PEDIATRIC SURGERY UPDATE ©
VOLUME 35, 2010
PSU Volume 35 No 01 JULY 2010
Portal Vein Thrombosis
Extrahepatic non-cirrhotic portal vein
thrombosis (PVT) is the second most frequent cause of portal
hypertension in the world. In extrahepatic portal vein obstruction
forward hepatopedal flow from the superior mesenteric vein and splenic
vein is impeded due to obstruction of the portal vein which results in
development of prehepatic portal hypertension and cavernomatous
replacement of the portal vein. The causes of PVT can be split into
four groups: direct injury to the portal vein and consequent thrombus
formation as it occurs in omphalitis or umbilical catheterization;
congenital malformation of the portal system associated with other
cardiovascular disorders; indirect factors that predispose to thrombus
formation in the portal system (sepsis, dehydration,
hypercoagulability); and idiopathic. Children with PVT present with
esophageal varices and bleeding, symptoms of secondary hypersplenism
(splenomegaly) and growth retardation. Anomalies associated with PVT
include heart and large vessel malformation and biliary
(cholelithiasis). With time the child develops collateral circulation
around the PVT along with coagulation disorders, ascites and biliary
cirrhosis. Diagnosis of PVT is established with Doppler ultrasound and
angio CT Scan. Variceal bleeding is managed with medication or
endoscopic sclerotherapy. Persistent bleeding following endoscopic
treatment, prominent splenomegaly with symptomatic hypersplenism,
growth retardation, and symptomatic portal biliopathy may need surgical
portosystemic shunt decompression the mesenteric-portal Rex shunt if
the umbilical portion of the intrahepatic left PV and the superior
mesenteric veins are patent. Otherwise, a splenorenal shunt is
warranted.
References:
1- Mack CL, Superina RA, Whitington PF: Surgical restoration of portal
flow corrects procoagulant and anticoagulant deficiencies associated
with extrahepatic portal vein thrombosis. J Pediatr. 2003 142(2):197-9,
2003
2- Gehrke I, John P, Blundell J, Pearson L, Williams A, de Ville de
Goyet J: Meso-portal bypass in children with portal vein thrombosis:
rapid increase of the intrahepatic portal venous flow after direct
portal hepatic reperfusion. J Pediatr Surg. 38(8):1137-40, 2003
3- Chiu B, Superina R: Extrahepatic portal vein thrombosis is
associated with an increased incidence of cholelithiasis. J Pediatr
Surg. 39(7):1059-61, 2004
4- Schettino GCM, Fagundes EDT, Roquete MLV et al: Portal vein
thrombosis in children and adolescents. Jornal de Pediatria. 82(3):
171-178, 2006
5- Maksoud-Filho JG, GonÃalves ME, Cardoso SR, Gibelli NE,
Tannuri U: Long-term follow-up of children with extrahepatic portal
vein obstruction: impact of an endoscopic sclerotherapy program on
bleeding episodes, hepatic function, hypersplenism, and mortality. J
Pediatr Surg. 44(10):1877-83, 2009
6- Sharif K, McKiernan P, de Ville de Goyet J: Mesoportal bypass for
extrahepatic portal vein obstruction in children: close to a cure
for most! J Pediatr Surg. 45(1):272-6. 2010
Congenital Hyperinsulinism
Congenital hyperinsulinism (CH) is the
most frequent cause of severe, persistent hypoglycemia in neonates and
young infants leading to seizures, developmental delay, and permanent
brain damage. The inappropriate oversecretion of insulin is responsible
for profound hypoglycemia which require aggressive treatment to prevent
severe and irreversible brain damage. CH is a heterogeneous
disorder with two histopathological lesions, diffuse and focal which
are clinically indistinguishable. Focal CH is characterized by a
sporadic somatic islet-cell hyperplasia. Diffuse CH corresponds to a
functional abnormality of insulin secretion in the whole pancreas and
involves several genes with different transmissions. Most cases are
caused by mutations in genes coding for either of the two subunits of
the beta-cell K(ATP) channel (ABCC8 and KCNJ11). In the diffuse
form the hyperinsulinism is due to a recessive mutation of both alleles
of these genes. To differentiate the focal from the diffuse form 18 F
DOPA PET and CT scan is needed. Initial management of CH consist of
diazoxide therapy. Focal lesions are effectively treated by
limited pancreatic resection while diffuse lesions which are
unresponsive to drug or dietary treatment require extensive
laparoscopic or open pancreatectomy. Pancreatic beta-cell dysfunction
persists following subtotal pancreatectomy of diffuse CH.
References:
1- de Lonlay P, Touati G, Robert JJ, Saudubray JM: Persistent
hyperinsulinaemic hypoglycaemia. Semin Neonatol. 7(1):95-100, 2002
2- Fournet JC, Junien C: The genetics of neonatal hyperinsulinism. Horm
Res. 59 Suppl 1:30-4, 2003
3- Lindley KJ, Spitz L: Surgery of persistent hyperinsulinaemic
hypoglycaemia. Semin Neonatol. 2003 Jun;8(3):259-65.
4- Bax KN, van der Zee DC: The laparoscopic approach toward
hyperinsulinism in children. Semin Pediatr Surg. 16(4):245-51, 2007
5- Palladino AA, Bennett MJ, Stanley CA: Hyperinsulinism in infancy and
childhood: when an insulin level is not always enough. Clin Chem.
54(2):256-63, 2008
6- Al-Shanafey S, Habib Z, AlNassar S: Laparoscopic pancreatectomy for
persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr Surg.
44(1):134-8, 2009
Dermatopathic Lymphadenopathy
Adenopathies are a source of concern
for both physicians and patients during the pediatric age. Diagnosis
can be suggested by fine needle aspiration cytology, but other times
complete lymph node removal is warranted. Most adenopathies in children
are benign in nature. Dermatopathic lymphadenopathy (DPL) is
characterized by non-neoplastic lymph node enlargement with reactive
process and is generally caused by chronic inflammatory skin disease.
DPL is usually caused by viral, bacterial, or other specific infections
(tattoo). The morphologic features helpful in the diagnosis of DPL on
fine needle aspiration cytology are melanin-laden macrophages with
variable pigment; large, histiocytic clusters with blood vessels at the
center; characteristic histiocytes, with elongated vesicular nuclei,
nuclear grooves, crumpled and convoluted nuclei and pseudonucleoli; and
absence of or very few tingible body macrophages. DPL is a harmless
cause of enlarged lymph nodes rarely difficult to differentiate from
mycosis fungoides or lymphoma.
References:
1- Hamazaki M, Taniguchi K, Murata H: Immunohistological study of
reactive follicular hyperplasia of lymph node in childhood. Rinsho
Byori. 40(4):423-8, 1992
2- Sudilovsky D, Cha I: Fine needle aspiration cytology of
dermatopathic lymphadenitis. Acta Cytol. 42(6):1341-6, 1998
3- Iyer VK, Kapila K, Verma K: Fine needle aspiration cytology of
dermatopathic lymphadenitis.
Acta Cytol. 42(6):1347-51, 1998
4- Zirkin HJ, Avinoach I, Edelwitz P: A tattoo and localized
lymphadenopathy: a case report. Cutis. 67(6):471-2, 2001
5- Winter LK, Spiegel JH, King T: Dermatopathic lymphadenitis of the
head and neck. J Cutan Pathol.34(2):195-7, 2007
6- Good DJ, Gascoyne RD: Atypical lymphoid hyperplasia mimicking
lymphoma. Hematol Oncol Clin North Am. 23(4):729-45, 2009
PSU Volume 35 No 02 AUGUST 2010
Pancreatic Neuroendocrine Tumors
Neuroendocrine tumors of the upper
gastrointestinal tract are mainly located in the pancreas, stomach or
duodenum. Pancreatic neuroendocrine tumors (PNET) are rare tumors found
in the pancreas that can be either functional or non-functional. PNET
are usually sporadic, but may also be associated with genetic syndromes
such as Von Hippel-Lindau and tuberous sclerosis. PNET are
non-functional in their majority. Functional PNET can secrete insulin,
gastrin, glucagon, somatostatin and vasoactive intestinal polypeptide.
Non-functional PNET can secrete non-active substance such as
neurotensin or chromogranin A. PNET are classified as well
differentiate tumor and well differentiated or poorly differentiated
carcinomas. Most are already metastatic by the time they are diagnosed
with liver the most common site followed by regional lymph nodes.
Surgery with curative intent is the mainstay of treatment for localized
or loco-regional disease. PNET in children should preferably undergo an
organ-sparing resection. Surgery as well as other forms of local
treatment like transarterial chemoembolization or radiofrequency
ablation can also improve prognosis in patients with liver metastases.
For the inoperable cases, cytotoxic therapy with compounds like
streptozotocin, 5-fluorouracil or doxorubicin can achieve modest
outcome. Treatment with somatostatin analogues like octreotide has been
proven to prolong progression-free survival in patients with metastatic
neuroendocrine tumors of midgut origin.
References:
1- Van Nieuwenhove Y, Vandaele S, Op de Beeck B, Delvaux G:
Neuroendocrine tumors of the pancreas. Surg Endosc. 17(10):1658-62, 2003
2- Brüwer M, Pahlov-Nejad T, Herbst H, Senninger N,
Schürmann G: Neuroendocrine tumors of the
gastroenteropancreatic system--reevaluation using the Capella
classification. Zentralbl Chir. 128(8):656-62, 2003
3- Langrehr JM, Bahra M, Kristiansen G, Neumann HP, Neumann LM,
Plöckinger U, Lopez-Hänninen E:
Neuroendocrine tumor of the pancreas and bilateral adrenal
pheochromocytomas. A
rare manifestation of von Hippel-Lindau disease in childhood. J Pediatr Surg. 42(7):1291-4, 2007
4- Breysem L, Kersemans P, Vanbeckevoort D, Ectors N, Smet MH:
Nonfunctioning neuroendocrine tumor of the pancreas in an 8-year-old
girl. JBR-BTR. 90(6):528-31, 2007
5- 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
6- Dimou AT, Syrigos KN, Saif MW: Neuroendocrine Tumors of the
Pancreas: What's New. JOP. J Pancreas (Online) 5; 11(2):135-138,
2010
ERCP in Children
Endoscopic retrograde
cholangiopancreatography (ERCP) is a very important diagnostic and
therapeutic tool for pancreaticobiliary disorders in children and
adults. Biliary indications for ERCP include common bile duct stones,
choledochal cyst, aberrant biliary anatomy, duct stricture, duct
hypoplasia, biliary atresia, postoperative bile leaks and bile duct
injury. ERCP can be therapeutic in extracting bile duct stones,
performing papilla sphincterotomy, cystoduodenostomy, stricture
dilatation and endoprosthesis placement. There is controversy whether
ERCP should be used routinely in choledocholithiasis since spontaneous
passage of common bile duct stones is common in children. Pancreatic
indications for ERCP include recurrent or chronic pancreatitis,
sclerosing cholangitis, pancreas divisum, pseudocysts, trauma and
choledochocele. Therapeutic options for ERCP in pancreatic disorders
include papillotomy and stent placement. General anesthesia will be
needed to perform ERCP in young children, mostly between the ages of
three and 12 years. The morbidity associated with ERCP is very low and
consists of pancreatitis (most common), hemorrhage, duodenal
perforation and cholangitis. Mortality is nil. ERCP is useful and safe
in children, but previous use of diagnostic ultrasound and MRCP to
detect morphologic changes should be encouraged to increase the
therapeutic efficacy of ERCP.
References:
1- 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
2- Cheng CL, Fogel EL, Sherman S, McHenry L, Watkins JL, Croffie JM,
Gupta SK, Fitzgerald JF, Lazzell-Pannell L, Schmidt S, Lehman GA:
Diagnostic and therapeutic endoscopic retrograde
cholangiopancreatography in children: a large series report. J Pediatr
Gastroenterol Nutr. 41(4):445-53, 2005
3- 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
4- Durakbasa CU, Balik E, Yamaner S, et al: Diagnostic and therapeutic
endoscopic retrograde cholangiopancreatography (ERCP) in children
and adolescents: experience in a single institution. Eur J Pediatr
Surg. 18(4):241-4, 2008
5-Shanmugam NP, Harrison PM, Devlin J, et al: Selective use of
endoscopic retrograde cholangiopancreatography in the diagnosis of
biliary atresia in infants younger than 100 days. J Pediatr
Gastroenterol Nutr. 49(4):435-41, 2009
6- Paris C, Bejjani J, Beaunoyer M, Ouimet A: Endoscopic retrograde
cholangiopancreatography is useful and safe in children. J
Pediatr Surg. 45(5):938-42, 2010
Gastrostomy Complications
Gastrostomy is a common and important
procedure that have demonstrated to improve growth of children with
developmental disabilities. A gastrostomy can be constructed open,
percutaneously or laparoscopically. The morbidity associated with
gastrostomy is significant. Most common reported complications in order
of increase frequency include granulation tissue, tube leakage,
accidental tube dislodgement, wound infection, blocked tube, bleeding,
gastroesophageal reflux and colocutaneous fistula. Granulation tissue
can be managed with topical silver nitrate application and Kenalog
oral-base ointment. Tube mechanical problems can be managed with
flushing, change of tube as soon as dislodged, avoiding pressure
necrosis from tight fit, and performing daily tube rotation.
Wound infection if associated with cellulitis might need systemic
antibiotics. Reflux is usually managed medically. Colocutaneous fistula
needs emergency operation. The open procedure causes more complications
that the percutaneous procedure. Laparoscopic gastrostomy in children
avoids the serious complications caused by a blind puncture through the
abdominal cavity when performing the percutaneous procedure. The
frequency of minor ongoing problems necessitates ongoing support of the
child and care of the gastrostomy.
References:
1- Day AS, Beasley SW, Meads A, Abbott GD: Morbidity associated with
gastrostomy placement in children demands an ongoing integrated
approach to care. N Z Med J. 114(1129):164-7, 2001
2- Cameron BH, Blair GK, Murphy JJ 3rd, Fraser GC: Morbidity in
neurologically impaired children after percutaneous endoscopic versus
Stamm gastrostomy. Gastrointest Endosc. 42(1):41-4, 1995
3- Jones VS, La Hei ER, Shun A: Laparoscopic gastrostomy: the preferred
method of gastrostomy in children. Pediatr Surg Int. 23(11):1085-9,
2007
4- Khattak IU, Kimber C, Kiely EM, Spitz L: Percutaneous endoscopic
gastrostomy in paediatric practice: complications and outcome. J
Pediatr Surg. 33(1):67-72, 1998
5- Zamakhshary M, Jamal M, Blair GK, Murphy JJ, Webber EM, Skarsgard
ED: Laparoscopic vs percutaneous endoscopic gastrostomy tube insertion:
a new pediatric gold standard? J Pediatr Surg. 40(5):859-62, 2005
6- Peters RT, Balduyck B, Nour S: Gastrostomy complications in infants
and children: a comparative study. Pediatr Surg Int. 26(7):707-9, 2010
PSU Volume 35 No 03 September 2010
Frey Procedure
Chronic pancreatitis, a disease
process more commonly found in adults than children, generally follows
a progressive course of both pancreatic exocrine (steatorrhea) and
endocrine (diabetes) insufficiency. Chronic pancreatitis in children is
commonly unresponsive to medical therapy and may result in addiction to
pain medication, dietary restrictions, absence from school, and
restriction in the life of the child. Two procedures, namely Puestow
(longitudinal pancreaticojejunostomy) and Duval (distal pancreatectomy
with caudal pancreaticojejunostomy), has varying success depending
mostly if the head of the pancreas is adequately decompressed or not.
The Frey operation is indicated on patients with chronic pancreatitis
who have "head dominant" disease. The Frey procedure consists of an
anterior resection of the head of the pancreas preserving the duodenum
along with a longitudinal pancreaticojejunostomy in order to
improve decompression of the head of the gland. Frey procedure has
proved to provide symptomatic relief and improvement in quality of life
in children and adults. Frey procedure is considered as the standard
procedure in patients with pancreatic head complications and ductal
dilatation associated with chronic pancreatitis. Postoperative
complications after Frey procedure are usually septic in nature and
likely to occur more often in patients in whom endoscopic pancreatic
stenting has been performed before surgical intervention.
References:
1- Frey CF, Child CG, Fry W: Pancreatectomy for chronic pancreatitis. Ann Surg. 184(4):403-13, 1976
2- Izbicki JR, Bloechle C, Knoefel WT, Kuechler T, Binmoeller KF,
Broelsch CE: Duodenum-preserving resection of the head of the pancreas
in chronic pancreatitis. A prospective, randomized trial. Ann Surg.
221(4):350-8, 1995
3- Amikura K, Arai K, Kobari M, Matsuno S: Surgery for chronic
pancreatitis--extended pancreaticojejunostomy. Hepatogastroenterology.
44(18):1547-53, 1997
4- Chaudhary A, Negi SS, Masood S, Thombare M: Complications after
Frey's procedure for chronic pancreatitis. Am J Surg. 188(3):277-81,
2004
5- Rollins MD, Meyers RL: Frey procedure for surgical management of chronic pancreatitis in children. 39(6): 817-820, 2004
6- Falconi M, Bassi C, Casetti L, Mantovani W, Mascetta G, Sartori N,
Frulloni L, Pederzoli P: Long-term results of Frey's procedure for
chronic pancreatitis: a longitudinal prospective study on 40 patients.
J Gastrointest Surg. 10(4):504-10, 2006
7- Chiang KC, Yeh CN, Hsu JT, Chen HM, Chen HY, Hwang TL, Jan YY, Chen
MF: Pancreaticoduodenectomy versus Frey's procedure for chronic
pancreatitis: preliminary data on outcome and pancreatic function. Surg
Today. 37(11):961-6, 2007
8- Andersen DK, Frey CF: The evolution of the surgical treatment of chronic pancreatitis. Ann Surg. 251(1):18-32, 2010
MRCP in Pancreatic Trauma
Magnetic resonance
cholangiopancreatography (MRCP) is a diagnostic method that uses
three-dimensional data sets for projection images, as well as arbitrary
cross-sectional images, of the pancreatic and biliary ducts. MRCP is
simple, comfortable, and requires no contrast media or radiation.
Secretin administration improves ductal visualization, particularly of
nondilated ducts. MRCP may be the diagnostic method of choice when ERCP
is contraindicated or fails. Pancreatic injury has a high morbidity and
mortality. The integrity of the main pancreatic duct is the most
important determinant of prognosis. In the setting of blunt
abdominal pancreatic trauma MRCP has a role in assessing pancreatic
ductal integrity along with specific complications such as pseudocyst
and posttraumatic strictures. Suspicion of ductal injury in MRCP
determines the need for subsequent endoscopic retrograde
cholangiopancreatogram (ERCP). In this era of conservative management
of pancreatic injury in children, ERCP is considered a golden standard
for identifying ductal injury offering the possibility of placing a
ductal stent as primary treatment. Transpapillary drainage is
especially effective in patients who have partial pancreatic duct
disruption that can be bridged and is used to treat
post-traumatic pancreatic pseudocysts.
References:
1- Takehara Y: MR pancreatography: technique and applications. Top Magn Reson Imaging. 8(5):290-301, 1996
2- Fulcher AS, Turner MA, Yelon JA, McClain LC, Broderick T, Ivatury
RR, Sugerman HJ: Magnetic resonance cholangiopancreatography (MRCP) in
the assessment of pancreatic duct trauma and its sequelae: preliminary
findings. J Trauma. 48(6):1001-7, 2000
3- Ragozzino A, Manfredi R, Scaglione M, De Ritis R, Romano S, Rotondo
A: The use of MRCP in the detection of pancreatic injuries after blunt
trauma. Emerg Radiol.10(1):14-8, 2003
4- Houben CH, Ade-Ajayi N, Patel S, Kane P, Karani J, Devlin J,
Harrison P, Davenport M: Traumatic pancreatic duct injury in children:
minimally invasive approach to management. J Pediatr Surg.
42(4):629-35, 2007
5- de Blaauw I, Winkelhorst JT, Rieu PN, van der Staak FH, Wijnen MH,
Severijnen RS, van Vugt AB, Wijnen RM: Pancreatic injury in children:
good outcome of nonoperative treatment. J Pediatr Surg. 43(9):1640-3,
2008
6- Bhasin DK, Rana SS, Rawal P: Endoscopic retrograde pancreatography
in pancreatic trauma: need to break the mental barrier. J Gastroenterol
Hepatol. 24(5):720-8, 2009
7- Rekhi S, Anderson SW, Rhea JT, Soto JA: Imaging of blunt pancreatic trauma. Emerg Radiol. 17(1):13-9, 2010
Pulmonary Blastoma
Pulmonary blastoma (PB) is a very rare
type of embryonal malignant lung tumor believe to arise from the
primitive interstitial mesenchyme of the lung. Other names coined to
this tumor include pulmonary sarcoma, embryonal sarcoma, pulmonary
rhabdomyosarcoma, embryonal rhabdomyosarcoma, and malignant
mesenchymoma. Most cases present before the age of six years. Three
pathologic types are recognized based on their morphologic appearance:
Type I is a cystic lesion that is not distinguishable from other cystic
lesions of the lungs. Type II is a cystic and solid mass, which may be
evident radiologically; and Type III is a solid high-grade sarcoma. It
is believed pulmonary blastomas can arise from a preexisting
cystic lung disease such as congenital adenomatoid malformation,
pulmonary sequestration, bronchogenic cysts and pneumatocele. CT scan
is utilized for diagnosis. A new solid component in an old cystic
lesion of the lung arise suspicious that we are dealing with PB and a
mandatory resection is warranted. Total tumor removal (lobectomy)
offers the only chance of a good long-term outcome. Adjuvant
chemotherapy is reserved for metastatic or residual disease.
References:
1- Kodaira Y, Akiyama H, Morikawa M, Shimizu K: Pulmonary blastoma in a child. J Pediatr Surg. 11(2):239-41, 1976
2- Seballos RM, Klein RL: Pulmonary blastoma in children: report of two
cases and review of the literature. J Pediatr Surg. 29(12):1553-6, 1994
3- Tagge EP, Mulvihill D, Chandler JC, Richardson M, Uflacker R,
Othersen HD: Childhood pleuropulmonary blastoma: caution against
nonoperative management of congenital lung cysts. J Pediatr Surg.
31(1):187-9, 1996
4- Lallier M, Bouchard S, Di Lorenzo M, Youssef S, Blanchard H,
Lapierre JG, Vischoff D, Tucci M, Brochu P: Pleuropulmonary blastoma: a
rare pathology with an even rarer presentation. J Pediatr Surg.
34(7):1057-9, 1999
5- Miniati DN, Chintagumpala M, Langston C, Dishop MK, Olutoye OO,
Nuchtern JG, Cass DL: Prenatal presentation and outcome of children
with pleuropulmonary blastoma. J Pediatr Surg. 41(1):66-71, 2006
6- Nasr A, Himidan S, Pastor AC, Taylor G, Kim PC: Is congenital
cystic adenomatoid malformation a premalignant lesion for
pleuropulmonary blastoma? J Pediatr Surg 45:1086–1089, 2010
PSU Volume 35 No 04 October 2010
CHAOS
Congenital high airway obstruction
syndrome (CHAOS) is a term coined in 1994 to development of complete or
near-complete obstruction of the fetal airway. Though rare, CHAOS is
not always incompatible with life. CHAOS is thought to occur when the
upper airway fails to recanalize around the 10th week of gestation
causing the obstruction. This obstruction blocks the normal egress of
fluid from the lung raising the intratracheal pressure and leading to
distension of the tracheobronchial tree and proliferative lung growth.
The lung expands, the diaphragm flattens and the heart is compressed in
the midline. The elevated intrathoracic pressure causes decreased
venous return, fetal cardiac failure with ascites, placentomegaly and
hydrops fetalis. Diagnosis is performed with prenatal ultrasound and
MRI with findings such as dilated airways distal to the obstruction,
large echogenic lungs, flattened diaphragm, fetal ascites and hydrops.
Most common cause of CHAOS is laryngeal atresia, followed by subglottic
stenosis, laryngeal/tracheal webs or agenesis. The prenatal natural
history and postnatal course of CHAOS depends on whether the airway
obstruction is complete. Survival depends on emergency tracheostomy
immediately after birth with the use of the ex-utero intrapartum
treatment (EXIT) procedure. The presence of hydrops is an ominous sign.
The presence of a pinpoint laryngotracheal or tracheoesophageal fistula
in some fetus with CHAOS will allow airway decompression with
resolution of ascites and diaphragmatic eversion.
References:
1- Hedrick MH, Ferro MM, Filly RA, Flake AW, Harrison MR, Adzick NS:
Congenital high airway obstruction syndrome (CHAOS): a potential for
perinatal intervention. J Pediatr Surg. 29(2):271-4, 1994
2- DeCou JM, Jones DC, Jacobs HD, Touloukian RJ: Successful ex utero
intrapartum treatment (EXIT) procedure for congenital high airway
obstruction syndrome (CHAOS) owing to laryngeal atresia. J
Pediatr Surg. 33(10):1563-5, 1998
3- Lim FY, Crombleholme TM, Hedrick HL, Flake AW, Johnson MP, Howell
LJ, Adzick NS: Congenital high airway obstruction syndrome: natural
history and management. J Pediatr Surg. 38(6):940-5, 2003
4- Shimabukuro F, Sakumoto K, Masamoto H, Asato Y, Yoshida T, Shinhama
A, Okubo E, Ishisoko A, Aoki Y: A case of congenital high airway
obstruction syndrome managed by ex utero intrapartum treatment: case
report and review of the literature. Am J Perinatol. 24(3):197-201, 2007
5- Vaikunth SS, Morris LM, Polzin W, Gottliebson W, Lim FY, Kline-Faith
B, Crombleholme TM: Congenital high airway obstruction syndrome
due to complete tracheal agenesis: an accident of nature with clues for
tracheal development and lessons in management. Fetal Diagn Ther.
26(2):93-7, 2009
6-Roybal JL, Liechty KW, Hedrick HL, Bebbington MW, Johnson MP, Coleman
BG, Adzick NS, Flake AW: Predicting the severity of congenital
high airway obstruction syndrome. J Pediatr Surg 45(8): 1633-1639, 2010
Thoracic Neuroblastoma
Neuroblastoma is the most common
malignant solid tumor in children with almost 20% of them arising in
the thorax. Thoracic neuroblastomas have a better prognosis than those
occurring in other parts of the body. Most cases present before the age
of one year. Mediastinal neuroblastoma becomes symptomatic earlier and
can be detected in an earlier favorable stage. The cell of origin is
the dorsal root sympathetic ganglion cell. In what respect biology
markers, thoracic neuroblastomas have favorable biological profiles
such as DNA index greater than one, significant lower N-myc
amplification, LDH level < 1500 and low ferritin levels. Clinically
the child might demonstrate cough, dyspnea, wheezing, neurogenic cord
compression, dancing eyes' syndrome and Horner syndrome. For diagnosis
the chest films suggest the presence of a posterior mediastinal mass.
CT scan is sensitive predicting chest wall involvement. MRI is very
sensitive for predicting lymph node extension, intraspinal extension
and chest wall involvement. Management consists of complete surgical
excision with adjuvant chemotherapy. Thoracoscopic resection of
neurogenic tumors achieved similar local control and disease-free
survival when compared with open resections. Results that were
accompanied by shorter hospital stay and decreased blood loss.
References:
1- Young DG: Thoracic neuroblastoma/ganglioneuroma. J Pediatr Surg. 1983 Feb;18(1):37-41
2- Adams GA, Shochat SJ, Smith EI, Shuster JJ, Joshi VV, Altshuler G,
Hayes FA, Nitschke R, McWilliams N, Castleberry RP: Thoracic
neuroblastoma: a Pediatric Oncology Group study. J Pediatr Surg.
28(3):372-7, 1993
3- Morris JA, Shcochat SJ, Smith EI, Look AT, Brodeur GM, Cantor AB,
Castleberry RP: Biological variables in thoracic neuroblastoma: a
Pediatric Oncology Group study. J Pediatr Surg. 30(2):296-302, 1995
4- Petty JK, Bensard DD, Partrick DA, Hendrickson RJ, Albano EA, Karrer
FM: Resection of neurogenic tumors in children: is thoracoscopy
superior to thoracotomy? J Am Coll Surg. 203(5):699-703, 2006
5- Demir HA, Yalçin B,
Büyükpamukçu N, Kale G,
Varan A, Akyüz C, Kutluk T,
Büyükpamukçu M: Thoracic
neuroblastic tumors in childhood. Pediatr Blood Cancer. 54(7):885-9,
2010
6- Fraga JC, Aydogdu B, Aufieri R, Silva GV, Schopf L, Takamatu E,
Brunetto A, Kiely E, Pierro A: Surgical treatment for pediatric
mediastinal neurogenic tumors. Ann Thorac Surg. 90(2):413-8, 2010
7- Malek MM, Mollen KP, Kane TD, Shah SR, Irwin C: Thoracic
neuroblastoma: a retrospective review of our institutional experience
with comparison of the thoracoscopic and open approaches to resection.
J Pediatr Surg. 45(8):1622-1626, 2010
Anal Achalasia
Internal anal sphincter achalasia
(IASA) is a condition with similar clinical presentation to
Hirschsprung's disease, but with ganglion cells present in the rectal
biopsy. Theories on pathogenesis include nitregic nerve depletion,
defective innervation of the neuromuscular junction and altered
distribution of interticial cells of Cajal, considered the pacemakers
of the bowel. Children with anal achalasia present with severe
constipation with or without soiling. Diagnosis of IASA is made with
anorectal manometry which shows the absence of the rectoinhibitory
reflex upon rectal balloon inflation with presence of ganglion cells
and normal acethycholinesterase activity in a rectal biopsy. Management
consists of posterior internal sphincter myotomy. Intrasphincteric
injection of Clostridium botulinum toxin has also been tried causing a
local and six-month transient denervation of the sphincter. The
majority of patients with internal anal sphincter achalasia can be
treated successfully by internal sphincter myectomy. In the long
term, a significant number of patients have been found to suffer from
soiling-related social problems.
References:
1- De Caluwé D, Yoneda A, Akl U, Puri P: Internal anal
sphincter achalasia: outcome after internal sphincter myectomy. J
Pediatr Surg. 36(5):736-8, 2001
2- Messineo A, Codrich D, Monai M, Martellossi S, Ventura A: The
treatment of internal anal sphincter achalasia with botulinum toxin.
Pediatr Surg Int. 17(7):521-3, 2001
3- Heikkinen M, Lindahl H, Rintala RJ: Long-term outcome after internal
sphincter myectomy for internal sphincter achalasia. Pediatr Surg Int.
21(2):84-7, 2005
4- Chumpitazi BP, Fishman SJ, Nurko S: Long-term clinical outcome after
botulinum toxin injection in children with nonrelaxing internal anal
sphincter.Am J Gastroenterol. 104(4):976-83, 2009
5- Doodnath R, Puri P: Long-term outcome of internal sphincter myectomy
in patients with internal anal sphincter achalasia. Pediatr Surg Int.
25(10):869-71, 2009
6- Doodnath R, Puri P: Internal anal sphincter achalasia. Semin Pediatr Surg. 18(4):246-8, 2009
PSU Volume 35 No 05 November 2010
Neonatal Liver Hemorrhage
Hemorrhage from the liver in the
neonatal period is a very serious and lethal injury. Neonatal liver
bleeding can occur spontaneously during a surgical procedure, the
result of birth trauma, associated with sepsis, coagulopathy
or an acquired liver malformation. Breech delivery can be
associated with spontaneous blunt hemorrhage into the liver, adrenals,
kidney or spleen. Symptoms of abdominal distension, a contiguous mass
effect in the right upper quadrant, anemia and shock appear 48 hours
after the event. Blunt hepatic hemorrhage has also been reported within
24 hours of birth in babies carrying a congenital cavernous subcapsular
liver hemangioma due to the tensile strength of this part of the liver.
Performing surgery in very small premature babies with necrotizing
enterocolitis can be associated with intraoperative spontaneous
bleeding from the liver. In these cases severe liver hemorrhage could
be stabilized by early liver tamponade using absorbable thrombostatic
sponges and polyglactin mesh, argon beam coagulation, thrombin and
fibrin glue application or topical hemostatic agents. Other times
recombinant factor VIIa has been needed to manage such events.
Significant increased fluid requirements and persistent hypotension are
ominous findings in the preoperative period and important predictors of
the development of liver hemorrhage in necrotizing enterocolitis. Since
bleeding occurs near the falciform ligament it is believe that it acts
as a fulcrum on which the expanding liver would tear.
References:
1- Strear CM, Graf JL, Albanese T et al: Successful Treatment of Liver
Hemorrhage in the Premature Infant. J Pediatr Surg 33(6): 849-851, 1998
2- Pumberger W, Kohlhauser C, Mayr M, Pomberger G: Severe liver
hemorrhage during laparotomy in very low birthweight infants. Acta
Paediatr. 91(11):1260-2, 2002
3- Metzelder ML, Springer A, August C, Willital GH: Neonatal
hemoperitoneum caused by a congenital liver angioma. J Pediatr Surg.
39(2):234-6, 2004
4- Amodio J, Fefferman N, Rivera R, Pinkney L, Strubel N: Idiopathic
intraparenchymal hematoma of the liver in a neonate. Pediatr Radiol.
34(4):358-61, 2004
5- Foss K: A case report of a low-birth-weight infant with a
subcapsular liver hematoma and spontaneous bowel perforation. Adv
Neonatal Care. 4(2):67-78, 2004
6- Filan PM, Mills JF, Clarnette TD, Ekert H, Ekert P: Spontaneous
liver hemorrhage during laparotomy for necrotizing enterocolitis: a
potential role for recombinant factor VIIa. J Pediatr. 147(6):857-9,
2005
7- Raghavan M, Stansfield J: Spontaneous liver hemorrhage during
laparotomy in a preterm infant. Paediatr Anaesth. 18(7):671-2, 2008
Solitary Rectal Ulcer Syndrome
Solitary rectal ulcer syndrome (SRUS)
is a very rare cause of rectal bleeding in children. It is more
commonly found in adults. Often goes unrecognized or misdiagnosed. In
common with adults there is often a long duration of symptoms prior to
diagnosis. The etiology of SRUS is multifactorial. Significant factors
include ischemia with trauma due to dysfunctional defecation associated
with anterior rectal prolapse. Some form of rectal prolapse is commonly
found during videoproctography. Clinically the child manifests with
bright rectal bleeding, constipation, mucous discharge, tenesmus,
perineal and lower abdominal pain. Most cases are males. Endoscopic
findings include ulcerated, polypoid, or plaque-like lesions
characteristically found in the anterior rectal wall. Histology shows
fibrous obliteration of the lamina propria with disorientation of
smooth muscle fibers and hypertrophy of the muscularis mucosa with
distorted crypts. The diagnosis is establish using endoscopic biopsy,
proctography and anorectal manometry. Most children respond well to
conservative therapy. Initial management of SRUS consist of bulk
laxatives, high fiber diet, biofeedback, behavior modification or
Argon plasma coagulation. Surgical management is reserved for
intractable cases and consist of local excisional procedures and/or
rectopexy.
References:
1-Godbole P, Botterill I, Newell SJ, Sagar PM, Stringer MD: Solitary
rectal ulcer syndrome in children. JR Coll Surg Edinb 45(6): 411-414,
2000
2- Kiriştioğlu I, Balkan E, Kiliç
N, DoÄŸruyol H: Solitary rectal ulcer syndrome in children.
Turk J Pediatr. 2000 Jan-Mar;42(1):56-60
3- Gabra HO, Roberts JP, Variend S, Shawis RN: Solitary rectal ulcer
syndrome in children. A report of three cases. Eur J Pediatr Surg. 2005
Jun;15(3):213-6.
4- MartÃn de Carpi J, Vilar P, Varea V: Solitary rectal ulcer
syndrome in childhood: a rare, benign, and probably misdiagnosed cause
of rectal bleeding. Report of three cases. Dis Colon Rectum. 2007
Apr;50(4):534-9
5- Keshtgar AS: Solitary rectal ulcer syndrome in children. Eur J Gastroenterol Hepatol. 2008 Feb;20(2):89-92.
6-Somani SK, Ghosh A, Avasthi G, Goyal R, Gupta P: Healing of solitary
rectal ulcers with multiple sessions of argon plasma coagulation. Dig
Endosc. 2010 Apr;22(2):107-11
Granulomatous Appendicitis
Granulomatous inflammation of the
appendix is a very rare form of appendicitis in children and adults. It
is usually associated with a secondary systemic disease process such as
Crohn's disease, foreign body reaction, tuberculosis, sarcoidosis,
schistosomiasis, or Yersinia pseudotuberculosis. With absence of the
above disorders it is termed primary or idiopathic granulomatous
appendicitis. Patients' presented with pain in the right lower quadrant
of the abdomen frequently associated with a mass and a protracted
preoperative course. Most children undergo primary appendectomy.
Prominent histologic features included epithelioid granulomas with
lymphoid cuffing, central necrosis, transmural inflammation with
lymphoid aggregates, mucosal ulceration, and cryptitis. Few children
develop Crohn's later in life and postoperative fistulas occur very
infrequently. Delayed or interval appendectomy specimens often have a
characteristic inflammatory pattern that includes granulomas,
xanthogranulomatous inflammation, mural fibrosis/thickening, and
transmural chronic inflammation, changes that may be misinterpreted as
Crohn disease. The prognosis of idiopathic granulomatous appendicitis
is favorable.
References
1- Allen DC, Biggart JD: Granulomatous disease in the vermiform appendix. J Clin Pathol. 36(6):632-8, 1983
2- Timmcke AE: Granulomatous appendicitis: is it Crohn's disease? Report of a case and review of
the literature. Am J Gastroenterol. 81(4):283-7, 1986
3- Mazziotti MV, Marley EF, Winthrop AL, Fitzgerald PG, Walton M,
Langer JC: Histopathologic analysis of interval appendectomy specimens:
support for the role of interval appendectomy. J Pediatr Surg.
32(6):806-9, 1997
4- Lamps LW, Madhusudhan KT, Greenson JK, Pierce RH, Massoll NA, Chiles
MC, Dean PJ, Scott MA: The role of Yersinia enterocolitica and Yersinia
pseudotuberculosis in granulomatous appendicitis: a histologic and
molecular study. Am J Surg Pathol. 25(4):508-15, 2001
5- Guo G, Greenson JK: Histopathology of interval (delayed)
appendectomy specimens: strong association with granulomatous and
xanthogranulomatous appendicitis. Am J Surg Pathol. 27(8):1147-51, 2003
6- Yayla D, Alpman BN, Dolek Y: Granulomatous appendicitis in a 12-year-old boy. J Pediatr Surg. 45(9):e27-9, 2010
PSU Volume 35 NO 06 December 2010
Pneumatosis Cystoides Intestinalis
Pneumatosis cystoides intestinalis
(PCI) is a condition characterized by formation of multiple gas-filled
cysts located within the wall (subserosa or submucosa) of the
gastrointestinal tract. They pneumatosis can be located in any point of
the GI tract. In neonates, PCI is associated with necrotizing
enterocolitis. Infant and older children can develop PCI associated
with immunosuppression (bone marrow transplant or chemotherapy),
collagen vascular disease, aganglionosis, colonoscopy, steroid therapy,
bacterial infection with Clostridium species, viral infection with
rotavirus and cytomegalovirus, short bowel syndrome, bowel obstruction,
and congenital heart disease. Following a mechanical (increase
intraluminal pressure), bacterial (producing gas) and biochemical (high
partial pressure of hydrogen) theory of origin of the pneumatosis, the
exact mechanism is still unknown. Clinical presentation includes
abdominal distension, bloody diarrhea, bilious vomits, lethargy and
hypotension. Management consists of aggressive fluid therapy, frequent
clinical and radiological examinations, systemic antibiotics, bowel
rest and nutritional support. Worrisome CT findings include thickening
of bowel wall, free fluid and periintestinal stranding. Surgery is
indicated if the child develops progressive deterioration, free air,
metabolic acidosis, severe hemorrhage, obstruction or signs of
peritoneal irritation.
References:
1- West KW, Rescorla FJ, Grosfeld JL, Vane DW: Pneumatosis intestinalis
in children beyond the neonatal period. J Pediatr Surg. 24(8):818-22,
1989
2- Reynolds HL Jr, Gauderer MW, Hrabovsky EE, Shurin SB: Pneumatosis
cystoides intestinalis in children beyond the first year of life:
manifestations and management. J Pediatr Surg. 26(12):1376-80, 1991
3- D'Agostino S, Fabbro MA, Musi L, Bozzola L: Pneumatosis cystoides
intestinalis: a rare cause of nonsurgical pneumoperitoneum in an
infant. J Pediatr Surg. 35(7):1106-8, 2000
4- Fenton LZ, Buonomo C: Benign pneumatosis in children. Pediatr Radiol. 30(11):786-93, 2000
5- Kurbegov AC, Sondheimer JM: Pneumatosis intestinalis in non-neonatal pediatric patients. Pediatrics. 108(2):402-6, 2001
6- Olson DE, Kim YW, Ying J, Donnelly LF: CT predictors for
differentiating benign and clinically worrisome pneumatosis
intestinalis in children beyond the neonatal period.Radiology.
253(2):513-9, 2009
Cells of Cajal
The interstitial cell of Cajal (ICC)
are the smooth muscle pacemakers cells of spontaneous motility in the
gut. The ICC network is widely distributed within the submucosal,
intramuscular and intermuscular (between the circular and longitudinal
muscles) layers of the gut wall. ICC serves as electrical pacemakers,
provides pathways for the active propagation of slow waves, are
mediators of enteric motor neurotransmission and play a role in
afferent neural signaling. This motor neurotransmission occurs through
specialized synapses that exist between enteric nerve terminals and
ICC. Digestive motility consists of non-propulsive mixing
(segmental) and propulsive (peristalsis) movement. Electron microscopy
and immunochemistry have demonstrated reduced number, density and
structural abnormalities in the ICC of gut motility conditions such as
achalasia, gastroesophageal reflux, gastroparesis, infantile pyloric
stenosis, segmental dilatation of the intestine, chronic intestinal
pseudo-obstruction, Hirschsprung's disease, neuronal intestinal
dysplasia, internal anal sphincter achalasia and slow transit
constipation. Gastrointestinal stromal tumors originate from the ICC.
In the gut musculature, ICC and mast cells are the only cells that have
prominent c-kit expression. c-Kit is a transmembrane protein kinase
which has as ligand stem cell factor and is involved in cell
development in a variety of cell lineages.
References:
1- Ward SM, Sanders KM: Involvement of intramuscular interstitial cells of Cajal in neuroeffector
transmission in the gastrointestinal tract. J Physiol. 576(Pt 3):675-82, 2006
2- Streutker CJ, Huizinga JD, Driman DK, Riddell RH: Interstitial cells
of Cajal in health and disease. Part I: normal ICC structure and
function with associated motility disorders. Histopathology.
50(2):176-89, 2007
3- Rolle U, Piaseczna-Piotrowska A, Puri P: Interstitial cells of Cajal
in the normal gut and in intestinal motility disorders of childhood.
Pediatr Surg Int. 23(12):1139-52, 2007
4- Negreanu LM, Assor P, Mateescu B, Cirstoiu C: Interstitial cells of
Cajal in the gut--a gastroenterologist's point of view. World J
Gastroenterol. 14(41):6285-8, 2008
5- Burns AJ, Roberts RR, Bornstein JC, Young HM: Development of the
enteric nervous system and its role in intestinal motility during fetal
and early postnatal stages. Semin Pediatr Surg. 18(4):196-205, 2009
6- Mostafa RM, Moustafa YM, Hamdy H: Interstitial cells of Cajal, the
Maestro in health and disease. World J Gastroenterol.
14;16(26):3239-48, 2010
7- Okada T, Sasaki F, Honda S, Cho K, Matsuno Y, Itoh T, Kubota KC,
Todo S: Disorders of interstitial cells of Cajal in a neonate with
segmental dilatation of the intestine. J Pediatr Surg.
45(6):e11-4, 2010
Postoperative Ileus
Ileus refers to any obstruction of the
intestine. Postoperative ileus occurs after a major surgical procedure
and is an important cause of postoperative discomfort and prolonged
hospital stay. Operations that involve large incision, extensive bowel
manipulation and peritoneal irritatives such as blood and pus more
commonly results in postop ileus. Ileus is characterized by lack of
coordinated and reduced peristalsis. The patients complain of
cramping, abdominal pain and nausea. No test confirms or excludes the
diagnosis of ileus. Ileus that fail to resolved after the fifth postop
day might be caused by an associated abscess, anastomotic leak,
inflammation, intussusception or early adhesion. Ileus is resolving
when the patient passes flatus or a bowel movement. Ileus resolves when
the patient tolerates oral feeding without significant abdominal
symptoms. After surgery the small bowel recovers first, the stomach
next and the colon last within a period of 3-5 days. Sympathetic nerve
activity due to peritoneal irritation reduces acetylcholine release and
inhibits bowel motility. Traditional treatment includes bowel
rest and NG suction. Novel approach includes early enteral feeding,
minimal to none NG suction, chewing gum (sham feeding), thoracic
epidural catheter, ketorolac, intraoperative steroids and use of
laparoscopy.
References:
1- Burd RS, Cartwright JA, Klein MD: Factors associated with the
resolution of postoperative ileus in newborn infants. Int J Surg
Investig. 2(6):499-502, 2001
2- Mattei P, Rombeau JL: Review of the pathophysiology and management of postoperative ileus.
World J Surg. 30(8):1382-91, 2006
3- Zhang Q, Zhao P: Influence of gum chewing on return of gastrointestinal function after gastric
abdominal surgery in children. Eur J Pediatr Surg. 18(1):44-6, 2008
4- Noble EJ, Harris R, Hosie KB, Thomas S, Lewis SJ: Gum chewing
reduces postoperative ileus? A systematic review and meta-analysis. Int
J Surg. 7(2):100-5, 2009
5- CavuÅŸoÄŸlu YH, Azili MN, Karaman A, Aslan MK,
Karaman I, Erdoğan D, Tütün O:
Does gum chewing reduce postoperative ileus after intestinal resection
in children? A prospective randomized controlled trial. Eur J Pediatr
Surg. 19(3):171-3, 2009