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CASE REPORT
Year : 2008  |  Volume : 52  |  Issue : 5  |  Page : 566 Table of Contents     

Anaesthesia for Nesidioblastosisin A New Born: A Case Report


1 Senior lecturer, Department of Anaesthesia, Christian Medical College, Vellore, India
2 P.G. Student, Department of Anaesthesia, Christian Medical College, Vellore, India
3 Professor, Department of Anaesthesia, Christian Medical College, Vellore, India
4 Professor, Department of Paediatric Surgery, Christian Medical College, Vellore, India

Date of Acceptance05-May-2008
Date of Web Publication19-Mar-2010

Correspondence Address:
K Sunitha Zachariah
Department of Anaesthesia, Christian Medical College, Vellore
India
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Source of Support: None, Conflict of Interest: None


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Congenital hyperinsulinism as seen in nesidioblastosis is the most common cause of non transient neonatal hypoglycaemia. The prime concern in nesidioblastosis is the prevention of dangerous hypoglycaemia which can lead to coma, brain damage or mental retardation. In this report we describe the presentation and perioperative course of a case of nesidioblastosis in a newborn who had persistent hypoglycaemia with convulsions in spite of high concen­trations of dextrose infusion. As sugars were low in spite of medical management, 95% pancreatectomy was done and the baby was discharged following discontinuation of intravenous fluids and starting breast feeds.

Keywords: Hypoglycemia, Hyperinsulinism, Nesidioblastosis, Anaesthesia


How to cite this article:
Zachariah K S, Karthikeyan C, Jacob R, Jacob C. Anaesthesia for Nesidioblastosisin A New Born: A Case Report. Indian J Anaesth 2008;52:566

How to cite this URL:
Zachariah K S, Karthikeyan C, Jacob R, Jacob C. Anaesthesia for Nesidioblastosisin A New Born: A Case Report. Indian J Anaesth [serial online] 2008 [cited 2020 Feb 19];52:566. Available from: http://www.ijaweb.org/text.asp?2008/52/5/566/60676


   Introduction Top


Nesidioblastosis [1] , the term coined by Laidlow in 1938 is now termed 'Persistent Hyperinsulinemic Hy­poglycemia of Infancy' (PHHI). It represents the most common cause of hyperinsulinism which can lead to brain damage or death secondary to severe hypoglycaemia 2 . Cornblath et al suggested the follow­ing thresholds for glucose administration 45mg% or less in infants with clinical manifestations compatible with hypoglycaemia or less than 36mg% in infants at risk of hypoglycaemia [2] . Initial management is mainly medical with high concentration glucose infusion, frequent high calorie feeds, intramuscular glucagon, hydrocortisone, diazoxide and diuretic [3],[4] . If unsuccessful, surgical re­section of the pancreas is done. We describe the perioperative course of this condition in a baby who presented with recurrent hypoglycaemic episodes.


   Case report Top


An eight hour old female baby weighing 3680 gms was admitted to neonatal ICU with irregular shallow breathing, apnoeic spells and cyanosis. On examina­tion, her periphery was cold, pulses feeble and capil­lary refill time more than two seconds. The mother was a non diabetic primigravida who had severe pre-ec­lampsia. The baby was delivered through a normal vagi­nal delivery with an APGAR score of 7 and 10 at the 1 st and 5 th minutes respectively. At the time of admis­sion, the baby needed inotropic and ventilatory sup­port. She was hypoglycaemic at birth and continued to have persistent hypoglycaemia in spite of both dex­trose 10-12.5% infusion at 22 ml/hour (12mg kg­-1 minute -1 ) and breast feeds. We used 12.5% as we were unable to control the sugars with 10% dextrose. We increased the concentration till we found the child was stable with infusions of 12.5 %. This was achieved by diluting 25% dextrose with 10% dextrose to achieve the particular concentration. Sugars were maintained between 20-45mg%. There was no ketonuria. The baby had one episode of hypoglycaemic seizures, which was managed with escalating doses of intravenous dex­trose and phenobarbitone. She was treated with diazoxide upto 15 mg.kg -1 day -1 and hydrochlorthiazide 3.5mg.kg -1 .day -1 . Since she did not show any response to these drugs, a possibility of nesidioblastosis was considered. The diagnosis was confirmed with simul­taneous assays of insulin and glucose [Table 1] and a 95% pancreatectomy was planned.

Prior to surgery, the baby was kept fasting for 3 hours, but 12.5% dextrose infusion was maintained at 20-22ml/hour. Monitoring included pulse oximetry, elec­trocardiogram, non-invasive blood pressure, endtidal carbo ndioxide, temperature, precordial stethoscope and glucometer- blood sugar and urine output monitoring[bladder was catheterized and the urine output measured with a syringe]. The glucometer sugar values were confirmed with laboratory sugar values.The baby was induced with fentanyl 3.5mcg , thiopentone 20mg and ventilated with oxygen, air and sevoflurane 2-4%. The trachea was intubated with a size 3.5 mm uncuffed endotracheal tube after paralyzing with 2mg atracurium. The anaesthesia was maintained with oxygen,air ,sevoflurane 1.5-2%. Intravenous fentanyl 4mcg and paracetamol suppository 80 mg were used for intraoperative analgesia. A 95% pancreatectomy, leaving a thin rim of pancreatic tissue to protect the pancreatico-duodenal vessels, was done. The tempera­ture was recorded using a nasopharyngeal probe and it was maintained between 35.5-36.5°C with the use of a forced air warming device and fluid warmer. Sugar monitoring was done at 15 minute intervals. Since the preoperative sugar was 220mg% 12.5%dextrose in­fusion was reduced to 2ml/hour and then stopped after 30 minutes since the random blood sugar was above 200mg%. 0.9% normal saline was used as replace­ment fluid at 25-30 ml/hour. Intraoperative sugars were maintained in the range of 239-250 mg% in the first two hours. The surgery lasted for four hours. By the conclusion of surgery, sugars came down [the lowest being 76mg%] so the 12.5% dextrose infusion was con­tinued at 20ml/hour and the last sugar after extubation was 156mg%. At the end of the surgery, the wound was infiltrated with 0.25% bupivacaine. Blood loss was approximately 50ml. 50ml of FFP and 50 ml of whole blood were transfused. The total urine output was 20ml. As the baby was normothermic (36.6°C)and wide awake, she was extubated and transferred to the nursery for post-operative monitoring.

The blood sugar levels remained very high post­operatively, so insulin 0.1U/kg was given with 5% of dextrose at 20ml/hour. After thirty hours, the baby was started on oral liquids and breast feeds, which she tol­erated well and maintained blood sugars. The insulin was stopped on the third postoperative day. As her sugar values remained normal she was discharged on the seventh post-operative day without any medications. On follow up two months later the baby was euglycemic. The histopathology report of the resected specimen showed nesidioblastosis (diffuse type) with focal ductal squamous metaplasia.


   Discussion Top


Patients with PHHI may present from birth to 18 months with symptoms of persistent hypoglycemia re­quiring frequent or continuous dextrose infusions. [3] They may be large for their gestational age because of chronic hyperinsulinism.

Nesidioblastosis is commonly inherited as an au­tosomal recessive defect. [3],[5] Familial cases also have been documented.The histological abnormalities in pan­creatic structure are of two types. [6] (1)Focal adenom­atous hyperplasia and (2)diffuse adenomatous hyper­ plasia.

Glucose requirements in infants is high 6-8mg.kg­-1 .minute -1 as compared to 2-3 mg.kg -1 .minute -1 in adults. In the fed state, insulin levels can rise to 50­150mcgU.ml -1 and falls to 5-10 mcgU.ml -1 with fast­ing. PHHI is suspected in an infant with severe hypogly­cemia who needs glucose infusion at a rate more than 10 mg.kg -1 .minute -1 to maintain euglycemia.. The diag­nosis is confirmed if there is an inappropriately high in­sulin-glucose ratio > 0.3-0.5 during hypoglycemia [3] . Supportive evidence is low serum ketones, free fatty acids and absence of acetonuria and acidosis. Mea­surement of C-peptide is also useful as its clearance is slower than that of insulin. [3],[6] Therapeutic approaches are glucose infusion 10-30 mg.kg -1 .minute -1 as required, high calorie frequent feeds, glucagons (IM) and hydrocortisone(IV) as emergency measures, diazoxide upto 15 mg.kg -1 .day -1 in 3 divided doses for 3 days but not longer. Diazoxide blocks insulin secretion by inhib­iting sulphonylurea receptors. [2],[3] Diazoxide is usually administered along with a thiazide diuretic (chlorothiazide ) which potentiates its hyperglycemic action and re­duces fluid retention. Octreotide ,a long acting analogue of somatostatin suppresses insulin secretion further down stream and can be given if there is no response to diazoxide.

Our patient had an insulin: blood sugar level ratio more than 0.3-0.5. [2] She was treated with 12mg.kg­-1 .minute -1 of glucose, diazoxide up to 15 mg.kg -1 .day -1 and hydrocortisone with sub optimal response. [6] There­fore a 95% pancreatectomy was planned, since a near total pancreatectomy is required for maintaining euglycemia. [7],[8] There was a rebound hyperglycemia af­ter resection, but it was controlled with a short period of insulin infusion and a reduced concentration of dex­trose. In conclusion, outcome of this rare condition can be poor but early diagnosis and intervention markedly reduces the morbidity and damage in these neonates.

 
   References Top

1.Laidlaw GF. Nesidioblastoma, the islet tumour of the pancreas. Am J Pathol 1938; 14:125-134.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2.La Franchi S. Hypoglycaemia of infancy and childhood.Paediatric clinics of North America 1987;34:961-982.  Back to cited text no. 2      
3.Desai M P.Hypoglycemia in infancy and childhood. Pae­diatric endocrine disorders1st edition.Orient Longman 2001;pp:335-347.  Back to cited text no. 3      
4.Cryer PE. Glucose homeostasis and hypoglycemia. In: Wilson JD, Foster DW, eds. Williams Textbook of Endo­crinology, 11th edn. Philadelphia: WB Saunders 2007;1525-1526.  Back to cited text no. 4      
5.Warne G.L, Dabadghao P, Boneh A.. Endocrinology of the newborn. Clinical pediatric endocrinology 4 th edition Charles G.D Brook, Blackwell Science 2001: pp:88­-90.  Back to cited text no. 5      
6.Stanley Charles A. Hyperinsulinism in infants and chil­dren. The Pediatric Clinics of North America 1997; 44:363­-373.  Back to cited text no. 6      
7.Desai M.P, J.V.Khatri. Persistent Hyperinsulinemic Hy­poglycemia of infancy. Indian pediatrics 1998;35 : 317­-327.  Back to cited text no. 7      
8.Harold Lovvorn, Nanca Michael L. Congenital hyperin­sulinism and the surgeon. Lesson learned over 30 years. Journal of Pediatric Surgery 1999;34:786- 793.  Back to cited text no. 8      



 
 
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