QUESTION OF THE WEEK - please email your answer by the next class.
(click here to read the answers to last week's questions)
At left is a strikingly precise self-portrait of a Dutch “Figurative Abstract” painter, who died at 38 from complications of his congenital condition. Which condition did he suffer from? Two of its features are prominently shown in this painting. (Click on the painting to enlarge it)
Fetal tracheal occlusion, which is now used to treat pulmonary hypoplasia, was initially described experimentally to demonstrate that fetal lungs actually produce fluid.Eighteen centuries earlier, Galen (Claudius Galenus, above right) used the same technique to prove the same phenomenon in which organ?
ANSWERS TO LAST WEEK'S QUESTIONS
1.What congenital anomaly is seen here – literally? The pediatrician who pioneered its diagnosis and treatment (and after whom, in part, the first palliative operation is named) later championed the awareness of another congenital condition – related to another feature of this cartoon character. Who was that pediatrician?
“Blue baby syndrome” most commonly refers to Tetralogy of Fallot, the most common cyanotic heart defect (cyanotic, because of the right-to-left shunt that allows deoxygenated systemic venous blood to bypass the lungs. Helen Taussig, a pediatric cardiologist and Pediatrician-in-Chief at the Johns Hopkins University School of Medicine, helped develop the Blalock-Taussig shunt – an anastomosis between the right subclavian artery and the right pulmonary artery to bypass the atresia of the main pulmonary artery, thereby directing blood to the lungs.
Later in life, Helen Taussig testified before Congress regarding the effects of thalidomide (which caused limb deformities, or dysmelia, in off-springs of mothers who took it in early pregnancy), and was instrumental in taking the drug off the market. There are many types of dysmelias – including phocomelia, most often seen with thalidomide, and oligodactyly (too few fingers) – a feature of this, and most other cartoon characters.
2. After the first trimester, amniotic fluid is mostly fetal urine. Oligohydramnios (too little amniotic fluid) can be dangerous to the fetus.
In which of these scenarios is it justifiable to create a direct connection between the fetal bladder and the amniotic cavity (vesico-amniotic, or V-A shunt), thereby bypassing the urethra?
- 33-week-gestation fetus with a 1-week history of ruptured membranes and oligohydramnios from amniotic fluid leak
- An 18-week-gestation fetus with absent kidneys and oligohydramnios
- A 22-week-gestation fetus with a distended bladder, distended ureters, normal amniotic fluid volume and adequate renal function, based on a needle aspiration of the fetal urine
- A 16-week-gestation fetus with severe oligohydramnios, a distended bladder and bright echogenic kidneys containing multiple cysts
- A 23-week-gestation fetus with absent amniotic fluid, a distended bladder and adequate renal function, based on a needle aspiration of the fetal urine
Prenatal shunting of the bladder should only be considered if
- there is oligohydramnios (which rules out # 3), AND
- kidney function is adequate, so that fetal urine can in fact me made and excreted to replenish amniotic fluid (rules out #2 and #4: bright, echogenic and cystic kidneys at 16 weeks suggests early and severe kidney damage), AND
- there is no equivalent or better postnatal treatment (#1 is out: at 33 weeks, this baby is better off being delivered)
The only scenario that might be considered is #5