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1
Question:

A 15-year-old girl is brought to the emergency department due to a severe headache, abdominal pain, nausea, and vomiting.  The patient reports an occipital headache that began 4 days ago and has increased in severity despite treatment with acetaminophen.  The nausea and vomiting began a day ago, and now the patient is unable to tolerate liquids.  The abdominal pain is constant but worsens with vomiting.  She has no chills, photophobia, changes in vision, or dizziness.  The patient is sexually active.  Her last menstrual period was 4 months ago.  She does not use tobacco, alcohol, or illicit drugs.  Temperature is 37.2 C (99 F), blood pressure is 150/90 mm Hg, and pulse is 90/min.  Cardiopulmonary examination is normal.  Abdominal examination shows right upper quadrant tenderness without rebound or rigidity.  There is a nontender, palpable mass that extends from the suprapubic bone up to the umbilicus.  Neurologic examination shows intact cranial nerves, no nystagmus, and no pain with neck flexion.  Bilateral lower extremities have 3+ deep tendon reflexes and sustained ankle clonus.  Serum β-hCG is elevated.  Which of the following is the most likely diagnosis in this patient?

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Hydatidiform mole

Clinical presentation

  • Abnormal vaginal bleeding ± hydropic tissue
  • Uterine enlargement > gestational age
  • Abnormally elevated β-hCG levels
  • Theca lutein ovarian cysts
  • Hyperemesis gravidarum
  • Preeclampsia with severe features  
  • Hyperthyroidism

Risk factors

  • Extremes of maternal age
  • History of hydatidiform mole

Diagnosis

  • "Snowstorm" appearance on ultrasound
  • Quantitative serum β-hCG
  • Histologic evaluation of uterine contents

Management

  • Dilation & suction curettage
  • Serial serum β-hCG post evacuation
  • Contraception for 6 months

This patient's hypertension, hyperreflexia (eg, clonus), and positive pregnancy test are concerning for preeclampsia with severe features.  In addition to new-onset hypertension, pre-eclamptic patients often have signs of end-organ damage such as right upper quadrant pain (due to hepatic swelling and stretching of the Glisson capsule) and headache.  Although preeclampsia typically presents in the late third trimester, preeclampsia at <20 weeks gestation can be a complication of hydatidiform mole (HM).

HM, a type of gestational trophoblastic disease, is caused by an abnormal fertilization that results in a nonviable gestation composed of abnormal placental tissue (eg, hyperplastic trophoblasts, hydropic trophoblastic villi).  The abnormal trophoblastic tissue proliferation can result in preeclampsia and leads to an enlarged uterus (eg, pelvic mass) that is greater than expected for gestational age and is filled with a heterogeneous mass of anechoic, cystic spaces (eg, "snowstorm" appearance on ultrasound).  Preeclampsia is likely due to abnormal placental spiral artery development, which causes placental hypoperfusion, placental ischemia, and maternal hypertension.  Treatment of HM is uterine evacuation (eg, suction curettage) to remove the trophoblastic tissue, after which the preeclampsia resolves.

(Choice A)  Cholecystitis can present with right upper quadrant pain, nausea, and vomiting.  However, cholecystitis is uncommon in adolescents and is not associated with hypertension, neurologic findings, or a pelvic mass.

(Choice B)  Bacterial meningitis can present with headache, nausea, and vomiting.  However, this patient's lack of fever and nuchal rigidity makes bacterial meningitis an unlikely diagnosis.

(Choice C)  Embryonal carcinoma, an aggressive ovarian cancer typically diagnosed in adolescents, can present with an abdominal mass and elevated β-hCG.  Patients typically have ascites rather than hypertension or neurologic symptoms.

(Choice D)  Fitz-Hugh Curtis syndrome (eg, perihepatitis) is a sequela of pelvic inflammatory disease that typically presents with pleuritic right upper quadrant pain.  However, patients typically have associated fever and hypotension (eg, sepsis); not neurologic symptoms.

(Choice F)  Viral encephalitis can present with headache and hyperreflexia.  This patient's lack of fever, photophobia, and cranial nerve deficits makes this diagnosis unlikely.

Educational objective:
Hydatidiform mole can present with preeclampsia with severe features at <20 weeks gestation.  The preeclampsia resolves after treatment of the hydatidiform mole.