Successful Management of Takayasu’s Arteritis with Pulmonary Hypertension in Pregnancy: A Rare Case

Introduction: Takayasu’s arteritis (TA) is a rare systemic, chronic inflam-matory, progressive, idiopathic disease of aorta and its main branches. Taka-yasu’s arteritis causes narrowing, occlusion and aneurysm of arteries. The eti-ology of TA is still unknown. It may be autoimmune or genetic in origin or infective diseases. It is commonly found in Asia and oriental countries. Worldwide annual incidence is 1.2 - 2.6 cases/million. Women are affected in 80% - 90% of cases with age of onset below 40 years. TA does not worsen or improve with pregnancy but has adverse effect on pregnancy in the form of abortion, Intrauterine fetal death, superimposed preeclampsia, Intrauterine growth restriction, abruption and congestive heart failure. Because the disease is common in women of childbearing age, management of pregnancy in these patients becomes important;however optimal management for pregnant pa-tients with this disease has not yet been established. Due to the manifold car-diovascular complications that can occur in the course of the disease, man-agement of pregnancies in TA patients is a challenge for the clinician. An in-terdisciplinary collaboration of obstetrician, rheumatologist, cardiologist and anaesthetist is necessary to improve maternal and fetal prognosis. This was the first reported case of TA in pregnancy in Hasan Sadikin Hospital with superimposed preeclampsia and pulmonary hypertension. Materials and Methods: The patient, G1P0A0 was diagnosed as TA since one year ago, and when the patient was pregnant, the patient routinely came to obstetric and rheumatology clinics. Angiographic examination showed dilatation of aorta ascendant, narrowing of aorta descendent at 8 thoracal vertebrae, and arcus aorta, abdominal aorta and the branches were without abnormalities that lead to type IIb TA. On physical examination blood pressure was 200/100 mmHg on the right arm, and 140/100 mm Hg on the left arm, right hand pulse 120 x/m, left hand pulse 113 x/m, respiration rate 20 x/m, temperature 36°C. Laboratory values showed Elevated C-reactive protein 6.8 Mg/dl, anemia hemoglobin 10.5 g/dL, elevated rate of sedimentation of blood 36 mm/hours, and proteinuria + 2. The results of other examinations: Glucose, Ureum, Creatinine, GOT, GPT and electrolytes were in normal limits. Thoracal examination/rontgen showed revealed cardiomegaly. Echocardiogram at 20 - 21 weeks gestational age showed an ejection fraction of 55%, hypertensive heart disease and pulmonal hypertension. The ultrasound examination at 36 - 37 weeks’ gestation showed a 2066 g estimated weight (IUGR simetris). Doppler of the umbilical artery showed a resistance index (RI) of 0.49, pulsatility index (PI) 0.70 with a positive end diastolic flow (EDF). Doppler of the median cerebry media artery showed a resistance index (RI) of 0.72, pulsatility index (PI) 1.26. Amniotic Fluid Index 11, 43 cm, Placenta anterior. The pregnancy was controlled tightly in our clinic. During pregnancy in the first trimester, the patient got methylprednisolone 1 × 4 mg, Azathioprin 1 × 50 mg, folic acid 1 × 1 mg. Additional therapy at second trimester were calcium 1000 mg, multivitamin 1 x/day, methyldopa 3 × 250 mg and Acetylsalicylic acid 1 × 80 mg. She was scheduled for a cesarean section at 37 - 38 weeks of pregnancy. Results: According to the American College of Rheumatology (ACR) 1990, our patient fulfilled 4 of 6 TA criteria: age Conclusions: The management of TA in pregnancy is still a challenge. A multidisciplinary team involving obstetrician, cardiologist, rheumatologist and anaesthetist is absolutely needed to get an optimal development of the pregnancy. The risk of complication on the mother and fetus might occur any time. Adequately controlled blood pressure and fetal monitoring might result for better outcome. Birth delivery (pervaginam or perabdominal) is still a problem according to obstetrical condition, general condition of the mother and other additional factors. Careful assessment, treatment of TA complication, and regular antenatal follow-up improve maternal and fetal outcome.

Alobar Holoprosencephaly with Duodenal Atresia: A Case Report

Introduction: Holoprosencephaly (HPE) is the most frequent malformation of the prosencephalon. It represents the absence or incomplete division of the prosencephalon during the 4th and 8th week of gestation. Its incidence is estimated to be 1 in 16,000 live births and 1 in 250 spontaneous abortions. According to the degree of cerebral involvements, it is classified into 3 types: alobar, semilobar, and lobar. Alobar is the most severe type. Duodenal atresia occurs in approximately 1:10,000 live births. It can be fatal unless diagnosed and surgically treated promptly. It is a less common cause of polyhydramnion. The diagnosis of duodenal obstruction may be suspected prior to the child’s birth by prenatal ultrasonography. The outcome of congenital duodenal obstructions, in terms of mortality rate, has improved over the last decades, mainly attributable to the improvement in the quality of prenatal diagnosis and neonatal intensive care. Embryological basis for etiology of duodenal atresia is thought to be due to errors of recanalization. Duodenal atresia is associated with other congenital anomalies in spite with holoprosencephaly. This is the only case (HPE with duodenal atresia) which is reported in Hasan Sadikin Hospital in the last five years. Materials and Methods: A patient G2P1A0 who had vaginal birth 6 years ago, without complication on mother and fetus was referred to Hasan Sadikin Hospital with abdominal discomfort as main complaint on her 33 - 34 weeks of pregnancy. Prenatal care was during the first and second trimester by midwife and sonography in satellite hospital. She had polyhydramnion and head abnormality and referred to Hasan Sadikin Hospital. Sonography examination showed alobar holoprosencephaly, abnormal facial (cleft lip and palate, absent nasal bone, hypotelorism), duodenal atresia and polyhydramnios (Amniotic Fluid Index 50 cm);other examinations were normal. Amnioreduction and karyotyping examination were done. After 3 weeks the amniotic fluid increased (Amniotic Fluid Index 45 cm) and the patient complaint recurrent abdominal discomfort, so second amnioreduction was done. Estimated fetal weight was 2500 gram, using Doppler examination umbilical arteries were in normal limits (pulsatility index = 1.08). Cesarean section on 38 weeks of pregnancy was done considering the abnormal head circumference and diameter as requested by the mother and the family. Results: The patient had poor prenatal care, late diagnosed congenital anomalies i.e. holoprosencephaly, facial anomaly and duodenal atresia. Fetal malformation might be clearly visible earlier if sonography examination on the first and second trimester was done appropriately, and mother and family were informed to choose the right action. Postnatal examination (MRI and USG) were not done. BNO examination revealed double bubble on the upper abdomen up to the left and right part. The baby was referred to the pediatric surgery with duodenal atresia and duodenostomy was planned. Genetic testing using conventional karyotype analysis ruled out aneuploidy of chromosomes 13, 18, 21, X and Y. No other risk factors such as smoking, alcohol, diabetes, infectious disease and drugs were before and during pregnancy. But she had bad environmental condition near a river with colored and bad smelled water from factory waste. </