Lymphangiomas have been subgrouped into:
- Cystic hygromas.
- Capillary lymphangioma.
- Cavernous lymphangioma.
This histologic subgrouping is clinically impractical because antenatal
scanning cannot differentiate between these groups.
- 75-90% are found in the neck. 20% are located in the axilla. 10% are
located in the extremities, trunk, abdomen or genitalia (1-4).
- 2-3% of the cervical cysts
extend into the mediastinum and diaphragm.
- Although 20% of hygromas of
the axilla are seen in live-born infants, only 5% are antenatally
diagnosed. Either they are underdiagnosed antenatally or they represent a
distinct pathology with an alternate pathophysiology (5,6).
Unlike classical cystic hygromas which form due to a failure of the
jugular-lymphatic sequence the cystic lymphangioma of the infant is regarded as
an embryonic tumor (like the hamartoma), formed by excessive growth of tissue
in its normal location (7). The histology of fetal axillary cystic hygromas are
different to axillary cystic lymphangiomas of live-born infants.
- Simple and non-septated.
- May resolve spontaneously in
utero.
- Differential Diagnosis.
- Benign cystic
teratoma.
- Benign cystic
hemangioma.
May be associated with chromosomal abnormalities. In Reichlers series (8),
three of five cysts were associated with chromosome abnormalities (trisomy 21
and 18).
- Shoulder dystocia during
delivery (9).
- Ninh TN, Ninh TX. Cystic
hygroma in children: A report of 126 cases. J Pediatr Surg 1974;9:191.
- Siegel MJ, McAlister WH,
Askin FN. Lymphangiomas in children. Report of 121 cases. J Can Assoc
Radiol 1979;30:99.
- Bhattacharyya NC, Yadav K,
Mitra SK et.al. Lymphangiomas in children. Aust NZ J Surg 1981;51:296.
- Van Cauwelaret P, Gruwez JA.
Experience with lymphangioma. Lymphology 1978;11:43.
- Bronshtein M, Bar-Hava I,
Blumenfeld Z et.al. The difference between septated and non septated
nuchal cystic hygroma in the early second trimester. Obstet Gynecol
1993;81:683.
- Nicolaides K, Shawwa M,
Brizot M et.al. Ultrasonographically detectable markers of fetal
chromosomal abnormalities. Ultrasound Obstet Gynecol 1993:59.
- Judity E, Allanson MD.
Lymphangioma. In: Stevenson RE, Hall JG, Goodman RM (eds). Human
malformation and related anomalies. New York, Oxford University Press,
1993, p288.
- Reichler A, Bronshtein M.
Early prenatal diagnosis of axillary cystic hygroma. J Ultrasound Med
1995;14:581-584.
- Weiss BD. Shoulder dystocia
caused by axillary cystic hygroma. J Fam Pract 1991;33:524-528.