PEDIATRIC SURGERY UPDATE ©
VOLUME 20, 2003
Volume 20 No 01 JANUARY 2003
Desmoplastic Small Round Cell Tumor
Within the non-rhabdomyosarcoma soft tissue tumors in children, the
Desmoplastic Small Round Cell Tumor (DSRCT) is an extremely locally aggressive
and rare tumor with predilection for children and adolescent patients and
predominant or exclusive intra-abdominal location. DSRCT is characterized
by a generally diffuse pattern of growth of small round cells with hyperchromatic
nuclei, scanty cytoplasm, patchy epithelial differentiation, immunohistochemical
co-expression of keratin and desmin intermediate filaments and a focal
but pronounced desmoplastic stromal component. This neoplasm exhibits a
predominantly intra-abdominal serosal pattern with frequent
pelvic extension, less frequent retroperitoneal involvement and
very rarely pulmonary and mediastinal spread. The child usually present
with a palpable, painful mass which may cause abdominal distension, constipation,
bowel or ureteral obstruction. On imaging bulky peritoneal soft-tissue
masses without an apparent organ-based primary site are characteristic
of intra-abdominal DSRCT. DSRCT can be mistaken for a rhabdomyosarcoma
as it contains cells with rhabdoid features. Cytogenetic studies showed
a t(11;22) translocation that differs from the Ewing's tumor translocation
and seems to be specific to this entity. Management consists of dose intensive
multimodal chemotherapy, aggressive surgery to resect visible disease,
local radiotherapy, and myeloablative chemotherapy with stem-cell rescue
in selected cases. Unfortunately the prognosis is very poor and most children
succumb to widespread metastasis disease.
References:
1- Yeoh G, Russell P, Wills EJ, Fleming S: Intra-abdominal
desmoplastic small round cell tumor. Pathology 25(2):197-202, 1993
2- Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley
KL, Ghavimi F, Merchant TE, Boulad F, Cheung NK, Bonilla MA, Crouch G,
Kelleher JF Jr, Steinherz PG, Gerald WL: Desmoplastic small round-cell
tumor: prolonged progression-free survival with aggressive multimodality
therapy. J Clin Oncol 14(5):1526-31, 1996
3- Leuschner I, Radig K, Harms D: Desmoplastic small
round cell tumor. Semin Diagn Pathol 13(3):204-12, 1996
4- Pickhardt PJ, Fisher AJ, Balfe DM, Dehner LP, Huettner
PC: Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic
correlation. Radiology 210(3):633-8, 1999
5- Quaglia MP, Brennan MF: The clinical approach to desmoplastic
small round cell tumor. Surg Oncol 9(2):77-81, 2000
Juvenile Fibroadenoma
Juvenile or giant fibroadenoma of the breast is a benign lesion that
can obtain a large formidable proportional size during breast development
in female adolescent patients. Most cases in children are seen between
the ages of 10 and 15 years. The tumor is solitary in most affected children
with a diameter of 4-6 centimeters. Multiple and bilateral involvement
has been reported in a few cases. Differential diagnosis includes cystosarcoma
phylloides, benign virginal hypertrophy (juvenile gigantomastia) or rhabdomyosarcoma.
FNA or Tru-cut needle biopsy can establish a precise histological diagnosis.
Growth is so fast that it can cause non-tender cellulitis of the skin by
way of stretching. Microscopically the tumor is characterized by a rich
cellular stroma and a prominent glandular epithelium. Juvenile adenofibromas
regardless of size, should be excised so as to preserve as much breast
tissue as possible. Management options include local excision with reconstruction,
reduction mammoplasty, or simple mastectomy with reconstruction.
References:
1- Musio F, Mozingo D, Otchy DP: Multiple, giant fibroadenoma.
Am Surg 57(7):438-41, 1991
2- Morimoto T, Komaki K, Mori T, Sasa M, Miki H, Inoue
H, Monden Y, Nakanishi H: Juvenile gigantomastia: report of a case. Surg
Today 23(3):260-4, 1993
3- Remadi S, Ismail A, Karpuz V, Finci V, Zacharie S,
Vassilakos P: Cellular (juvenile) fibroadenoma of the breast. A clinico-pathologic
and immunohistochemical study of 7 cases. Ann Pathol 14(6):392-7,
1994
4- Boothroyd A, Carty H: Breast masses in childhood and
adolescence. A presentation of 17 cases and a review of the literature.
Pediatr Radiol 24(2):81-4, 1994
5- Herrera L, Lugo-Vicente HL: Primary Embryonal Rhabdomyosarcoma
of the Breast in an Adolescent Female: A Case Report. J Pediatr Surg 33(10):1582-1584,
1998
6- Pacinda SJ, Ramzy I: Fine-needle aspiration of breast
masses. A review of its role in diagnosis and management in adolescent
patients. J Adolesc Health 23(1):3-6, 1998
Laparoscopic Bowel Resection
The enthusiasm brought by laparoscopic surgery results has expanded
its capabilities to other intra-abdominal surgical procedures such as isolated
or limited bowel resection in children. So far reported indications have
included: resection of a Meckel's diverticulum, post-enterocolitis small
bowel or colonic stricture, localized mesenteric cyst, inflammatory bowel
disease (IBD), intussusception, intestinal lymphangioma and duplication.
Technically the approach can include mobilization with extracorporeal resection
and anastomosis (video-assisted), resection with intracorporeal stapled
anastomosis or resection with hand-sewn end-to-end anastomosis depending
on the lesion, mobility, size of the bowel and ability of the surgeon.
Largest experience with lap bowel resection and anastomosis has been for
inflammatory bowel disease, specially Crohn's disease children. Laparoscopically
assisted colectomies can be performed safely in treating IBD. Laparoscopic-assisted
ileocolic resection has also been found a safe alternative to open surgery
in adolescent patients with Crohn disease. Lap bowel resection has offered
a faster recovery of pulmonary function, fewer complications, and shorter
length of stay compared with conventional surgery for selected patients
undergoing ileocolic resection for Crohn's disease.
References:
1- Wexner SD, Johansen OB: Laparoscopic bowel resection:
advantages and limitations. Ann Med 24(2):105-10, 1992
2- Meijerink WJ, Eijsbouts QA, Cuesta MA, van Hogezand
RA, Ringers J, Meuwissen SG, Griffioen G, Bemelman WA: Laparoscopically
assisted bowel surgery for inflammatory bowel disease. The
combined experiences of two academic centers. Surg Endosc
13(9):882-6, 1999
3- Hamel CT, Hildebrandt U, Weiss EG, Feifelz G, Wexner
SD: Laparoscopic surgery for inflammatory bowel disease. Surg Endosc
15(7):642-5, 2001
4- Diamond IR, Langer JC: Laparoscopic-assisted versus
open ileocolic resection for adolescent Crohn disease. J Pediatr Gastroenterol
Nutr 33(5):543-7, 2001
5- Milsom JW, Hammerhofer KA, Bohm B, Marcello P, Elson
P, Fazio VW: Prospective, randomized trial comparing laparoscopic vs. conventional
surgery for refractory ileocolic Crohn's disease. Dis Colon Rectum
44(1):1-8, 2001
6- Rothenberg SS: Laparoscopic Segmental Intestinal Resection.
Semin Pediatr Surg 11(4): 211-216, 2002
Volume 20 No 02 FEBRUARY 2003
Ondine's Curse
Congenital Central hypoventilation syndrome (CCHS), also known as Ondine
curse, is a rare disorder of ventilatory control characterized by a lack
of response to normal respiratory stimulants, especially hypercapnia. The
child develops prolonged apnea, cyanosis and hypoventilation in the absence
of cardiac, pulmonary or neuromuscular disease. Low respiratory rates during
sleep are characteristic of Ondine's curse. Initial mechanical ventilation
dependency is followed by ability to sustain respiration during awake periods.
Ondine's curse is believed to be caused by aberrant development of neural
crest tissue (neurocristopathy), and as such is associate with Hirschsprung's
disease mostly of the long segment variety. It can also be associated with
multifocal congenital neuroblastoma, ganglioneuroma, neuroma and hypothalamic
dysfunction. Abnormalities of the eye and autonomic nervous system are
also common. CCHS can potentially be managed with long-term assisted mechanical
ventilation, or electrodes surgically implanted on the phrenic nerve to
pace the diaphragm. Electrodes can be placed thoracoscopically. Diaphragmatic
pacing can result in successful weaning from mechanical ventilation, appears
to be effective in reducing pulmonary vascular resistance and pulmonary
hypertension, and improve the quality of life in children with CCHS. Diaphragmatic
fatigue and loss of phrenic nerve conductivity seem to be causes of failure
of electrical pacing. The prognosis for patients with CCHS is poor with
death resulting from pulmonary infections or cardiac failure due to pulmonary
hypertension from hypoxemia.
References:
1- Oren J, Kelly DH, Shannon DC: Long-term follow-up
of children with congenital central hypoventilation syndrome. Pediatrics
80(3):375-80, 1987
2- El-Halaby E, Coran AG: Hirschsprung's Disease Associated
with Ondine's Curse: Report of Three Cases and Review of the Literature.
J Pediatr Surg 29(4): 530-535, 1994
3- Flageole H, Adolph VR, Davis GM, Laberge JM, Nguyen
LT, Guttman FM: Diaphragmatic pacing in children with congenital central
alveolar hypoventilation syndrome. Surgery 118(1):25-8, 1995
4- Stovroff M, Dykes F, Teague WG: The complete spectrum
of neurocristopathy in an infant with congenital hypoventilation, Hirschsprung's
disease, and neuroblastoma. J Pediatr Surg 30(8):1218-21, 1995
5- Shaul DB, Danielson PD, McComb JG, Keens TG: Thoracoscopic
placement of phrenic nerve electrodes for diaphragmatic pacing in children.
J Pediatr Surg 37(7):974-8, 2002
Splenic Autotransplantation
Splenic autotransplantation (SAT) as a means of preserving some immunologic
function after emergent (trauma), or elective (Schistosomiasis and Gaucher's
disease) splenectomy in children has been reported in several studies.
Thinly slice splenic segments (around 20 to 30 gm tissue) are deposited
into a greater omentum pouch. Studies have shown that these implants grow
with time, taking around five years. More than 30% of normal splenic tissue
is needed for adequate filtration and immunologic clearance. After implantation
Technetium scanning is use to show viability of the graft. Fc-receptor
scintigram with IgG coated and Tc-labeled RBC are use to verify mononuclear
phagocyte function. Filtration capacity is follow-up with disappearance
of Howell-Jolly bodies and platelet numbers. Phagocytosis and Immunologic
capacity is done measuring tuftsin and IgM respectively, and NBT test for
evaluating phagocytic function of granulocytes. Complications associated
with SAT are adhesive small bowel obstruction, torsion and aseptic necrosis
of the transplant. A better antibiotic response to pneumococcal vaccine
is found in patients after splenectomy and autotransplantation. SAT is
simple and appears to preserve splenic function.
References:
1- Patel J, Williams JS, Shmigel B, Hinshaw JR: Preservation
of splenic function by autotransplantation of traumatized spleen in man.
Surgery 90(4):683-8, 1981
2- Tzoracoleftherakis E, Alivizatos V, Kalfarentzos
F, Androulakis J: Complications of splenic tissue reimplantation. Ann R
Coll Surg Engl 73(2):83-6, 1991
3- Miyano T, Yamataka A, Ohshiro K, Yamashiro Y: Heterotopic
splenic autotransplantation for splenomegaly secondary to Gaucher's disease--a
case of siblings. J Pediatr Surg 29(12):1572-4, 1994
4- Yamataka A, Fujiwara T, Tsuchioka T, Kurosu Y, Sunagawa
M: Heterotopic splenic autotransplantation in a neonate with splenic rupture,
leading to normal splenic function. J Pediatr Surg 31(2):239-40,
1996
5- Weber T, Hanisch E, Baum RP, Seufert RM: Late
results of heterotopic autotransplantation of splenic tissue into the greater
omentum. World J Surg 22(8):883-9, 1998
6- Leemans R, Manson W, Snijder JA, Smit JW, Klasen HJ,
The TH, Timens W: Immune response capacity after human splenic autotransplantation:
restoration of response to individual pneumococcal vaccine subtypes. Ann
Surg 229(2):279-85, 1999
Laparoscopic Varicocelectomy
Varicoceles develop during early adolescence. Varicocelectomy represents
the treatment of choice for scrotal varicocele associated with scrotal
pain or discomfort, testicular atrophy or infertility (low sperm count).
Prepubertal varicocelectomy is controversial. Traditionally, the surgical
procedure entailed an inguinal or high retroperitoneal open approach to
ligate the internal spermatic veins associated significant postop morbidity
(recurrence and postop hydrocele formation) and prolonged return to normal
activity. During the past ten years the technique has take advantage of
the laparoscopic approach including minimal surgical trauma, lower morbidity,
cost and time sparing, faster recovery, better microscopic dissection with
preservation of the spermatic artery along amenable bilateral ligation
without a second incision. The Palomo mass high retroperitoneal ligation
of the internal spermatic vessels results in a significant decrease in
failure rate as compared with artery-sparing procedures. The Palomo approach
is also safe after previous inguinal hernia repair. The procedure entails
mass clipping and division of the spermatic veins. Collateral veins should
be cauterized. Scrotal subdermal injection of methylene blue help contrast
delineates and preserve lymphatics vessels to reduce the incidence of postop
hydrocele formation.
References:
1- Hagood PG, Mehan DJ, Worischeck JH, Andrus CH, Parra
RO: Laparoscopic varicocelectomy: preliminary report of a new technique.
J Urol 147(1):73-6, 1992
2- Belloli G, Musi L, D'Agostino S: Laparoscopic surgery
for adolescent varicocele: preliminary report on 80 patients. J Pediatr
Surg 31(11):1488-90, 1996
3- Bebars GA, Zaki A, Dawood AR, El-Gohary MA:
Laparoscopic versus open high ligation of the testicular veins for the
treatment of varicocele. JSLS 4(3):209-13, 2000
4- Cohen RC: Laparoscopic varicocelectomy with preservation
of the testicular artery in adolescents. J Pediatr Surg 36(2):394-6,
2001
5- Esposito C, Monguzzi G, Gonzalez-Sabin MA, Rubino
R, Montinaro L, Papparella A, Esposito G, Settimi A, Mastroianni L, Zamparelli
M, Sacco R, Amici G, Damiano R, Innaro N: Results and complications of
laparoscopic surgery for pediatric varicocele. J Pediatr Surg 36(5):767-9,
2001
6- Richter F, Stock JA, LaSalle M, Sadeghi-Nejad
H, Hanna MK: Management of prepubertal varicoceles-results of a questionnaire
study among pediatric urologists and urologists with infertility
training. Urology 58(1):98-102, 2001
7- Barqawi A, Furness P 3rd, Koyle M: Laparoscopic Palomo
varicocelectomy in the adolescent is safe after previous ipsilateral inguinal
surgery. BJU Int 89(3):269-72, 2002
8- Podkamenev VV, Stalmakhovich VN, Urkov PS, Solovjev
AA, Iljin VP: Laparoscopic surgery for pediatric varicoceles: Randomized
controlled trial. J Pediatr Surg 37(5):727-9, 2002
Volume 20 No 03 MARCH 2003
Ostomy Prolapse
Though colostomies ileostomies or jejunostomies play a major role in
the management of gastrointestinal disorder in children they can be associated
with complications. These include skin excoriation, retraction, prolapse
and stricture. Most stomal creation is required during the neonatal period.
Stomal prolapse is the most common complications encounter in clinical
practice (20%) since very dilated bowel must be brought through a thin
abdominal wall. Colostomies sited in the transverse colon have the highest
incidence of prolapse. Whenever possible the stoma should be constructed
away from the surgical wound. End enterostomies rarely prolapse. Prolapses
are more common with loop enterostomies and usually involves the distal
non-functioning limb. Mild prolapse is usually inconsequential and requires
no revision. All prolapses (proximal or distal) should be manage as an
emergency trying initially to reduce it promptly before swelling, mechanical
obstruction or ischemia ensues. For reduction to be effective consciously
sedate the child, lubricate the bowel with jelly to avoid mucosal injury
and start from distal to proximal squeezing gently the intestinal wall.
If the prolapse becomes severe, recurrent, or occurs in the proximal limb
causing mechanical obstruction then surgical revision becomes necessary.
Some temporary measures suggested by other authors include purse string
suture based on the Thiersch principle or anchoring the bowel loop to the
skin away from the stoma. Whenever possible early closure of the stoma
should be done.
References:
1- Gauderer MW, Izant RJ Jr.: A technique for temporary
control of colostomy prolapse in children. J Pediatr Surg 20(6):653-5,
1985
2- Golladay ES, Bernay F, Wagner CW: Prevention of prolapse
in pediatric enterostomas with purse string technique. J Pediatr Surg
25(9):990-1, 1990
3- Millar AJ, Lakhoo K, Rode H, Ferreira MW, Brown RA,
Cywes S: Bowel stomas in infants and children. A 5-year audit of 203 patients.
S Afr J Surg 31(3):110-3, 1993
4- Cheung MT: Complications of an abdominal stoma: an
analysis of 322 stomas. Aust N Z J Surg 65(11):808-11, 1995
5- Nour S, Beck J, Stringer MD: Colostomy complications
in infants and children. Ann R Coll Surg Engl 78(6):526-30, 1996
6- Steinau G, Ruhl KM, Hornchen H, Schumpelick V: Enterostomy
complications in infancy and childhood. Langenbecks Arch Surg 386(5):346-9,
2001
7- Duchesne JC, Wang YZ, Weintraub SL, Boyle M, Hunt
JP: Stoma complications: a multivariate analysis. Am Surg 68(11):961-6,
2002
Pancreatitis in Choledochal Cysts
Choledochal cysts (CC) can produce abdominal pain, obstructive jaundice,
or pancreatitis. The recurrent abdominal pain associated to hyperamylasemia
has been called "pseudopancreatitis" since true inflammation of the pancreas
is absent. Acute edematous or necrotizing pancreatitis associated with
CC is very rare. It is believed that when the bile duct pressure increases
due to obstructive cholangitis pancreatic enzymes in bile may regurgitate
into the blood stream and produce hyperamylasemia. Cholangiovenous reflux
of amylase might cause hyperamylasemia. Other clinical studies have found
that amylase in the biliary tract have access to the blood stream probably
through a sinusoidal pathway by cholangiovenous reflux, and a lymphatic
pathway, via the Disse's space and denuded cyst wall with bile duct pressure
increases. Common denominator is an increase in ductal pressure of a partially
closed common channel system. Infants with CC do not show hyperamylasemia
due to physiologically low level of pancreatic amylase. The abdominal complaint
and hyperamylasemia can easily subside in a short period of time with conservative
treatment resulting in no demonstrable pancreatic dysfunction. Once this
occurs appropriate surgical management of the choledochal cyst which includes
cyst excision and Roux-en-Y hepatico-jejunostomy reconstruction should
be done.
References:
1- Todani T, Urushihara N, Watanabe Y, Toki A, Uemura
S, Sato Y, Morotomi Y: Pseudopancreatitis in choledochal cyst in children:
intraoperative study of amylase levels in the serum. J Pediatr Surg
25(3):303-6, 1990
2- Davenport M, Stringer MD, Howard ER: Biliary amylase
and congenital choledochal dilatation. J Pediatr Surg 30(3):474-7,
1995
3- Urushihara N, Todani T, Watanabe Y, Uemura S, Morotomi
Y, Wang ZQ: Does hyperamylasemia in choledochal cyst indicate true pancreatitis?
An experimental study. Eur J Pediatr Surg 5(3):139-42, 1995
4- Okada A, Higaki J, Nakamura T, Fukui Y, Kamata S:
Pancreatitis associated with choledochal cyst and other anomalies in childhood.
Br J Surg 82(6):829-32, 1995
5- Komuro H, Makino SI, Yasuda Y, Ishibashi T, Tahara
K, Nagai H: Pancreatic complications in choledochal cyst and their surgical
outcomes. World J Surg 25(12):1519-23, 2001
Parotitis
Acute parotitis is a self-limiting disease most commonly associated
with mumps (epidemic parotitis) in children. Other times the parotitis
is associated with bacterial infection progressing to frank suppuration.
Recurrent parotitis, also known as juvenile recurrent parotitis, is characterized
by a cyclic swelling of the parotid glands associated with discomfort and/or
pain in the absence of external inflammatory changes during a period of
several years. The condition mainly affects children between the ages of
three and six, males being more commonly affected. The symptoms peak in
the first year of school and usually begin to subside after puberty. Retrograde
infection induced by the mumps virus and upper respiratory infection play
a major role in the etiology of recurrent parotitis. Sialography demonstrates
sialectasia. Children with recurrent parotid swelling needs to be screened
for underlying systemic immune disorders such as Sjogren's syndrome. With
time the recurrent episodes reduce salivary flow, while increasing the
chloride, sodium, copper, albumin, IgA and lactoferrin concentration. Etiology
of juvenile recurrent parotitis is a combination of congenital malformation
of portions of the salivary ducts and a set-in infection. Treatment is
conservative.
References:
1- Park JW: Recurrent parotitis in childhood. Clin Pediatr
(Phial) 31(4):254-5, 1992
2- Mandel L, Kaynar A: Recurrent parotitis in children.
N Y State Dent J 61(2):22-5, 1995
3- Giglio MS, Landaeta M, Pinto ME: Microbiology of recurrent
parotitis. Pediatr Infect Dis J 16(4):386-90, 1997
4- Chitre VV, Premchandra DJ: Recurrent parotitis. Arch
Dis Child 77(4):359-63, 1997
Volume 20 No 04 APRIL 2003
Partial Splenectomy
Partial splenectomy (PS) is a safe and effective alternative for several
hemolytic disorders and hemoglobinopathies in children including hereditary
spherocytosis, sickle cell disease and b-thalassemia. It is the operation
of choice for all benign splenic conditions. The main objective of partial
splenectomy is preserve splenic contribution to host defense and reduce
the incidence of post splenectomy sepsis (4% risk; mortality rate 1.5%),
while reducing symptoms of hemolysis and sequestration from the systemic
disorder. The risk of septicemia is even higher in very young children
(< four years old). Technically, the goal of the procedure is to remove
80% of splenic tissue by preserving either the upper or lower pole. The
preserved remnant has not proved to reduce the possibility of post splenectomy
sepsis due to the prohibited large clinical trial needed, but has been
found to preserve partial phagocytic function and normal immunoglobulin
level, reduce Howell-Jolly bodies with confirmed visualization on scintigraphy.
In hereditary spherocytosis partial splenectomy has achieved two aims:
decrease red cell destruction and preserve the phagocytic and immunologic
functions of the spleen. Because of chronic hemolysis the size of the spleen
remnant has a tendency to increase with time, specially rapid the younger
the child. Follow-up is closely needed in these children to determine if
they need conversion to total splenectomy. For sickle cell anemia partial
splenectomy has greatly reduced the acute sequestration crisis, need for
hospitalization and subsequent transfusions but does not always preserve
splenic function. This is probably cause by progressive auto-infarction
of the splenic remnant. Since sickle cell patients autoinfarct between
36 and 60 months of life the procedure would benefit more children with
less than 48 months of age.
References:
1- Nouri A, de Montalembert M, Revillon Y, Girot R: Partial
splenectomy in sickle cell syndromes. Arch Dis Child 66(9):1070-2,
1991
2- Tchernia G, Gauthier F, Mielot F, Dommergues JP, Yvart
J, Chasis JA, Mohandas N: Initial assessment of the beneficial effect of
partial splenectomy in hereditary spherocytosis. Blood 81(8):2014-20,
1993
3- Svarch E, Vilorio P, Nordet I, Chesney A, Batista
JF, Torres L, Gonzalez A, de la Torre E: Partial splenectomy in children
with sickle cell disease and repeated episodes of splenic sequestration.
Hemoglobin 20(4):393-400, 1996
4- Kimber C, Spitz L, Drake D, Kiely E, Westaby S, Cozzi
F, Pierro A: Elective partial splenectomy in childhood. J Pediatr Surg
33(6):826-9, 1998
5- Idowu O, Hayes-Jordan A: Partial splenectomy in children
under 4 years of age with hemoglobinopathy. J Pediatr Surg 33(8):1251-3,
1998
6- al-Salem AH, al-Dabbous I, Bhamidibati P: The role
of partial splenectomy in children with thalassemia. Eur J Pediatr Surg
8(6):334-8, 1998
7- de Buys Roessingh AS, de Lagausie P, Rohrlich P, Berrebi
D, Aigrain Y: Follow-up of partial splenectomy in children with hereditary
spherocytosis. J Pediatr Surg 37(10):1459-63, 2002
8- Rice HE, Oldham KT, Hillery CA, Skinner MA, O'Hara
SM,
Ware RE: Clinical and hematologic benefits of partial splenectomy for congenital
hemolytic anemias in children. Ann Surg 237(2):281-8, 2003
Congenital Rectal Stenosis
Congenital rectal stenosis (CRS) is a rare anorectal malformation characterized
by a tubular defect in which an stenotic distal rectum partially communicates
with the distal anal canal. The stenosis is located at the natural limit
of rectum and anal canal (immediately above the pectinate line), runs for
one to two centimeters in distance while the child is born with a normal-looking
external anus. The defect has all the necessary elements responsible for
bowel control including excellent muscle component, an anal canal located
within the limits of the external sphincters with all the nerve ending
that afford normal sensation and a normal sacrum. CRS is usually detected
in the newborn during the initial physical examination. Rectal stimulation
can cause propulsive watery output. Local ischemia occurring late during
fetal life may be the mechanism responsible for the creation of rectal
stenosis of the middle and/or upper rectum. CRS is the hallmark finding
in the Currarino's triad (sacral bony abnormality, presacral mass and rectal
stenosis). Management of CRS consists of serial daily dilatations increasing
the size of the dilator progressively or continuous balloon distension.
Failure of conservative therapy (dilatation) should alert the physician
to the presence of an associated pathologic condition in the presacral
space. Chronic constipation is a common post-dilatation feature in these
patients.
References:
1- Malangoni MA, Grosfeld JL, Ballantine TV, Kleiman
M: Congenital rectal stenosis: a sign of a presacral pathologic condition.
Pediatrics 62(4):584-7, 1978
2- Stone HH, Wilkinson AW: Experimental production of
rectal stenosis and atresia in the rabbit. J Pediatr Surg 18(1):89-90,
1983
3- O'Riordain DS, O'Connell PR, Kirwan WO: Hereditary
sacral agenesis with presacral mass and anorectal stenosis: the Currarino
triad. Br J Surg 78(5):536-8, 1991
4- Zia-w-Miraj Ahmad M, Brereton RJ, Huskisson L: Rectal
atresia and stenosis. J Pediatr Surg 30(11):1546-50, 1995
Omental Infarction
Omental infarction is a very rare painful condition that can mimic appendicitis,
pyelitis or cholecystitis in children. This condition is different from
omental torsion which is usually associated with intraabdominal pathology
such as omental cysts, hernias, tumors or adhesions. Etiology of omental
infarction is unknown. Torsion occurs with omental long axis rotation resulting
in venous obstruction followed by arterial obstruction, infarction
and gangrene. The omentum usually rotates around the distal right gastro-epiploic
artery causing right lower abdominal pain. Obesity is a well-known predisposing
factor. All patients present with acute onset of right lower quadrant pain.
Clinically, the child develops local tenderness with peritoneal signs but
without gastrointestinal symptoms. CT scan (defined area of fat interspersed
with hyper attenuating streaks) is diagnostic. Laparoscopy will confirm
the diagnosis. Surgical resection of the infarcted omentum results in immediate
resolution of pain with minimal morbidity. Others believe that with preoperative
diagnosis resection should depend on symptoms.
References:
1- Puylaert JB: Right-sided segmental infarction of the
omentum: clinical, US, and CT findings. Radiology 185(1):169-72,
1992
2- Helmrath MA, Dorfman SR, Minifee PK, Bloss RS, Brandt
ML, DeBakey ME: Right lower quadrant pain in children caused by omental
infarction. Am J Surg 182(6):729-32, 2001
3- Cervellione RM, Camoglio FS, Bianchi S, Balducci T,
Dipaola G, Giacomello L, Chironi C, Erculiani E, Ottolenghi A: Secondary
omental torsion in children: report of two cases and review of the literature.
Pediatr Surg Int 18(2-3):184-6, 2002
4- Grattan-Smith JD, Blews DE, Brand T: Omental infarction
in pediatric patients: sonographic and CT findings. AJR Am J Roentgenol
178(6):1537-9, 2002
5- Varjavandi V, Lessin M, Kooros K, Fusunyan R, McCauley
R, Gilchrist B: Omental Infarction: Risk Factors in Children. J Pediatr
Surg 38(2): 233-235, 2003
Volume 20 N0 05 MAY 2003
Appendicitis
Appendicitis is still the most common condition requiring emergent abdominal
surgery in childhood caused by obstruction of the appendiceal lumen, most
commonly a fecalith. The obstruction distends the lumen leading to arterial
occlusion and infarction. Initially visceral periumbilical pain occurs
carried by afferent sympathetic fibers to T10 dermatome. With progression
of inflammation the pain shifts to the right lower quadrant. Anorexia,
nausea and vomiting follow. Point tenderness in the right lower quadrant
(or the persistence of right lower quadrant pain) is the most reliable
physical finding. Fever is usually present. Laboratory findings are an
elevated white blood cell count in most instances. Very high WBC's > 18,000
may indicate perforation. Radiographic findings may include ileus, appendicolith
(pathognomonic finding), splinting and abdominal wall edema. Ultrasound
and CT-scan have improved the diagnostic accuracy in children with suspected
appendicitis. Appendicitis is managed with appendectomy, open ( right lower
either quadrant horizontal muscle splitting incision) or laparoscopic.
Preoperative antibiotics and hydration are mandatory in all cases of suspected
appendicitis. All wounds are closed primarily without drains. Complicated
appendicitis (gangrenous and perforated) receives postoperative antibiotics
until the child clinical condition improves, fever subsides, ileus are
gone and the WBC count normalizes. Postoperative persistent fever, ileus
or leukocytosis mandates an imaging search (usually CT-Scan) for intraabdominal
collections. These collections can be managed with percutaneous drainage
and continued antibiotherapy.
References:
1- Santini I, Pacheco R, Lugo-Vicente HL: Perforated
Appendicitis in Children: Evaluation of a delayed diagnosis (Spanish).
PR Health Science J 14(4):263-267, 1995
2- Dilley A, Wesson D, Munden M, Hicks J, Brandt M, Minifee
P, Nuchtern J: The impact of ultrasound examinations on the management
of children with suspected appendicitis: a 3-year analysis. J Pediatr Surg
36(2):303-8, 2001
3- Gwynn LK: The diagnosis of acute appendicitis: clinical
assessment versus computed tomography evaluation. J Emerg Med 21(2):119-23,
2001
4- Meguerditchian AN, Prasil P, Cloutier R, Leclerc S,
Peloquin J, Roy G: Laparoscopic appendectomy in children: A favorable alternative
in simple and complicated appendicitis. J Pediatr Surg 37(5):695-8,
2002
5- Chen C, Botelho C, Cooper A, Hibberd P, Parsons SK:
Current practice patterns in the treatment of perforated appendicitis in
children. J Am Coll Surg 196(2):212-21, 2003
6- Emil S, Laberge JM, Mikhail P, Baican L, Flageole
H, Nguyen L, Shaw K: Appendicitis in children: A ten-year update of therapeutic
recommendations. J Pediatr Surg 38(2):236-42, 2003
7- Newman K, Ponsky T, Kittle K, Dyk L, Throop C, Gieseker
K, Sills M, Gilbert J: Appendicitis 2000: Variability in practice, outcomes,
and resource utilization at thirty pediatric hospitals. J Pediatr Surg
38(3):372-9, 2003
Acute Cholecystitis
Acute cholecystitis in children is a rare disease entity associated
with hemolytic diseases (sickle-cell disease, hereditary spherocytosis
and thalassemia), severe intercurrent illness and congenital anomalies.
Children presents with fever, nausea, vomiting, acute abdominal right upper
quadrant pain (positive Murphy' sign from a distended, tender gallbladder)
and leukocytosis. In a few patient a mass may be present in the right upper
quadrant. With jaundice the suspicion of common bile ducts stones should
be raised. Chronic cholecystitis with cholelithiasis is a more common presentation
in children than acute cholecystitis. Diagnosis can be establish with ultrasound
(thickened gallbladder wall) and use of HIDA bilioenteric studies
to asses patency of the cystic duct (procedure of choice). Initial management
consist of hydration and antibiotics to cool down the inflammatory process.
This is followed by early laparoscopic cholecystectomy except in patients
presenting with a gallbladder phlegmon later than seven days after the
onset of the attack. Laparoscopic cholecystectomy for acute cholecystitis
in patients with sickle cell disease has also been found to be safe and
recommended in experienced hands with adequate preoperative preparation
of the child.
References:
1- Ziv Y, Feigenberg Z, Dintsman M: Acute inflammation
and distension of the gall bladder in infancy. Aust Paediatr J 23(1):53-4,
1987
2- Coughlin JR, Mann DA: Detection of acute cholecystitis
in children. Can Assoc Radiol J 41(4):213-6, 1990
3- Reiss R, Nudelman I, Gutman C, Deutsch AA: Changing
trends in surgery for acute cholecystitis. World J Surg 14(5):567-70,
1990
4- Rescorla FJ, Grosfeld JL: Cholecystitis and cholelithiasis
in children. Semin Pediatr Surg 1(2):98-106, 1992
5- Lugo-Vicente HL: Trends in Management of Gallbladder
Disorders in Children. Pediatr Surg Internat 12(5-6):348-352, 1997
6- Holcomb GW 3rd, Morgan WM 3rd, Neblett WW 3rd, Pietsch
JB, O'Neill JA Jr, Shyr Y: Laparoscopic cholecystectomy in children: lessons
learned from the first 100 patients. J Pediatr Surg 34(8):1236-40,
1999
7- Avrutis O, Friedman SJ, Meshoulm J, Haskel L, Adler
S: Safety and success of early laparoscopic cholecystectomy for acute cholecystitis.
Surg Laparosc Endosc Percutan Tech 10(4):200-7, 2000
8- Sinha R, Sharma N: Acute cholecystitis and laparoscopic
cholecystectomy. JSLS 6(1):65-8, 2002
Surgical Ethics
Surgical ethics is based on recognition of the rights of patients who
require care by a surgeon. The four basic principles of ethics that we
will consider in a series of future reviews are beneficence, no-maleficence,
respect for autonomy and justice in what pertains the practice of surgery
and medicine. We must recognize that patients have seven basic rights:
1- the right not to be killed intentionally or negligently, 2- not to be
harmed by intent or negligence and 3- not to be deceived by the surgeon.
Patients also have the right to: 4- be adequately informed about the risks
and benefit of surgery, 5- be treated by a knowledgeable competent practitioner,
6- to have his or her health and well-being more highly valued than the
surgeon's own economic interest, and to 7- decide whether to accept treatment
under the conditions described. Surgeons must act as moral fiduciary of
the patient always avoiding them to come into self-interest conflicts.
Honesty is the general fiduciary commitment to protect and promote the
interest of the patient if surgical ethics is to guide the clinical judgement
and practice of surgeons in a comprehensive way.
References:
1- Mc Cullough LB, Jones JW, Brody BA: Surgical Ethics.
1st ed. New York: Oxford University Press, 1998
2- H. Tristram Engelhardt, Jr.: The Foundation of Bioethics,
2nd ed. New York: Oxford University Press, 1996
Volume 20 No 06 JUNE 2003
Cricopharyngeal Achalasia
Congenital cricopharyngeal achalasia (CCA) is an important but relatively
seldom diagnosed cause of dysphagia in children caused by failure of the
cricopharyngeal muscle to relax at the appropriate time during the third
period of swallowing mechanisms in the absence of other motor abnormalities.
Age of initial presentation ranges from birth to six months. Symptoms include
failure to thrive, regurgitation of food, choking, cyanosis, nasal reflux,
coughing and recurrent aspiration pneumonia. Cine-esophagogram with fluoroscopic
observation of the swallowing mechanism will establish the diagnosis in
most children. The characteristic sign is a round and regular posterior
narrowing on the posterior wall of the esophagus at the level of C4-C5
with enlargement of the hypopharynx. Esophageal motility studies will quantify
changes and also evaluate lower esophageal dysfunction not easily identified
in esophagograms. Though CCA can rarely be found as an isolated condition,
it is usually associated with neurologic deficit as seen in myelomeningocele
and Arnold Chiari malformations. Management of CCA includes positioning,
nasogastric lavage feedings, balloon dilatation of the upper esophagus
or surgical myotomy of the cricopharyngeal muscle. If all other measures
fails, cricopharyngeal myotomy is a safe and effective operation with excellent
results. Symptomatic relief is immediate and complete with no long-term
recurrence documented.
References:
1- Lernau OZ, Sherzer E, Mogle P, Nissan S: Congenital
cricopharyngeal achalasia treatment by dilatations. J Pediatr Surg
19(2):202-3, 1984
2- Skinner MA, Shorter NA: Primary neonatal cricopharyngeal
achalasia: a case report and review of the literature. J Pediatr Surg
27(12):1509-11, 1992
3- Raboei E, Luoma R: Neonatal cricopharyngeal achalasia--a
case report. Eur J Pediatr Surg 10(2):130-2, 2000
4- Brooks A, Millar AJ, Rode H: The surgical management
of cricopharyngeal achalasia in children. Int J Pediatr Otorhinolaryngol
56(1):1-7, 2000
5- Muraji T, Takamizawa S, Satoh S, Nishijima E, Tsugawa
C, Tamura A, Shimizu N: Congenital cricopharyngeal achalasia: Diagnosis
and surgical management. J Pediatr Surg 37(5):E12, 2002
Ganglioneuroma
Ganglioneuroma is a rare and benign neural mature crest tumor of the
sympathetic nervous system. They arise wherever sympathetic tissue exists
and may be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum,
and pelvis. The clinical presentation of most patients is that of an asymptomatic
slow growing solid mass in an older child. On many occasions the tumor
is found incidentally after simple chest films or abdominal imaging studies.
Imaging studies cannot differentiate a ganglioneuroma from its malignant
counterpart neuroblastoma. Rarely the child develops respiratory problems,
painless spinal deformity or neurologic deficit before the mass is found.
Most of these tumors are hormone silent. Histologically, ganglioneuroma
is completely differentiated and composed of mature ganglion cells, Schwann's
cells and neuropils. Ganglioneuromas frequently produce somatostatin and
vasoactive intestinal peptide (VIP). Management consists of surgical excision
whenever possible. Transperitoneal laparoscopic adrenalectomy has been
safely performed when this tumor arises from the adrenal gland.
References:
1- Young DG: Thoracic neuroblastoma/ganglioneuroma. J
Pediatr Surg 18(1):37-41, 1983
2- Girgert R, Schweizer P, Schwable J: Neuroblastoma:
induction of differentiation (Part I). Basical science in pediatric surgery.
Eur J Pediatr Surg 10(2):79-82, 2000
3- Miller KA, Albanese C, Harrison M, Farmer D, Ostlie
DJ, Gittes G, Holcomb GW 3rd: Experience with laparoscopic adrenalectomy
in pediatric patients. J Pediatr Surg 37(7):979-82, 2002
4- Lonergan GJ, Schwab CM, Suarez ES, Carlson CL: Neuroblastoma,
ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.
Radiographics 22(4):911-34, 2002
5- C. Thomas Blank: Neuroblastoma, in Andrassy "Pediatric
Surgical Pathology", WB Saunders Co, Philadelphia, 1998, pages 177-180.
Thoracoscopy
As we enter the 21st century, minimal invasive surgical procedure will
continue to demonstrate its superiority over conventional open surgery
in terms of pain control, convalescence, hospital stay, cosmesis and achieving
its purpose. Thoracoscopy using video-endoscopic technique is reliantly
replacing open thoracotomy in many chest conditions in children. As diagnostic
aid, thoracoscopy can be use for biopsy of lung, pleural, foregut and mediastinal
benign and malignant masses. Therapeutically, thoracoscopically has been
utilized for excision of esophageal duplication cysts, closure of patent
ductus arteriosus, pleurodesis, resection of lung (bullectomy, lobectomy
or segmentectomy), thymectomy, anterior spinal fusion procedures for scoliosis,
management of empyema, and resection of mediastinal cysts and tumors. Almost
the entire anterior and posterior mediastinum is visible by thoracoscopy.
Advantages of thoracoscopic procedure consist in less pain, less compromise
of pulmonary reserve, shorter hospital stay and costs. Contraindications
for thoracoscopy consist of inability to develop a pleural window with
the scope and patients in high pressure mechanical ventilation in need
of lung biopsy due to the high incidence of postop air leak. Once the thoracoscope
is in you should always perform an evaluation of the anatomy before committing
the child to an open thoracotomy.
References:
1- Rothenberg SS: Thoracoscopy in infants and children.
Semin Pediatr Surg 7(4):194-201, 1998
2- Michel JL, Revillon Y, Montupet P, Sauvat F, Sarnacki
S, Sayegh N, N-Fekete C: Thoracoscopic treatment of mediastinal cysts in
children. J Pediatr Surg 33(12):1745-8, 1998
3- Rothenberg SS: Thoracoscopic lung resection in children.
J Pediatr Surg 35(2):271-4, 2000
4- Kogut KA, Bufo AJ, Rothenberg SS, Lobe TE: Thoracoscopic
thymectomy for myasthenia gravis in children. J Pediatr Surg 35(11):1576-7,
2000
5- Subramaniam R, Joseph VT, Tan GM, Goh A, Chay OM:
Experience with video-assisted thoracoscopic surgery in the management
of complicated pneumonia in children. J Pediatr Surg 36(2):316-9,
2001
6- Partrick DA, Rothenberg SS: Thoracoscopic resection
of mediastinal masses in infants and children: an evaluation of technique
and results. J Pediatr Surg 36(8):1165-7, 2001
7- Partrick DA, Bensard DD, Teitelbaum DH, Geiger JD,
Strouse P, Harned RK: Successful thoracoscopic lung biopsy in children
utilizing preoperative CT-guided localization. J Pediatr Surg 37(7):970-3,
2002
8- Smith TJ, Rothenberg SS, Brooks M, Bealer J, Chang
J, Cook BA, Cullen JW: Thoracoscopic surgery in childhood cancer. J Pediatr
Hematol Oncol 24(6):429-35, 2002
9- Warmann S, Fuchs J, Jesch NK, Schrappe M, Ure BM:
A prospective study of minimally invasive techniques in pediatric surgical
oncology: preliminary report. Med Pediatr Oncol 40(3):155-7, 2003
10- Rodgers BM: The Role of Thoracoscopy in Pediatric
Surgical Practice. Semin Pediatr Surg 12(1): 62-70, 2003