Volume 10, Issue 1

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.

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.

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.

Obejctives Phenylketonuria (PKU) and hyperphenylalaninemia (HPHE) are metabolic disorders of phenylalanine (phe) metabolism due to the liver enzyme phe hydroxylase deficiency resulting in mental retardation. The only treatment of these disorders is the total or the partial breastfeeding replacement with special formula, respectively. Case presentation We aimed to evaluate the maternal stress degree in the two groups of mothers and compare the stress degree between mothers who were obliged to replace breastfeeding completely with those who replaced it partially. Mothers (n=50) were divided into two groups: n=25 with PKU and n=25 with HPHE infants. Mothers with PKU newborns were obliged to replace breastfeeding completely and feed their newborns with a common formula plus phe free medical food, whereas mothers with HPHE infants partially replaced breastfeeding with phe free medical food. Stress degrees were calculated using special questionnaire checklist by Bourne E. Psychological support was provided for both groups of mothers with stress. Conclusions Most mothers with PKU infants experienced high or severe stress, whereas mothers with HPHE newborns suffered from low and moderate symptoms and signs of stress. After psychological support most mothers with PKU newborns were ameliorated from stress symptoms; mothers with HPHE neonates experienced almost no stress symptoms. Mothers with PKU infants obliged to complete breastfeeding replacement experienced high and/or severe stress degrees as compared to mothers with HPHE newborns with partial breastfeeding replacement. Psychological support resulted in amelioration of both tested groups.

Objectives The population of female heart transplant recipients of reproductive age is increasing and pregnancy follow-up of these patients is important. Case presentation A 30-year-old patient who had a heart transplant due to viral myocarditis became pregnant spontaneously. A close follow-up by a multidisciplinary team allowed a normal pregnancy without maternal or fetal complications and the delivery of a healthy infant. Conclusions Successful pregnancy outcomes are possible in women who had a heart transplant. Careful and close surveillance by a multidisciplinary team is mandatory.

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.

Objectives Doppler measurement of fetal middle cerebral artery peak systolic velocity (MCA-PSV) is used to screen fetuses at risk of developing fetal anemia. This screening tool has demonstrated to be accurate to estimate the likelihood of fetal anemia and it has been used in the management of fetuses at this risk. It is rare to find in the literature cases of increased MCA-PSV in the absence of fetal anemia. We present the case of an elevated MCA-PSV in the absence of common causes of fetal anemia. Case presentation A 30-year-old woman at 21 weeks of gestation was referred to our unit with a left fetal renal agenesis and single umbilical artery diagnosis. The patient had a poorly controlled type 1 Diabetes with a booking HbA1C of 9.1%. At 31 weeks of gestation, MCA-PSV was raised (>1.5 multiples of the median) in the absence of any cause of fetal anemia and discrepancy of ventricular size with normal fetal heart function were seen. In a subsequent scan, thick interventricular septum was seen and hypertrophic cardiomyopathy (HC) was suggested. Follow-up ultrasonography showed a persistent MCA-PSV in a large for gestation age (LGA) fetus with HC. Due to the prenatal diagnosis of persistent elevated MCA-PSV in LGA fetus with diagnoses of HC in a poorly controlled type 1 diabetic patient labor was induced after fetal lung maturation. Biventricular hypertrophic cardiomyopathy was detected after birth. Conclusions HC may be an explanation for increased MCA-PSV without fetal anemia.

Objectives To describe a novel presentation of severely elevated procalcitonin (PCT) levels associated with postpartum fibroid degeneration. Case presentation We report a case of a 34-year-old woman with multiple large fibroids who was found to have fevers and a severely elevated PCT level of 34.03 ng/mL 2 days postpartum. MRI revealed carneous degeneration of her fibroids, and other etiologies such as infection were ruled out. She was successfully treated with the use of intravenous non-steroidal anti-inflammatory drugs and antibiotics, as a precaution. This report indicates procalcitonin may be elevated secondary to the inflammatory state caused by degenerating fibroids. Informed consent was obtained from all individuals included in this study. Conclusions Uterine leiomyomas, or fibroids, are tumors of the female reproductive tract affecting anywhere from 20–40% of women. One known complication of fibroids is degeneration, when the fibroid can infarct due to a decrease in blood supply. This can cause an inflammatory state with the release of multiple inflammatory markers. During pregnancy, routine markers such as white blood cell counts may be unreliable. Other markers such as procalcitonin are not well-studied in pregnancy. This case provides practitioners an example where the marker procalcitonin can be used to more accurately assess an inflammatory state during pregnancy. It also provides alterative diagnoses in the workup of postpartum fever.

Objectives This is the report of the first pregnant woman having COVID-19 in Vietnam. The patient was at 21 weeks of gestation and a high level of serum D-dimer and fibrinogen was observed from admission day to day 5. Case presentation The patient had lived in Russia before returning to Vietnam where she was diagnosed with COVID-19. Clinical characteristics of this patient were described and there was no major derangement of laboratory parameters. She was asymptomatic when being diagnosed and then developed mild symptoms of COVID-19 after three days of admission. The patient was treated with lopinavir/ritonavir, ampicillin/sulbactam, and Lovenox and continued being hospitalized until April 25th, 2020. Conclusions We presented the clinical picture of the first COVID-19 pregnant case in Vietnam. This case report highlighted the increased risk of developing coagulopathy even in mild and asymptomatic patients.

Objectives Lower urinary tract obstruction (LUTO) has different etiologies. The most common form is posterior urethral valve (PUVs). It is a condition that can lead to end stage renal disease (ESRD) in later life and severe renal and pulmonary damage in utero . Those born alive may end up with residual kidney disease leading to life long morbidity or death. This condition can be diagnosed through prenatal ultrasound but because diagnosis is done late when kidney injury has already happened it does not guarantee healthy survival after birth. There are various treatments in utero and after birth but they all have their complications. In developing countries diagnosis is possible but in most cases pregnant women with fetuses with PUVs present very late in pregnancy. In utero interventions are in most cases not possible and the only way out is expectant management with serial ultrasound assessment until the fetus has achieved level of maturity at which time it is delivered and further postnatal evaluations and treatment given. This series is aimed at highlighting the prenatal ultrasound features of PUVs and the management challenges faced by physicians in developing countries. Case presentation The three cases were managed in three different hospitals. All the cases presented at different gestational ages and different levels of fetal affectation. Because of these, outcomes of management and prognosis of each case differ. Conclusions Fetal PUVs pose specific challenges in management because of late presentation and lack of resource for intervention even where cases present early with minimal renal damage.

Objectives The aim of this article was to present a case of premature fetal closure of the ductus arteriosus (DA) of unknown cause. Case presentation A 32-year-old pregnant woman came for the regular prenatal visit at 36 + 1 weeks of gestation (WG) at which oligohydramnios and premature closure of DA were revealed. Use of non-steroidal anti-inflammatory drugs was excluded by the history, although the patient had the symptoms of common cold 2 weeks before the check-up taking more than 1,000 mL of strong chamomile tea daily till the day before the prenatal visit. The patient was hospitalized at 36 + 1 weeks of gestation due to premature closure of DA and oligohydramnios (amniotic fluid index = 4.5/3), which was the indication to deliver the baby by cesarean section at 36 + 6 WG (birth weight was 2,830 g, birth length 49 cm and head circumference 34 cm, Apgar score at 1 and 5 min were 9/9). Postnatal course was uneventful, and postnatal echocardiography at 12 h of life revealed functionally closed DA and mild dysfunction of the right ventricle, which completely resolved after 7 days. The mother and the baby were discharged home healthy, and were doing well 3 months after delivery. Conclusions Although the cause of premature closure of DA in most of the cases will remain undetected, thorough history sometimes with unexpected events should be taken under the consideration as possible causative factor for premature DA closure, as was drinking of high quantities of chamomile tea in our case.

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.

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.

Objectives The Coronavirus disease 2019 (COVID-19) pandemic has rapidly spread since its emergence in December 2019, and has been associated with severe morbidity and mortality. This report includes an in-depth discussion on the unique challenges that the obstetrical population provides when considering optimal management strategy. Case presentation We describe our approach to a preterm patient with high clinical suspicion for COVID-19 whose condition turned critical in the postpartum state. Conclusions Differences in physiology during pregnancy, and goals for reducing both maternal and fetal risks, provide challenges when considering intensive care management, delivery timing, and method of delivery.

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.

Objectives We present a case of immune-mediated diabetes mellitus, diagnosed in pregnancy upon presentation with diabetic ketoacidosis, found to have normal glucose control postpartum. Case presentation A 28-year-old medically uncomplicated G1P0 presented in diabetic ketoacidosis at 28.2 weeks gestation. Workup for pancreatic autoantibodies revealed indeterminate anti-islet cell antibodies and positive anti-glutamic acid antibodies. She was stabilized with intravenous fluids and insulin, and transitioned to long and short acting subcutaneous insulin. Her insulin requirements decreased over the course of her pregnancy. Spontaneous vaginal delivery occurred at 37 weeks. Her postpartum glucose control was normal without re-initiation of insulin. Conclusions The diagnosis of diabetic ketoacidosis during pregnancy should prompt further investigation into an underlying diagnosis of immune mediated diabetes. These patients should be followed closely in the postpartum period.

Objectives Thanks to the advances of modern medicine it has become possible to reach a fertile age even in the case of serious illnesses, enabling those patients to realize their desire to have children. This is also the case with the extremely heterogeneous, often autosomal dominantly inherited osteogenesis imperfecta. Due to a disruption in collagen synthesis those patients are faced with multiple fractures, spinal deformities and a decrease in pulmonary capacity throughout the course of their lives, depending on the subtype and severity of the disease. Obstetricians as well as anesthetists face major interdisciplinary challenges in the case of a pregnancy in those patients because of pregnancy-associated risks like uterine rupture, preterm birth and postpartum hemorrhage as well as risks associated with the nature of osteogenesis imperfecta itself, like bone fractures, spinal deformities and decreased mobility in the course of the progressing pregnancy. Mode of delivery should be planned individually in order to minimize maternal morbidity and mortality. In cases in which the fetus is as well affected by the disease, this aspect must be taken into consideration when it comes to supervision of pregnancy and planning of the birth mode. Case presentation We report the case of a woman with osteogenesis imperfecta type III who spontaneously conceived a pregnancy with a fetus who was also affected by the genetic disease. This constellation has up to now been reported by only few sources and requires supervision by an experienced perinatal center. Conclusions Osteogenesis imperfecta is not an exclusion diagnosis for family planning and a successful delivery. Nevertheless, depending on the form of the disease, mother and child can be severely affected by the disturbed collagen synthesis. Each patient has to be individually advised and cared for with the specific risks due to the type of Osteogenesis imperfecta. In the case of type III, due to extreme scoliosis, pelvic deformity and small growth, only a primary cesarean section can be performed.

Objectives Emanuel syndrome is a rare inherited syndrome, a correct in utero diagnosis allows effective management for ongoing and future pregnancies. Case presentation Here, we report a case of a complete non-mosaic trisomy 22, with several prenatal sonographic findings, that was diagnosed in utero at 15 weeks’ gestation and then it was confirmed with chromosomal analysis and postmortem examination. Conclusions Every anatomical difference should always be further investigated in order to achieve the correct diagnosis.

Objectives Lymphatic malformations are benign vascular anomalies comprised of lymphatic elements and most commonly occur in the neck. They may be diagnosed prenatally on ultrasound, and, in addition to confirming the diagnosis, fetal MRI can delineate the presence and extent of airway involvement. A variety of therapies are available for the treatment of lymphatic malformations including medical management, sclerotherapy, and surgery. Case presentation We report a case of a 28 year-old pregnant female diagnosed with a fetal neck mass by ultrasound at 21 weeks gestation. A fetal MRI was performed which demonstrated a multiseptated cystic neck mass most suggestive of a lymphatic malformation. The mass was noted to be intimately associated with the fetal airway. Follow up ultrasounds showed continued growth of the lesion throughout pregnancy. The baby was born by EXIT procedure and the lymphatic malformation was ultimately treated by a combination of medical treatment, sclerotherapy, and surgery. Conclusions Cervical lymphatic malformations have a classic imaging appearance and can enlarge throughout gestation as well as after delivery. The treatment plan typically involves a multidisciplinary approach.

Objectives To describe an infrequent case of bacteremia by Rothia dentocariosa in a newborn and to discuss its potential pathogenicity. Case presentation R. dentocariosa is an aerobic or facultative anaerobic gram-positive bacillus, common in the human oral cavity that has been isolated in cases of endocarditis, pneumonia, endophthalmitis and peritonitis in adults. Infections in the fetus and newborn have been scarcely reported. We present a full-term newborn with prolonged rupture of membranes, acute elevation of C-reactive protein, and isolation of R. dentocariosa in the blood culture. Endocarditis was ruled out and the patient did well with five days of intravenous broad spectrum antibiotics. To date, only a previous case of sepsis by R. dentocariosa in a neonate with meconium aspiration syndrome and an antenatal death of a full-term fetus with hemorrhagic brain lesions probably related to R. dentocariosa have been reported. Conclusions Until more information about the pathogenicity of this germ in the newborn is available, an individualized approach and a close clinical control of the patient with infectious risk factors and bacteremia by R. dentocariosa seem to be prudent.

Objectives To describe the association between the rapid normalization of pCO 2 after intubation in a patient with severe bronchopulmonary dysplasia managed with permissive hypercapnia, with the risk of developing hypocalcemia and seizures, and to make health care providers aware of this risk in similar cases. Case presentation An extreme premature infant, born at 25 weeks of gestational age (GA), developed a severe bronchopulmonary dysplasia (BPD) and, after several extubation failures could be managed with non-invasive ventilation and permissive hypercapnia, with capillary pCO 2 of up to 80 mmHg and pH >7.20. At 46 postmenstrual age (PMA) he was intubated because of severe hypercapnia and compensating metabolic alkalosis. About 20 h after intubation, after normalization of pH and pCO 2 , he developed hypocalcemia and seizures, that remitted after iCa normalization. A comparison between arterial and capillary blood gases showed a significantly greater correlation between pH and iCa in arterial than in capillary samples. Conclusions Our findings emphasize the importance of avoiding the abrupt reduction of pCO 2 and the close monitoring of acute metabolic changes after its correction in chronic patients with permissive hypercapnia, as well as the potential superiority of arterial samples over capillaries to improve the precision of this control.

Objectives Amniotic band syndrome and osteogenesis imperfecta are two distinct diseases that develop due to structural defects of the collagen protein. In our paper, we report the concurrence of these two diseases rarely seen in the newborn period. Case presentation A female infant born at 33rd gestational week was found to have constrictive bands in her right lower extremity and flexion contractures in distal joints of lower and upper extremities due to amniotic bands in postnatal physical examination. While being treated for respiratory difficulty, she was diagnosed with osteogenesis imperfecta and treated with bisphosphonates upon being found to suffer bilateral humeral fractures on the sixth day of life. She received respiratory support with mechanical ventilation due to respiratory tract complications related to osteogenesis imperfecta and died on the 384th day of life. Conclusions One should bear in mind that other collagen tissue diseases may accompany the amniotic band syndrome; this possibility should be definitely pursued if clinical suspicion exists.

Objectives To describe an infrequent association of multiple injuries in relation to perinatal trauma and the challenge of clinical examination for proper diagnosis. Case presentation A male newborn was born at 41 weeks gestational age to a multipara diabetic mother, by forceps. Apgar 8/9. Upon admission, he exhibited axial hypotonia, right brachial paresis, a large left parietal cephalohematoma, but no skull fractures or orbital injuries. He also showed ocular misalignment with marked esotropia of the right eye, ptosis and mild anisocoria, suggesting right Horner syndrome, and left facial palsy. During the oculo-cephalic reflex examination he exhibited a normal adduction and impaired abduction of the right eye. The cranial MRI showed an extensive left parietal cephalohematoma, with internal foci of recent bleeding, and supra and infratentorial laminar subdural hematomas. No lesions were evidenced in the cavernous sinuses. The recovery of extra and intraocular motility at one month of life in our patient highlighted the transitory nature of the lesions and was reassuring for the parents. Conclusions This case highlights the importance of a detailed clinical examination in the initial evaluation of a newborn at neurological risk after birth trauma. The association of sixth cranial nerve palsy, Horner syndrome, facial palsy, and brachial plexus injury have been scarcely referred in this context and, if not adequately interpreted, might suggest erroneous diagnoses with very different prognoses.

Objectives To describe the case of a preterm infant with intrauterine HSV infection. To discuss the epidemiology, features and complications associated with intrauterine HSV infection by conducting a review of medical literature. Case presentation A preterm male infant was born with diffuse skin erosions in various stages of healing suggestive of denuded bullae. Prenatal ultrasound showed evolving fetal cerebral ventriculomegaly, suspected Dandy-Walker malformation, and oligohydramnios. Neonatal skin swabs were positive for herpes simplex virus type 2 by polymerase chain reaction and the patient was treated with intravenous acyclovir from birth. Cranial ultrasound and magnetic resonance imaging findings showed severe progressive ex-vacuo ventricular dilatation consistent with intrauterine herpes simplex infection. Due to those findings and progressive multi-organ dysfunction, care was redirected to a palliative path and the child expired at 21 days of age. In retrospect, the mother had a few atypical vesicles on the left hand 4–5 weeks prior to delivery with a presumptive diagnosis of pompholyx. Conclusions Although confirmed intrauterine herpes simplex virus (HSV) infections are very rare, the potentially devastating prognosis and complications as evidenced by our case show that awareness of intrauterine HSV is critical for pediatricians as well as physicians involved in antenatal care. In order to adequately assess, treat and counsel pregnant women, more work is required to detect HSV infection, evaluate possible congenital infection and improve outcomes with fetal and neonatal treatment.

Objectives Birth injuries usually occur with two different mechanisms: trauma due to mechanic stress during labor and hypoxic-ischemic injury. Sometimes these two mechanisms can occur at the same time with a complex clinical picture. Case presentation The baby girl was born at 37 2/7 weeks after a prolonged second stage of labor, weighing 3,725 g, and was admitted to the Neonatal Intensive Care Unit with the diagnosis of hypoxic-ischemic encephalopathy. During follow up she developed multiorgan failure and severe anemia. On the third postnatal day, abdominal bleeding was detected. Laceration in the liver capsule was found and appeared to be the source of bleeding. Conclusions Abdominal bleeding secondary to mechanical laceration of the liver is hard to diagnose and may coexist with perinatal asphyxia.

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.

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.

Objectives Peripherally inserted central catheters (PICC) are used in the neonatal intensive care unit (NICU) setting for medication and nutrition administration. PICCs are easy to place and may remain inserted up to several weeks. Serious complications are rare. Cases of infection, dysfunction, thrombosis, malposition into other vessels, catheter migration, vessel erosion, perforation into pleura, pericardium, abdomen and even into the epidural space with extravasation have been reported [1, 2]. Case presentation We present the case of a preterm infant with a right leg inserted PICC with the tip supposedly being placed in the external iliac vein with further catheter migration into the abdominal wall during the course of treatment. Conclusions Our patient developed extravasation of lipid infusion, which was initially misinterpreted as an abscess due to signs of local inflammation.

Objectives Subcutaneous fat necrosis of the newborn (SCFN) is a rare panniculitis that can affect newborn patients who have experienced perinatal stress, hypoxia, or hypothermia. Risk factors include gestational diabetes and pre-eclampsia. This condition is usually self-limiting; however, it can lead to severe complications, including hypercalcemia. After diagnosis, it is important to monitor calcium levels. There is no current standard for how frequently these levels should be monitored. Case presentation We present a full-term African American male with multiple risk factors, who was diagnosed with SCFN. The patient’s hypercalcemia secondary to SCFN led to renal complications. Conclusions This case highlights the importance of early and frequent monitoring of calcium levels in patients with SCFN.

Objectives The objective of this clinical case report is to highlight the MRI features and staging system which may guide clinicians in determining further management. Case presentation Three different cases with fetal head and neck vascular malformation diagnosed during prenatal screening were presented. MRI demonstrates large cystic neck masses which may compromise fetal airway during delivery. Thus, this required multidisciplinary team management among obstetricians, otolaryngologists, pediatricians, anesthesiologists, and radiologists. A decision for complex birth delivery through the cesarean section aided with EXIT-to-airway procedure was made. Each of these procedures demonstrates the different challenges and outcomes of the neonates which correlated with the characterization and staging based on prenatal MRI. Conclusions EXIT-to-airway procedure in head and neck malformation may be beneficial in transiting complicated and potentially catastrophic delivery situations to a more controlled environment. However, it also needs to align with prenatal MRI evaluation, which provides a more objective assessment guide for the clinicians.

Objectives The aim of this article is to describe a clinical case of Scimitar syndrome, an unusual cause of respiratory distress in the neonatal period. This syndrome is a rare form of partial or total anomalous right lung venous drainage to the inferior caval vein, usually associated with right pulmonary hypoplasia. Case presentation The authors report a case of a 36-week-old newborn admitted to the neonatal intensive care unit with worsening respiratory distress. Chest radiography revealed a characteristic right pulmonary hypoplasia and a tubular structure paralleling the right heart border in the shape of a Turkish sword (“scimitar”). Chest computed tomography angiography confirmed the diagnosis. Conclusions Age of presentation and the severe malformations found, contributed to the patient’s poor outcome in this case.

Objectives We are presenting a unique case of severe laryngo-tracheo-esophageal cleft in a preterm infant who remained asymptomatic for several days after birth. Laryngo-tracheo-esophageal cleft (LTEC) is a rare congenital anomaly, representing approximately 0.2–1.5% of congenital laryngeal malformations [Merei JM, Hutson JM. Embryogenesis of tracheo esophageal anomalies: a review. Pediatr Surg Int 2002;18:319–26]. There is an abnormal, posterior, sagittal communication between the larynx and the pharynx, which may extend downward between the trachea and the esophagus. Case presentation We report a case of LTEC in a preterm newborn whose mother had severe polyhydramnios. A preterm 34-week gestation female newborn was born in our institution whose mother had severe polyhydramnios during third trimester. She was postnatally diagnosed to have type 3 LTEC by the 19th day after birth. In the presence of a large common tracheo-esophageal cavity, the baby did not develop aspiration or respiratory distress despite feeding from birth. Conclusions This case report illustrates the many factors and the probable mechanisms involved in preventing aspiration.

Objectives Urinary tract infections (UTIs) are common in childhood. Distal tubular dysfunction during a UTI is relatively common, but proximal tubular involvement is a unique feature in humans. Case presentation We present the first case of transient generalized proximal tubular dysfunction (renal Fanconi syndrome) in an infant with an UTI. During pregnancy, his mother was treated for Crohn’s disease with infliximab (last dose at 28 weeks of gestation). He presented at the age of six weeks with a reduced intake, and was found to have amino-aciduria, glucosuria, and urinary loss of potassium, bicarbonate and low-molecular-weight proteins. Within a few weeks after antibiotic treatment for the UTI, no proximal tubular disorder remained and the boy is doing well. Conclusions We hypothesize that the inflammatory response caused by the UTI was more profoundly present due to the maternal infliximab therapy, and thereby included not only the distal but also the proximal tubules.

Objectives To report an unusual presentation of a Lobular Capillary Hemangioma in a 48 h old neonate to inform practitioners on the importance of proper identification of the lesion, differential diagnosis and management. Case presentation We report the case of a newborn female presenting with a pedunculated mass on the right hand, port-wine colored that quickly turned dark purple indicating thrombosis. The mass was surgically excised without complications and histopathology analysis reported a LCH. An abdominal and renal ultrasound was requested to rule out any underlying abnormalities. At follow-up, two months later, there is adequate healing and no related complications. Conclusions Lobular Capillary Hemangioma is a rare vascular malformation that occurs at an early age in the head and neck in most cases. We report an unusual case of a newborn female patient with a pedunculated lobular hemangioma on the right hand. Surgical treatment was performed with good cosmetic results. Our case is relevant as it raises awareness of the different diagnoses a congenital mass can be and that we, as physicians, should take into consideration when making a diagnosis and treatment.

Objectives To describe challenges in diagnosis and treatment of congenital neonatal gangrene lesions associated with history of maternal coronavirus disease 2019 (COVID-19) infection. Case presentation A preterm neonate was born with upper extremity necrotic lesions and a history of active maternal COVID-19 infection. The etiology of his injury was challenging to deduce, despite extensive hypercoagulability work-up and biopsy of the lesion. Management, including partial forearm salvage and hand amputation is described. Conclusions Neonatal gangrene has various etiologies, including compartment syndrome and intrauterine thromboembolic phenomena. Maternal COVID-19 can cause intrauterine thrombotic events and need to be considered in a differential diagnosis.