VOLUME 31, 2008

PSU Volume 31 No 01 JULY 2008

Prophylactic Antibiotics

Antibiotics have an important role in preventing wound infections during emergent and elective surgery. The most critical factors in prevention of postoperative wound infections are sound judgment and proper technique of the surgeon and surgical team. Antibiotics prophylaxis in surgery is governed by such factors as: surgical wound classification (most important),  host immune system function and immune competence, host nutritional status, type and prolongation of the surgical procedure, hospital vs. community emerging ‘difficult to manage’ strains, and emergency vs. elective procedure According to wound type, clean procedures (hernias, excisions, biopsy) need no use of prophylactic antibiotics. Clean contaminated procedures (surgery entering airway, bowel, breast, urinary or bile lumen) should receive one preoperative dose of broad spectrum antibiotic followed by 24 hours postoperative prophylaxis. Contaminated and dirty surgical procedures (empyema, non-prepared bowel perforation, perforated appendicitis, infected urinary tract) should receive preoperative double antibiotic therapy continued according to individual laboratory and clinical condition in the postoperative period. Special considerations such immune, asplenic and nutritional compromised hosts should receive antibiotic prophylaxis. Prolonged procedures beyond three hours should receive an intraoperative dose.  Long-term hospitalized children submitted to surgery should receive high graded antibiotic therapy due to colonization with antibiotic resistant strains.

1- ACS Surgery: Principles and Practice, Chapter 1: Basic Surgical and Perioperative Considerations, pags 9 and 13, 2006
2- Lee Nichols R: Preventing Surgical Site Infections: A Surgeon’s Perspective. Emerging Infectious Diseases, 7(2): 220, 2001
3- Ichikawa S, Ishihara M, Okazaki T, et al: Prospective study of antibiotic protocol for managing surgical site infections in children. Journal of Pediatr Surgery 42: 1002-1007, 2007

Playground Injuries

Children sustaining injuries from playground equipment constitute a common cause of visiting the emergency room of Children hospitals. Injuries are usually minor, but sometimes serious head or extremity injury or even death can occur. Injuries consist of fractures (most common), contusion/abrasions, laceration, hematomas, strains/sprains and brain injury. In order of frequency monkey bars are responsible from the majority of cases, followed very closely by swings and slides. Geographically, they occur with almost the same frequency at school, recreation/sporting facilities and home. Body area injured consist of the upper extremity (most common), followed by head/neck, lower extremity and trunk. Most of the children are managed and released the same day. Monkey bars injuries are usually fractures, while swings at school cause most brain injuries (mechanism: a young child moves behind a moving swing). Upper extremity fractures due to climbing account for the majority of hospitalizations. Older children sustained more injuries on climbing apparatus, where younger children sustained more injuries on slides. Removing and replacing unsafe equipment is an effective strategy for preventing playground injuries.

1  Lillis KA, Jaffe DM: Playground injuries in children. Pediatr Emerg Care. 13(2):149 53, 1997
2  Macarthur C, Hu X, Wesson DE, Parkin PC: Risk factors for severe injuries associated with falls from playground equipment. Accid Anal Prev. 32(3):377 82, 2000
3  Phelan KJ, Khoury J, Kalkwarf HJ, Lanphear BP: Trends and patterns of playground injuries in United States children and adolescents. Ambul Pediatr. 1(4):227 33, 2001
4  Howard AW, MacArthur C, Willan A, Rothman L, Moses McKeag A, MacPherson AK: The effect of safer play equipment on playground injury rates among school children. CMAJ. 172(11):1443 6, 2005
5  Loder RT: The demographics of playground equipment injuries in children. J Pediatr Surg. 43(4):691 9, 2008

Internal Hernias

Internal hernias are a rare cause of bowel obstruction (5%) in children and adults. These mesenteric defects are most commonly acquired or congenital in nature. Acquired internal hernias occur postoperatively resulting from incomplete closure of surgically created mesenteric defects. Congenital mesenteric defects represent 10% of all internal hernias. Pathogenesis of these congenital defects includes regression of the dorsal mesentery, enlargement of a hypovascular area and compression of mesentery by the colon. Most congenital mesenteric defects that lead to herniation occur in the small bowel mesentery, are 2 to 3 cm wide and trap a loop of ileum. Herniation of small bowel through the falciform ligament and Winslow’s foramen has also been reported. Clinically, children shows sign of intermittent small bowel obstruction, nausea, vomiting, abdominal pain and distension which can follow into bowel incarceration or strangulation. Preoperative diagnosis is difficult and a high index of suspicion is needed to order appropiate studies for diagnosis such as upper bowel contrast studies or CT-Scan. Misdiagnosis results in delayed exploration which leads to bowel necrosis and death. Surgical exploration is the only means of definitive diagnosis.

1  Ozen? A, Ozdemir A, Coskun T: Internal hernia in adults. Int Surg. 83(2):167 70, 1998
2  Moran JM, Salas J, Sanjußn S, Amaya JL, Rinc?n P, Serrano A, Tallo EM: Paramesocolic hernias: consequences of delayed diagnosis. Report of three new cases. J Pediatr Surg. 39(1):112 6, 2004
3  Agresta F, Michelet I, Candiotto E, Bedin N: Incarcerated internal hernia of the small intestine through a breach of the broad ligament: two cases and a literature review. JSLS. 11(2):255 7, 2007
4  Mboyo A, Goura E, Massicot R, Flurin V, Legrand B, Repetto Germaine M, Caron Bataille S, Ndi? J: An exceptional cause of intestinal obstruction in a 2 year old boy: strangulated hernia of the ileum through Winslow's foramen. J Pediatr Surg. 43(1):e1 3, 2008
5- Gingalewski C, Lalikos J: An unusual cause of small bowel obstruction: herniation through a defect in the falciform ligament. J Pediatr Surg. 43(2):398 400, 2008
6  Page MT, Ricca RL, Resnick AS, Puder M, Fishman SJ: Newborn and toddler intestinal obstruction owing to congenital mesenteric defects. J Pediatr Surg 43(4): 755 758, 2008

PSU VOL 31 NO 02 AUGUST 2008

Visceral Myopathy

Bowel dysmotility leading to severe intractable constipation in children is a very serious unsolved condition in pediatric surgery. Constipation needs initial rectal biopsy to determine if the child has ganglion cells or not present. As histopathological studies increase their diagnostic acuity we have a more relevant understanding of the physiology of altered motility in the small and large intestine. One of those components are the interstitial cell of Cajal (ICC) of the bowel. ICC are known to be essential regulators of gastrointestinal motility, they are called the pacemaker cells of the smooth muscle of the gastrointestinal tract. Studies have revealed reduced numbers or the absence of ICC in small intestine and colon that do not exhibit normal peristaltic activity (chronic idiopathic constipation). In patients with slow-transit constipation, the number of ICC is significantly decreased in all layers except the outer longitudinal muscle layer, while the myenteric plexus shows moderate hypoganglionosis. Persistent dysmotility problems after pull-through operation in aganglionosis may be due to altered distribution and impaired function of ICC. Delay in the development of ICC in the gastrointestinal tract may be a cause of intestinal pseudoobstruction in the newborn. Such conditions as pyloric stenosis, Hirschsprung's disease, hypoganglionosis, intestinal neuronal dysplasia, internal anal sphincter achalasia, megacystis microcolon intestinal hypoperistalsis syndrome have been reported to be associated with loss or deficiency of ICC.

1- Huizinga JD: Neural injury, repair, and adaptation in the GI tract. IV. Pathophysiology of GI  motility related to interstitial cells of Cajal. Am J Physiol. 275(3 Pt 1):G381-6, 1998
2-  Wedel T, Spiegler J, Soellner S, Roblick UJ, Schiedeck TH, Bruch HP, Krammer HJ:
 Enteric nerves and interstitial cells of Cajal are altered in patients with slow-transit constipation and megacolon. Gastroenterology. 123(5):1459-67, 2002
3- Rolle U, Piotrowska AP, Nemeth L, Puri P: Altered distribution of interstitial cells of Cajal in Hirschsprung disease. Arch Pathol Lab Med. 126(8):928-33, 2002
4- Rolle U, Yoneda A, Solari V, Nemeth L, Puri P.: Abnormalities of C-Kit-positive cellular network in isolated hypoganglionosis. J Pediatr Surg. 37(5):709-14, 2002
5- Kenny SE, Vanderwinden JM, Rintala RJ, Connell MG, Lloyd DA, Vanderhaegen JJ, De Laet MH: Delayed maturation of the interstitial cells of Cajal: a new diagnosis for transient neonatal pseudoobstruction. Report of two cases. J Pediatr Surg. 33(1):94-8, 1998
6- Rolle U, Piaseczna-Piotrowska A, Puri P: Interstitial cells of Cajal in the normal gut and in intestinal motility disorders of childhood. Pediatr Surg Int. 23(12):1139-52, 2007

Endoscopic Injuries

The volume of gastrointestinal endoscopies done to children yearly has increased considerably over the last ten years. More children undergo diagnostic and therapeutic upper, lower and ERCP endoscopies. Most therapeutic endoscopic procedures are done on an ambulatory basis. The incidence of complications is near 0.06 to 0.5% depending on the procedure. Perforation of the colon during colonoscopy is the most serious endoscopic related injury in children due to the need of operative intervention from bacterial seeding and peritonitis. Upper endoscopy and ERCP entails complications such as mucosal tear, bleeding and bowel perforation. Most cases are amenable to nonoperative therapy. Bleeding complications might need transfusion, cauterization, endoscopic hemoclip or angiographic embolization. Perforation of the duodenum may need prompt surgical repair as they cause rapid chemical and bacterial peritonitis. Esophageal perforations are amenable to observation. Needless to say endoscopic procedures in children are very safe with a low incidence of complications. The more complicated the procedure, the higher the incidence of complications. Surgeons are always verb-called to assist such complications. Patients who survive the initial complications have excellent long-term outcomes.

1- Panieri E, Millar AJ, Rode H, Brown RA, Cywes S: Iatrogenic esophageal perforation in children: patterns of injury, presentation, management, and outcome. J Pediatr Surg. 31(7):890-5, 1996
2- Enns R, Eloubeidi MA, Mergener K, Jowell PS, Branch MS, Pappas TM, Baillie J: ERCP-related perforations: risk factors and management. Endoscopy. 34(4):293-8, 2002
3- Cobb WS, Heniford BT, Sigmon LB, Hasan R, Simms C, Kercher KW, Matthews BD: Colonoscopic perforations: incidence, management, and outcomes. Am Surg. 70(9):750-7, 2004
4- Abadir J, Emil S, Nguyen N:  Abdominal foregut perforations in children: a 10-year experience. J Pediatr Surg. 40(12):1903-7, 2005
5- Iqbal CW, Askegard-Giesmann JR, Pham TH, Ishitani MB, Moir CR: Pediatric endoscopic injuries: incidence, management, and outcomes. J Pediatr Surg. 43(5):911-5, 2008

Snowboard Injuries

We don't have snow in the tropics, but yearly thousands of kids suffer from snowboard injury. Unlike skiing, snowboard is a relatively new sport with a dramatic rise in popularity associated with serious injuries. Snowboarding males are more commonly affected than females, while skiers have a longer length of hospital stay. Skiers and snowboarder both sustain in order of higher frequency head, extremity (skeletal), and intra-abdominal injuries. Two-third occurs at licensed resorts, and one-third at parks or private property. Head trauma is the leading cause of death among both groups of sports. Curiously, helmet are required for all international snowboarding competitions, while is not usually utilized in recreational resorts. Risk of snowboard related injury was highest in beginners. Mean severity injury score and splenic injuries (snowboard spleen) are more commonly found in snowboarder than skiers since they do more aerial jumping maneuvers with a higher incidence of traumatic falls. Snowboarders who wore protective wrist guards were half as likely to sustain wrist injuries as those who did not wear guards. Elite snowboarders suffer more from knee than wrist injury.

1- Hackam DJ, Kreller M, Pearl RH: Snow-related recreational injuries in children: assessment of morbidity and management strategies. J Pediatr Surg. 35(9):1409-10, 2000
2- Machold W, Kwasny O, G ssler P, Kolonja A, Reddy B, Bauer E, Lehr S: Risk of injury through snowboarding. J Trauma. 48(6):1109-14, 2000
3- Idzikowski JR, Janes PC, Abbott PJ: Upper extremity snowboarding injuries. Ten-year results from the Colorado snowboard injury survey. Am J Sports Med. 28(6):825-32, 2000
4- Bladin C, McCrory P, Pogorzelski A: Snowboarding injuries : current trends and future directions. Sports Med. 34(2):133-9, 2004
5- Hagel B: Skiing and snowboarding injuries. Med Sport Sci. 48:74-119, 2005
6- Hayes JR, Groner JI: The increasing incidence of snowboard-related trauma. J Pediatr Surg. 43(5):928-30, 2008

PSU Volume 31 No 03 SEPTEMBER 2008

Jeune Syndrome

The two most common chest wall deformities in pediatric surgery are pectus excavatum and pectus carinatum. Pectus excavatum manifests itself during early childhood, while pectus carinatum is seen more commonly in early adolescent ages. Jeune syndrome is a rare autosomal recessive chest wall skeletal dysplasia also known as asphyxiating thoracic dystrophy or insufficiency. Almost all cases of Jeune syndrome has been described in children. The syndrome is characterized by respiratory distress, osseous dysplasia, and short stature. Patients generally die during the first months of life since the thoracic chest wall will not grow creating entrapment of lung and asphyxia. Besides a small thorax, varying degrees of rhizomelic brachymelia, polydactyly, pelvic abnormalities, renal anomalies, cystic lesions of the pancreas, retinal anomalies and hepatic fibrosis are also present in the syndrome. Diagnosis can be made prenatally. The life-saving procedures to expand the chests of infants born with Jeune asphyxiating thoracic dystrophy provide a static solution incapable of responding to the growth demands of thriving patients. Actual management consist either of dynamic lateral thoracic expansion with titanium struts or vertical expandable prosthetic titanium rib thoracoplasty creating additional chest wall that is formed of autologous tissue.

1- Giorgi PL, Gabrielli O, Bonifazi V, Catassi C, Coppa GV: Mild form of Jeune syndrome in two sisters. Am J Med Genet. 35(2):280-2, 1990
2- Chen CP, Lin SP, Liu FF, Jan SW, Lin SY, Lan CC: Prenatal diagnosis of asphyxiating thoracic dysplasia (Jeune syndrome). Am J Perinatol.  13(8):495-8, 1996
3- Kaddoura IL, Obeid MY, Mroueh SM, Nasser AA: Dynamic thoracoplasty for asphyxiating thoracic dystrophy. Ann Thorac Surg. 72(5):1755-8, 2001
4- Yerian LM, Brady L, Hart J: Hepatic manifestations of Jeune syndrome (asphyxiating thoracic dystrophy). Semin Liver Dis. 23(2):195-200, 2003
5- Davis JT, Long FR, Adler BH, Castile RG, Weinstein S: Lateral thoracic expansion for Jeune syndrome: evidence of rib healing and new bone formation. Ann Thorac Surg. 77(2):445-8, 2004
6- Waldhausen JH, Redding GJ, Song KM: Vertical expandable prosthetic titanium rib for thoracic insufficiency syndrome: a new method to treat an old problem. J Pediatr Surg. 42(1):76-80, 2007

Pectus Excavatum

Pectus excavatum (funnel chest) is the most common chest wall deformity in children. The depression in the sternum in relation to the costal cartilages causes a distressful cosmetic defect with mild to no changes in pulmonary and cardiac function. Symptoms of lack of endurance, shortness of breath with exercise, or chest pain are frequent. Indications for surgical treatment includes severe, symptomatic deformity; paradoxical respiratory chest wall motion; CT scan with a pectus index greater than 3.25, cardiac compression/displacement, pulmonary compression, pulmonary function studies showing restrictive disease, or other cardiac pathology secondary to compression of the heart. Originally repaired open using the Ratvich technique of costal cartilage removal, this has fallen into disuse and replaced with a minimally invasive technique called Nuss procedure where a titanium bar is placed behind the sternum and fixed to each side of the thoracic wall for two years before removal. Thoracoscopy while placing the bar behind the sternum helps avoid significant complications. Though the complications reported with the Nuss procedure are minimal, a few of them are significant such as laceration of the internal mammary artery, hemopericardium, recurrence of the pectus deformity after bar removal and bar displacement. There is a 1.5% incidence of postoperative wound infection. Surgical repair of the pectus excavatum improves cardiovascular function but there is no significant improvement in pulmonary function. The value of routine testing of pre- and postoperative lung function in patients with pectus excavatum is questionable. Good sternal elevation as measured by preoperative and postoperative CT scans can be achieved with the Nuss procedure regardless of the severity of chest depression or age. Results are good with excellent cosmetic results. Minimally invasive repair of pectus excavatum procedure and removal of the pectus bar should only occur in specialized institutions with wide experience in thoracic surgery.

1- Malek MH, Berger DE, Housh TJ, Marelich WD, Coburn JW, Beck TW: Cardiovascular function following surgical repair of pectus excavatum: a metaanalysis. Chest. 130(2):506-16, 2006
2- Shin S, Goretsky MJ, Kelly RE Jr, Gustin T, Nuss D: Infectious complications after the Nuss repair in a series of 863 patients. J Pediatr Surg. 42(1):87-92, 2007
3- Aronson DC, Bosgraaf RP, Merz EM, van Steenwijk RP, van Aalderen WM, van Baren R: Lung function after the minimal invasive pectus excavatum repair (Nuss procedure). World J Surg. 31(7):1518-22, 2007
4- Vegunta RK, Pacheco PE, Wallace LJ, Pearl RH: Complications associated with the Nuss procedure: continued evolution of the learning curve. Am J Surg. 195(3):313-6, 2008
5- Nakagawa Y, Uemura S, Nakaoka T, Yano T, Tanaka N: Evaluation of the Nuss procedure using pre- and postoperative computed tomographic index. J Pediatr Surg. 43(3):518-21, 2008
6- Kelly RE Jr.: Pectus excavatum: historical background, clinical picture, preoperative
evaluation and criteria for operation. Semin Pediatr Surg. 17(3):181-93, 2008
7- Nuss D: Minimally invasive surgical repair of pectus excavatum. Semin Pediatr Surg. 17(3):209-17, 2008

Trocar Hernias

With the advent of minimally invasive procedures the trocar (port site) hernia has emerged. A trocar hernia is an abdominal defect caused by the sheath of the trocar through which omentum or even small bowel can protrude (incisional hernia). Trocar hernias occur in 3% of all laparoscopic procedures. Risk factors which increase the development of a trocar hernia are: large trocar (10 mm or larger), trocar design, use of linea alba for port placement, pre-existing fascial defect, pre-school children and skinny constitution. When small bowel protrudes a Richter's hernia develops. The usual presentation is of crampy abdominal pain with nausea and vomiting. Treatment is reduction of the bowel that is incarcerated and then repair of the fascial defect. To avoid trocar hernias fascial closure of port sites of 10-mm in adults and 5-mm in children should be accomplish whenever possible. Trocar site hernias in infants are mainly of omental protrusion and occur within the first postoperative week.

1- Holzinger F, Klaiber C: Trocar site hernias. A rare but potentially dangerous complication of laparoscopic surgery. Chirurg. 73(9):899-904, 2002
2- Boughey JC, Nottingham JM, Walls AC: Richter's hernia in the laparoscopic era: four case reports and review of the literature. Surg Laparosc Endosc Percutan Tech. 13(1):55-8, 2003
3- Tonouchi H, Ohmori Y, Kobayashi M, Kusunoki M: Trocar site hernia. Arch Surg. 139(11):1248-56,2004
4- Mahmoud Uslu HY, Ustuner EH, Sozener U, Ozis SE, Turkcapar AG: Cannula site insertion technique prevents incisional hernia in laparoscopic fundoplication. Surg Laparosc Endosc Percutan Tech. 17(4):267-70, 2007
5- Paya K, Wurm J, Fakhari M, Felder-Puig R, Puig S: Trocar-site hernia as a typical postoperative complication of minimally invasive surgery among preschool children. Surg Endosc. Feb 13,2008

PSU Volume 31 No 04 OCTOBER 2008

Ethanol Lock

Children needing long-term parenteral nutrition or chemotherapy will do so with the help of a central venous catheter (CVC). With each episode of thrombosis or catheter-related infection (CRI) the rate of catheter turnover will increase and fewer veins will be accessible for such purposes. Most CRI can be managed with systemic antibiotics without removing the catheter. The use of 70% ethanol lock solution has been described for prevention and management of catheter-related infections in children and adults. Ethanol lock denatures proteins and is rapidly bactericidal and fungicidal in vitro, exhibits thrombolytic effects eliminating the need to maintain catheter patency. Ethanol lock has been used to dislodge lipids clots in occluded catheters. Patients who received prophylactic ethanol lock therapy have a 95% decrease in catheter replacements required. The use of antibiotic-lock therapy for CRI prevention has the theoretical disadvantage of increased antibiotic resistance. The ethanol lock volume can be something between 0.5 and 1 ml. Whether degradation of silicone catheters occurs with repeated ethanol instillation is unknown, but it should not be used with polyurethane catheters as it degrades the internal lumen. No severe clinical side effects of ethanol flush are observed. Mild symptoms that could occur include tiredness, headaches, dizziness, nausea, and light-headedness.

1- Dannenberg C, Bierbach U, Rothe A, Beer J, Körholz D: Ethanol-lock technique in the treatment of bloodstream infections in pediatric oncology patients with broviac catheter. J Pediatr Hematol Oncol. 25(8):616-21, 2003
2- Crnich CJ, Halfmann JA, Crone WC, Maki DG: The effects of prolonged ethanol exposure on the mechanical properties of polyurethane and silicone catheters used for intravascular access. Infect Control Hosp Epidemiol. 26(8):708-14, 2005
3- Onland W, Shin CE, Fustar S, Rushing T, Wong WY: Ethanol-lock technique for persistent bacteremia of long-term intravascular devices in pediatric patients. Arch Pediatr Adolesc Med. 160(10):1049-53, 2006
4- Opilla MT, Kirby DF, Edmond MB: Use of ethanol lock therapy to reduce the incidence of catheter-related bloodstream infections in home parenteral nutrition patients. JPEN J Parenter Enteral Nutr. 31(4):302-5, 2007
5- Broom J, Woods M, Allworth A, McCarthy J, Faoagali J, Macdonald S, Pithie A: Ethanol lock therapy to treat tunnelled central venous catheter-associated blood stream infections: results from a prospective trial. Scand J Infect Dis. 40(5):399-406, 2008
6- Mouw E, Chessman K, Lesher A, Tagge E: Use of an ethanol lock to prevent catheter-related infections in children with short bowel syndrome. J Pediatr Surg. 43(6):1025-9, 2008

Splenectomy for Sickle Cell Disease

Sickle cell disease (SCD) is a chronically hemolytic debilitating condition causing recurrent packed blood cell transfusions in children to manage or prevent complications such as anemia, stroke, acute chest syndrome, severe pain syndrome or splenic sequestration. The major indication for splenectomy in children with SCD consists of recurrent splenic sequestrations' episodes and severe hemolysis due to secondary hypersplenism. Clinically the sequestration is characterized by hypovolemia, worsening anemia, extreme reticulocytosis and a tender enlarged spleen. Hypersplenism is characterized by anemia, thrombocytopenia, or neutropenia usually associated with splenomegaly. Restoration of blood volume and circulating red cell mass forms the basis of management of sequestration episodes and hypersplenism in SCD children. Splenic sequestrations occur more often in children less than six years of age. Total laparoscopic splenectomy is recommended after managing the acute event to prevent further recurrences of splenic sequestration crisis and reverse hypersplenism. Lap splenectomy, even in functional asplenic (small spleens) individuals reduce the transfusion requirements (reducing iron overload), maintaining stable hematologic parameters and decreasing red cell turnover. The rate of postoperative acute chest syndrome is smaller with laparoscopic than open splenectomy. The risk of overwhelming postsplenectomy sepsis crisis has decreased significantly over the years due to preoperative immunization and use of prophylactic antibiotics.

1- Hendricks-Ferguson VL, Nelson MA: Laparoscopic splenectomy for splenic sequestration crisis. AORN J. 71(4):820-2, 2000
2- Overturf GD: Pneumococcal vaccination of children. Semin Pediatr Infect Dis. 13(3):155-64, 2002
3- Al-Salem AH: Indications and complications of splenectomy for children with sickle cell disease. J Pediatr Surg. 41(11):1909-15, 2006
4- Rescorla FJ, West KW, Engum SA, Grosfeld JL: Laparoscopic splenic procedures in children: experience in 231 children. Ann Surg. 246(4):683-7, 2007
5- Ghantous S, Al Mulhim S, Al Faris N, Abushullaih B, Shalak F, Yazbeck S: Acute chest syndrome after splenectomy in children with sickle cell disease. J Pediatr Surg. 43(5):861-4, 2008
6- Haricharan RN, Roberts JM, Morgan TL, Aprahamian CJ, Hardin WD, Hilliard LM, Georgeson KE, Barnhart DC: Splenectomy reduces packed red cell transfusion requirement in children with
sickle cell disease. J Pediatr Surg. 43(6):1052-6, 2008

Pulmonary Arteriovenous Fistula

Congenital pulmonary arteriovenous fistula (PAVF) is a rare vascular malformation where an abnormal connection develops between an artery and a vein in the lung of a child. The arteriovenous connection can be tortuous or direct aneurysmal and lacks an intervening capillary bed. Females are more often affected than males. PAVF can be either large or small, multiple or diffuse and unilateral or bilateral. The congenital variant of PAVF can be caused by a cavernous hemangioma, while the acquired form occurs after heart surgery, trauma, actinomycosis, amyloidosis, hepatic cirrhosis or cystic fibrosis. Symptoms include cyanosis, digital clubbing, exertional dyspnea, hemoptysis and hemothorax. Most PAVF cases affect the lower lobes. Diagnosis is made with angiography or 3-dimension spiral CT. Main indication for surgery is hypoxemia and prevention of neurological sequelae due to embolization. Management can entail segmentectomy, lobectomy, pneumonectomy, or recently the use of embolization with metal coils or silicon balloons. Excision has good results when the PAVF is single, large and located in one lobe.

1- Sheikhzadeh A, Paydar MH, Ghabussi P, Hashemian M, Yazdanyar A, Hakim HS: Pulmonary arteriovenous fistulas. Case presentations and clinical recognition. Herz. 8(3):179-86, 1983
2- Batinica S, Gagro A, Bradic I, Marinovic B: Congenital pulmonary arteriovenous fistula: a rare cause of cyanosis in childhood. Thorac Cardiovasc Surg. 39(2):105-6, 1991
3- Fiane AE, Stake G, Lindberg HL: Congenital pulmonary arteriovenous fistula. Eur J Cardiothorac Surg. 9(3):166-8, 1995
4- Bernstein HS, Brook MM, Silverman NH, Bristow J: Development of pulmonary arteriovenous fistulae in children after cavopulmonary shunt. Circulation. 92(9 Suppl):II309-14, 1995
5- Fraga JC, Favero E, Contelli F, Canani F: Surgical treatment of congenital pulmonary arteriovenous fistula in children. J Pediatr Surg. 43(7):1365-7, 2008

PSU Volume 31 No 05 NOVEMBER 2008

Reperfusion Injury

Hypovolemic shock whether hemorrhagic (trauma) or septic is the leading cause of morbidity and mortality in intensive care units. Reperfusion injury commences after physicians reoxygenate tissue that has undergo ischemic insults caused by hypoxia. The problem starts in the endothelial cells bed, since these cells are very sensitive to hypoxia and manifest damage by increasing cellular volume, loss of cytoskeletal organization, loss of adherence to the basement membrane, decreases in membrane fluidity and adherence of activated leukocytes. The location of this endothelial damage occurs at the level of arterioles, capillaries and venules. Specifically this last (venules) are the most common sites of inflammatory response due to reperfusion causing leukocyte-endothelial cell adhesion, transendothelial migration, platelet-leukocyte aggregation and enhanced oxidant production. Leukocytes use binding proteins to attach themselves to the endothelium and promotes damages liberating oxidative substances. The consumer of oxygen in the cell is the mitochondria.
There is an inability for the mitochondria to use oxygen during reperfusion leaving the cell in a cytopathic hypoxia (inability to produce ATP via oxidative phosphorylation). Endothelium is also damage in reperfusion injury due to oxidative radicals (superoxide and hydrogen peroxide). One of the most dramatic examples of reperfusion injury occurs during the development of necrotizing enterocolitis of babies.

1- Langer JC, Sohal SS, Blennerhassett P: Mucosal permeability after subclinical intestinal ischemia-reperfusion injury: an exploration of possible mechanisms. J Pediatr Surg. 30(4):568-72, 1995
2- Beuk RJ, oude Egbrink MG, Kurvers HA, Bonke HJ, Tangelder GJ, Heineman E: Ischemia/reperfusion injury in rat mesenteric venules: red blood cell velocity and leukocyte rolling. J Pediatr Surg. 31(4):512-5, 1996
3- Chan KL, Hui CW, Chan KW, Fung PC, Wo JY, Tipoe G, Tam PK: Revisiting ischemia and reperfusion injury as a possible cause of necrotizing enterocolitis: Role of nitric oxide and superoxide dismutase. J Pediatr Surg. 37(6):828-34, 2002
4- Stallion A, Kou TD, Latifi SQ, Miller KA, Dahms BB, Dudgeon DL, Levine AD: Ischemia/reperfusion: a clinically relevant model of intestinal injury yielding systemic inflammation. J Pediatr Surg. 40(3):470-7, 2005
5- Rushing GD, Britt LD: Reperfusion Injury after Hemorrhage. Ann Surg 247(6): 929-937, 2008

Mesenteric Vascular Occlusion

Mesenteric vascular occlusion causing infarction of the bowel is a very devastating disease rarely seen in the pediatric age group. Most cases are caused by mesenteric venous thrombosis. In children, mesenteric vascular thrombosis may occur both in idiopathic form or associated with a predisposing disease. Predisposing diseases includes thrombotic disorders causing a hypercoagulable state, cardiac diseases, diabetes mellitus, vasculitis (polyarteritis nodosa), artificial surfaces, trauma and surgery. Major difficulty is in establishing a prompt diagnosis since symptoms and signs mimic many disorders and bowel necrosis is already present when surgery is performed. Abdominal pain, distension, rigidity and tenderness are usually present. Persistent metabolic acidosis is a warning sign of bowel ischemia. Likewise imaging is nonspecific and can present with air-fluid levels, pneumatosis intestinalis, portal vein air, and thickened bowel loops. Selective SMA angiography is the most reliable diagnostic procedure in suspected cases and thrombolysis using urokinase or streptokinase is an alternative therapy. With failed patent arterial or venous visualization laparotomy is the next step in management.

1- Bognár M, Léb J, Dénes J: Mesenteric vascular occlusion in infants and children: report of two cases and review of the literature. Acta Paediatr Acad Sci Hung. 17(3):199-206, 1976
2- Meacham PW, Dean RH: Chronic mesenteric ischemia in childhood and adolescence. J Vasc Surg. 2(6):878-85, 1985
3- Oguzkurt P, Senocak ME, Ciftci AO, Tanyel FC, Büyükpamukçu N: Mesenteric vascular occlusion resulting in intestinal necrosis in children. J Pediatr Surg. 35(8):1161-4, 2000
4- Warshauer DM, Lee JK, Mauro MA, White GC 2nd: Superior mesenteric vein thrombosis with radiologically occult cause: a retrospective study of 43 cases. AJR Am J Roentgenol. 177(4):837-41, 2001
5- Dahshan A, Donovan K: Isolated superior mesenteric artery thrombosis: a rare cause for recurrent abdominal pain in a child. J Clin Gastroenterol. 34(5):554-6, 2002

Polyarteritis Nodosa

Polyarteritis nodosa (PAN) is an autoimmune mediated necrotizing vasculitis affecting principally  medium and small sized arteries which become swollen and damaged from attacks by rogue immune cells. PAN affects principally the skin and kidney, but almost every organ in the body is involved. In both the glomeruli and blood vessels, endothelial injury and subendothelial fibrin deposition are the earliest detectable ultrastructural changes. Boys and girls seem to be equally affected, with a peak at the age of ten years. Clinical symptoms vary depending on site of vascular involvement. Clinically children manifest fever, abdominal pain, vomiting, diarrhoea, weight loss, joint pains and skin rash. For surgeons, PAN can produce acute mesenteric vascular occlusion from venous thrombosis causing bowel ischemia. Unfortunately severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy. It can also produce acute cholecystitis. Cutaneous PAN can appear in children and has a benign and chronic course. Skin biopsy will make the diagnosis. The diagnosis of polyarteritis nodosa is difficult and often delayed. Management consists of steroids (prednisone) and immunosuppression (cyclophosphamide).

1- D'Agati V, Chander P, Nash M, Mancilla-Jimenez R: Idiopathic microscopic polyarteritis nodosa: ultrastructural observations on the renal vascular and glomerular lesions. Am J Kidney Dis. 7(1):95-110, 1986
2- Gündogdu HZ, Kale G, Tanyel FC, Büyükpamukçu N, Hiçsönmez A: Intestinal perforation as an initial presentation of polyarteritis nodosa in an 8-year-old boy. J Pediatr Surg. 28(4):632-4, 1993
3- Núñez Giralda A, Espejo Ortega M, Ibáñez Rubio M, Torrelo Fernández A, González, Medierod I, López Robledillo J: Childhood cutaneous polyarteritis nodosa. An Esp Pediatr. 54(5):506-9, 2001
4- Crankson SJ, Oda O, Al-Zaben AA, Al Suwairi W, Makanjoula D: Intestinal ischamemia in a child due to polyarteritis nodosa: a case report. Trop Gastroenterol. 27(1):41-3, 2006
5- Kendirli T, Yüksel S, Oral M, Unal N, Tulunay M, Dilek US, Yalçnkaya F: Fatal polyarteritis nodosa with gastrointestinal involvement in a child. Pediatr Emerg Care. 22(12):810-2, 2006

PSU Volume 31 No 06 DECEMBER 2008

Neonatal Appendicitis

Appendicitis can occur in any age group including newborns. Neonatal appendicitis is very rare, has a high perforation rate, morbidity and mortality. Most described cases occur in males (75%) which are born prematurely. Some of the reasons babies don't get appendicitis is due to the broad conical orifice of the appendix, the use of liquid diet, lack of fecalith and reduced lymphatic hyperplasia in the periappendiceal area in this age group. The diagnosis is delay in all cases due to the infrequent nature and only made during the exploratory laparotomy. Several causes of neonatal appendicitis are appraised. It is suspected is a form of localized necrotizing enterocolitis. This implicates some form of vascular insufficiency associated with perinatal asphyxia, cardiac anomalies or low flow states. Obstructive cecal distension associated with Hirschsprung's disease or meconium ileus (Cystic Fibrosis) causes increased pressure at the base of the appendix leading to perforation. Appendicitis can also be associated with an incarcerated inguinal hernia (Amyand's hernia). The babies show abdominal distension, signs of pain through irritability, restlessness, sleep disturbance, fever, vomiting, feeding intolerance and abnormal radiographic findings. Management is usually exploratory laparotomy with removal of the sick appendix, antibiotherapy and lavage of the abdominal cavity.

1- Stiefel D, Stallmach T, Sacher P: Acute appendicitis in neonates: complication or morbus sui generis? Pediatr Surg Int. 14(1-2):122-3, 1998
2- Martins JL, Peterlini FL, Martins EC: Neonatal acute appendicitis: a strangulated appendix in an incarcerated inguinal hernia. Pediatr Surg Int. 17(8):644-5, 2001
3- Efrati Y, Peer A, Klin B, Lotan G: Neonatal periappendicular abscess--updated treatment. J Pediatr Surg. 38(2):e5, 2003
4- Karaman A, Cavusoglu YH, Karaman I, Cakmak O: Seven cases of neonatal appendicitis with a review of the English language literature of the last century. Pediatr Surg Int. 19(11):707-9, 2003
5- Managoli S, Chaturvedi P, Vilhekar KY, Gupta D, Ghosh: Perforated acute appendicitis in a term neonate. Indian J Pediatr. 71(4):357-8, 2004
6- Jancelewicz T, Kim G, Miniati D: Neonatal appendicitis: a new look at an old zebra. J Pediatr Surg. 43(10):e1-5, 2008

Spinal Accessory Nerve Injury

The XI cranial nerve called the spinal accessory nerve provides motor innervation to two muscles in the neck: the sternocleidomastoid and upper portion of trapezius muscle. Injury to the spinal accessory nerve is usually iatrogenic and occurs most commonly after lymph node or other type of biopsy in the posterior triangle of the neck in children and adults. The paralysis of the trapezius disrupts the scapohumeral synchrony manifesting itself clinically as loss of shoulder motion, wing scapula, pain and a functional deficit. In adults the injury is usually recognized in the early postoperative period by shoulder pain and active shoulder motion dysfunction, while children have a later clinical presentation. To avoid damage during surgery use of loupe magnification is needed. Instead of a transverse incision, which is more pleasing cosmetically, a parallel incision along the posterior border of the sternocleidomastoid is safer. Because the nerve is adhered to the lymph node confirmation using nerve stimulator is needed. After the procedure the child should be examined to determined the integrity of the nerve in scapulothoracic and glenohumeral motion. With suspicion of nerve injury electrodiagnostic studies should be done. Surgical options after injury includes neurolysis or neuroma resection with primary microsurgical repair or with nerve graft reconstruction. Significant recovery is obtained after surgery.

1- Okajima S, Tamai K, Fujiwara H, Kobashi H, Hirata M, Kubo T: Surgical treatment for spinal accessory nerve injury. Microsurgery. 26(4):273-7, 2006
2- Boström D, Dahlin LB: Iatrogenic injury to the accessory nerve. Scand J Plast Reconstr Surg Hand Surg. 41(2):82-7, 2007
3- Lloyd S: Accessory nerve: anatomy and surgical identification. J Laryngol Otol. 121(12):1118-25, 2007
4- Kelley MJ, Kane TE, Leggin BG: Spinal accessory nerve palsy: associated signs and symptoms.  J Orthop Sports Phys Ther. 38(2):78-86, 2008
5- Grossman JA, Ruchelsman DE, Schwarzkopf R: Iatrogenic spinal accessory nerve injury in children. J Pediatr Surg. 43(9):1732-5, 2008

Posttraumatic Stress Disorder

Posttraumatic stress disorder (PTSD) is a constellation of symptoms associated with re-experience such as denial, avoidance and arousal after going through a life-threatening event. PTSD can affect children, parents and family members after a surgical experience. Several studies have demonstrated that symptoms of post-traumatic stress can be seen in young children undergoing bone marrow transplantation up to one year after transplant. Burn children with PTSD reported an impaired overall health related quality of life and limited physical (e.g., more bodily complaints) and emotional functioning (e.g., more feelings of sadness).  High levels of posttraumatic stress disorder symptoms are common in the recovery period after pediatric orthopaedic trauma, even among patients with relatively minor injuries. Children admitted to the hospital after injuries are at higher risk for such symptoms. Parents of children undergoing cardiopulmonary bypass surgery are at increased risk for intermediate and long-term psychological malfunctioning. Acute symptoms of PTSD in parents shortly after discharge of their child are a major risk factor for the development of chronic PTSD. In this era of prenatal ultrasound confirmation of surgical diagnosis is imperative that pediatric surgeons gather with affected parents and explain before embarking in the surgical care of their child.

1- Stuber ML, Nader K, Yasuda P, Pynoos RS, Cohen S: Stress responses after pediatric bone marrow transplantation: preliminary results of a prospective longitudinal study. J Am Acad Child Adolesc Psychiatry. 30(6):952-7, 1991
2- Landolt MA, Buehlmann C, Maag T, Schiestl C: Brief Report: Quality of Life Is Impaired in Pediatric Burn Survivors with Posttraumatic Stress Disorder.  J Pediatr Psychol. Sep 21, 2007.
3- Sanders MB, Starr AJ, Frawley WH, McNulty MJ, Niacaris TR: Posttraumatic stress symptoms in children recovering from minor orthopaedic injury and treatment. J Orthop Trauma. 19(9):623-8, 2005
4- Helfricht S, Latal B, Fischer JE, Tomaske M, Landolt MA: Surgery-related posttraumatic stress disorder in parents of children undergoing cardiopulmonary bypass surgery: a prospective cohort study. Pediatr Crit Care Med. 9(2):217-23, 2008
5- Nagata S, Funakosi S, Amae S, Yoshida S, Ambo H, Kudo A, Yokota A, Ueno T, Matsuoka H, Hayashi Y: Posttraumatic stress disorder in mothers of children who have undergone surgery for congenital disease at a pediatric surgery department. J Pediatr Surg. 43(8):1480-6, 2008

Journal Club