Int J Clin Exp Med 2015;8(8):14161-14165 www.ijcem.com /ISSN:1940-5901/IJCEM0009837

Case Report Anesthetic management of a parturient with mirror syndrome: a case report

Zhendong Xu, Yan Huan, Yueqi Zhang, Zhiqiang Liu

Department of Anesthesiology, Shanghai First Maternity and Infant Hospital, Tongji University School of Medicine, Shanghai 200040, China Received May 3, 2015; Accepted June 23, 2015; Epub August 15, 2015; Published August 30, 2015

Abstract: Mirror syndrome is a rare clinical entity consisting of fetal and placental hydrops with maternal edema. It is associated with an increase in fetal mortality and maternal morbility. We describe the anesthetic management of a parturient with Mirror syndrome complicated by HELLP syndrome and massive postpartum hemorrhage, who required general anesthesia for cesarean delivery.

Keywords: Anesthesia, mirror syndrome

Introduction and severe edema of vulva for 1 week was admitted to our hospital at 31 weeks and four Mirror syndrome is a rare obstetric entity that days’ gestation. The patient’s body weight was occurs in pregnant women and is secondary to 54.5 kg and height was 158 cm. She had no fetal and placental hydrops. The name was significant medical history and was healthy derived from the maternal signs and symptoms before the pregnancy. The baby was conceived that “mirror” those of the hydropic fetus and naturally and the patient underwent regular placenta [1, 2]. Patients often also have hemo- prenatal examinations. Ultrasonography per- dilutional anemia, hypertension, hypoprotein- formed in the second semester revealed a sin- emia and pulmonary edema [1]. Mirror syn- gleton with normal fetal umbilical blood flow but drome is not yet well recognized in clinical prac- thickening of the right ventricular myocardium. tice because of its rare incidence. The impera- Ultrasonography at 31 weeks’ gestation indi- tive treatment for mirror syndrome is to termi- cated moderate fetal growth restriction (9th nate pregnancy immediately by cesarean sec- percentile) with oligohydramnios, fetal pleural tion or induced labor if the cause of fetal effusion, and fetal hydrops. Magnetic reso- hydrops cannot be treated [3, 4]. However, the nance imaging of the fetus indicated subcuta- anesthetic management of maternal mirror neous edema of the entire body, massive syndrome has been rarely reported. The con- abdominal effusion and pericardial effusion. siderations and precautions of anesthetic man- agement were discussed in the current report. The placenta was significantly thickened with- In the report, a patient with mirror syndrome out signs of placental abruption or implanta- who was treated with cesarean section under tion. Physical examination showed that the anesthesia and followed by a postpartum hem- patient was of clear consciousness and a fair orrhage 5 hours postoperatively was success- general condition. Physical examination reve- fully treated by secondary surgery under gen- aled normal blood pressure (110/60 mmHg) eral anesthesia. with a heart rate of 80 beats/min and respira- tory rate of 15 breaths/min. Chest auscultation Case report revealed clear breath sounds without rales. Bilateral lower extremity pitting edema was A 28-year-old woman gravida 2 para 0 (G2P0) observed. Maternal laboratory tests showed: with severe bilateral lower extremity edema hemoglobin, 90.0 g/L; hematocrit, 29.6%; Anesthesia of mirror syndrome

(Cheetah Medical, Wilmington, DE, USA) was also used to measure the peripheral vascular resistance. Respiratory frequency was 20 breath/minute, and pulse oxygen saturation

(SpO2) was 95% on room air. Blood pressure was 123/75 mmHg with a heart rate of 116

beats/min. CVP was 9 cmH2O, NICOM monitor- ing indicated cardiac output (CO) of 6 L/min, total peripheral vascular resistance (TPR) of 1030 dynes·sec/cm5 (normal range, 800-1200 dynes·sec/cm5) and a total peripheral vascular resistance index (TPRI) of 1589 dynes·sec/ cm5/m2 (normal range, 1970-2390 dynes·sec/ cm5/m2).

Figure 1. The fetus with body and umbilical cord Ringers lactate solution was administered at a edema, and the accumulation of a large amount of rate of 200 mL/h. Cimetidine was injected at exudate in the abdominal cavity. dose of 250 mg once the patient was trans- ferred to the operating room. The patient was platelets, 124.4 × 109/L; aspartate amino- pre-oxygenated for 5 minutes, after which 60 transferase (AST), 76 U/L; alanine aminotrans- µg of remifentanil was injected over 30 sec- ferase (ALT), 71 U/L; albumin, 25 g/; uric acid, onds. Rapid induction was then performed with 806 µmol/L; total bile acid, 42 µmol/L; urea an injection of propofol 110 mg and succinyl- nitrogen, 6.7 mmol/L; and creatinine, 72 choline 100 mg, and cricoid pressure was µmol/L. The diagnosis of mirror syndrome was applied. Endotracheal intubation with Glide- made. scope video laryngoscope (Saturn Biomedical Systems Inc., Burnaby, BC, Canada) was easily On the third day after admission, the patient achieved using a 7.0-mm cuffed tube. Anes- presented increased dyspnea with a respirato- thesia was maintained with an infusion of pro- ry rate of 22 breaths/min, tachycardia and pofol at a rate of 300 mg/h with the addition of abdominal pain. The chest X-ray findings were sufentanyl after the umbilical cord was suggestive of pulmonary congestion. Laboratory clamped. The lungs were ventilated with posi- tests showed the following: hemoglobin, 80 tive end-expiratory pressure (PEEP) of 5 cmH2O. g/L; hematocrit, 26%; platelets, 90 × 109/L; ALT, 110 U/L; AST, 124 U/L; and 24-h urinary Obstetric surgery protein, 1.7 g/L. The features of HELLP (Hemolysis, Elevated Liver enzymes, and Low After verification of the correct tube position by Platelet counts) syndrome were noted, and the capnography, the obstetricians started the decision was made to perform an emergency operation. Three minutes after the incision, the cesarean section to terminate pregnancy. fetus was delivered. The fetus had severe total body edema and the accumulation of a large Anesthetic management amount of exudate in the abdominal cavity (Figure 1). The placenta and umbilical cord The patient was transferred to the operating were also severe edema. The fetus was imme- room and positioned supine with left uterine diately intubated with Apgar scores of 2 and 3 displacement and 30°C head elevation. A at 1 and 5 minutes, respectively. Resuscitation pre-anesthetic evaluation showed a class II was initiated by the neonatal team. Abdominal Mallampati airway with mild oral mucosa swell- paracentesis was performed and approximate- ing. Auscultation revealed diffuse crepitation at ly 40 mL of yellow liquid was drawn out. the base of both lungs. Standard monitoring Resuscitation attempts ultimately failed. was attached (electrocardiography, pulse oxim- etry, and non-invasive blood pressure) and a The patient received oxytocin 20 IU by intrave- right cervical cannula were inserted to mea- nous infusion followed by intravenous carbeto- sure the central venous pressure (CVP). A non- cin 100 µg after umbilical cord clamping. invasive cardiac output (NICOM) system However, the uterus did not sufficiently con-

14162 Int J Clin Exp Med 2015;8(8):14161-14165 Anesthesia of mirror syndrome tract despite carboprost tromethamine admin- including hypertension, edema and proteinuria, istration. To prevent atonic postpartum hemor- which makes it difficult to distinguish between rhage, bilateral ascending uterine artery liga- the two syndromes [1, 4]. Mirror syndrome and tion was performed and B-lynch sutures were preeclampsia may occur simultaneously [5]. used. During surgery, the patient was hemody- Hemodilution has been a distinct pathophysio- namically stable. Estimated blood loss was logical feature of mirror syndrome compared 1200 mL and two units of packed red blood to hemoconcentration in preeclampsia [6, 7]. cells (PRBC) were transfused. Albumin (10 g) Other important clinical features of mirror syn- and furosemide (20 mg) were administered. A drome are progressively elevated uric acid, pru- total of 500 mL lactated Ringers solution was ritus and dyspnea [1]. In the current case, the given during the entire procedure. The patient patient also had a significantly decreased was awakened and extubated once fully awake. platelet count, hypoproteinemia and slightly The early postoperative period in the post- elevated ALT and AST, and she might have had anesthesia care unit was uneventful. The HELLP syndrome. Her blood circulation had patient was then transferred to the ward 1 hour been monitored by NICOM, which revealed nor- later. mal measurements of CO, TPR, and TPRI, sug- gesting that peripheral vascular resistance was At 5 hours postoperative, the patient presented not elevated, while preeclampsia is usually with tachycardia and hypotension, and the associated with increased peripheral vascular wound drainage increased to 500 mL without resistance. Our finding indicated that TPR and vaginal bleeding. Laboratory values demon- TPRI could be used as parameters to differenti- strated hemoglobin of 55 g/L and hematocrit ate between the two syndromes. of 20%. Intraperitoneal hemorrhage may have occurred. Accordingly, the patient was trans- Mirror syndrome threatens both mother and ferred to the operating room immediately for fetus. Braun et al [1] reported that pulmonary emergency surgery to locate the source of the edema occurred in 21.4% of 56 patients with bleeding. Her blood pressure was 70/50 mmHg mirror syndrome and the average rate of intra- and she had tachycardia of 130 beats/min. The uterine death and stillbirth was 35.7%. Delivery invasive arterial blood pressure was monitored of the fetus remains the only way to reverse by radial artery cannulation. A phenylephrine maternal complications if attempts to resolve infusion was started to maintain her blood the hydrops fail [8]. pressure and three units of PRBC was trans- fused. Urgent surgical exploration was per- Studies on the anesthetic management of formed under general anesthesia with endotra- patients with mirror syndrome are rare. McCann cheal intubation, and the right ovary showed et al [7] reported the successful use of epidural marked edema with ovarian artery bleeding. analgesia in a patient with mirror syndrome at The bleeding was controlled after ligation of the 21 weeks’ gestation. Zlotnik et al [6] reported a right ovary. Approximately 3000 mL of blood case of mirror syndrome at 25 weeks’ gestation was suctioned from the peritoneal cavity. A in which the pregnancy was terminated by the total of 11 units of PRBC, eight units of fresh induction of labor. At the third stage of delivery, frozen plasma, and two units of platelets were the patient required manual revision under transfused. After the surgery, the patient was anesthesia of the undelivered placenta that transferred to the intensive care unit and extu- was retained by the uterus. Since she had no bated 1 hour later. The edema gradually van- epidural catheter in situ and her case was com- ished 3 days after operation and the patient plicated by HELLP syndrome and a difficult air- was discharged 6 days later uneventfully. way, awake fiberoptic intubation with subse- quent general anesthesia was performed. Discussion Tayler et al [2] recently reported a cesarean section under epidural anesthesia for a patient As an uncommon obstetric medical entity, mir- with mirror syndrome at 34 weeks’ gestation. ror syndrome is not well recognized and clini- The anesthetic management was relatively sim- cians still lack experience, especially regarding ple because the patient had edema only and no anesthetic management. Mirror syndrome HELLP syndrome, thrombocytopenia, anemia, shares many features with preeclampsia, pulmonary edema or dyspnea.

14163 Int J Clin Exp Med 2015;8(8):14161-14165 Anesthesia of mirror syndrome

The patient in the current case presented with attempts failed due to the neonate’s poor phys- severe overall edema that might complicate iological condition. epidural placement. Epidural hematoma forma- tion could be induced as a result of the progres- In summary, here general anesthesia was sively decreasing platelets. Also, ventilation administered for a patient with mirror syndrome support might be needed due to the pulmonary conducting cesarean section and the peripher- edema. Taking all of the above concerns into al vascular resistance was measured by NICOM consideration, epidural anesthesia was not the to differentiate it from preeclampsia. These optimal anesthetic procedure for this patient. features have not been reported before. Our Therefore, general anesthesia with endotrache- findings suggest that after careful planning is al intubation was chosen. However, the airway made for possible complications such as diffi- edema was suspected in the patient due to the cult airway, pulmonary edema and hemorrhage, edema of oral mucosa. As such, a smaller tra- general anesthesia may be safely administered cheal tube was utilized and preparation for dif- to a patient with mirror syndrome. ficult airway management was made accord- Disclosure of conflict of interest ingly. Fortunately, the endotracheal incubation was accomplished smoothly using a video None. laryngoscope. Volume control in combination with 5-cm H2O PEEP ventilation was used to Address correspondence to: Dr. Zhiqiang Liu, improve pulmonary function. Department of Anesthesiology, Shanghai First Maternity and Infant Hospital, Tongji University Fluid administration was restricted because School of Medicine, No.536, Changle Road, Jingan there was evidence of lung edema on the chest District, Shanghai 200040, China. E-mail: drliuzhq@ roentgenogram as well as hemodilutional ane- hotmail.com mia. Albumin and furosemide were used to improve serum albumin level and reduce vol- References ume overload. Volume status was evaluated by central venous pressure and stroke volume [1] Braun T, Brauer M, Fuchs I, Czernik C, variation was provided by NICOM. Dudenhausen JW, Henrich W, Sarioglu N. Mirror syndrome: a systematic review of fetal Uterine atony was found in the patient after associated conditions, maternal presentation labor, and blood loss was estimated to be and perinatal outcome. Fetal Diagn Ther 2010; approximately 1200 mL. Bilateral uterine artery 27: 191-203. ligation in combination with Blynch suturing [2] Tayler E, DeSimone C. Anesthetic management was performed, and various medicines were of maternal Mirror syndrome. Int J Obstet Anesth 2014; 23: 386-9. administered to reinforce uterine contraction [3] Brochot C, Collinet P, Provost N, Subtil D. Mirror and prevent hemorrhaging at that time. syndrome due to parvovirus B19 hydrops com- However, an acute hemorrhage occurred 5 plicated by severe maternal pulmonary effu- hours later after surgery, and a secondary sur- sion. Prenat Diagn 2006; 26: 179-80. gical exploration indicated active bleeding at [4] Gedikbasi A, Oztarhan K, Gunenc Z, Yildirim G, one ovary. It remains to be further elucidated Arslan O, Yildirim D, Ceylan Y. Preeclampsia whether the latter complication of hemorrhage due to fetal non-immune hydrops: mirror syn- is connected with mirror syndrome; however, it drome and review of literature. Hypertens is worthwhile for clinicians to be cautious that Pregnancy 2011; 30: 322-30. postpartum hemorrhage might occur in a [5] Carbillon L, Oury JF, Guerin JM, Azancot A, Blot patient with mirror syndrome [9]. P. Clinical biological features of Ballantyne syn- drome and the role of placental hydrops. Mirror syndrome is associated with an increase Obstet Gynecol Surv 1997; 52: 310-4. in perinatal mortality [9, 10]. The pediatricians [6] Zlotnik A, Gruenbaum SE, Gruenbaum BF, Koifman A, Rusabrov E. Awake fiberoptic intu- in this case were well prepared for neonatal bation and general anesthesia in a parturient resuscitation and the emergent medical treat- with mirror syndrome and a predicted difficult ments were carried out, including tracheal intu- airway. Isr Med Assoc J 2011; 13: 640-2. bation, umbilical vein catheter placement and [7] McCann SM, Emery SP, Vallejo MC. Anesthetic exudation extraction from the thoracic and management of a parturient with fetal sacro- abdominal cavities. However, the resuscitation coccygeal teratoma and mirror syndrome com-

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plicated by elevated hCG and subsequent hy- [10] Giacobbe A, Grasso R, Interdonato ML, Laganà perthyroidism. J Clin Anesth 2009; 21: 521-4. AS, Valentina G, Triolo O, Mancuso A. An un- [8] Finamore PS, Kontopoulos E, Price M, Giannina usual form of mirror syndrome: a case report. J G, Smulian JC. Mirror syndrome associated Matern Fetal Neonatal Med 2013; 26: 313-5. with sacrococcygeal teratoma: a case report. J Reprod Med 2007; 52: 225-7 [9] Zhao Y, Liu G, Wang J, Yang J, Shen D, Zhang X. Mirror syndrome in a Chinese hospital: diverse causes and maternal fetal features. J Matern Fetal Neonatal Med 2013; 26: 254-8.

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