Case presentation
A 27-year-old woman (G3 P2+0), with three confirmed pregnancies and two
previous deliveries, presented at 26 weeks of gestation in a tertiary
care hospital with a history of fever, low platelets, and deranged liver
function tests (LFTs) for two days. Her pregnancy was un-booked, and the
above-mentioned findings were detected at a local clinic. Before this
presentation, the patient was not on any medications.
On presentation to the hospital, physical examination revealed a
well-nourished and conscious woman who was oriented to time, place, and
person. Her pulse rate was 82 beat per minute, blood pressure 120/80
mmHg, and oxygen saturation of 95% at room air. Abdominal examination
revealed a height of the fundus corresponding to 26cm. Neurological
examination was non-significant and the Glasgow Coma Scale (GCS) was
15/15. The rest of the physical examination was unremarkable.
She was started on empiric antibiotics and further investigations were
done including Dengue NS1 antigen, LFTs, and Urine DR, to rule out other
causes of her symptoms. A few hours into admission, the patient
developed hypoglycemia for which she was intravenously given 25%
dextrose.
A chest x-ray was performed showing right pleural effusion and bilateral
atelectasis. Ultrasound of the abdomen confirmed fatty changes with a
normal-sized hypoechoic liver sparing periportal zones ruling out HELLP
(Figure 1). A provisional diagnosis of AFLP (and viral hepatitis) was
made.
A normal sonogram of the gall bladder, pancreas, spleen, kidney, and
urinary bladder was reported. The surface Antigen test showed no
reaction to HbsAg and HCV antigen, so ruling out viral hepatitis. A
confirmed diagnosis of AFLP was made.
12 hours into admission, the patient complained of abdominal pain,
vomiting, constipation, and headache, for which she was promptly
transferred to the intensive care unit (ICU). The patient developed
significant dyspnea with a total leukocyte count of 24.26 x 109/L,
severe anemia, and a left-sided consolidation on CXR.
She developed tachycardia with a pulse of 118/bpm. Fetal movements were
positive. Her hemoglobin level dropped from 11.3g/dL to 8.6g/dL, with a
prolonged APTT and INR, and her serum SGPT had risen to 3339 IU/L
recorded in Table 1. The patient became markedly drowsy, irritable, and
restless, and a diagnosis of Grade 1 (hepatic) encephalopathy was made,
for which she was given lactulose. Later patient progressed to Grade II
hepatic encephalopathy, with a GCS of 13/15.
Vaginal delivery was chosen over a cesarean to prevent further
deterioration of her condition. An alive male fetus was delivered, with
a poor APGAR score and was handed for resuscitation. Around 600-700ml of
clots were removed from the uterus.
An hour later, fresh bleeding was observed per vaginal examination. With
a contracted uterus, her perineum was inspected for tears, balloon
tamponade was performed. However, the patient continued to bleed and was
shifted to the operation theatre for uterine artery embolization. Her BP
was 120/ 83 mmHg, pulse 120 bpm, and O2 saturation of 93% at room air.
She was on continuous positive airway pressure (CPAP), and mannitol was
administered to protect renal function after the vascular procedure.
The procedure successfully stopped her bleeding. The patient was drowsy,
irritable, and not following commands, consistent with a progression to
grade III hepatic encephalopathy, with a GCS of 11/15. Antibiotic
treatment was continued.
Over the course of the next two days, the patient’s condition improved
steadily. CXR showed improvement and CT brain showed no cerebral edema
(Figure 2).
Approximately two months later, the patient returned to our facility
with a complaint of lower abdominal pain. On examination, her uterus was
bulky, with a thickened endometrium and necrotic debris sloughing off
through the vagina. She had developed uterine necrosis; a late and rare
complication of the uterine artery embolization that had been performed
for her postpartum hemorrhage. As a result, a total abdominal
hysterectomy was performed, after which she was discharged from the
hospital.
Discussion
Jaundice during pregnancy has many causes including but not limited to
intrahepatic cholestasis, cholelithiasis, viral hepatitis, pre-eclampsia
with or without HELLP syndrome, and AFLP. Intrahepatic cholestasis of
pregnancy (ICP) may present during the third trimester but it is mainly
characterized by pruritis in the absence of a skin rash with abnormal
LFTs (elevated transaminase and bile acids) and usually resolves after
birth, however, serum bilirubin concentration is rarely higher than
6mg/dl. Cholelithiasis may occur at any time during pregnancy and is
accompanied by pain in the right upper quadrant, and fever, and
ultrasonography (USG) is usually diagnostic.8 Acute
viral hepatitis in pregnancy presents as a systemic illness with fever,
nausea, vomiting, fatigue, jaundice, dark urine and pale stools , and
markedly elevated aminotransferase concentrations
(>500U/liter).
The incidence of HELLP syndrome is three times as much as that of
AFLP.9 The symptoms of our patient were initially
indicative of both HELLP and AFLP; nausea, vomiting, dyspnea, epigastric
abdominal pain, anorexia , jaundice, and hence supportive treatment for
AFLP and HELLP Syndrome was started. Further laboratory investigations
indicated severe coagulopathy, raised APTT, elevated serum transaminase
and bilirubin levels, hypoglycemia, an elevated ammonia value, and a low
albumin level, favoring the diagnosis of AFLP over HELLP syndrome.
In AFLP there is an inherited enzyme deficiency in beta-oxidation, due
to a defect in the enzyme LCHAD (long-chain hydroxy acyl-coenzyme A
dehydrogenase) predisposing them to AFLP resulting in progressive lipid
accumulation within the hepatocytes. However, other risk factors can
also be noted e.g., primigravida (first pregnancy), pre-eclampsia, male
fetus, and multiple gestation.10
Lastly, extrahepatic complications delayed maternal recovery for up to
four weeks after delivery. These trends in earlier studies make this
case report noteworthy as our patient not only presented late to us
after the development of hepatic encephalopathy, acute renal failure,
and DIC but also improved a week before delivery.
Conclusion
Early diagnosis and prompt management are essential to prevent maternal
and perinatal morbidity and mortality. AFLP can be managed with early
and prompt diagnosis through laboratory tests to reduce complications.
Further studies can be conducted to devise a relationship between
factors that can lead to termination of the pregnancy in patients with
hepatic encephalopathy and DIC so neonatal deaths can be reduced and a
solution can be stamped with regards to AFLP.
Competing Interests
None declared.
Funding
This study received no external funding.
Acknowledgment
None.
Consent for publication
An institutional approval letter was granted by Ziauddin Medical
University, Karachi, Pakistan for this case report.
Consent statement:
Written informed consent was obtained from the patient to publish this
report in accordance with the journal’s patient consent policy.
DATA AVAILABILITY STATEMENT
The data that support the findings of this study are available with the
corresponding author upon reasonable request.
Authors’ contributions
Conceptualization: Saba Saleem Qazi, Hania Khwaja,
Data Curation: Anusha Akhai, Shiza Danish
Formal Analysis: Hania Khwaja
Investigation: Shiza Danish
Methodology: Sabeeh Khawar Farooqui, Hania Khwaja
Writing (main draft): Sabeeh Khawar Farooqui, Saba Saleem Qazi, Anusha
Akhai, Muhammad Sohaib Asghar, Muhammad Junaid Tahir
Writing (review and editing): Muhammad Sohaib Asghar, Muhammad Junaid
Tahir