
==== Front
Cureus
Cureus
2168-8184
Cureus
2168-8184
Cureus Palo Alto (CA)

10.7759/cureus.66060
Gastroenterology
Pediatrics
Radiology
Portal Hypertension in Children: Investigating Umbilical Catheterization in the Neonatal Period
Muacevic Alexander
Adler John R
Bouhafs Nadia 1
Hamami Amal 1
Elouali Aziza 1
Babakhouya Abdeladim 1
Rkain Maria 12
1 Department of Pediatrics, Faculty of Medicine and Pharmacy of Oujda, Mohammed VI University Hospital Oujda, Oujda, MAR
2 Pediatric Gastroenterology, Centre Hospitalier Universitaire Mohammed VI Oujda, Oujda, MAR
Nadia Bouhafs bouhafsnadia4@gmail.com
3 8 2024
8 2024
16 8 e660603 8 2024
Copyright © 2024, Bouhafs et al.
2024
Bouhafs et al.
https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
This article is available from https://www.cureus.com/articles/276247-portal-hypertension-in-children-investigating-umbilical-catheterization-in-the-neonatal-period
Portal cavernoma is a major cause of extrahepatic portal hypertension (EHPH) in children. It is a serious condition, due to the frequency and severity of digestive hemorrhages secondary to the rupture of esophageal varices (EV). Neonatal umbilical catheterization is a significant risk factor for the development of portal vein thrombosis (PVT) and portal hypertension. We report a case of a five-year-old male who presented with upper gastrointestinal (GI) bleeding on ruptured esophageal varices resulting from a portal cavernoma, complicating neonatal umbilical vein catheterization. This case illustrates the risk of severe vascular complications, particularly portal hypertension that can result from neonatal umbilical vein catheterization.

umbilical catheterization
portal cavernoma in children
portal vein thrombosis
esophageal varices
hematemesis
==== Body
pmcIntroduction

Portal cavernoma (synonymous with portal vein thrombosis, PVT) comprises a network of veins whose caliber, initially millimetric or microscopic, is increased, thereby facilitating the flow of hepatopetal portal blood. This condition results from a natural compensatory mechanism that is triggered when the portal vein becomes obstructed. It is the consequence of thrombotic and chronic occlusion of the extrahepatic portal system [1]. Although the exact causes of PVT in children remain unknown, several risk factors have been identified and classified into three main groups: general causes (procoagulant state), local factors (abdominal infection, omphalitis, umbilical catheter), and vascular malformations [2]. Umbilical venous catheterization itself represents a risk factor for the development of PVT. In this report, we discuss a case of portal thrombosis in a five-year-old male due to umbilical catheterization, which was revealed at a later age. By sharing this clinical experience, we aim to raise awareness among healthcare professionals about the risks associated with umbilical catheterization and to promote optimized clinical practices to minimize these risks.

Case presentation

A five-year-old male presented to the pediatric emergency department two days after an episode of hematemesis, during which he had vomited an estimated 500 ml of blood. He had been born at a gestational age of 39 weeks with a birth weight of 3000 g following an uncomplicated pregnancy. His medical history revealed no abnormalities other than recurrent abdominal pain. The patient appeared pale on physical examination with discolored conjunctivae and tachycardia at 122 bpm; the other vital signs were normal. He had a palpable splenomegaly 2 cm below the rib cage, without hepatomegaly. There were no signs of chronic liver disease. The initial blood work showed severe anemia with thrombocytopenia (hemoglobin: 5.4 g/dl, normochromic normocytic, platelets: 118,000/µl, normal blood smear) and a prothrombin level of 62%. Liver function tests and coagulation profiles were normal (Table 1).

Table 1 Results of biological tests at admission

ALT: alanine aminotransferase; AST: aspartate aminotransferase; GGT: gamma-glutamyl transferase; MCHC: mean corpuscular hemoglobin concentration; MCV: mean corpuscular volume

Parameters	Results	References range	
Hemoglobin (g/dl)	 5.4	9.5-13.5	
MCV (fL)	83	80-100	
MCHC (g/dL)	32	32-36	
White blood cell count (E/mm3)	3170	5000-10,000	
Platelets (E/mm3)	118,000	150,000-400,000	
Prothrombin level (%)	62	70-120	
AST (IU/L)	29	8-30	
ALT (IU/L)	18	8-35	
GGT (IU/l)	11	10-71	

An esophagogastroduodenoscopy revealed multiple stage 3 esophageal and fundic varices, with no active bleeding. CT angiography showed a portal cavernoma with signs of portal hypertension (Figure 1).

Figure 1 CT angiography images in axial (A), parasagittal (B), and coronal (C) sections showing a portal cavernoma with portosystemic shunts

CT: computed tomography

Initially, the origin of the portal cavernoma was unclear. The patient's history did not indicate a predisposition to thrombosis; the thrombophilia assessment showed normal results and no vascular malformations. However, neonatal records revealed an admission to the neonatal ICU for neonatal asphyxia due to maternal-fetal infection and unconjugated hyperbilirubinemia due to rhesus incompatibility, complicated by nosocomial septicemia for which he had spent 28 days in the neonatal. An umbilical catheter had been inserted for transfusion and treatment for over 10 days. No complications had been reported in the following years. Our patient was treated with Sandostatin and beta-blockers and underwent three sessions of esophageal variceal ligation (Figure 2) with favorable initial progress while awaiting surgery.

Figure 2 Endoscopic images showing esophageal varices in the lower third of the esophagus before (A) and after (B) endoscopic ligation

Discussion

Portal cavernoma was first described in 1903 during the autopsy of a 44-year-old patient who died due to extensive mesenteric venous thrombosis [3]. In children, portal cavernoma is a major cause of "pre-hepatic" portal hypertension. It is a grave condition given the high rate and severity of digestive bleeding due to the rupture of esophageal varices (EV). In the United States, its incidence is estimated at 1% in the general population while a study in Morocco reported an incidence of about 1.45% [1]. Umbilical vein catheterization is a major risk factor for the development of portal cavernoma in children, with a rising prevalence (~60-70%) due to the procedure's widespread use in neonatal care [2]. A systematic review of the literature has revealed the considerable risk of PVT related to umbilical catheterization [4]. The combined average rate of neonatal PVT due to umbilical vein catheters (UVC) across studies was found to be 12% (range: 0-49%). However, a more recent multicenter survey including 187 children diagnosed with PVT (average age at diagnosis: four years) reported a history of neonatal UVC placement in 65% of cases [5].

Scientific literature highlights that UVC-related PVT is mainly associated with improper catheter tip placement [4]. The UVC should be centrally positioned, ideally at the junction between the inferior vena cava and the right atrium. Indeed, if the catheter tip is too low, it could be responsible for necrotizing enterocolitis, colon perforation, liver abscess, and PVT [6]. Therefore, careful evaluation of the UVC tip position is necessary to reduce catheter-related complications. A recent study found that an ultrasound assessment of UVC's position helped identify catheter position in 100% of cases compared with radiographic assessment [7]. Prospective studies have concluded that properly inserted UVCs do not cause PVT [4].

Besides improper catheter placement, which is the primary risk factor, several other factors can increase the risk of PVT in catheterized newborns. These factors include sepsis, trauma during insertion, transfusions, infusion of hypertonic solution [8-9], and solutions with significantly elevated serum calcium concentrations [10]. UVC-related PVT can sometimes resolve spontaneously. Kim et al.'s study involving 100 newborns found that 43% had asymptomatic PVT, with complete resolution in 56% during follow-up. The majority of cases remain unrecognized and are discovered later between the ages of 6-10 years, as in our patient [8].

Clinical signs of portal cavernoma are related to extrahepatic portal hypertension (EHPH). Gastrointestinal (GI) hemorrhages and splenomegaly are the most common clinical manifestations. Around 50% of children initially present with GI bleeding. Splenomegaly is found in over 90% of children with PVT, and ascites may be present in 8.3% of cases [11,12]. Growth retardation exists in 50% of the cases. The decreased hepatic portal flow and/or resistance to growth hormone have been proposed as pathophysiological hypotheses to explain this scenario [13]. Our patient had normal weight and height. Biologically, splenomegaly is accompanied by hypersplenism [11]. Chronic anemia is found in almost all cases, and thrombocytopenia and decreased prothrombin time (PT) are secondary to overconsumption by a mechanism of disseminated intravascular coagulation (DIC) within the capillaries forming the cavernoma. Liver function tests are generally unchanged. Abdominal Doppler ultrasound is the most commonly used diagnostic test, with a sensitivity of over 90%. Upper GI endoscopy must be performed in all cases, as it can reveal the presence of esophagogastric varices in approximately 84% of cases [11,14].

None of the studies in the literature encourage the use of beta-blockers in children to prevent digestive hemorrhage [15]. On the other hand, the effectiveness of primary prophylactic endoscopic treatment has been demonstrated [16]. Porto-systemic shunting is indicated if hemorrhagic complications persist despite endoscopic treatment, or in cases of symptomatic cholangiopathy. Mesenterico-Rex shunting or portal reperfusion, a more physiological technique, has shown promising results in the curative treatment of portal cavernomas [17].

Prevention of portal thrombosis related to umbilical catheterization in newborns is crucial. This involves correct catheter insertion, regular assessment of catheter position every 48 hours, close monitoring of newborns, especially those with additional risk factors, and minimizing the duration of catheter use (seven days maximum). If long-term central venous access (>4 days) is necessary, replacing the umbilical venous catheter with a percutaneous central venous catheter (PICC) or using ultrasound-guided central venous access may be beneficial. Manual liver retraction during the placement of an umbilical venous catheter improves the positioning rate of the UVC tip in the inferior vena cava. This maneuver is quick, easy to implement, and not associated with any adverse effects [18].

Conclusions

This case report illustrates portal hypertension as a late complication of invasive neonatal procedures. PVT secondary to umbilical catheterization is a significant complication requiring sharp vigilance. Raising awareness among healthcare professionals about the risks associated with umbilical catheterization and closely monitoring children who have undergone this procedure is essential for optimal management and prevention of thrombotic complications.  

Disclosures

Author Contributions

Human subjects: Consent was obtained or waived by all participants in this study.

Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following:

Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work.

Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work.

Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Concept and design:  Nadia Bouhafs, Amal Hamami, Abdeladim Babakhouya, Maria Rkain

Acquisition, analysis, or interpretation of data:  Nadia Bouhafs, Amal Hamami, Aziza Elouali, Abdeladim Babakhouya, Maria Rkain

Drafting of the manuscript:  Nadia Bouhafs, Aziza Elouali, Abdeladim Babakhouya

Critical review of the manuscript for important intellectual content:  Nadia Bouhafs, Amal Hamami, Aziza Elouali, Abdeladim Babakhouya, Maria Rkain

Supervision:  Nadia Bouhafs, Amal Hamami, Aziza Elouali, Abdeladim Babakhouya, Maria Rkain
==== Refs
References

1 Cavernous transformation of the portal vein in children: about 11 cases (Article in French) Pan Afr Med J Ilham T Mounia LI Moustapha H 277 19 2014 25870732
2 Portal cavernoma in children - complications and evolution Medichub Borcău DA Grama A Căinap S 4 6 6 2022
3 Portal cavenorma: diagnosis, aetiologies and consequences (Article in French) Ann Chir Vibert E Azoulay D Castaing D Bismuth H 745 750 127 2002 12538094
4 Incidence of umbilical vein catheter-associated thrombosis of the portal system: a systematic review and meta-analysis World J Hepatol Bersani I Piersigilli F Iacona G 1802 1815 13 2021 34904047
5 Etiology, presenting features and outcome of children with non-cirrhotic portal vein thrombosis: a multicentre national study Dig Liver Dis Di Giorgio A De Angelis P Cheli M 1179 1184 51 2019 30928422
6 Central versus low-lying umbilical venous catheters: a multicenter study of practices and complications Am J Perinatol El Ters N Claassen C Lancaster T 1198 1204 36 2019 30566998
7 Sonography for complete evaluation of neonatal intensive care unit central support devices: a pilot study J Ultrasound Med Saul D Ajayi S Schutzman DL Horrow MM 1465 1473 35 2016 27229130
8 Does umbilical vein catheterization lead to portal venous thrombosis? Prospective US evaluation in 100 neonates Radiology Kim JH Lee YS Kim SH Lee SK Lim MK Kim HS 645 650 219 2001 11376248
9 Neonatal portal vein thrombosis: diagnosis and management Semin Fetal Neonatal Med Williams S Chan AK 329 339 16 2011 21925985
10 Incidence and risk factors of subclinical umbilical catheter-related thrombosis in neonates Thromb Res Hwang JH Chung ML Lim YJ 21 25 194 2020 32563060
11 Extrahepatic portal vein thrombosis, an important cause of portal hypertension in children J Clin Med Grama A Pîrvan A Sîrbe C 42 44 10 2021
12 Extrahepatic portal vein obstruction in the pediatric age: a medical challenge J Clin Gastroenterol Treat Khodayar-Pardo P 32 34 2 2016
13 Portal-vein obstruction in children leads to growth retardation Hepatology Sarin SK Bansal A Sasan S Nigam A 229 233 15 1992 1735525
14 Portal vein thrombosis in children and adolescents: 20 years experience of a pediatric hepatology reference center Arq Gastroenterol Ferri PM Ferreira AR Fagundes ED Liu SM Roquete ML Penna FJ 69 76 49 2012 22481689
15 Guidelines for the diagnosis and treatment of extrahepatic portal vein obstruction (EHPVO) in children Ann Hepatol Flores-Calderón J Morán-Villota S Rouassant SH 0 12 Suppl 1 2013
16 Portal hypertension in children: high-risk varices, primary prophylaxis and consequences of bleeding J Hepatol Duché M Ducot B Ackermann O Guérin F Jacquemin E Bernard O 320 327 66 2017 27663417
17 Experience with alternate sources of venous inflow in the meso-Rex bypass operation: the coronary and splenic veins J Pediatr Surg Chiu B Pillai SB Sandler AD Superina RA 1199 1202 42 2007 17618880
18 Increased success rate in umbilical venous catheter positioning by posterior liver mobilization (Article in French) Arch Pediatr Pennaforte T Klosowski S Alexandre C Ghesquière J Rakza T Storme L 1440 1444 17 2010 20430598
