Anesthetic Management in a Case of MURCS Syndrome.

MURCS syndrome is a more severe form of Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, an acronym meaning aplasia/hypoplasia of Müllerian ducts (MU), congenital renal agenesis/ectopia (R), and cervical somite dysplasia (CS). A common presentation is primary amenorrhea in adolescent females. An anesthetist must consider the benefits and limitations of both regional and general anesthesia for these patients based on site of surgery and severity of malformations. We report successful anesthetic management of a 21-year-old female with MURCS syndrome scheduled for a creation of neovagina under spinal anesthesia using ultrasound guidance. Copyright:

INTRODUCTION

Around 70%–80% of women at adolescent age present in the context of primary amenorrhea.[1] The Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome is the most prevalent form of primary amenorrhea, contributing to 16% cases of primary amenorrhea.[2] It is caused due to arrest of Müllerian duct development at the 5thgestational week, characterized by congenital absence of the upper two-thirds of the vagina and an absent or rudimentary uterus in women who have normal development of secondary sexual characteristics and a 46XX karyotype.[1]

MURCS syndrome is a more severe form of MRKH syndrome, an acronym meaning aplasia/hypoplasia of Müllerian ducts (MU), congenital renal agenesis/ectopia (R), and cervical-somite dysplasia (CS). Isolated vaginal agenesis cases present in adolescence, while patients of MURCS can present at any age and depending on that they may be scheduled for correction of scoliosis, Klippel–Feil, renal defects, and cardiac lesions depending on the severity of existing malformation. The presence of associated malformations makes anesthetic management challenging for an anesthetist. Although there is enough surgical literature about this syndrome, there is a paucity of case reports on anesthetic management and considerations in a patient of MURCS syndrome.

We report successful conduct of spinal anesthesia in a patient of MURCS syndrome posted for creation of neovagina, who had L5 hemivertebra with absent posterior elements and mild scoliosis.

CASE REPORT

A 21-year-old female weighing 40 kg presented to a gynecologist with the absence of onset of menstrual cycles and chronic pelvic pain for the last 6 years. On preanesthetic evaluation, the patient was short statured (150 cm). Airway assessment showed short neck and low hairline. Spine, systemic examination, and blood investigations were normal.

An axial section of magnetic resonance imaging (MRI) abdomen and pelvis showed complete absence of uterus, cervix, and vagina, with normal ovaries. Same MRI showed absence of posterior elements of L5 vertebra and fusion of L5–S1 articular processes on the left side [Figure 1] and the presence of mild scoliosis toward the right side at lumbar level [Figure 2]. Neuraxial ultrasound was done to have clarity of space and needle path. Considering the presence of mild scoliosis, subarachnoid block planned at L4–5 interspace with midline needle insertion. After confirming nil per oral status, the patient was shifted to operating room. Under all aseptic precautions, spinal anesthesia was performed in sitting position with 0.5% hyperbaric bupivacaine 10 mg along with fentanyl 25 μg. After 10 min, the patient was taken in lithotomy position. The intraoperative period was uneventful with surgical duration of 90 min.

Figure 1

Coronal T2-weighted magnetic resonance image showing absent inferior articular process of L5 on the right side (arrow) and fusion of L5–S1 articular processes on the left side (white asterisk)

Figure 2

Coronal T2 magnetic resonance imaging of thoracolumbar spine reveals the presence of scoliosis at lower lumbar level with convexity toward the right (arrow)

DISCUSSION

Evaluation of a patient with primary amenorrhea posted for creation of neovagina should include careful assessment to search for any other associated malformations. If genital aplasia/hypoplasia is associated with any of extra genital abnormality (renal, skeletal, cardiac, and others), then it needs to be investigated in line of MURCS syndrome.

Skeletal abnormalities (12%–20% of women) such as Klippel–Feil anomaly, fused vertebrae, scoliosis, and radial aplasia may make both airway management and central neuraxial block challenging.[2] Procedures below umbilicus are normally preferred under central neuraxial block, but the presence of scoliosis may make this procedure difficult. Bilateral symmetrical spread of local anesthetics through intrathecal route cannot be predicted accurately in patients with scoliotic spine.[3] A modified paramedian approach with needle insertion toward convexity and ultrasound guidance offers advantages in patients with associated scoliosis.[4] Neuraxial ultrasound contributes to safety of lumbar central neuraxial blocks by providing anatomical information including the depth of the epidural space, the identity of a given intervertebral level, and the location of the midline and interspinous/interlaminar space.[5] Our patient did not have any finding on history and spine examination, but MRI revealed the presence of mild scoliosis and the absence of posterior elements at L5 level.

Administration of anesthesia, especially airway management, in these patients presents a significant challenge. This is due to the fusion of at least two cervical segments, short neck, low hair line, and restriction of neck motion. Presence of such features requires careful airway assessment to decide on type of anesthesia technique. Lateral radiographs, MRI of the cervical spine are required preoperatively to see extent of involvement. These patients are at high risk of neurological injury during laryngoscopy, and tracheal intubation as head extension or any sudden movements of the cervical spine may result in spinal cord compression. Therefore, one needs to maintain inline cervical stabilization, preferably using a video laryngoscope, and be prepared with a difficult airway cart including need for fiberoptic bronchoscope. Awake intubation or sedated fiberoptic intubation, while maintaining spontaneous ventilation, using dexmedetomidine and/or ketamine is a possible option.[6]

The upper urinary tract malformation (40%) is the most common association in patients of MURCS syndrome. In the presence of renal anomalies with altered renal function, drugs eliminated mainly by the kidney must be avoided or adjusted according to bispectral index and train of four.

Care should be taken while making lithotomy position because these patients are mostly short stature, and some may require pillows for support, due to associated spine deformity.

Rare cardiac associations such as tetralogy of Fallot, atrial septal defect, and pulmonary valvular stenosis dictate techniques of anesthesia depending on the clinical severity, electrocardiography, and echocardiography.[2] Aminoglycosides use for subacute bacterial endocarditis prophylaxis requires consideration due to associated renal derangement and hearing loss.

Structured systematic preanesthetic checkup may decrease the possibility of missing any associated malformation. Some additional investigations are warranted in case clinical assessment is suggestive of systemic involvement. Multisystem involvement requires meticulous preoperative evaluation and planning for smooth conduct of anesthesia in a patient of MURCS syndrome.

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 initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.