VOLUME 29, 2007

PSU Volume 29 No 01  JULY 2007

Preauricular Tags

Preauricular skin or chondrocutaneous branchial  remnant is not a rare finding in infants. Preauricular tags occur in five of every 1000 live births. They are congenital malformations that should be taken care for aesthetic reasons. Most preauricular tags are from branchial origin and contain cartilage. Excision is indicated after the age of six months under general endotracheal anesthesia as an ambulatory procedure. Cosmetic results are very good. Less than 5% of all preauricular tags are associated with other malformations of the ear/face region, most of them conductive type hearing impairment. Physical examination under such circumstances provides the first clue of a hearing disorder. Possibility increases if there is familial history of hearing impairment. When in doubt, audiometry is indicated. Another association with preauricular tag is the presence of a urinary tract anomaly which occurs in 8% of all cases. Such renal anomaly includes hydronephrosis caused by ureteropelvic obstruction or vesicoureteric reflux, and horse-shoe kidney. Some workers recommend that urinary tract ultrasonography be conducted in the routine assessment of infants with preauricular tags and multiple congenital anomalies. Specific disorders associated with tags that increase the yield of finding a renal disorder includes CHARGE association, Townes Brocks syndrome, branchio oto renal syndrome, Nager syndrome, and diabetic embryopathy. Routine renal ultrasonography is not necessary in the evaluation of children with isolated preauricular tags.

1- Kankkunen A, Thiringer K: Hearing impairment in connection with preauricular tags. Acta Paediatr Scand. 76(1):143 6, 1987
2- Atlan G, Egerszegi EP, Brochu P, Caouette Laberge L, Bortoluzzi P: Cervical chondrocutaneous branchial remnants. Plast Reconstr Surg. 100(1):32 9, 1997
3- Kohelet D, Arbel E: A prospective search for urinary tract abnormalities in infants with isolated
preauricular tags. Pediatrics. 105(5):E61, 2000
4- Wang RY, Earl DL, Ruder RO, Graham JM Jr.: Syndromic ear anomalies and renal ultrasounds. Pediatrics. 108(2):E32, 2001
5- Lizama M, Cavagnaro F, Arau R, Navarrete O, Fontanaz AM, Garcia CJ: Association of isolated preauricular tags and nephrourological anomalies: case control study. Pediatr Nephrol. 22(5):658 60, 2007

Blind Loop Syndrome

The blind loop, stagnant or contaminated loop syndrome are all synonymous names give to the same pathological disease entity. This occurs when a piece of bowel has stagnation of succus entericus with inability to propel it content causing bacterial overgrowth.  The resulting event of such bacterial overgrowth in the stagnant loop traduces itself into malabsorption (from bacterial breakdown of bile salts and deamination of protein), megaloblastic  anemia (from decrease Vitamin B-12 absorption), steatorrhea (from production of deconjugated bile salts toxic and ineffective for intestinal micelle formation), and fat-soluble vitamin deficiency. Clinically, blind loop syndrome is suggested by abdominal pain, feculent vomiting, steatorrhea and hypoalbuminemia. Most cases of blind loop syndrome in children occur after surgical procedures such as side to side small bowel anastomosis, side-to-side duodenojejunostomy most commonly cited. Proximal bowel dilatation after bypass procedures in duodenal or jejuno-ileal atresia can lead to the blind loop syndrome.  End-to side procedures such as hepaticojejunostomy can also lead to blind loop syndrome and episodes of cholangitis. Upper gastrointestinal series studies can localize the stagnant segment of bowel. Oral antibiotic  treatment can improve symptoms in some patients, though most cases will require operative excision or plication of the blind loop.

1- Seitz W, Mangold G, Gronniger J: The blind loop syndrome after side to side anastomoses of the gut. Leber Magen Darm. 7(2):84 90, 1977
2- Schwobel M, Hirsig J, Illi O, Battig U: The influence of small bowel contamination on the pathogenesis of bowel obstruction. Prog Pediatr Surg. 24:165 72, 1989
3- Stewart BA, Karrer FM, Hall RJ, Lilly JR: The blind loop syndrome in children. J Pediatr Surg. 25(8):905 8, 1990
4- Spigland N, Yazbeck S: Complications associated with surgical treatment of congenital intrinsic
duodenal obstruction. J Pediatr Surg. 25(11):1127 30, 1990
5- Grosfeld JL, Rescorla FJ: Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long term follow up. World J Surg. 17(3):301 9, 1993
6- Halkic N, Abdelmoumene A, Kianmanesh R, Vuilleumier H: Blind loop syndrome. Swiss Surg. 8(5):220 3, 2002

Caudal Block

Caudal block  anesthesia in infants and children is one of the most resourceful breakthrough of the past few years utilized during  general endotracheal anesthesia for pediatric surgical procedures. Caudal block provides perioperative and postoperative analgesia for such infraumbilical procedures as orthopedic, genitourinary, inguinal and lower extremity. The quality and level of the caudal blockade is dependent on the dose, volume, and concentration of the injected drug. This simple, safe and inexpensive regional anesthesia procedure reduces the general anesthesia needs during surgery affecting less the hemodynamics changes, and suppressing the metabolic and endocrine responses to stress while providing postoperative analgesia. Complications associated with caudal block are rare and includes dural puncture, vascular puncture, failure to identify the sacral hiatus and toxic reactions to local anesthetics. Caudal block is an excellent adjunct in inguinal and umbilical herniorrhaphies, hydrocelectomies, orchiopexy, circumcision and perirectal surgery. With caudal block opioids use can be avoided reducing such side-effects as respiratory depression, nausea, vomiting and delayed gastric emptying. Caudal block reduces the incidence of emergence agitation and pain scores following sevoflurane anesthesia.

1- Uguralp S, Mutus M, Koroglu A, Gurbuz N, Koltuksuz U, Demircan M: Regional anesthesia is a good alternative to general anesthesia in pediatric surgery: Experience in 1,554 children. J Pediatr Surg. 37(4):610 3, 2002
2- Tuncer S, Yosunkaya A, Reisli R, Tavlan A, CICekci F, Otelcioglu S: Effect of caudal block on stress responses in children. Pediatr Int. 46(1):53 7, 2004
3- Aouad MT, Kanazi GE, Siddik Sayyid SM, Gerges FJ, Rizk LB, Baraka AS: Preoperative caudal block prevents emergence agitation in children following sevoflurane anesthesia. Acta Anaesthesiol Scand. 49(3):300 4, 2005
4- Silvani P, Camporesi A, Agostino MR, Salvo I: Caudal anesthesia in pediatrics: an update. Minerva Anestesiol. 72(6):453 9, 2006
5- Davidson AJ, Ironfield CM, Skinner AV, Frawley GP: The effects of caudal local anesthesia blockade on the Bispectral Index during general anesthesia in children. Paediatr Anaesth. 16(8):828 33, 2006


PSU Volume 29 No 02 AUGUST 2007

Opsoclonus Myoclonus Ataxia

Opsoclonus-myoclonus-ataxia (OMA) syndrome is a rare paraneoplastic or paraviral neurologic syndrome commonly associated with neuroblastoma. Less than 3% of children with neuroblastoma develop OMA syndrome. Clinically the child with OMA syndrome develops an acute onset of rapid chaotic eye movements, myoclonic jerking of the limbs and extremity and ataxia. OMA is believed to be an immune-mediated disorder due to the detection of antineural, antineurofilament and anti-Hu  antibodies. The child with OMA syndrome caused by neuroblastoma has a favorable staged disease due to rapid work-up and findings that the patientís immune response to the tumor limits the metastatic and growth potential of the tumor. Rarely do these tumors shows n-MYC gene amplification, a poor prognostic finding. Children with neuroblastoma and OMA have an excellent survival. There is no correlation with duration of symptoms and late neurologic outcome. Most children respond to treatment of acute symptoms with steroids or ACTH. Late neurologic sequelae (delay in motor function, speech and cognition) of OMA children can be drastic and affect quality of life. Children with advanced stage disease require more intensive chemotherapy and have better outcomes with regard to late neurological sequelae.  The higher the immune response limits the spread of disease but increases the neurologic sequelae.

1- Rudnick E, Khakoo Y, Antunes NL, Seeger RC, Brodeur GM, Shimada H, Gerbing RB, Stram DO, Matthay KK: Opsoclonus myoclonus ataxia syndrome in neuroblastoma: clinical outcome and
antineuronal antibodies a report from the Children's Cancer Group Study. Med Pediatr Oncol. 36(6):612 22, 2001
2- Cooper R, Khakoo Y, Matthay KK, Lukens JN, Seeger RC, Stram DO, Gerbing RB, Nakagawa A, Shimada H: Opsoclonus myoclonus ataxia syndrome in neuroblastoma: histopathologic
features a report from the Children's Cancer Group. Med Pediatr Oncol. 36(6):623 9, 2001
3- Pranzatelli MR, Tate ED, Wheeler A, Bass N, Gold AP, Griebel ML, Gumbinas M, Heydemann PT, Holt PJ, Jacob P, Kotagal S, Minarcik CJ, Schub HS: Screening for autoantibodies in children with opsoclonus myoclonus ataxia. Pediatr Neurol. 27(5):384 7, 2002
4- Klein A, Schmitt B, Boltshauser E: Long term outcome of ten children with opsoclonus myoclonus syndrome. Eur J Pediatr. 166(4):359 363, 2007
5- Ertle F, Behnisch W, Al Mulla NA, Bessisso M, Rating D, Mechtersheimer G, Hero B, Kulozik AE: Treatment of neuroblastoma related opsoclonus myoclonus ataxia syndrome with high dose dexamethasone pulses. Pediatr Blood Cancer. Jan 16; [Epub ahead of print], 2007
6- Rostasy K, Wilken B, Baumann M, Muller Deile K, Bieber I, Gartner J, Moller P, Angelini P, Hero B: High dose pulsatile dexamethasone therapy in children with opsoclonus myoclonus syndrome. Neuropediatrics. 37(5):291 5, 2006


Common bile ducts stones (choledocholithiasis) are usually secondary stones from the gallbladder that migrate through the cystic duct causing acute or chronic biliary tract obstruction (obstructive jaundice), pancreatitis or cholangitis.  Most common bile duct stones in children pass spontaneously without significant complications. Rarely, children can also develop primary common bile ducts stones. Up to 15% of children harboring gallstones can harbor asymptomatic common bile duct stones. Recurrent right upper quadrant pain is the most common clinical presentation. Most children have idiopathic gallstones. Ultrasound is the first study which will reveal whether the child has common bile duct dilatation with the presence of stones. This is accompanied with measurement of liver function tests and pancreatic enzyme. Once dilatation of the common bile duct or stones is suspected, an MRCP is indicated. With good sonographic evidence of common bile ducts stones or a positive MRCP an ERCP should be performed to accomplish endoscopic sphincterotomy with extraction of the biliary duct stones. This is followed in the next few days with laparoscopic removal of the gallbladder. The endoscopic approach to choledocholithiasis produces less morbidity, less possibility of long term common bile duct stenosis, and permits preop visualization of the biliary tree.  If the endoscopic stone extraction cannot be accomplished with ERCP, then open or laparoscopic common bile duct exploration is indicated.

1- Lugo Vicente HL: Trends in management of gallbladder disorders in children. Pediatr Surg Int. 12(5 6):348 52, 1997
2- Newman KD, Powell DM, Holcomb GW 3rd: The management of choledocholithiasis in children in the era of laparoscopic cholecystectomy. J Pediatr Surg. 32(7):1116 9, 1997
3- Kumar R, Nguyen K, Shun A: Gallstones and common bile duct calculi in infancy and childhood. Aust N Z J Surg. 70(3):188 91, 2000
4- Griffin N, Wastle ML, Dunn WK, Ryder SD, Beckingham IJ: Magnetic resonance cholangiopancreatography versus endoscopic retrograde cholangiopancreatography in the diagnosis of choledocholithiasis. Eur J Gastroenterol Hepatol. 15(7):809 13, 2003
5- Mah D, Wales P, Njere I, Kortan P, Masiakos P, Kim PC: Management of suspected common bile duct stones in children: role of selective intraoperative cholangiogram and endoscopic retrograde cholangiopancreatography. J Pediatr Surg. 39(6):808 12, 2004
6- Vrochides DV, Sorrells DL Jr, Kurkchubasche AG, Wesselhoeft CW Jr, Tracy TF Jr, Luks FI: Is there a role for routine preoperative endoscopic retrograde cholangiopancreatography for suspected choledocholithiasis in children? Arch Surg. 140(4):359 61, 2005
7- Issa H, Al Haddad A, Al Salem AH: Diagnostic and therapeutic ERCP in the pediatric age group. Pediatr Surg Int. 23(2):111 6, 2007
8- Fitoz S, Erden A, Boruban S: Magnetic resonance cholangiopancreatography of biliary system abnormalities in children. Clin Imaging. 31(2):93 101, 2007

Cholelithiasis in SCD

Cholelithiasis is a frequent complication of Sickle Cell Disease (SCD) in children. Prevalence of developing pigmented stones can range from thirty to 50%. Even the prevalence of developing common bile duct stones in children with SCD is higher than the general population. Complications related to cholelithiasis include biliary colic, pancreatitis, cholecystitis and obstructive jaundice. Children with SCD should be screened for cholelithiasis since the age of eight years using abdominal ultrasound. Once cholelithiasis is identified removal of the sick gallbladder should be accomplished regardless if the child is asymptomatic or not. Preoperative transfusion reduces the morbidity of the surgical procedure.  Elective laparoscopic cholecystectomy should be the gold standard in children with SCD and asymptomatic cholelithiasis to prevent the potential complications of biliary colic, acute cholecystitis, and choledocholithiasis, which lead to major risks, discomfort, and longer hospital stay. Laparoscopic cholecystectomy results in a shorter hospital stay with fewer postoperative complications than open operation in patients with sickle cell disease and is the procedure of choice in the treatment of cholelithiasis in such patients.

1- Al Salem AH, Qaisruddin S: The significance of biliary sludge in children with sickle cell disease. Pediatr Surg Int. 13(1):14 6, 1998
2- Leandros E, Kymionis GD, Konstadoulakis MM, Albanopoulos K, Dimitrakakis K, Gomatos I, Androulakis G: Laparoscopic or open cholecystectomy in patients with sickle cell disease: which
approach is superior? Eur J Surg. 166(11):859 61, 2000
3- Seleem MI, Al Hashemy AM, Meshref SS: Mini laparoscopic cholecystectomy in children under 10 years of age with sickle cell disease. ANZ J Surg. 75(7):562 5, 2005
4- Curro G, Meo A, Ippolito D, Pusiol A, Cucinotta E: Asymptomatic cholelithiasis in children with sickle cell disease: early or delayed cholecystectomy? Ann Surg. 245(1):126 9, 2007

PSU Volume 29 No 03 SEPTEMBER 2007

Multinodular Goiter

Non-toxic multinodular goiter (MNG) refers to multinodular enlargement of the thyroid gland without overt hormone output. MNG is rare in children affecting primordially adolescent kids. The etiology of pediatric MNG appears multifactorial including autoimmune and familial factors (familial form has increased incidence of malignancy). Children presents with asymptomatic progressive nodular enlargement of both lobes of the thyroid gland. Work-up should include neck ultrasonography, thyroid scintigraphy, thyroid hormone levels, assessment of autoantibodies (antimicrosomal, antithyroid), aspiration cytology and histological examination. In populations with iodine deficiency, multinodular goiter is endemic. MNG follows an initial phase of hyperplastic goiter or results from the generation of several individual nodules. Alterations of the stromal and vascular tissues as well as the occurrence of somatic mutations are contributing factors. Histological examination of removed affected glands shows multiple adenomas with areas of epithelial hyperplasia, hemorrhage, and calcification. MNG has an 8% potential for malignant transformation in the form of papillary carcinoma, mostly increased in familial cases, those that have received cervical irradiation and presence of cervical adenopathies. Indications for surgery in non-toxic MNG includes compression symptoms such as painful or difficulty in swallowing, breathing discomfort, suspicion of carcinoma or cosmetic. Total thyroidectomy seems to be the most effective surgical procedure with lower morbidity than subtotal thyroidectomy.

1- Hurley DL, Gharib H: Evaluation and management of multinodular goiter. Otolaryngol Clin North Am. 29(4):527-40, 1996
2- Uccheddu A, Cois A, Licheri S: The choice of the intervention in the surgical treatment of nontoxic diffuse multinodular goiter. Minerva Chir. 51(1-2):25-32, 1996
3- Orgiazzi J: Multinodular goiter: natural history. Ann Chir. 53(3):233-6, 1999
4- Al-Fifi S, Rodd C: Multinodular goiter in children. J Pediatr Endocrinol Metab. 14(6):749-56, 2001
5- Hisham AN, Azlina AF, Aina EN, Sarojah A: Total thyroidectomy: the procedure of choice for multinodular goitre. Eur J Surg. 167(6):403-5, 2001
6- Rios A, Rodriguez JM, Canteras M, Galindo PJ, Balsalobre MD, Parrilla P: Risk factors for malignancy in multinodular goitres. Eur J Surg Oncol. 30(1):58-62, 2004

Neonatal Mastitis

Neonatal mastitis is a disorder specific to newborn breasts that usually occurs between the second and fourth week of life. All cases usually occur in full term infants with a sex ratio of 2:1 (females: males). Bilateralism is rare. Systemic spread or extra mammary foci even rarer. Clinically the baby presents with a swollen, tender, erythematous breast bud. The etiology of the mastitis includes skin organism most commonly staphylococcus aureus (85%) followed by gram negative bacilli and mycobacterium. Management of neonatal mastitis consists of intravenous antibiotics and warm compresses. Beta-lactamase-resistant antibiotics are a reasonable empirical initial treatment pending culture results. If the child develops fluctuation, then needle aspiration or incision and drainage should be done through a circumareolar incision taking care not to excise normal breast tissue. Surgical drainage encouraged resolution of the inflammatory process, aids in the recovery of the organism, and assured appropriate drug therapy. The immediate prognosis of neonatal mastitis is excellent though abscess formation requiring incision may be detrimental for later breast development.

1- Turbey WJ, Buntain WL, Dudgeon DL: The surgical management of pediatric breast masses. Pediatrics. 56(5):736-9, 1975
2- Walsh M, McIntosh K: Neonatal mastitis. Clin Pediatr (Phila). 1986 25(8):395-9
3- Efrat M, Mogilner JG, Iujtman M, Eldemberg D, Kunin J, Eldar S: Neonatal mastitis--diagnosis and treatment. Isr J Med Sci. 31(9):558-60, 1995
4- Stricker T, Navratil F, Sennhauser FH: Mastitis in early infancy. Acta Paediatr. 94(2):166-9, 2005
5- Faden H: Mastitis in children from birth to 17 years. Pediatr Infect Dis J. 24(12):1113, 2005

Secondary Hyperparathyroidism

Secondary hyperparathyroidism refers to a condition seen almost exclusively in the context of chronic renal failure. Due to the low plasma concentration of ionized calcium in children with end stage renal disease there is compensatory hyperplasia of the parathyroid glands with subsequent chronic increase in parathyroid hormone levels. This leads to a severe osteopenia known as renal osteodystrophy leading to skeletal deformities. Most children are managed with medication and Vitamin D with efforts to maintain a normal calcium and phosphate concentration. The pathogenesis of this condition is completely or partially corrected after successful renal transplantation. With refractory medical management, symptomatic disease (bone pain, muscle weakness and pruritus) or biochemical signs (hypercalcemia, and high plasma alkaline phosphatase and parathyroid hormone concentrations) the need for parathyroidectomy arises. Management consists of surgical exploration of the neck with removal of four parathyroid glands and immediate autotransplantation into the forearm. Recurrence of symptoms and parathyroid hormone elevation occurs when the patient has a fifth parathyroid gland, with incomplete surgery or more commonly due to recurrent hyperplasia of the grafted gland.

1- Albertucci M, Zielinski CM, Rothberg M, Sterpetti A, Klingman R, Ronk JF: Surgical treatment of the parathyroid gland in patients with end-stage renal disease. Surg Gynecol Obstet. 167(1):49-52, 1988
2- Ross AJ 3rd: Parathyroid surgery in children. Prog Pediatr Surg. 26:48-59, 1991
3- Bessell JR, Proudman WD, Parkyn RF, Disney AP: Parathyroidectomy in the treatment of patients with chronic renal failure: a 10-year review. Br J Surg. 80(1):40-2, 1993
4- Koch Nogueira PC, David L, Cochat P: Evolution of secondary hyperparathyroidism after renal transplantation. Pediatr Nephrol. 14(4):342-6, 2000
5- Tasic V: Management of renal osteodystrophy in children. Turk J Pediatr. 47 Suppl:13-8, 2005

PSU Volume 29 No 04 OCTOBER 2007

Hemoport Fragmentation

Hemoports catheters play a vital role in providing continuous central venous access for such therapy as parenteral alimentation, long-term antibiotics and cancer chemotherapy in children and adults. The tip of the hemoport catheter should lie within the superior vena cava or right atrial junction during placement. Placement can be done through the external or internal jugular vein or using the subclavian vein with the port usually lying infraclavicularly in the anterior chest area. Very rarely fragmentation with embolization of the port catheter can occur specially during removal of the port. Incidence of catheter fractures is 0.1%. Fracture is suspected if the catheter offers resistance to removal and/or the length removed is too short. The fragmented retained catheter can cause endocarditis, thrombosis, pulmonary abscess, dysrhythmia or sudden death. Causes of fracture include manufacturing defect, mechanical trauma, excessive hydrostatic pressure when flushing or infusing, material degradation, stress due to constant motion, deposition of fibrin, clot or calcium within the catheter, or pinching between the clavicle and the first rib. The fragmented catheter should be differentiated from a calcified "ghost" cast by CT-Scan. The fragmented catheter can stay within the vascular vessel, or embolized into the right heart or pulmonary arteries. Management should consist of percutaneous endovascular retrieval by an invasive cardiologist or radiologist.

1- Klotz HP, Schopke W, Kohler A, Pestalozzi B, Largiader F: Catheter fracture: a rare complication of totally implantable subclavian venous access devices. J Surg Oncol. 62(3):222-5, 1996
2- Fuenfer MM, Georgeson KE, Cain WS, Colvin EV, Johnson WH Jr, Pearce FB, McMahon WS: Etiology and retrieval of retained central venous catheter fragments within the heart and great vessels of infants and children. J Pediatr Surg. 33(3):454-6, 1998
3- Jones SA, Giacomantonio M: A complication associated with central line removal in the pediatric population: retained fixed catheter fragments. J Pediatr Surg. 38(4):594-6, 2003
4- Giretti R, Caruselli M, Zannini R, de Vivo E, Piattellini G, Bechi P, Catani F, Amici M, Santelli F, Pagni R: Dislocation of central venous catheters in paediatric patients. J Vasc Access. 7(3):132-5, 2006
5- Wilson GJ, van Noesel MM, Hop WC, van de Ven C: The catheter is stuck: complications experienced during removal of a totally implantable venous access device. A single-center study in 200 children. J Pediatr Surg. 41(10):1694-8, 2006
6- Hughes DB, Ullery BW, Spigland N: Formation of a calcified "cast" in a long-term indwelling central venous catheter: a case report. J Pediatr Surg. 41(11):1927-9, 2006

Psoas Abscess

Psoas abscess refers to a suppurative infection confined beneath the fascia of the psoas muscle. They occur rarely in infants and children.  Psoas abscess are predominantly primary meaning without another focus of infection in the body of the child. They are seen predominantly in younger children of developing tropical countries. Most primary psoas abscess are caused by Staphylococcus Aureus. Secondary psoas abscess is more common in Europe and North America with predominantly enteric bacterias and occurs after extension from Crohn's disease, appendicitis, cancer, urological infections or from an osseous source like spine, ileum or sacroiliac joint. The clinical syndrome can be confuse and presents with hip pain, and limping with flexion of the hip, positive psoas sign, or inability to ambulate. A mass associated with tenderness is palpable in the iliac fossa, lower abdomen or pelvis or the inguinal region. Peritoneal irritation is uncommon since the fascia covering the psoas muscle prevents spreading of abscess to retroperitoneum or free peritoneal space. Fever, elevated ESR and leukocytosis are almost always presenting signs. Either ultrasound or more specifically CT-Scan will reveal the diagnosis. Management consists of intravenous antibiotics and adequate drainage. Drainage can be accomplished with percutaneous techniques or extraperitoneal operative drainage. Morbidity and mortality are related to delay in management.

1- Santaella RO, Fishman EK, Lipsett PA: Primary vs secondary iliopsoas abscess. Presentation, microbiology, and treatment. Arch Surg. 130(12):1309-13, 1995
2- Lucas AP, Carneiro R, Viegas M, Pascoal J, Sousa-Santos A: Ilio-psoas abscess in the neonate. Eur J Pediatr Surg. 7(3):186-7, 1997
3- Kadambari D, Jagdish S: Primary pyogenic psoas abscess in children. Pediatr Surg Int. 16(5-6):408-10, 2000
4- Kleiner O, Cohen Z, Barki Y, Mares AJ: Unusual presentation of psoas abscess in a child. J Pediatr Surg. 36(12):1859-60, 2001
5- Al-Shaikhi A, Shaw K, Laberge JM: A rapidly growing flank mass in an infant. J Pediatr Surg. 38(9):1415-7, 2003
6- Yano T, Takamatsu H, Noguchi H, Tahara H, Kaji T, Saruwatari Y, Sakai I: Iliopsoas abscess in the neonate. J Pediatr Surg. 39(7):e13-5, 2004
7- Katara AN, Shah RS, Bhandarkar DS, Unadkat RJ: Retroperitoneoscopic drainage of a psoas abscess. J Pediatr Surg. 39(9):e4-5, 2004
8- Garner JP, Meiring PD, Ravi K, Gupta R: Psoas abscess - not as rare as we think? Colorectal Dis. 9(3):269-74, 2007

Eosinophilic Esophagitis

Eosinophilic esophagitis is a primary pediatric disorder characterized by severe isolated eosinophilic infiltration of the esophagus unresponsive to acid blockade but responsive to removal of dietary food allergens. Infants and children demonstrate symptoms of vomiting, failure to thrive, abdominal pain, while older children and adults complain of dysphagia, heartburn, food impaction and chest pain.  They can also present with food allergen sensitization, atopic disorders, allergic rhinitis, asthma, elevated IgE levels and peripheral eosinophilia. Esophagogram can demonstrate a distal stricture making the differential diagnosis include reflux stricture. The diagnosis is made during esophagoscopy with multiple biopsy showing more than 15 eosinophils per high power field. Goal of therapy is to alleviate signs and symptoms. This includes diet modifications to avoid allergens, elemental diets,  systemic steroids, swallowed aerolized topical steroids, montelukast (a leukotriene-receptor antagonist), and histamine receptors antagonists. Once the child or adult develops a stricture that does not responds to medical or dietary management esophageal dilatation is a therapeutic option. A new emerging therapy includes neutralizing antibodies against human interleukin-5.

1- Liacouras CA, Spergel JM, Rucheli E, et al: Eosinophilic Esophagitis: A 10-year experience in 381 children. Clinical Gastroenterology and Hepatology 3: 1198-1206, 2005
2- Castell DO: Eosinophilic Esophagitis. Practical Gastroenterology. September 2005, pags. 41-48
3- Noel RJ, Rothenberg ME: Eosinophilic esophagitis. Curr Opin Pediatr. 17(6):690-4, 2005
4- Yan BM, Shaffer EA: Eosinophilic esophagitis: a newly established cause of dysphagia. World J Gastroenterol. 12(15):2328-34, 2006
5- Stein ML, Collins MH, Villanueva JM, Kushner JP, Putnam PE, Buckmeier BK, Filipovich AH, Assa'ad AH, Rothenberg ME: Anti-IL-5 (mepolizumab) therapy for eosinophilic esophagitis. J Allergy Clin Immunol. 118(6):1312-9, 2006
6- Shah A, Hirano I: Treatment of eosinophilic esophagitis: Drugs, diet or dilatation? Current Gastroenterology Reports 9: 181-188, 2007

PSU Volume 29 NO 05 NOVEMBER 2007

Splenic Hemangiomas

Vascular tumors of the spleen in infants and children include in order of higher frequency hemangiomas, littoral cell angiomas, angiosarcomas, and hamartomas. Splenic hemangiomas are rare benign tumors that present clinically with enlargement of the spleen, thrombocytopenia and microangiopathic hemolytic anemia. Initial work-up should include ultrasound and computed tomography scans of the abdomen. MRI is very helpful since can diagnose the hemangioma and help it differentiate from a hamartoma of the spleen. At MRI splenic hemangiomas showed signal intensity characteristics and enhancement patterns similar to those described for other body hemangiomas, while hamartomas demonstrated diffuse heterogeneous enhancement on images obtained early after administration of contrast material and became more uniformly enhanced on delayed images. Scintigraphic studies are mandatory to confirm diagnosis. Primary hemangiosarcoma of the spleen is an aggressive neoplasia which occurs in a previously splenic benign hemangioma. In such cases children present with symptoms of recurrent fever, malaise, and abdominal pain. Management consists of total splenectomy. Should the hemangioma be massive preoperative splenic artery embolization can be achieved. Antiangiogenic therapy has been reported efficacious in large lesions.

1- Sordillo EM, Sordillo PP, Hajdu SI: Primary hemangiosarcoma of the spleen: report of four cases. Med Pediatr Oncol. 9(4):319-24, 1981
2- Ramani M, Reinhold C, Semelka RC, Siegelman ES, Liang L, Ascher SM, Brown JJ, Eisen RN, Bret PM: Splenic hemangiomas and hamartomas: MR imaging characteristics of 28 lesions. Radiology. 202(1):166-72, 1997
3- Arber DA, Strickler JG, Chen YY, Weiss LM: Splenic vascular tumors: a histologic, immunophenotypic, and virologic study. Am J Surg Pathol. 21(7):827-35, 1997
4- Pampin C, Devillers A, Treguier C, Fremond B, Moisan A, Goasguen J, Le Gall E: Intratumoral consumption of indium-111-labeled platelets in a child with splenic hemangioma and thrombocytopenia. J Pediatr Hematol Oncol. 22(3):256-8, 2000
5- Goyal A, Babu SN, Kim V, Katariya S, Rao KL: Hemangioendothelioma of liver and spleen: trauma-induced consumptive coagulopathy. J Pediatr Surg. 37(10):E29, 2002
6- Islam S, Newman EA, Strouse PJ, Geiger JD: Antiangiogenic therapy for a large splenic hemangioma. Pediatr Surg Int. 21(12):1007-10, 2005

Pancreatic Carcinoma

Tumors of the pancreas can arise from exocrine or endocrine cells. Adenocarcinoma of the pancreas is an extremely rare tumor found during the pediatric age. Most adenocarcinomas of the pancreas are non-islet cell lesions of ductal origin with more than 70% located in the head of the pancreas. Others are acinar cell carcinomas and nonfunctional islet cell carcinomas. More than half the cases are females with a mean age of nine years (range three months to 18 years). As in adults this is a very aggressive malignant neoplasm with early metastatic spread to lymph nodes and liver. While the majority of pancreatic tumors are exocrine lesions of ductal origin, acinar cell tumors are more commonly observed in children and associated with a better prognosis. Children with pancreatic ductal carcinoma presents with abdominal and back pain, vomiting, obstructive jaundice, a palpable mass and weight loss. Diagnostic imaging should consist of ultrasound, CT-Scan and MRI to look for chances of surgical resectability. Genetic markers such as CEA, C19-9, and alpha fetoprotein should be obtained. Whenever feasible and in the event of no metastatic disease management should consist of surgical resection of the tumor. Body and tail tumors can be dealt with distal pancreatectomy, while head of pancreas tumors will need pancreaticoduodenectomy (Whipple procedure). The response rate with chemotherapy and/or radiotherapy is very poor. The prognosis with metastatic disease is dismal.

1- Smith JH 3rd, Baugh C, Reppun T: Obstructive jaundice secondary to pancreatic adenocarcinoma in a seven-year-old male. J Pediatr Surg. 20(2):184-5, 1985
2- Newman K, Vates T, Duffy L, Anderson K: Pancreatoduodenectomy with preservation of the stomach and pylorus: a safe and effective alternative in children.  Pediatr Surg. 27(10):1334-5, 1992
3- Vejcho S: Carcinoma of the pancreas in childhood: a case report of long term survival. J Med Assoc Thai. 76(3):177-83, 1993
4- Le Borgne J, de Calan L, Partensky C: Cystadenomas and cystadenocarcinomas of the pancreas: a multiinstitutional retrospective study of 398 cases. French Surgical Association. Ann Surg. 230(2):152-61, 1999
5- Shorter NA, Glick RD, Klimstra DS, Brennan MF, Laquaglia MP: Malignant pancreatic tumors in childhood and adolescence: The Memorial Sloan-Kettering experience, 1967 to present. J Pediatr Surg. 37(6):887-92, 2002

Hepatic Lymphangiomas

Lymphangiomas found within the liver are an extremely rare finding exclusively present in children and adolescent. Most reported cases occur as part of a diffuse body involvement with lymphangioma including spleen, kidneys, gastrointestinal tract, mediastinum and lung. Histologically the lesion presents with multiple smooth-walled cysts filled with serous fluid and blood looking like multiple intercommunicating cavernous spaces separated by thin-walled septa and lined by a flattened endothelium. Children develop hepatomegaly and right upper quadrant pain.  Lymphangiomas are benign lesion, but within the liver they can produce symptoms due to progressive compression of vital structures. Diagnostic imaging consists of ultrasound, CT-Scan and MRI. Management consists of resection if a surgical liver anatomic lesion or pedunculated is identified, while total hepatectomy and orthotopic liver transplantation is used with symptomatic diffuse hepatic involvement. Sclerotherapy utilized in other lymphangiomas in the rest of the body is of no benefit with hepatic lesions.

1- Asch MJ, Cohen AH, Moore TC: Hepatic and splenic lymphangiomatosis with skeletal involvement: report of a case and review of the literature. Surgery. 76(2):334-9, 1974
2- Morán Penco JM, Borella F, Garibaldi L, Toma P, Dodero P: Hepatic hemangiolymphangioma in an infant. An Esp Pediatr. 19(5):411-3, 1983
3- Tepetes K, Selby R, Webb M, Madariaga JR, Iwatsuki S, Starzl TE: Orthotopic liver transplantation for benign hepatic neoplasms. Arch Surg. 130(2):153-6, 1995
4- Koh CC, Sheu JC: Hepatic lymphangioma--a case report. Pediatr Surg Int. 16(7):515-6, 2000
5- Chan SC, Huang SF, Lee WC, Wan YL: Solitary hepatic lymphangioma--a case report. Int J Clin Pract Suppl. 147:100-2, 2005
6- Nzegwu MA, Ekenze SO, Okafor OC, Anyanwu PA, Odetunde OA, Olusina DB: Solitary hepatic lymphangioma in an infant. J Perinat Med. 35(2):164-5, 2007

PSU Volume 29 NO 06 DECEMBER 2007

Pleomorphic Adenoma

Less than 5% of all major primary salivary gland neoplasms occur in children. The major salivary glands comprised the parotid, submandibular and sublingual. The first decade of life, and particularly the first two years of life, has a preponderance of benign neoplasms. Most common benign neoplasm affecting the major salivary gland in children are vascular in nature such as hemangiomas, hemangioendotheliomas and lymphangiomas. Pleomorphic adenoma, also known as mixed tumor, is the most common benign epithelial salivary gland tumor throughout childhood. Pleomorphic adenoma usually arises in the lateral portion of the parotid gland, grows slowly as a painless mass with peak ages between nine and 14 years. Most affected cases are males. The mass is usually less than 3 centimeters in diameter, firm and freely mobile. Extremely rarely a pleomorphic adenoma will arise in a submandibular or sublingual gland.  CT Scan and MRI are helpful in determining the extent of the lesion and surgical planning. Fine needle aspiration biopsy has a high accuracy and sensitivity rate revealing the histologic nature of the tumor. Management of pleomorphic adenoma entail excision of the tumor with a rim of normal salivary gland tissue circumferentially. This means superficial or total parotidectomy with nerve preservation or reconstruction if at all possible. Enucleation carries a high percent of recurrence. Redo surgery carries an even higher rate of fascial nerve damage. Long-term follow up is recommended.

1- Callender DL, Frankenthaler RA, Luna MA, Lee SS, Goepfert H: Salivary gland neoplasms in children. Arch Otolaryngol Head Neck Surg. 118(5):472-6, 1992
2- Kessler A, Handler SD: Salivary gland neoplasms in children: a 10-year survey at the Children's Hospital of Philadelphia. Int J Pediatr Otorhinolaryngol. 29(3):195-202, 1994
3- Boccato P, Altavilla G, Blandamura S: Fine needle aspiration biopsy of salivary gland lesions. A reappraisal of pitfalls and problems. Acta Cytol. 42(4):888-98, 1998
4- Garcia-Perla A, Muñoz-Ramos M, Infante-Cossio P, Mayorga-Jimenez F, Gutierrez-Perez JL, Garcia-Perla A: Pleomorphic adenoma of the parotid in childhood. J Craniomaxillofac Surg. 30(4):242-5, 2002
5- Ethunandan M, Ethunandan A, Macpherson D, Conroy B, Pratt C: Parotid neoplasms in children: experience of diagnosis and management in a district general hospital. Int J Oral Maxillofac Surg. 32(4):373-7, 2003
6- Guzzo M, Ferrari A, Marcon I, Collini P, Gandola L, Pizzi N, Casanova M, Mattavelli F, Scaramellini G: Salivary gland neoplasms in children: the experience of the Istituto Nazionale Tumori of Milan. Pediatr Blood Cancer. 47(6):806-10, 2006
7- Rodriguez KH, Vargas S, Robson C, Perez-Atayde A, Shamberger R, McGill TJ, Healy GB, Rahbar R: Pleomorphic adenoma of the parotid gland in children. Int J Pediatr Otorhinolaryngol. 71(11):1717-23, 2007

Bowel Lymphomas

Lymphomas of the gastrointestinal tract are the most common type of primary extranodal lymphomas, accounting for five to 10% of all non-Hodgkin's lymphomas. The most frequent symptoms are abdominal pain, nausea, vomiting and weight loss since they will present clinically as an abdominal mass, bowel obstruction, perforation, bleeding or intussusception. In the majority of children the diagnosis is made at urgent exploration. The tumor is located most commonly in the proximal and distal  terminal ileum. Mean age of diagnosis is nine years with male predominance. The most common type of lymphoma encounter in children is non-endemic Burkitt's lymphoma. Localized disease, low stage disease and complete resection favors survival in lymphoma, whereas age, sex, and urgency of operation had no influence on survival. Spontaneous bowel perforation from the lymphoma or during surgical manipulation increases the chances of dying from the disease and significantly reduce survival due to delay in instituting chemotherapy. The mainstay of management of bowel lymphomas consists of resection and chemotherapy if at all possible while not increasing the rate of perforation.

1- Zinzani PL, Magagnoli M, Pagliani G, Bendandi M, Gherlinzoni F, Merla E, Salvucci M, Tura S: Primary intestinal lymphoma: clinical and therapeutic features of 32 patients. Haematologica. 82(3):305-8, 1997
2- Bethel CA, Bhattacharyya N, Hutchinson C, Ruymann F, Cooney DR: Alimentary tract malignancies in children. J Pediatr Surg. 32(7):1004-8, 1997
3- Yanchar NL, Bass J: Poor outcome of gastrointestinal perforations associated with childhood abdominal non-Hodgkin's lymphoma. J Pediatr Surg. 34(7):1169-74, 1999
4- Pandey M, Kothari KC, Wadhwa MK, Patel HP, Patel SM, Patel DD: Primary malignant large bowel lymphoma. Am Surg. 68(2):121-6, 2002
5- Goldberg SR, Godder K, Lanning DA: Successful treatment of a bowel perforation after chemotherapy for Burkitt lymphoma. J Pediatr Surg. 42(3):E1-3, 2007
6- Ara C, Coban S, Kayaalp C, Yilmaz S, Kirimlioglu V: Spontaneous intestinal perforation due to non-Hodgkin's lymphoma: evaluation of eight cases. Dig Dis Sci. 52(8):1752-6, 2007

Stump Appendicitis

Appendectomy for inflammation of the appendix is one of the most common urgent procedure performed in children and adults. The procedure consists of ligating the sick appendix near the wall of the cecum. Developing inflammation of the remnant stump (residual appendicitis) is a very rare condition that occurs when there has been incomplete resection of the organ and is estimated to be seen more commonly following the advent of laparoscopic appendectomy. The clinical picture is similar to the original disease. The child develops vomiting, fever, epigastric or periumbilical pain that radiates toward the right lower quadrant with leukocytosis. Since they already have an appendectomy, physicians are reluctant to consider this diagnosis again and tend to observe these cases, the reason why the rate of appendiceal stump perforation is extremely high. CT Scan will help demonstrate an inflammatory process in the right lower quadrant with fat stranding or abscess formation. Management consists of re-appendectomy very near the wall of the cecum, cecorrhaphy or right hemicolectomy. Avoidance of a long appendiceal stump is the only suggested means by which to reduce its occurrence.

1- Walsh DC, Roediger WE: Stump appendicitis--a potential problem after laparoscopic appendicectomy. Surg Laparosc Endosc. 7(4):357-8, 1997
2- Gupta R, Gernshiemer J, Golden J, Narra N, Haydock T: Abdominal pain secondary to stump appendicitis in a child. J Emerg Med. 18(4):431-3, 2000
3- Mangi AA, Berger DL: Stump appendicitis. Am Surg. 66(8):739-41, 2000
4- Aschkenasy MT, Rybicki FJ: Acute appendicitis of the appendiceal stump. J Emerg Med. 28(1):41-3, 2005
5- Liang MK, Lo HG, Marks JL: Stump appendicitis: a comprehensive review of literature. Am Surg. 72(2):162-6, 2006
6- Uludag M, Isgor A, Basak M: Stump appendicitis is a rare delayed complication of appendectomy: A case report. World J Gastroenterol. 12(33):5401-3, 2006
7-Yigit T, Mentes O, Eryilmaz M, Balkan M, Uzar AI, Kozak O: Stump resections resulting from incomplete operations. Am Surg. 73(1):75-8, 2007

Journal Club