the birth prevalence was 0.08 per 1000 pregnancies overall (singleton and multiple). Using combined data from NorSTAMP and STORK, the rate of fetal death (at < 24 weeks' gestation), including terminations of pregnancy and selective feticide, was 31.8% (54/170); the overall perinatal mortality rate was 14.7% (17/116), ranging from 69.2% at < 30 weeks to 4.5% at ≥ 33 weeks' gestation. MCMA twins that survived in utero beyond 24 weeks were delivered, usually by Cesarean section, at a median of 33 (interquartile range, 32-34) weeks of gestation. CONCLUSIONS: In MCMA twins surviving beyond 24 weeks of gestation, there was a higher survival rate compared with in previous decades, presumably due to early diagnosis, close surveillance and elective birth around 32-34 weeks of gestation. High perinatal mortality at early gestations was attributed mainly to extreme prematurity due to preterm spontaneous labor. Copyright © 2018 ISUOG. Published by John Wiley & Sons Ltd.

Six-month survival of a monochorionic monoamniotic twin with sirenomelia.

BACKGROUND: Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period. CASE: A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6 months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes. CONCLUSION: Infants with sirenomelia, even those with end-stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.

Lethal Cord Entanglement.

Cord entanglement and conjoined twins are unique complications of monoamniotic monochorionic pregnancy. This case report describes a case of monoamniotic twins' intrauterine demise due to lethal cord entanglement. A 26-year-old unbooked primigravida was reported in the emergency labor room at 32 weeks with the complaint of loss of fetal movements since the previous day. On obstetric examination, the uterus appeared enlarged and discordant with the gestational age, multiple fetal parts were felt on palpation, and the pelvic grip was empty. Doppler could not detect any fetal heartbeat. The emergency obstetric sonography scan showed twins, the first in breech and the second in vertex presentation. Fetal heart sound was not found for either of the twins. The patient was counseled, and the decision was taken to terminate the pregnancy by lower-segment cesarean section. During the cesarean to deliver the babies, monochorionic and monoamniotic pregnancy was confirmed.