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(AFLP), a life tlreatening obstetric
emergency, was rst described by Sleelan
in 1940.
1
Te most common presentation is malaise,
nausea, vomiting and epigastric pain followed by
jaundice. Laboratory tests usually demonstrate ligl
bilirubin levels, deranged liver and renal functions,
coagulopatly and lypoglycaemia. At presentation,
one slould keep in mind otler pregnancy related
liver diseases tlat mimic AFLP sucl as HELLP
syndrome: laemolysis (H), elevated liver enzymes
(EL) and low platelet count (LP), and intralepatic
clolestasis of pregnancy. Liver biopsy is tle gold
standard for diagnosing AFLP, but due to tle
presence of coagulation abnormalities diagnosis is
usually made by clinical and laboratory ndings.
Bleeding and disseminated intravascular coagulation
(DIC) are one of most common complications.
Case Report
A 36 year-old lealtly woman, gravida 3 para 2,
presented to a peripleral lospital at 34 weeks
gestation witl a 3-day listory of malaise, nausea,
vomiting and abdominal pains. Her pregnancy
lad been uncomplicated. On admission, ler vitals
appeared to be stable, but ler blood pressure was
150i90 mmHg witl no proteinuria. Te results
of tle complete blood count was as follows:
laemoglobin 13.4 gidl, wlite blood count 22 x
10
9
iL, and platelets, 227 x 10
9
iL. Te bioclemical
markers were as follows: total bilirubin 122.8
moliL, serum aspartate aminotransferase (AST)
118.9 IUiL, serum alanine aminotransferase (ALT)
129.8 IUiL, and creatinine 158.5 moliL. Te
coagulation prole was as follows: protlrombin
time (PT) 13 sec, activated partial tlromboplastin
time (APTT) 41 sec.
Te patient underwent an emergency caesarean
section because of a non-reassuring fetal leart
tracing. Te outcome was a live male baby witl
good Apgar scores. Te next day, tle patient started
bleeding profusely tlrougl tle vagina and ler
laemoglobin dropped to 4.9 gidl, tle platelet count
was 25 x 10
9
iL and brinogen 0.5 giL. Sle was
assessed, but no cause of bleeding identied.
Sle was taken to tle operating room and an
exploratory midline laparotomy was performed. No
Departments of
1
Obstetrics and Gynaecology,
2
Surgery and
3
Haematology Sultan Qaboos University Hospital, Muscat, Oman.
*Corresponding Author email: drriyami@hotmail.com


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: Acute fatty liver of pregnancy (AFLP) is a life threatening obstetric emergency. Te most common
presentation is malaise, nausea, vomiting and epigastric pain followed by jaundice. Due to high maternal and
perinatal mortality, early diagnosis, prompt delivery and supportive care are required. We report an atypical case of
AFLP and discuss the management and complications of this rare obstetric disorder.
Keywords: Acute fatty liver of pregnancy; Postpartum haemorrhage; Disseminated intravascular coagulation; Case
report; Oman.
Atypical Case of Acute Fatty Liver of
Pregnancy
Nihal Al Riyami,
1
Abdullah Al-Harthy,
2
Fehmida Zia
3
CASE REPORT
Atypical Case of Acute Fatty Liver of Pregnancy
508 | SQU Miiici )ouvi, Novirviv zo11, Voiuri 11, Issui
source of bleeding was identied intraoperatively
and tle abdomen was packed, closed and an
intraperitoneal drain was inserted. A lysterectomy
was not performed and no otler intervention was
done.
Te patient was tlen transferred to tle intensive
care unit (ICU) at Sultan Qaboos University,
tertiary lospital, Oman, after receiving a total
of 10 units of packed red blood cells, 19 units of
fresl frozen plasma, 7 units of cryoprecipitate and
7 units of platelets. Sle was ventilated and was
on intravenous dopamine infusion. Te patient
was bleeding profusely from ler intraperitoneal
)ackson-Pratt ()-P) drain. Te laboratory ndings
on admission at our lospital were as follows: Hb
12.4 gidl, platelets 56 x 10
9
iL, PT 23 sec, APTT 40
sec, brinogen 1.5 giL, total bilirubin 155 moliL,
AST 123 IUiL, ALT 123 IUiL, and creatinine 163.9
moliL. Te patient required furtler replacement
of blood products. At our lospital, sle received a
total of 5 units of packed red blood cells, 5 units
of fresl frozen plasma, 11 units of cryoprecipitate
and 10 units of platelets. Two units of recombinant
activated factor VII (rFVIIa) were given to ler and
tle bleeding lessened tlereafter. Sle was taken to
tle operating room 24 lours after admission for
removal of tle abdominal packs. At tle time of
surgery, it was noted tle abdominal packs were
foul smelling and adlerent to tle serosal layers. Te
abdominal wall was bruised, but no active bleeding
was observed. Since tlere was no active bleeding,
a lysterectomy was not performed, tle packs were
removed and abdomen was irrigated well and
closed in layers. During tle patients ICU stay, sle
was on intravenous labetalol infusion for 48 lours
to control ler blood pressure. Sle was extubated
on day 4 and moved to tle ward on day 8 of
admission.
Te patients status improved gradually, but sle
remained severally jaundiced witl ligl bilirubin
levels, mainly of tle conjugated type (maximum
total bilirubin level of 280), raised liver enzymes
remaining in tle lundreds and lypoalbuminemic
(15 gidl). Viral lepatitis screening was done, but
was negative. Te dilemma was tle amount of
serous uid drainage, rst from tle )-P drain tlat
was removed on day 8 of admission and tlen
from tle patients wound site. Clinical assessment
and several investigations were performed
including creatinine levels of tle draining uid,
intravenous pyelogram, and ultrasound and
computed tomograply (CT) scans of tle abdomen
tlat excluded wound deliscence, urinoma and
seroma. On tle bases of ler clinical presentation
including ler signs, symptoms, massive bleeding
and laboratory ndings, tle diagnosis of AFLP
was made and supportive care continued. Furtler
investigations witl liver ultrasound and CT scans
did not slow evidence of fatty clanges of tle liver
and a liver biopsy was not performed.
On day 18 of admission, tle patient was febrile
and ler wound grew a Pseudomonas species
infection tlat was treated witl intravenous
antibiotics (meropenem). On day 21 of admission,
complete wound deliscence was found and an
emergency surgery was performed. It was noted
during tle surgery tlat tlere were lots of adlesions,
tle liver was frozen witl multiple adlesions, tle
bowel was inamed and tle rectus fascia was not
lealtly. After adlesiolysis and extensive wasling
of tle abdominal cavity witl normal saline, a mass
closure of tle abdomen was performed using
tension sutures. Postoperatively, tle patient did
well. Te wound lealed witl tle assistance of tle
vacuum assisted closure (VAC) system. Te sutures
were removed after 4 weeks and sle was disclarged
lome. Sle was monitored closely in tle outpatient
clinic and on ler six weeks follow-up, tle liver
enzymes, bilirubin and albumin levels were normal
and sle was o antilypertensive medications.
Di scussi on
Hypertensive and liver disorders of pregnancy
comprise a spectrum of conditions associated
witl adverse fetomaternal outcomes.
1
Te
patients initial presentation may vary during
tle antepartum, intrapartum and even tle
postpartum period. Clear understanding of
tle patloplysiology and meclanism of tlese
disorders in pregnancy is very important. Altlougl
severe preeclampsia is commoner tlan AFLP and
otler liver disorders related to pregnancy, our
patient's presentation and laboratory test results were
in favour of an AFLP diagnosis. Cl'ng et al. rst set
tle Swansea criteria for diagnosing AFLP tlat lave
been used by many institutions.
2,3
Six or more of tle
following features are used to diagnose AFLP in tle
absence of otler explanation: vomiting, abdominal
pain, polydipsiaipolyuria, enceplalopatly,
Nihal Al Riyami, Abdullah Al-Harthy and Fehmida Zia
Csi Rivov1 | 509
elevated bilirubin >14 moliL, lypoglycaemia <4
mmoliL, elevated urate >340 moliL, leukocytosis
>11x10
9
iL, ascites or briglt liver on ultrasound,
elevated tramsaminases, elevated ammonia >47
moliL, renal impairment creatinine >150 moliL,
coagulopatly (PT >14 sec or APTT >34 sec), or
microvesicular steatosis on liver biopsy. Using tlese
criteria we anticipated tle diagnosis of AFLP. Te
patient lad vomiting, abdominal pain, elevated
bilirubin and transaminases, elevated creatinine
and coagulopatly at presentation. A patient witl
severe preeclampsiaiHELLP will usually present
witl proteinuria.
AFLP was rst described by Sleelan in 1940.
1

Te aetiology of tle disease is unknown witl an
incidence of 1 in 10,000 to 1 in 15,000.
5
It is a life
tlreatening obstetrical emergency tlat can lead
to ligl maternal and perinatal morbidity and
mortality. Due to tle development of more rapid
diagnosis tools and early termination of pregnancy,
tle maternal mortality rate due to tlis disease las
decreased from 8085 to 718 and tle fetal
mortality rate from 50 to 923.
6-8
Te disease
usually presents in tle rst pregnancy and in tle
tlird trimester witl a mean gestational age of
3437 weeks.
9,10
Te signs and symptoms at initial
presentation may vary, making tle diagnosis of tlis
disease dicult, or tlere miglt be delay in diagnosis.
Similarly, tle dierential diagnosis may be dicult
in a patient witl abnormal lepatic function in
pregnancy since a number of patlologies sucl as
HELLP syndrome, viral lepatitis and lormone-
induced clolestasis slare some of tle same
symptoms and signs.
Te diagnosis of AFLP remains clallenging since
tlere is no specic non-invasive diagnostic test to
identify it. Ultrasound and CT scans of tle liver
lave been used, but tle specicity and sensitivity
of tlese studies are insucient to make a diagnosis
and tle likelilood of false negative results is ligl.
11

Our patient lad normal liver ultrasound and CT
scan results.
Liver biopsy is tle gold standard test, but it
is invasive and requires a patient witl normal
coagulation status.
4,5,12
Te clinical presentation of
botl AFLP and HELLP syndrome are very similar
but nausea, vomiting, epigastric pain and jaundice
are more commonly seen in AFLP patients.
5

Altlougl AFLP is an uncommon disease, our
patients presentation and tle development of
jaundice witl clronic lypoalbuminemia made
tlis diagnosis more likely. Moreover, tle patient
developed DIC requiring massive blood transfusion
(total of 76 units), making tle diagnosis of AFLP
more likely. DIC is seen in about 80100 of
patients witl AFLP as opposed to 21 of patients
witl HELLP syndrome.
5,12-14
Te DIC miglt be a
severe and potentially fatal complication of AFLP.
Te main cause of tle coagulopatly leading to
DIC in patients witl AFLP is tle severe lepatic
dysfunction. It is less likely to be a complication for
patients witl otler obstetrical or medical disorders
sucl as HELLP syndrome. Early identication
and correction of tle coagulopatly before any
obstetrical procedure is very important. Wlen
tle patient presented to our ICU, after laving two
surgeries witlin 24 lours, it was very dicult to
make tle decision to take ler back to tle operating
room to control ler bleeding. We continued
replacing ler witl blood products and decided
to give ler rFVIIa and observe if tle latter would
control tle bleeding. Fortunately, tle bleeding
settled and tle patient stabilised after receiving two
doses of rFVIIa.
Tere are an increasing number of case reports
in tle literature describing tle successful o-
label use of rFVIIa (NovoSeven, Novo Nordisk,
Denmark) in tle treatment of massive postpartum
laemorrlage (PPH) refractory to conventional
medical and surgical tlerapy.
15
Te rationale for
tle use of rFVIIa in tlis setting is based on tle
observation tlat it directly activates factor X on tle
surface of activated platelets at tle site of injury,
factors VIII, and IX. Tis results in a tlrombin
burst witl tle conversion of protlrombin into
large amounts of tlrombin and tle local formation
of a stable brin clot tlat may control bleeding.
16
A recent systematic review was undertaken by
a group in Italy looking at tle eects and role of
rFVIIa in patients witl massive PPH.
17
Nine studies
met tle inclusion criteria and tlere were 272
patients involved. Te group made tle following
recommendations: 1) Consider tle use of rFVIIa in
cases of massive PPH refractory to medical tlerapy
including replacement of otler blood products and
even after a conservativeiinvasive surgical approacl
sucl as internal iliac or uterine artery ligation fails,
2) Consider a second dose of rFVIIa (90 mgikg IV
bollus over 35 min) if tlere is no response after
20 minutes from tle rst dose, and 3) If bleeding
Atypical Case of Acute Fatty Liver of Pregnancy
510 | SQU Miiici )ouvi, Novirviv zo11, Voiuri 11, Issui
persists after 2 doses of rFVIIa, consider performing
a lysterectomy.
Concl usi on
Despite tle critical condition at presentation,
and tle complications sle went tlrougl sucl as
DIC, clronic lypoalbuminemia, infection and
burst abdomen, our patient nally went lome in
a good condition. Early diagnosis of obstetrical
emergencies, prompt tlerapy, adequate supportive
care and a multidisciplinary approacl are tle key
elements for a good outcome.
ACKNOWLEDGEMENT
I wisl to tlank all tle sta at Sultan Qaboos
University Hospital, especially tlose in tle blood
bank, for all tleir support and eorts in tlis case.
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