PEDIATRIC SURGERY UPDATE ©
VOLUME 14, 2000
Volume 14 No 01 JANUARY 2000
Rhabdomyosarcoma - Genetics
Most rhabdomyosarcomas (RMS) occurs sporadically. A few are associated
to syndromes such as Beckwith-Wiedemann, Li-Fraumeni, and Neurofibromatosis
I. Risk factors in the development of RMS include maternal use of marijuana
& cocaine, exposure to radiation, and maternal history of stillbirth.
Alveolar (aRMS) and embryonal RMS (eRMS) are the most genetically studied
sarcomas in children. The most consistent genetic mutation identified in
more than 70% of aRMS is translocation of chromosomes 2 and 13, t(2;13)q35-37;q14).
The PAX3 loci in chromosome 2 fuses to the FKHR (fork head in RMS) domain
of chromosome 13 creating a powerful chimeric PAX3-FKHR gene. Another of
the reported translocation is t(1;13)(p36;q14) involving chromosome 1 and
13 in 10% of aRMS. In this variant Chromosome 1 locus encoding PAX7 fused
to FKHR in chromosome 13 resulting in another chimeric transcript PAX7-FKHR.PAX7-FKHR
tumors tend to occur in younger patients, more often in the extremity,
more often localized lesions and are associated with significantly longer
event-free survival. Identification of fusion gene status may be a useful
diagnostic tool in differentiating RMS from other round cell tumors. eRMS
contains frequent allelic loss on chromosome 11 (11p15), a genetic feature
specific for this type of tumor.The presence of a consistent region of
allelic loss is indicative of the presence of a tumor suppressor gene that
is inactivated. This leads to overexpression of insulin-like growth factor
II gene that is known to play a role in the development of embryonal tumors.
Other alterations associated with eRMS are distint patterns of chromosomal
gains in contrast with aRMS which shows genomic amplification. Both tumors
share alterations in the p53 gene at the germline level contributing to
increase susceptibility to other tumors characteristics of the Li-Fraumeni
syndrome.
References
1- Barr FG: Molecular Genetics and Pathogenesis of Rhabdomyosarcoma.
J Pediatr Hematol Oncol 19(6): 483-491, 1997
2- Rubnitz JE, Crist WM: Molecular Genetics of Childhood
Cancer: Implications for Pathogenesis, Diagnosis, and Treatment. Pediatrics
100(1): 101-108, 1997
3- Kelly KM, Womer RB, Sorensen PH, Xiong QB, Barr
FG: Common and variant gene fusions predict distinct clinical phenotypes
in rhabdomyosarcoma. J Clin Oncol 15(5):1831-6, 1997
4- Frascella E, Toffolatti L, Rosolen A: Normal and rearranged
PAX3 expression in human rhabdomyosarcoma. Cancer Genet Cytogenet 15;102(2):104-9,
1998
5- Bernasconi M, Remppis A, Fredericks WJ: Induction
of apoptosis in rhabdomyosarcoma cells through down-regulation of PAX proteins.
Proc Natl Acad Sci U S A 12;93(23):13164-9, 1996
6- Weber-Hall S, Anderson J, McManus A, Abe S, Nojima
T, Pinkerton R, Pritchard-Jones K, Shipley J: Gains, losses, and amplification
of genomic material in rhabdomyosarcoma analyzed by comparative genomic
hybridization. Cancer Res 15;56(14):3220-4, 1996
7- Grosfeld JL: Risk-Based Management: Current Concepts
of Treating Malignant Solid Tumors of Childhood. J Am Coll Surg 189(4):
407-425, 1999
Intestinal Non-Hodgkin Lymphoma
In 1.6% of all ileo-colic intussusception in children a malignant Non-Hodgkin
Lymphoma (NHL) is the culprit. Primary gastrointestinal NHL usually present
with a median age of eight years, colicky abdominal pain, bloody stools
and palpable mass. Is the most frequent extranodal lymphoma. Other times
symptoms take the form of nonspecific abdominal pain, intestinal obstruction
or mimic appendicitis. Intestinal NHL has rapid doubling times (12-36 hrs)
making it sensitive to cytotoxic drugs. During presentation localized or
disseminated disease is seen evenly. Children with actual or simulated
acute abdominal conditions are the ones that receive early therapy and
have best survival (Stage I/II disease). Bulk disease in the abdomen as
predicted by levels of LDH, interleukin II receptor and B2 microglobulin
level determines outcome. Complete resection, absence of bone marrow and
CNS involvement offers the best five-year survival rates (80-97%). Suspicious
lymph nodes should be removed, but functional impairment is not justified.
Most of these children will receive minimal adjuvant chemotherapy. In case
of large retroperitoneal tumors, disseminated or metastatic disease aggressive
debulking should be avoided and the role of surgery should be aimed at
establishing a tissue diagnosis as fast as possible. Debulking is associated
with greater complications and delay in instituting primary chemotherapy.
Bowel perforation carries the worst prognosis.
References
1- Wayne ER, Campbell JB, Kosloske AM, Burrington JD:
Intussusception in the older child- suspect lymphosarcoma. J Pediatr Surg
11(5):789-94, 1976
2- Stovroff MC, Coran AG, Hutchinson RJ: The role of
surgery in American Burkitt's lymphoma in children. J Pediatr Surg 26(10):1235-8,
1991
3- LaQuaglia MP, Stolar CJ, Krailo M, Exelby P: The role
of surgery in abdominal non-Hodgkin's lymphoma: experience from the Childrens
Cancer Study Group. J Pediatr Surg 27(2):230-5, 1992
4- Watanabe Y, Ito T, Horibe K, Ishiguro Y, Nimura Y:
Advanced primary non-Hodgkin's lymphoma of the small intestine in childhood:
report of four cases. Surg Today 24(11):1023-7, 1994
5- Reiter A, Zimmermann W, Zimmermann M, von Schweinitz
D, Riehm H, Mildenberger H: The role of initial laparotomy and second-look
surgery in the treatment of abdominal B-cell non-Hodgkin's lymphoma of
childhood. A report of the BFM Group. Eur J Pediatr Surg 4(2):74-81, 1994
6- Gahukamble DB, Khamage AS: Limitations of surgery
in intraabdominal Burkitt's lymphoma in children. J Pediatr Surg 30(4):519-22,
1995
7- Yanchar NL, Bass J: Poor outcome of gastrointestinal
perforations associated with childhood abdominal non-Hodgkin's lymphoma.
J Pediatr Surg 34(7):1169-74, 1999
Ectopic Pancreas
Ectopic pancreatic tissue is found incidentally or may cause problems
when associated with a duplication cyst, a Meckel diverticulum, in the
stomach, in a congenital duodenal diaphragm and in the ileum (as lead point
of an intussusception). The presence of ectopic tissue in patients with
Meckel's diverticulum is a main risk for occurrence of an acute nonmechanical
complication. Most heterotopic pancreas identified incidentally in asymptomatic
children occurs as a patch of tissue in the serosal surface of the proximal
jejunum with fully formed acinar tissue, islets and draining ducts. Incidental
removal is not indicated unless the location harbingers problems. Ectopic
pancreatic tissue is susceptible to drugs also.
References
1- Artigas V, Calabuig R, Badia F, Rius X, Allende L:
Meckel's diverticulum: value of ectopic tissue. Am J Surg 151(5):631-4,
1986
2- Pang LC: Pancreatic heterotopia: a reappraisal and
clinicopathologic analysis of 32 cases. South Med J ;81(10):1264-75, 1988
3- Salman B, Besbas N, Coskun T, Yilmazbayhan D, Sarialioglu
F: Intussusception due to ectopic pancreatic tissue in a nine-month-old
child. Turk J Pediatr 34(4):255-8, 1992
4- Rubesin SE, Furth EE, Birnbaum BA, Rowling SE: Ectopic
pancreas complicated by pancreatitis and pseudocyst formation mimicking
jejunal diverticulitis. Br J Radiol 70:311-3, 1997
5- Abel R, Keen CE, Bingham JB, Maynard J, Agrawal MR:
Heterotopic pancreas as lead point in intussusception: new variant of vitellointestinal
tract malformation. Pediatr Dev Pathol 2(4):367-70, 1999
Volume 14 No 02 FEBRUARY 2000
Neuroblastoma - Genetics
Neuroblastoma (NB) is a malignant tumor of the sympathetic system that
develops from the neural crests: sympathetic ganglion cells and adrenal
glands. NB can behave seemingly benign, undergo spontaneous regression,
mature into a benign ganglioneuroma or progress to kill its host. The most
characteristic genetic abnormality of NB is deletion of the short arm of
chromosome 1 (1p) occurring in 70% of primary diploid tumors. Loss or inactivation
of a tumor suppressor gene at this site is critical for progression of
neuroblastoma. Loss of heterozygosity in chromosome 14 long arm (14q) has
also been identified in 50% of NB cells studied with no clinical behavior
identified. Gain of chromosome 17 is associated with more aggressive tumors.
Another consistent chromosomal aberration identified in 25% of NB
is the presence of double-minute chromosomes producing multiple copies
of the oncogene N-myc. N-myc activation results in tumor formation
and is strongly associated with advance stages of disease and poor outcome
independent of the stage of the tumor or age of the patient. Most NB cells
are diploid and many are hyperdiploid. Hyperdiploidy is a good prognostic
feature of NB, while diploid tumors at any age and hyperdiploid in older
patients carry a worse prognosis requiring more intensive treatment. TRK
receptors (A, B and C) are detected in 90% of NB and correlates inversely
with N-myc expression. High TRK-A correlates with improved survival playing
a role in the propensity of NB to regress or differentiate. TRK-B is associated
with more matured tumors and TRK-C with lower stage tumors. High levels
of TRK expression are associated with better prognosis, earlier stage,
lower patient age and lack of N-myc expression.
References
1- Rubnitz JE, Crist WM: Molecular Genetics of Childhood
Cancer: Implications for Pathogenesis, Diagnosis, and Treatment. Pediatrics
100(1): 101-108, 1997
2- Kreissman SG: Molecular Genetics: Toward an Understanding
of Childhood Cancer. Sem Pediatr Surg 2(1): 2-10, 1993
3- Castleberry RP: Clinical and biologic features in
the prognosis and treatment of neuroblastoma. Curr Opin Oncol 4(1):116-23,
1992
4- Tracy T Jr, Weber TR: Current concepts in neuroblastoma.
Surg Annu 1:227-45, 1992
5- Hata Y, Sasaki F, Naito H, et al: Late recurrence
in neuroblastoma. J Pediatr Surg 26(12):1417-9, 1991
6- Ciccarone V, Spengler BA, Meyers MB, et al: Phenotypic
diversification in human neuroblastoma cells: Expression of distinct neural
crest lineages. Cancer Res 49: 219-225, 1989
7- Brodeur GM, Maris JM, Yamashiro DJ, et al: Biology
and Genetics of Human Neuroblastomas. J Pediatr Hematol Oncol 19(2): 93-101,
1997
Recurrent Abdominal Pain
Recurrent abdominal pain (RAP) severe enough to seek surgical advice
is a common problem in children. Most of the children with RAP are females.
During evaluation multiple imaging (US, CT-Scan, MRI, HIDA-CCK, barium
studies) and endoscopic diagnostic studies are done, most of which are
not too helpful in arriving with a diagnosis. Symptoms wane spontaneously
in almost one-third of these cases after a 4-6 week period of conservative
observation. With persistent symptoms a diagnostic laparoscopy can be offered
due to its superior visualization of pelvic and abdominal structures. Those
with right lower quadrant pain are an important group, since adhesions,
chronic appendiceal inflammation and inspissated appendicolith have been
identified. With laparoscopy for RAP the yield of abdominal pathology will
increase (chronic appendicitis, cysts, Meckel's and hernias), school loss
will minimize and an economic benefit will be obtained. The lap procedure
has lent itself to a variety of patients because of its ease, safety, and
diagnostic accuracy.
References
1-Stringel G, Berezin SH, Bostwick HE, Halata MS: Laparoscopy
in the Management of Children with Chronic Recurrent Abdominal Pain. JSLS
Vol. 3:215-219, 1999.
2 -Stylianos S, Stein JE, Flanigan LM, Hechtman DH: Laparoscopy
for Diagnosis and Treatment of Recurrent Abdominal Pain in Children. J
Pediatr Surg. 31(8): 1158-1160, 1996
3- Miller K, Mayer E, Moritz E: The role of laparoscopy
in chronic and recurrent abdominal pain. Am J Surg 172(4):353-6, 1996
4- Morfesis FA, Ahmad F: Use of laparoscopy in the treatment
of acute and chronic right lower quadrant pain. J Ky Med Assoc 94(1):16-21,
1996
5- Schier F, Waldschmidt J: Laparoscopy in children with
ill-defined abdominal pain. Surg Endosc 8(2):97-9, 1994
Muscle Biopsy
Muscle biopsy is a standard procedure to help establish a diagnosis
in case of suspecting myopathy in children. Though the procedure is technically
easy, complications can occur the result of not using appropriate anesthesia
strategy. We encourage initial sedation with midazolin, followed by ketamine
or propofol along with local anesthesia (xylocaine/marcaine 1-2% mixture)
in this cases. We strongly discourage and avoid the use of halogenated
gases or muscle paralysis whenever possible. Most frequent cause of cardiac
arrest causing sudden death following anesthesia in a child suspected of
having a muscular dystrophy (Duchenne, or any other type) with elevated
CPK level is the result of rhabdomyolysis caused by halogenated gases (halothane,
isoflurane, sevoflurane, etc.) or muscle-paralysis drugs (succinylcholine,
suxamethonium). Rhabdomyolysis triggers severe hyperkalemia, hyperphosphatemia,
hypocalcemia, massive increases in creatine kinase, aspartate aminotransferase,
and alanine aminotransferase concentrations, and high ion gap metabolic
acidosis. Following unexplained anesthetic deaths, pathologists should
examine body fluid electrolytes and skeletal muscle for myopathy and dystrophin.
References
1- Obata R, Yasumi Y, Suzuki A, Nakajima Y, Sato S: Rhabdomyolysis
in association with Duchenne's muscular dystrophy. Can J Anaesth 46(6):564-6,
1999
2- Miller ED Jr, Sanders DB, Rowlingson JC, Berry FA
Jr, Sussman MD, Epstein RM: Anesthesia-induced rhabdomyolysis in a patient
with Duchenne's muscular dystrophy. Anesthesiology 48(2):146-8, 1978
3- Sethna NF, Rockoff MA, Worthen HM, Rosnow JM: Anesthesia-related
complications in children with Duchenne muscular dystrophy. Anesthesiology
68(3):462-5, 1988
4- Sullivan M, Thompson WK, Hill GD: Succinylcholine-induced
cardiac arrest in children with undiagnosed myopathy. Can J Anaesth 41(6):497-501,
1994
5- McKishnie JD, Muir JM, Girvan DP: Anaesthesia induced
rhabdomyolysis--a case report. Can Anaesth Soc J 30(3 Pt 1):295-8, 1983
6- Moore WE, Watson RL, Summary JJ: Massive myoglobinuria
precipitated by halothane and succinylcholine in a member of a family with
elevation of serum creatine phosphokinase. Anesth Analg 55(5):680-2, 1976
Volume 14 No 03 MARCH 2000
Frantz Tumor
Papillary cystic tumor of the pancreas, also known as Frantz tumor (FT)
since 1959, occurs predominantly in girls and young women (mean age 21
years). Abdominal pain and a slowly growing incidentally found epigastric
mass is the most common complains, associated at times with weight loss,
anorexia and vomiting. FT is well-encapsulated, shows solid and hemorrhagic
patterns, contain PAS-positive cytoplasmic or prozymogen granules as seen
in acinar cell tumors and behaves as a low-grade malignancy. CT scans suggest
the diagnosis (thick capsule, calcifications, mixed solid and cystic patterns,
grows toward the outside of the pancreas). Differential diagnosis includes
traumatic pseudocysts, serous and mucinous cystadenomas of the pancreas.
Immunohistochemically the tumor is positive for alpha-1-antitrypsin while
negative for insulin and glucagon. Complete removal is the treatment of
choice for tumor arising in any part of the pancreas. FT is frequently
amenable to local resection and has a good long-term survival rate after
excision. Metastasis (liver) or local recurrence occurs in 10% of cases.
Older age at diagnosis or recurrence disease increases the malignant biological
behavior of the tumor. Radiotherapy and, or chemotherapy are of no use
for its treatment.
References
1- Todani T, Shimada K, Watanabe Y, Toki A, Fujii T:
Frantz's tumor: a papillary and cystic tumor of the pancreas in girls.
J Pediatr Surg 23(2):116-21, 1988
2- Tait N, Greenberg ML, Richardson AJ, Osborn RA, Little
JM: Frantz's tumour: papillary and cystic carcinoma of the pancreas. Aust
N Z J Surg 65(4):237-41, 1995
3- Horisawa M, Niinomi N, Sato T, Yokoi S, Oda K, Ichikawa
M, Hayakawa S: Frantz's tumor (solid and cystic tumor of the pancreas)
with liver metastasis: successful treatment and long-term follow-up. J
Pediatr Surg 30(5):724-6, 1995
4- Yang YJ, Chen JS, Chen CJ, Lin PW, Chang KC,Tzeng
CC: Papillary cystic tumor of the pancreas in children. Scand J Gastroenterol
31(12):1223-7, 1996
5- Rivera M, Ortiz VN, Duran N, Trujillo O: Solid and
papillary neoplasm of the pancreas: a case presentation. Bol Asoc Med P
R 90(4-6):91-2, 1998
6- Ooi LL, Ho GH, Chew SP, Low CH, Soo KC: Cystic tumours
of the pancreas: a diagnostic dilemma. Aust N Z J Surg 1998 Dec;68(12):844-6
Bile Duct Injury
Laparoscopic cholecystectomy (LC) has replaced the open procedure as
the treatment of choice in gallbladder disorders. With the lap technique
an increase two- to threefold in the incidence (0.5%) of bile duct injuries
(BDI) has also occurred. Inapropiate anatomy identification and aberrant
situations are the most common cause of BDI. BDI can be classified into
transection, lacerations, leaks or strictures. Disease complexity, increasing
age, male gender and admission to a teaching hospital are associated with
an increase risk of injury. Classic injuries involve clipping & dividing
the CBD for the cystic duct. Other BDI may result from stenting injury,
thermal injury, lose cystic duct clip or an accessory "Luschka" duct. Such
injuries cause two principal clinical manifestations: 1) bile leaks with
pain and secondary bile peritonitis, and 2) biliary obstruction due to
partial or complete hepatic or common duct ligation (jaundice) or late
stricture formation. US, HIDA scan or CT-Scan followed by ERCP or PTC may
help diagnosed and define the exact location and nature of the BDI. Complete
proximal obstruction may need transhepatic biliary catheters in each radical
duct to relieve obstruction. A bile leak may need percutaneous drainage
for sepsis control. Cystic or accessory ducts leaks can be dealt with endoscopic
ampullary stenting or re-laparoscopic ligation if identified early. The
most effective means of reconstructing a major BDI is using roux-en-y hepaticojejunostomy
after inflammation and adhesions of the initial insult have subsided. Intraoperative
cholangiography appears to protect against BDI by preventing misidentification
of anatomy. Conversion to open cholecystectomy is essential in avoiding
these injuries when the anatomy remains uncertain.
References
1- Ferguson CM; Rattner DW; Warshaw AL: Bile duct injury
in laparoscopic cholecystectomy. Surg Laparosc Endosc 2(1):1-7, 1992
2- Davidoff AM; Pappas TN; Murray EA; Hilleren DJ, Johnson
RD; Baker ME; Newman GE; Cotton PB; Meyers WC: Mechanisms of major biliary
injury during laparoscopic cholecystectomy. Ann Surg 215(3):196-202,
1992
3- Lee VS; Chari RS; Cucchiaro G; Meyers WC: Complications
of laparoscopic cholecystectomy. Am J Surg 165(4):527-32, 1993
4- Branum G; Schmitt C; Baillie J; Suhocki P; Baker M,
Davidoff A; Branch S; Chari R; Cucchiaro G; Murray E; et al: Management
of major biliary complications after laparoscopic cholecystectomy. Ann
Surg 217(5):532-40, 1993
5- Bergman JJ; van den Brink GR; Rauws EA; de Wit L,
Obertop H; Huibregtse K; Tytgat GN; Gouma DJ: Treatment of bile duct lesions
after laparoscopic cholecystectomy. Gut 38(1):141-7, 1996
6- Lillermoe KD; Martin SA; Cameron JL; Yeo CJ, Talamini
MA; Kaushal S; Coleman J; Venbrux AC; Savader SJ, Osterman FA; Pitt HA:
Major bile duct injuries during laparoscopic cholecystectomy. Follow-up
after combined surgical and radiologic management. Ann Surg 225(5):459-68,
1997
7- MacFadyen Jr BV; Vecchio R; Ricardo AE; Mathis CR:
Bile duct injury after laparoscopic cholecystectomy. The United States
experience. Surg Endosc 12(4):315-21, 1998
8- Fletcher DR, Hobbs MS, Tan P, Valinsky LJ, Hockey
RL: Complications of cholecystectomy: risks of the laparoscopic approach
and protective effects of operative cholangiography: a population-based
study. Ann Surg 229(4):449-57, 1999
Septate GB
Congenital anomalies of the gallbladder includes agenesis (congenital
absence), duplications, and septation. Finding a Septate gallbladder (GB)
is a very rare event that could herald biliary problems. The septum (which
contains smooth muscle fibers) usually divides the GB into two chambers.
The anomaly in endoderm occurs during the second month of fetal life when
the GB lumen develops. Mostly the malformation runs asymptomatic and it
is found accidentally without clinical relevance unless the pinpoint communication
between the cavities causes stagnation, inflammation or stone formation.
Children present with history of chronic biliary colicky abdominal pain
associated with nausea and vomiting. Symptoms are caused by pressure in
the small chambers of the GB along with delay emptying. Diagnosis is made
using Ultrasound (flat fundus, elongated and dilated GB) couple with biliary
scintigraphy (HIDA Scan). Symptomatic children should be managed with laparoscopic
cholecystectomy.
References
1- Esper E, Kaufman DB, Crary GS, Snover DC, Leonard
AS: Septate Gallbladder with Cholelithiasis: A Cause of Chronic Abdominal
Pain in a 6-Year-Old Child. J Pediatr Surg 27 (2): 1560-1562, 1992
2- Doyle TC: Flattened fundus sign of the septate gallbladder.
Gastrointest Radiol 9(4):345-7, 1984
3- Mrhac L, Zakko S, Ibrahim A: Imaging of septate gallbladder.
Clin Nucl Med 24(3):209-10, 1999
Volume 14 No 04 APRIL 2000
Gallstone Pancreatitis
Gallstone pancreatitis (GP) continue to be the most common complication
of cholelithiasis in children. The most frequently encountered mechanisms
of gallstone pancreatitis appear to be impacted stones, congenital pathology
in the ampullary area, and passage of small stones through the common bile
duct (CBD), with temporary obstruction and inflammation followed by disruption
of pancreatic ductules and/or acinar cell membrane. Should the stone impact
the ampulla jaundice could result. Management of the pancreatitis during
the acute episode is supportive (bowel decompression, bowel rest and total
parenteral nutrition), and the course of the disease for the most part
is benign (low Ranson's criteria). When patients are discharged before
cholecystectomy, the recurrence rate of acute biliary pancreatitis that
requires emergency readmission is unacceptably high. Emergency endoscopic
retrograde cholangiopancreaticogram (ERCP) with papillotomy has been found
to reduce mortality and biliary sepsis in the early acute situation. Considering
the relatively low incidence of CBD stones in GP routine preoperative ERCP
is not indicated. Preop ERCP can be restricted to patients with cholangitis,
persistent hyperbilirubinemia, CBD stones or persistent hyperamylasemia.
Laparoscopic cholecystectomy should be done during the same admission after
symptoms of pancreatitis and hyperamylasemia abates. Intraoperative cholangiography
needs to be done to visualize the anatomy and address common bile duct
stones.
References
1- Frei GJ, Frei VT, Thirlby RC, McClelland RN: Biliary
pancreatitis: clinical presentation and surgical management. Am J Surg
151(1):170-5, 1986
2- Beshlian K, Ryan JA Jr: Pancreatitis in teenagers.
Am J Surg 152(1):133-8, 1986
3- Albu E, Buiumsohn A, Lopez R, Gerst PH: Gallstone
pancreatitis in adolescents. J Pediatr Surg 22(10):960-2, 1987
4- Fan ST, Lai EC, Mok FP, Lo CM, Zheng SS, Wong J: Early
treatment of acute biliary pancreatitis by endoscopic papillotomy. N Engl
J Med 28;328(4):228-32, 1993
5- Lugo-Vicente HL: Trends in management of gallbladder
disorders in children. Pediatr Surg Int 12(5-6):348-52, 1997
6- Bulkin AJ, Tebyani N, Dorazio RA: Gallstone pancreatitis
in the era of laparoscopic cholecystectomy. Am Surg 63(10):900-3, 1997
7- Chang L, Lo SK, Stabile BE, Lewis RJ, de Virgilio
C: Gallstone pancreatitis: a prospective study on the incidence of cholangitis
and clinical predictors of retained common bile duct stones. Am J Gastroenterol
93(4):527-31, 1998
8- Holcomb GW 3rd, Morgan WM 3rd, Neblett WW 3rd: Laparoscopic
cholecystectomy in children: lessons learned from the first 100 patients.
J Pediatr Surg 34(8):1236-40, 1999
Mirizzi's Syndrome
Mirizzi syndrome (MS) is an unusual finding of biliary tree diseases.
Refers to partial mechanical obstruction of the common hepatic duct owing
to compression by a stone impacted in the infundibulum, Hartmann's pouch
or cystic duct, or due to the inflammatory reaction resulting from compression.
The typical diagnostic signs of MS are dilatation of the common hepatic
duct & radicals above the level of a gallstone impacted in the cystic
duct, with normal duct width below the stone. Obstructive jaundice, pain
and cholangitis are the common presentations of this condition. Diagnosis
may require a combination of ultrasonography, computed tomography, and
cholangiography (percutaneous or endoscopic retrograde). MS is classified
as type I (stenosis of the common hepatic duct due to an impacted cystic
duct stone), type II (fistula between cystic and common hepatic duct),
type III (hepatic duct stenosis due to a stone), and type IV (hepatic duct
stenosis due to cholecystitis). Management depends on the type identified
and may consist of open partial cholecystectomy and choledochoplasty with
a gallbladder flap, common bile duct exploration with t-tube placement
or bilio-enteric anastomosis. Laparoscopic technique is fraught with bile
duct injury increasing further the morbidity. The morbidity and mortality
associated with this rare syndrome can be relatively high and a significant
number of patients may develop late biliary strictures.
References
1- Becker CD, Hassler H, Terrier F: Preoperative diagnosis
of the Mirizzi syndrome: limitations of sonography and computed tomography.
AJR Am J Roentgenol 143(3):591-6, 1984
2- Didlake R, Haick AJ: Mirizzi's syndrome. An uncommon
cause of biliary obstruction. Am Surg 56(4):268-9, 1990
3- Ibrarullah M, Saxena R, Sikora SS, Kapoor VK, Saraswat
VA, Kaushik SP: Mirizzi's syndrome: identification and management strategy.
Aust N Z J Surg 63(10):802-6, 1993
4- Toursarkissian B, Holley DT, Kearney PA, McGrath PC,
Zweng TN: Mirizzi's syndrome. South Med J 87(4):471-5, 1994
5- Nagakawa T, Ohta T, Kayahara M, Ueno K, Konishi I,
Sanada H, Miyazaki I: A new classification of Mirizzi syndrome from diagnostic
and therapeutic viewpoints. Hepatogastroenterology 44(13):63-7, 1997
6- Karakoyunlar O, Sivrel E, Koc O, Denecli AG: Mirizzi's
syndrome must be ruled out in the differential diagnosis of any patients
with obstructive jaundice. Hepatogastroenterology 46(28):2178-82, 1999
Prenatal Inguinal Hernia
The diagnosis of an inguinal hernia (IH) has been made prenatally with
the help of sonography in a few cases. Moving, echo-free, cystlike structures
representing peristalsis within trapped loops of bowel in an abnormally
enlarged scrotum are the main ultrasonographic finding. There is no need
to change the route of birth due to the presence of a prenatal IH. The
differential diagnosis consists of other masses protruding from the abdominal
wall such as omphalocele or from the perineal region (sacrococcygeal teratoma,
undescended testis and communicating hydrocele). After delivery the
diagnosis is confirmed during the initial physical exam of the baby by
finding a reducible bulge in the inguinal area. Repair of the inguinal
hernia should be done before discharge from the hospital to avoid feeding
difficulties, incarceration, strangulation or gonadal infarction. Prematures
have a higher incidence of developing complications from the IH than term
infants.
References
1- Ober KJ, Smith CV: Prenatal ultrasound diagnosis of
a fetal inguinal hernia containing small bowel. Obstet Gynecol 78(5 Pt
2):905-6, 1991
2- Meizner I, Levy A, Katz M, Simhon T, Glezerman M:
Prenatal ultrasonographic diagnosis of fetal scrotal inguinal hernia. Am
J Obstet Gynecol 166(3):907-9, 1992
3- Shipp TD, Benacerraf BR: Scrotal inguinal hernia in
a fetus: sonographic diagnosis. AJR Am J Roentgenol 165(6):1494-5, 1995
4- Paladini D, Palmieri S, Morelli PM, Forleo F, Morra
T, Salviati M, Zampella C, D'Angelo A, Martinelli P: Fetal inguinoscrotal
hernia: prenatal ultrasound diagnosis and pathogenetic evaluation. Ultrasound
Obstet Gynecol 7(2):145-6, 1996
Volume 14 No 05 MAY 2000
Dumping Syndrome
Dumping syndrome (DS) refers to a combination of vasomotor symptoms
that occurs while the patient is eating (especially carbohydrates) and
includes: faintness, weakness, dizziness, tachycardia, diaphoresis and
the need to assume a reclining position. The child may develop early satiety,
nausea, bloating and cramps with explosive bowel movement. DS is the physiologic
response (release of various humoral agents from the gut: bradykinins,
serotonins, enteroglucagon along with extracellular fluid shifts) to an
altered movement of large amounts of hyperosmotic gastric chyme into the
proximal small bowel. DS develops after 1- bypass (gastrojejunostomy),
ablation (pyloroplasty) or alteration (antireflux surgery) of the pyloric
sphincter mechanism, 2- removal of part of the stomach, or 3- malplacement
of a feeding gastrostomy tube. Fundoplication is the most common cause
of DS in children. The diagnosis is made by demonstrating an abnormal response
to an orally administered glucose challenge. Radionuclide studies confirm
the presence of rapid gastric emptying. Cows' milk formula and meals made
with cooked starch provoke dumping symptoms, hyperglycemia and hyperinsulinemia.
Diet modification (frequent small meals high in protein, fat emulsions
and uncooked corn starch - lactose free formulas) is the best useful
medical therapy. In other words not overeating, reducing carbohydrates
in food, abstaining from dairy products and eliminating fluids with meals.
Pectin 5-15 g/day divided into six doses can be added to the diet. Late
DS refers to hypoglycemia that result from innapropiate timing of insulin
release.
References
1- Hirsig J, Baals H, Tuchschmid P, Spitz L: Dumping
syndrome following Nissen's fundoplication: a cause for refusal to feed.
J Pediatr Surg 19(2):155-7, 1984
2- Caulfield ME, Wyllie R, Firor HV, Michener W: Dumping
syndrome in children. J Pediatr 110(2):212-5, 1987
3- Khoshoo V, Reifen RM, Gold BD, Sherman PM: Nutritional
manipulation in the management of dumping syndrome. Arch Dis Child 66(12):1447-8,
1991
4- Khoshoo V, Roberts PL, Loe WA, Golladay ES: Nutritional
management of dumping syndrome associated with antireflux surgery. J Pediatr
Surg 29(11):1452-4, 1994
5- Samuk I, Afriat R, Horne T, Bistritzer T, Barr J:
Dumping syndrome following Nissen fundoplication, diagnosis, and treatment.
J Pediatr Gastroenterol Nutr 23(3):235-40, 1996
6- Borovoy J, Furuta L, Nurko S: Benefit of uncooked
cornstarch in the management of children with dumping syndrome fed exclusively
by gastrostomy. Am J Gastroenterol 93(5):814-8, 1998
Postoperative Intussusception
Intussusception can occur a few days after a surgical procedure in children.
The diagnosis is delayed because symptoms mimic common postoperative complaints.
Suspicion should be raised when an apparently favorable postoperative course
is suddenly complicated by intestinal obstruction. Postoperative intussusception
(POI) is generally characterized by intermittent abdominal pain, irritability
and vomiting. Initial symptoms included bilious vomiting or increased nasogastric
drainage (after initial return of gut function). An abdominal mass is not
usually palpable, and few children have bloody stools. Plain abdominal
radiographs revealed dilated loops of bowel and multiple air-fluid levels.
Ultrasound can identify a target lesion characteristic of intussusception.
POI can follow Nissen fundoplication, tumor resection, small bowel procedure,
pull-through procedures, nephrectomy and even gastrocystoplasty. Most are
ileo-ileal or jejuno-ileal and will need surgical reduction. Diagnostic
delay results in ischemic bowel needing resection.
References
1- Hulbert WC Jr, Valvo JR, Caldamone AA, Putnam TC:
Intussusception following resection of Wilms tumor. Urology 21(6):578-80,
1983
2- West KW, Stephens B, Rescorla FJ, Vane DW: Postoperative
intussusception: experience with 36 cases in children. Surgery 104(4):781-7,
1988
3- Holcomb GW 3d, Ross AJ 3d, O'Neill JA Jr: Postoperative
intussusception: increasing frequency or increasing awareness? South Med
J 84(11):1334-9, 1991
4- Jequier S, Argyropoulou M, Bugmann P: Ultrasonography
of jejunal intussusception in children. Can Assoc Radiol J 46(4):285-90,
1995
5- Linke F, Eble F, Berger S: Postoperative intussusception
in childhood. Pediatr Surg Int 14(3):175-7, 1998
6- de Vries S, Sleeboom C, Aronson DC: Postoperative
intussusception in children. Br J Surg 86(1):81-3, 1999
Appendicolith
Appendicitis is usually diagnosed from signs, symptoms, results of simple
laboratory tests a/o simple abdominal films. After simple abdominal films
an appendicolith (coprolith, fecalith, retained barium or foreign body)
is sometimes found in the symptomatic child with right lower quadrant pain
or less commonly in an asymptomatic situation. In the child WITH SYMPTOMS
of low abdominal pain this finding should be followed by appendectomy.
Appendiceal fecaliths and calculi play a role in the pathogenesis of appendicitis
and are associated with perforation and gangrene. In the ASYMPTOMATIC situation
a prophylactic appendectomy is NOT justified when an appendicolith, retained
barium or another foreign body within the lumen of the appendix is identified.
A normal appendix will expel the appendicolith or barium in a variable
period. The parents should be informed that appendicitis may develop and
that the child should seek a physician if abdominal symptoms develop. A
note should appear in the record explaining this conversation.
References
1- Maenza RL, Smith L, Wolfson AB: The myth of the fecalith.
Am J Emerg Med 14(4):394-7, 1996
2- Nitecki S, Karmeli R, Sarr MG: Appendiceal calculi
and fecaliths as indications for appendectomy. Surg Gynecol Obstet 171(3):185-8,
1990
3- Maglinte DD, Bush ML, Aruta EV, Bullington GE: Retained
barium n the appendix: diagnostic and clinical significance. AJR Am J Roentgenol
137(3):529-33, 1981
4- Brender JD, Marcuse EK, Koepsell TD, Hatch EI: Childhood
appendicitis: factors associated with perforation. Pediatrics 76(2):301-6,
1985
5- Meier DE: Appendicitis in a barium-filled appendix.
South Med J 74(4):493-4, 1981
6- Santini I, Pacheco R, Lugo-Vicente HL: Apéndice
Peforada en Niños:Evaluacion de un Diagnóstico Tardío.
PR Health Science J 14(4):263-267, 1995
Volume 14 No 06 JUNE 2000
Müllerian Duct Syndrome
Müllerian Duct Syndrome (MDS) refers to a genetic disorder of male
pseudohermaphroditism (46 XY karyotype) characterized by normal masculinization
of the external genitalia and the presence of uterus and fallopian tubes.
Most cases are discovered during surgery for undescended testis, inguinal
hernia or transverse testicular ectopia. MDS is caused by a deficient activity
(most cases) or receptor insensitivity of antimullerian hormone (also known
as Müllerian inhibitor factor). This hormone is produced by testicular
Sertoli cells and is responsible of producing fetal regression of Müllerian
structures (uterus & fallopian tubes) in genetic males. Most cases
are transmitted as autosomal recessive restricted to males (sex-linked).
Anatomic variants include fallopian tube or uterus within the inguinal
canal, testis and tubes in a hernia sac or bilateral cryptorchidism with
the testes embedded in the broad ligaments. The vas deferens is intimately
adhered to the uterus lateral wall. Initial procedure consists of hernia
repair, replacement of structure within pelvis and karyotype. After diagnosis
follow-up management has been controversial. A few suggest partial removal
of the uterus (leaving vas deferens intact on a thin pedicle of myometrium)
and fallopian tubes with testicular fixation. Most content that surgical
excision of persistent MDS structure may result in ischemic or traumatic
damage to the vasa deferentia and testes and optimal management is orchiopexy
leaving the uterus and fallopian tubes in situ. The testis in MDS are at
risk of malignant degeneration.
References
1- Loeff DS, Imbeaud S, Reyes HM, Meller JL, Rosenthal
IM: Surgical and Genetic Aspects of Persistent Müllerian Duct Syndrome.
J Pediatr Surg 29(1): 61-65, 1994
2- Belville C, Josso N, Picard JY: Persistence of müllerian
derivatives in males. Am J Med Genet 89(4):218-223, 1999
3- Rizk DE, Ezimokhai M, Hussein AS, Gerami S, Deb P:
Persistent müllerian duct syndrome. Arch Gynecol Obstet 261(2):105-7,
1998
4- Karnak I, Tanyel FC, Akcoren Z, Hicsonmez A: Transverse
testicular ectopia with persistent müllerian duct syndrome. J Pediatr
Surg. 32(9):1362-4, 1997
Bleeding Nipple
Breast enlargement is commonly seen in newborns babies, a condition
associated with clear or milky nipple discharge. Maternal hormones are
considered the culprit. On rare occasions the mother of an infant will
bring to you the attention that the child is having intermittent episodes
of bleeding through the nipple. In infants it is a benign, self-limited
condition that should be managed conservatively (it could take six months
to go away). The main reason of bloody nipple discharge is mammary ductal
ectasia, which extends down to the collecting tubules. Mammary duct ectasia
was first reported in 1983, characterized by dilatation of the subareolar
duct system, and by inflammatory reaction and fibrosis. The infant's own
endocrine system is responsible for breast enlargement and mammary duct
ectasia, though infection has also been postulated as etiologic factor.
Surgical procedures should be avoided, because injury to the breast bud
may cause permanent damage. Stimulation or massages to the breast should
also be avoided. When associated with hypertrophied mammary glands, prepubertal
gynecomastia should be sought.
References
1- Gershin T, Mogilner JG: [Bloody nipple discharge in
an infant]. Harefuah 122(8):505-6, 1992
2- Iester A, Debbia C, Franzone G, Scribanis R, Puleo
MG, Buzzanca C, Di Bella E, Alpigiani MG: [Mammary duct ectasia in children].
Minerva Pediatr 47(10):417-21, 1995
3- Olcay I, Gokoz A: Infantile gynecomastia with bloody
nipple discharge. J Pediatr Surg 27(1):103-4, 1992
4- Bober E, Ozer E, Akgur F, Buyukgebiz A: Bilateral
breast masses and bloody nipple discharge in a two year-old boy. J Pediatr
Endocrinol Metab 9(3):419-21, 1996
5- Stringel G, Perelman A, Jimenez C: Infantile mammary
duct ectasia: a cause of bloody nipple discharge. J Pediatr Surg 21(8):671-4,
1986
6- Berkowitz CD, Inkelish SH: Bloody nipple discharge
in infancy. J Pediatr 103:755-758, 1984
Pilonidal Sinus
Pilonidal sinus disease (PSD) is the result of a chronic inflammatory
reaction of the subcutaneous tissue of the low back from midline skin orifices
that ingest hair shafts in hirsute buttocks. PSD arises from hair follicles
in the skin, appears after puberty and affects mostly males adolescents.
Male sex, adolescence and a familial disposition are associated with the
development of PSD. Minor local trauma (‘Jeep Disease' in military personnel)
and overweight are the most important factors for development of symptomatic
PSD. Primary closure after excision of PSD has frequently been complicated
by wound breakdowns. Sepsis and hematoma formation are the main causes
of wound breakdowns. PSD is best treated in the acute situation with follicle
removal and lateral drainage. The method is suited to outpatient management
and gives minimal disability and good long-term control. In the chronic
situation conservative therapy (meticulous hair control by natal
cleft shaving, perineal hygiene, and limited lateral incision/drainage
for abscess) effectively controls PSD in the nonoperative outpatient setting
while promoting near-normal work status preferred over excisional operations.
References
1- Bascom J: Pilonidal disease: long-term results of
follicle removal. Dis Colon Rectum 26(12):800-7, 1983
2- Golladay ES, Wagner CW: Pediatric pilonidal disease:
a method of management. South Med J 83(8):922-4, 1990
3- Armstrong JH, Barcia PJ: Pilonidal sinus disease.
The conservative approach. Arch Surg 129(9):914-7, 1994
4- Sondenaa K, Andersen E, Nesvik I, Soreide JA: Patient
characteristics and symptoms in chronic pilonidal sinus disease. Int J
Colorectal Dis 10(1):39-42, 1995