DR.C.ANANDHI DNB RESIDENT SOUTHERN RAILWAY HEADQUARTERS HOSPITAL

Transcription

1 DR.C.ANANDHI DNB RESIDENT SOUTHERN RAILWAY HEADQUARTERS HOSPITAL

2 Navasakthi 2 years female toddler born as preterm(36 wks) with birth wt 2.73 kg by emergency LSCS:Indication -severe hypertension. Mother - Type 2 DM on oral medication and Insulin. Hypothyroid on Eltroxin. Essential hypertension on Nifidipine.

3 Neonatal period- had respiratory distress, hypotonic and lethargic and diagnosed to have congenital hypothyroidism. Except for thyroid profile all investigations were normal. Baby discharged on day 7 with Eltroxin 50 microgram od.

4 1 month to 5 month baby thrived well with good thyroid control. But visited op with 3 episodes of unexplained tachypnoea. Cardiology opinion obtained. Ecg,echo normal.

5 At 6 months infant had c/ofast breathing for 2 days, Clinical featureshr-180/min BP-140/100 mmhg in rt upper limb. RR-62/min.

6 Anthropometry normal. Milestones normal. CVS normal RS-normal ABDOMEN-normal CNS-normal Suspected to have eltroxin induced hyperthyroidism and investigated.

7 Investigation: Hb-13 Pcv-37 TLC-14,800 Platelet-2,28,000 RFT- normal LFT normal serum electrolytes -normal TSH 2 mu/ml(normal) T3-2.2nmol/l;FT4-14 pmol/l (normal)

8 ECG-left ventricular hypertrophy Echo-normal USG KUB-normal

9 Plasma adrenaline<33.3pg/ml-normal nor-adrenaline<133.3pg/ml-normal Urine 24 hrs metanephrine 0.10mg/24 hrs 24 hrs vma 0.8mg/24 hrs Serum cortisol-7.58 ( ) micgm/dl Aldosterone (40-310)ng/l Renin- normal

10 CT Renal angiogram: Renal arteries single on both sides,no evidence of stenosis or aneurysm. Renal veins single on both sides. Abnormal large splenorenal shunt between splenic vein and left renal vein. Left renal vein dilated measuring 1.1cm Shunt measured 0.6cm. Portal vein narrowed measuring 0.2 cm.

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14 CONGENITAL HYPOTHYROIDISM CONGENITAL SPLENORENAL SHUNT HYPERTENSION

15 Management: Hypertension: Infant was initially started with T.Propranolol 1.5 mg bd, since control of bp not achieved dose increased to 2.5mg tid. Infant was under regular follow up for hypothyroidism and hypertension. At around 11 months bp was again high and dose increased to 5 mg bd. Hypothyroid:Eltroxin 50 microgram od.

16 In view of rarity of case and to decide on surgical options opinion of Hepatologist/Hepatobiliary Surgeons obtained.

17 Serum ammonia normal CT abdomen-spleno renal shunt, Normal appearing liver and spleen. UGI scopy normal. CT guided liver biopsy- normal.

18 Presently the child is 2 yrs old with Normal anthropometry Normal milestones Hypothyroidism under control Hypertension under control (cause???) Congenital splenorenal shunt with no complications.

19 Definition: CPSVS is defined as occurring in the absence of primary liver disease and in the presence of either a large shunt vessel or hemangioma that is related to vascular developmental anomalies.

20 Embryology absence of portal vein is due to excessive involution of the peri-intestinal vitelline venous loop or total failure of the vitelline veins to establish the critical anastomosis with the hepatic sinusoids or umbilical veins. Extrahepatic portosystemic shunts originate with the persistence of subcardinohepatic anastomosis with the vitelline veins.

21 CLASSIFICATION In 1994 Morgan and Supreina classified Type I-there is a complete end-to-side portocaval fistula with no portal flow to the liver. Type II- shunts occur as a side-to-side portocaval fistula or as any number of other PSS including gastrorenal, splenorenal, and portorenal shunts.

22 In 1997, Howard and Davenport applied the Abernethy eponym to portocaval shunts and recognized the type I and type II variants. I - No intrahepatic portal flow (CAPV or type I Abernethy malformation). II -Partial shunt with preserved hepatic portal flow (type II Abernethy malformation)

23 IIa - Arising from left or right portal vein (includes PDV) IIb - Arising from main portal vein (including its bifurcation or splenomesenteric confluence) IIc - Arising from the mesenteric, gastric, or splenic veins.

24 Type I frequent in girls. multiple malformations as polysplenia, malrotation, and cardiac anomalies coexist. Liver transplantation, which offers the only option for cure, is reserved for patients with refractory symptoms despite medical management.

25 Type II no gender preponderance. Clinical presentation: jaundice,hyperammonemia, coagulopathy,neurocognitive dysfunction, in newborn galactosemia with normal enzyme levels. Symptoms of encephalopathy may not present until adulthood.

26 Complications : Hepatopulmonary syndrome Pulmonary artery hypertension Porto systemic encephalopathy Heart failure Glomerulonephritis Liver tumors

27 Investigation: Doppler utrasonography -primary diagnosis CT and MR angio for classification. Radiolabelled per rectal portal scintigraphy to diagnose shunt ratio. Antenatal USG detection has also been done.

28 Natural course : Depends on shunt ratio and patient age. Spontaneous closure is expected to occur in the first 2 years of life when an intrahepatic portosystemic shunt is found; Close follow-up is recommended because most of them are asymptomatic.

29 Mild metabolic abnormalities relieved by medical therapy with protein-free diet and oral administration of lactulose and branched-chain amino acids.

30 SURGICAL: shunt ratios above 60% is corrected due to the risk of encephalopathy and liver dysfunction. Both embolization and surgical correction of the shunt have been described. Resection is the treatment of choice in patients with liver tumors associated with extrahepatic portosystemic shunting.

31 Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Ohno T, Muneuchi J, Ihara K, Yuge T, Kanaya Y, Yamaki S, Hara T. Pediatrics Apr;121(4):e Epub 2008 Mar 24.

32 Nine patients with congenital portosystemic venous shunt were studied from January 1990 through September patients had history of neonatal hypergalactosemia. 6 patients had history of PAH median age at diagnosis 12 years and 3 months.

33 Laparoscopic Correction of Congenital Portosystemic Shunt in Children Kimura, Takuya PhD*; Soh, Hideki MD*; Hasegawa, Toshimichi PhD*; Sasaki, Takashi PhD Surgical Laparoscopy, Endoscopy & Percutaneous Techniques: October Volume 14 - Issue 5 - pp

34 Management and classification of type II congenital portosystemic shunts Timothy B. Lautz, Niramol Tantemsapya, Erin Rowell, Riccardo A. Superina Department of Surgery, Children's Memorial Hospital, Feinberg School of Medicine of Northwestern University, Box 57, Chicago, IL 60614, USA Journal of pediatric surgery, Journal of Pediatric Surgery (2011) 46,

35 Journal of Vascular and Interventional Radiology Volume 22, Issue 8, August 2011, Pages Portal Venous Remodeling After Endovascular Reduction of Pediatric Autogenous Portosystemic Shunts.

36 OPEN FOR DISCUSSION

37 THANK YOU