PEDIATRIC SURGERY UPDATE ©
VOLUME 38, 2012
PSU Volume 38 No 01 JANUARY 2012
Dopamine-producing Pheochromocytoma
Pheochromocytomas are
neuroendocrine tumors that produce, metabolize and secrete
catecholamines such as norepinephrine and epinephrine. In very rare
occasions hormonally active pheochromocytomas can produce and secrete
only dopamine as the only catecholamine. Predominance of dopamine in
this tumors and lack of production of other catecholamines is due to
deficiency in tumor cells of dopamine-B-hydroxylase, the enzyme that
converts dopamine into norepinephrine. Dopamine-producing
pheochromocytomas are usually found as extraadrenal paragangliomas.
Clinically patients harboring these tumors are normotensive. Most
patients are diagnosed after identifying the adrenal or extraadrenal
position of the tumor during incidental imaging or as the result of
symptoms caused by the space-occupying tumor. Due to lack of hormonally
associated symptoms these tumors can grow into large size. Tumors that
produce dopamine can be identified by high plasma or urine
concentrations of dopamine, or high plasma levels of free
methoxytyramine. The incidence of malignancy is higher in this type of
pheochromocytoma. Nausea, vomiting, flushing and orthostatic
hypotension are a few of the symptoms exhibited by these patients. MRI
is the imaging of choice to localize these tumors. Management consists
of resection of the tumor. Persistent elevation of blood pressure after
tumor resection have been documented. PET-Scan is more specific
than MIBG in localizing residual disease.
References:
1- Minamiguchi N, Inui E, Nukui M: [A case of dopamine-secreting pheochromocytoma]. Hinyokika Kiyo. 45(12):831-3, 1999
2- Yasunari K, Kohno M, Minami M, Kano H, Ohhira M, Nakamura K,
Yoshikawa J: A dopamine-secreting pheochromocytoma. J Cardiovasc
Pharmacol. 36 Suppl 2:S75-7, 2000
3- Awada SH, Grisham A, Woods SE: Large dopamine-secreting pheochromocytoma: case report. South Med J. 96(9):914-7, 2003
4- Eisenhofer G, Goldstein DS, Sullivan P, Csako G, Brouwers FM, Lai
EW, Adams KT, Pacak K: Biochemical and clinical manifestations of
dopamine-producing paragangliomas: utility of plasma methoxytyramine. J
Clin Endocrinol Metab. 90(4):2068-75, 2005
5- Tam V, Ng KF, Fung LM, Wong YY, Chan MH, Lam CW, Tam S, Lam CW: The
importance of the interpretation of urine catecholamines is essential
for the diagnosis and management of patient with dopamine-secreting
paraganglioma. Ann Clin Biochem. 42(Pt 1):73-7, 2005
6- Foo SH, Chan SP, Ananda V, Rajasingam V: Dopamine-secreting
phaeochromocytomas and paragangliomas: clinical features and
management. Singapore Med J. 51(5):e89-93, 2010
7- Dubois LA, Gray DK: Dopamine-secreting pheochromocytomas: in search of a syndrome. World J Surg. 29(7):909-13, 2005
Laparoscopy for Ventriculoperitoneal Shunts
Ventriculoperitoneal (VP) shunt
is the standard treatment for hydrocephalus in children and adults.
Malfunctioning VP shunts causing increase intracranial pressure needs
surgical revision. Such malfunctioning can occur due to obstruction
from peritoneal adhesion, multiple infections, ascites and pseudocysts
formation, or mechanical causes such as catheter fracture,
disconnection, migration and misplacement. VP shunt failure is common
with 25-40% occurring within the first year, and 50% by the second
year. Laparoscopy has an important role in initial VP shunt placement
and later revisions. Laparoscopy reduce the trauma to the abdominal
wall decreasing adhesion formation and optimizing visualization during
placement. With VP shunt failure laparoscopy can be both diagnostic and
therapeutic. While visualizing the entire abdominal cavity the causes
of failure can be identified, lysis of adhesions can be performed,
retrieval of disconnected shunt can be accomplished, flow of CSF fluid
can be observed and proper placement of the catheter can be obtained
The technique is safe with a very low morbidity rate. The use of
laparoscopy assisted VP shunt revision is advocated for patients with
multiple previous shunt revisions, prior abdominal surgery, previous
intraperitoneal infections, broken devices, obesity or CSF pseudocysts.
References:
1- Khosrovi H, Kaufman HH, Hrabovsky E, Bloomfield SM, Prabhu V,
el-Kadi HA: Laparoscopic-assisted distal ventriculoperitoneal shunt
placement. Surg Neurol. 49(2):127-34, 1998
2- Jackson CC, Chwals WJ, Frim DM: A single-incision laparoscopic
technique for retrieval and replacement of disconnected
ventriculoperitoneal shunt tubing found in the peritoneum.Pediatr
Neurosurg. 36(4):175-7, 2002
3- Kirshtein B, Benifla M, Roy-Shapira A, Merkin V, Melamed I, Cohen Z,
Cohen A: Laparoscopically guided distal ventriculoperitoneal shunt
placement. Surg Laparosc Endosc Percutan Tech. 14(5):276-8, 2004
4- Bani A, Hassler WE: Laparoscopy-guided insertion of peritoneal catheters in ventriculoperitoneal
shunt procedures: analysis of 39 children. Pediatr Neurosurg. 42(3):156-8, 2006
5- Yu S, Bensard DD, Partrick DA, Petty JK, Karrer FM, Hendrickson RJ:
Laparoscopic guidance or revision of ventriculoperitoneal shunts in
children.JSLS. 10(1):122-5, 2006
6- Johnson BW, Pimpalwar A: Laparoscopic-assisted placement of
ventriculo-peritoneal shunt tips in children with multiple previous
open abdominal ventriculo-peritoneal shunt surgeries. Eur J Pediatr
Surg. 19(2):79-82, 2009
7- Martin K, Baird R, Farmer JP, et al: The use of laparoscopy in
ventriculoperitoneal shunt revisions. J Pediatr Surg. 46(11):
2146-2150, 2011
Magnet-assisted Laparoscopic Surgery
Magnets have been introduced in
the armamentarium of laparoscopic surgery to recapture the
triangulation that is affordable by conventional laparoscopy while
decreasing the number and size of the abdominal incisions utilized.
Specialized magnetic grasper are inserted into the peritoneal cavity
through the port cannula and attached to intraabdominal organs. These
magnets are controlled by another external magnet placed on top of the
abdominal wall. The magnet grasper moves to provide further traction on
an organ without the additional need of another port. With the magnet
you can retract the liver, stomach, lung tissue, gallbladder providing
traction and facilitating exposure. Magnet-assisted laparoscopy is safe
and effective means of reducing the number and size of abdominal
incisions while improving exposure, triangulation, and the ergonomics
of the procedure.
References:
1- Rothenberg SS, Shipman K, Yoder S: Experience with modified
single-port laparoscopic procedures in children. J Laparoendosc Adv
Surg Tech A. 19(5):695-8, 2009
2- Garey CL, Laituri CA, Ostlie DJ, Snyder CL, Andrews WS, Holcomb GW
3rd, St Peter SD: Single-incision laparoscopic surgery in children:
initial single-center experience. J Pediatr Surg. 46(5):904-7,
2011
3- de Armas IA, Garcia I, Pimpalwar A: Laparoscopic single port surgery
in children using Triport: our early experience. Pediatr Surg Int.
27(9):985-9, 2011
4- Dutta S: Early experience with single incision laparoscopic surgery:
eliminating the scar from abdominal operations. J Pediatr
Surg. 44(9):1741-5, 2009
5- Padilla BE, Dominguez G, Millan C, Martinez-Ferro M: The use of
magnets with single-site umbilical laparoscopic surgery. Semin Pediatr
Surg. 20(4):224-31, 2011
PSU Vulome 38 NO 02 FEBRUARY 2012
Middle Aortic Syndrome
Middle aortic syndrome (MAS) is a rare
condition involving diffuse narrowing of the distal thoracic or
abdominal aorta involving its visceral and renal branches. The etiology
of MAS is unknown and occurs in young patients. MAS have been
associated with neurofibromatosis, Alagille's and William syndrome. The
mesenteric vascular stenosis is clinically silent, while the renal
stenosis is responsible for the clinical picture of hypertension in
these children. Absent femoral pulses and an abdominal bruit are also
present. Bilateral involvement is common. Ultrasonography is the
primary screening technique. Biplanar arteriography is the standard
imaging to establish the diagnosis and involvement of the disease.
Arteriography shows a smooth segmental narrowing of the abdominal aorta
with concomitant stenosis at the origins of the renal arteries. Primary
treatment goals are preservation of functional renal tissue and
amelioration of the renal hypertension. Therapeutic approaches included
medical management, percutaneous transluminal angioplasty and/or
surgical intervention. Repair of the narrowing requires prosthetic
material for bypass or patch reconstruction. Techniques used to
revascularize the kidney included thoracoabdominal to infrarenal aortic
bypass with renal artery reimplantation, splenorenal bypass,
gastroduodenal to renal bypass, aortorenal bypass and
autotransplantation.
References:
1- Panayiotopoulos YP, Tyrrell MR, Koffman G, Reidy JF, Haycock GB,
Taylor PR: Mid-aortic syndrome presenting in childhood. Br J Surg.
83(2):235-40, 1996
2- Tummolo A, Marks SD, Stadermann M, Roebuck DJ, McLaren CA, Hamilton G, Dillon MJ,
Tullus K: Mid-aortic syndrome: long-term outcome of 36 children. Pediatr Nephrol. 24(11):2225-32, 2009
3- Lin YJ, Hwang B, Lee PC, Yang LY, Meng CC: Mid-aortic syndrome: a
case report and review of the literature. Int J Cardiol. 123(3):348-52,
2008
4- Chocrón de Benzaquen S, Munoz Lopez M, Madrid Aris AD,
Castellote Alonso A, Enriquez G, Nieto Rey JL: [Mid-aortic syndrome:
case studies from a paediatric nephrology department]. An Pediatr
(Barc). 75(1):33-9, 2011
5- Bleacher J, Turner ME, Quivers E, Schwartz MZ: Renal
autotransplantation for renovascular hypertension caused by midaortic
syndrome. J Pediatr Surg. 32(2):248-50, 1997
6- Sethna CB, Kaplan BS, Cahill AM, Velazquez OC, Meyers KE: Idiopathic
mid-aortic syndrome in children. Pediatr Nephrol. 23(7):1135-42, 2008
Ciliated Hepatic Foregut Cyst
Ciliated Hepatic Foregut Cyst (CHFC)
is a rare cystic benign disease of the liver increasingly diagnosed.
Most patients with CHFC are asymptomatic at the time of
diagnosis. The most common symptom is right upper quadrant pain. In
almost all cases the cyst is located within the left liver segment IV
or in close proximity. Average size of the cyst is 3.6 cm with most
cyst unilocular, avascular and lying beneath the anterior surface of
the liver. Cyst content is viscous or mucinous. It is the presence of
shared histological features in the form of pseudostratified ciliated
columnar epithelium that leads to label these cysts of foregut origin.
The origin of CHFC is detachment and migration of foregut with
subsequent entrapment in the liver. Infantile presentation is in the
form of an abdominal mass due to the larger size. In infants the cyst
may have bile duct communication and can develop squamous metaplasia,
reason why excision and ligation of the biliary communication should be
preferred to internal drainage. CT will show a complex hypodense cystic
mass without enhancement. FNA cytology will show the ciliated
pseudostratified columnar cells and establish the diagnosis. The
presence of a tough fibrous outer layer in the cyst makes it amenable
to mobilization and enucleation without spillage. This can be
accomplished by laparoscopy or open surgery.
References:
1- Kim S, White FV, McAlister W, Shepherd R, Mychaliska G: Ciliated
hepatic foregut cyst in a young child. J Pediatr Surg. 40(11):e51-3,
2005
2- Stringer MD, Jones MO, Woodley H, Wyatt J: Ciliated hepatic foregut cyst. J Pediatr Surg. 41(6):1180-3, 2006
3- Betalli P, Gobbi D, Talenti E, Alaggio R, Gamba P, Zanon GF:
Ciliated hepatic foregut cyst: from antenatal diagnosis to
surgery.Pediatr Radiol. 38(2):230-2, 2008
4- Sharma S, Dean AG, Corn A, Kohli V, Wright HI, Sebastian A, Jabbour
N: Ciliated hepatic foregut cyst: an increasingly diagnosed
condition.Hepatobiliary Pancreat Dis Int. 7(6):581-9, 2008
5- Goodman MD, Mak GZ, Reynolds JP, Tevar AD, Pritts TA: Laparoscopic
excision of a ciliated hepatic foregut cyst.JSLS. 13(1):96-100, 2009
6- Zaydfudim V, Rosen MJ, Gillis LA, Correa H, Lovvorn HN 3rd, Pinson
CW, Kelly BS Jr: Ciliated hepatic foregut cysts in children. Pediatr
Surg Int. 26(7):753-7, 2010
7- Guerin F, Hadhri R, Fabre M, Pariente D, Fouquet V, Martelli H,
Gauthier F, Branchereau S: Prenatal and postnatal Ciliated Hepatic
Foregut Cysts in infants. J Pediatr Surg. 45(3):E9-14, 2010
ALTE
An apparent life-threatening event
(ALTE) is defined as an episode that is frightening to the caretaker
and is characterized by some combination of apnea (which can be central
or occasionally obstructive), color change (usually cyanotic or pallid
but occasionally erythematous or plethoric), marked change in muscle
tone (usually limpness), choking or gagging. Although the natural
history of ALTE is most often benign, there is a risk for subsequent
morbidity and mortality. The most common causes of ALTE are
gastroesophageal reflux, seizure activity and lower respiratory tract
infections, specially respiratory syncytial virus infection. Other
causes include otolaryngology conditions, inborn errors in
metabolism and cardiac diseases. The incidence of sudden infant death
syndrome is reported to be higher after ALTE episodes. Tow mechanisms
explain why reflux can result in ALTE: chemoreflex in the larynx
prompted by acid fluid and stimulation of the distal esophagus by the
gastric content. Children with ALTE and gastroesophageal reflux, other
cardiac, respiratory and neurological causes appropriately excluded
should be considered candidates for fundoplication since medical
management carries a high risk of recurrent ALTE. After fundoplication
the incidence of ALTE is significantly reduced.
References:
1- National Institutes of Health Consensus Development Conference on
Infantile Apnea and Home Monitoring. Pediatrics 79:292- 299, 1987
2- McGovern MC, Smith MB: Causes of apparent life threatening events in
infants: a systematic review. Arch Dis Child. 89(11):1043-8, 2004
3- Dewolfe CC: Apparent life-threatening event: a review. Pediatr Clin North Am. 52(4):1127-46, 2005
4- Esani N, Hodgman JE, Ehsani N, Hoppenbrouwers T: Apparent
life-threatening events and sudden infant death syndrome: comparison
of risk factors. J Pediatr. 152(3):365-70, 2008
5- Valusek PA, St Peter SD, Tsao K, et al. The use of fundoplication
for prevention of apparent life-threatening events. J Pediatr Surg
42:1022-1024, 2007
6- Tirosh E, Ariov-Antebi N, Cohen A: Autonomic function,
gastroesophageal reflux in apparent life threatening event. Clin Auton
Res. 20(3):161-6, 2010
PSU Volume 38 No 03 MARCH 2012
Bile Duct Bifurcation
Duplication, or more appropriately
phrase bifurcation of the bile ducts is an anomaly associated to
duodenal atresia. This anomaly can lead to find gas in the distal bowel
of a duodenal atresia. The bifurcated bile duct communicates between
the proximal and distal atetric segments bypassing gas between them. A
bifid biliary system inserts at blind upper and lower pouches of the
duodenum, and the common bile duct inserts in a Y fashion. The
incidence of duodenal atresia with an anomalous bifurcated bile duct
conduit is higher than is thought and occurs more frequently than that
associated with duodenal stenosis. Down syndrome is highly associated
with cases of an anomalous bifurcated bile duct conduit. Contrast
studies are generally not performed in the typical clinical and
radiographic evaluation of duodenal atresia; however, an upper
gastrointestinal series can demonstrate the anomalous bile duct
bifurcation or duplication. The presence of such anomaly can predispose
the patient to cholestasis and cholangitis due to duodeno-biliary
reflux. The management of duodenal atresia consists of
duodenoduodenostomy. Care should be taken at surgery to avoid
obstruction or injury to the anomalous bifurcated bile duct, and
operative cholangiography may be useful to document continued bile duct
patency following repair of the atresia.
References:
1- Knechtle SJ, Filston HC: Anomalous biliary ducts associated with duodenal atresia. J Pediatr Surg. 25(12):1266-9, 1990
2- Panuel M, Bourliere-Najean B, Delarue A, Viard L, Faure F, Devred P:
[Duodenal atresia with bifid termination of the common bile duct]. Arch
Fr Pediatr. 49(4):365-7, 1992
3- Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y: Embryogenesis
of pancreaticobiliary maljunction inferred from development of duodenal
atresia. J Hepatobiliary Pancreat Surg. 6(1):50-4, 1999
4- Tashjian DB, Moriarty KP: Duodenal atresia with an anomalous common
bile duct masquerading as a midgut volvulus. J Pediatr Surg.
36(6):956-7, 2001
5- Mali V, Wagener S, Sharif K, Millar AJ: Foregut atresias and bile
duct anomalies: rare, infrequent or common? Pediatr Surg Int.
23(9):889-95, 2007
6- Komuro H, Ono K, Hoshino N, Urita Y, Gotoh C, Fujishiro J, Shinkai
T, Ikebukuro K: Bile duct duplication as a cause of distal bowel gas in
neonatal duodenal obstruction. J Pediatr Surg. 46(12):2301-4, 2011
Microvillous Inclusion Disease
Microvillous Inclusion Disease (MVID)
or microvillous atrophy is a very rare congenital disorder of the
intestinal epithelial cells that present with persistent
life-threatening watery diarrhea and is characterized by morphological
enterocyte abnormalities. Onset of diarrhea most often occurs within
the first days of life (early) or in the first two months of life
(late). MVID is very rare and transmitted as an autosomal recessive
trait. Patients can lose up to 30% of body weight. Small bowel biopsy
is diagnostic revealing villous atrophy without significant crypt
hyperplasia. Periodic acid-Schiff positive granules accumulate in the
apical cytoplasm of immature intestinal epithelial cells. Children with
MVID are totally dependent on parenteral nutrition. Long-term outcome
is generally poor, due to metabolic decompensation, repeated states of
dehydration, infectious and liver complications related to the
parenteral nutrition. Relentlessly the child develops intestinal
failure secondary to the diarrhea. There is no specific treatment for
MVID other than hydration and parenteral nutrition. Isolated intestinal
transplantation or combined liver–small bowel transplantation is
a last treatment option when significant liver disease exists.
Replacement of the conventional soybean oil–based lipid emulsions
with an omega-3–rich product have been found to rapidly reverse
parenteral nutrition-induced cholestasis in patients with MVID.
References:
1- Wilson W, Scott RB, Pinto A, Robertson MA: Intractable diarrhea in a
newborn infant: microvillous inclusion disease. Can J Gastroenterol.
15(1):61-4, 2001
2- Ruemmele FM, Schmitz J, Goulet O: Microvillous inclusion disease (microvillous atrophy). Orphanet J Rare Dis. 26;1:22, 2006
3- Iancu TC, Mahajnah M, Manov I, Shaoul R: Microvillous inclusion
disease: ultrastructural variability. Ultrastruct Pathol. 31(3):173-88,
2007
4- Halac U, Lacaille F, Joly F, Hugot JP, Talbotec C, Colomb V,
Ruemmele FM, Goulet O: Microvillous inclusion disease: how to improve
the prognosis of a severe congenital enterocyte disorder. J Pediatr
Gastroenterol Nutr. 52(4):460-5, 2011
5- Fuchs J, Fallon EM, Gura KM, Puder M: Use of an omega-3 fatty
acid-based emulsion in the treatment of parenteral nutrition-induced
cholestasis in patients with microvillous inclusion disease. J Pediatr
Surg. 46(12):2376-82, 2011
Endoluminal Intestinal Lengthening
Endoluminal intestinal lengthening is
an option still in experimental phase for intestinal lengthening in
cases of short bowel syndrome. Mechanical force is a viable method for
increasing intestinal length while preserving the intestinal function.
Distraction enterogenesis or the application of forces to the small
bowel has been shown to increase length through the induction of
cellular proliferation. Some designs previously used for such purposes
consist of screws, hydraulic pistons and remotely controlled ratcheting
devices. Experimental studies have demonstrated the feasibility of
using a polymer-coated spring capsule intraluminally placed in a piece
of intestine of experimental animals for timed deployment of an
expanding device for bowel Lengthening. The restored jejunal segment
had an increase in crypt depth and no difference in villus height
compared with normal jejunum. Sucrase activity in the restored segment
was not different from that in normal jejunum. Using these
methods the small bowel can be lengthened three to fourfold times its
original length. Mechanical lengthening may be a useful technique to
increase intestinal length in patients with short bowel syndrome.
References:
1- Shekherdimian S, Scott A, Chan A, Dunn JC: Intestinal lengthening in
rats after massive small intestinal resection. Surgery. 146(2):291-5,
2009
2- Park J, Puapong DP, Wu BM, Atkinson JB, Dunn JC: Enterogenesis by
mechanical lengthening: morphology and function of the lengthened small
intestine. J Pediatr Surg. 39(12):1823-7, 2004
3- Chang PC, Mendoza J, Park J, Lam MM, Wu B, Atkinson JB, Dunn JC:
Sustainability of mechanically lengthened bowel in rats. J Pediatr
Surg. 41(12):2019-22, 2006
4- Stark R, Zupekan T, Bondada S, Dunn JC: Restoration of mechanically
lengthened jejunum into intestinal continuity in rats. J Pediatr Surg.
46(12):2321-6, 2011
5- Stark R, Panduranga M, Carman G, Dunn JC: Development of an
endoluminal intestinal lengthening capsule. J Pediatr Surg.
47(1):136-41, 2012
PSU Volume 38 NO 04 APRIL 2012
Ovarian Transposition
Ionizing radiation used in adjuvant
management of malignancy can have an adverse effect on gonadal function
at all ages. The ovaries are exposed to significant doses of
irradiation when radiotherapy is used to managed pelvic and abdominal
malignant diseases such as Hodgkin's lymphoma, cervical and rectal
cancer to children before childbearing age. Half of the follicles are
lost when a dose of 4 Gy is used. The risk of premature ovarian failure
increases significantly with increasing doses of abdominal pelvic
irradiation. Ovarian transposition, or repositioning the ovaries out of
the irradiation field can preserve ovarian function before pelvic
irradiation. Ovarian transposition can be done laparoscopically prior
to pelvic irradiation. The procedure can reduce damage caused by
radiotherapy but does not protect against damage caused by systemic
chemotherapy. The laparoscopic procedure for ovarian transposition is
highly efficient, can be done as outpatient and is associated with few
postoperative complications. Proper location to fix the transposed
ovaries depends on the planned irradiation field. For cervical cancer
the ovaries are transposed high and lateral above the pelvic rim, while
for pelvic lymph node irradiation they are placed medially or
preferably laterally. Surgical complications reported includes injury
to the ovarian vasculature, fallopian tube infarction and ovarian cyst
formation.
References:
1- Hays DM, Fryer CJ, Pringle KC, Collins RD, Hutchinson RJ, O'Neill
JA, Constine LS, Heller RM, Davis PC, Nachman J, et al: An evaluation
of abdominal staging procedures performed in pediatric patients with
advanced Hodgkin's disease: a report from the Childrens Cancer Study
Group.J Pediatr Surg. 27(9):1175-80. 1992
2- Héloury Y, Guiberteau V, Sagot P, Plattner V, Baron M,
Rogez JM: Laparoscopy in adnexal pathology in the child: a study of 28
cases. Eur J Pediatr Surg. 3(2):75-8, 1993
3- Williams RS, Littell RD, Mendenhall NP: Laparoscopic oophoropexy and
ovarian function in the treatment of Hodgkin disease. Cancer.
86(10):2138-42, 1999
4- Meirow D, Nugent D: The effects of radiotherapy and chemotherapy on female reproduction. Hum Reprod Update. 7(6):535-43, 2001
5- Terenziani M, Piva L, Meazza C, Gandola L, Cefalo G, Merola M:
Oophoropexy: a relevant role in preservation of ovarian function after
pelvic irradiation. Fertil Steril. 91(3):935.e15-6, 2009
6- Han SS, Kim YH, Lee SH, Kim GJ, Kim HJ, Kim JW, Park NH, Song YS,
Kang SB: Underuse of ovarian transposition in reproductive-aged cancer
patients treated by primary or adjuvant pelvic irradiation. J Obstet
Gynaecol Res. 37(7):825-9, 2011
7- Morris SN, Ryley D: Fertility preservation: nonsurgical and surgical options. Semin Reprod Med. 29(2):147-54, 2011
Adrenal Cysts
Adrenal cysts are rare, usually found
incidentally during autopsy series. Adrenal cysts are usually
asymptomatic; mostly an occasional discovery during ultrasound or CT
done for other reason. Acute abdominal or flank pain may be presented
in some cases. From the histological point of view adrenal cysts
are vascular or endothelial, hemorrhagic or pseudocyst and
epithelial-lined or "true" adrenal cysts. Less than 10% of adrenal
cysts are malignant. The most common types are epithelial and
pseudocysts. Intra-cystic hemorrhage spontaneously or post-traumatic
may be present. Due to the asymptomatic nature of the cyst they can
attain large sizes usually in the range of 10 cm when diagnosed.
Preoperative CT-guided aspiration for cytology and biopsy are useful
technique to establish a diagnosis. Resection of the cyst should be
performed for cysts larger than 3 cm, symptomatic, suspicion of
malignancy, hormonally active or rapidly enlarging. Either laparoscopic
or open are standard methods to remove the cyst along with the adrenal
gland associated. Laparoscopic adrenalectomy or cyst unroofing is a
safe and effective treatment for benign adrenal cysts with the
advantages of a shorter hospital stay, less blood loss and enhanced
cosmesis.
References:
1- El-Hefnawy AS, El Garba M, Osman Y, Eraky I, El Mekresh M, Ibrahim
el-H: Surgical management of adrenal cysts: single-institution
experience. BJU Int. 104(6):847-50, 2009
2- Poiana C, Carsote M, Chirita C, Terzea D, Paun S, Beuran M: Giant adrenal cyst: case study. J Med Life. 3(3):308-13, 2010
3- Guazzoni G, Montorsi F, Rigatti P, Lanzi R, Pontiroli AE, Silvestre
P, Breda G: Laparoscopic unroofing of adrenal cysts. Eur Urol.
31(4):499-502, 1997
4- Pradeep PV, Mishra AK, Aggarwal V, Bhargav PR, Gupta SK, Agarwal A:
Adrenal cysts: an institutional experience. World J Surg.
30(10):1817-20, 2006
5- Lal TG, Kaulback KR, Bombonati A, Palazzo JP, Jeffrey RB, Weigel RJ:
Surgical management of adrenal cysts. Am Surg. 69(9):812-4, 2003
6- Castillo OA, Litvak JP, Kerkebe M, Urena RD: Laparoscopic management
of symptomatic and large adrenal cysts. J Urol. 173(3):915-7, 2005
Measuring IAP
The abdominal compartment syndrome
(ACS) is a clinical syndrome caused by persistency elevated
intraabdominal pressure (IAP) leading to decreased venous return and
cardiac output, increase intracranial pressure, impaired ventilation,
and kidney and bowel end-organ damage. The most common etiologies of
ACS in children are isolated head trauma, abdominal trauma, surgery for
abdominal wall defect, bowel ischemia/necrosis and meningococcemia. The
most accurate method for measurement of IAP is directly via an
intraperitoneal catheter. Due to invasiveness, the indirect method of
measuring IAP using the intravesical method has prevailed as the gold
standard. The bladder technique requires that the bladder be infused
with a certain amount of saline, to ensure that there is a conductive
fluid column between the bladder and the transducer. The method uses a
Foley catheter inserted into the bladder while the bladder is filled
with saline. The closest correlation with intraabdominal pressure
occurs when a volume of 1 ml/kg of weight is utilized in children. The
higher the bladder filling volume, the higher the overestimation of
IAP. Optimal patient position for IAP measurement is supine, taken at
end-expiration with the transducer calibrated to the level of the
mid-axillary line. Infusion of saline at room temperature causes higher
bladder pressure due to contraction of the detrusor bladder muscle.
Mean IAP in critically ill children is 7 +/- 3 mm Hg. Above 12 mm Hg
IAP is elevated.
References:
1- Chiumello D, Tallarini F, Chierichetti M, Polli F, Li Bassi G, Motta
G, Azzari S, Carsenzola C, Gattinoni L: The effect of different volumes
and temperatures of saline on the bladder pressure measurement in
critically ill patients. Crit Care. 11(4):R82, 2007
2- Gudmundsson FF, Viste A, Gislason H, Svanes K: Comparison of
different methods for measuring intra-abdominal pressure. Intensive
Care Med. 28(4):509-14, 2002
3- Zengerink I, McBeth PB, Zygun DA, Ranson K, Ball CG, Laupland KB,
Widder S, Kirkpatrick AW: Validation and experience with a simple
continuous intra-abdominal pressure measurement technique in a
multidisciplinary medical/surgical critical care unit. J Trauma.
64(5):1159-64, 2008
4- Balogh Z, Jones F, D'Amours S, Parr M, Sugrue M: Continuous
intra-abdominal pressure measurement technique. Am J Surg.
188(6):679-84, 2004
5- Suominen PK, Pakarinen MP, Rautiainen P, Mattila I, Sairanen
H: Comparison of direct and intravesical measurement of intraabdominal
pressure in children. J Pediatr Surg. 41(8):1381-5, 2006
6- Ejike JC, Bahjri K, Mathur M: What is the normal intra-abdominal
pressure in critically ill children and how should we measure it? Crit
Care Med. 36(7):2157-62, 2008
7- Davis PJ, Koottayi S, Taylor A, Butt WW: Comparison of indirect
methods of measuring intra-abdominal pressure in children.
Intensive Care Med. 31(3):471-5, 2005
PSU Volume 38 NO 05 MAY 2012
Extravasation Injury
Extravasation injury (EI) during
intravenous therapy is a significant source of morbidity, mortality and
liability in children and adults. The spectrum of injury goes from mild
irritation without inflammation to tissue necrosis depending if the
agent extravasated is a vesicant, irritant or flare producing.
Vesicants can produce tissue necrosis by absorption from local tissue
and DNA binding. They should be administered by central vein route.
Patients at increased risk of EI include the very young or old with
impaired venous circulation and lymphatic drainage, and the critically
ill child. Device related factors include metal needles, large gauge
catheters, poorly secured IV cannulas, IV placed in antecubital fossa,
dorsum of hand or near joint area, and catheter dysfunction by
separation, breakage or dislodgement. Patients should be informed of
risk of EI and encourage to notify the nurse of any change in
sensation, swelling, leakage, pain, machine flow malfunction or
burning. Management consists of immediate discontinuance of infusion,
aspirate residual extravasated fluid and removal of the peripheral
catheter. Affected extremity should be elevated. Avoidance of the
extremity for future cannulation or blood pressure recording. Prompt
consultation with the wound nurse should follow. Serial photographs are
useful to monitor wound progress. Conservative local care is given and
plastic and physical therapy are consulted as needed.
References:
1- Garland JS, Dunne WM Jr, Havens P, Hintermeyer M, Bozzette MA,
Wincek J, Bromberger T, Seavers M: Peripheral intravenous catheter
complications in critically ill children: a prospective study.
Pediatrics. 89(6 Pt 2):1145-50, 1992
2- Gault DT: Extravasation injuries. Br J Plast Surg. 46(2):91-6, 1993
3- Kumar RJ, Pegg SP, Kimble RM: Management of extravasation injuries. ANZ J Surg. 71(5):285-9, 2001
4- Tong R: Preventing extravasation injuries in neonates. Paediatr Nurs. 19(8):22-5, 2007
5- Rose RE, Felix R, Crawford-Sykes A, Venugopal R, Wharfe G, Arscott
G: Extravasation injuries. West Indian Med J. 57(1):40-7, 2008
6- Amjad I, Murphy T, Nylander-Housholder L, Ranft A: A new approach to
management of intravenous infiltration in pediatric patients:
pathophysiology, classification, and treatment. J Infus Nurs.
34(4):242-9, 2011
Hürthle Cell Neoplasms
Oncocytic or Hürthle cell adenoma
and carcinoma represent less than 5% of all thyroid neoplasms in
children and adults. The distinction between Hürthle cell adenoma
and carcinoma is histologically determined by the presence of vascular
or capsular invasion in the latter. Hürthle cells are large
polygonal eosinophilic cells with pleomorphic hyperchromatic nuclei and
fine granular cytoplasm containing an abundance of mitochondria
commonly associated with Hashimoto thyroiditis, nodular goiter and
well-differentiated thyroid cancer. Hürthle cell tumors contain
increased number of mitochondria with structural abnormalities
resembling patients with mitochondrial disease or myopathy. Adenomas
are more common than carcinomas. Hürthle carcinoma is more
aggressive, produces more metastasis, has a lower survival rate and low
uptake for radioiodine. Hürthle adenomas can be managed with
hemithyroidectomy, while the carcinoma variety will need total
thyroidectomy. Since most Hürthle cell neoplasms secrete
thyroglobulin it can be used to detect recurrent disease. Hürthle
cell tumor size is predictive of malignancy with adenomas on the
average smaller than carcinomas (2 cm vs. 4 cm). Hürthle carcinoma
has a prognosis that is reliably predicted by degree of invasion, tumor
size, extrathyroidal disease extension, and initial nodal or distant
metastasis. Molecular expression of high Ki-67 phenotype proliferative
index correlates with recurrence and tumor-related mortality among
Hürthle cell tumors.
References:
1- Chen H, Nicol TL, Zeiger MA, Dooley WC, Ladenson PW, Cooper DS,
Ringel M, Parkerson S, Allo M, Udelsman R: Hürthle cell neoplasms
of the thyroid: are there factors predictive of malignancy? Ann Surg.
227(4):542-6, 1998
2- Hoos A, Stojadinovic A, Singh B, Dudas ME, Leung DH, Shaha AR, Shah
JP, Brennan MF, Cordon-Cardo C, Ghossein R: Clinical significance of
molecular expression profiles of Hürthle cell tumors of the
thyroid gland analyzed via tissue microarrays. Am J Pathol.
160(1):175-83, 2002
3- Stojadinovic A, Hoos A, Ghossein RA, Urist MJ, Leung DH, Spiro RH,
Shah JP, Brennan MF, Singh B, Shaha AR: Hürthle cell carcinoma: a
60-year experience. Ann Surg Oncol. 9(2):197-203, 2002
4- Maximo V, Soares P, Lima J, Cameselle-Teijeiro J, Sobrinho-Simaes M:
Mitochondrial DNA somatic mutations (point mutations and large
deletions) and mitochondrial DNA variants in human thyroid pathology: a
study with emphasis on Hürthle cell tumors. Am J Pathol.
160(5):1857-65, 2002
5- Besic N, Hocevar M, Zgajnar J, Petric R, Pilko G: Aggressiveness of
therapy and prognosis of patients with Hürthle cell papillary
thyroid carcinoma. Thyroid. 16(1):67-72, 2006
6- Bremer AA, Feldman BJ, Iezza G, Clark OH, Rosenthal SM: Report of a
Hürthle cell neoplasm in a peripubertal girl. Thyroid.
17(2):175-8, 2007
7- Sippel RS, Elaraj DM, Khanafshar E, Zarnegar R, Kebebew E, Duh QY,
Clark OH: Tumor size predicts malignant potential in Hürthle cell
neoplasms of the thyroid. World J Surg. 32(5):702-7, 2008
Cloacogenic Polyps
Inflammatory cloacogenic polyps are
very rarely found in the pediatric age. They arise from the
transitional zone of the anal canal, but can extend proximally toward
the sigmoid colon. Characterized histologically by marked hyperplasia
of the muscularis mucosa with extension of smooth muscle and fibrous
stroma into the lamina propria. The typical presentation of the patient
is difficulty with defecation and passage of mucous and blood per
rectum. The polyps can prolapse; this is due to the malfunction of the
internal anal sphincter; and the smooth muscle that covers the rectum.
During endoscopy they can appear polypoid with flat base. The polyps
vary in size from 3-4 cm in diameter, and have a sessile
appearance. Inflammatory cloacogenic polyp is related to solitary
rectal ulcer syndrome and is most likely due to prolapse of the
anorectal transition zone. Cloacogenic polyps are not a neoplasm
arising from a preexistent normal transitional epithelium but a
nonspecific regenerative process. Management consists of endoscopic
removal of the polyps. Those unable to be removed endoscopically or
endorectal will need sigmoidectomy with low anterior
resection.
References:
1- Ciriza de Los Rios C, Tomas Moro E, Garcia Duran F, et al:
[Inflammatory cloacogenic polyps: a rare cause of rectal bleeding].
Gastroenterol Hepatol. 30(8):461-4, 2007
2- Calva-Rodriguez R, Gonzalez-Palafox MA, Rivera-Dominguez ME, et al:
[Inflammatory cloacogenic polyp]. Rev Gastroenterol Mex. 72(4):371-5,
2007
3- Washington K, Rourk MH Jr, McDonagh D, Oldham KT: Inflammatory
cloacogenic polyp in a child: part of the spectrum of solitary rectal
ulcer syndrome. Pediatr Pathol. 13(4):409-14, 1993
4- Bass J, Soucy P, Walton M, Nizalik E: Inflammatory cloacogenic polyps in children. J Pediatr Surg. 30(4):585-8, 1995
5- Poon KK, Mills S, Booth IW, Murphy MS: Inflammatory cloacogenic
polyp: an unrecognized cause of hematochezia and tenesmus in childhood.
J Pediatr. 130(2):327-9, 1997
6- Siafakas C, Vottler TP, Andersen JM: Rectal prolapse in pediatrics. Clin Pediatr (Phila). 38(2):63-72, 1999
PSU Volume 38 NO 06 JUNE 2012
Pulmonary Metastasectomy
The most common thoracic pediatric
malignancy is pulmonary metastasis secondary to solid tumors. Survival
has been improved with the use of pulmonary metastasectomy. By
examining tumor types individually it is seen that certain histologies
(adrenocortical carcinoma, alveolar soft part sarcoma, osteosarcoma)
mandate surgical metastasectomy for patient survival. Other pediatric
tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive and
application of metastasectomy is controversial, though a possible
benefit for Ewing's cases has been found recently when combined with
radiotherapy. In Wilms, pulmonary metastasectomy is indicated if
complete remission can be achieved to avoid lung irradiation. In the
case of neuroblastoma, differentiated thyroid cancer, rhabdomyosarcoma,
metastasectomy is seldom performed except in highly unusual situations.
During metastasectomy wedge resection is more commonly performed than
anatomic resection in order to minimized the volume of resected lung
tissue. Significant longer survival is observed for patient after
complete resection, with five or fewer metastatic nodules, unilateral
disease, and disease-free interval of more than two years. Performing
metastasectomy at least three months after detection might
significantly improve the prognosis. Other factors associated with
dismal prognosis are poor chemonecrosis and central distribution
instead of peripheral location of the metastasis. The open thoracotomy
approach is preferred since the pulmonary nodule must be manually
palpated for an optimal resection.
References:
1- Kayton ML: Pulmonary metastasectomy in pediatric patients. Thorac Surg Clin.16(2):167-83, 2006
2- Tronc F, Conter C, Marec-Berard P, Bossard N, Remontet L, Orsini A,
Gamondes JP, Louis D: Prognostic factors and long-term results of
pulmonary metastasectomy for pediatric histologies.Eur J Cardiothorac
Surg. 34(6):1240-6, 2008
3- Tanaka Y, Maniwa Y, Nishio W, Yoshimura M, Okita Y: The optimal
timing to resect pulmonary metastasis. Eur J Cardiothorac Surg.
33(6):1135-8, 2008
4- Rasalkar DD, Chu WC, Lee V, Paunipagar BK, Cheng FW, Li CK:
Pulmonary metastases in children with osteosarcoma: characteristics and
impact on patient survival.Pediatr Radiol. 41(2):227-36, 2011
5- Letourneau PA, Shackett B, Xiao L, Trent J, Tsao KJ, Lally K,
Hayes-Jordan A: Resection of pulmonary metastases in pediatric patients
with Ewing sarcoma improves survival.J Pediatr Surg. 46(2):332-5, 2011
6- Warmann SW, Furtwängler R, Blumenstock G, Armeanu S,
Nourkami N, Leuschner I, Schenk JP, Graf N, Fuchs J: Tumor biology
influences the prognosis of nephroblastoma patients with primary
pulmonary metastases: results from SIOP 93-01/GPOH and SIOP
2001/GPOH.Ann Surg. 254(1):155-62, 2011
7- Letourneau PA, Xiao L, Harting MT, Lally KP, Cox CS Jr, Andrassy RJ,
Hayes-Jordan AA: Location of pulmonary metastasis in pediatric
osteosarcoma is predictive of outcome. J Pediatr Surg. 46(7):1333-7,
2011
Magnet Ingestion
Most foreign body ingestion in infant
and children passes through the gastrointestinal tract without causing
significant sequelae. Surgical intervention is generally required if an
object becomes lodged in the gastrointestinal tract or if the material
has a harmful effect such as the corrosive effect of batteries. With
rare-earth magnets present in many small toys, the situation can be
very different. A single magnet ingestion is innocuous and is expected
to pass through the GI tract. Unfortunately, a misdiagnosis and
misconception that a solitary magnet has been ingested when in fact
they are two or more joined together may lead to a delay in diagnosis
and subsequent severe and possibly preventable complications. The
ingestion of multiple magnets can cause bowel obstruction, volvulus,
perforation or internal bowel fistula formation owning to pressure
necrosis from magnet attraction. Pressure necrosis and fistula
formation can be a gradual process resulting in minimal physical
examination findings. In many of the toys the magnets are embedded in
plastic parts that are easily detachable. If in the stomach, the magnet
should be removed endoscopically. If the history, clinical findings and
imaging are suggestive of multiple magnetic ingestion early
intervention using laparoscopy or open surgery is indicated to prevent
serious life-threatening complications. It is imperative health
authorities give more information to parents and physicians about the
potential risk of small magnetic toys in children.
References:
1- Hernandez Anselmi E, Gutierrez San Roman C, Barrios Fontoba JE, et
al: Intestinal perforation caused by magnetic toys. J Pediatr Surg.
42(3):E13-6, 2007
2- Alzahem AM, Soundappan SS, Jefferies H, Cass DT: Ingested magnets
and gastrointestinal complications. J Paediatr Child Health.
43(6):497-8, 2007
3- Butterworth J, Feltis B: Toy magnet ingestion in children: revising the algorithm. J Pediatr Surg. 42(12):e3-5, 2007
4- Dutta S, Barzin A: Multiple magnet ingestion as a source of severe gastrointestinal complications
requiring surgical intervention. Arch Pediatr Adolesc Med. 162(2):123-5, 2008
5- Naji H, Isacson D, Svensson JF, Wester T: Bowel injuries caused by
ingestion of multiple magnets in children: a growing hazard. Pediatr
Surg Int. 28(4):367-7, 2012
6-Salimi A, Kooraki S, Esfahani SA, Mehdizadeh M: Multiple magnet
ingestion: Is there a role for early surgical intervention? Ann Saudi
Med. 32(1):93-6, 2012
HIPEC
Hyperthermic intraperitoneal
chemotherapy (HIPEC) is an alternative of management based on the fact
that hyperthermia and chemotherapy have synergistic cytotoxicity for
microscopic carcinomatous disease. In adults it has been applied
successfully for extensive peritoneal disease associated with such
tumors as mesothelioma, appendiceal, colonic, gastric and ovarian
carcinoma. During closed-technique HIPEC the skin is temporarily closed
and chemotherapy is delivered at supranormal temperatures. Drugs known
to have synergy with hyperthermia include mitomycin C, doxorubicin and
cisplatin. HIPEC has been found to be safe and improve median survival
in children with dermoplastic small round cell tumor after complete
surgical excision. Adult and pediatric patient undergoing HIPEC are a
highly selected group who does not have uncontrolled disease outside
the abdominal cavity. HIPEC and cytoreductive surgery is not
recommended for palliative purposes. Other rare instances where HIPEC
has been used effectively in children include peritoneal metastasis
from melanoma, signet cell colonic carcinoma and Wilms's tumor.
Indications for HIPEC include resectability to no visible disease,
active disease limited to the abdomen, no liver metastasis, normal
liver and kidney function and disease partially responsive to
neoadjuvant chemotherapy.
References:
1- Reingruber B, Boettcher MI, Klein P, Hohenberger W, Pelz JO:
Hyperthermic intraperitoneal chemoperfusion is an option for treatment
of peritoneal carcinomatosis in children. J Pediatr Surg. 42(9):E17-21,
2007
2- Hayes-Jordan A, Green H, Fitzgerald N, Xiao L, Anderson P: Novel
treatment for desmoplastic small round cell tumor: hyperthermic
intraperitoneal perfusion. J Pediatr Surg. 45(5):1000-6, 2010
3- Msika S, Gruden E, Sarnacki S, Orbach D, Philippe-Chomette P, Castel
B, Sabata JM, Flamant Y, Kianmanesh R: Cytoreductive surgery associated
to hyperthermic intraperitoneal chemoperfusion
for desmoplastic round small cell tumor with peritoneal carcinomatosis
in young patients. J Pediatr Surg. 45(8):1617-21, 2010
4- Owusu-Agyemang P, Arunkumar R, Green H, Hurst D, Landoski K,
Hayes-Jordan A: Anesthetic Management and Renal Function in Pediatric
Patients Undergoing Cytoreductive Surgery with Continuous Hyperthermic
Intraperitoneal Chemotherapy (HIPEC) with Cisplatin. Ann Surg Oncol.
2012 Mar 27.
5- Hayes-Jordan A, Green H, Ludwig J, Anderson P: Toxicity of
hyperthermic intraperitoneal chemotherapy (HIPEC) in pediatric patients
with sarcomatosis/carcinomatosis: Early experience and phase 1 results.
Pediatr Blood Cancer. 2012 Apr 10. doi: 10.1002/pbc.24160.