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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 manifestations1,2. 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 newborns3. 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 heterogeneity3. 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 cell2,3.
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%.
Table 1: Timeline.
| Date | Medical and surgical events | Details |
|---|---|---|
| 21/07/2012–24/09/2012 | Prenatal consultations | The patient received 7 prenatal consultations. No intercurrences |
| 2/08/2012 | Cardiology evaluation | The physical examination found a normal NIBP and HR; absence of cyanosis and jugular-vein distention and rhythmic heart sounds with a reinforcement of the second in aortic focus. The electrocardiogram showed sinus rhythm, with a 60o axis and premature ventricular contractions alternated with normal beats |
| 17/08/2012 | Transthoracic color Doppler echocardiography | Aortic dilation was evident from the root to the ascending portion. See details in diagnostic evaluation part |
| 15/09/2012 | Risk assessment | Class II of the Goldman index was assigned, and recommendations were made for the intraoperative period: avoid adrenergic and overhydration, provide antihypertensive measures, and conditional use of amiodarone for treating extrasystoles |
| 25/09/2012 | Medical board | The patient was scheduled for elective surgery with the following suggestions: continue with cardiological indications, prepare the availability of compatible red blood cell concentrate and ICU accommodation for postoperative monitoring |
| 26/09/2012 | Cardiology evaluation | The Goldman classification and indications of the previous consultation were identical because the echocardiogram findings did not evidence risk at cardiac function |
| 29/09/2012 | Color Doppler ultrasound | A single pregnancy of 33.5 weeks was found by fetal biometrics, normal Doppler flows (umbilical and middle cerebral arteries), and a small fetus for gestational age |
| 10/10/2012 | Preoperative anesthesia evaluation | Airway evaluation: permeable, complete dentition, class II of modified Mallampati, central trachea, thyromental distance > 6 cm, interincisor distance > 5 cm |
| 11/10/2012 | Cesarean section | She was in PACU for 3 hours, then went to ICU for monitoring and management. Four doses of cefazolin 2 g EV were completed every 6 hours and then the therapy was discontinued |
| 12/10/2012 | ICU discharge | She was hospitalized in general ward |
| 15/07/2012 | Hospital discharge | She received medical indications but there were no appointment dates for Cardiology and Gynecology offices at medical record |
| 21/10/2012 | Reentering by the Emergency Service | She was admitted to trauma shock unit with a diagnosis of distributive (septic) vs cardiogenic shock |
| 22/10/2012 | Readmission to ICU | The dead of the patient occurred |
NIBP = non-invasive blood pressure; HR = heart rate; ICU = Intensive Care Unit; PACU = Postanesthesia Care Unit.
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 15o. 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.
Table 2: Case reports and case series of cesarean section in MFS parturients performed with CSEA.
| Author | Dose and level | Presence of MFS comorbidities | Result | Observations |
|---|---|---|---|---|
| Ben Letaifa | CSEA at L3-L4 interspace. SD: hyperbaric Bup 5 mg + fentanyl 10 μg + morphine 150 μg. ED: (after 20 min of SD administration) lidocaine 2% 5 mL + fentanyl 10 μg (3 boluses with an interval of 5 min per catheter) | Presence of aortic dissection with MV dysfunction | Suitable blockage higher than T4 | Preload with ringer lactate 500 mL. EV infusion of ephedrine 60 mg. SP: 100-150 mmHg. DP: 60-90 mmHg. HR: 100-120 bpm |
| Saeki | CSEA at T12-L1 level. ED: none. SD: 2.2 mL 0.5% isobaric Bup | MV regurgitation, aortic dissection | Suitable blockage at T6 level | Aortic dissection managed with medical treatment |
| Baghirzada | Case 1: epidural catheter at L3-L4. Spinal puncture at L4-L5 interspace. SD: 0.75% hyperbaric Bup 9 mg. ED: Bup 0.5% 150 mg | Case 1: dilated aortic root (42 mm), MV prolapse, bicuspid AV, significant DE (lumbar dural sac area of 4.71 cm2), cervicothoracic scoliosis | Case 1: suitable block (ED anesthetic titrated during 15 min for reaching T4 level) | Case 1: used NCOM. Baseline SP was maintained with phenylephrine EV boluses of 0.1 mg. Aortic root repairing surgery five days after CS |
| Case 2: epidural catheter at L2-L3 level. SD: 0.75% hyperbaric Bup 13.5 mg + fentanyl 10 μg + morphine 100 μg | Case 2: moderate DE (lumbar dural sac area of 3.61 cm2), dilated aortic root (45 mm) | Case 2: suitable block (bilateral T5 sensory level obtained after 15 min, catheter not used for anesthesia) | Case 2: used NCOM | |
| Clayton | Reported 7 cases managed with CSEA. Inadequate blocks received: SD: 0.5% hyperbaric Bup 2.8 mL (mean initial dose) (range 1.5-4 mL) + opioids | — | 5 blocks were inadequate: 4 of the 5 CSEA parturients required additional measures despite having received ED | Additional measures: 1 patient received general anesthesia, 1 repeated spinal, 2 EV opiates |
| Heck (only CSEA cases) | Case 1: ED: none. SD: Bup 17.5 mg + DM 300 μg | Case 1: aortic dilatation (42 mm) | Case 1-2: adequate blocks | Case 4: converted to general anesthesia |
| Case 2: ED: 0.5% L-Bup 10 mL. SD: Bup 10 mg + DM 300 μg | Case 5: aortic dilatation (59 mm) | Cases 3-7: inadequate blocks | Case 7: repeated spinal anesthesia (0.5% Bup 1 mL) | |
| Case 3: SD: Bup 7.5 mg. ED: 0.5% Bup 5 mL | Cases 3 and 5-7: supplementary analgesia with alfentanil | |||
| Case 4: SD: Bup 12.5 mg + DM 300 μg. ED: 2% lidocaine 5 mL + 0.5% L-Bup 10 mL | Cases 5-7: received nitrous oxide | |||
| Case 5: SD: Bup 14 mg + DM 300 μg. ED: none | ||||
| Case 6: SD: Bup 17 mg + DM 300 μg. ED: 0.5% L-Bup 23 mL | ||||
| Case 7: SD: Bup 14 mg + DM 300 μg. ED: 0.5% L-Bup 10 mL + 2% lidocaine (plus adrenaline/bicarbonate) 10 mL | ||||
| Sakurai | ED (T12-L1 interspace): 10 mL normal saline + 2% lidocaine 10 mL per catheter (twice). SD (L3-L4 interspace): 0.5% hyperbaric Bup 11 mg + fentanyl 10 μg | Aortic root dilatation (39 mm), DE, scoliosis | Adequate sensory block at T11-T6 (beginning and end of surgery, respectively) | 4 mg ephedrine for one episode of hypotension. Hypesthesia and severe postdural puncture headache after CS |
| Yang | SD: 0.5% hyperbaric Bup 8 mg + 10 μg fentanyl. ED: 2% lidocaine 8 mL + 0.75% ropivacaine 8 mL per catheter (titrated over 20 min) | Aortic regurgitation (repaired aortic root aneurysm during childhood) | Adequate sensory block at T4 level | SP above 100 mmHg (invasive) maintained with ephedrine 4 mg EV (twice) |
| Coffman | SD: 0.75% Bup 0.5 mL + fentanyl 15 μg + morphine 100 μg (L3-L4 interspace). ED: 2% lidocaine + 1:200,000 epinephrine 5 mL (4 doses) in 10 min (1st catheter). 2-chloroprocaine 5 mL (4 doses) in 10 min (2nd catheter, L2-L3 interspace) | Aortic root replacement caused by aortic dissection one year before CS | Failed block. converted to general anesthesia. CS performed in lateral decubitus position | Monitored invasive pressure. Phenylephrine EV infusion (25-50 μg/min) + 20 μg of epinephrine (two episodes of hypotension) |
| Naud | Doses provided in CSEA not reported | Dilated aortic root (49 mm), DE | CS without complications. Patient was delivered in lateral decubitus position | Valve-sparing aortic root repair on 5th day after CS |
MFS = Marfan syndrome; CSEA = combined spinal-epidural anesthesia; Bup = bupivacaine; min = minute; L-Bup = levobupivacaine; SD = subarachnoid dose; ED = epidural dose; MV = mitral valve; EV = endovenous; SP = systolic pressure; DP = diastolic pressure; HR = heart rate; DE = dural ectasia; AV = aortic valve; NCOM = noninvasive cardiac output monitor; CO = cardiac output; CS = cesarean section; DM = diamorphine.
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 controversial5.
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 reports4,5,8,9. 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, pneumothorax10, and also the aspiration risk during induction and neonatal depression caused by opioids11. 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 DE4 or even in patients without symptoms of this condition12. 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 anesthetic4,5,12, and consequently, a high rate of failures during spinal blocks12. Likewise, the severity of DE may be related to the extent of anesthetics administered intrathecally5. 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 level4.
There are several reports of failures of neuraxial techniques even though CSEA or high anesthetic doses have been administered to compensate for the DE alterations8,12. The radiological appearance of the lumbar spine (including the dural sac) may not correlate with the clinical response to neuraxial anesthesia8,13. 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 criteria14, 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 sepsis15. 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 endpoints16. 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 patients17. 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.
