In reply to “Anesthesia for cesarean delivery in a patient with Klippel-Trenaunay Syndrome”: a mini case series in our institution

We read with interest the case reported by de Avelar Texeira et al.¹ Safe anesthesia management for deliveries complicated by Klippel-Trenaunay Syndrome (KTS) is challenging for potential difficult airway management owing to the soft tissue hypertrophy and possible undetected hemangiomas, hemorrhagic complications as result of pelvic varicosities injured during surgery, presence of neuraxial vascular malformation.

We managed two Cesarean Sections (CS) in the same parturient (Patient 1) and one vaginal delivery in a different patient (Patient 2) both affected by KTS, opting for spinal anesthesia in the first case and epidural analgesia in the second one. Patient 1 at her first urgent CS² at 38 weeks of gestation revealed Body Mass Index (BMI) 39.8 kg.m^-2, varicosities of the right leg, Mallampati Class III, no evidence of port-wine stains on her back and normal coagulation profile. She had been scheduled for Magnetic Resonance Imaging (MRI) to determine the presence of Arterio-Venous Malformations (AVM) in the pelvis, spinal cord before the delivery, but she had not yet undergone. Basing on MRI of lumbar spine performed 6 years before showing no AVM we decided for spinal anesthesia. Sixteen months later Patient 1 was referred to our Anesthesia Preadmission Clinic. A MRI performed at 27 weeks showed absence of abdominal, pelvic and spinal AVM. She had BMI 36.7 kg.m^-2 and normal coagulation profile. Considering her history and new MRI we performed a spinal anesthesia for her elective CS. Anesthesia and surgery were both uneventful.

Patient 2 had BMI 37.5 kg.m^-2, vascular overgrowth of the left leg, hemangioma of sacral area (Fig. 1), Mallampati class 3. Her history revealed a pelvic ultrasound Doppler documenting only a 6 mm left vulvar varicosity, a lumbar MRI negative for spinal/epidural AVM and a successful spinal anesthesia three years before. After spontaneous labor initiation an epidural catheter was inserted in the L3-L4 interspace using ultrasound assisted technique. Analgesia maintained with programmed intermittent epidural bolus. Labor proceeded uneventfully and a liveborn female infant was delivered 4 h later.

Figure 1
Hemangioma of sacral area.

Our choices differed from de Avelar Texeira et al. and it was mainly for the dissimilar clinical manifestations of the KTS. Our parturients had no history of cerebral hemangioma, paresis or other neurological manifestations except for pain crisis of the affected limb. We agree with the authors that documented back angiomas, hemiparesis and total absence of spine imaging should discourage the use of neuroaxial blocks but two points are still not clear in the case presented¹: why not use neuroaxial block as in the two previous caesarean sections? Why despite abdominal angiotomography being available is there no information about lumbar spine?

In our opinion general anesthesia in KTS could be more risky then neuraxial block for two crucial reasons: potential bleeding of unknown airway AVM in particular if difficult (Mallampati class 3) management is predicted, rupture of undetected cerebral hemangiomas as result of sudden blood pressure increase during laringoscopy. Considering these issues neuraxial block supported by at least a negative spine MRI seems to be the safest option.

References

• ¹
  Teixeira CEFA, Braga AFA, Braga FSDS, Carvalho VH, Costa RMD, Brighenti GIT. Anesthesia for cesarean delivery in a patient with Klippel-Trenaunay syndrome. Rev Bras Anestesiol. 2018;68:641-4.
• ²
  Gonnella GL, Scorzoni M, Catarci S, Zanfini BA, Draisci G. Successful use of spinal anesthesia for an urgent cesarean section in a parturient with a severe Klippel-Trénaunay syndrome. Korean J Anesthesiol. 2018;71:411-2.

Publication Dates

• Publication in this collection
  01 July 2020
• Date of issue
  Jan-Feb 2020

History

• Received
  9 July 2019