Case+6+Discussion

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Ultrasound Findings

Hepatorenal view: Shrunken liver with irregular margins surrounded by free fluid subdiaphragmatic and in morrisons pouch. Right kidney with no hydronephrosis normal in size.

Spleno-renal view: Splenomegaly with homogenous appearance, no masses, or cysts.. No hydronephrosis of left kidney, with subdiaphragmatic free fluid observed.

Transverse pelvic view: Intrauterine pregnancy with posterior placenta, dating (in other windows) to 18 weeks. Large amount of free fluid seen in abdomen. Fluid appearing clear with no layering (layering being suggestive of blood)

Case 6 Discussion:

Putting all of our obtained information together we have acute mental status changes in an 18 week pregnant 35 y/o female with free fluid in the abdomen, shrunken fibrotic liver, splenomegaly, and low platelets with normal vitals in the absence of fever or poteinuria suggestive of a cirrhotic-like clinical picture in pregnancy. Other etiology such as pre-eclampsia (or HELLP), meningitis, cerebral malaria are essentially ruled out with limited wrk-up and exam. Why would a young pregnant woman present with signs and symptoms of cirrhosis in the developing world?

This young lady’s heartbreaking case is Hep B surface antigen positive. And though hepatitis flares are common in the immunodeficient state of pregnancy, our findings suggest much more: look closely at that liver window and a shrunken nodular edged liver is seen, with a corresponding massive spleen and ascites suggestive of cirrhosis. The limited labwork available with isolated thrombocytopenia and later an elevated INR (2.0) agree with the above picture. Finally, this cirrhosis diagnosis in the context of asterixis suggests the etiology of our mental status changes: hepatic encephalopathy.

And for the record, yet again, ultrasound rapidly enabled a diagnosis in this resource poor setting allowing quick movement on a clinical course.

Hepatitis B vaccine is available in most sub-Saharan countries especially within the last decade for children. However vaccine campaigns continue to have poor reach in especially rural areas, as well as adults who missed the series as children.. Our patient was unfortunately unvaccinated and at the young age of 35 already developed cirrhotic changes.

Though we don’t know for sure, it is likely that a flare during this pregnancy may have contributed to the acute first time development of encephalopathy and ascites (patient denies any previous episodes of confusion, swelling, or distention). Cirrhosis in pregnancy is certainly ‘high risk’ for multiple reasons contributed to organ failure, however limited case reports and expert consensus suggest that specifically the highest risk of cirrhosis in pregnancy pertains to variceal bleeding which increases with gestational age (starting 2nd tri), culminating in highest risk with valsalva maneuvers at the time of delivery.

Management of this clinical situation includes a variety of layers from pregnancy counseling at an early stage to antepartum management,to intrapartum management. As previously stated given the nature of risk involved with pregnancy in cirrhosis, care should be taken to offer all menstruating women with cirrhosis birth control counseling/offering. And in the event of a rare pregnancy (cirrhosis itself is associated with low risk of pregnancy) an honest discussion with the mother regarding the high risk of the pregnancy and the elective nature of continuation should ensue. This is options counseling in the context of significant maternal morbidity/mortality and in countries where elective abortion is illegal, should qualify as a medically necessary termination of the pregnancy. Our patient was counseled in her native language and chose to continue the pregnancy.

In addition to routine prenatal care, management of cirrhosis in pregnancy involves the routine measures of diuresis (excepting usage of spironolactone – class D), low salt intake, leg elevation, etc… In cases of HE lactulose is useful and rifaximin (though not available in sub-Saharan Africa) in refractory cases. Experts recommend upper endoscopy for variceal identification and banding in optimally the second trimester or later. Given no option for scoping in our context, it is reasonable to start the patient on a low dose of beta blocker for prophylaxis given the high mortality associated with variceal rupture in this decompensated cirrhotic patient.

There are no RCTs (nor will there ever be) regarding delivery methods in this clinical context. Initially it may seem desirable to avoid valsalva maneuvers and deliver by C-section, however with an INR of 1.9 this is not advisable. One could surmise in a milder case (with less severe organ dysfunction and preserved INR) this may be an option. However, based on case report reviews and expert opinion the likely best method of delivery for this patient will be epidural placement at time of natural labor, passive decent, with instrument delivery sparing maternal valsalva maneuvers.

Half of all worldwide chronic Hep B cases are secondary to vertical (and preventable) transmission. So managing the specific Hepatitis B component of this situation – which we presume to be chronic Hep B in the context of the significant liver dysfunction – is absolutely paramount both from maternal and child perspectives. From the maternal perspective the rationale to treat patients with hep B is tor educe the risk of progression of liver disease as well as transmission to the child. Naturally in the setting of decompensated cirrhosis the indication is clear to prevent an further damage therefore the mother was started on lamivudine (which is readily available and class C however with long anecdotal experience of safety in pregnancy in dense HIV+ regions). Transmission rates have been reported as high as 9% in maternal HepB patients to child, however the high protective efficacy (95%) of neonatal vaccinations suggest that most infections occur during birth. Prophylactic administration of HBIG would be optimal but not available, however the vaccination is freely available in the routine three-series for first 6 months of life. It appears the highest risk factor for transmission involves a high maternal HBV viral load which behooves the use of antivirals during the pregnancy.

Our patient lives about a half day’s walk from the health center, a usual barrier to care in obstetrical care. Many women not only often don’t deliver in health facilities due to this issue, but even receive no prenatal care in this context. One interesting project in global health (now currently in construction here) is the building of maternal waiting homes which allow uncomplicated pregnant women to board (full board including food) starting at 38 weeks, and complicated pregnancies earlier as needed. This is one further barrier reduction to high quality obstetrical care in remote and poor regions. In addition, programs linking poor patients to transportation money and food allotments has been shown to improve outcomes in this complicated medical situation specifically improving compliance to visit attendance.