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Prenatally diagnosed cystic lymphangioma in infants
N Suzuki1, Y Tsuchida, A Takahashi
1Department of Surgery, Gunma Children's Medical Center, Setagun, Japan.
Insights
Prenatal diagnosis of cystic lymphangioma (CL) allows for planned delivery and improved outcomes. Early diagnosis in pregnancy indicates associated anomalies and potential for poor prognosis.
Area of Science:
- Medical Science
- Pediatric Surgery
- Fetal Medicine
Background:
- Prenatally diagnosed cystic lymphangioma (CL) often presents with chromosomal anomalies and hydrops fetalis.
- Cervical CL can cause neonatal respiratory distress, complicating management.
- Large CL size and associated symptoms pose treatment challenges.
Purpose of the Study:
- To evaluate the impact of prenatal diagnosis on the management and outcomes of cystic lymphangioma.
- To compare outcomes between prenatally diagnosed and undiagnosed cases of CL.
- To identify factors influencing prognosis in infants with CL.
Main Methods:
- Retrospective review of 11 prenatally diagnosed CL cases (1988-1997).
- Categorization into delivered (D) and non-delivered (non-D) groups.
- Comparison with 13 infants with prenatally undiagnosed CL.
Main Results:
- Massive CLs diagnosed prenatally often involved the head/neck and were associated with hydrops.
- Delivered infants with CL experienced respiratory distress, requiring interventions like tracheostomy.
- Undiagnosed CL cases also presented with respiratory distress, with one fatality due to complications.
Conclusions:
- Prenatal diagnosis facilitates planned delivery and prompt postnatal care, improving prognosis.
- Early and massive CL diagnosis suggests associated anomalies and a guarded outlook.
- Effective management strategies are crucial for improving outcomes in CL patients.
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.
Methods:
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).
Results:
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.
Conclusions:
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.