PEDIATRIC SURGERY UPDATE ©
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.
References:
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.
References:
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.
References:
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 patient’s 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.
References:
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
Choledocholithiasis
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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.
References:
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