Introduction. Extra-hepatic portal vein obstruction (EHPVO) is one of the
most often causes of portal hypertension in children. Objective. Establishing
the importance of shunt surgery in combination with partial spleen resection
in selected pediatric patients with EHPVO, enormous splenomegaly and severe
hypersplenism. Methods. Distal splenorenal shunt (DSRS) with partial spleen
resection was performed in 22 children age from 2 to 17 years with EHPVO.
Indications for surgery were pain and abdominal discomfort caused by spleen
enlargement, as well as symptomatic hypersplenism with leucopenia,
thrombocytopenia and anemia. The partial spleen resection was performed by
ligation of blood vessels to caudal two thirds of the spleen. After ischemic
parenchymal demarcation transection with electrocautery LigaSure was
performed with preservation of 20-30% of spleen tissue, and then Warren DSRS
was created. Platelet and leucocytes counts and liver function tests were
obtained before, one month and one year after surgery. Growth was assessed
with SD scores (Z scores) for height, weight and body mass index at the time
of surgery and one year later. Results. In all patients postoperative period
was without significant complications. Platelets and leucocytes counts were
normalized. Patency rate of shunts was 100%. Two significant shunts stenosis
were observed and successfully treated with percutaneous angioplasty. During
the follow-up period (1 to 9 years) all patients were asymptomatic, with
improved quality of life and growth. Conclusion. Results of our study
indicate that shunt surgery with a partial spleen resection is an effective
and safe procedure for patients with enormous splenomegaly and severe
hypersplenism caused by EHPVO.
Airway management of a paediatric patient with temporomandibular joint ankylosis with extra hepatic portal vein obstruction, splenomegaly, hypersplenism, and obstructive sleep apnoea for shunt surgery: A unique challenge