Uterine cavernous haemangioma in pregnancy

Abstract

We report on the key sonographic feature of uterine cavernous haemangiomas in pregnancy, a rare condition with only 14 cases reported in the literature including this case. A key feature of our case is post‐partum follow‐up ultrasound investigation up to 6 months. This condition is associated with morbidity and mortality, correct identification can prevent catastrophic outcomes.

Vascular anomalies (benign soft tissue lesions) involving the adult female genital tract are extremely uncommon.1 The International Society for the Study of Vascular Anomalies (ISSVA) reclassified vascular lesions into tumours and malformations. According to this classification vascular anomalies referred to as haemangioma in the past are now separated into true haemangioma and vascular malformations on the basis of physical findings, clinical behaviour, histological findings and cellular kinetics.2 True hemangiomas undergo spontaneous regression by puberty, therefore, most of the vascular malformations found in adults belong to the second group. These permanent malformations are categorised into arterial, venous, capillary, lymphatic and mixed. Venous malformations are the most common and are typically described as cavernous haemangiomas. These lesions can occur throughout the female reproductive tract including myometrium, endometrium, cervix and vaginal wall. Venous malformations very rarely affect the uterus and are rarely described in pregnancy. There are the significant complications associated with this rare finding. We would like to report an obstetric case including post‐partum follow‐up and a review of reported cases of a uterine cavernous haemangioma diagnosed in pregnancy.

Case report

A healthy 28‐year‐old G3P2 was sent to our unit at 20 weeks gestation for review for a suspected partial mole. Her previous pregnancies were uncomplicated, both deliveries were Caesarean sections. Ultrasound examination revealed an anatomically normal and appropriately grown fetus and in particular, the placenta appeared normal. Extensive cystic spaces were noted within the myometrium (Figures 1, 2, 3), confirmed by MRI (Figure 4). In view of the normal pregnancy findings trophoblastic disease was considered to be unlikely. A literature search with keywords ‘massive cysts and uterus’ revealed a similar case3 and a reply4 to that publication led to the provisional ultrasound diagnosis of a cavernous uterine haemangioma. A reported maternal feature is anaemia, most likely due to sequestrated blood within the venous spaces. This was also the case with our patient (Hb 90 g/L). We arranged frequent surveillance and our patient developed significant discomfort at 30 weeks due to the massive distended uterus. An elective LSCS with a multidisciplinary team was performed at 34 + 5 weeks without complication. The baby weighed 2480 g with Apgars of 9¹, 9⁵ and the neonatal course was uncomplicated. There was no significant blood loss. Recovery was uneventful and in keeping with previous reported cases, the haemoglobin returned to normal spontaneously when the sequestrated blood returned to the circulation – see results below (Figure 5).

Figure 1.

Extensive cystic spaces in the myometrium at 20 weeks gestation.

Figure 2.

Low level venous flow demonstrated with colour Doppler imaging.

Figure 3.

Massive expansion at 30 weeks.

Figure 4.

MRI sagittal view of the uterus at 32 weeks demonstrating a thickened anterior myometrium with cystic spaces.

Figure 5.

Excerpt from blood profile demonstrating improvement in haemoglobin following delivery. No transfusion was necessary.

A pelvic scan 6 months post delivery revealed a moderately enlarged uterus (12 × 55 × 86 mm) with small cystic spaces (Figures 6 and 7) involving the anterior wall of the uterus. The patient was amenorrhoeic due to hormonal contraception.

Figure 6.

Sagittal view of the uterus at 6 months post‐partum demonstrating cystic spaces within the anterior myometrium.

Figure 7.

Transverse view of uterus 6 months post‐partum demonstrating cystic spaces within the myometrium.

Discussion

To the best of our knowledge, there are 13 reported cases of uterine venous malformations involving pregnancy summarised in Table 1. There was one maternal deaths reported, two fetal deaths, two post‐partum hysterectomies and significant blood loss (>1000 mL) in seven patients. These significant outcomes make the identification of uterine venous malformations important in order to prevent catastrophic outcomes. The main feature of these lesions is a diffusely thickened myometrium with extensive small cystic spaces. Colour flow Doppler imaging typically demonstrates low velocity flow, or no discernible flow within these spaces. This appearance is often mistaken as a form of trophoblastic disease, highlighting the importance of βHCG levels as a way to differentiate between these conditions.

Table 1.

Reported cases of uterine cavernous haemangioma in pregnancy

Case	Publication	Patient age/history	Presentation	Antenatal imaging	Delivery	Outcome	Diagnosis and comments
1	Corbacioglu Esmer et al.5	25 yo P0 Cutaneous haemangioma	Fetal growth restriction at 25/40	US: diffusely thickened myometrium with numerous echolucent areas Low velocity blood flow with mixed arterial and venous pattern	LSCS at 32 weeks (fetal distress)	Uterus preserved. No significant post‐partum bleeding	Cavernous haemangioma (MRI performed 1 week post‐partum)
2	Bhavsar et al.6	25 yo G1P2	Collapse 1/52 postpartum	Not reported	LSCS (twins)	Maternal death (pulmonary embolus)	Cavernous haemangioma (histopathology)
3	Benjamin et al.7	27 yo P1	Profuse PV bleeding at 11 weeks postpartum	Not reported	LSCS at term (failure to progress)	Hysterectomy	Diffuse haemangioma (histopathology)
4	Djunic et al.8	33 yo P0. Congenital diffuse haemangioma lower leg, gluteal region and labia	Uterine abnormalities on US at 24/40. Low grade DIC at 26/40	Not reported	LSCS (FDIU at 28/40 gestation)	Uterus preserved	Diffuse cavernous haemangioma with significant disseminated intravascular coagulation (histopathology)
5	Virk et al.9	21 yo G3P2	Elective LSCS (2 previous LSCS) Mild anaemia	Not reported	Caesarean section at term	Peri‐partum hysterectomy due to uncontrollable bleeding	Cavernous haemangioma (histopathology)
6	Comstock et al.10	age not reported G1P0	Large for dates at 27/40	US: diffusely thickened with numerous tubular echolucent areas No demonstrable blood flow within cystic spaces	LSCS at 40/40 (failure to progress)	Uterus preserved 1700 mL blood loss	Cavernous haemangioma (histopathology on biopsy specimen)
7	Uotila et al.3	25 yo G1P0	Uterine enlargement and tenderness at 18/40	US: thickened multi‐cystic uterine wall No demonstrable blood flow within cystic spaces MRI: thickened uterine wall	LSCS at 30/40 (low grade DIC and anaemia)	Uterus preserved 3000 mL blood loss	No definitive diagnosis (histopathology of biopsy specimen)
8	Thanner et al.11	26 yo P0 Hx aneurysmal malformations lower body. LMWH since 10/40 for elevated D‐dimers	18/40 referral for suspected haematoma of uterine wall	US: diffusely thickened myometrium with numerous vascular channels	LSCS at term for fetal bradycardia	Uterus preserved. Amniotic fluid embolism and uterine atony. Massive secondary PPH at day 13 requiring transfusion	Angioleimyomatous hyperplasia (histopathology of biopsy specimen reported)
9	Sütterlin et al.12	26 yo G2P2	17/40 referred for abdominal pain	US: thickened myometrium with numerous vascular channels ‘wild’ blood flow within cystic spaces	Classical CS at 41 + 2/40	Uterus preserved 600 mL blood loss	Pregnancy induced vascular ectasia and oedema (histopathology)
10	Weissman et al.13	30 yo G3P2	33/40 referred for suspicion of incomplete molar pregnancy following US Asymptomatic	US: thickened myometrium with lacunar formations Turbulent blood flow	Uncomplicated vaginal delivery at 41/40	Uterus preserved. Uncomplicated ante/postnatal course. Follow‐up ultrasound at 6 weeks post‐partum demonstrated significant reduction in size of lesion	Cavernous haemangioma (based on antenatal US appearances)
11	Lotgering et al.14	32 yo P	Enlarged uterus at 14/40	US: findings suggestive of uterine haemangioma	Uncomplicated vaginal delivery at term	Uterus preserved. No reported complications.	Cavernous haemangioma (based on antenatal US appearances)
12	Hadlock et al.15	15 yo G2P1	22/40 uncertain dates Mild anaemia	US: ‘snowflake’ pattern in myometrium	LSCS 40/40 (previous LSCS)	Uterus preserved 1500 mL blood loss	Dilated mural venous channels in myometrium (noted at LSCS)
13	Dawood et al.16	32 yo P0 Congenital haemangioma on left side of face and trunk	Abdominal pain at 36/40 Maternal anaemia	Not reported	LSCS at 36/40 for failure to progress	FDIU 36/40 Uterus preserved (resection of haemangioma) Haemoperitoneum 1500 mL requiring transfusion	Numerous vascular channels consistent with haemangioma (histopathology of resected tissue)

G, gravida; P, para; US, ultrasound; LSCS, lower segment caesarean section; PPH, postpartum haemorrhage; CS, caesarean section; PV, per vagina; DIC, disseminated intravascular coagulation; FDIU, fetal death in utero; MRI, magnetic resonance imaging.

There are case reports of pregnancies in patients with Klippel–Trenaunay–Weber syndrome (OMIM 149000) where angiomatosis has affected the uterus.17, 18 The sonographic description of a ‘spongy appearance’ to the uterine wall was described by Verheijen et al.18 While this appearance is similar to that described in case reports of uterine cavernous haemangioma, the Klippel–Trenaunay–Weber lesions tend to be focal. These lesions have been reported to regress after pregnancy. These cases have not been included in Table 1.

It is worthwhile reflecting on possible reasons for abnormal vascular proliferation in pregnancy. Studies have demonstrated that oestrogen can increased the mitotic rate in the endothelial cells that line these capillaries, and in addition indirect angiogenic factors such as EPCs, VEGF and others can be released due to increased circulating oestrogen. Corticosteroids are utilised in the treatment of some vascular proliferative diseases; this may be considered in the management of future cases.19

The key to the management of uterine cavernous haemangioma is recognition of the underlying condition and potential for severe complications, proper preparation, including consultant obstetrician and anaesthetist involvement, availability of blood products and care in a tertiary level hospital with intensive care availability if required. Understanding the association with anaemia also allows an expectant approach post surgery in stable cases to allow for the natural return to normal levels.