OEIS complex-using MRI in diagnostic: Two case reports.

OEIS complex is an acronym for a specific, extremely rare, grouping of more commonly occurring congenital malformations consisting of an omphalocele (O), exstrophy of the cloaca (E), imperforate anus (I) and spinal defects (S). It is a midline defect occurring in early blastogenesis or in mesodermal migration. The incidence is not known, it has been estimated to be 1 in 200,000-400,000 pregnancies. The etiology is thought to be heterogeneous. Most cases occur sporadically but several reports show occurrence in siblings, concurrent occurrence in monozygotic twins, and more frequent occurrence in IVF pregnancies. We report two cases of OEIS complex. Case I was recognized postnatally in twin gestation. Case II, an IVF pregnancy, was diagnosed prenatally at 35 weeks gestation by ultrasound (US) and further confirmed by magnetic resonance imaging (MRI). The purpose of this report is to present two additional cases of this rare malformation, give further evidence of the OEIS complex occurring more commonly in multiple gestations (monozygotic twins) and IVF pregnancies and illustrate the importance of MRI in prenatal diagnostics in addition to US providing better prenatal counseling, perinatal care, and planning of reconstructive surgical management.

Incidence of congenital diaphragmatic hernia in Olmsted County, Minnesota: a population-based study.

OBJECTIVE: The rate of congenital diaphragmatic hernia (CDH) varies, but most reports estimate the incidence to be less than three per 10,000 births. Our objective was to document the incidence of CDH in a geographically well-defined population using available resources for highly accurate incident case ascertainment. METHODS: We ascertained CDH cases in Olmsted County, Minnesota, United States of America, from 1981 through 2014 using resources of the Rochester Epidemiology Project. Overall and sex-specific incidence rates were calculated, and hospital survival was assessed. RESULTS: A total of 26 incident CDH cases were identified; the overall incidence of 3.6 (95% CI, 2.2-5.0) per 10,000 did not differ significantly over the 34-year study period (p = .28). The estimated incidence was 4.3 (95% CI, 2.2-6.5) for male infants and 2.9 (95% CI, 1.1-4.6) for female infants. The percentage of cases diagnosed prenatally was 33% from 1981 through 2000 and 50% from 2001 through 2014. The rate of survival to discharge in the two periods was 50% and 88%. CONCLUSION: The incidence of CDH in Olmsted County exceeds the majority of published rates, which most likely can be attributed to our comprehensive case ascertainment.

Health-related quality of life in children and adolescents with congenital diaphragmatic hernia: a cross-sectional study.

BACKGROUND: Patients with congenital diaphragmatic hernia (CDH) have a high residual morbidity rate. We compared self-reported health-related quality of life (HRQoL) between patients with CDH and healthy children. METHODS: Forty-five patients with CDH who were born from January 1, 1990, through February 15, 2015, were matched to healthy, age-matched control participants at a 1:2 ratio. The health records of the study participants were reviewed to determine comorbid conditions, and HRQoL was assessed by both the participants and their parents with the Pediatric Quality of Life Inventory (PedsQL). The HRQoL scores of the patients with CDH and the control participants were compared by using analysis of variance to adjust for age group and sex. Among patients with CDH, analysis of variance was used to compare HRQoL scores across groups defined according to their characteristics at initial hospitalization, postdischarge events, and comorbid conditions. RESULTS: Compared with control participants, patients with CDH had lower mean PedsQL scores, as reported by the parent and child, for the physical and psychosocial domains (P < 0.001). Risk factors associated with lower parent-reported HRQoL included bronchopulmonary dysplasia, longer initial hospitalization, severe cognitive impairment, and orthopedic symptoms; among patients with CDH, low HRQoL was associated with chronic respiratory issues. CONCLUSION: Patients with CDH had lower HRQoL compared with healthy participants. Parent-reported HRQoL tended to be higher than child-reported HRQoL. Results were also inconsistent for the risk factors associated with HRQoL obtained by using child- and parent-reported scores. Therefore, when interpreting HRQoL in CDH survivors, a proxy report should not be considered a substitute for a child's self-report.

Congenital Diaphragmatic Hernia: The Side of Diaphragmatic Defect and Associated Nondiaphragmatic Malformations.

Background Congenital diaphragmatic hernia (CDH) has different clinical presentations depending on whether it is right sided (R-CDH) or left sided (L-CDH). Some have suggested that L-CDH and R-CDH may represent different syndromic phenotypes. This theory would be indirectly supported if different nondiaphragmatic anomalies were associated with laterality. We assessed whether CDH laterality is associated with specific types of nondiaphragmatic anomalies. Methods Cases of CDH were retrospectively identified from five centers, and associated congenital anomalies, prenatal diagnosis, demographics, birth characteristics, and side of the CDH were analyzed. CDH characteristics were summarized according to the absence (isolated) or presence (complex) of nondiaphragmatic malformations. Results Among 228 neonates with CDH, 140 (61%) had isolated CDH and 88 (39%) had complex CDH. Complex CDH was significantly associated with being small for gestational age (odds ratio [95% confidence interval, CI]: 8.3 [1.9-35.7]; p = 0.005) and having L-CDH (odds ratio [95% CI]: 3.6 [1.5-8.9]; p = 0.005). The overall proportion with anomalies differed by side (42% for L-CDH, 23% for R-CDH; p = 0.02), but the rates of anomalies in specific organ systems did not differ. Conclusion The rate of associated nondiaphragmatic anomalies by specific organ system did not differ between L-CDH and R-CDH, which suggests that they represent the same phenotypic entity.

Congenital diaphragmatic hernia: outcomes of neonates treated at Mayo Clinic with and without extracorporeal membrane oxygenation.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a rare anomaly with high mortality and long-term comorbid conditions. AIMS: Our aim was to describe the presenting characteristics, treatment, and outcomes of consecutive patients with CDH treated at our institution. METHODS: We performed a retrospective cohort study and identified consecutive neonates treated for CDH from 2001 to 2015 at our institution. For all patients identified, we reviewed hospital and postdischarge data for neonatal, disease, and treatment characteristics. We determined hospital survival overall and also according to the presence of prenatal diagnosis, liver herniation into the chest (liver up), and the use of extracorporeal membrane oxygenation (ECMO) in addition to surgery. We evaluated postdischarge chronic conditions in patients with at least one year of follow-up. RESULTS: Thirty-eight neonates were admitted for treatment during the study period. In three who were in extremis, life support was withdrawn. The other 35 underwent surgical repair, of whom eight received ECMO. The overall survival was 79% (30/38). Survival for those who had surgical correction of CDH but did not need ECMO was 89% (24/27); it was 75% (6/8) for those who received ECMO and had surgery. Hospital survival was lower for liver-up vs liver-down CDH (61% [11/18] vs 95% [19/20]; odds ratio, 0.08; 95% CI, 0.01-0.77; P = 0.01). Among survivors, the median duration of hospitalization was 31 (interquartile range, 20-73) days. Major chronic pulmonary and gastrointestinal disorders, failure to thrive, and neurodevelopmental delays were the most noted comorbid conditions after discharge, and all were more prevalent in those who required ECMO. CONCLUSION: The overall survival of neonates with CDH was 79%. Intrathoracic liver herniation was associated with more frequent use of ECMO and greater mortality. A substantial number of survivors, especially those who required ECMO, experienced chronic conditions after discharge.

Hernia Sac Presence Portends Better Survivability of Isolated Congenital Diaphragmatic Hernia with "Liver-Up".

Objective The objective of this study was to investigate the prognostic value of a hernia sac in isolated congenital diaphragmatic hernia (CDH) with intrathoracic liver herniation ("liver-up"). Study Design A retrospective study from the single tertiary center. Isolated "liver-up" CDH neonates referred to our institution between 2000 and 2015 were reviewed for the presence or absence of a hernia sac. Association between the presence of a hernia sac and survival was assessed. Results Over the study period, there were 29 isolated CDH patients with "liver-up" who were treated, 7 (24%) had a sac, and 22 (76%) did not. Demographics were similar between groups. However, disease acuity, assessed from lower Apgar scores (p = 0.044), lower probability of survival (p = 0.037), and lower admission oxygenation (p = 0.027), was higher in neonates without a sac. Hospital survival was significantly higher for those with sac compared with those without (7/7, 100 vs. 7/22, 32%, p = 0.002). Conclusion The presence of a hernia sac may be associated with better survival for isolated "liver-up" CDH. As the presence of sac can be prenatally detected, it may be a useful marker to aid perinatal decision making.

Neurocognitive outcomes in congenital diaphragmatic hernia survivors: a cross-sectional prospective study.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) survivors may have persistent neurocognitive delays. We assessed neurodevelopmental outcomes in CDH survivors from infancy to late teenage years. METHODS: A cross-sectional study was conducted on 37 CDH survivors to examine neurocognitive functioning. Overall cognitive score was tested with the early learning composite (ELC) of Mullen Scales of Early Learning (n=19), and Full Scale IQ (FSIQ) of Wechsler Intelligence Scale for Children-Fourth Edition (n=18). RESULTS: ELC was 85.7±16.4, lower than the expected norm of 100, P=0.004, and 6 survivors had moderate, and 3 severe delay, which is not greater than expected in the general population (P=0.148). FSIQ was 99.6±19.1, consistent with the expected norm of 100, P=0.922, and 3 survivors had moderate and 2 severe delay, which is greater than expected (P=0.048). Although ELC was lower than FSIQ (P=0.024), within each testing group overall cognitive ability was not associated with participant age (ELC, P=0.732; FSIQ, P=0.909). Longer hospital stay was the only factor found to be consistently associated with a worse cognitive score across all participants in our cohort. CONCLUSIONS: A high percentage of survivors with CDH have moderate to severe cognitive impairment suggesting that these subjects warrant early testing with implementation of therapeutic and educational interventions.

Survival prediction of high-risk outborn neonates with congenital diaphragmatic hernia from capillary blood gases.

BACKGROUND: The extent of lung hypoplasia in neonates with congenital diaphragmatic hernia (CDH) can be assessed from gas exchange. We examined the role of preductal capillary blood gases in prognosticating outcome in patients with CDH. METHODS: We retrospectively reviewed demographic data, disease characteristics, and preductal capillary blood gases on admission and within 24 h following admission for 44 high-risk outborn neonates. All neonates were intubated after delivery due to acute respiratory distress, and were emergently transferred via ground ambulance to our unit between 1/2000 and 12/2014. The main outcome measure was survival to hospital discharge and explanatory variables of interest were preductal capillary blood gases obtained on admission and during the first 24 h following admission. RESULTS: Higher ratio of preductal partial pressure of oxygen to fraction of inspired oxygen (PcO2/FIO2) on admission predicted survival (AUC = 0.69, P = 0.04). However, some neonates substantially improve PcO2/FIO2 following initiation of treatment. Among neonates who survived at least 24 h, the highest preductal PcO2/FIO2 achieved in the initial 24 h was the strongest predictor of survival (AUC = 0.87, P = 0.002). Nonsurvivors had a mean admission preductal PcCO2 higher than survivors (91 ± 31 vs. 70 ± 25 mmHg, P = 0.02), and their PcCO2 remained high during the first 24 h of treatment. CONCLUSION: The inability to achieve adequate gas exchange within 24 h of initiation of intensive care treatment is an ominous sign in high-risk outborn neonates with CDH. We suggest that improvement of oxygenation during the first 24 h, along with other relevant clinical signs, should be used when making decisions regarding treatment options in these critically ill neonates.

Cardiopulmonary exercise performance is reduced in congenital diaphragmatic hernia survivors.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is associated with lung hypoplasia. CDH survivors may have pulmonary morbidity that can decrease cardiopulmonary exercise. We aimed to examine whether cardiopulmonary exercise testing (CPET) results differ in CDH survivors versus healthy age-matched controls and whether CPET results among CDH survivors differ according to self-reported daily activity. METHODS: In one medical center in Croatia, CDH survivors-patients with surgically corrected CDH who were alive at age 5 years-were invited to participate in spirometry and CPET. Values were compared with those of controls matched 2:1 by age and sex for each CDH survivor aged 7 years or older. RESULTS: Among 27 CDH survivors aged 5-20 years, 13 (48%) had continued symptoms or spirometric evidence of pulmonary disease. Compared with controls (n = 44), survivors (n = 22) had lower peak oxygen consumption (V˙O2 mean [SD], 35.7 [6.9] vs. 45.3 [8.2] ml/kg per min; P < 0.001). At peak exercise, V˙O2/heart rate (P < 0.001), tidal volume (P = 0.005), and minute ventilation (P < 0.001) were lower in survivors, but the maximal respiratory rate was not different (P = 0.72). Among survivors, mean (SD) V˙O2peak (ml/kg per min) differed by self-reported activity level: athletic, 40.3 (5.0); normal, 35.8 (6.5); and sedentary, 32.1 (6.8) (by ANOVA, P = 0.10 across three groups and P = 0.04 athletic vs. sedentary). CONCLUSION: More than half of CDH survivors continue to have chronic pulmonary disease. CDH survivors had lower aerobic exercise capacity than controls. Self-reporting information on daily activities may identify CDH patients with low V˙O2max who may benefit from physical training. Pediatr Pulmonol. 2016;51:1320-1329. © 2016 Wiley Periodicals, Inc.

Epidemiology and Outcomes of Congenital Diaphragmatic Hernia in Croatia: A Population-Based Study.

BACKGROUND: Outcomes of neonates with congenital diaphragmatic hernia (CDH) are variable; reports are frequently limited to the experience of single tertiary care centres-a possible source of bias. Population-based studies decrease survivor bias and provide additional insight into this high-mortality condition. The objective of this study was to examine the incidence and outcomes of CDH in Croatia. METHODS: All cases of CDH in Croatia from 2001 through 2013 were ascertained from public health records. Overall and sex- and region-specific incidence rates were calculated, and characteristics associated with 1-year survival were assessed. RESULTS: We identified 145 cases of CDH during the study period, for an incidence of 2.67 per 10 000 total births. The incidence did not differ by calendar year (P = 0.38) or geographic region (P = 0.67). There was a slightly higher incidence among males (rate ratio, 1.37, 95% CI 0.99, 1.91). The 1-year survival rate was 33.1% for the entire cohort and 47.9% for liveborns who received any treatment at an intensive care unit. From multivariable analysis, survival was decreased in neonates with left CDH, liver up (odds ratio 0.1, 95% CI, 0.03, 0.4) and increased when treated in a centre with higher case volume (odds ratio 12.8, 95% CI, 2.2, 72.1). CONCLUSIONS: The incidence of CDH in Croatia is within the range of previous reports. Survival was substantially higher in neonates treated in a centre with higher case volume, which suggests that centralisation of medical care for CDH may be warranted in Croatia.

Survival of outborns with congenital diaphragmatic hernia: the role of protective ventilation, early presentation and transport distance: a retrospective cohort study.

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a congenital malformation associated with life-threatening pulmonary dysfunction and high neonatal mortality. Outcomes are improved with protective ventilation, less severe pulmonary pathology, and the proximity of the treating center to the site of delivery. The major CDH treatment center in Croatia lacks a maternity ward, thus all CDH patients are transferred from local Zagreb hospitals or remote areas (outborns). In 2000 this center adopted protective ventilation for CDH management. In the present study we assess the roles of protective ventilation, transport distance, and severity of pulmonary pathology on survival of neonates with CDH. METHODS: The study was divided into Epoch I, (1990-1999, traditional ventilation to achieve normocapnia), and Epoch II, (2000-2014, protective ventilation with permissive hypercapnia). Patients were categorized by transfer distance (local hospital or remote locations) and by acuity of respiratory distress after delivery (early presentation-occurring at birth, or late presentation, ≥ 6 h after delivery). Survival between epochs, types of transfers, and acuity of presentation were assessed. An additional analysis was assessed for the potential association between survival and end-capillary blood CO2 (PcCO2), an indirect measure of pulmonary pathology. RESULTS: There were 83 neonates, 26 in Epoch I, and 57 in Epoch II. In Epoch I 11 patients (42%) survived, and in Epoch II 38 (67%) (P = 0.039). Survival with early presentation (N = 63) was 48 % and with late presentation 95% (P <0.001). Among early presentation, survival was higher in Epoch II vs. Epoch I (57% vs. 26%, P = 0.031). From multiple logistic regression analysis restricted to neonates with early presentation and adjusting for severity of disease, survival was improved in Epoch II (OR 4.8, 95%CI 1.3-18.0, P = 0.019). Survival was unrelated to distance of transfer but improved with lower partial pressure of PcCO2 on admission (OR 1.16, 95%CI 1.01-1.33 per 5 mmHg decrease, P = 0.031). CONCLUSIONS: The introduction of protective ventilation was associated with improved survival in neonates with early presentation. Survival did not differ between local and remote transfers, but primarily depended on severity of pulmonary pathology as inferred from admission capillary PcCO2.

Malformations associated with congenital diaphragmatic hernia: Impact on survival.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with high mortality. Survival is influenced by the extent of pulmonary hypoplasia and additional congenital defects. The purpose of this study was to assess the association of congenital anomalies and admission capillary carbon dioxide levels (PcCO2), as a measure of extent of pulmonary hypoplasia, on survival in neonates with CDH. METHODS: This is a retrospective review of neonates with CDH admitted to a tertiary neonatal intensive care unit between 1990 and 2014. Logistic regression was used to assess whether hospital survival was associated with admission PcCO2 or associated anomalies (isolated CDH, CDH with cardiovascular anomalies, and CDH with noncardiac anomalies). The probabilities of survival (POS) score, based on birth weight and 5-min Apgar as defined by the Congenital Diaphragmatic Hernia Study Group were included as a covariate. RESULTS: Of 97 patients, 55 had additional malformations (cardiovascular n=12, noncardiac anomalies n=43). POS was lower in CDH with other anomalies compared to isolated CDH. Survival rate was 61.9%, 53.5% and 41.7% in isolated CDH, CDH with noncardiac anomalies and CDH with cardiovascular anomalies, respectively. After adjusting for POS score the likelihood of survival in CDH groups with additional anomalies was similar to isolated CDH (OR 0.95, 95% CI 0.22-4.15, and 1.10, 0.39-3.08, for CDH with and without cardiovascular anomalies, respectively). After adjusting for POS score, lower PcCO2 levels (OR=1.25 per 5mmHg decrease, P=0.003) were associated with better survival. CONCLUSIONS: Neonates with CDH have a high prevalence of congenital malformations. However, after adjusting for POS score the presence of additional anomalies was not associated with survival. The POS score and admission PcCO2 were important prognosticating factors for survival.

Brain drain: final year medical students' intentions of training abroad.

BACKGROUND: In Croatia, a new European Union (EU) member state since July 2013, there is already a shortage of around 3280 doctors to reach the European average. OBJECTIVES: To investigate the emigration intentions of the current cohort of final year medical students at Zabreb School of Medicine. METHODS: An electronic questionnaire was used in June 2013 to assess the attitudes of 232 final year medical students towards working conditions abroad and expectations for career opportunities in Croatia following accession to the EU. RESULTS: With an overall response rate of 87%, more than half of the surveyed students (106/202, 53%) intended to travel abroad, either for specialty (52/202, 26%) or subspecialty (54/202, 27%) training. More female students (58/135, 43%) than male students (17/62, 27%) indicated they would not emigrate. Most attractive emigration destinations were: Germany (34/121, 28%), USA (19/121, 16%), the UK (19/121, 16%), Switzerland (16/121, 13%) and Canada (11/121, 9%). The most important goals that respondents aimed to achieve through training abroad were to excel professionally (45/120, 38%), to prosper financially (20/120, 17%) and to acquire new experiences and international exposure (31/120, 26%). CONCLUSIONS: Students' motivating factors, goals for and positive beliefs about training abroad, as well as negative expectations regarding career opportunities in Croatia, may point towards actions that could be taken to help make Croatia a country that facilitates medical education and professional career development of young doctors.

Severe liver injury while using umbilical venous catheter: case series and literature review.

OBJECTIVE: Proper position of umbilical venous catheter (UVC) is of importance to avoid severe complications. We review clinical presentations of neonates with UVC who developed catheter-associated liver injury. STUDY DESIGN: We reviewed institutional intensive care database (2008-2013) and identified neonates with UVCs who developed severe hepatic injury. We recorded admission diagnosis, gestational age, birth weight, number of days the umbilical catheter was in place, its radiological position at insertion and at the time of injury, presenting clinical signs, and outcomes. RESULTS: Of 1,081 neonates, 9 (0.8% [95% exact binomial confidence interval, 0.4-1.6%]) with UVC developed severe hepatic injury. All had the UVC malpositioned within the liver circulation. All presentations were life threatening, with acute abdominal distension (hepatomegaly) being the most consistent sign. Two neonates died from complications which were unrelated to catheter-associated liver injury. CONCLUSIONS: In all neonates with liver injury, UVC was malpositioned within the portal circulation. Despite the fact that our report provides only circumstantial evidence for the mechanism of injury, it supports reports which suggest that "low" UVC position increases potential for this type of complication. Acute onset of abdominal distension in a neonate with UVC should prompt ultrasonographic evaluation of position of the catheter tip.

Anti-neutrophil cytoplasmic antibody positivity in five children with systemic lupus erythematosus--what is the importance of this finding?

Juvenile systemic lupus erythematosus (JSLE) is a systemic autoimmune chronic disease that can affect any part of the body. It is characterized by the formation of antibodies against nuclear antigens. Vasculitis may be found in SLE, but it scarcely complies with anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) criteria. We report five cases of severe JSLE associated with AAV diagnosed between 1991 and 2013 in three university-based tertiary care centers. The patients (3 girls and 2 boys, aged 12 to 17) presented with a severe clinical picture and the following features: cytopenia (n=5), autoimmune hepatitis (n=3), lupus nephritis (n=1), pancreatitis (n=1), secondary antiphospholipid syndrome (n=2), impending respiratory failure (n=2), and gastrointestinal bleeding (n=1). All patients were proteinase 3 (PR3) ANCA positive, while two of them were myeloperoxidase (MPO) and PR3 ANCAs positive at the same time. They were treated with corticosteroids and immunosuppressive drugs. Remission of the disease was achieved in three patients. The course of the disease was worsening in two patients and we included rituximab (anti-CD20) in therapy. All of our patients presented as the most severe SLE patients, who must be diagnosed as soon as possible and treated very intensively. Since the comorbidity of JSLE and AAV occurs very rarely in children, presentation of such patients, their clinical pictures, treatment, and the course of the diseases are experiences that can be of great help.

Management of delayed hemorrhage after partial vocal cord cordectomy.

A patient developed brisk airway bleeding after being discharged earlier that day following an uneventful partial vocal cord cordectomy. This emergent complication required management by the team of anesthesiologists and otorhinolaryngologists. A plausible explanation for the occurrence of this complication was the presence of aberrant arterial blood supply in the area of the vocal cords where the surgery was performed.

Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures.

BACKGROUND: Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are associated with life-threatening perioperative complications, including rhabdomyolysis, hyperkalemia, and hyperthermia. Current recommendations contraindicate use of succinylcholine and volatile anesthetics; however, the latter recommendation remains controversial. OBJECTIVE: To review the perioperative outcomes of patients with DMD and BMD. METHODS: We reviewed records of patients with DMD or BMD who underwent anesthetic management at our institution from January 1990 through December 2011. RESULTS: We identified 47 patients (DMD, 37; BMD, 10) who underwent 117 anesthetic exposures (DMD, 101; BMD, 16). Volatile anesthetic agents were used 66 times (DMD, 59; BMD, 7). One patient with undiagnosed BMD received succinylcholine and developed acute rhabdomyolysis and hyperkalemic cardiac arrest. All other major complications were attributed to the procedure (i.e., large bleeding), to preexisting comorbidities (i.e., respiratory failure, cardiac disease), or to both. CONCLUSIONS: Use of succinylcholine in children with dystrophinopathy is contraindicated. These patients have significant comorbidities and are frequently undergoing extensive operations; complications related to these factors can develop, as evidenced by our series. These complications may occur with use of volatile and nonvolatile anesthetics. However, because most of our patients were older than 8 years at the time of surgery, our observation cannot be generalized to younger dystrophin-deficient children.

Isolated cor triatriatum sinistrum and pregnancy: case report and review of the literature.

PURPOSE: Isolated cor triatriatum sinistrum (CTS) is a heart malformation in which a perforated fibromuscular membrane divides the left atrium into two chambers. When communication between these chambers is restricted, a patient may have signs and symptoms of mitral stenosis. The later stages of pregnancy are associated with tachycardia and increases in intravascular volume. We describe how this altered physiology may affect pregnant women with asymptomatic CTS. We also review the literature relating to pregnancy in patients with CTS. CLINICAL FEATURES: A 30-yr-old primigravida, at 40 weeks of gestation with pre-pregnancy diagnosed asymptomatic CTS, was admitted for delivery. She had no cardiac symptoms during pregnancy, and her vaginal delivery under epidural analgesia was uneventful. This cardiac malformation is infrequently described in pregnant women, but a literature review showed that the physiology of late pregnancy with increases in hemodynamic variables may result in cardiac decompensation. CONCLUSION: While our patient with isolated CTS and an unrestrictive intra-atrial membrane had an asymptomatic pregnancy and an uneventful labour, the literature review suggests that the increase in intravascular volume and heart rate that occurs during late pregnancy and after delivery may result in cardiac decompensation, even in patients with asymptomatic CTS.

Pregnancy and vaginal delivery in epidural analgesia in woman with cerebrospinal fluid shunt.

Hydrocephalus is a medical condition characterized by enlargement of cerebral ventricles due to abnormal cerebrospinal fluid accumulation. Hydrocephalic women with cerebrospinal fluid (CSF) shunts are now surviving to reproductive age, but still there are doubts regarding the mode of delivery, analgesia and anesthesia. Postpartal complications are more frequently described in deliveries ended by cesarean section than in spontaneous vaginal deliveries. We present a case of labor in the 32-year old woman, with congenital hydrocephalus and a preexisting ventriculoperitoneal (VP) shunt. After thorough review of current literature, we came to conclusion that without absolute neurosurgical indication or acute development of listed symptoms (headaches, irritability, light sensitivity, hyperesthesia nausea, vomiting, vertigo, migraines, seizures, weakness in the arms or legs, strabismus and double vision) the best way to finish the pregnancy of woman with VP shunt is spontaneous vaginal delivery with the use of epidural analgesia, mediolateral episiotomy and vacuum extraction.