The clinical features of patients with PTLS are variable [4]. It is important to underline that some of them may bring troubles to the anesthesia. And early referral and multidisciplinary assessment are very important. Firstly, tracheal intubation or insertion of laryngeal mask airway may be difficult because of facial dysmorphisms and obstructive sleep apnea. These dysmorphisms are present in 43.1% of patients, such as down-slanting palpebral fissures, frontal bossing, low-set and posteriorly rotated ears, and broad mouth. Neira-Fresneda recommended otolaryngological evaluation to assess anatomy causes of obstructive sleep apnea [5]. Secondly, preoperative cardiovascular examination is necessary. Soler-Alfonso found 10 cardiovascular anomalies in 24 patients [6]. Aortic dilation has been reported in patients with PTLS, even without a history of heart defect or cardiothoracic surgery [6]. Echocardiographic screening and electrocardiographic exam were recommended at diagnosis with attention to the aortic root and great vessels. If abnormal, consult a cardiologist. If normal, reevaluate periodically after normal echocardiogram every 2–3 years in childhood and adolescence, every 4–5 years in adulthood for aortic root dilation [5]. In our case, the patient was diagnosed with PLTS during pregnancy and was transferred to our hospital on the weekend so that we could not do multidisciplinary assessment earlier in the antenatal management. Since the medical condition there was limited and the referral system has not been well established in China, she was not taken seriously at first. However, as she was admitted to our hospital, we arranged a multidisciplinary consultation including nephrologist, neurologist, otolaryngologist. And the routine preoperative airway evaluations and cardiovascular examinations were normal. Anesthesiologists can use ultrasound technology for more comprehensive assessments in the future.
We found only one case was reported by Fernández Urbón about the anesthetic management of a child with PTLS [3]. The child was a 21-month-old girl who presented with ogival palate, retrognathia, broad filtrum, axial hypotonia and hypertonia in lower extremities. She was intervened for inguinal hernia and underwent general anesthesia with propofol combined with ilioinguinal and iliohypogastric nerve block. She was discharged the next day. We agreed with Fernández Urbón’s advocation that anesthesiologists should avoid using drugs which could cause malignant hyperthermia, such as depolarizing muscle relaxants and inhaled anesthetics. In our case, we prepared propofol and rocuronium which had specific antagonists. Furthermore, neuraxial anesthesia may be difficult in the patients of PTLS. On the one hand, poor communication and cooperation may predispose a patient`s inability to maintain stillness during needle puncture and neuraxial anesthesia may not be administered successfully. On the other hand, scoliosis may increase the difficulty of puncture technique or risk of complication [7]. In our case, fortunately, spinal anesthesia was successfully performed at the first attempt without perioperative complications. If not, recent studies have shown that spinal ultrasound can be used for difficult puncture [8].
To date, the case may be the first reported spinal anesthesia for the parturient with PTLS. Careful and comprehensive preoperative evaluation is very important for patients with Potocki -Lupski syndrome during to the varieties of clinical manifestations. In the future, we not only need more cases to explore whether spinal anesthesia is safe and effective for cesarean section, but also we need to investigate whether general anesthesia is suitable for parturients with PTLS.