Volume 7, Issue 1

Abnormal invasive placenta (AIP) is subdivided into total, partial and focal types. A diagnosis of total AIP is made when the entire placenta adheres to the uterine myometrium. We experienced a patient with total AIP who had a history of an emergency cesarean section at 36 weeks of gestation due to placental abruption, and massive hemorrhage due to severe uterine atony treated conservatively with blood transfusion. From our experience with the present case, we hypothesize that total AIP resulted from thinning of the entire endometrium, as can occur with an ischemic change of the entire uterus after disruption of Nitabuch’s layer in severe placental abruption and intramuscular extravasation with uterine atony. This situation might adversely affect placental implantation in the current pregnancy.

Pregnancy-associated atypical hemolytic uremic syndrome (p-aHUS) is a rare disorder, with an estimated incidence of 1 in 25,000 pregnancies [Fakhouri F, Roumenina L, Provot F, Sallee M, Caillard S, Couzi L, et al. Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations. J Am Soc Nephrol. 2010;21:859–67.]. Unlike classic hemolytic uremic syndrome (HUS), aHUS is not related to Escherichia coli 0157:H7 infections. Rather, it arises from uncontrolled alternative complement pathway activation leading to diffuse endothelial damage. The formation of the resulting fibrin and platelet microthrombi in the vasculature leads to hemolysis, thrombocytopenia and ischemic end-organ damage in the form of acute kidney injury [Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009;361:1676–87; Legendre CM, Licht C, Muus P, Greenbaum LA, Babu S, Bedrosian C, et al. Terminal complement inhibitor eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med. 2013;368:2169–81; Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:60; Shen YM. Clinical evaluation of thrombotic microangiopathy: identification of patients with suspected atypical hemolytic uremic syndrome. Thromb J. 2016;14(Suppl 1):19.]. Triggers for hyperactivation of the complement pathway include infection, inflammation, malignancy, endothelium-affecting drugs, maternal-fetal hemorrhage and pre-eclampsia [Shen YM. Clinical evaluation of thrombotic microangiopathy: identification of patients with suspected atypical hemolytic uremic syndrome. Thromb J. 2016;14(Suppl 1):19.]. Thirty percent of individuals with aHUS are found to have mutations in the genes encoding complement regulatory proteins, such as protein factor H, complement factor I and complement 3 [Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. N Engl J Med. 2009;361:1676–87; Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:60.]. Outcomes of an untreated aHUS are poor: up to 50% of patients with aHUS progress to end-stage renal disease within a year and 25% die during the acute phase [Loirat C, Fremeaux-Bacchi V. Atypical hemolytic uremic syndrome. Orphanet J Rare Dis. 2011;6:60; Laurence J, Haller H, Mannucci PM, Nangaku M, Praga M, Rodriguez de Cordoba S. Atypical hemolytic uremic syndrome (aHUS): essential aspects of an accurate diagnosis. Clin Adv Hematol Oncol. 2016;14(Suppl 11):2–15.]. We present an unusual case of a 37-year-old primigravida who developed p-aHUS in the setting of hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. She was successfully treated with a relatively novel medication; eculizumab, a terminal complement inhibitor. In contrast to previous reports of long-term treatment, she received a total of six doses of eculizumab and remained in remission at 12 months postpartum.

Objective To compare the sensitivity and specificity of leukocyte esterase activity (LEA) to histopathological examination in diagnosing chorioamnionitis. Methods We compared the diagnostic tests performed at Dr. Mohammad Hoesin Hospital, Palembang, Indonesia, from September 2015 to April 2016. Ninety-one pregnant women were included in the study. The LEA and histopathological examination were carried out with neonatal sepsis the main outcome. Data were analyzed using the SPSS version 21.0 and MedCalc statistics. Results Chorioamnionitis was detected in 54 (77.1%) patients with a gestational age ≥37 weeks and in 16 (22.9%) patients with a gestational age <37 weeks. The duration of membrane rupture was significantly associated with chorioamnionitis (P = 0.001 and P = 0.011). Neonatal sepsis was also significantly associated with chorioamnionitis in both groups (P = 0.014 and P = 0.036). A LEA value with the cut-off point >0.5 was able to significantly predict chorioamnionitis with 98.6% sensitivity and 95.2% specificity, providing better accuracy in diagnosing chorioamnionitis in the preterm pregnancy group. Conclusion LEA had a very good predictive value for chorioamnionitis with better accuracy in diagnosing chorioamnionitis in preterm pregnancy.

Background Deficiency of factor XII (FXII) is widely considered to have a detrimental effect on pregnancy. Several reports underline the increased risk for antenatal complications with few published case reports of uncomplicated deliveries. The main objective of our article is to perform a systematic review to highlight pregnancies with severe deficiency of FXII that have been delivered uneventfully, along with presenting our relative case of a woman with severe deficiency of FXII. Materials and methods A systematic review was performed in the Pubmed database. Inclusion criteria were considered to be case reports and case series presenting delivery of uncomplicated pregnancies in women with severe FXII deficiency. Medical records of our patient were also reviewed in terms of signs and symptoms, laboratory and imaging examinations and neonatal outcomes. Results There were 62 abstracts derived while 44 were assessed for eligibility. There were finally three case reports of women with FXII deficiency delivering live newborns and one case series of 12 women with a final outcome of 19 deliveries. Regarding our case presentation, the woman with FXII levels <12%, after a neonatal death because of extreme prematurity (24 weeks + 4 days), was set in regular follow-up and treatment with bemiparin natriate, 3.5 mg/kg and acetylsalicylic acid, 100 mg/day. She finally managed to have her second pregnancy delivered at 38 weeks + 3 days, her third pregnancy ended up as a miscarriage and her fourth pregnancy was also delivered at 37 weeks + 4 days. Conclusion Despite the increased risk for antenatal complications, appropriate follow-up of pregnancies with severe FXII pregnancy may finally lead to an uneventful delivery.

Splenic rupture in pregnancy is a rare event. It is generally due to trauma or an underlying splenic pathology. Timely diagnosis and intervention of splenic rupture is essential given the high rates of associated maternal and fetal morbidity and mortality. This case illustrates a 38 year old lady in the third trimester of pregnancy who presented with maternal collapse and a non-reassuring foetal heart rate tracing following a fall at home one week previously. A massive hemoperitoneum was identified at caesarean section and a splenectomy was performed. Histological examination revealed a littoral cell angioma of the spleen. This vascular tumour arising in the splenic red pulp sinuses is a rare entity and specific immunophenotypic features help distinguish it from other vascular tumours of the spleen. Both trauma and an underlying splenic pathology led to splenic rupture in this unique case.

Objective This is a rare presentation of a fetus with a right mainstem bronchus occlusion leading to congenital high airway obstruction syndrome (CHAOS). Methods A review of 15 articles reporting on the use of the ex utero intrapartum treatment (EXIT) procedure. Results A total of 22 reported cases of CHAOS had undergone the EXIT procedure from 1994 to 2016. Seventy-seven percent of fetuses that underwent EXIT were alive at the last point of follow-up, but only 36% were alive without any sequelae. Conclusion CHAOS is a rare malformation, but after the development of EXIT, survival is a viable option. It is associated with other malformations, but its etiology is entirely unknown.

The severe scarring and distortion which follows genital mutilation may make subsequent childbirth difficult or traumatic. Deinfibulation has been advocated and practiced in hospitals. We present a patient who deinfibulated herself during a successful home delivery, and presented to hospital thereafter.

Mullerian anomalies occur in about 0.5% of reproductive-aged women; many remain asymptomatic only being discovered during surgery or on imaging done for unrelated reasons. These result from the abnormal formation, fusion or resorption of the mullerian duct during development. A unicornuate uterus with a rudimentary horn is one of the rarer subtypes, with such anomalies making up about 5% of all mullerian anomalies. Pregnancy in a non-communicating uterine horn (NCUH) is extremely rare, with an incidence between 1:100,000 and 1:140,000. There is a high risk of uterine rupture if pregnancy occurs in a non-communicating uterine horn. We present here a case of a non-communicating rudimentary horn pregnancy, with a successful outcome.

Pregnancy with uncorrected tetralogy of Fallot (TOF) is a rare event, and increases the risk for fetal growth restriction and adverse outcomes related to worsening right-to-left shunt, cyanosis, pulmonary hypertension and arrhythmia. Obstetric management is made on a case-by-case basis. Patients who remain stable can achieve term pregnancies and the mode of delivery can be based on obstetric indications. We present the case of a 21-year-old primigravida with baseline cyanosis, oxygen saturation 90% at rest (74% with activities), hematocrit 50%, and echocardiogram showing pulmonary atresia, ventricular septal defect (VSD), and major aorto-pulmonary collateral arteries (MAPCA). We managed her with aspirin, metoprolol and oxygen supplementation. She delivered at 38 weeks (fetal growth restriction) via cesarean; she received epidural and endocarditis prophylaxis. Three years later, she presented pregnant and had a repeat cesarean at term. Both newborns had normal structural hearts. Knowledge of the cardiovascular physiology during pregnancy and baseline assessment, and a multidisciplinary management are key aspects in the management of these cases during pregnancy.

Background Acute myocardial infarction (AMI) in pregnancy is a rare event and of the causes, coronary artery vasospasm (CAV) is considered even more uncommon. Purpose We present a new case report of a woman at 32 weeks of pregnancy with an AMI from CAV with a normal coronary angiogram. We performed a systematic review of similar cases of spontaneous AMI related to CAV to better understand its characteristics and management. AMI was defined as elevated cardiac enzymes (troponin or CKMB) with chest pain and/or electrocardiogram (EKG) changes consistent with ischemia. Methods We use the terms “acute myocardial infarction”, “myocardial infarction”, “coronary artery vasospasm” and “pregnancy” for our PubMed review. We also evaluated all references in identified manuscripts. Six cases of AMI in pregnancy due to CAV have been reported as of November 2016, including ours. Results and conclusion Six cases of AMI due to CAV during pregnancy or postpartum are reported in the literature, including ours. Patients experiencing this condition tend to be of advanced maternal age, multigravida and in their third trimester or postpartum. Successful management with a combination of long acting nitrates and/or calcium channel blockers achieved symptomatic control in all published cases. Obstetric outcomes were mostly normal, with the majority experiencing uncomplicated deliveries at term.

Metaphyseal corner fractures (MCFs) in infants are strongly associated with inflicted injuries. These fractures occur due to shearing forces and involve the chondro-osseous junction in skeletally immature bones. We present the unique case of a MCF of the distal femur caused in utero by external cephalic version (ECV). The illustration of this case links the mechanism to the injury. It is critical for this mechanism to be understood to prevent an erroneous diagnosis of child abuse.

Hydrothorax is the accumulation of fluid in the pleural space. A large fetal pleural effusion (hydrothorax) was identified within the right hemithorax in a 22-year-old woman at 39 weeks of gestation. Because the fetal hydrothorax was detected at 39 weeks of gestation, we did not perform any intervention antenatally. A male fetus was delivered by an elective cesarean section at 39 weeks of gestation. He did not require intubation, ventilation or resuscitation in the operating room, and had a good health status. The baby was followed-up in the neonatal intensive care unit. Six days after the birth, the pleural effusion (hydrothorax) was completely resolved spontaneously. Conservative management may be regarded as an option for isolated fetal hydrothorax in uncomplicated, stable cases without hydrops.

Congenital myotonic dystrophy type 1 presents with severe generalized weakness, hypotonia and respiratory involvement after birth with high mortality and poor outcome among survivors. We report on a patient that prenatally showed polyhydramnios and arthrogypotic attitude. Postnatal examination was compatible with the diagnosis of congenital myopathy. A rare finding associated with the patient was chylothorax. Genetic testing confirmed the diagnosis of myotonic dystrophy. Few prenatal and neonatal cases of congenital myotonic dystrophy associated with chylothorax have been reported in the literature. We reviewed all cases reported to date showing congenital myopathic weakness in association with chylothorax to delineate the clinical manifestations that allow an early diagnosis and management of this syndrome. Possible mechanisms to explain the association between myopathy and chylothorax are also discussed.

Air leaks are known complications associated with mechanical ventilation, with a higher incidence in more premature babies. Pneumothorax and pneumomediastinum are the most common ones and the majority would resolve spontaneously without active intervention. Subcutaneous emphysema is very rare, with few reported cases in neonates. We report here a case of extensive subcutaneous emphysema, pneumothorax and pneumomediastinum in a late preterm baby developed while on nasal continuous positive airway pressure (nCPAP) respiratory support.

Background Brain abscesses are possible but very uncommon complications of bacterial sepsis and meningitis in neonates. We report a case of multiple brain abscesses in a preterm neonate as a complication of Serratia marcescens sepsis. Case The female preterm weighing 1990 g was delivered by cesarean section at 32 weeks of gestation. Apart from moderate respiratory distress syndrome (RDS), the baby was in a good condition with no indicators of perinatal infection. On the 3 rd day of life, the clinical status deteriorated and the sepsis screen was positive. The baby was intubated and, along with other intensive measures, treated with high doses of vancomycin and imipenem. Serattia marcescens was isolated in hemoculture. The baby clinicaly improved in the following days, but the cranial ultrasound revealed multiple hypoechoic lesions in parietal lobes bilaterally. Magnetic resonance imaging (MRI) of the brain showed multiple (five) hypodense lesions with the peripheral enhancement suggestive of intra-parenchymal abscesses. The neurosurgical consilium suggested conservative treatment with antibiotics and weekly neuroimiging follow-up. The antibiotic treatment was conducted for a total of 8 weeks. The final MRI showed a total regresion of previous abscesses with the formation of small cavitations. The clinical and neurological examination of the baby was normal as was the EEG. The baby was discharged with a recommendation of neurological follow-up. Conclusion A multidisciplinary team approach, including neurosurgeons, neonatologists and infectious disease specialists, is needed for a decision on treatment of brain abscesses in neonates. Serial imaging is important in the assessment of the efficacy of treatment.

Congenital hemangiomata are rare benign vascular tumors, presenting as fully mature lesions at birth. Three types have been described; the “rapidly involuting congenital hemangioma” (RICH), the “non-involuting congenital hemangioma” (NICH)and the “partially involuting congenital hemangioma” (PICH). We herein report on a RICH type congenital hemangioma, identified during a fetal morphology ultrasound performed at 19 weeks’ gestation. Early diagnosis allowed close surveillance of the fetus and neonate and observation of the natural course of this lesion. Although being a potentially life-threatening condition, no intervention was required. It presented as a 4 cm vascular tumor on the forehead at time of the cesarean section and diminished rapidly over the first 4 neonatal months, resulting in some skin excess and discoloration which is expected to entirely resolve by 14 months of age.

We present a case of a term male neonate born with congenital diaphragmatic hernia (CDH), double-outlet right ventricle (DORV) and a “picture of genetic syndrome”, antenatally diagnosed, without any confirmation of a particular syndrome as no amnioparacentesis was performed. The postnatal chromosomal analysis revealed trisomy 18.

Hemolytic disease of the fetus and newborn (HDFN) due to anti-D antibodies is a well-known complication of rhesus (Rh) incompatibility, encountered in D-positive babies born to alloimmunized D-negative mothers who have been sensitized during previous labor or abortion. Here, we report a case of significant hemolytic disease of the newborn due to the presence of anti-D antibodies in an Rh-positive baby born to an Rh-positive mother. The boy presented at day 1 of life with neonatal jaundice and required intensive phototherapy. His hemoglobin (Hb) concentration gradually dropped from 17 g/dL to 6.6 g/dL. The blood bank workup revealed O Rh-positive blood group, with a positive direct antiglobulin test (DAT) and confirmed the presence of anti-D antibodies. His mother was typed as O Rh positive, with a negative DAT and positive anti-D. He required two blood transfusions, and his Hb stabilized at the age of 7 weeks. Anti-D HDFN is a rare complication of Rh-positive and Rh-weak positive pregnancies. The lack of awareness of this phenomenon is often a source of confusion for clinicians. A literature review of similar cases and possible explanations are discussed.

Cholestasis in the first days of life is uncommon in neonates. Neonatal cholestasis is usually associated with shock, sepsis, alloimmunity, metabolic disorders or biliary obstruction. A congenital intrahepatic portosystemic shunt results from failed involution of primordial liver vessels during the first days of life. Resulting shunts can lead to hepatic encephalopathy or liver tumors. A congenital intrahepatic portosystemic shunt should be considered when an alternative explanation cannot be found. In most cases, congenital intrahepatic portosystemic shunts will involute spontaneously by 1–2 years of age; however, surgical or radiologic closure may be needed.

Pulmonary interstitial emphysema (PIE) is most commonly seen in the setting of preterm, low birth weight neonates with lung disease. It exists on a spectrum with pneumomediastinum and pneumothorax and is often a transient phenomenon. This condition has been rarely reported in neonates while only on nasal continuous positive airway pressure (CPAP) without mechanical ventilation, but only as a localized presentation. We present a case of a late preterm neonate with diffuse PIE complicated by bilateral pneumothoraces, requiring chest tubes, with congenital thyroid aplasia as well.