WBR0373

Author	[[PageAuthor::Rim Halaby, M.D. [1]]]
Exam Type	ExamType::USMLE Step 1
Main Category	MainCategory::Embryology
Sub Category	SubCategory::Renal
Prompt	[[Prompt::A 27 year old pregnant gravida 1 para 0 presents to the emergency department at 18 weeks gestation with rupture of membranes. Upon questioning, she did not receive any prenatal care and was not followed by an obstetrician. Following appropriate intervention, the patient’s fetus is evacuated but is not viable. Inspection of the fetus reveals clubbed feet and cranial anomalies. Which of the following additional findings is also expected to be most likely present on autopsy?]]
Answer A	AnswerA::Horseshoe kidney
Answer A Explanation	AnswerAExp::Horseshoe kidney is caused by the fusion of the inferior poles of both kidneys. It is not associated with Potter’s syndrome.
Answer B	AnswerB::Ventricular septal defect (VSD)
Answer B Explanation	AnswerBExp::VSD is a the most common congenital heart defect. However, it is not directly associated with Potter’s syndrome.
Answer C	AnswerC::Renal agenesis
Answer C Explanation	AnswerCExp::Oligohydramnios, commonly associated with bilateral renal agenesis, can give rise to Potter’s syndrome.
Answer D	AnswerD::Thymic aplasia
Answer D Explanation	AnswerDExp::Thymic aplasia is a characteristic finding in DiGeorge syndrome, a congenital disorder due to 22q11 deletion. DiGeorge syndrome is characterized by congenital heart disease, abnormal facies, thymic aplasia, cleft palate, and hypocalcemia.
Answer E	AnswerE::Mandibular hypoplasia
Answer E Explanation	AnswerEExp::Mandibular hypoplasia is a manifestation of Treacher-Collins syndrome. It is caused by failure of neural crest migration of the first branchial arch.
Right Answer	RightAnswer::C
Explanation	[[Explanation::Potter’s Syndrome is a triad of clubbed feet, pulmonary hypoplasia, and cranial anomalies. Oligohydramnios, commonly associated with bilateral renal agenesis, can give rise to Potter’s syndrome. Other causes of oligohydramnios include ureteral or urethral atresia, polycystic kidney disease, renal hypoplasia, and uteroplacental insufficiency. The incidence of Potter’s syndrome is related to the degree of oligohydramnios and to the gestational age at which rupture takes place. Approximately 50% of infants with rupture at 19 weeks’ gestation or earlier are affected with Potter’s syndrome. Educational Objective: Oligohydramnios, commonly caused by bilateral renal agenesis, can give rise to Potter’s syndrome. The latter is characterized by the triad of: clubbed feet, pulmonary hypoplasia, and facial anomalies. Reference: Medina TM, Hill DA. Preterm premature rupture of membranes: Diagnosis and management. Am Fam Phys. 2006; 73(4):659-664. Educational Objective: References: ]]
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