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Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 103-105

Anesthetic management of a teen with MURCS variant with tetralogy of Fallot for vaginoplasty

Department of Anaesthesia, Jehangir Hospital, Pune, Maharashtra, India

Date of Submission27-Mar-2019
Date of Acceptance15-May-2019
Date of Web Publication28-Aug-2019

Correspondence Address:
Dr. Avantika Sandeep Bhat
Kakade Nagar, A Building, S. No. 284, Tanaji Nagar, Chinchwad, Pune - 411 033, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/TheIAForum.TheIAForum_24_19

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MRKH (Mayer Rockytonsky Kuster Houster) Syndrome is a rare congenital disorder of reproductive system affecting females (Incidence is 1:4500 newborn girls). There are 2 types of this syndrome. Type 1 involves maldevelopment of only the reproductive system. Type 2 is also known as MURCS syndrome which involves reproductive, cardiovascular systems, renal agenesis, somatic, vertebral and hearing defects. The MURCS variant with congenital heart defects is very rare type. Morbidity and mortality of patients having corrected Tetralogy of Fallot (TOF) depends upon the severity of residual right ventricular outflow tract (RVOT) obstruction and the amount of pulmonary regurgitation. Successful anaesthetic management of such a case is similar to a tightrope walk. Acute kidney injury (AKI) to the single kidney and deranged coagulation profile were additional challenges that we addressed to during the conduct of this case.

Keywords: Corrected tetralogy of Fallot, dexmedetomidine, Mayer–Rokitansky–Kuster–Hauser syndrome, vaginoplasty

How to cite this article:
Bhat AS, Yadav A. Anesthetic management of a teen with MURCS variant with tetralogy of Fallot for vaginoplasty. Indian Anaesth Forum 2019;20:103-5

How to cite this URL:
Bhat AS, Yadav A. Anesthetic management of a teen with MURCS variant with tetralogy of Fallot for vaginoplasty. Indian Anaesth Forum [serial online] 2019 [cited 2020 Aug 12];20:103-5. Available from: http://www.theiaforum.org/text.asp?2019/20/2/103/265643

  Introduction Top

Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome is a rare congenital disorder of the reproductive system affecting females (incidence, 1:4500 newborn girls).[1] It is characterized by underdeveloped or absent uterus, cervix, and vagina. These females have a chromosomal pattern of 46 XX. The ovaries, external genitalia, and secondary sexual characteristics develop normally. Primary amenorrhea is the most common presentation.

Successful anesthetic management of an operated case of tetralogy of Fallot (TOF)[2],[3],[4],[5] demands a critical judgement for maintaining a normal sinus rhythm and systemic vascular resistance to achieve a normal cardiac output.

Acute kidney injury (AKI) to the single kidney and deranged coagulation profile were the additional challenges that we addressed to during this case.

  Case Report Top

Our patient was a 13-year-old girl, weighing 48 kg, with complaints of pain in the abdomen of unknown origin for 3 months. The patient was an operated case of TOF. On further evaluation, she was found to have uterine didelphys with partial vaginal atresia, leading to hematometrocolpos and left renal atresia. Thus, she was diagnosed of having the Type 2 of MRKH syndrome – the mullerian duct aplasia-renal agenesis-cervicothoracic somite dysplasia (MURCS) variant. She was posted for drainage of the hematometrocolpos and a corrective vaginoplasty.

Recent echocardiographic examination of the patient revealed severe residual right ventricular outflow tract (RVOT) obstruction with pulmonary regurgitation with a pressure gradient of 80 mmHg. The right atrium (RA) and the right ventricle (RV) were found to be dilated with mild leak around the ventricular septal defect patch. Vegetations were found to be present around RVOT and the pulmonary valve. Left ventricular ejection fraction was 60%. The patient was under cardiologist's treatment with tablet propranolol 40 mg twice a day and tablet carvedilol 12.5 mg twice a day. She also received antibiotic prophylaxis for bacterial endocarditis.

On preoperative examination, the patient had a heart rate of 68/min with low-volume regular pulse and a systolic murmur. Blood pressure was 110/70 mmHg. Room air saturation was 95%. Grade 2 pallor was noticed.

Laboratory tests revealed that hemoglobin (Hb) dropped from 12.2 gm% to 8.4 gm% over 3 months. International normalized ratio (INR) showed an increasing trend from 1.1 to 4.0. The same was treated with Vitamin K and fresh frozen plasma (FFP). INR on the day of surgery was 1.6. Serum creatinine increased from 0.8 to 8.0 mg/dL over 3 months. AKI was treated conservatively as it was thought to have been due to excessive nonsteroidal anti-inflammatory drug (NSAID) usage. Hence, we stopped all the NSAIDs and treated the patient with adequate hydration along with furosemide. Serum creatinine value dropped to 3.8 mg/dL with this treatment.

On the day of surgery, high-risk consent was obtained. Blood and blood products were kept ready. The patient was given her morning dose of propranolol 40 mg. All standard monitors were attached in the operation theater. The preoperative vitals were within normal limits. After preoxygenation, midazolam 50 μg/kg was given intravenously (IV) followed by fentanyl 2 μg/kg and etomidate 0.3 mg/kg. Lignocaine 2% was given IV at a dose of 1 mg/kg to avoid the intubation response. Intubation was done with injection atracurium 0.5 mg/kg as muscle relaxant. The patient was ventilated with pressure control ventilation with oxygen–air combination in 50%–50% and sevoflurane 2%. Her right internal jugular vein and right radial artery were cannulated for continuous hemodynamic monitoring.

Tranexamic acid at a dose of 10 mg/kg was given, and dexmedetomidine infusion was started at the rate of 0.4 μg/kg/h. The ongoing blood losses were replaced by packed cell volume (PCV) and FFP and platelets in 1:1:1 ratio. Total blood loss was 1050 mL which was replaced by blood and blood products at a volume of 1300 mL and 600 mL of lactated Ringer's solution, respectively. Calcium gluconate at a dose of 15 mg/kg and magnesium sulfate at a dose of 20 mg/kg were given along with blood. Total urine output of the patient was 1000 mL over 4 h.

At the end of the surgery, the residual neuromuscular blockade was reversed with glycopyrrolate 10 μg/kg and neostigmine 0.05 mg/kg given IV, and the trachea was extubated in a slightly deeper plane of anesthesia. The patient was wide awake, was pain free, and was hemodynamically stable after extubation.

  Discussion Top

There are two types of MRKH syndrome.[1] Type 1 involves maldevelopment of only the reproductive system of the patient. Type 2 is also known as MURCS syndrome which involves the reproductive and cardiovascular systems and renal agenesis, somatic, vertebral, and hearing defects. The MURCS variant with congenital heart defects is a rarity.

The postoperative morbidity and mortality of an operated case of TOF depends on the severity of residual RVOT obstruction and pulmonary regurgitation.[2],[3],[4],[5] In our case, there was severe RVOT obstruction and mild pulmonary regurgitation, leading to dilatation of the RA and RV, reflecting volume as well as pressure overload. Hence, the maintenance of normovolemia as well as normal systemic vascular resistance to avoid hypotension was important. On the contrary, increase in volume would lead to heart failure. Similarly, avoiding tachycardia was critical to avoid increase in O2 consumption, increase in RVOT obstruction, and decrease in end-diastolic volume. Very low heart rate would lead to ballooning of the heart which, in turn, leads to cardiac failure. Prevention of hypoxia and acidosis, and maintaining normothermia, was of paramount importance. RV dysfunction and failure is the most significant morbidity associated with TOF repair and the same is very difficult to treat.[2],[3],[4],[5]

As etomidate is a highly cardio stable drug, it was preferred over propofol to avoid hypotension.[6] Subarachnoid block was avoided as it would lead to profound hypotension. Epidural block was best avoided in view of the deranged coagulation profile. It further narrowed down the choice of analgesics for us. Providing very good analgesia was mandatory in this case to prevent tachycardia and hypertension. Dexmedetomidine[7] infusion was used as the primary analgesic drug. It is a selective alpha-2 agonist and provides excellent analgesia and sedation without much respiratory depression. It also has a renoprotective[8] effect.

We kept a very low threshold for early blood transfusion because of low Hb and deranged coagulation profile. Furthermore, from a cardiac point of view, it was necessary to maintain normovolemia all throughout to avoid hypotension and tachycardia. A low Hb level also leads to compromise in the oxygen-carrying capacity of blood. Hence, a timely replacement of PCV, FFP, and platelets was given in a 1:1:1 ratio.[9]

  Conclusion Top

Administration of anesthesia to a case of corrected TOF is like a tightrope walk. Maintaining stable intraoperative vitals is very difficult and yet of utmost importance. Unilateral renal atresia, recent AKI, deranged coagulation profile, and anemia were the other challenges of the present case. Our plan of general anesthesia and analgesia with dexmedetomidine infusion worked out very well for this patient. Invasive monitoring was an added advantage. Stable intraoperative hemodynamic parameters and good intraoperative as well as postoperative pain management led us to a safe conduct of anesthesia and favorable postoperative outcome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Morcel K, Camborieux L; Programme de Recherches sur les Aplasies Müllériennes, Guerrier D. Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome. Orphanet J Rare Dis 2007;2:13.  Back to cited text no. 1
Cannesson M, Earing MG, Collange V, Kersten JR. Anesthesia for noncardiac surgery in adults with congenital heart disease. Anesthesiology 2009;111:432-40.  Back to cited text no. 2
Cho YK, Ma JS. Right ventricular failure in congenital heart disease. Korean J Pediatr 2013;56:101-6.  Back to cited text no. 3
Köhler D, Arnold R, Loukanov T, Gorenflo M. Right ventricular failure and pathobiology in patients with congenital heart disease – Implications for long-term follow-up. Front Pediatr 2013;1:37.  Back to cited text no. 4
Menghraj SJ. Anaesthetic considerations in children with congenital heart disease undergoing non-cardiac surgery. Indian J Anaesth 2012;56:491-5.  Back to cited text no. 5
Sarkar M, Laussen PC, Zurakowski D, Shukla A, Kussman B, Odegard KC. Hemodynamic responses to etomidate on induction of anesthesia in pediatric patients. Anesth Analg 2005;101:645-50.  Back to cited text no. 6
Klamt JG, de Andrade Vicente WV, Garcia LV, Ferreira CA. Effects of dexmedetomidine-fentanyl infusion on blood pressure and heart rate during cardiac surgery in children. Anesthesiol Res Pract 2010; Article ID 869049, p. 7.  Back to cited text no. 7
Salah M, El-Tawil T, Nasr S, Nosser T. Does dexmedetomidine affect renal outcome in patients with renal impairment undergoing CABG? Egypt J Cardiothorac Anesth 2013;7:7.  Back to cited text no. 8
  [Full text]  
Miller TE. New evidence in trauma resuscitation – Is 1:1:1 the answer? Perioper Med (Lond) 2013;2:13.  Back to cited text no. 9


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