Missing or making the timely diagnosis of acute fatty liver of pregnancy (AFLP): lessons learned.

OBJECTIVE: To review the presenting signs, symptoms, laboratory findings, clinical courses, complications, and maternal outcomes in women determined on medicolegal review to have had acute fatty liver of pregnancy (AFLP). METHODS: We retrospectively studied case files from women with medical and/or hypertensive complications of pregnancy which had been reviewed by the first author, deidentified and divested of legal documents so that only medical-related information was retained for later research and teaching purposes. Main outcomes are 12 lessons learned from this review to be offered for the future benefit of clinicians encountering patients with AFLP. RESULTS: Eleven women with AFLP were identified within the 161 file database. Signs and symptoms present in 91-100% of women with AFLP were altered sensorium, a fast resting maternal pulse >100/min, gastrointestinal complaints, muscle pain and evidence of fetal compromise/stillbirth. Laboratory findings in 91-100% revealed abnormal liver, renal and coagulation tests, hyperuricemia, and very low serum glucose and albumin. A correct initial diagnosis of AFLP was made for only three women; the most common incorrect presumptive diagnosis was HELLP syndrome. Four women survived. Twelve major findings are discussed. CONCLUSION: Rapid, timely and thorough evaluation of third trimester women presenting with A (Altered mental status changes/extreme sleepiness), F (Fast resting pulse >100 in the absence of fever or heart causation), L (Liver-related gastrointestinal complaints) and P (Pain as myalgias and body aches), often in association with evidence of fetal compromise or stillbirth, should initiate efforts to investigate a possible diagnosis of AFLP. Adoption of the concepts summarized including core initial laboratory testing, immediate patient transfer to tertiary care, and aggressive correction of consumptive coagulopathy could form the basis of a patient safety bundle to guide future management for patients with AFLP.

Maternal morbidity and mortality in pregnant/postpartum women with suspected HELLP syndrome identifiable as probable thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome by high LDH to AST ratio.

OBJECTIVE: To describe findings in 8 women initially diagnosed as presumptive HELLP Syndrome, eventually confirmed as TTP/aHUS as distinguished by elevated calculated LDH:AST ratio > 22:1. METHODS: All medicolegal files of patients evaluated between 1986 and 2015 with presumptive HELLP syndrome but later determined to have TTP/aHUS had LDH:AST ratios evaluated throughout care. RESULTS: Fifty-eight pregnant/postpartum women presented with a diagnosis of presumptive HELLP syndrome. In the final analysis, 8 women had TTP/aHUS characterized by severe thrombocytopenia (<20 000/µl) at admission, rare epigastric pain, and the consistent demonstration of a very high calculated total LDH to AST ratio. This calculation greatly exceeded 22:1 with TTP/aHUS (mean = 32:1) versus 2:1 with HELLP and could be consistently demonstrated throughout care. Six of 8 women with TTP/aHUS died. CONCLUSION: Correctly distinguishing between HELLP syndrome versus an imitator disorder continues to challenge obstetric specialists. This medicolegal data supplements prior findings supporting the concept of the LDH:AST ratio as a useful screening tool for clinicians to differentiate TTP/aHUS apart from HELLP syndrome in order to facilitate earlier hematology consultation, patient referral to tertiary care and emergent hemotherapy for these mothers.

Second trimester presentation of preeclampsia and choriocarcinoma in a primigravida with live birth.

Choriocarcinoma in the second trimester with a normal appearing live fetus is rare. A primigravida presented at 24 weeks' gestation with 5 days of worsening dyspnea and multiple widespread small lung nodules. Pelvic ultrasound revealed a normal intrauterine live singleton fetus with an extrauterine mass. Gestational hypertension progressed to preeclampsia with severe features and onset of vaginal bleeding. Cesarean delivery was undertaken with liveborn delivery and removal of an intrauterine mass confirmed to be choriocarcinoma. Postpartum treatment with multi-agent chemotherapy was initiated. The newborn thrived; the mother has no evidence of residual disease.

Milestones in the quest for best management of patients with HELLP syndrome (microangiopathic hemolytic anemia, hepatic dysfunction, thrombocytopenia).

In the 3 decades since the original description and naming of the dangerous pregnancy complication called HELLP syndrome, many milestones have been passed in pursuit of a better understanding of the disorder and its best management. It is worthwhile to review the milestones through which we have passed internationally in obstetrics as the peril that this disorder poses to pregnancy has been exposed. Factors of importance for emphasis will be definitions, reasons why the triple classification system impacts practice, the important role glucocorticoids have in reversing the pathogenesis of the disorder if used early, the excellent results reported for mothers when all components of the Mississippi Protocol are utilized for patient care, and the place of plasma exchange in special situations. Our challenge for the future is to delay or prevent HELLP's development prior to potential viability so that stillbirths and very preterm losses can be reduced or eliminated.

Approaches to reduce urinary tract injury during management of placenta accreta, increta, and percreta: a systematic review.

OBJECTIVE: A systematic review of the literature was conducted to answer the following question: are there enhancements to standard peripartum hysterectomy technique that minimize unintentional urinary tract (UT) injury in pregnancies complicated by invasive placental attachment (INPLAT)? METHODS: A PubMed search of English language articles on INPLAT published by June 2010 was conducted. Data regarding the following parameters was required for inclusion in the quantitative analysis of the review's objective: (1) type of INPLAT, (2) details pertaining to medical and surgical management of INPLAT, and (3) complications, if any, associated with management. An attempt was made to identify approaches that may lower the risk of unintentional UT injury. RESULTS: Most cases (285 of 292) were managed by hysterectomy. There were 83 (29%) cases of unintentional UT injury. Antenatal diagnosis of INPLAT lowered the rate of UT injury (39% vs. 63%; P = 0.04). Information regarding surgical technique or medical management was available for 90 cases; 14 of these underwent a standard hysterectomy technique. Methotrexate treatment and 11 modifications of the surgical technique were associated with 16% unintentional UT injury rate as opposed to 57% for standard hysterectomy (P = 0.002). The use of ureteral stents reduced risk of urologic injury (P = 0.01). Multiple logistic regression analysis identified antenatal diagnosis as the significant predictor of an intact UT. CONCLUSIONS: Antenatal diagnosis of INPLAT is paramount to minimize UT injury. Utilization of management modifications identified in this review may reduce urologic injury due to INPLAT.