Infantile hepatic hemangioma has substantial arteriovenous shunting which may lead to cardiovascular compromise and hydrops fetalis. It may present as hepatomegaly since the entire liver is involved in most cases. As mortality is very high, a high index of suspicion is required to make a diagnosis and common complications arising out it, especially in the presence of cutaneous hemangioma. We present a 2-month-old baby born at term presented with features suggestive of sepsis with multiple cutaneous hemangiomas, and on evaluation, there was congestive cardiac failure, which was initially thought of cardiac origin but subsequently came out to be arteriovenous shunting of blood in liver

Dhingra, Arti

Goel, Anil K

Shah, Seema

English

Atharva Scientific Publications (E-Jounals)

Vol 5 | Issue 7 | July 2018 Indian J Child Health 507
Case Report
Diffuse neonatal hemangiomatosis presenting as congestive cardiac failure - A 
case report
Arti Dhingra1, Anil K Goel2, Seema Shah3
From 1Associate Professor, Department of Paediatrics, SHKM Government medical college, Nuh, Haryana, 2Additional Professor and Head, 
Department of Paediatrics, AIIMS, Raipur, Chhattisgarh, 3Associate Professor, Department of Biochemistry, SSPGPGTI, Noida, Uttar Pradesh, India
Correspondence to: Dr. Anil K Goel, Additional Professor and Head, Department of Paediatrics, AIIMS, Raipur, Chhattisgarh, India. 
E-mail: akgoel@aiimsraipur.edu.in
Received – 16 May 2018 Initial Review – 05 June 2018 Accepted – 26 June 2018
Congestive cardiac failure in late neonatal period, the usual practice is to evaluate for heart disease and related hyperdynamic state, leading to decompensation. Although 
diffuse hepatic hemangiomatosis (DHH) is a rare disorder, an 
uncommon disease in the neonate can present from skin lesion 
to grave complications. In our case, the infant presented with 
unexplained cardiac failure, which was difficult to explain in a 
setting of the absence of other features. A high index of suspicion 
is required in such multiple and midline hemangiomas, subjecting 
to search for internal organ involvement and association of 
high-output heart failure which can result in early intervention 
and decrease morbidity because, if left untreated or delayed 
recognition can result in high mortality.
CASE REPORT
A 2 months female infant came to pediatric emergency with 
complaints of excessive crying and poor feeding for 9 days 
and cough and rapid breathing for 4 days duration. There was 
no history of fever, vomiting, jaundice, cyanosis, or symptoms 
suggestive of urosepsis. The baby was born to mother G1P1, at 
37 weeks 3 days gestation by assisted vaginal delivery with breech 
presentation in hospital. The baby was exclusively breastfed since 
birth. The baby had multiple cutaneous hemangiomas all over 
the body including face, back, lower limbs, and abdomen. They 
were 20–30 in number, size ranging from pinpoint to 2.5 cm in 
diameter, largest being on the right thigh (Fig. 1). Mother also 
noticed increase in number of hemangioma since birth. The 
baby was completely asymptomatic till the present episode. On 
admission, examination reveals that the baby had respiratory 
distress with features suggestive of congestive cardiac failure 
in the form of tachypnea (RR–60/min), tachycardia (165/min), 
gallop rhythm and tender hepatomegaly, liver 6.5 cm below the 
right costal margin (liver span 9 cm), capillary refill time was 2 s, 
and SpO2 94–96% in room air. Clinically, no added sound heard 
in precordium except gallop rhythm.
In view of multiple cutaneous hemangiomas, possibility of 
visceral hemangioma especially hemangioma of liver was kept, 
which might have predisposed the patient to congenital cervical 
fusion. Ultrasonography abdomen and contrast-enhanced 
computed tomography (CECT) abdomen showed multiple 
hypoattenuating liver masses in both lobes of liver showing 
enhancement paralleling the vascular pool suggestive of multiple 
hemangiomas in liver, maximum diameter being 3.1 cm in 
both lobes of liver (Fig. 2). Other investigations (CECT chest 
and magnetic resonance imaging brain, X-ray of lumbosacral 
region) and ophthalmologic examination were done to rule out 
the presence of hemangioma(s) in other organs, but we could 
not find any such lesion in the specified area under scanner. 
Echocardiography was also normal which ruled out any structural 
malformation of the heart.
Pediatrics surgery opinion was taken and the baby was put 
on decongestive measures in the form of furosemide (2 mg/
day), digoxin (40 mics/kg), and prednisolone (1 mg/kg), and 
propranolol (2 mg/kg) in conventional doses along with other 
supportive treatments. The patient improved clinically as regard 
to decrease in respiratory distress and liver size. Attendants 
requested discharge on request on the 7th day because of personal 
problems, and the baby was discharged on prednisolone, 
propranolol, diuretics, and digoxin orally on standard 
ABSTRACT
Infantile hepatic hemangioma has substantial arteriovenous shunting which may lead to cardiovascular compromise and hydrops 
fetalis. It may present as hepatomegaly since the entire liver is involved in most cases. As mortality is very high, a high index 
of suspicion is required to make a diagnosis and common complications arising out it, especially in the presence of cutaneous 
hemangioma. We present a 2-month-old baby born at term presented with features suggestive of sepsis with multiple cutaneous 
hemangiomas, and on evaluation, there was congestive cardiac failure, which was initially thought of cardiac origin but subsequently 
came out to be arteriovenous shunting of blood in liver.
Key words: Diffuse hepatic hemangiomatosis, Hemangioma, Hydrops fetalis
Dhingra and Goel Unusual presentation of diffuse neonatal hemangiomatosis
Vol 5 | Issue 7 | July 2018 Indian J Child Health 508
recommended doses. Mother was advised to come for follow-up 
ultrasound of liver to look for decrease in size of hemangioma 
and hepatic embolization if no response could be observed. 
The patient was lost to follow-up. The baby died at home after 
10 days of discharge as informed by attendants telephonically 
when they were called up for follow-up.
DISCUSSION
DHH is not a common disorder in the neonate, characterized by 
extensive replacement of liver parenchyma with hemangiomatous 
lesions [1]. It is frequently associated with a palpable abdominal 
mass and high-output heart failure, with high mortality rate in 
neonates. Diffuse neonatal hemangiomatosis (DNH) is a fatal 
disease characterized by multiple hemangiomas affecting the 
skin and visceral organs, although malignant transformation is 
rare. Typically, these hemangiomas have their onset at birth or 
during the 1st month of life [2]. Liver hemangioma is the most 
common hepatic vascular anomalies in infancy accounting for 
90% of these lesions. Multiple numbers may be found in 10-20% 
cases [3]. Horii et al. found in a prospective multicentric 
study involving 201 patients with cutaneous hemangiomas an 
incidence of 16% of hepatic lesions in infants with five or more 
cutaneous infantile hemangiomas versus 0% in those with fewer 
than five [4]. In another study by Dickie et al. in 26 patients 
with hepatic hemangioma found that none was having none, 
single, or three cutaneous hemangiomas. That is the reason 
screening is important in patient with any number of cutaneous 
hemangioma that presents with hepatomegaly with an abdominal 
ultrasonography [5].
It is very important to differentiate this from the more common 
multiple cutaneous hemangiomatosis in which internal organs 
are spared [6]. The liver is the most common extracutaneous site 
(64%), but virtually any organ may be affected including brain 
(52%), gastrointestinal (GI) tract (52%), lungs (52%), and eyes [7]. 
Like isolated hemangioma, DNH proliferates, and then involutes, 
but extracutaneous involvement may lead to life-threatening 
complications. Screening with abdominal ultrasonography and 
other radio imaging is often recommended in patients with more 
than five cutaneous hemangiomas. These infants carry a greater 
frequency of hepatic hemangioma in comparison with those with 
fewer than five cutaneous hemangiomas [4].
Death usually occurs within the first 10 weeks of life as 
a result of AV shunting in the liver causing increased cardiac 
output and congestive heart failure as occurred in our case [2]. 
In addition, other complications include hemorrhage, obstructive 
jaundice, and consumptive coagulopathy [8]. Without any 
treatment, the mortality rate is as high as 77%, but with 
appropriate treatment, the mortality can be reduced to 27% [8]. 
Diffuse liver hemangiomas, which may occur in the absence 
of skin hemangiomas, causes massive liver enlargement with 
compartment syndrome of abdomen, impaired ventilation, 
decreased venous return, and resulting in higher mortality [9]. 
Kasabach–Merritt phenomenon is again a condition associated 
with cutaneous vascular tumor with thrombocytopenia and/or 
coagulopathy. This condition is more commonly associated with 
Kaposiform hemangioendothelioma and tufted angioma and truly 
not associated with infantile hemangioma [10]. However, diffuse 
hepatic hemangioma can develop even in patients with single 
cutaneous hemangioma [11]. This highlights the importance of 
physical examination and screening ultrasound in these patients.
Emergent treatment is required in such condition as otherwise 
disease is fatal. Multiple treatment modalities are available and 
corticosteroids are the mainstay of treatment, especially in life-
threatening conditions. Corticosteroid stops the proliferation 
of blood vessels and induces early involution of hemangiomas. 
Usual dose is 2–3 mg/kg/day which can be increased to 
5 mg/kg/day, although associated with more adverse effects [12]. 
Other medical therapies include interferon 2b alpha, vincristine/
vinblastine, and cyclophosphamide can be given, which works 
by inhibiting angiogenesis but used rarely due to adverse effects.
A non-selective beta blocker, propranolol (1–3 mg/kg/day) 
has been successfully used for cutaneous hemangioma, has also 
shown good results in symptomatic hepatic hemangioma. Exact 
mechanism of action of propranolol is unknown, probably acts 
by decreasing renin production and causing decreased vascular 
endothelial growth factor production and also possibly related to 
vasoconstriction [7,13]. A recent meta-analysis done by Lou et 
al., in 2013, involving 324 infantile hemangiomas showed that the 
efficacy of propranolol was greater than other therapies such as 
steroids, interferon, and vincristine in treating this condition with 
less severe side effects [14]. The striking effect of propranolol 
Figure 1: Child with diffuse hemangiomatosis
Figure 2: Contrast-enhanced computed tomography abdomen 
showing multiple hepatic hemangiomas
Dhingra and Goel Unusual presentation of diffuse neonatal hemangiomatosis
Vol 5 | Issue 7 | July 2018 Indian J Child Health 509
on infantile hemangioma can be attributed to three molecular 
mechanisms: Vasoconstriction, inhibition of angiogenesis, and 
induction of apoptosis [15]. Hypotension, bradycardia, and 
hypoglycemia are well-recognized side effects of propranolol 
and need to be monitored closely. In our patient, propranolol 
treatment was well tolerated and was associated with dramatic 
improvement in the cardiac function. Hepatic embolization or 
surgical resection is recommended for focal lesions with direct 
shunt as a means of controlling heart failure refractory to medical 
treatment [16].
In our case, the patient had multiple hepatic hemangiomas in 
addition to cutaneous hemangioma. Most hepatic hemangiomas 
are asymptomatic and can become symptomatic with 
(1) high-output heart failure due to AV shunting, (2) associated 
hypothyroidism leading to overproduction of iodothyronine 
deiodinase, and (3) Kasabach merit syndrome presenting with 
coagulopathy and thrombocytopenia. Our patient had high-output 
cardiac failure for which furosemide, digoxin, and propranolol 
were started in addition to prednisolone. The patient did not come 
for follow-up and we could have considered upgrading medical 
therapy, hepatic artery embolization, or surgical resection in view 
of poor response to medical therapy.
CONCLUSION
Diffuse hemangiomatosis of liver with coexisting congestive heart 
failure, an evidence of AV shunting of blood without extrahepatic 
involvement, is not uncommon in infancy. Health awareness of 
the existence of this uncommon entity and supported by screening 
radio imaging in every case of any number of cutaneous 
hemangioma can help reach an accurate and early diagnosis, 
and therefore, early management of these patients can reduce 
morbidity to a large extent.
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Funding: None; Conflict of Interest: None Stated.
How to cite this article: Dhingra A, Goel AK, Shah S. Diffuse neonatal 
hemangiomatosis presenting as congestive cardiac failure - A case report. 
Indian J Child Health. 2018; 5(7):507-509.


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