PEDIATRIC SURGERY UPDATE ©
VOLUME 24, 2005
Volume 24 No 01 JANUARY 2005
Human Tail
The human tails refers to a extremely rare and benign condition where
a child is born with a persistent vestigial tail-like cutaneous structure
in the lumbosacrococcygeal region. Pathologically, two types of human tails
have been recognized: the true and pseudo human tail. The true human tail
arises from the most distal remnant of the embryonic tail lacking bone,
cartilage, notochord and spinal cord. Contains a central core of mature
fatty tissue divided into small lobules by thin fibrous septa with small
blood vessels and nerve fibers scattered. The true tail arises by retention
of structures found normally in fetal development. It may be as long as
13 cm, can move and contract, and occurs twice as often in males as in
females. The pseudotail is a short, stump-like structure. Spina bifida
(dysraphism) is the most frequent coexisting anomaly in both anatomical
variants (50%). Other associated lesions include tethered cord syndrome,
lipomas, teratomas and gliomas. Investigation of children born with human
tail appendages should include a thorough neurological examination, plain
x-ray films of the lumbo-sacral region and contrast MRI imaging looking
for dysraphism and associated lesions. Management consist of surgical excision
of the vestigial tail and repair of the dysraphism. Long-term follow-up
for possible sequelae after surgery, especially in cases with spinal dysraphism,
is necessary.
References:
1- Belzberg AJ, Myles ST, Trevenen CL: The human tail
and spinal dysraphism. J Pediatr Surg 26(10):1243-5, 1991
2- Dao AH, Netsky MG: Human tails and pseudotails. Hum
Pathol 15(5):449-53, 1984
3- Dubrow TJ, Wackym PA, Lesavoy MA: Detailing the human
tail. Ann Plast Surg 20(4):340-4, 1988
4- Lu FL, Wang PJ, Teng RJ, Yau KI: The human tail. Pediatr
Neurol 19(3):230-3, 1998
5- Gonul E, Izci Y, Onguru O, Timurkaynak E, Seber N:
The human tail associated with intraspinal lipoma: case report. Minim Invasive
Neurosurg 43(4):215-8, 2000
6- Noack F, Reusche E, Gembruch U: Prenatal diagnosis
of 'true tail' with cartilage content? Fetal Diagn Ther 18(4):226-9, 2003
7- Muthukumar N: The "human tail": a rare cause of tethered
cord: a case report. Spine 29(20):E476-8, 2004
Vaginal Prolapse
Vaginal prolapse in the newborn period is a condition reported rarely.
It usually occurs during the first few days of life and presents as a tumor
mass protruding from the vulva. The genital prolapse can include both the
vagina and uterus. Genital prolapse in this age group is associated with
a congenital pelvic neuropathy (myelomeningocele and spina bifida oculta),
lesions affecting the development of the levator muscles, primary support
of the pelvic organs. Though most cases are seen in babies with central
nervous system defects, the condition can also be seen in normal newborn
babies born with intrauterine growth retardation. Initial management consists
of digital manual reduction. Unfortunately, most cases are often resistant
to simple reduction. If the prolapse recurs the wearing of a tiny pessary
made from a rolled and tied one-inch penrose drain is introduced into the
vagina. The pessary can be removed for cleansing and continued to be used
for several weeks until the lax tissues adhere laterally. Should the prolapse
be severe and repetitive, temporary sewing together the posterior half
of the labia minora and majora together can be done. The suture is removed
after they have been in place for two weeks. On a few occasions ventral
suspension of the uterus has been deemed necessary.
References:
1- Johnson A, Unger SW, Rodgers BM: Uterine prolapse
in the neonate. J Pediatr Surg 19(2):210-1, 1984
2- Shuwarger D, Young RL: Management of neonatal genital
prolapse: case reports and historic review. Obstet Gynecol 66(3 Suppl):61S-63S,
1985
3- Carpenter SE, Rock JA: Procidentia in the newborn.
Int J Gynaecol Obstet 25(2):151-3, 1987
4- Bayatpour M, McCann J, Harris T, Phelps H: Neonatal
genital prolapse. Pediatrics 90(3):465-6, 1992
5- Banieghbal B, Fonseca J: Surgical management of uterine
prolapse in an infant. Eur J Pediatr Surg 8(2):119-20, 1998
6- McGlone L, Patole S: Neonatal genital prolapse. J
Paediatr Child Health 40(3):156-7, 2004
Fibroadenoma
Fibroadenoma is a common benign tumor found in the breast
of adolescent girls. It is also considered the most common discrete solid
mass found within the adolescent breast tissue. Most girls harboring a
fibroadenoma have between thirteen and 16 years of age, the tumor is slow
growing, tends to develop in the upper outer quadrant and is more common
in African-American race. Though females may develop breast masses early
in life, the risk of malignancy is extremely low. The tumor is usually
solitary, with an average diameter of two to 4 cm, characterized by rich
cellular stroma and prominent glandular epithelium. At physical exam the
mass feels like a well-circumscribed movable nodule. Fibroadenomas may
be related to an exaggerated local response to the estrogenic effects of
puberty. Mammography, due to the inherent radiation risk and dense fibrous
tissue, is not recommended for routine screening or routine imaging of
breast masses in adolescents. Alternatively, sonography of the breast is
diagnostic on most cases. The tumor looks well-circumscribe, hyperechoic
and homogenous on ultrasound. Ten percent of cases harbor a giant juvenile
fibroadenoma, a large lesion that distorts the normal breast architecture
eroding through the skin and areolar complex. Management of fibroadenoma
could be observation or cryoablation. Growing, symptomatic or anxiety-producing
masses should be managed with excision through a periareolar incision to
preserve cosmesis.
References:
1- Simmons PS: Diagnostic considerations in breast disorders of children
and adolescents. Obstet Gynecol Clin North Am 19(1):91-102, 1992
2- West KW, Rescorla FJ, Scherer LR 3rd, Grosfeld JL: Diagnosis and
treatment of symptomatic breast masses in the pediatric population.
J Pediatr Surg 30(2):182-6, 1995
3- Dehner LP, Hill DA, Deschryver K: Pathology of the breast in children,
adolescents, and young adults. Semin Diagn Pathol 16(3):235-47, 1999
4- Elsheikh A, Keramopoulos A, Lazaris D, Ambela C, Louvrou N, Michalas
S: Breast tumors during adolescence. Eur J Gynaecol Oncol 21(4):408-10,
2000
5- Onuigbo W: Breast fibroadenoma in teenage females. Turk J Pediatr
45(4):326-8, 2003
6- Kaufman CS, Bachman B, Littrup PJ, Freeman-Gibb LA, White M, Carolin
K, Francescatti D, Stocks LH, Smith JS, Henry CA, Bailey L, Harness JK,
Simmons R: Cryoablation treatment of benign breast lesions with 12-month
follow-up. Am J Surg 188(4):340-8, 2004
Volume 24 No 02 FEBRUARY 2005
Adrenal Incidentaloma
With the advent of potent imaging studies during the eighties a group
of adult patients was found with incidentally discovered adrenal masses,
hence the term coined of adrenal Incidentaloma. At the time, masses below
a size of three centimeters were observed with follow-up studies for spontaneous
regression. Most cases resulted in benign non-functioning adenomas which
disappeared with time. The situation in children is different. A mass identified
in the adrenal gland is cause for concern. In infancy and childhood the
most common adrenal mass is the neuroblastoma, a malignant neural crest
tumor. Initial diagnosis of an adrenal mass in a child is made with Ultrasound,
which is also used to document regression of uncomplicated neonatal adrenal
hemorrhage. Further radiological assessment of an adrenal incidentaloma
in a child should include CT-Scan and MRI. MRI can accurately distinguish
adrenal adenomas from adenocarcinoma, pheochromocytoma and neuroblastomas.
Endocrine tests evaluating pituitary-adrenal function (urinary excretion
of 17-hydroxycorticosteroids, 17-ketosteroids and catecholamines, plasma
concentrations of ACTH, cortisol, DHEAS, androstenedione and testosterone,
dexamethasone suppression test and corticotrophin-releasing hormone stimulation
test) should be part of the work-up. Should biochemical studies revealed
no hormonal related disease (Cushing, hyperaldosteronism, pheochromocytoma,
etc.) a histological diagnosis should be obtained by either CT-guided fine
needle biopsy or surgical resection. In the event of no diagnosis, adrenal
tumor resection should be done.
References:
1- Caplan RH, Kisken WA, Huiras CM: Incidentally discovered
adrenal masses. Minn Med 74(8):23-6, 1991
2- Kasperlik-Zeluska AA, Roslonowska E, Slowinska-Srzednicka
J, Migdalska B, Jeske W, Makowska A, Snochowska H: Incidentally discovered
adrenal mass (incidentaloma): investigation and management of 208 patients.
Clin Endocrinol (Oxf) 46(1):29-37, 1997
3- Angeli A, Osella G, Ali A, Terzolo M: Adrenal incidentaloma:
an overview of clinical and epidemiological data from the National Italian
Study Group. Horm Res 47(4-6):279-83, 1997
4- Agrons GA, Lonergan GJ, Dickey GE, Perez-Monte JE:
Adrenocortical neoplasms in children: radiologic-pathologic correlation.
Radiographics 19(4):989-1008, 1999
5- Masiakos PT, Gerstle JT, Cheang T, Viero S, Kim PC,
Wales P: Is surgery necessary for incidentally discovered adrenal masses
in children? J Pediatr Surg 39(5):754-8, 2004
Pneumomediastinum
Spontaneous pneumomediastinum is a self-limited condition rarely seen
in infants and children. Alveolar rupture secondary to increased pressure
(such as effected by barotrauma) or over distension leads to air dissection
along perivascular and peribronchial tissues up to the hilum of the mediastinum
and the soft tissue of the neck (Macklin effect). Clinically the child
develops dyspnea, sore throat, sudden chest pain radiating to the back
or neck, subcutaneous emphysema and Hamman‘s sign. Rarely, the massive
subcutaneous emphysema can cause respiratory compromise requiring emergency
releasing incisions. Some of the causes of spontaneous pneumomediastinum
include asthma (most common cause), mechanical ventilation, intubation,
Valsalva, foreign body, trauma, esophageal perforation and drug inhalation.
Diagnosis is established with simple chest films (PA and lateral are needed).
Management is conservative (bed rest and analgesics). A few cases need
mechanical or high frequency oscillatory ventilation. Most children clinically
improve during the next three days with resolution of the pneumomediastinum
by seven days.
References:
1- Burton EM, Riggs W Jr, Kaufman RA, Houston CS: Pneumomediastinum
caused by foreign body aspiration in children. Pediatr Radiol 20(1-2):45-7,
1989
2- Taylor J, Dibbins A, Sobel DB: Neonatal pneumomediastinum:
indications for, and complication of, treatment. Crit Care Med 21(2):296-8,
1993
3- McHugh TP: Pneumomediastinum following penetrating
oral trauma. Pediatr Emerg Care 13(3):211-3, 1997
4- Miyahara K, Ichihara T, Watanabe T: Successful use
of high frequency oscillatory ventilation for pneumomediastinum. Ann Thorac
Cardiovasc Surg 5(1):49-51, 1999
5- Damore DT, Dayan PS: Medical causes of pneumomediastinum
in children. Clin Pediatr (Phila) 40(2):87-91, 2001
6- Chapdelaine J, Beaunoyer M, Daigneault P, Berube D,
Butter A, Ouimet A, St-Vil D: Spontaneous pneumomediastinum: are we overinvestigating?
J Pediatr Surg 39(5):681-4, 2004
Adhesive Bond
Adhesive bond refers to a group of cyanoacrylate tissue glue adhesive
used as an alternative for skin wound closure, instead of suture. The most
extensive use of adhesive bond is for the repair of simple traumatic skin
laceration in the emergency room. Advantages of using adhesive bond include
rapid achievement of tissue union, need to use or remove suture is eliminated,
application is less painful than suturing, more efficient use of physician
time, and cyanoacrylates have a significant antimicrobial effect against
gram-positive organisms. Cosmetic appearance is similar to that obtained
after suture skin closure. Early wound deshicence is the most common complication
of use of tissue adhesive closure, seen in up to 5% of cases. The adhesive
bond is a sterile sealed unit terminally sterilized by gamma radiation.
When compared, suturing is cheaper than using the adhesive bond. The use
of adhesive bonds is an ideal alternative to conventional suturing for
the cutaneous closure of low tension lacerations in children with a long-term
cosmetic outcome comparable to conventional suturing. Parent satisfaction
is greater with the use of adhesive bond.
References:
1- Bruns TB, Simon HK, McLario DJ, Sullivan KM, Wood
RJ, Anand KJ: Laceration repair using a tissue adhesive in a children's
emergency department. Pediatrics 98(4 Pt 1):673-5, 1996
2- Simon HK, McLario DJ, Bruns TB, Zempsky WT, Wood RJ,
Sullivan KM: Long-term appearance of lacerations repaired using a tissue
adhesive. Pediatrics 99(2):193-5, 1997
3- Singer AJ, Hollander JE, Valentine SM, Turque TW,
McCuskey CF, Quinn JV: Prospective, randomized, controlled trial of tissue
adhesive (2-octylcyanoacrylate) vs standard wound closure techniques for
laceration repair. Stony Brook Octylcyanoacrylate Study Group. Acad Emerg
Med 5(2):94-9, 1998
4- Barnett P, Jarman FC, Goodge J, Silk G, Aickin R:
Randomized trial of histoacryl blue tissue adhesive glue versus suturing
in the repair of paediatric lacerations. J Paediatr Child Health 34(6):548-50,
1998
5- Amiel GE, Sukhotnik I, Kawar B, Siplovich L: Use of
N-butyl-2-cyanoacrylate in elective surgical incisions--long term outcomes.
Am Coll Surg 189(1):21-5, 1999
6- Ong CC, Jacobsen AS, Joseph VT: Comparing wound closure
using tissue glue versus subcuticular suture for pediatric surgical incisions:
a prospective, randomized trial. Pediatr Surg Int 18(5-6):553-5, 2002
7- van den Ende ED, Vriens PWHE, Allema JH, Breslau PJ:
Adhesive bonds or percutaneous absorbable suture for closure of surgical
wounds in children. Results of a prospective randomized trial. J Pediatr
Surg 39(8): 1249-1251, 2004
Volume 24 No 03 MARCH 2005
STEP
Short bowel syndrome is a very serious gastrointestinal disorder characterized
by the absence of significant length of bowel capable of normal digestion
and absorption. It is estimated that more than 70% of small bowel length
must be lost to develop a short bowel syndrome. The three most common causes
of short bowel syndrome in the pediatric age are necrotizing enterocolitis,
midgut volvulus and gastroschisis. Intestinal adaptation can occur when
the neonate is left with more than thirty (30) centimeters of small bowel
with an intact ileo-cecal valve. Though the prospect of bowel transplant
continues to develop better forms of avoiding acute rejection, still the
median survival after transplantation is short (mean of 15 months). Recently,
a novel experimental procedure, has attained the attention of surgeons
managing this devastating disease complication. The operation is termed
serial transverse enteroplasty (STEP) procedure. After short bowel ensues
the process of adaptation includes mucosal hyperplasia and bowel dilatation.
The STEP procedure is based on the anatomic principle that the blood supply
to the bowel comes from the mesenteric border traversing along the
perpendicular long axis of the bowel. Multiple stapler lines are placed
perpendicularly alternating the direction of the stapler creating a channel
of bowel smaller in diameter and longer in length than the original bowel.
Advantages of STEP: easy to do, no anastomosis needed, does not result
in intestinal obstruction, mesentery is not jeopardized, the length is
almost double, the tapering is customizable, and can be performed in sequence
after a successful Bianchi procedure. STEP could become the lengthening
bowel procedure for short bowel syndrome.
References:
1- Kim HB, Fauza D, Garza J, Oh JT, Nurko S, Jaksic T:
Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure.
J Pediatr Surg 38(3):425-9, 2003
2- Kim HB, Lee PW, Garza J, Duggan C, Fauza D, Jaksic
T: erial transverse enteroplasty for short bowel syndrome: a case report.
J Pediatr Surg 38(6):881-5, 2003.
3- Tannuri U: Comment on: J Pediatr Surg 38(6):881-5,
2003. J Pediatr Surg 38(3):425-9, 2003. Serial transverse enteroplasty
(STEP): a novel bowel lengthening procedure, and serial transverse enteroplasty
for short bowel syndrome. J Pediatr Surg 38(12):1845, 2003; author reply
1845-6
Peritoneal Gliomatosis
Peritoneal Gliomatosis (PG) is a rare complication of solid mature or
immature ovarian teratomas. Occurs with rupture of the tumor capsule causing
multiple mature glial implantation (neural tumor tissue) on the parietal,
visceral peritoneum, omentum or space of Douglas. GP is found in childhood,
adolescence, as well as in young women. Peritoneal gliomatosis can be considered
to be implantation metastases. The nodules may vary in size, but are usually
around 3 mm in diameter. Peritoneal gliomatosis is a benign condition.
Mean age at time of diagnosis is eleven years. Most cases described are
occurs with mature ovarian teratoma. The prognosis depends chiefly on the
degree of maturity of the implants. In mature GP, usually no additional
chemotherapy is necessary; in immature GP, chemotherapy can induce maturation
of the implants. The fate of the glial tissue is still not clear, but the
nodule can persist without detectable changes after more than fifteen years
of the initial operation, can undergo fibrosis and disappear, or may transform
into malignant glial or teratomatous tissue. Close follow-up of these patients
is mandatory. The few cases originating from immature teratoma can developed
into malignant transformation. Gliomatosis peritonei in extremely rare
circunstances has been reported due to transport of glial tissue from the
cerebrospinal fluid into the peritoneal cavity via a ventriculo-peritoneal
shunt.
References:
1- Truong LD, Jurco S 3rd, McGavran MH: Gliomatosis peritonei.
Report of two cases and review of literature. Am J Surg Pathol 6(5):443-9,
1982
2- El Shafie M, Furay RW, Chablani LV: Ovarian teratoma
with peritoneal and lymph node metastases of mature glial tissue: a benign
condition. J Surg Oncol 27(1):18-22, 1984
3- Harms D, Janig U, Gobel U: Gliomatosis peritonei in
childhood and adolescence. Clinicopathological study of 13 cases including
immunohistochemical findings. Pathol Res Pract 184(4):422-30, 1989
4- Chaung JH, Chen L: Ovarian teratoma with gliomatosis
peritonei. J Pediatr Surg 27(5):662-4, 1992
5- Dadmanesh F, Miller DM, Swenerton KD, Clement PB:
Gliomatosis peritonei with malignant transformation. Mod Pathol 10(6):597-601,
1997
6- Hill DA, Dehner LP, White FV, Langer JC: Gliomatosis
peritonei as a complication of a ventriculoperitoneal shunt: case report
and review of the literature. J Pediatr Surg 35(3):497-9, 2000
Nevus Sebaceous
Nevus sebaceous of Jadassohn is a congenital hamartomatous skin lesion
occurring mostly on the scalp, face and neck. At physical exam the lesion
is well circunscribe with a yellow-orange smooth plaque appearance. Borders
become irregular with puberty and hormonal changes. Malignant transformation
has been reported in 10% of cases occurring only after puberty. The nevus
sebaceous can transformed into a basal or squamous cell carcinoma. The
lesion will not go away spontaneously. It is uncommon for malignancy to
develop in a sebaceous nevus before puberty. Due to the risk of malignant
transformation and the difficulty in follow-up of this children with time,
early complete excision for prophylaxis is recommended in cases of nevus
sebaceous. Excision must encompassed clean deep and lateral margins of
resection to be effective. Large lesion will benefit from use of tissue
expanders.
References:
1- Hagan WE: Nevus sebaceus of Jadassohn: the head and
neck manifestations. Laryngoscope 97(8 Pt 1):909-14, 1987
2- Goldstein GD, Whitaker DC, Argenyi ZB, Bardach J:
Basal cell carcinoma arising in a sebaceous nevus during childhood. J Am
Acad Dermatol 18(2 Pt 2):429-30, 1988
3- Weng CJ, Tsai YC, Chen TJ: Jadassohn's nevus sebaceous
of the head and face. Ann Plast Surg 25(2):100-2, 1990
4- Beer GM, Widder W, Cierpka K, Kompatscher P, Meyer
VE: Malignant tumors associated with nevus sebaceous: therapeutic consequences.
Aesthetic Plast Surg 23(3):224-7, 1999
5- Dunkin CS, Abouzeid M, Sarangapani K: Malignant transformation
in congenital sebaceous naevi in childhood. J R Coll Surg Edinb 46(5):303-6,
2001
6- Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick
SJ, Thaller S: Should nevus sebaceus of Jadassohn in children be excised?
A study of 757 cases, and literature review. J Craniofac Surg 14(5):658-60,
2003
Volume 24 No 04 APRIL 2005
Thalassemia
Thalassemia, also known as Mediterranean or Cooley's anemia, is a genetically
determined heterogeneous group of hemoglobinopathies affecting the
synthesis of hemoglobin alpha and/or beta-globin chains. This autosomal
dominant illness can occur in major (homozygous), intermediate or minor
(heterozygous) varieties. A defect in the synthesis of hemoglobin subunits
results in the accumulation of intracellular particles in the red blood
cell contributing to early destruction by the spleen. The anemia of thalassemia
is an inborn error of metabolism causing large amounts of fetal hemoglobin
to be produced, instead of adult hemoglobin. With the block in iron metabolism,
large deposits of iron occur throughout the body. Heterozygous (minor)
thalassemia child is asymptomatic, while the major or intermediate thalassemia
child develops severe chronic anemia, jaundice, hepatosplenomegaly, frontal
bossing and growth retardation. With continued hemolysis come gallbladder
pigment stones (25%), hypersplenism and splenic infarcts. Splenectomy is
palliative and indicated for the management of chronically transfused patients
in order to increase red blood cell survival and decrease transfusion requirements.
Cardiac iron overload is the most frequent cause of death from chronic
transfusion therapy. After splenectomy, a high incidence of sepsis and
portal vein thrombosis (hypercoagulable state) has been reported. Partial
splenectomy reduces transfusion requirements for a limited amount of time
due to regrowth of the splenic remnant.
References:
1- Cohen AR, Galanello R, Pennell DJ, Cunningham MJ,
Vichinsky E: Thalassemia. Hematology (Am Soc Hematol Educ Program). 14-34,
2004
2- Fujita F, Lyass S, Otsuka K, Giordano L, Rosenbaum
DL, Khalili TM, Phillips EH: Portal vein thrombosis following splenectomy:
identification of risk factors. Am Surg 69(11):951-6, 2003
3- Al-Salem AH, Nasserulla Z: Splenectomy for children
with thalassemia. Int Surg 87(4):269-73, 2002
4- 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
5- Laopodis V, Kritikos E, Rizzoti L, Stefanidis P, Klonaris
P, Tzardis P: Laparoscopic splenectomy in beta-thalassemia major patients.
Advantages and disadvantages. Surg Endosc 12(7):944-7, 1998
6- Skarsgard E, Doski J, Jaksic T, Wesson D, Shandling
B, Ein S, Babyn P, Heiss K, Hu X: Thrombosis of the portal venous system
after splenectomy for pediatric hematologic disease. J Pediatr Surg 28(9):1109-12,
1993
Latex Allergy
Allergy to natural rubber latex is an increasing common condition in
both children and health care workers. Almost 20 to 40% of children with
spina bifida are allergic to latex. Other affected persons are health care
workers, latex industry workers, immune compromised individuals, children
with bladder exstrophy, anorectal anomalies, and persons with positive
risk factors such as multiple surgical procedures. Chemical additives in
latex gloves can cause an irritant or allergic contact dermatitis. Latex
proteins are responsible for most of the immediate IgE-mediated hypersensitivity
allergic reactions. Symptoms range from rhinitis, conjunctivitis and urticaria
to intraoperative anaphylaxis and death. Skin prick testing with natural
rubber latex and glove tests are safe diagnostic procedure. In children
with spina bifida significant and independent risk factors identified for
latex sensitization are multiple interventions and higher levels of total
serum IgE. The only currently available treatment is complete avoidance
of latex. For children with known history of latex allergy premedication
with antihistamines and steroids is in order.
References:
1- Birmingham PK, Suresh S: Latex allergy in children:
diagnosis and management. Indian J Pediatr 66(5):717-24, 1999
2- Degenhardt P, Golla S, Wahn F, Niggemann B: Latex
allergy in pediatric surgery is dependent on repeated operations in the
first year of life. J Pediatr Surg 36(10):1535-9, 2001
3- Zucker-Pinchoff B, Stadtmauer GJ: Latex allergy. Mt
Sinai J Med 69(1-2):88-95, 2002
4- Cremer R, Kleine-Diepenbruck U, Hering F, Holschneider
AM: Reduction of latex sensitization in spina bifida patients by a primary
prophylaxis programme (five years experience). Eur J Pediatr Surg 12 Suppl
1:S19-21, 2002
5- Sparta G, Kemper MJ, Gerber AC, Goetschel P, Neuhaus
TJ: Latex allergy in children with urological malformation and chronic
renal failure. J Urol 171(4):1647-9, 2004
6- Kimata H: Latex allergy in infants younger than 1
year. Clin Exp Allergy 34(12):1910-5, 2004
Hermansky-Pudlak Colitis
Hermansky-Pudlak syndrome is a triad of tyrosine-positive oculocutaneous
albinism, platelet dysfunction, and the deposition of an abnormal ceroid-like
pigment in the tissues. Ceroid lipofuscin is accumulated in lysosomes organelles.
Children with Hermansky-Pudlak syndrome can suffer from pulmonary fibrosis,
renal failure, and cardiomyopathy besides other complications. All identified
affected patients in the northwest Puerto Rico are homozygous for a 16-bp
duplication in exon 15 of a recently cloned gene. This duplication is associated
with a broad range of pigmentation and an increased risk of restrictive
lung disease in adults. Several families with the syndrome rarely suffer
from granulomatous colitis, a unique type of inflammatory bowel disease
with clinical features suggestive of idiopathic ulcerative colitis and
pathologic features suggestive of Crohn's disease with perineal and perirectal
involvement. Diagnosis is established with endoscopy. Management
is similar to cases with inflammatory bowel disease directed toward pathogenetic
mechanisms. Corticosteroids, sulphasalazine and the new salicylates, the
immunosuppressants azathioprine, 6-MP and, more recently, cyclosporin and
metronidazole have become the accepted and standard forms of treatment.
Some cases are refractory to medical treatment needing segmental resection
of the affected bowel.
References:
1- Sherman A, Genuth L, Hazzi CG, Balthazar EJ, Schinella
RA: Perirectal abscess in the Hermansky-Pudlak syndrome. Am J Gastroenterol.
84(5):552-6, 1989
2- Witkop CJ, Nunez Babcock M, Rao GH, Gaudier F, Summers
CG, Shanahan F, Harmon KR, Townsend D, Sedano HO, King RA, et al: Albinism
and Hermansky-Pudlak syndrome in Puerto Rico. Bol Asoc Med P R. 82(8):333-9,
1990
3- Schinella RA, Greco MA, Cobert BL, Denmark LW, Cox
RP: Hermansky-Pudlak syndrome with granulomatous colitis. Ann Intern Med.
92(1):20-3, 1980
4- Gahl WA, Brantly M, Kaiser-Kupfer MI, Iwata F, Hazelwood
S, Shotelersuk V, Duffy LF, Kuehl EM, Troendle J, Bernardini I: Genetic
defects and clinical characteristics of patients with a form of
oculocutaneous albinism (Hermansky-Pudlak syndrome).
N Engl J Med. 338(18):1258-64, 1998
5- Sandberg-Gertzen H, Eid R, Jarnerot G: Hermansky-Pudlak
syndrome with colitis and pulmonary fibrosis. Scand J Gastroenterol. 34(10):1055-6,
1999
6- Mahadeo R, Markowitz J, Fisher S, Daum F: Hermansky-Pudlak
syndrome with granulomatous colitis in children. J Pediatr. 118(6):904-6,
1991
Volume 24 No 05 MAY 2005
Cardiac Tamponade
Vascular access using central lines is essential in managing acutely
ill, chronically disease and cancer pediatric patients. Vascular access
can be obtained either through the neck or groin using the external jugular,
internal jugular, subclavian or saphenous vein. Potentially lethal complications
of central venous catheter placement consist of arrhythmias, pneumothorax,
hemothorax, vascula injury and cardiac tamponade. Cardiac tamponade is
some very rare complication of venous access. Tamponade may be an acute
or late complication and is usually associated with the effusion of intravenous
fluid into the pericardium. Most cases occur acutely during intraoperative
placement. In either setting symptoms of tamponade includes chest pain,
hypotension, increase central venous pressure, low oxygen saturation, bradycardia
and cardiac arrest. The perforation can occur in the superior vena cava,
atrium, ventricle or pulmonary artery. Immediate recognition of pericardial
tamponade followed by pericardiocentesis are crucial factors in survival.
Contrast infusion is valuable in evaluating this complication of central
line placement. In children, most central venous access should be performed
in the operating room whenever possible. After insertion, position of the
catheter in the central venous circulation should be documented by radiographic
means on a hard-film copy. Any deviation in the child's hemodynamic stability
during placement or afterward should herald the coming of a lethal complication
and managed accordingly.
References:
1- Hansbrough JF, Narrod JA, Stiegman GV: Cardiac perforation
and tamponade from a malpositioned subclavian dialysis catheter. Nephron
32(4):363-4, 1982
2- Hunt LB, Olshansky B, Hiratzka LF: Cardiac tamponade
caused by pulmonary artery perforation after central venous catheterization.
JPEN J Parenter Enteral Nutr 8(6):711-3, 1984
3- Krauss D, Schmidt GA: Cardiac tamponade and contralateral
hemothorax after subclavian vein
catheterization. Chest 99(2):517-8, 1991
4- Bagwell CE, Salzberg AM, Sonnino RE, Haynes JH: Potentially
lethal complications of central venous catheter placement. J Pediatr Surg
35(5):709-13, 2000
5- Shields LB, Hunsaker DM, Hunsaker JC 3rd: Iatrogenic
catheter-related cardiac tamponade: a case report of fatal hydropericardium
following subcutaneous implantation of a chemotherapeutic injection port.
J Forensic Sci 48(2):414-8, 2003
Accessory Splenic Torsion
It is estimated that 10% of the general population carries an accessory
spleen. Accessory spleens are situated on the hilum of the spleen,
splenic artery, pancreas, splenocolic ligament, greater omentum, mesenterium,
adnexal region and scrotum. Trauma, torsion and hematologic hemolytic conditions
affect an accessory spleen. A careful search should be made for accessory
spleens, as they should be removed at the time of primary splenectomy to
avoid a second operation later in life. Torsion with infarction of an accessory
spleen must be considered as a rare cause of acute abdominal pain in childhood.
Accessory splenic torsion causes acute diffuse or localized (left upper
quadrant) abdominal pain sometimes undistinguishable from that caused by
acute appendicitis or intussusception. Most affected children develop an
intraperitoneal inflammatory mass. Preoperative diagnostic imaging
is unable to point to the diagnosis. Ultrasound shows a round, hypoechoic,
solid mass. CT Scan demonstrates a low-density mass with peripheral enhancement
after intravenous contrast medium. MRI can be helpful in the differential
diagnosis of infarction by suggesting hemorrhagic necrosis on the T2-weighted
images. Diagnosis is corroborated during laparoscopy or laparotomy. Accessory
splenectomy is curative.
References:
1- Broker FH, Khettry J, Filler RM, Treves S: Splenic
torsion and accessory spleen: a scintigraphic demonstration. J Pediatr
Surg 10(6):913-5, 1975
2- Appel MF, Bart JB: The surgical and hematologic significance
of accessory spleens. Surg Gynecol Obstet 143(2):191-2, 1976
3- Seo T, Ito T, Watanabe Y, Umeda T: Torsion of an accessory
spleen presenting as an acute abdomen with an inflammatory mass. US, CT,
and MRI findings. Pediatr Radiol 24(7):532-4, 1994
4- Chateil JF, Arboucalot F, Perel Y, Roy D, Vergnes
P, Diard F: Acute torsion of an accessory spleen. J Radiol 77(3):209-11,
1996
5- Valls C, Mones L, Guma A, Lopez-Calonge E: Torsion
of a wandering accessory spleen: CT findings. Abdom Imaging 23(2):194-5,
1998
6- Perez Fontan FJ, Soler R, Santos M, Facio I: Accessory
spleen torsion: US, CT and MR findings. Eur Radiol 11(3):509-12, 2001
7- Wacha M, Danis J, Wayand W: Laparoscopic resection
of an accessory spleen in a patient with chronic lower abdominal pain.
Surg Endosc 16(8):1242-3. Epub 2002 May 23, 2002
Sinus Histiocytosis
Sinus histiocytosis with massive lymphadenopathy (SHML), also known
as Rosai-Dorfman disease, is a benign condition that occurs mainly in children,
characterized by a protracted course with painless bilateral enlargement
of the cervical lymph nodes, fever, leucocytosis, mild anemia, raised erythrocyte
sedimentation rate and hypergammaglobulinemia. Extranodal involvement in
SHML occurs in the skin, upper respiratory tract, and bone. Diagnosis is
confirmed with histologic evidence of involved lymph nodes characterized
by an exuberant intrasinusoidal histiocytic proliferation. SHML can be
associated with retropharyngeal obstructive symptoms, mediastinal enlargement
and orbital enlargement. Prognosis has been found to correlate both with
the number of nodal groups and number of extranodal system involvement.
Children with SHML may have a variably expressed immunodeficiency that
predisposes them to recurrent infections. In general, management is expectant
waiting for spontaneous regression. Cytotoxic chemotherapeutic agents have
been utilized for life-threatening complications of SHML.
References:
1- Suarez CR, Zeller WP, Silberman S, Rust G, Messmore
H: Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy.
Am J Pediatr Hematol Oncol 5(3):235-41, 1983
2- Foucar E, Rosai J, Dorfman R: Sinus histiocytosis
with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity.
Semin Diagn Pathol 7(1):19-73, 1990
3- Maennle DL, Grierson HL, Gnarra DG, Weisenburger DD:
Sinus histiocytosis with massive lymphadenopathy: a spectrum of disease
associated with immune dysfunction. Pediatr Pathol 11(3):399-412, 1991
4- Brau RH, Sosa IJ, Marcial-Seoane MA: Sinus histiocytosis
with massive lymphadenopathy (Rosai-Dorfman disease) and extranodal involvement
of the orbit. P R Health Sci J 14(2):145-9, 1995
5- Moore SW, Schneider JW, Schaaf HS: Diagnostic aspects
of cervical lymphadenopathy in children in the developing world: a study
of 1,877 surgical specimens. Pediatr Surg Int 19(4):240-4. Epub 2003 Apr
17, 2003
Volume 24 No 06 JUNE 2005
Oral Gastrografin
Gastrografin is a valuable contrast material used by radiologists for
studies of the gastrointestinal tract. Is a water-soluble highly osmolar
material (1900 mOsm) composed of sodium diatrizoate, meglumine amidotrizoate
and a wetting agent (polysorbate 80). Gastrografin has a therapeutic value
in cases of partial mechanical bowel obstruction, postoperative ileus,
dissolution of barium-impacted ileus and stomal dysfunction after gastric
resection. Possible mechanisms of action of Gastrografin come from its
hyperosmolality promoting proximal bowel distension, increasing the gradient
pressure across the obstructing segments, decreasing bowel edema and enhancing
bowel motility. A dose of 100 ml of Gastrografin in adults, or 20-50
ml in children is injected via a nasogastric tube and supine plain abdominal
radiographs are taken at 30 min and four hrs after administration. At this
time if contrast passes to the colon a non-operative course is followed.
With clear-cut off sign or absence of contrast material in the cecum in
the next 24 hours a diagnosis of unrelieved mechanical obstruction is entertained
and surgery probably needed. For absolute diagnosis of successful resolution
the abdominal pain should disappear, the abdomen should appear flat and
soft, the nasogastric output normalized and the child should have another
spontaneous bowel action. Omnipaque, an isosmolar water soluble agent
retains its radiographic density in the small bowel better than Gastrografin
being a better alternative than Gastrografin in follow-through examinations
of intestinal obstruction.
References:
1- Joyce WP, Delaney PV, Gorey TF, Fitzpatrick JM: The
value of water-soluble contrast radiology in the management of acute small
bowel obstruction. Ann R Coll Surg Engl 74(6):422-5, 1992
2- Assalia A, Schein M, Kopelman D, Hirshberg A, Hashmonai
M: Therapeutic effect of oral Gastrografin in adhesive, partial small-bowel
obstruction: a prospective randomized trial. Surgery 115(4):433-7, 1994
3- Chung CC, Meng WC, Yu SC, Leung KL, Lau WY, Li AK:
A prospective study on the use of water-soluble contrast follow-through
radiology in the management of small bowel obstruction. Aust N Z J Surg
66(9):598-601, 1996
4- Chen SC, Lin FY, Lee PH, Yu SC, Wang SM, Chang KJ:
Water-soluble contrast study predicts the need for early surgery in adhesive
small bowel obstruction. Br J Surg 85(12):1692-4, 1998
5- Blackmon S, Lucius C, Wilson JP, Duncan T, Wilson
R, Mason EM, Ramshaw B: The use of water-soluble contrast in evaluating
clinically equivocal small bowel obstruction. Am Surg 66(3):238-42, 2000
6- Choi HK, Chu KW, Law WL: Therapeutic value of gastrografin
in adhesive small bowel obstruction after unsuccessful conservative treatment:
a prospective randomized trial. Ann Surg 236(1):1-6, 2002
7- Biondo S, Pares D, Mora L, Marti Rague J, Kreisler
E, Jaurrieta E: Randomized clinical study of Gastrografin administration
in patients with adhesive small bowel obstruction. Br J Surg 90(5):542-6,
2003
8- Roadley G, Cranshaw I, Young M, Hill AG: Role of Gastrografin
in assigning patients to a non-operative course in adhesive small bowel
obstruction. ANZ J Surg 74(10):830-2, 2004
Ectopic Testis
Whenever a child is born with an empty scrotum, the physical examination
should include a diligent palpable search for the undescended testis in
the inguinal, femoral, perineal or medial thigh areas. Testes palpable
in areas away from the normal descent from the retroperitoneum to the scrotum
are termed ectopic testis. An ectopic testis is caused by mislocation of
the ipsilateral genito-femoral nerve controlled stimulation causing the
gubernaculum to migrate to the wrong site because the chemotactic signal
is arising from this wrong place. Testes palpable in the inguinal canal
or found intra-abdominally are termed undescended. Compared with undescended
testes, ectopic testes are extremely rare found most commonly in the perineal
ipsilateral area. Other sites include the femoral canal, suprapubic region
(at base of the penis), medial thigh, preperitoneal, umbilical, contralateral
scrotum or associated with gastroschisis. The perineal testis is particularly
subject to trauma. Management is orchiopexy as soon as the diagnosis is
established. The most effective route of approach for repair is inguinal
allowing replacement of the testis into the corresponding hemiscrotum without
difficulty. Other surgeons use a low scrotal approach due to the low incidence
of concomitant hernia. Because of the histopathologic features involved,
prognosis is better than that associated with cryptorchidism.
References:
1- Murphy DM, Butler MR: Preperitoneal ectopic testis:
a case report. J Pediatr Surg 20(1):93-4, 1985
2- Gauderer MW: Gastroschisis and extraabdominal ectopic
testis: simultaneous repair. J Pediatr Surg 22(7):657-9, 1987
3- Maidenberg M: A case of an ectopic testis in the perineum.
Prog Urol 3(2):268-71, 1993
4- Celayir AC, Sander S, Elicevik M: Timing of surgery
in perineal ectopic testes: analysis of 16 cases. Pediatr Surg Int 17(2-3):167-8,
2001
5- Parsons JK, Ferrer F, Docimo SG: The low scrotal approach
to the ectopic or ascended testicle: prevalence of a patent processus vaginalis.
J Urol 169(5):1832-3, 2003
6- Hutson JM, Hasthorpe S: Testicular descent and cryptorchidism:
the state of the art in 2004. J Pediatr Surg 40(2) :297-302, 2005
Gastrointestinal Stromal Tumor
Gastrointestinal stromal tumor (GIST), previously known as gastric leiomyoblastoma,
is an uncommon nonepithelial mesenchymal kit-positive (CD117 antigen) tumor
of the gastrointestinal tract. GIST are the most common mesenchymal tumors
of the gastrointestinal tract. Cell of origin is the intersticial cell
of Cajal. The frequency of malignant GIST is 20-30% of the frequency of
all soft-tissue sarcomas, but small benign tumors often found incidentally
during unrelated surgery or autopsy are more common. GIST occurs in children,
young adults or on a familial basis. Most involved children are girls with
symptoms of abdominal pain and anemia. CT-Scan or MRI suggests the diagnosis.
Most GIST appears in the stomach (submucosal mass), followed by the intestine
and rarely the colon. Metastasis occurs to the liver. Large tumors (> 5
cm) with high mitotic activity are associated with bad prognosis. Management
consist of complete surgical resection with prophylactic omentectomy to
reduce the recurrence of GIST. GIST have lower survival rate and more resistance
to chemotherapy.
References:
1- Oguzkurt P, Akcoren Z, Senocak ME, Caglar M, Buyukpamukcu
N: A huge gastric stromal tumor in a 13-year-old girl. Turk J Pediatr 44(1):65-8,
2002
2- Miettinen M, Majidi M, Lasota J: Pathology and
diagnostic criteria of gastrointestinal stromal tumors (GISTs): a review.
Eur J Cancer 38 Suppl 5:S39-51, 2002
3- Durham MM, Gow KW, Shehata BM, Katzenstein HM, Lorenzo
RL, Ricketts RR: Gastrointestinal stromal tumors arising from the stomach:
a report of three children. J Pediatr Surg 39(10):1495-9, 2004
4- Geramizadeh B, Bahador A, Ganjei-Azar P, Asadi A:
Neonatal gastrointestinal stromal tumor. Report of a case and review of
literature. J Pediatr Surg 40(3):572-4, 2005
5-Prakash S, Sarran L, Socci N, Dematteo RP, Eisenstat
J, Greco AM, Maki RG, Wexler LH, Laquaglia MP, Besmer P, Antonescu CR:
Gastrointestinal Stromal Tumors in Children and Young Adults: A Clinicopathologic,
Molecular, and Genomic Study of 15 Cases and Review of the Literature.
J Pediatr Hematol Oncol 27(4):179-187, 2005