|Year : 2008 | Volume
| Issue : 5 | Page : 566
Anaesthesia for Nesidioblastosisin A New Born: A Case Report
K Sunitha Zachariah1, C Karthikeyan2, Rebecca Jacob3, Chacko Jacob4
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 Acceptance||05-May-2008|
|Date of Web Publication||19-Mar-2010|
K Sunitha Zachariah
Department of Anaesthesia, Christian Medical College, Vellore
Source of Support: None, Conflict of Interest: None
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 Sep 25];52:566. Available from: http://www.ijaweb.org/text.asp?2008/52/5/566/60676
| Introduction|| |
Nesidioblastosis  , 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  . Initial management is mainly medical with high concentration glucose infusion, frequent high calorie feeds, intramuscular glucagon, hydrocortisone, diazoxide and diuretic , . 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|| |
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|| |
Patients with PHHI may present from birth to 18 months with symptoms of persistent hypoglycemia requiring frequent or continuous dextrose infusions.  They may be large for their gestational age because of chronic hyperinsulinism.
Nesidioblastosis is commonly inherited as an autosomal recessive defect. , Familial cases also have been documented.The histological abnormalities in pancreatic structure are of two types.  (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  . 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. , 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. , 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.  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.  Therefore a 95% pancreatectomy was planned, since a near total pancreatectomy is required for maintaining euglycemia. , 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.
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