PEDIATRIC SURGERY UPDATE ©
VOLUME 09, 1997
VOL 09 NO 01 JULY 1997
Adreno Cortical Carcinoma
Adreno-cortical carcinoma (ACC) is a rare potentially fatal tumor during
childhood. It occurs with significant frequency in Southern Brazil. Virilization
alone (pubic and/or body hair, clitoris or penis enlargement, and adult
voice), or associated to Cushing's syndrome from corticosteroid hypersecretion
is the most prominent feature. Feminizing tumors are uncommon and nonfunctional
tumors rare. Most children are females with a median age of four years.
Other signs include: abdominal mass, hypertension, seizures, and weight
loss. Features associated with malignancy included high levels of 17-keto
steroids, tumor weight greater than 100 g, tumor size greater than 200
cm3, and histologic evidence of diffuse growth pattern, vascular invasion,
and tumor cell necrosis. CT Scan is the single most important modality
in assessing primary and metastatic disease at diagnosis, and plays an
important role in defining the extent of the primary lesion preoperatively.
Early diagnosis and aggressive surgical extirpation may lead to an increased
survival of children with ACC. An anterior thoracoabdominal approach is
favored for suspected malignancies with en bloc resection. There is no
evidence that adjuvant therapy provides any additional benefit. The prognosis
is age related and dependent on the resectability of the tumor. Children
with small adrenocortical tumors have an excellent prognosis with surgery
as sole therapy.
References
1- Daneman A, Chan HS, Martin J: Adrenal carcinoma
and adenoma in children: a review of 17 patients. Pediatr Radiol 13(1):11-8,
1983
2- Kay R, Schumacher OP, Tank ES: Adrenocortical carcinoma
in children. J Urol 130(6):1130-2, 1983
3- Neblett WW, Frexes-Steed M, Scott HW Jr: Experience
with adrenocortical neoplasms in childhood. Am Surg 53(3):117-25, 1987
4- Chudler RM, Kay R: Adrenocortical carcinoma in children.
Urol Clin North Am 16(3):469-79, 1989
5- Ribeiro RC, Sandrini Neto RS, Schell MJ, Lacerda L,
Sambaio GA, Cat I: Adrenocortical carcinoma in children: a study of 40
cases. J Clin Oncol 8(1):67-74, 1990
6- Arico M, Bossi G, Livieri C, Raiteri E, Severi F: Partial
response after intensive chemotherapy for adrenal cortical carcinoma in
a child. Med Pediatr Oncol 20(3):246-8, 1992
7- Sabbaga CC, Avilla SG, Schulz C. Garbers JC, Blucher
D: Adrenocortical carcinoma in children: clinical aspects and prognosis.
J Pediatr Surg 28(6):841-3, 1993
8- al-Salem AH, Abu-Srair HA: Recurrent adrenocortical
carcinoma in a 4 year old girl. Aust N Z J Surg 64(10):723-5, 1994
9- Teinturier C, Brugieres L, Lemerle J, Chaussain JL,
Bougneres PF: [Adrenocortical carcinoma in children: retrospective study
of 54 cases]. Arch Pediatr 3(3):235-40, 1996
10- Michalkiewicz EL, Sandrini R, Bugg MF, Cristofani
L, Caran E, Cardoso AM, de Lacerda L, Ribeiro RC: Clinical characteristics
of small functioning adrenocortical tumors in children. Med Pediatr Oncol
28(3):175-8, 1997
Pheochromocytoma
Pheochromocytoma is a rare APUD (amine precursor uptake decarboxylate)
tumor in children. A well differentiated, functionally mature neoplasm
that originates mainly from the adrenal medulla, other times from the sympathetic
chain, the paraganglia or organ of Zuckerkandl. Most patients are males,
with an average age of 9-11 years showing sustained hypertension (80%);
24% of these tumors are bilateral, 15% multiple and 15% extra-adrenal in
location. Other signs are: headache, sweating, blurred vision, fatigue,
palpitations, and anorexia. An increase familial incidence as a single
Mendelian dominance is also noted in the pediatric age group. Affected
patients should be screened for MEN 2. The most accurate method of diagnosis
is cathecolamines determination in blood and urine (VMA). CT-Scan will
localize most tumors, and the yield can be increased with the complementary
use of MIBG (meta-iodo-benzylguanidine), an adrenergic tissue localizing
agent, particularly in extra-adrenal or metastatic lesions. Management
consists of preoperative control of hypertension with alpha adrenergic
blocking agents followed by transabdominal surgical resection under strict
intra-operative monitoring. The high percentage of children with bilateral,
extra-adrenal, and multiple tumors warrants the need for long-term follow-up.
Metastatic well-differentiated tumors are better managed with radioactive
MIBG. In the malignant variety chemotherapy should be used for residual
disease after surgery and for metastatic disease.
References
1- Lugo-Vicente HL, Marquez-Grau E: Diagnostic and
Therapeutic Problems arising from a Celiac Axis Pheochromocytoma in a Child:
A Case Report. Boletin Asoc Med PR 79:325-328, 1987
2- Ein SH, Weitzman S, Thorner P, Seagram CG, Filler RM:
Pediatric malignant pheochromocytoma. J Pediatr Surg 29(9):1197-201,
1994
3- Gelfand MJ: Meta-iodo-benzylguanidine in children.
Semin Nucl Med 23(3):231-42, 1993
4- Aguilo F, Tamayo N, Vazquez-Quintana E, Rabell
V, Haddock L, Allende M, Pagan H, Gonzalez A: Pheochromocytoma: a twenty
year experience at the University Hospital [published erratum appears in
P R Health Sci J 11(1):6] P R Health Sci J 10(3):135-42, 1991
5- Daneman A: Adrenal neoplasms in children. Semin Roentgenol
23(3):205-15, 1988
6- Revillon Y, Daher P, Jan D, Buisson C, Bonnerot V,
Martelli H, Nihoul-Fekete C: Pheochromocytoma in children: 15 cases. J
Pediatr Surg 27(7):910-1, 1992
7- Khafagi FA, Shapiro B, Fischer M, Sisson JC, Hutchinson
R, Beierwaltes WH: Pheochromocytoma and functioning paraganglioma in childhood
and adolescence: role of iodine 131 metaiodobenzylguanidine. Eur J Nucl
Med 18(3):191-8, 1991
8- Fonkalsrud EW: Pheochromocytoma in childhood.
Prog Pediatr Surg 26:103-11, 1991
9- Ein SH, Shandling B, Wesson D, Filler Rm: Recurrent
pheochromocytomas in children. J Pediatr Surg 25(10):1063-5, 1990
Pilomatrixoma
Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign
tumor of the skin originating from hair sheath cells. Most cases occur
in children less than ten-years of age with a 2:1 female to male ratio.
Presents clinically as asymptomatic superficial slow-growing dermal or
subcutaneous mass most commonly located in the head and neck or upper extremity.
Histology shows two characteristic epithelial cells: the basophilic and
ghost cells, and calcification is usually present (these features can help
the diagnosis when using FNA cytology). Pilomatrixoma can be confused with
an epidermal inclusion cyst, lymph node, or calcified hematoma. The "faceted
stone sign" (palpation of a tumor with a faceted rocky consistency) is
evident. Management consists of local excision, and recurrences are very
rare. Prognosis after removal is excellent. Multiple familial pilomatricomas
could be considered a cutaneous marker of Gardner syndrome.
References
1- Hawkins DB, Chen WT: Pilomatrixoma of the head and
neck in children. Int J Pediatr Otorhinolaryngol 8(3):215-23, 1985
2- Ganz H: [Is Malherbe calcifying epithelioma in the
ENT area of a rare tumor?] HNO 34(7):301-4, 1986
3- Solanki P, Ramzy I, Durr N, Henkes D: Pilomatrixoma.
Cytologic features with differential diagnostic considerations. Arch Pathol
Lab Med 111(3):294-7, 1987
4- Nield DV, Saad MN, Ali MH: Aggressive pilomatrixoma
in a child: a case report. Br J Plast Surg 39(1):139-41, 1986
5- Grinspan Bozza NO: [Pilomatrixoma. A series of 18 surgically
treated cases. A sign for its diagnosis] Med Cutan Ibero Lat Am 17(6):387-90,
1989
6- Unger P, Watson C, Phelps RG, Danque P, Bernard P:
Fine needle aspiration cytology of pilomatrixoma (calcifying epithelioma
of Malherbe). Report of a case. Acta Cytol 34(6):847-50, 1990
7- Marrogi AJ, Wick MR, Dehner LP: Pilomatrical neoplasms
in children and young adults. Am J Dermatopathol 14(2):87-94, 1992
8- Wells NJ, Blair GK, Magee JF, Whiteman DM: Pilomatrixoma:
a common, benign childhood skin tumor. Can J Surg 37(6):483-6, 1994
9- Pujol RM, Casanova JM, Egido R, Pujol J, de Moragas
JM: Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome?
Pediatr Dermatol 12(4):331-5, 1995
10- Kumar N, Verma K, Fine needle aspiration (FNA) cytology
of pilomatrixoma. Cytopathology 7(2):125-31, 1996
Vol 09 No 02 AUGUST 1997
Spherocytosis
Hereditary spherocytosis is a clinically heterogenous autosomal
dominant red blood cell membrane disorder that causes anemia. The genetic
defect results in deficiency of spectrin, the largest and most abundant
structural protein of the erythrocyte membrane skeleton. The affected RBC
loses its biconcave shape, strength and flexibility to the stress of circulation,
becomes round and is trapped and destructs early in the spleen. Initial
symptoms are those of pallor, jaundice and chronic anemia, followed by
splenomegaly. Hemolytic crises are triggered by intercurrent infections.
Pigment gallstones are common after the first decade of life. Labs' findings
are those of many spherocytes in the peripheral smear, 8-10 mg% hemoglobin,
elevated reticulocyte count, increase erythropoeisis in the bone marrow,
and negative Coomb's test. Erythrocytes' shows increased osmotic fragility
with autohemolysis in hypotonic solutions. Definitive therapy consists
of splenectomy. This condition is the most common indication for elective
splenectomy in children. The risk of overwhelming postsplenectomy sepsis
makes it advisable to delay splenectomy until after six years of age unless
the child becomes transfusion dependent. At the time of splenectomy, it
is important to identify and remove accessory spleens. If gallstones are
present, cholecystectomy should be done. A low content of spectrin and
high percentage of microcyte has been used as determinants of early splenectomy
as judge by the clinical severity of the disease process. Howell-Jolly
bodies in erythrocyte are identified after total splenectomy.
References
1- Burke BE,Shotton DM: Erythrocyte membrane skeleton
abnormalities in hereditary spherocytosis. Br J Haematol 54(2):173-87,
1983
2- Schroter W, Kahsnitz E: Diagnosis of hereditary spherocytosis
in newborn infants. J Pediatr 103(3):460-3, 1983
3- Lux SE: A genetic defect in the binding of protein
4.1 to spectrin in a kindred with hereditary spherocytosis. N Engl
J Med 25;307(22):1367-74, 1982
4- Orringer EP, Sheldon GF: Hereditary spherocytosis.
Recent experience and current concepts of pathophysiology. Ann Surg 203(1):34-9,
1986
5- Eber SW, Armbrust R, Schroter W: Variable clinical
severity of hereditary spherocytosis: relation to erythrocytic spectrin
concentration, osmotic fragility, and autohemolysis. J Pediatr 117(3):409-16,
1990
6- Koh MT, Ng SC: Hereditary spherocytosis: a study of
16 patients from University Hospital, Kuala Lumpur. Singapore Med J 32(1):67-9,
1991
7- Gigot JF, Legrand M, Cadiere GB, Delvaux G, de Ville
de Goyet J, de Neve de Roden A, Van Vyve E, Hourlay P, Etienne J, Njinou
B, et al: Is laparoscopic splenectomy a justified approach in hematologic
disorders? Preliminary results of a prospective multicenter study. Belgian
Group for Endoscopic Surgery. Int Surg 80(4):299-303, 1995
8- Hicks BA, Thompson WR, Rogers ZR, Guzzetta PC: Laparoscopic
splenectomy in childhood hematologic disorders. J Laparoendosc Surg 6 Suppl
1:S31-4, 1996
Cystosarcoma Phylloides
First described by Muller in 1838, cystosarcoma phylloides (CSP) is
a rare breast neoplasm of fibroepithelial origin rarely seen in adolescent
females. Phylloides tumors have been histologically classified as benign,
borderline, or malignant. Classification of malignancy is based on stromal
findings of cellular atypia, anaplasia, and degree of mitotic activity.
Malignancy is similar to sarcomas of the breast. Presentation of CSP is
that of a relative large, movable, painless mass sought to be a fibroadenoma.
Lack complete encapsulation and extends into surrounding tissue in multiple
projections of different sizes. Young patients elicited a short history
with rapid growth of the lesion. FNA cytology is often diagnostic of CSP.
Management depends on histopathology. Benign lesions can be managed with
local excision (enucleation) using a cosmetic incision. Borderline or malignant
CSP should be treated with wide local excision (simple mastectomy) or re-excision
to negative margins. Failure of complete excision results in local recurrence.
Metastasis are bloodborn involving most frequently the lung and bones.
Axillary dissection is recommended only if nodes are palpable. Adjuvant
radiation is necessary, if wide local spread of the tumor is present and
a resection of the lesion with a 2-cm tumorfree zone is not possible. Close
followup is mandatory.
References
1- Mollitt DL, Golladay ES, Gloster ES, Jimenez JF: Cystosarcoma
Phylloides in the Adolescent Female. J Pediatr Surg 22(10): 907-910, 1987
2- Hines JR, Murad TM, Beal JM: Prognostic indicators
in cystosarcoma phylloides. Am J Surg 153(3):276-80, 1987
3- Blanckaert D, Lecourt O, Loeuille GA, Six J, Laurent
JC: [Phyllodes tumor of the breast in an 11-year-old child] Pediatrie 43(5):405-8,
1988
4- Sheen-Chen SM, Chou FF, Chen WJ: Cystosarcoma phylloides
of the breast: a review of clinical, pathological and therapeutic option
in 18 cases. Int Surg 76(2):101-4, 1991
5- Vesely F, Baco E, Hudcova D: [Cystosarcoma phylloides
in adolescent women] Rozhl Chir 71(9):456-63, 1992
6- Mallebre B, Ebert A, Perez-Canto A, Hopp H, Opril F,
Weitzel H: [Cystosarcoma phylloides of the breast. A retrospective analysis
of 12 cases] Geburtshilfe Frauenheilkd 56(1):35-40, 1996
7- Briggs R, Walters M. Rosenthal D: Cystosarcoma phylloides
in adolescent female patients. Am J Surg 146: 712-714, 1983
8- Moore MP, Kinne DW: Breast Sarcoma. Surg Clin NA 76(2):
383-392, 1996
Vaginal Agenesis
It is estimated that vaginal atresia (or agenesis) occurs in every one
to 5-10,000 birth, the result of the Mayer-Rokitansky syndrome (partial
or complete absence of the uterus with an absent or hypoplastic vagina),
or an intersexual condition. In either case the vagina may be represented
by a solid epithelial cord. Usually there is concomitant aplasia of the
upper part of the paramesonephric ducts so that the uterus and tubes are
rudimentary. The ovaries are usually normal. Associated anomalies
of the urinary tract are frequent. Those cases not diagnosed in early infancy
will eventually present during adolescence with primary amenorrhea and
cyclic pelvic abdominal pain. US, CT, and recently MRI is extremely useful
in delineating the anatomic malformation. Many ingenious procedures have
been devised for creation of a neovagina in children: skin-flaps and bowel
(colon) replacement vaginoplasty. They are accomplished during puberty.
References
1- Martinez-Mora J, Castellvi IA, Lopez-Ortiz P: Neovagina
in Vaginal Agenesis: Surgical Methods and Long-Term Results. J Pediatr
Surg 27(1): 10-14, 1992
2- Strubbe EH, Willemsen WN, Lemmens JA, Thijn CJ,
Rolland R: Mayer-Rokitansky-Kuster-Hauser syndrome: distinction between
two forms based on excretory urographic, sonographic, and laparoscopic
findings. AJR Am J Roentgenol 160(2):331-4, 1993
3- Orozco-Sanchez J, Neri-Vela R, Flores-Mendez MS, Sandoval-Sevilla
SF, Leon-Cordova K: [Congenital atresia of the vagina] Bol Med Hosp Infant
Mex 48(9):648-55, 1991
4- Hitchcock RJ, Malone PS: Colovaginoplasty in infants
and children. Br J Urol 73(2):196-9, 1994
5- Salvatore CA, Lodovicci O: Vaginal agenesis: an analysis
of ninety cases. Acta Obstet Gynecol Scand 57(1):89-94, 1978
Vol 09 No 03 SEPTEMBER 1997
Idiopathic Thrombocytopenic Purpura
Idiopathic thrombocytopenic purpura (ITP) is probably the most frequent
cause of persistent thrombocytopenia in children. Antedated by a viral
(usually respiratory) infection, ITP is characterized by acute hemorraghic
manifestations such as petechia, purpura, and mucous membrane bleeding
(epistaxis). Platelet number and survival time are reduced significantly
causing prolonged bleeding time. This is caused by rapid destruction of
the reticuloendothelial system (spleen, bone marrow, and liver) due to
IgG binding to platelets (autoinmune). Acute ITP regresses spontaneously
within 3 to 6 months in 80% of cases. Initial management may consist of
short steroid course and/or immunoglobulin. Chronic ITP sets itself if
thrombocytopenia persists longer than six months. When this occurs, most
cases are asymptomatic except during intercurrent infectious process. Platelet
counts below 50,000/mm3, failure of medical therapy, long-term child restriction,
or persistent bleeding may need splenectomy. Complete clinical cure is
obtained in 70-80% of instances following splenectomy with a low operative
risk. Recurrent symptoms may be caused by a missed accessory spleen.
Laparoscopic splenectomy is a safe, cost-effective procedure with reduced
hospital stay, faster recovery, better cosmetic results and least trauma.
Isolated splenic thrombocytolysis and hyperplasia of megakaryocytopoiesis
and of splenic follicles after splenectomy correlated with better stable
remission and platelet counts. The response to splenectomy has been found
to correlate directly with the response to immunoglobulin therapy. Intermittent
immunoglobulin therapy with observation is a reasonable alternative until
spontaneous remission occurs.
References
1- E. Thomas Boles: The Spleen. In Medad Schiller ‘Pediatric
Surgery of the Liver, Pancreas and Spleen', W.B. Saunders, 1991, pag 210-212
2- Facon T, Caulier MT, Fenaux P, Plantier I, Marchandise
X, Ribet M, Jouet JP, Bauters F: Accessory spleen in recurrent chronic
immune thrombocytopenic purpura. Am J Hematol 41(3):184-9, 1992
3- Reid MM: Chronic idiopathic thrombocytopenic purpura:
incidence, treatment, and outcome. Arch Dis Child 72(2):125-8, 1995
4- Yoshida K, Yamazaki Y, Mizuno R, Yamadera H, Hara A,
Yoshizawa J, Kanai M: Laparoscopic splenectomy in children. Preliminary
results and comparison with the open technique. Surg Endosc 9(12):1279-82,
1995
5- Winde G, Schmid KW, Lugering N, Fischer R, Brandt B,
Berns T, Bunte H: Results and prognostic factors of splenectomy in idiopathic
thrombocytopenic purpura. J Am Coll Surg 183(6):565-74, 1996
6- Law C, Marcaccio M, Tam P, Heddle N, Kelton JG: High-dose
intravenous immune globulin and the response to splenectomy in patients
with idiopathic thrombocytopenic purpura. N Engl J Med 22;336(21):1494-8,
1997
Malone Stoma
This safe and highly effective novel technique was introduced by PS
Malone in 1990. Consist of building a continent appendicocecostomy through
which the cecum is intermittently catheterized for administration of an
antegrade enema to manage fecal soiling, incontinence, and even intractable
constipation if nonsurgical management has failed. This includes children
with anorectal malformations, neuropathic etiology (spina bifida, spinal
cord injury), and sphincteric trauma. Originally described as reversing
the appendix and reimplanting it into the cecum with a submucous tunnel
to diminish reflux, the procedure has undergone several modifications such
as orthotopic cecal imbrication, the fixation of the ileocecal region at
the inner side of the abdominal wall after creation of an appendicocutaneous
catheterizable stoma, laparoscopic approach, tubularized cecal or
ileal flap in those without an appendix, and the use of a button. The child
will catheterize once or twice a day washing his distal colon. Most
families are satisfied since the child spends more time in normal activities
and school. A level of commitment by the child and family is needed. Children
with colonic motility disorders and incontinence may not beneficiate from
this procedure. The stoma might break, stenosed, closed, or bleed in a
few cases. If the child is socially continent with a regimen of enemas,
suppositories or washouts, he should continue that way.
References
1- Malone PS, Ransley PG, Kiely EM: Preliminary report:
The antegrade continent enema. Lancet 336: 1217-1218, 1990
2- Squire R, Kiely EM, Carr B, Ransley PG, Duffy PG: The
Clinical Application of the Malone Antegrade Colonic Enema. J Pediatr Surg
28(8): 1012-1015, 1993
3- Griffiths DM, Malone PS: The Malone Antegrade Continence
Enema. J Pediatr Surg 30(1): 68-71, 1995
4- Goepel M, Sperling H, Stohrer M, Otto T, Rubben H:
Management of neurogenic fecal incontinence in myelodysplastic children
by a modified continent appendiceal stoma and antegrade colonic enema.
Urology 49(5):758-61, 1997
5- Yamamoto T, Kubo H, Honzumi M: Fecal incontinence
successfully managed by antegrade continence enema in children: a report
of two cases. Surg Today 26(12):1024-8, 1996
6- Kiely EM, Ade-Ajayi N, Wheeler R: Antegrade continence
enemas in the management of intractable faecal incontinence. J R Soc Med
88(2):103P-104P, 1995
RET Oncogene
The RET proto-oncogene is a protein tyrosine kinase gene (Ret protein)
expressed in the cells derived from the neural crest. Germline mutations
in the RET gene have been associated with neuroblastoma, pheochromocytoma,
multiple endocrine neoplasia (MEN) 2, familial medullary thyroid carcinoma
(MTC), radiation-induced thyroid papillary carcinoma, and recently Hirschsprung's
disease. The Ret protein might have a critical role in the embryogenesis
of the enteric nervous system. RET analysis is a suitable method to detect
asymptomatic children with MEN at risk to develop MTC allowing us to consider
thyroidectomy at a very early stage of neoplasm development (C-cell hyperplasia)
or prophylactically.
References
1- Lyonnet S, Edery P, Mulligan LM, Pelet A, Dow
E, Abel L, Holder S, Nihoul-Fekete C, Ponder BA, Munnich A: [Mutations
of RET proto-oncogene in Hirschsprung disease] C R Acad Sci III 317(4):358-62,
1994
2- Rossel M, Schuffenecker I, Schlumberger M, Bonnardel
C, Modigliani E, Gardet P, Navarro J, Luo Y, Romeo G, Lenoir G, et al:
Detection of a germline mutation at codon 918 of the RET proto-oncogene
in French MEN 2B families. Hum Genet 95(4):403-6, 1995
3- Martucciello G, Favre A, Takahashi M, Jasonni V: Immunohistochemical
localization of RET protein in Hirschsprung's disease. J Pediatr Surg 30(3):433-6,
1995
4- Fugazzola L, Pilotti S, Pinchera A, Vorontsova TV,
Mondellini P, Bongarzone I, Greco A, Astakhova L, Butti MG, Demidchik EP,
et al: Oncogenic rearrangements of the RET proto-oncogene in papillary
thyroid carcinomas from children exposed to the Chernobyl nuclear accident.
Cancer Res 55(23):5617-20, 1995
5- Komminoth P, Kunz EK, Matias-Guiu X, Hiort O, Christiansen
G, Colomer A, Roth J, Heitz PU: Analysis of RET protooncogene point mutations
distinguishes heritable from nonheritable medullary thyroid carcinomas.
Cancer 76(3):479-89, 1995
6- Shimotake T, Iwai N, Inoue K, Inazawa J, Nishisho I:
Germline mutations of the RET proto-oncogene in pedigree with MEN type
2A: DNA analysis and its implications for pediatric surgery. J Pediatr
Surg 31(6):779-81, 1996
Vol 09 No 4 OCTOBER 1997
Perforated Appendicitis
Perforated appendicitis continues to occur at a similar rate than twenty
years ago, although there are scattered reports that children with symptoms
of appendicitis evaluated in managed care systems have shown a tendency
to lower perforation rates. Morbidity, mortality and longer hospital stays
takes its toll when this vestigial organ inflames and ruptures. Each stage
in appendicitis has a unique form of management. The standard goal of therapy
for children with perforated appendicitis is: aggressive hydration, immediate
appendectomy, systemic antibiotic therapy, copious peritoneal saline irrigation,
wound irrigation, and primary closure in the best hands. Open peritoneal
lavage has been found superior to intraperitoneal tube drainage or
closed postoperative peritoneal lavage in the management of perforated
appendicitis in children. Some authors have found that the technique of
peritoneal lavage rather than the disease process itself may be responsible
for some postoperative complications such as small bowel adhesions and
obstruction. Leaving the wound open brings painful local care, augment
absence from school, increases the hospital stay, and is not necessary
in most children. Subcuticular incisional closure results in minimal wound
care and excellent cosmetic results. Lavage of the surgical wound
with physiologic serum is an effective, safe and inexpensive method to
prevent infection of the wound following appendectomy.
References
1- Lund DP, Murphy EU: Management of perforated appendicitis
in children: a decade of aggressive treatment. J Pediatr Surg 29(8):1130-3;
discussion 1133-4, 1994
2- Samelson SL, Reyes HM: Management of perforated
appendicitis in children--revisited. Arch Surg 122(6):691-6, 1987
3-Toki A, Ogura K, Horimi T, Tokuoka H, Todani T,
Watanabe Y, Uemura S, Urushihara N, Noda T, Sato Y, et al: Peritoneal lavage
versus drainage for perforated appendicitis in children. Surg Today 25(3):207-10,
1995
4-Buanes TA, Andersen GP, Jacobsen U, Nygaard K: Perforated
appendicitis with generalized peritonitis. Prospective, randomized evaluation
of closed postoperative peritoneal lavage. Eur J Surg 157(4):277-9, 1991
5- Hallerback B, Andersson C, Englund N, Glise H,
Nihlberg A, Solhaug J, Wahlstrom B: A prospective randomized study of continuous
peritoneal lavage postoperatively in the treatment of purulent peritonitis.
Surg Gynecol Obstet 163(5):433-6, 1986
6- Putnam TC, Gagliano N, Emmens RW: Appendicitis
in children. Surg Gynecol Obstet 170(6):527-32, 1990
7- Karp MP, Caldarola VA, Cooney DR, Allen JE, Jewett
TC Jr: The avoidable excesses in the management of perforated appendicitis
in children. J Pediatr Surg 21(6):506-10, 1986
8- Santini I; Lugo-Vicente HL; Pacheco
R: [Perforated appendicitis in children: evaluation of delayed diagnosis].
P R Health Sci J 14(4):263-7, 1995
Nephrectomy in Neuroblastoma
Removal of a kidney involved with neuroblastoma (NB) should be done
for radiographic and intraoperative evidence of gross kidney involvement.
A recent study group from Japan for advanced NB (Stage III and IV)
evaluated the preservation of the ipsilateral kidney at surgery on subsequent
length of survival showing a better survival in the group of children whose
kidney was preserved, although they confess bias in the selection of patients
since the decision (for either nephrectomy or kidney preservation) was
not randomized. Kidney invasion in NB is by direct penetration through
the capsule or by extension along blood vessels. For curability, stage
III needs pathological (histological) evidence of gross tumor removal to
be effective therapy. When the tumor is adhere to vital structures
you should not risk injury to nerves, major blood vessels or organs (this
includes the kidney) by performing radical excision since there is no improvement
in survival. Furthermore unilateral nephrectomy could bring a higher risk
of chemotherapyinduced renal impairment. The main reasons to avoid nephrectomy
in cases of advanced NB are that survival is not improved, and morbidity/mortality
is increased.
References
1- Tsuchida Y, Yokoyama J, Kaneko M, Uchino J, Iwafuchi
M, Makino S, Matsuyama S, Takahashi H, Okabe I, Hashizume K, et al: Therapeutic
significance of surgery in advanced neuroblastoma: a report from the study
group of Japan. J Pediatr Surg 27(5):616-22, 1992
2- Albregts AE, Cohen MD, Galliani CA: Neuroblastoma invading
the kidney. J Pediatr Surg 29(7):930-3, 1994
3- Powis MR, Imeson JD, Holmes SJ: The effect of complete
excision on stage III neuroblastoma: a report of the European Neuroblastoma
Study Group. J Pediatr Surg 31(4):516-9, 1996
4- DeCou JM, Bowman LC, Rao BN, Santana VM, Furman WL,
Luo X, Lobe TE, Kumar M: Infants with metastatic neuroblastoma have
improved survival with resection of the primary tumor. J Pediatr Surg 30(7):937-40;
discussion 940-1, 1995
5- Schell M, Cochat P, Hadj-Aissa A, Bouffet E, Dubourg
L, Brunat-Mentigny M: Renal function following unilateral nephrectomy for
neuroblastoma and Wilms' tumour. Pediatr Nephrol 9(5):579-82, 1995
Preauricular Sinus
Preauricular sinus is a common congenital condition that does not always
cause symptoms. It arises from the anterior aspect of the helix, not branchial
cleft remnant related. The sinus passes through the skin anteriorly to
end in a racemose group of pre-auricular cysts. Its origin can be traced
to imperfect fusion of the six tubercles that form the pinna. Symptoms
occur after an episode of infection. Recurrent infection may lead to development
of a pre-auricular ulcer. Complete excision of the sinus tract and its
associated cyst down to the temporalis fascia effect a cure. Surgery should
be avoided during incipient infection. Excision under general anesthesia
with the use of an extended incision with removal of all epithelial component
down to the temporalis fascia will be usually needed. Massaging the sinus
may avoid longterm occlusion of its lumen with its attendant possibility
of infection.
References
1- Hornibrook J, Robertson MS: The management of preauricular
sinus. N Z Med J 11;97(747):18-9, 1984
2- Chami RG, Apesos J: Treatment of asymptomatic preauricular
sinuses: challenging conventional wisdom. Ann Plast Surg 23(5):406-11,
1989
3- Joseph VT, Jacobsen AS: Single stage excision of preauricular
sinus. Aust N Z J Surg 65(4):254-6, 1995
4- Currie AR, King WW, Vlantis AC, Li AK: Pitfalls in
the management of preauricular sinuses. Br J Surg 83(12):1722-4, 1996
PRAPS
June 1997 marked the long awaited creation of the Puerto Rico Association
of Pediatric Surgeons (PRAPS). ‘Pediatric Surgery Update' becomes the official
publication of PRAPS.
Vol 09 No 5 NOVEMBER 1997
Primary Hyperparathyroidism
Primary Hyperparathyroidism (HPT) considered once a rare disease has
an incidence of 0.01% to 0.02% since the availability of immunocytochemical
screening tests. HPT is more prevalent in females, with a ratio of 3:2.
Children are rarely afflicted by this condition. Dehydration and respiratory
distress result in higher mortality rates in neonatal and infantile HPT.
Hyperplastic parathyroid tissue is the most prevalent histopathological
finding during this developmental stage. After the age of one, adenomas,
a collection of chief cells surrounded by a rim of normal tissue at the
outer perimeter of the gland, are more commonly found. Nonspecific and
diffuse symptoms such as fatigue, nausea, vomiting, constipation, weakness,
dizziness, weight loss, failure to thrive, and personality changes are
frequently found on initial presentation of the illness. Skeletal lesions
are often instrumental in the pediatric setting, with an incidence of 44%
in children. Other complications less common in children are renal calculi,
pancreatitis, neuromuscular dysfunction, pseudogout and anemia. A diagnosis
should be suspected by elevated calcium levels and diminished phosphorus
levels in blood chemistry studies. Other studies such as ultrasonography
and isotope scans are helpful tools in diagnosing the condition. Management
is surgical. Indications for surgery are the presence of complications
(such as pancreatitis, renal calculi, etc.), life-threatening hypercalcemia,
hypercalciuria, sex, and a young patient.
References
1 - Cope, Oliver: The story of primary hyperparathyroidism
at the Massachusetts General Hospital. New England Journal of Medicine
66: 1174-82, 1966
2 - Bilezikien, John P: Primary Hyperparathyroidism. Primer
on the Metabolic Bone Disease and Disorders of Mineral Metabolism 2nd edition.
Lippincott-Raven pag. 155-58.
3 - Bjernulf A, Hall K, Sjogren I, et al: Primary hyperparathyroidism
in children. Acta Paediatr Scand 59:249-58, 1970.
4 - Allo M, Thompson NW, Harness JK, et al: Primary hyperparathyroidism
in children, adolescents and young adults. World Journal of Surgery. 6
:771-5, 1982
5 - Huang, et al :Primary Hyperparathyroidism in children
: Report of a case and a brief review of the literature. Journal of the
Formosan Medical Association 92:1095-8, 1993
Ewing's Sarcoma
Ewing's sarcoma is the second most common primary malignant bone tumor
of children. Comprise 25% of primary bone tumors. This small round blue
cell neoplasm most commonly arises from the diaphysis of long bones (femur,
humerus, tibia and fibula). Current thinking links Ewing's sarcoma and
primitive neuroectodermal tumor by a shared chromosomal translocation
(11;22)(q24;q12) and by evidence of neuroectodermal origin. Other
less common sites of development are pelvis, ribs, jaw and vertebra. Soft
tissue extension is common and extensive. Extraosseous origin has been
reported infrequently. The tumor is clinically characterized by pain, tenderness
and motion limitless. Pathologic fractures may be present. Constitutional
symptoms such as weight loss, malaise, fever and chills may also be present.
Ewing's is a destructive lesion of bone associated with periosteal reaction.
Findings in simple films include onion-skin formation, endosteal buttressing
and formation of Codman's triangle. Only a high quality biopsy can determine
histological diagnosis. Multiple agent chemotherapy is essential for long-term
survival. Multi agent protocols are superior to singleagent therapies.
Radiation therapy and surgery are employed for local control of primary
tumors. Disease-free survival rate is now between 60 and 70%. Children
with metastatic disease and pelvic primary have a worse prognosis.
References
1 - Jurgens HF: Ewing's sarcoma and peripheral primitive
neuroectodermal tumor. Curr Opin Oncol 6(4):391-6, 1994
2 - Vlasak R, Sim FH: Ewing's sarcoma. Orthop Clin North
Am 27(3):591-603, 1996
3 - Christie DR, Bilous AM, Carr PJ: Diagnostic difficulties
in extraosseous Ewing's sarcoma: a proposal for diagnostic criteria. Australas
Radiol 41(1):22-8, 1997
4 - Meyers PA: Malignant bone tumors in children: Ewing's
sarcoma. Hematol Oncol Clin North Am 1(4):667-73, 1987
5 - Horowitz ME: Ewing's sarcoma: current status of diagnosis
and treatment. Oncology (Huntingt) 3(3):101-6; discussion 106-9, 1989
6 - Hays DM: Pediatric Surgical Oncology. Grune &
Stratton Pub. 1986 pag 245-248.
7 - Granowetter L: Ewing's sarcoma. Curr Opin Oncol 3(4):684-8,
1991
8 - Gentet JC, Panuel M, Sheiner C,
Coze C, Capodano AM, Bollini G, Bernard JL, Raybaud C:
[Ewing's tumor: current knowledge and --ignorance]. Pediatrie (Bucur) 47(12):799-807,
1992
Carcinoids
This argentaffin cell tumor causes interest because of its diverse
presentation, hormonal secretion, and malignant potential. The carcinoid
is the most common neoplasm of the GI tract in childhood and may occur
at any site along the alimentary tract. Above the diaphragm is commonly
identified in the bronchus, and below the diaphragm in the appendix. Female
predominates, the tumor is seldom life-threatening, and children rarely
develop hormonal hypersecretion of 5-hydroxy indole acetic acid (Carcinoid
syndrome). Carcinoids are usually discovered as an incidental finding during
surgery done for other reasons. The appendix tumor arise from subepithelial
endocrine cells with exclusive growth in the lamina propria beneath the
epithelial crypts. Most tumors are found in the tip of the appendix. Simple
appendectomy is curative in most cases. Tumors larger than 2 cm invading
neighboring structures may need right hemicolectomy. Long term follow-up
is imperative.
References
1 - Moartel CL, Weiland LH, Telander RL: Carcinoid Tumor
of the Appendix in the First Two Decades of Life. J Pediatr Surg 25 (10):
1073-1075, 1990
2 - Olney JR, Urdaneta LF, Al-Jurf AS, Jochimsen PR, Shirazi
SS: Carcinoid tumors of the gastrointestinal tract. Am Surg 51(1):37-41,
1985
3 - Moertel CG, Weiland LH, Nagorney DM, Dockerty MB:
Carcinoid tumor of the appendix: treatment and prognosis. N Engl J Med
31;317(27):1699-701, 1987
4 - Jonsson T, Johannsson JH, Hallgrimsson JG: Carcinoid
tumors of the appendix in children younger than 16 years. A retrospective
clinical and pathologic study. Acta Chir Scand 155(2):113-6, 1989
5 - Parkes SE, Muir KR, al Sheyyab M, Cameron AH, Pincott
JR, Raafat F, Mann JR: Carcinoid tumours of the appendix in children 1957-1986:
incidence, treatment and outcome [see comments] Br J Surg 80(4):502-4,
1993
6 - Corpron CA, Black CT, Herzog CE, Sellin RV,
Lally KP, Andrassy RJ: A half century of experience with carcinoid
tumors in children. Am J Surg 170(6):606-8, 1995
Vol 09 No 6 DECEMBER 1997
Gastric Volvulus
Gastric volvulus (GV) is a rare cause of regurgitation, retching or
vomiting in children produced by an abnormal rotation of one part of the
stomach around another. The volvulus can be either organoaxial if
it develops along a line joining the hiatus and pylorus, mesenteroaxial
if it occurs along the greater and lesser curvature axis (most common form
seen in children), or a combination of both. GV may be idiopathic or secondary
to other disorder and may be acute or chronic in nature. Most GV are secondary
to deficient fixation of the gastric ligaments, acute in nature and
associated to another underlying disorder, most commonly a diaphragmatic
defect (eventration or hiatal hernias). Other associations include asplenia,
pre-duodenal portal vein and previous Nissen fundoplication. The main symptoms
of secondary GV are vomiting and respiratory difficulty whereas those of
idiopathic GV are abdominal distension, pain and weight loss with or without
failure to thrive. Borchardt's triad (unproductive vomiting, epigastric
distension, and inability to pass NG tube) is not always present in the
pediatric patients. The diagnosis is suspected with plain abdominal films
(large air-fluid level) and confirmed with upper contrast GI studies. A
nasogastric tube will often relieve the acute situation. Surgical therapy
is mandatory for the simultaneous correction of both the anatomical defects
of gastric fixation and malposition and the diaphragmatic problem. Through
an abdominal approach the stomach is decompressed, the volvulus is untwisted,
the associated anomaly and cruras are repaired, and gastric fixation (gastropexy)
is done. Anterior gastropexy in at least two places will prevent recurrent
volvulus; gastrostomy alone may be followed by recurrence. Laparoscopy
can accomplish this task.
References
1- Youssef SA, Di Lorenzo M, Yazbeck S,
Ducharme JC: [Gastric volvulus in children] Chir Pediatr 28(1):39-42, 1987
2- Cameron AE, Howard ER: Gastric volvulus in childhood.
J Pediatr Surg 22(10):944-7, 1987
3- Honna T, Kamii Y, Tsuchida Y: Idiopathic gastric volvulus
in infancy and childhood. J Pediatr Surg 25(7):707-10, 1990
4- Fung KP, Rubin S, Scott RB: Gastric volvulus complicating
Nissen fundoplication. J Pediatr Surg 25(12):1242-3, 1990
5- Alawadhi A, Chou S, Soucy P: Gastric volvulus--a late
complication of gastrostomy. Can J Surg 34(5):485-6, 1991
6- Miller DL, Pasquale MD, Seneca RP, Hodin E: Gastric
volvulus in the pediatric population. Arch Surg 126(9):1146-9, 1991
7- Cameron BH, Blair GK: Laparoscopic-guided gastropexy
for intermittent gastric volvulus. J Pediatr Surg 28(12):1628-9, 1993
8- McIntyre RC Jr, Bensard DD, Karrer FM, Hall RJ, Lilly
JR: The pediatric diaphragm in acute gastric volvulus. J Am Coll
Surg 178(3):234-8, 1994
9- Andiran F, Tanyel FC, Balkanci F, Hicsonmez A: Acute
abdomen due to gastric volvulus: diagnostic value of a single plain radiograph.
Pediatr Radiol 25 Suppl 1:S240, 1995
10- Georgacopulo P, Pavanello P, Shweiki F, Guerra D:
[Gastric volvulus in childhood] Minerva Pediatr 48(6):275-8, 1996
11- Leitao B, Mota CR, Enes C, Ferreira P, Vieira P, Requeijo
D: Acute gastric volvulus and congenital posterolateral diaphragmatic hernia.
Eur J Pediatr Surg 7(2):106-8, 1997
12- Other Conditions of the Upper Gastrointestinal Tract,
In Marc Rowe ‘Essentials of Pediatric Surgery', Mosby Publisher 1995, pag.
504-505
13- Cameron BH, Vajarvandi V, Blair GK et al. The
intermittent and variable features of gastric volvulus in childhood.
Pediatr Surg Int 10:26-29, 1995
Giant Cystic Meconium Peritonitis
Giant Cystic Meconium Peritonitis (GCMP) is an unusual form of intrauterine
bowel perforation. The bowel perforation persists after birth and the peritoneal
cavity becomes transformed into a huge meconium-filled giant pseudocyst
with a thick membrane lining. The correct diagnosis can be reached either
prenatally or soon after birth with the help of Ultrasonography or simple
abdominal films respectively. Characteristically, a large cyst with dense
calcifications or eggshell appearance is diagnostic. Intrabdominal calcifications
once thought pathognomonic of meconium peritonitis can also be seen in
other conditions such as hamartomas, adrenal hemorrhage or hemangiomas.
Clinically the baby shows severe abdominal distension, bilious vomiting
and failure to pass meconium. Delay in diagnosis may colonize and infect
the pseudocyst creating a septic atmosphere. Associated cystic fibrosis
should always be thought. The etiology of GCMP might be an intrauterine
bowel atresia perforation, volvulus, intussusception or vascular accident.
Remaining bowel is short. Management consists of decortication or removal
of the cyst membrane with re-anastomosis of the GI tract if the surrounding
inflammation permits. Other times temporary enterostomy may be necessary.
The prognosis once dismal has improved in the last twenty years.
References
1- Kolawole TM, Bankole MA, Olurin EO, Familusi JB: Meconium
peritonitis presenting as giant cysts in neonates. Br J Radiol 46(551):964-7,
1973
2- Careskey JM, Grosfeld JL, Weber TR, Malangoni
MA: Giant cystic meconium peritonitis (GCMP): improved management based
on clinical and laboratory observations. J Pediatr Surg 17(5):482-9,
1982
3- Marchildon MB: Meconium Peritonitis and Spontaneous
Gastric Perforation. Clinics in Perinatology. 5(1): 79, 1976
4- Chitnis MR, Natarajan VM, Phadke DM, Golhar KB:
Giant cystic meconium peritonitis. Indian J Pediatr 58(4):554-5, 1991
5- Neuhauser EBD: The roentgen diagnosis of fetal meconium
peritonitis. AJR 51: 421-424, 1944
Congenital Mesoblastic Nephroma
Congenital Mesoblastic Nephroma (CMN), a fetal renal hamartoma, is the
most common renal tumor in neonates. Usually presenting as an asymptomatic
abdominal mass occupying most of the involved kidney, CMN is characterized
by benign clinical behavior, gross appearance similar to uterine fibroids,
and excellent prognosis after removal. Prenatally diagnosed CMN may be
associated with polyhydramnios, low birth weight and premature labor. Large
tumors might benefit from cesarean section. CT Scan shows a solid neoplasm
with intrarenal distortion of the collecting system. Microscopically, typical
CMN is composed of sheets of spindleshaped cells. Atypical CMN (cellular
variant) is a potentially more aggressive tumor composed mainly of primitive
mesenchymal cells, but also usually contains varying numbers of differentiating
fibroblasts and myofibroblasts. Management of CMN consists of simple nephrectomy
with lymph node sampling to detect rare cases simulating malignancy.
References
1- Snyder HM 3d, Lack EE, Chetty-Baktavizian
A, Bauer SB, Colodny AH, Retik AB: Congenital mesoblastic nephroma: relationship
to other renal tumors of infancy. J Urol 126(4):513-6, 1981
2- Gerber A, Gold JH, Bustamante S, Lorch V, Mirza
M: Congenital mesoblastic nephroma. J Pediatr Surg 16(5):758-9, 1981
3- Chan HS, Cheng MY, Mancer K, Payton D, Weitzman
SS, Kotecha P, Daneman A: Congenital mesoblastic nephroma: a clinicoradiologic
study of 17 cases representing the pathologic spectrum of the disease.
J Pediatr 111(1):64-70, 1987
4- Matsumura M, Nishi T, Sasaki Y, Yamada R, Yamamoto
H, Ohhama Y, Tanaka Y, Kurosu F, Amano K: Prenatal diagnosis and treatment
strategy for congenital mesoblastic nephroma. J Pediatr Surg 28(12):1607-9,
1993