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 Table of Contents  
CASE REPORT
Year : 2023  |  Volume : 12  |  Issue : 1  |  Page : 42-46

Down syndrome with umbilical cord hematoma: Case report and review of literature


1 Department of Neonatology, Neonatal Intensive Care Unit, Dr. Sulaiman Alhabib Hospital, Buraydah, Al Qassim, Saudia Arabia
2 Departemnt of Neonatology, Dr Sulaiman Alhabib Hospital, Buraydah, Al Qassim, Saudia Arabia
3 Department of Pediatric, College of Medicine, Qassim University, Buraydah, Al Qassim, Saudia Arabia

Date of Submission21-Nov-2022
Date of Decision11-Nov-2022
Date of Acceptance27-Nov-2022
Date of Web Publication03-Jan-2023

Correspondence Address:
Ammara Naeem
Neonatal Intensive Care Unit, Dr. Sulaiman Alhabib Hospital, Buraydah, Al Qassim
Saudia Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jcn.jcn_103_22

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  Abstract 


Umbilical cord hematoma (UCH) is the extravasation of blood in the umbilical cord. The condition is rare and asymptomatic but very important to recognize because it can potentially compromise fetal blood flow. Hence, UCH increases the chances of fetal mortality and morbidity. We are presenting the case report of a large but asymptomatic UCH in a newborn baby with Down syndrome. To the best of our knowledge, the association between Down syndrome and UCH has not been reported in medical literature till the time of writing of this manuscript.

Keywords: Down syndrome, mortality, newborn baby, umbilical cord hematoma


How to cite this article:
Naeem A, Rahman S, Abdulghani MH, Almalki Y. Down syndrome with umbilical cord hematoma: Case report and review of literature. J Clin Neonatol 2023;12:42-6

How to cite this URL:
Naeem A, Rahman S, Abdulghani MH, Almalki Y. Down syndrome with umbilical cord hematoma: Case report and review of literature. J Clin Neonatol [serial online] 2023 [cited 2023 Jan 27];12:42-6. Available from: https://www.jcnonweb.com/text.asp?2023/12/1/42/366891




  Introduction Top


Umbilical cord hematoma (UCH) is a rare condition. The reported incidence is 1 in 5500–1 in 11,000 pregnancies.[1] In the majority of cases, the bleeding is venous in origin. In 10% of cases, bleeding originates from the umbilical artery. The exact etiology of (UCH) is unknown. It can be associated with anomalies of the umbilical cord, bleeding disorders, instrumentation, any invasive procedure during pregnancy, placental abruption, and torsion of the umbilical cord.[2] Presentation varies from total asymptomatic cases to fetal death due to compromise of fetal blood supply and hypoxia.[2],[3] Along with the presentation of a case of Down syndrome associated with UCH, we are presenting a review of medical literature on this subject.


  Case Report Top


A baby boy was delivered at 39 weeks of gestation through Kiwi-assisted vaginal delivery due to fetal distress. Mother was a 30-year-old primigravida with an uneventful pregnancy. She had no past history of any significant illness. During admission to the labor ward, mother experienced one episode of hypotension and fainting which improved after fluid resuscitation. At this time, there was fetal bradycardia for 4 min which improved spontaneously. At the time of delivery, there was fetal distress and bradycardia so Kiwi was applied.

At delivery, the baby was active with APGAR scores of 8 and 9 at 1 and 5 min, respectively. Cord blood gas was normal. No resuscitation was required. Birth weight was 2900 g. The umbilical cord was unusually thick with a 5 cm long hematoma starting at the base of the umbilical cord [Figure 1]. On examination, the baby had obvious clinical features of Down syndrome. The baby was admitted to the neonatal intensive care unit due to tachypnea and mild respiratory distress which was diagnosed with transient tachypnea of newborn which required oxygen through a high-flow nasal cannula. The flow was gradually weaned to room air within the first 6 h of life.
Figure 1: Umblical cord hematoma

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Ultrasound of the abdomen and base of the umbilical cord could not rule out the possibility of an umbilical hernia. Hence, a computed tomography (CT) scan was done which ruled out umbilical hernia and confirmed the diagnosis of UCH. The baby was discharged home on day 5 of life on full oral feeding with a follow-up appointment in outpatient.


  Discussion Top


UCH is a rare clinical condition which can be associated with significant neonatal mortality and morbidity. Ours is the first case report of UCH associated with Down syndrome. The reported incidence of UCH varies from 1 per 5000 to 11,000.[1] The hematoma is usually above the base of the cord. In our case, it extended to the base of the cord which is unusual. The bleeding in the umbilical cord is venous in origin in almost 90% of cases. The underlying cause remains unknown in the majority of cases. Intrapartum infection, any instrumentation, amniocentesis, anomalies of the cord, and trauma can increase the risk of UCH.[2],[3],[4] Antenatal diagnosis can be made by careful antenatal scan, amniocentesis (if there is blood in amniotic fluid), or umbilical Doppler. Sepulveda et al.[5] reported two cases of UCH which presented in antenatal scan as an umbilical cystic mass in the second trimester and later lead to fetal death. Any cystic structure associated with the umbilical cord should raise suspicion of UCH.[6] After the delivery, diagnosis is made by careful clinical examination of the cord and placenta.[7] Abdominal ultrasound is helpful in diagnosing hematoma and excluding the possibility of abdominal wall defects and umbilical hernia. In difficult cases, CT/magnetic resonance imaging (MRI) is very helpful.

We reviewed 15 case reports, including 17 patients published between 2008 and 2022, to identify risk factors, predisposing conditions, and outcomes of UCH [Table 1]. Scutiero et al. published a case report with a literature review in 2018.[1] Our review also includes cases from Scutiero et al. review. Almost all cases of UCH went undiagnosed in the antenatal period and no predisposing factors were found. There was no association of cord anomalies other than rupture of vein or artery. No abnormalities were found on gross or microscopic examination of the umbilical cord. There was no history of instrumentation or any invasive procedures in any of these cases. In three cases, mother had chorioamnionitis which might have been associated with poor outcomes.[9] There is a strong association of fetal distress with UCH due to a compromise of fetal blood supply, leading to asphyxia which was confirmed on biopsy of one case of stillbirth.[1] In our case report, there was a history of fetal bradycardia but the baby was delivered with good APGAR scores without any need for resuscitation.
Table 1: Published Case reports of Umblcial Cord Hematoma

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Diagnosis of UCH can be established by naked eye examination of the cord and placenta and by histopathology of the umbilical cord.[10] In our case, the umbilical cord was unusually thick with a 5 cm long black hematoma, starting from the base. On cutting the cord, no abnormal structures were noted in the umbilical cord.

The outcome of UCH varies from asymptomatic normal healthy infant to fetal death. We found two cases of stillbirth associated with UCH. No clear cause of fetal death was identified other than umbilical hematoma, leading to acute asphyxia.[1],[14] There are reported cases of fetal distress and hypoxic-ischemic encephalopathy (HIE) associated with UCH. In our review of these cases, there was evidence of fetal distress in the form of cardiotocography changes, reduce fetal movements, bradycardia, and poor APGAR score in the majority of cases. Three babies developed moderate-to-severe HIE requiring therapeutic hypothermia.[11],[15],[16] There was one case of cerebral palsy following HIE-induced brain insult.[15] There was one reported case of neonatal death on day 6 due to severe HIE and multiorgan failure.[16] If UCH is diagnosed in the antenatal period, there is a need for a high degree of vigilance for the management of possible HIE as prompt management can improve outcome.[19]


  Summary Top


Antenatal diagnosis of an umbilical cord mass should raise the suspicion of UCH. The fetus should be closely monitored for any signs of compromise and delivery should be planned accordingly. After birth, the umbilical cord should be cut carefully and any possibility of umbilical cord herniation must be excluded by ultrasound, CT, or MRI as required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the guardian has given their consent for the child's images and other clinical information to be reported in the journal. The guardian understands that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Scutiero G, Bernardi G, Iannone P, Nappi L, Morano D, Greco P. Umbilical cord hematoma: A case report and review of the literature. Obstet Gynecol Int 2018;2018:2610980. Available from: https://doi.org/10.1155/2018/2610980. [Last accessed on 2022/10/10].  Back to cited text no. 1
    
2.
Khatiwada P, Alsabri M, Wiredu S, Kusum V, Kiran V. Spontaneous umbilical cord hematoma. Cureus 2021;13:e13048.  Back to cited text no. 2
    
3.
Gualandri G, Rivasi F, Santunione AL, Silingardi E. Spontaneous umbilical cord hematoma: An unusual cause of fetal mortality: A report of 3 cases and review of the literature. Am J Forensic Med Pathol 2008;29:185-90.  Back to cited text no. 3
    
4.
Arora PK, Mohandas S, McAndrew S, Karody V. Spontaneous umbilical cord hematoma. J Pediatr 2017;184:233-233.e1.  Back to cited text no. 4
    
5.
Sepulveda W, Wong AE, Gonzalez R, Vasquez P, Gutierrez J. Fetal death due to umbilical cord hematoma: A rare complication of umbilical cord cyst. J Matern Fetal Neonatal Med 2005;18:387-90.  Back to cited text no. 5
    
6.
Brown R, Nicolaides K. Umbilical cord hematoma associated with an umbilical cord cyst and fetal death at 28 weeks of gestation. J Ultrasound Med 2000;19:223-5.  Back to cited text no. 6
    
7.
Available from: https://www.dovemed.com/diseases-conditions/hematoma-umbilical-cord/. [Last accessed on 2022 Nov 16].  Back to cited text no. 7
    
8.
Hooper KE, Sebire P. Spontaneous umbilical cord hematoma. Arch Dis child (F & N Ed) 2016;101:F332.  Back to cited text no. 8
    
9.
Foles AI, Gago CP, Soares J, Aguiar M, Knoblich MC, Tuna ML. Umbilical cord hematoma in a preterm newborn. Einstein (Sao Paulo) 2022;20:eAI0008.  Back to cited text no. 9
    
10.
Boughaba N. Spontaneous umbilical cord hematoma. EC Paediatr 2019;8:01-3.  Back to cited text no. 10
    
11.
Pezron J, Tellai L, Tourneux P. Spontaneous umbilical cord hematoma with a favorable outcome. Arch Pediatr 2020;27:380-2.  Back to cited text no. 11
    
12.
Mota F, Oliveira N, Fonseca M, Mimoso G. Spontaneous umbilical cord hematoma. BMJ Case Rep 2019;12:e229952.  Back to cited text no. 12
    
13.
McAdams RM, Chabra S. Umbilical cord hematoma and adrenal haemorrhage in a macrosomic neonate with anaemia. BMJ Case Rep 2016;2016:bcr2015214140. Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4746510/. [Last accessed on 2022/10/10].  Back to cited text no. 13
    
14.
Abraham A, Rathore S, Gupta M, Benjamin SJ. Umbilical cord hematoma causing still birth – A case report. J Clin Diagn Res 2015;9:D01-2.  Back to cited text no. 14
    
15.
Tonni G, Bonasoni MP, De Felice C, Rossi A, Tonni S. Histopathological findings in spontaneous hematoma of the umbilical cord: Severe hypoxic-ischemic encephalopathy in a term survived newborn. Am J Forensic Med Pathol 2015;36:254-6.  Back to cited text no. 15
    
16.
Jouannelle C, Giansily-Blaizot M, Monpoux F, Casagrande F, Poirée M, Bérard E. Spontaneous umbilical cord hematoma and congenital factor VII deficiency. Haemophilia 2012;18:e24-5.  Back to cited text no. 16
    
17.
Barbati A, Cacace MG, Fratini D, Ceccarelli T, Capanna F, Di Renzo GC. Umbilical cord hematoma with altered fetal heart rate. J Obstet Gynaecol 2009;29:150-1.  Back to cited text no. 17
    
18.
Towers CV, Juratsch CE, Garite TJ. The fetal heart monitor tracing in pregnancies complicated by a spontaneous umbilical cord hematoma. J Perinatol 2009;29:517-20.  Back to cited text no. 18
    
19.
Sánchez-Códez MI, Lubián-Gutiérrez M, Méndez-Abad P. Umbilical cord hematoma. Fetal Pediatr Pathol 2019;38:359-60.  Back to cited text no. 19
    


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