|
 
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| CASE REPORT |
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| Year : 2023 | Volume
: 3
| Issue : 3 | Page : 167-170 |
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Early onset portal hypertension due to silent extensive portal vein thrombosis following umbilical vein catheterization
Surbhi Latawa, Ritu Sharma, Anuradha Rai, Dhulika Dhingra
Department of Paediatrics, CNBC, New Delhi, India
| Date of Submission | 21-Mar-2023 |
| Date of Decision | 13-Jun-2023 |
| Date of Acceptance | 21-Jun-2023 |
| Date of Web Publication | 14-Aug-2023 |
Correspondence Address:
Dr. Anuradha Rai
CNBC, New Delhi
India
 Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ipcares.ipcares_63_23
Background: Umbilical vein catheterization is a routine procedure in neonatal intensive care units. This invasive procedure, though easy and useful, can result in disastrous complications. Clinical Description: A 28 day old baby boy presented with umbilical sepsis along with multiple skin abscesses. He had a history of double volume exchange transfusion for Glucose 6 Phosphate deficiency induced hyperbilirubinemia, during the postnatal period. Ultrasound whole abdomen done to look for intra-abdominal abscesses revealed extensive portal vein thrombosis including the main portal vein and its branches with cavernoma formation. Upper gastrointestinal endoscopy revealed Grade II esophageal varices suggestive of the development of portal hypertension. Management and Outcome: Baby was started on subcutaneous enoxaparin which continued for 3 months. Follow up ultrasound showed complete obliteration of portal vein, replaced by cavernomas with development of grade II esophageal varices seen on upper gastrointestinal endoscopy. Conclusion: This case highlights how umbilical vein catheterization in the neonatal period can lead to catastrophic complications such as obliteration of the entire main portal vein and its branches, formation of portal cavernoma, portal hypertension, and esophageal varices, all of which can develop silently, at a very early age.
Keywords: Neonate, portal cavernoma, umbilical sepsis, varices
How to cite this article:
Latawa S, Sharma R, Rai A, Dhingra D. Early onset portal hypertension due to silent extensive portal vein thrombosis following umbilical vein catheterization. Indian Pediatr Case Rep 2023;3:167-70 |
How to cite this URL:
Latawa S, Sharma R, Rai A, Dhingra D. Early onset portal hypertension due to silent extensive portal vein thrombosis following umbilical vein catheterization. Indian Pediatr Case Rep [serial online] 2023 [cited 2023 Sep 26];3:167-70. Available from: http://www.ipcares.org/text.asp?2023/3/3/167/383630 |
Newborns, especially preterm babies, admitted to neonatal intensive care units frequently need long-term central venous access for providing total parenteral nutrition (TPN), administration of drugs and blood products, or exchange transfusion.[1] The umbilical venous route is accessible and most preferred for this purpose, thus making umbilical venous catheterization (UVC) a common procedure in such neonates. However, it can cause vascular endothelial damage and disrupt the blood flow, leading to thrombosis.[2] Retrospective studies as well as autopsy findings have reported the prevalence of UVC-related portal vein thrombosis (PVT) ranging from 1.3% to 43% depending on the duration of placement, position of the tip of the catheter, method of insertion of the catheter and other factors.[2],[3] A PVT may remain asymptomatic in the neonatal period but later may lead to serious complications like portal hypertension, which is often diagnosed incidentally by ultrasonography (USG) done for some other indications.[4],[5]
We report an unusually early presentation of portal hypertension in a young infant, due to an unsuspected underlying extensive PVT preceded by umbilical vein catheterisation.
| Clinical Description | |  |
A 28-day-old male neonate, the first issue of a nonconsanguineously married couple, born via lower segment caesarian section, in view of nonprogression of labor, at 38 weeks of gestation, with a birth weight of 2.9 kg (appropriate for gestational age) with an uneventful antenatal period, presented to us with fever, pus discharge from the umbilicus, abdominal distension, and abscess formation at multiple sites for the past 15 days.
His postnatal history revealed deep progressive jaundice developing since the 4th day of life, for which medical opinion was sought elsewhere. Investigations had revealed maximum serum bilirubin of 51.8 mg/dL (total), with an unconjugated fraction being 49 mg/dL. The baby had a blood group of B+ and mother's blood group was AB+. On further investigations, the baby was diagnosed to have Glucose-6-phosphate dehydrogenase deficiency (G6PD), with enzyme levels of 0.49 U/gHb (ref 7–20.5 U/gHb), for which the baby was hospitalized for 4 days. He was treated with double surface phototherapy and the baby underwent double volume exchange transfusion (DVET) through UVC. Following the procedure, the total bilirubin levels dropped to 19.9 mg/dL and gradually reduced out of phototherapy range to 10.2 mg/dL. The baby had no other medical illness since then and was exclusively breastfed and gaining weight.
At the time of presentation to our institute, the infant was found to be febrile, with a temperature of 101°F, heart rate of 160/min, well-felt pulses with capillary refill time <2 s, and respiratory rate of 65/min. He had a weight of 3.65 kg (weight/age Z-score of 0), length of 57 cm (length for age Z-score of +3), and a head circumference of 36 cm (50th percentile).
Local examination revealed two discrete rounded swellings of diameter 4 cm × 4 cm and 4 cm × 2 cm present on the right shoulder and left ankle, respectively, which were erythematous, warm and tender to touch but there was no restriction of joint movement. On systemic examination, the abdomen was found to be diffusely distended, but soft, with dilated veins over the abdomen filling from below to upward. Umbilicus was erythematous, everted, with periumbilical edema with discharging pus from umbilicus. The liver was firm, palpable 3 cm below right costal margin in midclavicular line, with a total span of 9 cm with discrete margins. The spleen was palpable 2.5 cm below the left costal margin in the splenic axis. Respiratory, cardiac, and neurological examinations were unremarkable.
| Management and Outcome | | |
The baby was investigated for septic screen and pus culture was also sent. The reports showed hemoglobin of 11.4 g/dL, total leukocyte count of 18,000 cells/mm3, with neutrophils 60%, lymphocytes 35%, and platelet count of 373 × 109 cells/L. The C-reactive protein was raised 100.4 mg/dL, alanine transaminase 10 IU/L, aspartate transaminase 24 IU/L, alkaline phosphatase 170 U/L, serum albumin 2.60 mg/dL, total protein 6.22 mg/dL, gamma-glutamyl transferase (GGT) 341 IU/L, prothrombin time 12.8, international normalized ratio– 0.89 with normal renal functions (urea 12 mg/dL and creatinine 0.11 mg/dL). The pus aspirated from the abscesses, showed the growth of Methicillin-resistant Staphylococcus aureus (MRSA) sensitive to linezolid, gentamicin, vancomycin, and cotrimoxazole.
Initially, the infant was started on empirical therapy with intravenous amoxicillin + clavulanic acid, with incision and drainage of abscesses, followed by regular dressing. The infant was accepting breastfeeds well, so no intravenous fluids were required. After obtaining pus culture reports, the antibiotics were changed to intravenous vancomycin.
The baby was further worked up for deep-seated abscesses. USG of abdomen showed multiple intrahepatic focal hypoechoic lesions of varying sizes in both the lobes of the liver suggestive of multiple liver abscesses along with an unexpected incidental finding of portal vein prominence with intraluminal partial thrombosis. The repeat USG abdomen done after a week on the 37th day of life showed persistence of the same findings. In addition, it showed partial thrombosis of the left and right branch of the portal vein, along with a portal cavernoma. Contrast-enhanced computed tomography of the abdomen showed complete thrombosis of the left umbilical vein and the portal venous system with portal cavernoma. There was complete thrombosis of the left portal vein, right portal vein, and main portal vein. The splenic vein and the superior mesenteric vein were patent [Figure 1]a, [Figure 1]b, [Figure 1]c. Upper gastrointestinal (UGI) endoscopy was done on the 72nd day of life, which showed long two columns of Grade II varices in esophagus, suggestive of the development of portal hypertension [Figure 2]. | Figure 1: (a) CECT coronal plane shows portal vein is replaced by multiple collaterals as depicted by yellow arrows. (b) CECT axial plane showing hypodense filling defect in right and left portal vein branches shown by yellow arrows. (c) CECT axial plane showing necrotic lesion in posterosuperior segments of the right lobe of liver suggestive of liver abscess shown by yellow arrows
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 | Figure 2: UGI endoscopy showing grade 2 esophageal varices (white arrows)
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Thus, the final diagnosis of the infant's condition was MRSA septicemia with multiple skin, umbilical and deep-seated abscesses, associated with extensive PVT and portal hypertension with Grade II esophageal varices, following umbilical vein catheterization for DVET due to G6PD deficiency induced hyperbilirubinemia in the neonatal period.
The infant was started on a therapeutic dose of subcutaneous enoxaparin (1.5 mg/kg/dose q 12 hourly) and was planned to continue enoxaparin for 3 months. The infant improved clinically. There was no further fever since the 2nd day of admission and the infant continued to feed well with normal reflexes. The umbilical discharge subsided within 1 week. The shoulder and ankle abscesses healed with regular dressing over 2–3 weeks. The follow-up investigations showed hemoglobin of 9.1 g/dL, total leukocyte count of 12,400 cells/mm3, with neutrophils 35%, lymphocytes 60%, and platelet count of 314 × 109 cells/L. The C-reactive protein decreased to 14.5 mg/dL, alanine transaminase 41 IU/L, aspartate transaminase – 24 IU/L, alkaline phosphatase 225 U/L, serum albumin 3.86 mg/dL, total protein 6.23 mg/dL, and GGT 64 IU/L, with normal renal functions (urea 16 mg/dL, creatinine 0.18 mg/dL). Vancomycin was administered for a total of 28 days, followed by oral linezolid for another 14 days. Enoxaparin was continued and the last follow up done at 4.5 months of age, the infant was asymptomatic, thriving well with the repeat USG reporting difficulty in identifying the main portal vein, which was replaced by a tortuous patent cavernoma, with bilateral portal vein branches being partially obliterated [Figure 3]a and [Figure 3]b. | Figure 3: (a and b) Follow-up ultrasound and color Doppler at 4.5 months of age (3 months' post enoxaparin) showing multiple portal cavernomas
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| Discussion | | |
The case described above depicts a remarkable picture of an unsuspected extensive PVT, culminating in portal hypertension with the formation of Grade II varices, at a very early age, within 2 months of life, the infant being hitherto asymptomatic. The diagnosis of PVT was incidental while looking for the presence of deep-seated abscesses in USG, in the infant who had presented with skin and soft-tissue abscesses.
Neonatal PVT is rare but has been increasing over the years with increasing intensive care stays and advancement in interventional procedures. Literature shows a neonatal PVT rate of 36/1000 neonatal intensive care admissions.[3] Newborns are generally asymptomatic, and diagnosis is made incidentally by ultrasound.[6]
The umbilical vein is patent and accessible during the early neonatal period and a good choice for central line catheterization.[5] Umbilical vein catheterization for purposes such as DVET or TPN is one of the most frequently attributed risk factors for the development of neonatal PVT, others being sepsis, especially umbilical sepsis, and thrombophilia. There are several mechanisms by which umbilical venous catheters may cause thrombosis, including damage to vessel walls, disrupted blood flow, the infusion of substances such as elements of TPN, drugs, and thrombogenic catheter materials.
However, it has been seen that the majority of PVT developing in the neonatal period, recover spontaneously and do not develop further complications such as portal hypertension.[7] The occluded thrombus, if does not resolve, leads to the formation of many small periportal collaterals around it, to supply blood to the liver, known as cavernous transformation of the portal vein.
In a retrospective study of 133 (128 neonates), infants with PVT, Morag et al.,[3] found that the median age of diagnosis was 7 days, with a history of UVC insertion in 73%, of which 46% had insertion in an appropriate position. They found that 27% of infants had a poor outcome (portal hypertension or lobar atrophy), which was significantly associated with an initial diagnosis of Grade III PVT and a low or intra-hepatically placed UVC. Further, treatment with anticoagulation did not benefit outcome. Other studies also suggest that the duration of UVC use and the location of the catheter tip correlate with the incidence of PVT.[8] In our case, there was extensive PVT involving the entire main portal vein and both branches by the time it was detected by us at just 6 weeks of age, implying that the process must have started much earlier.
Other factors which potentiate the formation of PVT in neonates with UVC include low birth weight, hypercoagulability, hypoxia, infection, sepsis, congenital malformations for which two etiopathogenic theories have been proposed: (a) congenital agenesis of the portal vein leading to the development of periportal collateral vessels and (b) hemangioma of the portal vein,[9] and gestational diabetes mellitus.[3] In our case, the baby had a history of UVC insertion in the immediate postnatal period as well as umbilical sepsis at 1 month of age progressing to systemic sepsis, both contributing to such an extensive PVT and further development of esophageal varices and portal hypertension at such an early age.
The best method for the treatment of neonatal PVT is not clear. Of the 74 neonates with PVT, studied by Bhatt et al.,[4] only 26% received anticoagulant therapy while 74% remained untreated. After a mean follow-up duration of 16.6 months, USG showed complete thrombus resolution in 63% of treated versus 58% of nontreated neonates and partial resolution in 15% versus 11% of untreated neonates. Irrespective of the status of thrombus resolution, majority (96%) had no complications.
It is generally recommended that an asymptomatic infant with incidentally detected PVT should be kept under close surveillance with serial ultrasound.[10] If the thrombus shows signs of extension or occlusion of the portal venous tract, or clinical deterioration, antithrombotic therapy with unfractionated or LMWH and thrombolytic therapy with recombinant tissue plasminogen activator or urokinase may be considered.[11] We also started our patient on LMWH and planned it for at least 3 months, with routine USG follow-up. Intravenous streptokinase has been rarely used.[12]
Long-term complications of neonatal PVT include liver lobe atrophy, portal hypertension, esophageal varices and gastrointestinal bleeding, portal collaterals, and portal cavernomas.[6] The most dreaded complication of PVT in long-term is portal hypertension. However, whether early intervention and treatment can alter morbidities in later childhood, is doubtful. Morag et al.,[13] in a retrospective study found that among 70 patients, who had PVT as neonates, 28% had asymptomatic left lobar atrophy in childhood, 7% had slowly progressive splenomegaly and 3% required shunting because of the progression of portal hypertension. In our case, after the sepsis had been treated, repeated serial USG showed the formation of portal cavernoma and by the 72nd day of life, the infant showed the presence of esophageal varices on UGI endoscopy, indicating the development of portal hypertension. The development of portal hypertension at such an early age is hardly reported.
Our case highlights how the catastrophic complications such as obliteration of the entire main portal vein and its branches, formation of portal cavernoma, portal hypertension, and esophageal varices, can develop silently, at a very early age, in an infant with a history of UVC in the immediate postnatal period. The placement of UVC should be done sparingly in neonates, only after thoroughly assessing the need for UVC and possible alternatives. The position of the catheter tip should be confirmed by USG and the catheter should be kept for as minimum duration as possible. The baby should be followed up with serial USGs after the placement of the UVC catheter and if PVT is demonstrated, close surveillance or antithrombotic therapy should be considered to prevent or early detect long-term complications.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
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