Kang Yanming, Ma Yushan, Jiang Xiaoqin, Lin Xuemei, Zhao Fumin
Department of anesthesiology.
Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education.
Medicine (Baltimore). 2019 Aug;98(31):e16670. doi: 10.1097/MD.0000000000016670.
Fetal giant cervical cyst (FGCC) is a rare congenital anomaly. Sometimes FGCC may extend into the mediastinum, and result in severe tracheal compression, which is a life-threatening event at birth.
We present a rare case of FGCC, which extended from the right neck into the superior mediastinum, and resulted in severe tracheal compression.
An FGCC was observed by ultrasonography and magnetic resonance imaging (MRI) at 27+4 weeks' gestation (WG). Fetal MRI at 35+1 WG showed that the FGCC was 3.3 × 8.2 × 7.5 cm and extended from the right neck into the superior mediastinum. Severe tracheal compression was observed and the inside diameter of the narrowest section of tracheostenosis appeared thread-like and measured only 0.1 cm.
Cervical cyst reduction was performed prenatally under ultrasound guidance to alleviate the tracheal compression and maximize the chance of fetal survival 2 days before birth. At 36+3 WG, cesarean section was performed, and a female neonate was immediately delivered and intubated (3.5-mm tube) by an experienced anesthesiologist. Neonatal intralesional sclerotherapy and cystic component aspiration as guided by digital subtraction angiography were performed under general anesthesia. Anesthesia was maintained only with sevoflurane 3% in 2 L/min oxygen. Extubation was performed soon after surgery.
The neonate recovered uneventfully and was discharged 2 days postoperatively. After 140 days of follow-up, the neonate had recovered completely.
If an FGCC is suspected by abdominal ultrasound, a fetal MRI is recommended to assess the severity of tracheal compression before birth, if feasible. An anesthesiologist should assess the risk of intubation failure at birth according to those results. If fetal severe tracheal compression is detected and it may result in inability of intubation at birth, prenatal cervical cyst reduction under ultrasound guidance may be effective for alleviating tracheal compression at birth, if feasible. This could maximize the chance of fetal survival. Improvement of fetal short- and long-term outcomes is important.
胎儿巨大宫颈囊肿(FGCC)是一种罕见的先天性异常。有时FGCC可能延伸至纵隔,导致严重气管受压,这在出生时是危及生命的事件。
我们报告一例罕见的FGCC病例,其从右颈部延伸至上纵隔,导致严重气管受压。
在妊娠27 + 4周(WG)时通过超声检查和磁共振成像(MRI)观察到FGCC。妊娠35 + 1周时的胎儿MRI显示FGCC大小为3.3×8.2×7.5 cm,从右颈部延伸至上纵隔。观察到严重气管受压,气管狭窄最窄处内径呈线状,仅0.1 cm。
在出生前2天,在超声引导下进行产前宫颈囊肿减压,以减轻气管受压并最大限度提高胎儿存活机会。妊娠36 + 3周时,行剖宫产,一名女婴立即娩出,由经验丰富的麻醉医生立即插管(3.5 mm导管)。在全身麻醉下,在数字减影血管造影引导下进行新生儿病灶内硬化治疗和囊肿成分抽吸。仅用3%七氟醚在2 L/min氧气中维持麻醉。术后不久即行拔管。
新生儿恢复顺利,术后2天出院。随访140天后,新生儿已完全康复。
如果腹部超声怀疑有FGCC,可行的话,建议进行胎儿MRI以评估出生前气管受压的严重程度。麻醉医生应根据这些结果评估出生时插管失败的风险。如果检测到胎儿严重气管受压且可能导致出生时无法插管,可行的话,超声引导下的产前宫颈囊肿减压可能有效减轻出生时的气管受压。这可最大限度提高胎儿存活机会。改善胎儿短期和长期结局很重要。