TÇCD 2011 29. National Pediatric Surgery Congress and 27. National Congress of The Egyptian Pediatric Surgical Association

Background/Purpose: Nesidioblastosis is a name used to describe congenital hypoglycemia. It is the most common cause of persistent Hypoglycemia in infancy with consequences such as seizures and brain damage. Treatment yielding avoidance of these sequele is essential. When medical therapy fails or complications are anticipated; early pancreatectomy is recommended to maintain euglycemia. The aim of this study is short term evaluation of Mansoura experience of near total pancreatectomy for Nesidioblastosis.

Patient and methods:During the period of May 2002 to May 2010, 13  patients suffering of persistent  hyperinsulinaemic  hypoglycemia (nesidioblastosis) were treated by near total (90-95%)pancreatectomy after failure of medical treatment .They were 8 females and 5 males and their ages at time of surgery ranged from 20 days to 27months. They all suffered symptoms of nesidioblastosis;thermoregulatory problemsin 5 cases,tremors in  5, seizures in  3, irritability in  5, convulsions in 3,respiratory distress in 8, apnea in  4,hypotonia in 4, lethargy in 5 and feeding difficulty in 4.All cases were diagnosed following strict criteria that included bouts of hypoglycemia without acidosis coinciding withincreased insulin level. Follow up ranged from 3 months to 8 years.

Results:primary success occurred in 2cases (15%), 4 more cases (31%) sufferedtemporary diabetes mellitus and one case (8%) had a temporary pancreatic fistula. Eventually  total cure occurred in 7 cases( 54%). 6 cases (46%) suffered a variety of complications; persistent hypoglycemia in 2 cases, Persistent diabetes mellitus in 3, developmental delay in one , persistent neurological deficit  in 2  and one case of mortality.

 

Conclusion: Near total (90-95%)pancreatectomy proves to be a suitable procedure for treatment of nesidioblastosis not responding to medical treatment and should be performed as early as possible.

 

Keywords: Pancreatoctomy, nesidioblastosis, congenital hypoglycemia

Background/Purpose: Nesidioblastosis is a name used to describe congenital hypoglycemia. It is the most common cause of persistent Hypoglycemia in infancy with consequences such as seizures and brain damage. Treatment yielding avoidance of these sequele is essential. When medical therapy fails or complications are anticipated; early pancreatectomy is recommended to maintain euglycemia. The aim of this study is short term evaluation of Mansoura experience of near total pancreatectomy for Nesidioblastosis.

Patient and methods:During the period of May 2002 to May 2010, 13  patients suffering of persistent  hyperinsulinaemic  hypoglycemia (nesidioblastosis) were treated by near total (90-95%)pancreatectomy after failure of medical treatment .They were 8 females and 5 males and their ages at time of surgery ranged from 20 days to 27months. They all suffered symptoms of nesidioblastosis;thermoregulatory problemsin 5 cases,tremors in  5, seizures in  3, irritability in  5, convulsions in 3,respiratory distress in 8, apnea in  4,hypotonia in 4, lethargy in 5 and feeding difficulty in 4.All cases were diagnosed following strict criteria that included bouts of hypoglycemia without acidosis coinciding withincreased insulin level. Follow up ranged from 3 months to 8 years.

Results:primary success occurred in 2cases (15%), 4 more cases (31%) sufferedtemporary diabetes mellitus and one case (8%) had a temporary pancreatic fistula. Eventually  total cure occurred in 7 cases( 54%). 6 cases (46%) suffered a variety of complications; persistent hypoglycemia in 2 cases, Persistent diabetes mellitus in 3, developmental delay in one , persistent neurological deficit  in 2  and one case of mortality.

 

Conclusion: Near total (90-95%)pancreatectomy proves to be a suitable procedure for treatment of nesidioblastosis not responding to medical treatment and should be performed as early as possible.

 

Keywords: Pancreatoctomy, nesidioblastosis, congenital hypoglycemia