PEDIATRIC SURGERY UPDATE ©
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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).
References:
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.
References:
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.
References:
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.
References:
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