Laurencio-Ambrosio, M.D., John N¹; Shiraishi-Zapata, M.D. MS, Carlos J²; Lee, M.D., Howard³

Language: English
References: 17
Page: 300-304
PDF size: 254.05 Kb.

ABSTRACT

Introduction: Marfan syndrome is an inherited disorder that affects connective tissue. Case: We report the anesthetic management of a parturient with Marfan syndrome scheduled for an elective C-section. Successful use of a combined spinal-epidural technique was used to provide neuraxial anesthesia; however, she presented an unfavorable evolution due to maternal sepsis. Likewise, a literature review of combined spinal-epidural anesthesia for C-sections in Marfan syndrome pregnant women was performed. Conclusion: Anesthetic management of parturients affected by Marfan syndrome during the cesarean section can be challenging. Strict blood pressure control during the intraoperative period has cornerstone importance. Likewise, neuraxial techniques have a significant percentage of failure in these patients.

ABBREVIATIONS:

• MFS = Marfan syndrome.
• ME = Extracellular matrix.
• CARE guidelines = Case report guidelines.
• ICD = International Classification of Diseases.
• ASA = American Society of Anesthesiologists.
• NIBP = Non-invasive blood pressure.
• IBP = Invasive blood pressure.
• CVC = Central venous catheter.
• HR = Heart rate.
• PACU = Postanesthesia Care Unit.
• RR = Respiratory rate.
• ICU = Intensive Care Unit.
• APACHE = Acute Physiology, Age, Chronic Health Evaluation.
• Hgb = Hemoglobin.
• ECG = Electrocardiogram.
• WHO = World Health Organization.
• SOFA = Sequential (Sepsis-related) Organ Failure Assessment Score.
• MAP = Mean arterial pressure.

INTRODUCTION

Marfan syndrome (MFS) is an autosomal dominant disease first described in 1896. It affects the connective tissue and has a specific phenotype characterized by cardiovascular, musculoskeletal and ocular manifestations^(1,2). Prevalence rates range from 1.5 to 17.2 per every 100,000 individuals. Approximately, 1 in 5,000 to 1 in 10,000 live newborns⁽³⁾. Most cases are caused by a mutation in the fibrillin-1 gene located on chromosome 15 (15q21.1). Patients with this mutation have wide phenotypic heterogeneity⁽³⁾. Fibrillin-1 is a structural protein, that is inserted into the extracellular matrix (ME) as a component of microfibrils; thus, its mutation causes a weakening of the structure of the ME, increased activity of the transforming growth factor-beta and loss of the interactions between ME and the cell^(2,3).

A case of cesarean section (CS) performed with combined spinal-epidural anesthesia in an MFS parturient, is reported with the Hospital Ethics Committee's written consent and adheres to CARE Case reports guidelines.

PATIENT INFORMATION

A 24-year-old nulliparous at 37 weeks' gestation, was admitted for an elective cesarean section as she had cephalopelvic disproportion (ICD-10 O33.1) in the absence of uterine dynamics and positive fetal maturity tests. She was diagnosed with MFS and had successfully had two other surgeries including correction of lens subluxation and dilation and curettag for incomplete spontaneous abortion (the type of anesthesia received in these procedures is unknown).

CLINICAL FINDINGS

On date of service, her weight and height were 62 kg and 1.73 m, respectively. She received three cardiology evaluations as shown in Table 1. In the first evaluation, a diagnostic echocardiogram was performed (see diagnostic evaluation). She was medically managed with metoprolol 50 mg orally per day. Given her medical history, she was deemed an ASA grade III according to the ASA physical status classification system. A review of her perioperative laboratory studies noted a hematocrit level of 29.8%.

DIAGNOSTIC EVALUATION

A transthoracic echocardiogram was performed seven weeks before surgery which noted an ejection fraction of 69%. It was also significant for aortic root dilation (41.7 mm in diameter) as well as mild aortic insufficiency, and grade I diastolic dysfunction. The pulmonary arterial pressure and pericardium were normal.

THERAPEUTIC INTERVENTION

The patient entered the operating room with non-invasive blood pressure (NIBP) of 60/40 mmHg, although the rest of the vital functions were normal. A 500 mL fluid bolus was immediately given (normal saline). A triple lumen 7 French central venous catheter (CVC) was placed for vasopressor and/or inotrope therapy. Additionally, a 20-gauge arterial catheter was placed to monitor the patient's hemodynamics. Norepinephrine (0.05 μg/kg/min) was initiated, with a notable improvement of her hemodynamics (phenylephrine for EV use is unavailable in the country). She was placed in the left lateral decubitus position for skin infiltration with 1% lidocaine without epinephrine in L²-L³ and a combined spinal-epidural anesthesia (CSEA) was placed without difficulty. An initial spinal dose of 5 mg of bupivacaine 0.5% with 20 μg of fentanyl was administered. Finally, a 20 G epidural catheter was threaded into the epidural space. She was immediately repositioned supine and placed in left lateral tilt 15^o. Initially, an epidural supplement of 3 mL of 2% lidocaine without adrenaline was administered per catheter to reach the level of T4. During surgery, she was resuscitated with 1,100 mL of crystalloids as well as 1,200 mL of 3.5% haemaccel. The infant was delivered within 3 minutes of incision, with an Apgar score of 8 points at the first and fifth minute. The rest of the surgical procedure was uneventful. The estimated blood loss was 900 mL. She remained on a norepinephrine infusion at 0.05 μg/kg/min throughout the duration of the procedure.

Postoperatively, the norepinephrine was stopped in the Postanesthesia Care Unit (PACU). The epidural catheter was removed after administering 1.5 mg of morphine. Furthermore, a compatible red blood cell concentrate was transfused for anemia correction (post-transfusion hemoglobin of 8.6 g/dL).

The patient was transferred to the Intensive Care Unit (ICU) for monitoring. After 24 hours, she was discharged to the general ward. Cardiology follow-up was unrevealing, and she was discharged home on postoperative day 4.

RESULTS

Six days after the discharge, she was readmitted with fever and abdominal pain. Upon initial assessment, she was found to be hypotensive (70/40 mmHg). On admission, she presented dyspnea, chest pain, and syncope. Restlessness, tachypnea (RR 22 breaths/minute), bilateral rales on pulmonary auscultation, and anuria were evidenced, being hospitalized with the diagnosis of distributive (septic) vs cardiogenic shock and respiratory failure. Hemogram showed a leukocyte count of 11.84 per 10³ and hemoglobin of 7.32 g/dL. Also, the arterial blood gases test showed metabolic acidosis, respiratory alkalosis, and a serum lactate level greater than 2. Biochemical and coagulation studies were normal. However, echocardiogram was not available at that time. Given her perilous clinical condition, she was intubated and placed on mechanical ventilation. A new CVC was placed for infusion of norepinephrine at an adjustable dose.

After admission to the ICU, dopamine and dobutamine infusions were initiated. Her clinical status continued to deteriorate and subsequently had a cardiac arrest. She received 30 minutes of ACLS, however, ROSC was never achieved. Death was communicated to the relatives and the proceedings for the autopsy of the law were initiated. This examination evidenced multiple organic damage as the basic cause of the patient's death.

DISCUSSION

We performed a peer-reviewed literature search about CSEA management for CS in MFS pregnant women using Medline, SCOPUS, EBSCO, ScienceDirect, and Cochrane database. The search included all studies published without language restriction from the start of databases to June 2020. The search included the following MeSH terms: cesarean section, cesarean delivery, Marfan syndrome, anesthesia. The included reports are shown in Table 2.

The anesthetic management of patients with MFS can be challenging due to the lack of literature, dilatation of aortic root and ascending aorta, and presence of dural ectasia (DE) (widening of the spinal canal with the erosion of the vertebral body, widening of the neural foramina, or existence of meningocele)⁽⁴⁾. Furthermore, which anesthetic technique is most appropriate for these parturients is still controversial⁽⁵⁾.

A CSEA was performed for avoiding hemodynamic response to laryngoscopy, orotracheal intubation, and surgery (hypertension, heart rate, myocardial contractility, and aortic wall stress)^(5-7) since our patient had aortic root dilatation as in previous reports^(4,5,8,9). This technique can prevent the possibility of other problems during general anesthesia in MFS patients such as difficult airway, luxation of mandibular, and cervical joints, respiratory restrictive disorder, pneumothorax⁽¹⁰⁾, and also the aspiration risk during induction and neonatal depression caused by opioids⁽¹¹⁾. Likewise, invasive pressure monitoring was performed because of aortic involvement.

In previous reports of CS under CSEA in MFS parturients, it has been suggested to consider this technique in the presence of DE⁽⁴⁾ or even in patients without symptoms of this condition⁽¹²⁾. DE can cause an increase in the capacity and the cerebrospinal fluid of the lumbosacral dural space, causing a restriction of the extension of the intrathecal anesthetic^(4,5,12), and consequently, a high rate of failures during spinal blocks⁽¹²⁾. Likewise, the severity of DE may be related to the extent of anesthetics administered intrathecally⁽⁵⁾. The epidural space in patients affected with DE may also have an increased size along with the dural sac, thus requiring a greater volume of a local anesthetic to achieve an adequate level⁽⁴⁾.

There are several reports of failures of neuraxial techniques even though CSEA or high anesthetic doses have been administered to compensate for the DE alterations^(8,12). The radiological appearance of the lumbar spine (including the dural sac) may not correlate with the clinical response to neuraxial anesthesia^(8,13). Also, DE gradually worsens with age, so a prior adequate spinal block does not ensure the success of future neuraxial blocks (including CSEA)⁽⁵⁾. Although our patient had no clinical features of DE, a magnetic resonance imaging of the lumbar spine was not performed to rule it out completely.

Based on the above, it should take in mind that neuraxial techniques have a significant percentage of failure in these patients and that strict blood pressure control, avoiding hypertension and hypotension, during the intraoperative period has cornerstone importance.

Our patient had a presumption of infection and a SOFA (Sequential [sepsis-related] Organ Failure Assessment Score) score greater than 2 points (anuria, mean arterial pressure [MAP] less than 70 mmHg, Glasgow score of 13 points) during hospital readmission, which in addition to the need for vasopressors to maintain MAP greater than 65 mmHg and a lactate value higher than 2 mmol/L, allows recognizing a diagnosis of septic shock according to current criteria⁽¹⁴⁾, which was confirmed by necropsy. Indeed, the recognition of maternal sepsis is problematic because gestational physiological changes overlap with the hemodynamic changes of the initial picture of sepsis (for example, the leukocyte elevation that is a normal finding in pregnancy and the expanded plasma volume that permits compensating for longer before a rapid deterioration)⁽¹⁵⁾. Therefore, a high degree of suspicion including a detailed history and examination is important for the early recognition of maternal sepsis⁽¹⁵⁾. Along with this, we must admit that, in this case, early follow-up by the outpatient office could have allowed recognizing a scenario compatible with sepsis.

Finally, current guidelines from the Surviving Sepsis Campaign recommend fluid resuscitation with crystalloids over colloids. They also advised volume replacement with albumin in case of the need for large amounts of crystalloids due to failure to achieve predefined hemodynamic endpoints⁽¹⁶⁾. Caution against the use of colloids in the case of dextrans and gelatins was the result of limited safety evidence. However, hydroxyethyl starch solutions were associated with kidney injury and increased mortality in critically ill patients⁽¹⁷⁾. In this case, a gelatin solution was used due to contribute maintaining an adequate mean arterial pressure until the arrival of the red blood cell concentrate.

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AFFILIATIONS

¹ Staff Anesthesiologist, Department of Anesthesiology. Hospital María Auxiliadora. Lima, Perú.

² Staff Anesthesiologist, Service of Surgical Center and Anesthesiology, Hospital II Talara. Professor of the Second Specialization Unit, Facultad de Ciencias de la Salud, Universidad Nacional de Piura. Perú.

³ Assistant Professor. Department of Anesthesiology. Northwestern University. Feinberg School of Medicine. Chicago, Illinois, USA.

Disclosure of funding received for the work: None.

Conflict of interest: None.

CORRESPONDENCE

Carlos J Shiraishi-Zapata. E-mail: shiraishi52@hotmail.com, cshiraishiz@unp.edu.pe

Received: 05-11-2020. Accepted: 09-03-2021