HELLP syndrome is a life-threatening condition that can make pregnancy difficult. Named because of 3 features of the condition: Hemolysis Elevated Liver enzyme levels and Low Platelet levels. It usually occurs in the last 3 months of pregnancy but can also begin shortly after delivery.
- Several nonspecific symptoms may be present in women with HELLP syndrome. Symptoms may include fatigue; depression; excessive dehydration and weight gain; headache; nausea and vomiting; right or middle upper abdominal pain; blurred vision; and rarely nosebleeds or blisters.
- The cause of HELLP syndrome is unknown but a number of risk factors have been associated with the condition. It is more common in women with preeclampsia or eclampsia. If not diagnosed and treated early, HELLP syndrome can cause serious complications for the mother and baby.
- The main treatment is to deliver the baby immediately if the mother or the baby is in distress, even if it happens early. Treatment may also include necessary medications for the mother or baby and blood transfusions for severe bleeding problems.
The etiology and pathophysiology are still poorly understood by several hypotheses:
- Immune-mediated: maternal rejection of acute reactions to fetal antigens
- Placenta-mediated liver injury
- Systemic inflammatory response syndrome in the setting of pre-eclampsia.
The prevalence of HELLP syndrome is 0.5% to 0.9%. Approximately 70% occur during the third trimester of pregnancy and the remainder within 48 hours of delivery. The mortality of women with HELLP syndrome ranges from 0 to 24% and perinatal mortality rates up to 37%.
Presentation is variable and may include malaise epigastric and/or right upper quadrant pain with nausea and vomiting. Some may experience symptoms that are not virus-like. The symptoms continue to improve.
- Hypertension and proteinuria (main signs of pre-eclampsia) may be absent or minimal.
- The average gestational age before HELLP syndrome was 34 weeks.
- Patients typically have multiple births and are over 35 years of age.
- Patients are usually overweight and develop edema in 50% of cases.
- The situation is worse at night.  .
The dominant features are primarily related to hepatic (liver) effects:
- hepatomegaly: especially the right lobe
- hemorrhagic subcapsular hematoma rupture
- hepatic infarction
Image 2: Liver position
Treatment / Management
HELLP syndrome can quickly become life-threatening for both mother and baby. The recommendation is therefore to refer patients consistently and strictly monitor laboratory values.
- Upon admission, patients should be treated as pre-eclampsia aggressively and should be given magnesium sulfate to prevent seizures along with antihypertensive therapy.
- Maternal and fetal examination should be performed throughout each step of management as immediate delivery is generally recommended for HELLP patients themselves
- For those with a stable maternal-fetal situation between 24 and 34 weeks of gestation a recommendation is to administer corticosteroids then deliver 24 hours after the last dose. See also Neonatal Respiratory Distress Syndrome. Steroids are not only beneficial for the baby to develop the lungs, but they are also beneficial for improved laboratory values in patients especially in elevated platelet counts.
- Some patients may benefit from red cell and plasma transfusions.
Figure: Proposed protocol in the treatment of pts with HELLP Syndrome
[Chart from Journal of The American Family Physician: HELLP Syndrome]
HELLP syndrome is a life-threatening condition. Early diagnosis and treatment along with intensive maternal and neonatal care can help reduce mortality in HELLP syndrome.
- The mortality rate of women with HELLP syndrome ranges from 0%-24% with perinatal mortality rates as high as 37% (poor perinatal prognosis from low fetal incidence of fetal hypoxia and premature asphyxia and delivery low weight).
Maternal death occurs due to disseminated intravascular coagulation (DIC) placental abruption post delivery bleeding or renal failure.
- DIC occurs in 15% to 62.5% of the cases.
- Spontaneous uterine fibroids occur in 11% to 25% of women with HELLP syndrome.
- Postpartum hemorrhage occurs in 12.5% to 40%
- Cognitive impairment in 36% to 50%.
Patients with HELLP syndrome have a 19%-27% risk of developing HELLP syndrome in a subsequent pregnancy.
Physical Therapy Management
Because of the severity and risk of maternal mortality, substance abuse is not indicated in the treatment of HELLP syndrome.
- Abdominal weakness and cleansing can be seen in patients after surgery.
- Most patients are advised to wait 4-6 weeks before resuming physical activity and exercise.
HELLP Syndrome my be misdiagnosed as any of the below conditions:
- Viral Hepatitis
- Acute fatty liver of pregnancy
- Haemolytic uremic syndrome
- Thrombotic thrombocytopenic purpura
- Systemic lupus erythematosus
- Subscapular Liver Hematoma in HELLP Syndrome: A Case Report. [see article in Gastroenterology Research].
- Rare Diseases hellp syndrome Available: https://rarediseases.info.nih.gov/diseases/8528/hellp-syndrome (accessed 5.9.2021)
- Radiopedia HELLP syndrome Available:https://radiopaedia.org/articles/hellp-syndrome (accessed 6.10.2021)
- Khalid F, Tonismae T. HELLP Syndrome. StatPearls [Internet]. 2020 Jul 31.Available:https://www.statpearls.com/articlelibrary/viewarticle/22684/ (accessed 5.10.2021)
- Padden MO. HELLP syndrome: recognition and perinatal management. American family physician. 1999 Sep;60(3):829-36.
- Haram, K. Svendsen, E. Abildgaard, U. The HELLP syndrome: Clinical issues and management. A Review. BMC Pregnancy Childbirth [serial online]. 2009; 9:8.
- Cesarean Birth; Post Partum Patient Education Material: Ohio State University Medical Center. Can be located online at http://medicalcenter.osu.edu/PatientEd/Materials/PDFDocs/women-in/post-par/cesarean.pdf. Accessed 25 March 2011.
- Kapan M. Evsen MS. Gumas M.. Onder A. Tekbas G., Subscapular Liver Hematoma in HELLP Syndrome: Case Report. Gastroenterology Research. June 2010. 3(3). 144-146.