Dr Anakha V Ajay ,Dr Naveen Viswanath(Dept of Pediatric surgery) Dr Sajith Kesavan Dr Greeshma Issac (Dept of pediatric intensive care) DrCJayakumar AIMS ,KOCHI
Three month old 38 week old ba with birth weight of 2.8kg, exclusively breast fed presented with abdominal distension and decreased urine output since two days. Mother also noticed that child had difficulty in feeding and poor activity since 2 days.Child was noted to have on and off abdominal distension which they thought to be gaseous was managed conservatively.
As the symptom persisted USG Abdomen done showed large liver lesion with multiple prominent intraluminal vascular channels with variation in mid and proximal aorta
No history of jaundice or pale coloured stools. History of mother taking herbal medications present 2 days back.At presentation child was alert, conscious with stable vitals. No facial dysmorphism. No neurocutaneous markers or cutaneous haemangiomas noted, Pallor present. Auxology revealed weight of 4.7kg(between 15th-50th centile), length of 56cm(at 50th centile), head circumference-38cm(between 15th-50th centile).
Abdominal examination revealed distended abdomen with palpable liver till level of umbilicus.
Blood investigations done showed normal counts with negative CRP,
HB-5.89g/dl.
T.Bilirubin-3.77mg/dl, D.Bilirubin-1.42mg/dl, T.Protein-4.1g/dl, Albumin-3.1g/dl, SGOT-83IU/L, SGPT-14.6IU/L,AFP-2722ng/ml,
Urea-8.6mg/dl,Creatinine-0.37mg/dl.
Preop serology was negative.
INR-1.9
TFT was altered(hypothyroidism).
CECT abdomen done was suggestive of large hepatic hemangioma involving entire left lobe and part of right lobe of liver.
Child was started on IV Steroids , propranolol and furosomide
Inj IV methyl prednisolone was started at a dose of 30mg/kg for 3 days and was gradually changed to oral.
Red blood cell transfusion given in view of low hemoglobin.
Fresh frozen plasma transfusion was also given in view of suspicion of bleed as ba had low hemoglobin and altered coagulation profile. USG Abdomen done following treatment courses showed decrease in dimensions of hemangioma.
Child was also started on thyroxine in view of altered TFT at dose 25 microgram/kg due consumption of thyroxine in the haemangioma
Child improved symptomatically and hence discharged on propranolol and furoped.
CT Abdomen showing hepatic hemangioma.
Infantile hepatic hemangioma(IHH) is the most common benign hepatic vascular tumour of infancy, comprising approximately 1% of all childhood tumors.
The tumour can present during fetal or neonatal period as a hepatic mass. Common presentations include abdominal distension and a palapable hepatic mass. Child can also present with tumor complications such as coagulopathy, heart failure or jaundice.Child can also have associated hypothyroidism(increased type 3 iodothyronine produced hemangiomas). Clinico-radio-pathological correlation is required for definite diagnosis.
In 3% cases there will be elevated alpha feto protein.
Frequent complications such as congestive cardiac failure(due to substantial arteriovenous shunting), thrombocytopenia,anemia and Kasabach-Merritt syndrome(consumptive coagulopathy)should be investigated.
The natural history of IHH in infancy is a rapid proliferative growth phase in the first six months of life, followed regression and involution. Corticosteroids are the treatment of choice for IHH.
If the child remains asymptomatic, no treatment may be needed. If symptoms of high output cardiac failure occur first line therapy is propranolol and furosomide . Surgical resection has an essential role for symptomatic patients with medical treatment failure. Further more prenatal diagnosis is essential for better patient outcomes due to prompt treatment in neonatal period.
Prognosis-Poor prognostic factors include heart failure, diffuse HIH, abdominal compartment syndrome. Proliferative phase >2 years of age or new lesion after 1 year of age has worst outcome.
Take home message- The significance of interdisciplinary collaboration among pediatricians, hepatologists and surgeons in the management of this rare but potentially serious condition would be beneficial.