An 11-year-old girl with lipoprotein lipase deficiency experienced recurring episodes of abdominal pain. She initially underwent appendectomy for suspected appendicitis; however, the appendix was normal. Pancreatitis was subsequently identified as the cause of her pain

Klerk, J.B.C. (Johannes) de

Postema, R.R. (Roelf)

Walraven, L.A. (L.) van

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Severe Acute Necrotizing Pancreatitis Associated with LipoproteinLipase Deficiency in ChildhoodBy L.A. van Walraven, J.B.C. de Klerk, and R.R. PostemaRotterdam, The NetherlandsAn 11-year-old girl with lipoprotein lipase deficiency experi-enced recurring episodes of abdominal pain. She initiallyunderwent appendectomy for suspected appendicitis; how-ever, the appendix was normal. Pancreatitis was subse-quently identified as the cause of her pain.J Pediatr Surg 38:1407-1408. © 2003 Elsevier Inc. All rightsreserved.INDEX WORDS: Lipoprotein lipase deficiency, acute abdo-men, necrotizing pancreatitis.THE INCIDENCE of acute pancreatitis in childhoodis unknown, but it is suspected to be more commonthan currently recognized. Diagnosis remains difficult,especially in infancy, because of the nonspecific clinicalfeatures and the small window period to establish thecorrect diagnosis.1-3CASE REPORTAn 11-year-old girl, known in our center with lipoprotein lipasedeficiency (autosomal recessive disorder with an incidence of1/1.000.000 newborns), was hospitalized elsewhere with severe ab-dominal pain. The diagnosis of lipoprotein lipase (LPL) was notknown. An emergency laparotomy was undertaken during the firstadmission. She was thought to have peritonitis based on appendicitis,and classic open appendectomy was performed. After 2 days, theabdominal pain disappeared, and the appendicitis was believed to bethe source of her symptoms. She left the hospital without complains.The later microscopic findings did not confirmed the diagnosis appen-dicitis (appendix sana).The second attack of abdominal pain was 3 months later, and duringthis hospitalization, she was transferred after 4 days to our centerbecause of clinical decline. During both hospitalizations, the surgicalteam excluded the diagnosis of acute pancreatitis based on a normalserum amylase concentration (100 IU/L).We found a very ill girl with severe abdominal and back pain andileus with persisting vomiting. Temperature was 38.8°C, and the serumamylase estimation was 379 U/L. The other findings were C reactiveprotein (CRP), 36 mg/L; white blood cell count (WBC), 17 10E9/L;triglyceride level, 33 mmol/L; and lipase level, 5,060 U/L. The patientwas not on her diet for LPL so there was a strong suspicion ofpancreatitis in this case.The computed tomography (CT) scan showed an severe pancreatitis(Balthazar E) with 10% necrosis of the corpus (Computed TopographySeverity Index 6) (Fig 1). An intensive therapy was performed on ourintensive care unit. The fluid, electrolyte, and mineral balance wasrestored and maintained, and carention, total parenteral nutrition, andpain relief were given by infusion.Early antibiotics were started (imipenem-cilastatin) because of atemperature of 39.1°C, WBC of 17 10E9/l, and CRP of 211 mg/L.Administration of heparin was not necessary because the plasmatriglyceride concentration fell and normalized in 2 days. The second CTscan showed a more severe image with peripancreatic fluid collectionsbut there was clinical improvement, so intensive medical treatment wascontinued (Fig 2). After 4 weeks of treatment she left the hospital withher diet for LPL without any complains and with normal endocrine andexocrine pancreas functions.DISCUSSIONAcute pancreatitis is seen commonly in adult surgicalpractice. In contrast, acute pancreatitis in children is rareand diagnosis often is delayed. In this case, the massiveelevation of plasma triglycerides, which often is clini-cally silent, resulted in 2 severe attacks of pancreatitis.In our hospital, the triglyceride concentration alreadydropped and, as a result, the amylase concentration rose(379 U/L). At admission in the other hospital the amylaseconcentration was normal (100 U/L). The treatment forlipoprotein lipase deficiency is aimed at making the dietlow enough in long chain fatty acids to keep the patientasymtomatic and free of recurrent bouts of pain. Thediagnosis of pancreatitis was missed because of a diag-nostic problem that arose when only the serum amylaseconcentration was considered in this patient with severeabdominal pain.The value of serum amylase is reduced by high serumtriglycerides, so pancreatitis may occur with a normalamylase concentration in patients with hypertriglyceri-daemia.4,5 A great variety of etiologies of acute pancre-atitis are evident in children; idiopathic (22%), trauma(20%), infection (15%), biliary tract disease (14%),drugs (13%), congenital anomalies (5%), hereditary,miscellaneous, and, as in this case, metabolic disorders.5From the Department of General Surgery, Academic Hospital Di-jkzigt and the Departments of Metabolic Disorders and PaediatricSurgery, Academic Children’s Hospital-Sophia, Rotterdam, The Neth-erlands.Address reprint requests to Laurens A. van Walraven, MD, Depart-ment of Surgery, Academic Hospital Dijkzigt, Dr Molenwaterplein 40,3015 GD, Rotterdam, The Netherlands.© 2003 Elsevier Inc. All rights reserved.0022-3468/03/3809-0029$30.00/0doi:10.1016/S0022-3468(03)00409-31407Journal of Pediatric Surgery, Vol 38, No 9 (September), 2003: pp 1407-1408The clinical features are not specific, but sudden onsetof severe abdominal pain in the epigastric upper quadrantmay be seen in acute pancreatitis with radiation to theback or left shoulder.In children with acute pancreatitis, initially, the serumamylase levels are normal in 10% to 15% of the patients.Serum lipase is more specific than amylase for acuteinflammatory pancreatic disease and should be deter-mined when pancreatitis is suspected, and the amylaselevel is normal. Furthermore, these enzyme assays arenot useful in infancy because of the physiologic defiencyof both enzymes.2,3 Ultrasound scan and CT scan areuseful in looking for edema, necrosis, abscess, orpseudocyst of the pancreas.The clinical awareness still is the most importantfactor leading to correct diagnosis, and hopefully futurenovel diagnostic markers (serum trypsinogen 2 and al-pha-1-antitrypsin). will be commercially available soon.REFERENCES1. Haddock G, Youngson GG, Mackinlay GA, et al: Acute pancre-atitis in children: A 15-year review. J Pediatr Surg 6:719-722, 19942. Weizman ZVI: Acute pancreatitis in childhood: Research ofpathogenesis and clinical implications. Can J Gastroenterol 11:249-253, 19973. Meyha DI: Acute and chronic pancreatitis in childhood. IndianJ Pediatr 66:S81-S86, 19994. Ohmoto K, Neishi Y, Miyake I, et al: Severe acute pancreatitisassociated with hyperlipidemia: Report of two cases and review of theliterature in Japan. Hepatogastroenerol 46:2986-2990, 19995. Sharma P, Lim S, James D, et al: Pancreatitis may occur with anormal amylase concentration in hypertriglycerideamia. BMJ 313:1265, 1996Fig 1. CT scan at admission. Severe acute necrotizing pancreati-tis, Balthazar E, with visible necrosis in the corpus of the pancreas.Fig 2. CT scan after 1 week. More peripancreatic collections arevisible.1408 VAN WALRAVEN, DE KLERK, AND POSTEMA

Severe acute necrotizing pancreatitis associated with lipoprotein lipase deficiency in childhood

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Severe acute necrotizing pancreatitis associated with lipoprotein lipase deficiency in childhood

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