Suzuki N, Tsuchida Y, Takahashi A, Kuroiwa M, Ikeda H, Mohara J, Hatakeyama S, Koizumi T
Department of Surgery, Gunma Children's Medical Center, Setagun, Japan.
J Pediatr Surg. 1998 Nov;33(11):1599-604. doi: 10.1016/s0022-3468(98)90589-9.
BACKGROUND/PURPOSE: Prenatally diagnosed cystic lymphangioma (CL) is often associated with chromosomal anomalies, hydrops fetalis, and, in the case of cervical CL, occasionally respiratory distress just after birth. Often it is difficult to treat prenatally diagnosed CL because of the large size and associated symptoms.
Between 1988 and 1997, 11 cases of prenatally diagnosed CL were treated. Five pregnancies were terminated electively and one aborted spontaneously (nondelivered, non-D group), and another five delivered (delivered, D group). Thirteen infants nondiagnosed prenatally were also seen in the same period (prenatally nondiagnosed, without pre-D group).
In the non-D group, massive CLs were diagnosed by ultrasound scan between 16 and 20 weeks' gestation. They occurred in the head and neck in all six cases, and four had associated pleural effusions or hydrops. In the D group, CL occurred in the face and neck in four cases. Three were born by cesarean section with neonatologists and surgeons standing by, then transferred to our hospital immediately after birth. Respiratory distress appeared in two cases; tracheostomy was carried out in one case. One patient with a huge CL in the chest and abdominal wall necessitated partial resection after OK-432 sclerotherapy. In the without pre-D group, there was CL in the face or neck in 10 cases, abdominal wall in two cases, and chest wall in one case. Respiratory distress was observed in three cases of cervical CL, and tracheostomy was required in two cases. One patient with hypoxic brain damage sequela died of pneumonia afterward.
Prenatal diagnosis permits planned delivery, prompt postnatal resuscitation, and improve prognosis. Infants with massive CL diagnosed early in pregnancy have other associated anomalies as well as hydrops fetalis, and a poor outcome or difficult management can be expected.
背景/目的：产前诊断的囊状淋巴管瘤（CL）常与染色体异常、胎儿水肿相关，对于颈部CL，出生后偶尔会出现呼吸窘迫。由于瘤体体积大及相关症状，产前诊断的CL往往难以治疗。
1988年至1997年间，对11例产前诊断的CL进行了治疗。5例选择性终止妊娠，1例自然流产（未分娩，非分娩组），另外5例分娩（分娩组，D组）。同期还观察了13例产前未诊断出的婴儿（产前未诊断，无产前诊断组）。
在非分娩组中，妊娠16至20周期间通过超声扫描诊断出巨大CL。6例均发生于头颈部，4例伴有胸腔积液或水肿。在分娩组中，4例CL发生于面部和颈部。3例剖宫产出生，新生儿科医生和外科医生待命，出生后立即转入我院。2例出现呼吸窘迫；1例行气管切开术。1例胸部和腹壁巨大CL患者在OK - 432硬化治疗后需要部分切除。在无产前诊断组中，10例CL位于面部或颈部，2例位于腹壁，1例位于胸壁。3例颈部CL患者出现呼吸窘迫，2例需要气管切开术。1例有缺氧性脑损伤后遗症的患者随后死于肺炎。
产前诊断有助于计划分娩、产后及时复苏并改善预后。妊娠早期诊断出巨大CL的婴儿除胎儿水肿外还伴有其他相关异常，预后不良或治疗困难。
