Myomectomy scar pregnancy ‒ a serious, but scarcely reported entity: literature review and an instructive case

Objectives Uterine myomas are a frequent finding in reproductive age women with an estimated incidence 12–25%. 1. Treatment of uterine myomas to facilitate good pregnancy rates and outcome, such as hysteroscopic, laparoscopic, abdominal resection uterine artery embolization among others were evaluated in terms of pregnancy outcome. While the literature is replete of the pregnancy complication of uterine rupture after myomectomies, 2–4 there are very few publications evaluate a relatively rare pregnancy complication associated with placental implantation within the uterine cavity at the site of the previous myomectomy, namely the myomectomy scar pregnancy (MSP). Despite their relative rarity, this type of pathologically adherent placenta rightfully belongs to the well-known entity of placenta accreta spectrum (PAS). Case presentation We present a complicated case of MSP and review the available literature to raise attention to its clinical appearance, its prenatal diagnosis so appropriate intrapartum management can be planned. Conclusions Despite the rarity of MSP, continuous attention should be given at every single routinely scheduled or indication driven obstetrical US scan following myomectomies to evaluate the placental site implantation regardless of the route and technique of their initial surgical procedure.

Extremely preterm infant with persistent peeling skin: X-linked ichthyosis imitates prematurity

Objectives X-linked ichthyosis (XLI) is a genetic disorder caused by a deficiency in steroid sulfatase, an enzyme which catalyzes a reaction in estrone synthesis. The disorder primarily manifests as dry, scaly skin which may be difficult to diagnose in extremely preterm infants, as the dermatological features may be falsely attributed to a normal variant of skin for this population. Case presentation In this case report, we describe a male with XLI, born at 24 weeks gestation, who had persistent dry, flaky and hyperpigmented skin. This case is notable for the age of diagnosis in an extremely premature infant; day of life 105. In addition, this infant experienced out of proportion bronchopulmonary symptoms that we postulate may be linked to the steroid sulfatase deficiency, as estrogen is a mediator of surfactant production. Conclusions This report underscores the need to potentially evaluate persistent dry, flaky skin in the preterm infant, as XLI may also impact long term neurodevelopmental outcomes.

A case of severe SARS-CoV-2 infection with negative nasopharyngeal PCR in pregnancy

Objectives SARS-CoV-2 remains a pressing issue for our obstetric community during the current pandemic. We present a case of a 22-year-old nulliparous woman 31 weeks pregnant, with significant clinical SARS-CoV-2 disease, in the setting of negative nasopharyngeal PCR testing but positive IgG antibodies. Case presentation This was a 22-year-old patient 31 weeks pregnant who presented with fever, tachycardia, and subsequently preterm premature rupture of membranes (PPROM) and pulmonary emboli with multifocal pneumonia. The patient underwent three negative SARS-CoV-2 tests via nasopharyngeal PCR testing during her hospital stay as well as a negative workup for fever. After a cesarean section for worsening maternal status after 7 days of hospitalization, the patient was admitted for worsening clinical status to the SICU. At the time of SICU admission patient was found to have serum IgG positive antibodies and was managed with intubation, antibiotics, and anticoagulation. Patient eventually left hospital against medical advice on hospital day 16 on oral antibiotics but was found to be recovering well at later outpatient follow up. Conclusions Diagnosis of SARS-CoV-2 remains a complicated picture in the setting of testing limitations. This case highlights an antepartum clinical presentation of severe SARS-CoV-2 and recommends a high clinical suspicion for diagnosis of SARS-CoV-2 and initiation of treatment in the pregnant population, even in the presence of negative nasopharyngeal PCR testing.

Further insights into unusual acrania-exencephaly-anencephaly sequence caused by amniotic band – first trimester fetoscopic correlation with two- and three-dimensional ultrasound

Objectives Antenatal detection of acrania-exencephaly-anencephaly (AEA) sequence beyond 10 completed weeks of gestation is usually straight-forward. An earlier detection and classification of the causative conditions prior to disaggregation of exposed dysplastic brain tissue remains challenging. Case presentation We present two- and three-dimensional ultrasound correlated with fetoscopic findings of an unusual type of neural tube defect at 11 gestational weeks caused by an amniotic constriction ring resulting in cystic degeneration of the developing skull. Conclusions By giving further illustrative insights into early defective brain development, this report confirms recent findings of an unusual subtype of acrania-exencephaly-anencephaly sequence suggesting early disruption of the developing brain, following an amniotic entrapment of the skull.

Postpartum ovarian vein thrombosis

Objectives Postpartum ovarian vein thrombosis (POVT) is a rare pathology that can lead to severe complications such as sepsis, extension of the thrombus leading to organ failure, and pulmonary embolism. It therefore requires early recognition and prompt treatment. Case presentation A patient with right POVT presented four days after delivery with acute right-sided abdominal pain and fever. Appendicitis was initially considered, before an abdominal-pelvic computed tomography raised the suspicion of POVT, subsequently confirmed through transabdominal ultrasound. Antibiotics and anticoagulation were initiated, with rapid clinical improvement and complete resolution of the thrombus three months later. Conclusions Diagnosing POVT is challenging as it clinically mimics other more frequent conditions. It is rare but life-threatening and should be considered in all females presenting with abdominal pain and fever in the postpartum period.

Obstetrical history of a family with combined oxidative phosphorylation deficiency 3 and methylenetetrahydrofolate reductase polymorphisms

Objectives Recurrent pregnancy loss (RPL) is a devastating complication of pregnancy with various etiologic backgrounds. Case presentation We present a case of combined oxidative phosphorylation deficiency 3 (COXPD3) carrier pregnant woman with Methylenetetrahydrofolate reductase (MTHFR) polymorphisms. She had five pregnancy losses and a postpartum death due to COXPD3. The patient was admitted to our clinic for the first time at her seventh pregnancy with oocyte donation. The patient was registered in a special antenatal care program and delivered a healthy baby at term. Her eighth pregnancy was terminated due to COXPD3 which was prenatally diagnosed. Conclusions Comprehensive and individualized approaches are necessary in RPL cases to obtain optimal outcomes.

Management of a patient in the state of total occlusion of aorta due to Takayasu arteritis in preconceptional and pregnancy period

Objectives Total aortic occlusion is a severe complication of Takayasu arteritis (TAK). Pregnancy follow-up in the state of total aortic occlusion due to TAK has not been reported before. Case presentation A 35 year-old nulliparous woman with total aortic occlusion in the distal aorta due to TAK, admitted with pregnancy desire. She had developed a collateral vessel system which has maintained the lower body circulation. She was informed about the potential risks after an evaluation and she admitted to our clinic at the seventh week of pregnancy, and acetylsalicylic acid was prescribed. At 20th gestational week anomaly screening was in normal limits although the uterine artery Doppler had lower S/D, PI and RI values. She was followed-up regularly in every two weeks. Vascular examination was performed by using an ankle brachial index (ABI) by duplex ultrasound. At 20th gestational week ankle brachial index score was 0.8–0.9 which indicates mild disease. Around 28th gestational week her claudication got worse again and ABI was in moderate level (0.5–0.8) and low molecular weight heparin was started. Until 37th gestational week her disease was stable, ABI was above 0.5, her blood pressure was in normal limits, no vascular complication occurred and the baby’s growth percentile was at 25th centile. At 37th gestational week a 2,640 g baby was delivered. Patient was discharged without any complications at third post-operative day. Conclusions Complicated TAK patients may have good obstetric outcomes with a multidisciplinary approach in experienced tertiary centers.

Spontaneous resolution of fetal ascites secondary to gastrointestinal abnormality

Objectives Isolated fetal ascites carries an uncertain prognosis and broad differential diagnosis. When detected on prenatal sonography, a thorough evaluation is warranted to exclude development of hydrops and search for an underlying condition. While gastrointestinal abnormalities account for approximately 20% of cases of fetal ascites, surgical correction is commonly required postnatally. While there have been reports of isolated fetal ascites resolving in utero, spontaneous resolution of the causative gastrointestinal abnormality is unusual. Case presentation We report a case of a multiparous 33-year-old found to have moderate fetal ascites and a complex fetal abdominal mass near the small bowel detected by ultrasound at 32 weeks with spontaneous resolution of both ascites and mass by 37 weeks. Following the delivery of a normal neonate, we suspect the mass and ascites to have been produced by a small bowel rupture resulting in meconium peritonitis. Conclusions When fetal ascites with late gestational onset has spontaneous resolution in utero and hydrops never develops, there is generally a favorable prognosis and normal neonatal outcome.

Thrombospondin domain1-related congenital chylothorax in an infant with maple syrup urine disease: a challenging case

Objectives Congenital chylothorax is a rare entity with various etiologies ranging from anatomical to genetic causes. If associated with non-immune hydrops fetalis mortality rates can reach up to 98%. Treatment is challenging and mostly supportive, with no standard guidelines. Case presentation We describe the unique and challenging course of a late preterm infant with non-immune hydrops fetalis (NIHF), and recurrent chylothorax attributed to homozygous mutations in thrombospondin domain1 (THSD1) gene. The infant was also affected with maple syrup urine disease (MSUD), which further complicated the clinical course. Treatment was supportive by means of chest tubes, intubation, mechanical ventilation, and eventually he was tracheostomized and discharged home on home BiPAP ventilation and required prolonged use of octreotide to prevent re-accumulation of chylothorax. Conclusions THSD1 gene has a role in vascular permeability and its mutation in our patient caused congenital chylothorax and NIHF and is also associated with other features such as vascular malformations.