Table 1.

Prenatal detection of KTW syndrome: Our cases and review of the literature

	GA (w/g)	Limb involvement	Hand/foot anomalies	Trunk involvement	Internal lesions	Associated US-findings	Outcome
Case no./references
Case 1	20	Left leg	–	+	+	Polyhydramnios	TOP
Case 2	32	Left leg	Abnormal disposition of the toes	+	−	–	SD at 39 w/g 4 d/o: died due to KMS
Case 3	24	Left leg	–	+	+	–	TOP
Case 4	20	Right arm	Ectrodactyly of the right hand	+	+	Ascites	TOP
Hatjis et al.3	34a	Left leg, right arm	–	+	−	Polyhydramnios	SD at 36 w/g Complete resolution of lesions at 10 m/o
Warhit et al.4	32	Left leg	–	−	−	–	CS at 39 w/g Live at 21 d/o old, observation
Seoud et al.5	16a	Left arm	–	+	Testes, adrenal glands	–	TOP
Lewis et al.6	22	Both legs and arms	Overgrowth of the second toes of both feet.	+	Retroperitoneal space, pericardium, cataracts	–	TOP
Shalev et al.7	33	Right leg	–	+	−	Polyhydramnios at 33 w/g	ID at 33 w/g Died at Day 4
Mor et al.8	30a	Left leg	–	−	−	Anasarca, polyhydramnios, bulky placenta	SD at 37 w/g Port wine naevi on the arms, trunk and the left leg. 6 d/o: resolution of hydrops 6 m/o: no regression of lesions
Drose et al.9	29a	Right leg	Hypoplastic thumbs, syndactyly with hypoplastic fingernails of the both hands	−	Cataracts	Cardiomegaly, hepatomegaly, ventriculomegaly, polyhydramnios	CS at 35 w/g Died shortly after birth
Heydanus et al.10	20	Right leg	–	+	−	Cardiomegaly	TOP
Hayashi et al.11	19a	Both legs	–	+	Retroperitoneal space	Polyhydramnios	TOP
Jorgenson et al.12	19	Left leg	–	−	Liver	–	TOP
Yankowitz et al.13	17	Right leg	–	−	−	Ventriculomegaly, pleural effusion	TOP
Meizner et al.14	14a	Both legs	Marked left foot hypoplasia	+	Intestine	–	TOP
Christenson et al.15	34	Right leg	–	−	−	Cardiomegaly, thickened placenta	CS at 34 w/g KMS, intensive conservative treatment, embolisation 4 d/o: cardiovascular decompensation, amputation at the right hip 6 m/o: normal development
Paladini et al.16 Case 1	17	Left leg	–	+	Intestine, liver, kidneys	–	TOP
Paladini et al.16 Case 2	18	Right leg	–	+	−	Polyhydramnios, pericardial effusion	TOP
Roberts et al.17	19	Right arm, left leg	–	+	−	34 w/g: left-sided hydrocele	CS at 37 w/g 22 d/o: Excision of the chest wall lesion, radical axillary dissection. Conservative management of the residual lesions
Shih et al.18	15	Left leg	–	+	−	Ventriculomegaly	TOP
Jeanty19	NR	Leg	–	−	Abdomen	–	NR
Mejia et al.20	20	Right leg	–	+	Intestine	–	SD
Goncalves et al.21	30a	Right leg	–	+	−	–	CS at 37 w/g Conservative management 9 m/o: port wine stains appeared at the right thigh and knee, no resolution of the mass, varicosities of the affected leg. Expectant management up to two years
Martin et al.22	20	Left leg	–	+	Thorax, pelvis	Oligohydramnios, hypoplastic thorax	TOP
Zoppi et al.23	28	Right leg	Both feet: macrodactyly, hypertrophy of the second toe; widely spaced I and II toes	+	Abdomen, pelvis, bowel, right kidney	Cardiomegaly	Emergency CS at 32 w/g Died 8 h after delivery due to KMS
Assimakopoulos et al.24	22a	Both legs	–	+	Small intestine	–	SD at 38 w/g Expectant management 13 m/o – reduction in size of abdomen lesion
Sahinoglu et al.25	24	Left leg	–	−	−	Umbilical cord haemangioma	Preterm SD at 26 w/g Died 10 h later because of pulmonary immaturity
Peng et al.26 Case 1	28	Left leg	–	+	Retroperitoneal space	33 w/g: polyhydramnios	CS at 37 w/g 4 d/o: KMS, E. coli sepsis few days later 10 d/o: died
Peng et al.26 Case 2	35a	–	–	−	-	Ascites, pleural effusion, cardiomegaly	ID at 35 w/g Haemangiomas and varicosity of the right leg, vascular malformation. KMS 5 and 7 d/o: embolisation 15 d/o: died
Chen et al.27	22	Right leg	-	+	-	Ascites, hydrocele	SD at 36 w/g 2 m/o – partial resolution of the ascites
Coombs et al.28	24	Both legs	Hemihypertrophy, syndactyly of the right foot, left hallux hypertrophy	+	Retroperitoneal space	Hypoplasia/aplasia of the deep venous system	CS at 38 w/g 7 m/o – deep venous system anomaly, including persistent embryonal veins
Volkov et al.29	22	Both arms	–	+	−	–	TOP
Al-Asali et al.30	22	Both legs	Talipes equinovarus, abduction of the thumb and syndactyly of the right hand	+	−	Polyhydramnios	PD at 33 wks 14 d/o: died due to KMS
Horenstein31	NR	Left leg	Hypertrophy of fingers on the both hands, syndactyly of the right hand. Left foot toes hypertrophy, syndactyly	+	Abdomen	–	SD
Cakiroglu et al.32	25	Right leg	–	+	−	Persistent embryonic lateral marginal veins	TOP
Tanaka et al.33	24	Left leg	–	+	Retroperitoneal space	24 w/g: no 27 w/g: hydrops, cardiomegaly, acute foetal anaemia	Emergency CS at 27 w/g Death at 40 min after delivery due to KMS

Note: Manifestations found only postnatally are italicised. GA: gestational age; NR: not reported; TOP: termination of pregnancy; SD: spontaneous delivery; PD: preterm delivery; ID: inducted delivery; CS: Caesarean section; KMS: Kasabach–Merritt syndrome; d/o: days old, m/o: months old; w/g: weeks' gestation.

^aFinal diagnosis was made only after birth.