PEDIATRIC SURGERY UPDATE ©
VOLUME 40, 2013
PSU Volume 40 No 01 JANUARY 2013
Von Willebrand's Disease
Von Willebrand's disease (vWD) is the
most common inherited bleeding disorder characterized by quantitative
or qualitative defects in Von Willebrand factor with a prevalence of 1%
in the world population. Von Willebrand factor (vWF) is a large
multimeric glycoprotein contained in plasma, platelets and endothelial
cells that causes platelet adhesion and aggregation following
microvascular injury, and in addition is a carrier for coagulation
factor VIII. Patients with vWD manifest dual hemostatic defect
characterized by prolonged bleeding time and low plasma level of factor
VIII. Deficiency in vWF results in mucocutaneous bleeding, including
epistaxis, menorrhagia, and excessive bleeding after trauma or surgery.
Classification of vWD is based on the combined results of multiple
laboratory tests related to vWF amount and activity as well as the
relative amounts of large vWF multimers as determined by gel
electrophoresis. vWD is classified as Type 1, seen in 70-80% of all
cases, when there is a modest and variable reduction in vWF; Type 2
(20%) if there is qualitative abnormalities in vWF and factor VIII
levels; and Type 3 (1-3%) when the patient present near complete
absence of vWF. Management of type 1 consists of administration of
desmopressin and antifibrinolytics which increases vWF and factor VIII
levels. Plasma
derived vWF and factor VIII concentrate is utilized to
manage type 2 and 3 patients undergoing major surgery and in patients
who are unresponsive or desmopressin is contraindicated. VWF:ristocetin
cofactor activity may be useful in evaluating the response to vWD
treatment in patients who require replacement therapy.
References:
1- Schneppenheim R: The pathophysiology of von Willebrand disease: therapeutic implications. Thromb Res. 128 Suppl 1:S3-7, 2011
2- O'Brien SH: Common management issues in pediatric patients with mild
bleeding disorders. Semin Thromb Hemost. 38(7):720-6, 2012
3- Gill JC, Shapiro A, Valentino LA, Bernstein J, Friedman C, Nichols
WL, Manco-Johnson M: von Willebrand factor/factor VIII concentrate
(Humate-P) for management of elective surgery in adults and children
with von Willebrand disease. Haemophilia. 17(6):895-905, 2011
4- Thompson AR, Gill JC, Ewenstein BM, Mueller-Velten G, Schwartz BA;
Humate-P Study Group: Successful treatment for patients with von
Willebrand disease undergoing urgent surgery using factor VIII/VWF
concentrate (Humate-P). Haemophilia. 10(1):42-51, 2004
5- Federici AB, Mannucci PM: Advances in the genetics and treatment of
von Willebrand disease. Curr Opin Pediatr. 14(1):23-33, 2002
6- Rodriguez KD, Sun GH, Pike F, Mandel EM, Casselbrant ML, Chi DH:
Post-tonsillectomy bleeding in children with von Willebrand disease: a
single-institution experience. Otolaryngol Head Neck Surg.
142(5):715-21, 2010
7- Mannucci PM, Chediak J, Hanna W, et al: Treatment of von Willebrand
disease with high-purity factor VIII/von Willebrand factor concentrate:
a prospective, multicenter study. Blood. 99: 450-456, 2002
LAST
With increase availability of
ultrasound guidance use of regional anesthesia has increased. Local
anesthetic systemic toxicity (LAST) is a complication associated with
regional anesthesia difficult to manage and potentially fatal. LAST
affect either or both the cardiovascular and central nervous systems
depending on the free plasma concentration of the local anesthetic
used. This is most commonly the result of intravascular injection
causing high blood concentration of the anesthetic. CNS excitation
(agitation, auditory change and metallic taste) progresses to seizures
or CNS depression (drowsiness, coma, and respiratory arrest). This is
followed by CVS excitation (tachycardia, ventricular arrhythmia, and
hypertension) then depression (bradycardia, conduction block, asystole,
and cardiac depression). Anesthetic cardiotoxicity primarily arises
from a blockade of sodium channels. Aspiration prior to injection
and use of intravascular marker such as adrenaline can reduce the
incidence of accidental intravascular injection. Bupivacaine,
levobupivacaine, and ropivacaine are longer acting and more toxic drugs
with bupivacaine being the most cardiotoxic. Plasma concentration
depends on amount injected and site of injection. Highest plasma
concentration occurs with intercostal, epidural and brachial blocks.
Combining anesthetic of rapid onset with longer duration anesthetics
can be dangerous as individual safe doses with multiple drugs are
unknown. Successful resuscitation of LAST, especially with cardiac
collapse, consists of lipid emulsion (intralipid rescue). Proposed
mechanisms of action include the intralipid acting as a "lipid sink"
extracting the lipophilic local anesthetic from plasma and tissues,
interference with sodium channel binding and reversal of the anesthetic
induced inhibition of myocardial fatty acid oxidation restoring
myocardial ATP supply. Adequate monitoring throughout the procedure is
essential to detect early signs of toxicity.
References:
1- Ciechanewicz S, Patil V: Lipid Emulsion for Local Anesthetic
Systemic Toxicity. Anesthesiology Research and Practice. Anesthesiol
Res Pract. 2012:131784, 2012
2- Kosh MC, Miller AD, Michels JE: Intravenous lipid emulsion for
treatment of local anesthetic toxicity. Therapeutics and Clinical Risk
Management. 6: 449-451, 2012
3- Dillane D, Finucane BT: Local anesthetic systemic toxicity. Can J Anaesth. 57(4):368-80, 2010
4- Burch MS, McAllister RK, Meyer TA: Treatment of local-anesthetic
toxicity with lipid emulsion therapy. Am J Health Syst Pharm.
15;68(2):125-9, 2011
5- Mercado P, Weinberg GL: Local anesthetic systemic toxicity: prevention and treatment. Anesthesiol Clin. 29(2):233-42, 2011
6- Lannqvist PA: Toxicity of local anesthetic drugs: a pediatric perspective. Paediatr Anaesth. 22(1):39-43, 2012
7- Wolfe JW, Butterworth JF: Local anesthetic systemic toxicity: update
on mechanisms and treatment. Curr Opin Anaesthesiol. 24(5):561-6, 2011
8- Weinberg GL: Lipid emulsion infusion: Resuscitation for local
anesthetic and other drugs overdose. Anesthesiology 117:180-87, 2012
Hypoganglionosis
Hypoganglionosis is a very rare entity
of intestinal innervation disorder surrounded by a controversial aura
of existence. No genetic basis or mutation has been associated with
this condition. Hypoganglionosis is reported in 0.3 to 6% of rectal
biopsies and mostly diagnosed in the preschool child. Hypoganglionosis
is defined as 40% reduction in the number of nerve cells in the bowel
wall. Most common presenting symptoms consist of intestinal
obstruction, severe chronic constipation, ileus and enterocolitis. A
full-thickness bowel specimen is required for the diagnosis of
hypoganglionosis with findings of sparse and small myenteric ganglia,
absent or low acetylcholinesterase activity, hypertrophy of muscularis
mucosa, mucosa and circular muscle. Interticial cell of Cajal have been
reported to be decreased in hypoganglionosis. Hypoganglionosis is
managed similarly to Hirschsprung's disease with resection of the
affected bowel and some form of subsequent pull-through procedure.
Complications associated with management of hypoganglionosis consist of
enterocolitis, chronic constipation, overflow encopresis and need of
redo pull-through due to residual disease. Mortality is associated with
enterocolitis and short bowel complications.
References:
1- Kubota A, Yamauchi K, Yonekura T, Kosumi T, Oyanagi H, Mushiake S,
Nakayama M, Imura K, Okada A: Clinicopathologic relationship of
hypoganglionosis. J Pediatr Surg. 36(6):898-900, 2001
2- 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
3- Zhang HY, Feng JX, Huang L, Wang G, Wei MF, Weng YZ: Diagnosis and
surgical treatment of isolated hypoganglionosis. World J Pediatr.
4(4):295-300, 2008
4- Dingemann J, Puri P: Isolated hypoganglionosis: systematic review of a rare intestinal innervation
defect. Pediatr Surg Int. 26(11):1111-5, 2010
5- Watanabe Y, Takasu H, Sumida W: A preliminary report on the
significance of excessively long segment congenital hypoganglionosis
management during early infancy. J Pediatr Surg. 46(8):1572-7, 2011
6- Puri P, Gosemann JH: Variants of Hirschsprung disease. Semin Pediatr Surg. 21(4):310-8, 2012
PSU Volume 40 No 02 FEBRUARY 2013
Closed Gastroschisis
Gastroschisis is a congenital
abdominal wall defect occurring toward the right of the insertion of
the umbilical vessels. Bowel and viscera protrude through a
full-thickness small defect. Contact of the exposed bowel to the
amniotic fluid produces serositis with edema and foreshortening of the
bowel. The diagnosis is made prenatally using ultrasound. Most babies
are born from young mothers. Closed gastroschisis refers to a small
group of gastroschisis (6%) where there is spontaneous antenatal
closure of the abdominal ring around the prolapsed intestine. Abdominal
ring closure in gastroschisis produces several sequelae such as
complete midgut infarction, intestinal resorption, small right-sided
mummified midgut remnant, or simple luminal occlusion without vascular
impairment. In most cases there is some remnant of the extraabdominal
bowel identified to the right of the umbilicus, either a shrunken
nonviable mass or and obvious gangrenous bowel of normal length.
Needless to say the baby ends with dilated proximal bowel due to the
created intestinal atresia and short bowel from the ischemic event.
Survival in closed gastroschisis depends on the length of proximal
remnant bowel left within the abdominal cavity. Close antenatal
monitoring may prevent severe bowel loss in some cases using bowel
dilatation as an index of suspicion and performing prenatal birth in
such situations. Mortality of closed gastroschisis can approach 75% due
to short bowel complications such as sepsis and liver failure.
References:
1- Davenport M, Haugen S, Greenough A, Nicolaides K: Closed
gastroschisis: Antenatal and postnatal features. J Pediatr Surg.
36(12):1834-7, 2001
2- Basaran UN, Inan M, Gacer F, Yardim T, Pul M: Prenatally closed
gastroschisis with midgut atresia. Pediatr Surg Int. 18(5-6):550-2,
2002
3- Winter LW, Giuseppetti M, Breuer CK: A case report of midgut atresia
and spontaneous closure of gastroschisis. Pediatr Surg Int.
21(5):415-6, 2005
4- Vogler SA, Fenton SJ, Scaife ER, et al: Closed gastroschisis: total
parenteral nutrition-free survival with aggressive attempts at bowel
preservation and intestinal adaptation. J Pediatr Surg. 43: 1006, 1010,
2008
5- Houben C, Davenport M, Ade-Ajayi N, Flack N, Patel S: Closing
gastroschisis: diagnosis, management, and outcomes. J Pediatr Surg.
44(2):343-7, 2009
6- Kassa AM, Lilja HE: Predictors of postnatal outcome in neonates with
gastroschisis. J Pediatr Surg. 46(11):2108-14, 2011
Laparoscopic Excision Choledochal Cyst
The management of choledochal cyst
entails complete excision of the cyst and reconstruction using
roux-en-y hepaticojejunostomy in most cases or hepaticoduodenostomy.
During the last ten years there has been a tendency for the procedure
to be performed laparoscopically. Laparoscopic excision of choledochal
cysts with reconstruction is feasible and can be performed safely with
a low intraoperative complication rate. The laparoscopic technique
includes excision of the gallbladder and cyst, followed by a Roux-en-Y
anastomosis constructed after exteriorization of the small bowel via an
infraumbilical trocar incision or intracorporeally. After repositioning
of the bowel, an end-to-side hepaticojejunostomy is carried out
laparoscopically. Dissection close to the cyst wall is mandatory to
prevent injury to the hepatic vessels, especially the portal vein.
Hepaticoduodenostomy is s simple procedure than hepaticojejunostomy,
with shorter operating time reducing the risk of postop adhesions, can
be performed totally laparoscopically giving superior cosmetic results,
but carries a higher incidence of postop cholangitis and bile-reflux
gastritis. Laparoscopy presents a longer operation duration than
laparotomy and requires more instruments. Less blood loss and chances
of transfusion is seen in the laparoscopic group due to the improved
accuracy provided by the magnified view. Cases without severe
infection, without common hepatic duct or left/right duct strictures,
and those without a cyst deeply embedded in the pancreas can be left
undrained. In neonates the laparoscopic procedure curtails further
complications of the cyst and reverses the derangement of liver
function associated with the choledochal cyst.
References:
1- Li L, Feng W, Jing-Bo F, Qi-Zhi Y, Gang L, Liu-Ming H, Yu L, Jun J,
Ping W: Laparoscopic-assisted total cyst excision of choledochal cyst
and Roux-en-Y hepatoenterostomy.J Pediatr Surg. 39(11):1663-6, 2004
2- Liu SL, Li L, Hou WY, Zhang J, Huang LM, Li X, Xie HW, Cheng W:
Laparoscopic excision of choledochal cyst and Roux-en-Y
hepaticojejunostomy in symptomatic neonates. J Pediatr Surg.
44(3):508-11, 2009
3- Nguyen Thanh L, Hien PD, Dung le A, Son TN: Laparoscopic repair for
choledochal cyst: lessons learned from 190 cases. J Pediatr Surg.
45(3):540-4, 2010
4- Liuming H, Hongwu Z, Gang L, Jun J, Wenying H, Wong KK, Miao X,
Qizhi Y, Jun Z, Shuli L, Li L: The effect of laparoscopic excision vs
open excision in children with choledochal cyst: a midterm follow-up
study. J Pediatr Surg. 46(4):662-5, 2011
5- Diao M, Li L, Cheng W: To drain or not to drain in Roux-en-Y
hepatojejunostomy for children with choledochal cysts in the
laparoscopic era: a prospective randomized study. J Pediatr Surg.
47(8):1485-9, 2012
6- Wang B, Feng Q, Mao JX, Liu L, Wong KK: Early experience with
laparoscopic excision of choledochal cyst in 41 children. J Pediatr
Surg. 47(12):2175-8. 2012
Radiation Exposure
Children are more radiosensitive than
adults. The use of CT-Scans in abdominal pain, trauma and a diverse
diagnostic entities has increased significantly during the past twenty
years. CT-Scans deliver non-uniform radiation doses across the body.
Several studies have reported significant increase cancer risk after
radiation exposure in the range received from multiple CT-Scans. Since
the bone marrow and the brain tissue are very radiosensitive, leukemia
and brain tumors are the most common tumors developing after ionizing
radiation. For children with normal life expectancy the lifetime excess
risk of any incident cancer for a head CT scan is about one cancer per
1000 head CT scans for young children (<5 years), decreasing to
about one cancer per 2000 scans for exposure at age 15 years. For an
abdominal or pelvic CT scan the lifetime risks for children are one
cancer per 500 scans irrespective of age at exposure. Means to reduce
this risky situation include using low-dose abdominal CT-Scans
and alternative diagnostic procedures such as ultrasound and MRI
when applicable. Ultrasound has been found to be an excellent tool as
initial diagnostic modality in abdominal pain suspicion of
appendicitis. There is wide agreement that the benefits of an indicated
CT scan far outweigh the risks.
References:
1- Kim K, Kim YH, Kim SY, Kim S, Lee YJ, Kim KP, Lee HS, Ahn S, Kim T,
Hwang SS, Song KJ, Kang SB, Kim DW, Park SH, Lee KH: Low-dose abdominal
CT for evaluating suspected appendicitis. N Engl J Med.
366(17):1596-605, 2012
2- Pearce MS, Salotti JA, Little MP, McHugh K, Lee C, Kim KP, Howe NL,
Ronckers CM, Rajaraman P, Sir Craft AW, Parker L, Berrington de
Gonzalez A: Radiation exposure from CT scans in childhood and
subsequent risk of leukaemia and brain tumours: a retrospective cohort
study. Lancet. 380(9840):499-505, 2012
3-Calvert C, Strauss KJ, Mooney DP: Variation in computed tomography
radiation dose in community hospitals. J Pediatr Surg.
47(6):1167-9, 2012
4-Freeman K, Strauchler D, Miller TS: Impact of socioeconomic status on
ionizing radiation exposure from medical imaging in children. J Am Coll
Radiol. 9(11):799-80, 2012.
5- Thirumoorthi AS, Fefferman NR, Ginsburg HB, Kuenzler KA, Tomita SS:
Managing radiation exposure in children-reexamining the role of
ultrasound in the diagnosis of appendicitis.J Pediatr Surg.
47(12):2268-72, 2012
6- Brody AS, Frush DP, Huda W, Brent RL: Radiation Risk to Children from Computed Tomography. Pediatrics. 120:677-682, 2007
PSU Volume 40 NO 03 MARCH 2013
Malignant Pancreatic Tumors
Malignant tumors originating primarily
in the pancreas occur rarely in children. They mostly arise from the
exocrine cells of the pancreas, as most endocrine pancreatic tumors are
benign. Malignant pancreatic tumors comprise in order of frequency:
solid pseudopapillary tumor, pancreatoblastoma, ductal adenocarcinoma,
acinar cell carcinoma and a few malignant endocrine tumors. Pancreatic
tumors in children most commonly present with a palpable mass or
abdominal pain. Jaundice occurs less frequent than in adult. Primary
imaging includes ultrasound, CT-Scan, MRI. This can be followed by
surgery or fine-needle image-guided biopsy to establish a histological
diagnosis and plan further therapy. This second option of biopsy is
used when the child present with distant metastasis or an unresectable
primary tumor. The two most common malignant pancreatic tumors are the
solid pseudopapillary tumor (SPT) and the pancreatoblastoma.
Pancreatoblastoma is a more commonly found in the first decade of life
and is a more aggressive tumor, while SPT is more common in females
during adolescent years and caries a better overall prognosis. About
one third pancreaticoblastoma cases are metastatic at the time of
diagnosis. These tumors can be responsive to chemotherapy and
radiation. Preoperative chemotherapy can successfully decrease tumor
size and allow a more complete surgical resection. Adequate management
of malignant pancreatic tumor is complete surgical resection. On
multivariate analysis, histologic type is the only factor that
significantly predicts survival. Patients with poorly differentiated
carcinoma shows the worst survival probability.
References:
1- 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
2- Tapia B, Ahrens W, Kenney B, Touloukian R, Reyes-Magica M: Acinar
cell carcinoma versus solid pseudopapillary tumor of the pancreas in
children: a comparison of two rare and overlapping entities with review
of the literature. Pediatr Dev Pathol. 11(5):384-90, 2008
3- Yu DC, Kozakewich HP, Perez-Atayde AR, Shamberger RC, Weldon CB:
Childhood pancreatic tumors: a single institution experience. J Pediatr
Surg. 44(12):2267-72, 2009
4- Park M, Koh KN, Kim BE, Im HJ, Kim DY, Seo JJ: Pancreatic neoplasms
in childhood and adolescence. J Pediatr Hematol Oncol. 33(4):295-300,
2011
5- Marchegiani G, Crippa S, Malleo G, Partelli S, Capelli P, Pederzoli
P, Falconi M: Surgical treatment of pancreatic tumors in childhood and
adolescence: uncommon neoplasms with favorable outcome. Pancreatology.
11(4):383-9, 2011
6- Rojas Y, Warneke CL, Dhamne CA, Tsao K, Nuchtern JG, Lally KP,
Vasudevan SA, Hayes-Jordan AA, Cass DL, Herzog CE, Hicks MJ, Kim ES,
Austin MT: Primary malignant pancreatic neoplasms in children and
adolescents: A 20year experience. J Pediatr Surg. 47(12):2199-204, 2012
Rectovestibular Fistula with Normal Anus
Rectovestibular fistula with a normal
anus is a rare anorectal malformation affecting females patients. It is
also known as anovestibular fistula or H-type rectovestibular fistula.
Is more commonly found in Asia. Most cases present with early infant
history of fecal discharge through an external opening in the posterior
vestibule of the genitalia. A few will develop a perineal vulvar
abscess before the fistula becomes visible. Anal stenosis has been
associated with this condition. The embryology of this fistula is
though to represent a persisting cloacal canal or an interruption
of the dorsal part of the embryonal cloacal membrane by an isolated
defect. The fistula uniformly extends from the vestibular fourchette to
the anterior wall of the rectum one to 3 cm above the dentate line.
Contrast enemas and endoscopic studies are not very useful as
diagnostic aid. The diagnosis is best confirmed during an exam under
anesthesia passing a small probe from the vestibule to the internal
rectal orifice. Management of rectovestibular fistula has included
perineal repair, vestibuloanal pull-through, anterior perineal
anorectoplasty, fistulectomy and limited posterior sagittal
anorectoplasty. To avoid a colostomy bowel preparation and systemic
antibiotics are necessary. Most common complications are recurrence and
wound deshicence. Fistula recurrence occurs when blood perfusion in the
local rectal tissue is poor and separation of the divided ends of the
fistula fails. It is important to close the fistula orifice using
healthy anterior rectal wall.
References:
1- Tsugawa C, Nishijima E, Muraji T, Satoh S, Kimura K: Surgical repair
of rectovestibular fistula with normal anus.J Pediatr Surg.
34(11):1703-5, 1999
2- Yazici M, Etensel B, Gursoy H, Ozklsaclk S: Congenital H-typ anovestibuler fistula. World J Gastroenterol 9(4): 881-882, 2003
3- Banu T, Hannan MJ, Hoque M, Aziz MA, Lakhoo K: Anovestibular fistula with normal anus. J Pediatr Surg. 43:526-529, 2008
4- Li L, Zhang TC, Zhou CB, Pang WB, Chen YJ, Zhang JZ: Rectovestibular
fistula with normal anus: a simple resection or an extensive perineal
dissection? J Pediatr Surg. 45:519-524, 2010
5- Kim SM, Park YJ, Ahn SM, Oh JT, Han SJ: Infantile vulvar abscess
with a normal anus: a suspicious sign of rectovestibular fistula.
Yonsei Med J. 51(5):717-21, 2010
6- Lawal TA, Chatoorgoon K, Bischoff A, Pena A, Levitt MA: Management
of H-type rectovestibular and rectovaginal fistulas. J Pediatr Surg.
46:1226-1230, 2011
Duodenum Inversum
Duodenum inversum refers to a
congenital anomaly where the third portion of the duodenum is located
to the right of the second portion or above the duodenal bulb. In other
words the third portion instead of continuing leftward toward Treitz,
reverses direction and travels in a superior posterior track prior to
crossing the midline above the pancreas. Most cases occur in male
adults. Duodenum inversum is associated with nonspecific symptoms such
as epigastric discomfort, nausea, distension or duodenal obstruction.
Other associated conditions include cholelithiasis, pancreatitis and
annular pancreas. The condition can mimic mesenteric artery syndrome.
Diagnosis is confirmed with UGIS. Management of duodenum inversum
without obstruction is conservative (antacids, antispasmodics). With
duodenal obstruction surgery may be required. The obstruction is due to
fibrotic bands. Lysis can be performed laparoscopically.
References:
1- Childress MH: Duodenum inversum. J Natl Med Assoc. 71(5):515-6, 1979
2- Long FR, Kramer SS, Markowitz RI, Taylor GE, Liacouras CA:
Intestinal malrotation in children: tutorial on radiographic diagnosis
in difficult cases. Radiology. 198(3):775-80, 1996
3- Long FR, Mutabagani KH, Caniano DA, Dumont RC: Duodenum inversum
mimicking mesenteric artery syndrome. Pediatr Radiol. 29(8):602-4, 1999
4- Azhough R, Bayat A, Hashemzadeh S, Khaki AA, Motayagheni N, Tarzamni
MK: The combination of annular pancreas and duodenum inversum
presenting with delayed gastric emptying, pain, and feeding
intolerance. Am J Gastroenterol. 104(5):1328-9, 2009
5- Kim ME, Fallon SC, Bisset GS, Mazziotti MV, Brandt ML: Duodenum
inversum: A report and review of the literature. J Pediatr Surg.
48(1):E47-E49, 2013
PSU Volume 40 No 04 APRIL 2013
Trunk Rhabdomyosarcoma
Rhabdomyosarcoma (RMS) is the most
common soft tissue sarcoma in children comprising one half of pediatric
soft tissue sarcomas and 15% of all pediatric solid tumors. RMS can
arise anywhere in the body and survival has been found highly dependent
on such variables as tumor site, extent of disease and management. RMS
arising in the trunk area is associated with a poor prognosis. The
trunk includes chest wall, abdomen and paraspinal region. Trunk RMS
presents as a painless, firm mass that increases rapidly in size. The
diagnosis is made by incisional or excisional biopsy of the mass. Most
patients with trunk RMS have embryonal histology. Adolescent patients
with trunk RMS fare worse than younger children. This is due to the
high incidence of unfavorable (alveolar) histology in adolescent cases.
Factors associated with unfavorable outcomes after managing trunk RMS
includes tumor size greater than 5 cm, advance stage at
presentation, alveolar histology, lymph node involvement, and local and
distant tumor recurrence. Complete surgical resection including the
full-thickness of the abdominal or chest wall when feasible with clear
tumor margins is the cornerstone of effective and curative management.
The abdominal wall is a site at which tumors can be resected widely
with minimal morbidity and the majority of wounds can be closed
primarily or with substitution using a biologic mesh. When upfront
surgery is not feasible, neoadjuvant chemotherapy followed by delayed
surgical resection should be considered. Patient with bulk residual
tumor remaining after surgery or metastatic disease requires adjuvant
therapy (radiotherapy and chemotherapy).
References:
1- Beech TR, Moss RL, Anderson JA: What comprises appropriate therapy
for children/adolescents with rhabdomyosarcoma arising in the abdominal
wall? A report from the Intergroup Rhabdomyosarcoma Study Group. J
Pediatr Surg. 34(5): 668-671, 1999
2- Tabrizi P, Letts M: Childhood rhabdomyosarcoma of the trunk and extremities. Am J Orthop (Belle Mead NJ). 28(8):440-6, 1999
3 Chui CH, Billups CA, Pappo AS, Rao BN, Spunt SL: Predictors of
outcome in children and adolescents with rhabdomyosarcoma of the
trunk--the St Jude Children's Research Hospital experience. J Pediatr
Surg. 40(11):1691-5, 2005
4- Hayes-Jordan A, Stoner JA, Anderson JR, Rodeberg D, Weiner G, Meyer
WH, Hawkins S, Arndt CA, Paidas C; Children's Oncology Group: The
impact of surgical excision in chest wall rhabdomyosarcoma: a report
from the Children's Oncology Group. J Pediatr Surg. 43(5):831-6, 2008
5- Raney RB, Anderson JR, Brown KL, Huh WW, Maurer HM, Meyer WH, Parham DM, Rodeberg
DA, Wolden SL, Donaldson SS; Soft-Tissue Sarcoma Committee of the
Children's Oncology Group Arcadia California USA. Treatment results for
patients with localized, completely resected (Group I) alveolar
rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG)
protocols III and IV, 1984-1997: a report from the Children's Oncology
Group. Pediatr Blood Cancer. 55(4):612-6, 2010
Gorham's Disease
Gorham's disease is a very rare
lymphatic vascular malformation involving bone and leading to massive
osteolysis. The disease is also known as vanishing bone syndrome or
Gorham-Stout syndrome. The cause of the disease is not known but there
is evidence that massive osteolysis is associated with increased
osteoclast activity and increase sensitivity of osteoclast precursors
to interleukin 6 which promotes bone resorption. There is no gender or
race predilection. Gorham's disease affects more commonly adolescent
and young adults. The disease mainly affects the shoulder, pelvis, and
skull crossing joint boundaries. Mandible, ribs, scapula, humerus,
pelvis and femur can be involved. The child presents with dull pain,
swelling of the affected area and pathological fractures. Involvement
of pleura and vertebrae is associated with poor prognosis since it
leads to chylothorax and nerve root compression. Chylothorax occurs in
one-fifth of all cases. Diagnosis of Gorham's disease is difficult. MRI
shows evidence of massive osteolysis. The affected bone undergoes
resorption replaced by hyper vascular fibrous tissue. Histology shows a
non malignant proliferation of dilated thin walled vascular and
lymphatic channels with associated loss of bone matrix. Splenic lesions
and soft-tissue involvement are common in patients with Gorham's
disease. Management of Gorham's disease includes sclerotherapy,
immunotherapy, radiotherapy and surgery. Surgery to replace the
affected bone with a graft or prothesis has also been used successfully
in early and localized disease. Death occurs due to malnutrition,
lymphocytopenia or infection.
References:
1- Szabo C, Habre W: Gorham syndrome: anaesthetic management. Anaesthesia. 55(2):157-9, 2000
2- Prasanna R, Sankar J, Ramachandran P: Gorhams disease: vanishing bone syndrome. Indian Pediatr. 46(3):255-6, 2009
3- Venkatramani R, Ma NS, Pitukcheewanont P, Malogolowkin MH,
Mascarenhas L: Gorham's disease and diffuse lymphangiomatosis in
children and adolescents. Pediatr Blood Cancer. 56(4):667-70, 2011
4- Wells K, Gray Hazard FK: Gorham's disease: diagnostic utility of an
autopsy for a rare bone disease. J Pediatr Health Care. 25(6):391-8,
2011
5- Kotecha R, Mascarenhas L, Jackson HA, Venkatramani R: Radiological
features of Gorham's disease. Clin Radiol. 67(8):782-8, 2012
6- Situma M, Alexander A, Weiselthaler N, Millar A: An aggressive
lymphatic malformation (Gorham's Disease) leading to death of a
child. J Pediatr Surg. 48(1):239-42, 2013
Laparoscopic Peritoneal Cannula Placement
Peritoneal dialysis is preferred to
hemodialysis to manage chronic renal failure in children because it has
a lower incidence of serious complications, is more cost-effective and
improves patient nutrition and independence. The peritoneal cannula for
such purposes can be place using an open conventional surgical
technique, percutaneously or laparoscopically. Laparoscopic placement
of peritoneal dialysis catheters or revision allows complete
visualization of the peritoneal cavity placing the catheter under
direct vision, preferably in the pelvis. Suturing the catheter tip into
the pelvis is associated with a low rate of catheter migration. In
addition a more complete omentectomy can be performed and lysis of
adhesions can be accomplished to increase the peritoneal absorptive
surface. Omentectomy is beneficial during placement of peritoneal
dialysis catheters to prevent blockage of the catheter. Laparoscopy can
allow for the rescue of block catheters in cases of revisions due to
malfunction. Others procedures that can be done concomitantly using the
laparoscopic technique include repair of inguinal hernias, gastrostomy
tube placement, kidney biopsy and cholecystectomy. Peritoneal dialysis
is delayed as long as possible to allow for healing of the incision to
prevent leakage. Other recognize benefits of the laparoscopic technique
include better cosmetic results, less postoperative pain and shorter
hospital stay.
References:
1- Mattioli G, Castagnetti M, Verrina E, Trivelli A, Torre M, Jasonni
V, Perfumo F: Laparoscopic-assisted peritoneal dialysis catheter
implantation in pediatric patients. Urology. 2007 Jun;69(6):1185-9.
2- Lu CT, Watson DI, Elias TJ, Faull RJ, Clarkson AR, Bannister KM:
Laparoscopic placement of peritoneal dialysis catheters: 7 years
experience. ANZ J Surg. 2003 Mar;73(3):109-11
3- Carrillo SA, Ghersi MM, Unger SW: Laparoscopic-assisted peritoneal
dialysis catheter placement: a microinvasive technique. Surg Endosc.
2007 May;21(5):825-9.
4- Skipper K, Dickerman R, Dunn E: Laparoscopic placement and revision
of peritoneal dialysis catheters. JSLS. 1999 Jan-Mar;3(1):63-5
5- Lee M, Donovan JF: Laparoscopic omentectomy for salvage of peritoneal dialysis catheters. J Endourol. 2002 May;16(4):241-4.
6- Stringel G, McBride W, Weiss R: Laparoscopic placement of peritoneal
dialysis catheters in children. J Pediatr Surg. 2008 May;43(5):857-60
PSU Volume 40 NO 05 MAY 2013
Hibernoma
Hibernoma is a rare benign lipomatous
tumor of brown fat origin initially described as a pseudolipoma. The
name hibernoma was used to characterize the resemblance of this tumor
to the brown fat found in hibernating animals. Brown fat has a
thermoregulatory role in neonates and fetus. Hibernoma can develop in
sites where brown fat is still present in humans such as interscapular
region, axilla, neck, mediastinum, periaortic area, retroperitoneum and
peripherally in the limbs. Findings brown fat cells in a lipomatous
tumor is diagnostic of hibernoma. Malignant potential has not been
demonstrated. Clinically, hibernomas present as a slow growing painless
solitary mass. They produce symptoms by virtue of compression of
adjacent structures or by torsion if the developed a pedicle. Most
hibernoma are found in young adults with an average age of 38 years.
Though rare in the pediatric age, hibernomas can affect the thigh,
back, chest, neck, breast, abdominal wall and spine of children. The
diagnosis of hibernoma by imaging is difficult. CT and MRI are used
indistinctly showing a well-defined hypointense mass with septations
that enhances with IV contrast. They do not have increased FDG uptake
on PET imaging. Malignancy cannot be excluded safely by imaging
modalities. Standard of care for managing hybernoma is complete
surgical excision. Recurrence is very rare after complete excision of
this tumorous mass.
References:
1- Baskurt E, Padgett DM, Matsumoto JA: Multiple hibernomas in a
1-month-old female infant. AJNR Am J Neuroradiol. 25(8):1443-5, 2004
2- Hardes J, Scheil-Bertram S, Hartwig E, Gebert C, Gosheger G, Schulte
M: Sonographic findings of hibernoma. A report of two cases. J Clin
Ultrasound. 33(6):298-301, 2005
3- Yalniz E, Alicioglu B, Oz Puyan F: Hibernoma: a benign lipomatous tumor mimicking liposarcoma. J BUON. 13(1):127-9, 2008
4- Whitaker JK, Motiwale S: Hibernoma in a 16-year-old boy. Scott Med J. 56(1):59, 2011
5- Papathanassiou ZG, Alberghini M, Taieb S, Errani C, Picci P, Vanel
D: Imaging of hibernomas: A retrospective study on twelve cases. Clin
Sarcoma Res. 25;1(1):3, 2011
6- Guidry CA, McGahren ED, Rodgers BM, Kane BJ: Pediatric
cervicomediastinal hibernoma: a case report. J Pediatr Surg.
48(1):258-61, 2013
Pulse Oximeter Probe Injuries
The pulse oximeter is an essential
noninvasive tool developed to monitor the arterial hemoglobin
saturation of children and adults. It as essential device routinely
used in the intensive care units, operating rooms, emergency rooms,
recovery rooms and during transfer of patients. The oximeter probe has
two components: a light source that uses a light emitting diode
reflecting direct red and infrared light into the skin, and a
photodetector that measures the absorption of light by hemoglobin. As
any device in medicine, injury can result from the continuous use of
the oximeter probe. The most common injury is an electrical burn caused
by a damage probe or a short circuit. Other injuries include thermal
burns due to overheating of the light emitting diode of the probe,
distal ischemia with digit gangrene owning to pressure of the probe,
dermatologic reaction from the chemicals in the probe, skin necrosis
and digital sensory loss. Thinner skin, lower fat content and high
water content makes the pediatric population more predisposed to such
injury with deeper and more extensive damage. Low cardiac output, poor
peripheral circulation, vasopressor therapy, hypotension, hypoxia,
hypothermia and arterial cannulation of the affected extremity are
considered associated factors implicated in the pathogenesis of pulse
oximeter probe induced finger injury. Severely ill patients, as
indicated by their need for more aggressive vasopressors, are more
likely to develop pulse oximeter induced digital injury. Routine
examination and rotation of the probe every 4 to 6 hours as a
prevention measure is essential to avoid such injury. Capillary filling
time should be checked and noted in the record.
References:
1- Chemello PD, Nelson SR, Wolford LM: Finger injury resulting from
pulse oximeter probe during orthognathic surgery. Oral Surg Oral Med
Oral Pathol. 69(2):161-3, 1990
2- Kohjiro M, Koga K, Komori M, Fukutome T, Yasaka B, Urakami H: [A
case report of a burn produced by the probe of a pulse oximeter].
Masui. 41(12):1991-3, 1992
3- Lin CW, Wang HZ, Hsieh KS: Pulse oximeter-associated toe injuries in
a premature neonate: a case report. Zhonghua Yi Xue Za Zhi
(Taipei). 62(12):914-6, 1999
4- Wille J, Braams R, van Haren WH, van der Werken C: Pulse
oximeter-induced digital injury: frequency rate and possible causative
factors. Crit Care Med. 28(10):3555-7, 2000
5- Punj J, Jaryal A, Mahalingam S, Mukundan C, Pandey R, Darlong V,
Chandralekha: Toe gangrene in an infant subsequent to application of
adult-type pulse oximeter probe for 10 min. J Anesth. 24(4):630-2, 2010
6- Ceran C, Taner OF, Tekin F, Tezcan S, Tekin O, Civelek B: Management
of pulse oximeter probe-induced finger injuries in children: report of
two consecutive cases and review of the literature. J Pediatr Surg.
47(11):e27-9, 2012
Pterygium Colli
Pterygium colli or better known as web
neck is a physical characteristic of several syndromes including
Ulrrich-Turner, Klippel-Feil and Escobar syndrome. Pterygium colli is
characterized by bilateral webbing of the neck extending from the
mastoid to the acromion. The webbing in the neck is caused by fibrotic
bands in the fascia colli superficialis which has showed muscular
elements in postmortem specimens. Other cases have an excess of skin.
Webbing is the result of large bilateral subcutaneous blebs and cysts
particularly of the neck which obliterate throughout the course of
fetal development, leading to cutis laxa in the newborn and by further
shrinkage and scarring of the subcutaneous tissue to the true webbing
of the neck. Correction of this deformity can be accomplished by
ellipsoid excision of the skin, excision of the fibrous bands and
multiple z-plasty reconstruction of the excess skin. The operative
procedure is a purely elective intervention and most of these patients
do not have any functional impairment. It is recommended the procedure
be done before the child begins school to avoid peer pressure and
stigma. The most common complication is hypertrophied scarring, but
cosmetic results in general are very good.
References:
1- Doletskia SIa, PolonetskiaVA: [Surgical correction of pterygium
colli in children with hereditary pathology]. Khirurgiia (Mosk).
(7):37-41, 1988
2- Rossillon D, De Mey A, Lejour M: Pterygium colli: surgical treatment.Br J Plast Surg. 42(2):178-83, 1989
3- Hoffmann J, Cornelius CP, Schwenzer K, Schwenzer N: [Possibilities
for correcting the neck profile in pterygium colli]. Mund Kiefer
Gesichtschir. Suppl 1:S141-3, 1997
4- Leandris M, Ricbourg B: [Surgical treatment of pterygium colli. A case report and review of the
literature]. Ann Chir Plast Esthet. 42(6):615-22, 1997
5- Posso CM, Wolff GA, Suarez LD: [Pterigium colli: secondary surgical
correction of one severe case]. Ann Chir Plast Esthet. 57(3):299-303,
2012
6- Reichenberger MA, Goertz O, Lehnhardt M, Germann G, Ryssel H,
Czermak C, Kolbenschlag J: Surgical correction of pterygium colli. J
Pediatr Surg. 48(2):464-9, 2013
PSU Volume 40 NO 06 JUNE 2013
Desmosis Coli
Constipation is a very serious
condition in children. Medical manageable conditions associated with
chronic constipation can be classified into normal transit
constipation, slow transit constipation and disorders of defecation
with retention in the rectum. The most common, slow transit
constipation, can be caused by genetic links, dysfunctional enteric
nerves, decrease levels of substance P, reduced numbers of interticial
cells of Cajal and disorders of connective tissue synthesis. Desmosis
coli is a disturbance of the intramural connective tissue mesh network
of the colonic wall which leads to a hypoperistalsis syndrome with
chronic constipation in the absence of any anomaly of the vegetative
gut innervation. The peristaltic movement of the gut is a function of
the alternating contraction and relaxation of circular and longitudinal
muscles. This movement is induced by a tendon-like connective-tissue
net in the circular and longitudinal muscles, which are both rooted in
a connective-tissue plexus layer. This connective tissue layer is not
developed in children with desmosis coli. Clinically, the child
develops hypoperistalsis or aperistalsis with massive elongation and
dilatation of the colon. Hirschsprung's disease can coexist with
desmosis coli as a familiar trait. The enteric nervous system is normal
or near-normal in the affected areas of desmosis coli. Two major
subtypes of desmosis can be distinguished: the rare congenital
(primary) aplastic desmosis of childhood, and the more common atrophic
(secondary) desmosis of adulthood typically incomplete and associated
with a hypoperistaltic syndrome. Seromuscular biopsies are required to
prove desmosis in gastrointestinal dysmotility disorders. Management of
desmosis coli is controversial.
References:
1- Meier-Ruge WA, Holschneider AM, Scharli: New pathogenetic aspects of
gut dysmotility in aplastic and hypoplastic desmosis of early
childhood. Pediatr Surg Int. 17(2-3):140-3, 2001
2- Meier-Ruge WA: Desmosis of the colon: a working hypothesis of primary chronic constipation.
Eur J Pediatr Surg. 8(5):299-303, 1998
3- Marshall DG, Meier-Ruge WA, Chakravarti A, Langer JC: Chronic
constipation due to Hirschsprung's disease and desmosis coli in a
family. Pediatr Surg Int. 18(2-3):110-4, 2002
4- Habner U, Meier-Ruge W, Halsband H: Four cases of desmosis coli:
severe chronic constipation, massive dilatation of the colon, and
hypoperistalsis due to of changes in the colonic connective-tissue net.
Pediatr Surg Int. 18(2-3):198-203, 2002
5- Meier-Ruge WA, Bruder E: [The morphological characteristics of
aplastic and atrophic desmosis of the intestine]. Pathologe.
28(2):149-54, 2007
6- Bruhin-Feichter S, Meier-Ruge W, Martucciello G, Bruder E:
Connective tissue in gut development: a key player in motility and in
intestinal desmosis. Eur J Pediatr Surg. 22(6):445-59, 2012
Transfer Burn Center
Burns are a major source of injury in
children that must be managed within the confines of specialized
centers whenever possible to obtain best results in morbidity,
mortality and rehabilitation. Burns are classified as First degree
(partial thickness, superficial, red, sometimes painful), Second degree
(partial thickness, skin may be red, blistered, swollen, very painful),
or Third degree (full-thickness, whitish, charred, translucent, no pin
prick sensation in burned area). The American Burn Association has
established strict criteria for admission or transfer of infants and
children to such regional burn centers. They include: 1- Children with
less than 10 years of age with burns compromising more than 10% of
total body surface area. 2- Children with age above the 10 years with
more than 20% total body surface area burned. 3- Children of all ages
with full-thickness burn above the 5% of total body surface area. 4-
Children with evidence of inhalation injury. 5- Burn to face, eyes,
ears, genitalia or joints. 6- Any burn associated with a major fracture
or significant traumatic injury. 7- Third degree burns in any age
group. 8- Chemical burns. 9- Electrical burns. 10- Burn injury in
patients with preexisting medical disorders that could complicate
management, prolong recovery, or affect mortality. 10- Burned children
in hospitals without qualified personnel or equipment for the care of
children. 11- Burn injury in patients who will require special social,
emotional, or rehabilitative intervention.
References:
1- www.ameriburn.org
Rapunzel Syndrome
Bezoars are concretion of human or
vegetable fibers that accumulate in the gastrointestinal tract. The
most common type of bezoar is the trichobezoar which is mostly made of
hair or hairlike fibers. Trichobezoars most commonly present during
adolescent years and during the second decade of life. Most cases of
trichobezoars occur in female patients. Eating hair denotes an
underlying psychiatry disorder. Most trichobezoars lodge within the
body of the stomach. When the trichobezoar extends beyond the pyloric
muscle and beyond as a tail is called Rapunzel syndrome. The distal end
of the bezoar may be in the jejunum, ileum or the colon Most children
with trichobezoars suffer from psychiatry disorders including
trichotillomania (pulling out of their own hair) and trichophagia
(eating of hair). Hair is retained in the folds of the gastric mucosa,
and as it accumulates the peristalsis creates an enmeshed ball. The
stomach dilates significantly. Decomposition and fermentation of fats
give the bezoar and the child breath a putrid smell. The black color of
the bezoar is due to the effect of acid on hair protein
denaturalization. Most cases are asymptomatic for a long period of
time. Most common presenting signs are abdominal pain, nausea,
vomiting, obstruction and peritonitis. Other times children present
with anorexia, weight loss, hematemesis or intussusception. Bezoars can
cause gastric ulceration, obstructive jaundice, acute pancreatitis, and
gastric emphysema. Imaging (UGIS, Ultrasound and CT-Scan) shows the
bezoar as a mass or filling defect and are diagnostic. Endoscopic
retrieval of the bezoar is rarely a definitive treatment. Management of
trichobezoar includes removal by gastrotomy and/or enterotomy and
behavioral therapy to avoid recurrence.
References:
1- Ventura DE, Herbella FA, Schettini ST, Delmonte C: Rapunzel syndrome
with a fatal outcome in a neglected child. J Pediatr Surg.
40(10):1665-7, 2005
2- Naik S, Gupta V, Naik S, Rangole A, Chaudhary AK, Jain P, Sharma AK:
Rapunzel syndrome reviewed and redefined. Dig Surg. 24(3):157-61, 2007
3- Gonuguntla V, Joshi DD: Rapunzel syndrome: a comprehensive review of
an unusual case of trichobezoar. Clin Med Res. 7(3):99-102, 2009
4- Crawley AJ, Guillerman RP: Rapunzel syndrome. Pediatr Radiol. 40 Suppl 1:S100, 2010
5- Kohler JE, Millie M, Neuger E. Trichobezoar causing pancreatitis:
first reported case of Rapunzel syndrome in a boy in North America. J
Pediatr Surg. 47(3):e17-9, 2012
6- Fallon SC, Slater BJ, Larimer EL, Brandt ML, Lopez ME. The surgical
management of Rapunzel syndrome: a case series and literature review. J
Pediatr Surg. 48(4):830-4, 2013