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Umbilical cord hematoma/aneurysm
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Umbilical cord hematoma, aneurysm and varix

 

Bottom Line

 

            Umbilical cord hematomas are caused by hemorrhage into the umbilical cord. They are infrequent, one in thousands of deliveries. They have a high risk of causing fetal asphyxia or death. They produce a red mass or swelling in the umbilical cord easily seen on gross examination.

            Injection of blood during transfusion into Wharton’s jelly may cause some fetal heart rate changes, but is absorbed without further compromise. A hemorrhage from a needle stick laceration can cause rapid fetal death. Most hematomas are spontaneous, and may have no effect, cause fetal distress, or cause asphyxia and death. A ruptured umbilical vein could fill the Wharton’s jelly until compression of the distal (fetal) portion of the umbilical vein collapses. Arterial bleeding likewise could tamponade umbilical venous flow.

There does not appear to be a relationship to the size of the hematoma and the outcome, although significant hematomas are usually over 3 cm in diameter. The experience with transfusion suggests that acutely there is little compliance in Wharton’s jelly. The most reasonable explanation for large diameter hemorrhages is that they are at the site of a preformed aneurismal dilatation of the vessel. The pathology of umbilical cord hematomas seldom shows more than a thinned, dilated vein. There may be acute inflammation as part of the chorioamnionitis but this has no apparent relationship to the hemorrhage. The thinned vein wall is consistent with the concept that most hematomas are from a ruptured aneurysm. Certainly, an aneurysm starting from a point of weakness in the umbilical vein wall and finally expanding to the point of rupture is the only convincing explanation of most cord hematomas. This leaves unexplained why a weakness develops in the vessel wall in the first place, but does give some hope that the risk of cord hematoma could be detected prenatally by ultrasound, especially since most hematomas occur near the umbilicus or the placental insertion. Most hematomas do not cause stillbirth or fetal distress until late gestation and often not until labor.

            Both umbilical venous and arterial aneurysms have been described in the literature. Those of the vein may be called varix, but this can be confusing, because of the more frequently reported varix of the intra-abdominal portion of the umbilical vein. Although fresh thrombus and fetal death has been reported in umbilical vein aneurysm, the reports have not documented the pathology in sufficient detail. Not every clot in an umbilical vessel is a thrombus. Umbilical artery aneurysms have been infrequently reported but those cases have had a high incidence of fetal death, single umbilical artery and trisomy 18. The arterial wall may show calcification, and it is possible that the etiology of umbilical artery aneurysm is different from venous aneurysm.

There are no small vessels or vaso vasorum in the umbilical cord with the exception of the area around the umbilicus, or sometimes associated with the omphalo-mesenteric remnant which is also is near the umbilicus. Unfortunately, this is one portion of the cord that is not routinely evaluated microscopically since it remains attached to the infant. There is one reported case of external hemorrhage from the umbilical cord associated with gastric mucosa from an omphalo-mesenteric remnant, not surprising as this remnant is a continuation of the Meckel diverticulum of the ileum that may also hemorrhage from aberrant gastric mucosa. However, this mechanism has not been documented to have occurred in cord hematomas.

            The bottom line is that we do not know the etiology of umbilical cord hematomas. Umbilical vessel aneurysms are a likely precursor of hematomas, but we do not know the cause or natural history of such aneurysms. Cord hematomas are one cause of fetal distress in labor, and they are a cause of stillbirth prior to labor.

 

Literature Review

 

Case reports of umbilical cord hematomas:

 

Two infants had witnessed onset of bradycardia and fetal death and hypoxic-ischemic encephalopathy respectively[1]. Both infants had severe chorioamnionitis and severe funisitis. The size of the hematomas in is not described. Both hematomas were near the placental insertion. The vein wall in both cases was disrupted. There are no illustrations of the specimens.

 

One case of an umbilical cord mass without Doppler flow detected at 30 weeks gestation had a normal term delivery with Apgar scores of 8 and 9 at one and five minutes[2]. The mass several centimeters from the cord insertion measured 10 x 6 x 6 cm. It is described as having fresh blood clot within a cystic lumen, and dilatation and destruction of the wall of one artery. A lesion this size had it occurred from a sudden hemorrhage prior to 30 weeks should have obstructed at least umbilical venous blood flow. The lesion is much more likely an arterial aneurysm, but no histology was published.

 

An umbilical cord hematoma is reported immediately adjacent to the umbilicus in an infant with a short, 17cm, umbilical cord at term[3]. The labor was complicated by an episode of late fetal heart rate decelerations which recovered, then repetitive late decelerations during the third stage of labor. The Apgar scores were 9 and 10 at one and five minutes and the newborn hematocrit was 33%. The hematoma was 3 x 4 x 4 cm and was left attached to the infant, and regressed without pathological examination. The remaining placenta and umbilical cord were normal. While not explicitly discussed in the paper, the idea is that torsion late in labor would have torn umbilical cord vessels at the umbilicus, creating a hematoma that may have mildly compromised fetal gas exchange, but did not cause significant asphyxia or fetal hemorrhage. There was no mention of fetal hypokinesia sequence.

 

Two infants with a cystic structure detected by ultrasound near the fetal insertion of the cord detected at 24 – 25 weeks of gestation dies in utero in association with an umbilical cord hematoma near the cyst[4]. Perhaps due to translation the pathology is unclear as both state “hematoma of the umbilical vessels”, which to me is uninterpretable. The first infant died the day after detection of the cyst at 24 weeks. The pathology reports a patent urachus and an allantoic cyst with the hematoma between the cyst and the abdominal wall. The picture of the cyst is compatible with the ultrasound measurements of 1.5 x 1.6 cm, and hemorrhage was not in the cyst. In the second case, the infant was stillborn at 37 weeks of gestation and mother had already gone into early labor when the death was detected. The cyst is larger and was called a pseudocyst, but the gross anatomy is similar. The diameters of the hematomas would be consistent with death from umbilical vascular compression. It seems unlikely that these two cases are simple coincidental association of allantoic cyst and proximal cord hematoma, but there is insufficient pathological detail to understand the anatomical relationship. The first infant had a single umbilical artery, while the second had normal arteries. The authors speculate about the relationship of the cyst to the hematoma.

 

A cord hematoma was visualized by abdominal ultrasonography in a 32 week fetal death[5]. The authors contend that the approximately 100 ml hematoma likely caused death by exsanguination rather than by vascular compression. The hematoma appears to lie adjacent to the cord rather than within the cord. Histology demonstrated disruption of the umbilical vein.

 

A case of witnessed fetal distress and fetal death demonstrated a 50 ml hematoma was noted 6 cm above the velamentous insertion of the cord that ruptured with delivery[6]. Sections demonstrated a thinned ruptured vein as well as thinning of an artery. Funisitis was present, but is described as not being around the vein. The hematoma appears eccentric to the cord.

 

A case of an umbilical cord hematoma producing fetal distress but with a good outcome following emergency Cesarean section demonstrated a 2 cm diameter, 5 cm long hematoma 5 cm form the placental insertion[7]. The vein showed acute inflammation and a fragmented wall.

 

Two cases of hematomas without adverse outcome were reported which were unusual[8]. In the first, the mother had a cord prolapse and the witnessed development of a hematoma forming in the middle of the extruded loop. Pressure on the hematoma caused a cessation of the fetal pulse, which returned if pressure was removed. The child was rapidly delivered by forceps, and the child was pale and at 10 hours was moderately anemic with hemoglobin of 13.4 g/dl. The histology “showed the damaged vessel to be an artery”. This might be an example of arterial constriction stopping the hemorrhage, or maybe just a matter of prompt delivery. The cause of the hemorrhage in the exposed cord is puzzling, and could it be related to normal arterial post delivery constriction. The second case had a hematoma noted near the fetal insertion of the cord and another mid-cord associated with a true knot. The latter ruptured at delivery, but the cord was quickly clamped. The pathology found the hematoma associated with a degenerated vein with evidence of thrombus. These lesions likely were venous aneurysms.

 

The often cited review by Dippel of the older literature (often in German) of cord hematoma explicitly does not consider vein aneurysms since these rapidly rupture the amnion sheath and result in hemorrhage but not hematoma[9]. He does concede that some hematomas may arise from rupture of small varices. Based on his material from John Hopkins the incidence of umbilical cord hematoma is 8 in 44,043 deliveries, about 1 in 5,500. He sites two other authors with higher incidences but notes that include cases of rupture and or iatrogenic causes. He reviews an additional 28 cases from the literature, and finds syphilis to be infrequent, a fetal mortality of 50%, a majority within 5 cm of the umbilicus, and 28 of the vein compared to 3 of the artery. Not all case reports had complete information. The pathological descriptions vary although arterial lesions all had an “anomaly” of the wall. The authors had many theories of etiology, often mechanical, but seldom explaining why a relatively common situation such as cord wrapping, rupture of membranes etc. caused the uncommon cord hematoma. One case demonstrated trauma at the site of prolapse in front of the fetal head. This paper reviews two observed cases. One infant was a stillborn with a 3.5x2 cm hemorrhage starting at the umbilicus and associated with a dilated umbilical vein with a thinned wall. The other occurred in an uncomplicated term birth weight delivery. The hematoma occurred 3 cm from the umbilicus and extended to 12 cm and “The circumference of the cord was not materially increased by the presence of the hemorrhage.” The hematoma in a portion follows the helix of the vessels and is not circumferential. The text says it surrounds the vein, but the photos show it around the artery which has an intimal cushion unlike the vein.

 

 

A term infant with an uncomplicated labor without fetal monitoring had a normal heart rate 2 hours prior to the detection of a lack of fetal heart tones[10]. A cord hematoma was found at delivery which appeared to surround the vein but not the arteries, although no disruption of the vein was identified. The fetus had 2.5 x 1.5 cm unruptured subcapsular hematoma of the liver.

 

There is a paper describing umbilical cord hematoma among ultrasound detected lesions, but the description is more likely thrombus in an aneurysm at the placental surface[11].

 

A case of uncomplicated term labor with a hematoma at the placental insertion of the cord did find a tear in the umbilical vein and necrosis and calcification of the vein wall[12]. These findings suggest a hemorrhage from around an aneurysm.

 

A post term mother presented with a stillborn infant with a proximal cord hematoma with histological evidence of thinning of the umbilical vein even in an area beyond the hematoma[13].

 

A small hematoma in the middle of the cord in a stillborn infant was attributed to dissection of an aneurysm of the umbilical vein[14]. My copy of the photograph is poor, but it is not clear that is the vein since there is another dilated vessel that could be vein adjacent to the aneurysm. On the other hand only a venous dissection would have been fatal. There may be more to the case as the 28 week fetus weighed only 310 grams and the placenta weighed 380 grams.

 

An proximal umbilical cord hematoma was associated with loss of beat to beat variability prior to delivery, thick meconium in the amniotic fluid, and Apgar scores of 6 and 10 at one and five minutes respectively[15]. The source of the hemorrhage could not be determined microscopically. 

 

A term infant developed decreased beat to beat variability and bradycardia prompting an emergency Cesarean section[16]. The Apgar scores were 8 and 9 at one and five minutes. Mid umbilical cord there was a hematoma due to rupture of what appears to be a widened umbilical vein. There was a single umbilical artery.

 

A term infant rescued by Cesarean section for an auscultated bradycardia survived without complications except anemia and a persistent bradycardia postnatally[17]. The proximal hematoma was large, 5 cm diameter for 10 cm of length. The pathology could not discover the source of hemorrhage. They report another case of the sudden cessation of fetal heart beat during labor with delivery of stillborn infant with a proximal hematoma measuring 6 cm diameter by 7 cm of length. A third case had loss of fetal movement early in labor with delivery of a stillborn infant with a proximal approximately 2 cm diameter (estimated from photograph) by 5 cm length. The pathology in the latter two cases was reported as rupture of the umbilical vein.

 

Another report of three cases and more recent review of the literature reported observed a sudden bradycardia followed by vaginal delivery, another infant after two hours of loss of beat to beat variability followed by Cesarean section, a third infant with development of sinsusoidal fetal heart rate delivered vaginally[18]. The first infant had an one minute Apgar score of 1, but could not be resuscitated. The second could not be resuscitated, and the third was resuscitated but died of complications of asphyxia at 3 hours of age. All three had mid cord hematomas with thinning of the wall of the umbilical vein, and the third case had acute inflammation.

 

A fetal heart rate of 108 and a scalp pH of 7.13 led to a Cesarean section of a mother in labor[19]. The infant had an Apgar score of 1 at 5 minutes, but recovered at 8 minutes of age. The umbilical cord had a hematoma 10 cm from the umbilicus.

 

A report of a normal delivery found a fusiform hematoma 15 cm from the cord insertion into the placenta[20]. The histology demonstrated aneurismal dilatation and thinning of the umbilical cord with focal medial necrosis. There was thrombus in the vein near the hematoma.

 

A term fetus that developed sudden bradycardia was rescued by Cesarean section with Apgar scores of 7 at one minute, 9 at five minutes[21]. There was a cord hematoma near the placenta with a ruptured vessel probably a vein but no histopathology. The umbilical cord was pathologically short (14 cm).

 

A normal delivery was reported with a large cord hematoma 15 cm from the placenta[22]. The vein was “moderately” dilated with a point of rupture into Wharton’s jelly.

 

A term infant was evaluated for 12 hours of decreased movement during which an abnormal non-stress test led to Cesarean section, Apgar scores of 2 and 8, and early seizures and hypotonia[23]. The cord was thickened by hemorrhage for 13 cm starting at the umbilicus. A second case in the same report was of a term stillborn infant with a massive cord hematoma starting at the placental insertion.

 

A report of three cases of similar large cord hematomas starting at the umbilicus had respectively mild asphyxia, severe asphyxia, and stillbirth[24]. The middle case had two days of decreased fetal movement prior to delivery. In the latter two cases, microscopic examination failed to find the source of hemorrhage.

 

 

Summary of features in above cases

ref

Gestation at birth

Distance from umbilicus

Size of hematoma

Outcome

[4]

24

0 cm

3x diam

IUFD

 

37

0 cm

3x diam

IUFD

[3]

40

0 cm

3x 3 x4

Transient distress

[2]

37

1 cm

10x6x6

normal

[1]

38

At placenta

 

IUFD

[1]

41

 

 

asphyxia

[5]

32

2 cm

6 x 9 x3

IUFD

[6]

34

Near placenta

2x2x1 ruptured

IUFD

[7]

43

Near placenta

2x2x5

Fetal distress

[8]

41

Mid cord

 

Mild anemia

[8]

36

6 cm  + mid

4x4x4 + ruptured

Normal (2 hematomas)

[9]

39

0 cm

3.5 x 2

IUFD

[9]

 

3 cm

9 cm long, thin

Normal

[25]

25

1 cm

4 cm diam x 12 cm

IUFD

[10]

40

0 cm

3-3.9 cm diam x 13 cm

IUFD

[13]

44

0 cm

2 cm diam x 5 cm

IUFD

[12]

41

At placental insertion

6 cm diam

normal

[14]

28

middle

1.5 cm diam x 3 cm

IUFD

[15]

41

0 cm

3cm diam x 9 cm

Normal*

[16]

40

12 cm from placenta (42 cm total)

Max diameter 3.2 cm

Normal*

[17]

42

0 cm

5 cm diam x 10 cm

Normal* anemic

[17]

 

Near umbilicus

6 cm diam x 7 cm

IUFD

[17]

40

0 cm

2 cm diam x 5 cm

IUFD

[18]

40

15 cm from placenta (52 cm total)

7cm

Intrapartum asphyxia  and non-rescusitable

[18]

39

5 cm from placenta (total 43 cm)

4 cm

Intrapartum asphyxia  and non-rescusitable

[18]

41

10 cm from placenta (total 37 cm)

1 cm long

Intrapartum asphyxia  and death at 3 hours

[19]

39

10 cm

5 cm long, 3 cm diameter

Moderate asphyxia

[20]

Term?

15 cm from placenta

6 cm long 2 cm wide

Normal

[21]

41

3 cm from placenta

4 cm long, < 2 cm diameter

Normal

[22]

38

15 cm from placenta

8 cm long, 4 cm diameter

Normal

[23]

40

0 cm

13 cm long, narrow

Moderate asphyxia

[23]

37

At placental insertion

5.5 cm diameter, long

IUFD

[24]

41

0 cm

7 cm long, 3.5 cm diameter

Mild asphyxia

[24]

44

0 cm

9 cm long, 2 cm diameter

Severe asphyxia

[24]

41

0 cm

12 cm long, 3 cm diameter

IUFD

 

IUFD = intrauterine fetal death

 

Iatrogenic laceration of fetal vessels

 

A fetus with trisomy 18 died within 5 minutes of the formation of a cord hematoma at the placental insertion following funipuncture with a 20 gauge needle. This infant had a single umbilical artery, and hemorrhage dissected beneath the amnion over the placental surface[26].

 

Three days after an amniocentesis of slightly blood stained fluid with fetal blood, sonography revealed fetal death and an umbilical cord hematoma[25]. The karyotype was trisomy 18. The cord hematoma sections did not find the cause of the hematoma but there was three vessel funisitis. The post amniocentesis ultrasound found no evidence of hemorrhage.

 

A report of a laceration of both the umbilical vein and an artery following amniocentesis for lung maturation showed spurting of blood on ultrasound, and fetal bradycardia[27]. The heart rate recovered, but emergency Cesarean section was preformed delivering a healthy, non anemic infant with bloody amniotic fluid. There is no illustration of the pathology.

 

At 17 weeks of gestation, a hematoma was observed at the fetal end of the umbilical cord 24 hours after amniocentesis measuring 2 cm x 5 cm with normal fetal cardiac activity[28]. The report does not indicate, but likely the 2 cm measurement was the diameter. Ten days later the cord hematoma was not detectable. The infant had an uncomplicated term delivery.

 

An in-vitro study of umbilical cord puncture found that amniotic fluid immersion resulted in a slower mean bleeding time than saline immersion, but the standard variation was large.

 

Hematomas caused by injection of blood into the cord during intrauterine transfusion

 

In a reported case a cord hematoma was seen in retrospect to have formed during the last two minutes of transfusion[29]. Two and one half minutes later bradycardia occurred and lasted twenty minutes followed by a tachycardia. A pulsed Doppler after the period of bradycardia demonstrated a significant difference in blood velocity proximal and distal to the hematoma.  Seven weeks later the infant was delivered and no abnormality of the umbilical cord was recognized.

 

A study of three cases of inadvertent injection of blood into Wharton’s jelly that were stopped when the needle displacement was recognized resulted in two cases of transient bradycardia, and one of persistent bradycardia resulting in immediate Cesarean section[30]. The illustrations demonstrate very small amounts of blood in the cord.

 

A report of a 4 cm hematoma formed by transfusion of blood into the cord had twp minutes of bradycardia with normal cord venous blood gas (pH 7.36)[31]. The child was delivered 2 weeks later without complications. Histology demonstrated the hematoma but did not find compression of the vein. The authors suggest that the hematoma induces bradycardia, possibly by venous spasm, but not compression. In my opinion this does not prove the case since the blood could have dispersed in Wharton’s jelly in the minutes following the initial injection.

 

A review of 50 umbilical cords collected between 1 hour and 20 weeks of cordocentesis demonstrated only one macroscopic cord hematoma[32]. This occurred in the recipient twin of a twin transfusion syndrome. The hematoma developed within an hour of the needle puncture. The twin was found dead 6 days later, but the report has no further information about the timing of death. At delivery the hematoma was fusiform, 6 cm long and 3.5 cm in diameter, although the entire cord was “edematous”.  The cords delivered within 48 hours showed the needle tract. Cords with longer in utero duration showed healing of the puncture wound although with some persistent thinning of the injured segment. Four cases had a small hematoma encircling the vessel which was not hemodynamically significant.

 

Umbilical vein varix or aneurysm

 

A report of 17 umbilical cords with marked segmental thinning of an umbilical vein (13) or artery (4) taken from 1100 placentas from a delivery base of  9,600 deliveries suggested an association with fetal malformations, but did not try to prove that the association was significant[33]. The cases are inherently biased by the obstetrical selection of placentas to submit to pathology. The authors did describe focal thinning to just one or two smooth muscle cells, which was not called an aneurysm since there was no gross lesion. The thinned areas were as likely to be directed to the center of the cord as to the surface.

 

An umbilical vein varix 4cm from the placenta, 3 cm diameter and 10 cm long was found in a 71 cm long cord in a term fetus with intrauterine death in early labor[34].  Death was attributed to fresh thrombus in the umbilical varix, but this was not illustrated. There was a single umbilical artery. An umbilical cord varix diagnosed by ultrasound at 30 weeks of gestation was 7 cm long and 2 cm diameter, extending 1 cm into the abdominal wall, but at delivery at 39 weeks gestation the entire aneurysm was contained in the cut cord[35]. By 33 weeks of gestation the aneurysm had extended to 15 cm in length. The delivery was induced because of maternal jaundice but was otherwise uncomplicated.

 

Another approximately 2-3  cm diameter umbilical vein varix, 2 cm from the abdomen was found incidentally during scanning for placenta previa[36] . Because of decreased bio-physical profile the infant was delivered by Cesarean section without complications.

 

An umbilical vein aneurysm, or varix, 8 cm from the umbilicus measuring 8.5 x 6 x 5 cm with internal thrombus was reported in an uncomplicated term delivery[37]

Another umbilical vein varix with thrombus diagnosed prenatally was delivered without complication at 32 weeks of gestation[38]. The varix was first seen at 26 weeks of gestation.

 

A case of a 4 cm venous aneurysm 10 cm from the placental insertion of a 50 cm cord was found in a term fetus dying during early labor[39]. The authors claim there is a fresh mural thrombus in an artery as well, but the image shows a normally constricted artery. Both arteries appear constricted 13 hours after fetal death which is unusual. They describe the villi as large, immature and hypervascular, but they are not illustrated.

 

Most cases and case series of umbilical vein varices refer to lesions in the intra-abdominal portion of the umbilical vein.

 

 

Umbilical cord varices are the rule in mesenchymal dysplasia of the placenta, I will review that topic later, but there is one report of a prenatally diagnosed varix in mesenchymal dysplasia that is relevant since Doppler studies demonstrated persistently uncompromised flow through the varix (7x3x5 cm)[40].  The pathological examination demonstrated “extensive, long-standing mural thrombi”.

 

Other reports of thrombi in umbilical veins are suspect since acute thrombi may be difficult to distinguish from post mortem or even post partum clotted blood. Overdiagnosis seems to be the case in the numerous thrombi reported in a review of umbilical thrombosis in which the veins were normal and the infants either had normal Apgars or were from a series of stillbirths[41].

 

Umbilical artery aneurysm

 

We described a calcified aneurysm of an umbilical artery that resulted in fetal death without evidence of hemorrhage[42]. The aneurysm had turbulent flow on Doppler studies and only a single umbilical artery. The cause of death was unclear but kinking of the cord due to the large size (5 cm diameter, 13 cm long) of the mass was suggested.

 

A report of a similar case of umbilical artery aneurysm reviewed the 8 other published cases[43]. Notably five has single umbilical artery and four has trisomy 18 (three overlapped with both).Four were stillborn and none of these were known to have trisomy 18.

 

Of the other published cases, one with trisomy 18 demonstrated a typical artery aneurysm with a calcification of the intima and thinned media, but was unusual in that a fistula had formed between the artery aneurysm and the umbilical vein[44]. The pregnancy was terminated. The publication of the three fetuses with trisomy 18 is notable because all three had single umbilical artery; the location of the aneurysm was at the placental insertion; the aneurysms were all less or equal to 3 cm in diameter; and all three infants were liveborn[45]. A mid cord arterial aneurysm associated with tortuosity (pseudoknot) resulted in intrauterine death at 36 weeks of gestation[46]. The fusiform aneurysm measured 8 cm in length and 3 cm in diameter. The cause of death was hypothesized to be compression of the vein and other artery.

An arterial aneurysm reported after the above cases were reviewed, was detected by prenatal ultrasound at 24 weeks of gestation in a fetus with a single umbilical artery, and a unilateral multi-cystic dysplastic kidney[47]. Amniotic fluid karyotype was 46,XX. The aneurysm was adjacent to a pseudocyst at the umbilicus, and measured 2.6 by 1.7 cm. The fetus died at 26 weeks of gestation. Another subsequent case associated with a single left umbilical artery and severe intrauterine growth restriction was detected at 22 weeks of gestation[48]. The aneurysm near the placental insertion increased in size from 1.6 cm to 2.5 cm at 27 weeks of gestation. A karyotype was normal and studies for infection were normal. The patient was next seen at 34 weeks of gestation with an intrauterine death, same size aneurysm, and a birth weight of 242 g.

 

 

References:

 

 

 

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