PEDIATRIC SURGERY UPDATE ©
VOLUME 17, 2001
Volume 17 No 01 JULY 2001
Hidradenitis Suppurativa
Hidradenitis suppurativa refers to a chronic relapsing cutaneous inflammation
of the apocrine glands of the body. Chronic hidradenitis suppurativa can
affect, in order of preference, the axilla, perineum, scrotum, inguinal,
scalp, palms of the hand, soles of the feet and the submammary region.
Hidradenitis suppurativa usually manifests itself after puberty (androgen-dependent
disorder). Females are more commonly affected than males. The inflammatory
process begins as a local occluding spongiform infundibulo-folliculitis
of a sweat gland with subsequent rupture and secondary bacterial infection.
Symptoms include pain, swelling, purulent discharge, and pruritus of the
affected region. Associated medical conditions include diabetes and obesity.
Initial management consists of general hygienic measures with antibiotics,
antiandrogens and estrogens. Surgery is needed when the condition is at
an advanced stage with cellulitis and scarring. Nonoperative treatment
is disappointing. Total excision of all apocrine-bearing axillary tissue
with primary closure is the treatment of choice. Operative treatment can
be safely accomplished even when draining sinuses are present. Recurrence
results from inadequate excision or an unusually wide distribution of apocrine
glands, but physical factors such as obesity, local pressure, and skin
maceration play a role. Radical surgery gives good symptomatic control
of severe hidradenitis suppurativa of the axilla, inguinoperineal, and
perianal regions but is less satisfactory for submammary disease.
References:
1- Harrison BJ, Mudge M, Hughes LE: Recurrence after
surgical treatment of hidradenitis suppurativa. Br Med J 294(6570):487-9,
1987
2- Lewis F, Messenger AG, Wales JK: Hidradenitis suppurativa
as a presenting feature of premature adrenarche. Br J Dermatol 129(4):447-8,
1993
3- Boer J, Weltevreden EF: Hidradenitis suppurativa or
acne inversa. A clinicopathological study of early lesions. Br J Dermatol
135(5):721-5, 1996
4- Wenzel FG, Horn TD: Nonneoplastic disorders of the
eccrine glands. J Am Acad Dermatol 38(1):1-17, 1998
5- Mengesha YM, Holcombe TC, Hansen RC: Prepubertal hidradenitis
suppurativa: two case reports and review of the literature. Pediatr Dermatol
16(4):292-6, 1999
6- Naimer SA, Zvulunov A, Ben-Amitai D, Landau M: Plantar
hidradenitis in children induced by exposure to wet footwear. Pediatr Emerg
Care 16(3):182-3, 2000
Desmoid Tumors
Desmoid fibromatosis (DF) is a benign, locally aggressive tumor, with
a strong propensity for infiltrative growth and local recurrence.
More than 50% of the tumors develop within the first five years of life
as an asymptomatic, firm, solid mass. DF can be found on the head and neck,
upper or lower extremities, the abdomen or the trunk. The lower extremities
are the most frequent sites of manifestation. The most frequent types are
the infantile fibromatosis (head, neck, shoulder, upper arm or thigh),
extra-abdominal fibromatosis (chest wall, back and thigh) and fibromatosis
colli (neck). Superficial lesions tend to be slow growing, small and rarely
involve deep structures. Deep-seated DF tends to be faster growing, larger
and involves deeper structures (extra-abdominal). Except fibromatosis colli
that tends to regress spontaneously, infantile and extra-abdominal DF is
best managed by gross total resection achieving negative margins unless
tumor excision is either particularly dangerous or likely to result in
significant physical handicap. Radiation or chemotherapy is most often
used with recurrent disease or as an alternative to mutilating surgery.
Adjuvant radiation therapy improves local control. DF have no tendency
to metastasize. It is believed they should be treated as low-grade malignancies
with documentation of histologic margins and close clinical follow-up.
References:
1- Ayala AG, Ro JY, Goepfert H, Cangir A, Khorsand J,
Flake G: Desmoid fibromatosis: a clinicopathologic study of 25 children.
Semin Diagn Pathol 3(2):138-50, 1986
2- Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM:
Problems in diagnosis and management of desmoid tumors. Am J Surg 159(5):450-3,
1990
3- Humar A, Chou S, Carpenter B: Fibromatosis in infancy
and childhood: the spectrum. J Pediatr Surg 28(11):1446-50, 1993
4- Faulkner LB, Hajdu SI, Kher U, La Quaglia M, Exelby
PR, Heller G, Wollner N: Pediatric desmoid tumor: retrospective analysis
of 63 cases. J Clin Oncol 13(11):2813-8, 1995
5- Lewis JJ, Boland PJ, Leung DH, Woodruff JM, Brennan
MF: The enigma of desmoid tumors. Ann Surg 229(6):866-72, 1999
6- Merchant TE, Nguyen D, Walter AW, Pappo AS, Kun LE,
Rao BN: Long-term results with radiation therapy for pediatric desmoid
tumors. Int J Radiat Oncol Biol Phys 47(5):1267-71, 2000
Dieulafoy Lesion
First described by a French surgeon in 1897, Dieulafoy's lesion (DL)
also known as cirsoid aneurysm, is a rare cause of massive upper gastrointestinal
hemorrhage. Most DL occurs due to an abnormal submucosa artery in the stomach
particularly along the lesser curvature in the region supplied by the left
gastric artery and within six centimeters of the gastroesophageal junction.
They have also been reported to occur in the jejunum and rectum. The problem
with this lesion is that bleeding is intermittent and massive. With fiberoptic
endoscopy the source of upper and lower gastrointestinal bleeding can be
identified in 80 to 85% of children. The endoscopic appearance of
DL may range from a pinpoint dot (two to 5 mm), clot, or tortuous vessel,
to blood oozing or spurting from normal mucosa. There is no surrounding
mucosal ulceration. Other times diagnosis is made at operation, as endoscopy
and arteriography fail to identify the lesion. Repeated endoscopies might
be needed. Management consists of endoscopic sclerotherapy injection and
laser photocoagulation. If this fails, surgery follows. Simple ligation
of the bleeding dot is all that is required to control the hemorraghe.
References:
1- McClave SA, Goldschmid S, Cunningham JT, Boyd WP:
Dieulafoy's cirsoid aneurysm of the duodenum. Dig Dis Sci 33(7):801-5,
1988
2- Tooson JD, Marsano LS, Gates LK: Pediatric rectal
Dieulafoy's lesion. Am J Gastroenterol 90(12):2232-3, 1995
3- Driver CP, Bruce J: An unusual cause of massive gastric
bleeding in a child. J Pediatr Surg 32(12):1749-50, 1997
4- Skok P: Endoscopic hemostasis in exulceratio simplex-Dieulafoy's
disease hemorrhage: a review of 25 cases. Endoscopy 30(7):590-4, 1998
5-Stockwell JA, Werner HA, Marsano LS: Dieulafoy's lesion
in an infant: a rare cause of massive gastrointestinal bleeding. J Pediatr
Gastroenterol Nutr 31(1):68-70, 2000
Volume 17 No 02 AUGUST 2001
Jugular Phlebectasia
A mass that appears in the neck upon straining (Valsalva maneuver),
coughing, sneezing or crying may be the result of a laryngocele, jugular
phlebectasia or superior mediastinal tumor. Jugular phlebectasia (also
known as venous congenital cyst, venous aneurysm, venous ectasia or essential
venous dilatation) refers to an isolated abnormal fusiform or saccular
dilatation of the internal jugular vein and it usually present with a swelling
in the right posterior triangle of the neck. Most patients are children,
boys being more twice as often affected as girls. Phlebectasia may affect
any vein in the neck, especially in this sequence: internal jugular, external
jugular, anterior jugular and the superficial communicans. Jugular phlebectasia
is an asymptomatic benign condition whose etiology is unknown. Absence
of a wide mediastinum or air in the mass on simple chest films eliminates
a mediastinal tumor or laryngocele respectively. Non-invasive diagnosis
of jugular phlebectasia can be achieved using ultrasonography combined
with Doppler flow imaging and spiral computerized tomography scan with
contrast. No treatment is indicated for this benign self-limiting condition,
except for the few patients who complain of symptoms (feeling of constriction,
choking, bluish discoloration, thrombosis, discomfort during physical activity
or tongue pain) and require surgical removal of the affected vein. Surgical
removal for cosmetic purposes alone consists of a unilateral excision of
the internal or external jugular vein, a procedure that produces no gross
side-effects.
References:
1- Bowdler DA, Singh SD: Internal jugular phlebectasia.
Int J Pediatr Otorhinolaryngol 12(2):165-71, 1986
2- Nwako FA, Agugua NE, Udeh CA, Osuorji RI: Jugular
phlebectasia. J Pediatr Surg 24(3):303-5, 1989
3- Yokomori K, Kubo K, Kanamori Y, Takemura T, Yamamoto
T: Internal jugular phlebectasia in two siblings: manometric and histopathologic
studies of the pathogenesis. J Pediatr Surg 25(7):762-5, 1990
4-Balik E, Erdener A, Taneli C, Mevsim A, Sayan A, Yuce
G: Jugular phlebectasia in children. Eur J Pediatr Surg 3(1):46-7, 1993
5- Pul N, Pul M: External jugular phlebectasia in children.
Eur J Pediatr 154(4):275-6, 1995
6- Sander S, Elicevik M, Unal M, Vural O: Jugular phlebectasia
in children: is it rare or ignored? J Pediatr Surg 34(12):1829-32, 1999
Ganglion Cysts
Ganglion cysts are synovial cysts that appear in the wrist or foot after
minor trauma or stress. Most occur in the dorsal wrist area. Presence of
a colorless to pale-yellow gelatinous material in the aspirate is pathognomonic
of ganglion cysts. FNA smears are monotonous showing abundant mucoid material,
single cells resembling histiocytes, a few tight clusters of cells, some
collagen fibers, and some red blood cells with altered shapes. Serial microscopic
studies have shown evidence of a one way valvelike system between the affected
joint and the ganglion. Diagnosis is physical, though ultrasound findings
(cysts with a mean diameter of 1.4 cm and projection into the joint or
tendon) are of help. Management consists of excision. Recurrence rates
are high ranging between 10 and 35%. Ganglion cysts in the volar aspect
of the wrist have a higher incidence of postoperative complications (nerve
and radial artery damage). Intralesional injection of hyaluronidase has
been found a safe, fast, well accepted and cost-effective alternative to
surgical excision.
References:
1- Oertel YC, Beckner ME, Engler WF: Cytologic diagnosis
and ultrastructure of fine-needle aspirates of ganglion cysts. Arch Pathol
Lab Med 110(10):938-42, 1986
2- Paivansalo M, Jalovaara P: Ultrasound findings of
ganglions of the wrist. Eur J Radiol 13(3):178-80, 1991
3- Otu AA: Wrist and hand ganglion treatment with hyaluronidase
injection and fine needle aspiration: a tropical African perspective. J
R Coll Surg Edinb 37(6):405-7, 1992
4- Gundes H, Cirpici Y, Sarlak A, Muezzinoglu S: Prognosis
of wrist ganglion operations. Acta Orthop Belg 66(4):363-7, 2000
Anal Warts
Warts in the perianal region of prepubertal infant or child most commonly
are the result of human papilloma virus (HPV) infestation. Main clinical
manifestations of anal warts are cauliflower-like Condylomata Acuminata
that usually involves moist surfaces, keratotic and smooth papular warts
usually on dry surfaces, and subclinical flat warts that can be found on
any mucosal or cutaneous surface. Mode of transmission can occur from an
infected maternal birth canal (perinatally), by autoinoculation or heteroinoculation
from common hand warts, through sexual abuse and possibly indirect transmission
via fomites. Often, the mode of transmission is unknown. HPV-DNA typing
is a useful technique that helps identify the genital types involved (6
or 11, 16 or 18) alerting the physician to proceed with a careful assessment
for sexual abuse. Predisposing factors for anal warts include social problems,
lack of hygiene, promiscuity, diabetes and ammoniacal erythema. The appearance
of the papilloma is diagnostic. Management includes cytotoxic agents (podophyllin,
podophyllotoxin and fluorouracil), destructive procedures (scissor excision,
cryotherapy, electrocautery, and laser photocoagulation) and recently topical
interferon hydrogel. In case of transmission by sexual abuse child protection
is warranted.
References:
1- Armstrong DK, Handley JM: Anogenital warts in prepubertal
children: pathogenesis, HPV typing and management. Int J STD AIDS 8(2):78-81,
1997
2- Derksen DJ: Children with condylomata acuminata. J
Fam Pract 34(4):419-23, 1992
3- Martinon Sanchez F, Martinon Sanchez ML, Farina Guerrero
P, Michelena del Riego M, Mato Prada J: Condyloma acuminatum in children.
An Esp Pediatr 28(1):15-8, 1988
4- Handley JM, Maw RD, Bingham EA, Horner T, Bharucha
H, Swann A, Lawther H, Dinsmore WW: Anogenital warts in children. Clin
Exp Dermatol 18(3):241-7, 1993
5- Obalek S, Jablonska S, Favre M, Walczak L, Orth G:
Condylomata acuminata in children: frequent association with human papillomaviruses
responsible for cutaneous warts. J Am Acad Dermatol 23(2 Pt 1):205-13,
1990
6- Boyd AS: Condylomata acuminata in the pediatric population.
Am J Dis Child 144(7):817-24, 1990
Volume 17 No 03 SEPTEMBER 2001
Duodenal Duplications
Bowel duplications are congenital anomalies that occur anywhere in the
gastrointestinal tract from mouth to anus. Duplications are spherical or
tubular in shape, located in or adjacent to the wall of part of the gastrointestinal
tract (dorsal or mesenteric side of the native bowel), have smooth muscle
in their walls, and are lined by alimentary tract mucosa. Duodenal duplications
(DD) comprise 5 to 10% of all bowel duplications. DD usually present with
a palpable abdominal mass, obstructive symptoms (vomiting), bleeding (from
heterotopic gastric mucosa), perforation or pancreatitis. Most develop
symptoms during the first two years of life. DD are usually attached by
a common muscularis posteromedial to the duodenum, partly embedded in the
pancreas with no communication with the lumen. Diagnosis can be established
by
the finding of "gut signature" on ultrasound - the hyperechoic inner layer
produced by the mucosa surrounded by a hypoechoic outer layer caused by
smooth muscle. CT, ERCP and MR-Cholangiography can identify location, size,
associated structures and bile ductal anatomy. Management of duodenal duplications
depends on the association of this lesion to the native duodenum, pancreas
and biliary system. Preop or intraoperative cholangiography should be done
whenever the biliary system is involved. Preferred management is complete
excision whenever possible, but mucosal stripping or marsupialization are
acceptable alternatives.
References:
1- Macpherson RI: Gastrointestinal tract duplications:
clinical, pathologic, etiologic, and radiologic considerations. Radiographics
13(5):1063-80, 1993
2- Stern L, Warner BW: Gastrointestinal Duplications.
Seminars Pediatr Surg 9(3): 135-140, 2000
3- Lyer CP, Mahour GH: Duplications of the Alimentary
Tract in Infants and Children. J Pediatr Surg 30(9): 1267-1270, 1995
4- Siddiqui AM, Shamberger RC, Filler RM, Perez-Atayde
AR, Lillehei CW: Enteric Duplications of the Pancreatic Head: Definite
Management by Local Resection. J Pediatr Surg 33(7): 1117-1121, 1998
5- Lad RJ, Fitzgerald P, Jacobson K: An unusual cause
of recurrent pancreatitis: duodenal duplication cyst. Can J Gastroenterol
14(4):341-5, 2000
6- Stringer MD, Spitz L, Abel R, Kiely E, Drake DP, Agrawal
M, Stark Y, Brereton RJ: Management of alimentary tract duplication in
children. Br J Surg 82(1):74-8, 1995
Gynecomastia
Gynecomastia refers to abnormal breast enlargement in males. In children,
gynecomastia can be classified as simple pubertal, pathological, general
obesity and pectoral muscle hypertrophy. Most cases of gynecomastia are
simple pubertal associated to a transient or permanent disturbance in steroid
hormone physiology occurring when the male breast is exposed to a decreased
ratio of androgen to estrogen. Pubertal gynecomastia can be managed non-operatively
since breast enlargement start one year after the onset of puberty and
subside two years later. Pathological gynecomastia is associated with drug
use (steroids, digitalis, spironolactone, marijuana), chronic liver disease
or malignancy (Leydig cell tumor of the testis). General obesity is associated
with fat deposition surrounding breast tissue that lends itself to weight
reduction. Diagnosis is by history and physical exam. Routine endocrine
work-up is not cost effective. Persistent pain, uncertain diagnosis and
cosmetic reasons (embarrassment or distress) are the major reasons for
operation. Subcutaneous mastectomy through a periareolar incision gives
the best cosmetic results. Common late postoperative sequelae are inverted
areolae, hypopigmentation and hypoesthesia of the areolar region.
References:
1- Mahoney CP: Adolescent gynecomastia. Differential
diagnosis and management. Pediatr Clin North Am 37(6):1389-404, 1990
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- Park AJ, Lamberty BG: Gynaecomastia: have Webster's
lessons been ignored?. J R Coll Surg Edinb 43(2):89-92, 1998
4- Bowers SP, Pearlman NW, McIntyre RC Jr, Finlayson
CA, Huerd S: Cost-effective management of gynecomastia. Am J Surg 176(6):638-41,
1998
5- Mellor SG, McCutchan JD: Gynaecomastia and occult
Leydig cell tumour of the testis. Br J Urol 63(4):420-2, 1989
6- Persichetti P, Berloco M, Casadei RM, Marangi GF,
Di Lella F, Nobili AM: Gynecomastia and the complete circumareolar approach
in the surgical management of skin redundancy. Plast Reconstr Surg 107(4):948-54,
2001
Idiopathic Bowel Perforation
Bowel perforation causing pneumoperitoneum during the neonatal period
is usually associated with necrotizing enterocolitis (leading cause), atresias,
meconium ileus, and Hirschsprung's disease. Other times they are iatrogenic
(misplaced tubes, vigorous resuscitation and drug-related). There is a
group of newborns with spontaneous isolated bowel perforation and no evidence
of the above disease disorder termed idiopathic bowel perforation (IBP).
IBP can occur in the stomach, small, large bowel, and appendix. The perforation
is single, small, on the antimesenteric wall, measuring less than one cm
with almost minimal surrounding bowel necrosis. A suggested etiologic factor
consists of ischemic necrosis secondary to a very localized vascular accident.
Diagnosis is made after finding pneumoperitoneum is found in simple abdominal
films. After resuscitation, hydration and antibiotherapy, management is
surgical. Gastric perforations are sutured and in proximal bowel perforations
resection and anastomosis can be done. In distal bowel perforations ganglionic
segment should be exteriorized. Hirschsprung's disease should be excluded
by multiple seromuscular frozen section and rectal biopsy. IBP carries
a good prognosis with a survival rate above 80%.
References:
1- Bax NM, Pearse RG, Dommering N, Molenaar JC: Perforation
of the appendix in the neonatal period. J Pediatr Surg 15(2):200-2, 1980
2- Zamir O, Shapira SC, Udassin R, Peleg O, Arad I, Nissan
S: Gastrointestinal perforations in the neonatal period. Am J Perinatol
5(2):131-3, 1988
3- Zamir O, Goldberg M, Udassin R, Peleg O, Nissan S,
Eyal F: Idiopathic gastrointestinal perforation in the neonate. J Pediatr
Surg 23(4):335-7, 1988
4- Weinberg G, Kleinhaus S, Boley SJ: Idiopathic intestinal
perforations in the newborn: an increasingly common entity. J Pediatr Surg
24(10):1007-8, 1989
5- St-Vil D, LeBouthillier G, Luks FI, Bensoussan
AL, Blanchard H, Youssef S: Neonatal gastrointestinal perforations. J Pediatr
Surg 27(10):1340-2, 1992
6- Harms K, Ludtke FE, Lepsien G, Speer CP: Idiopathic
intestinal perforations in premature infants without evidence of necrotizing
enterocolitis. Eur J Pediatr Surg 5(1):30-3, 1995
Volume 17 No 04 OCTOBER 2001
Congenital Esophageal Stenosis
Esophageal stenosis in children can be of congenital (5%) or most commonly
acquired nature (95%). Acquired stenosis is the result of repaired esophageal
atresia, caustic injury, penetrating injury or reflux esophagitis. Congenital
esophageal stenosis (CES) can be the result of a membranous diaphragm,
segmental hypertrophy of the muscularis and submucosal layer (submucosal
fibrosis), or presence of ectopic tracheobronchial rest. CES most commonly
affect the middle and distal third of the esophagus and rarely cause symptoms
in the neonatal period. Symptoms can be vomiting of undigested food, regurgitation,
food impaction, difficulty swallowing solid and failure to thrive. CES
affecting the upper third of the esophagus is very rare and usually produce
respiratory symptoms such as stridor and repeated respiratory infections.
Esophageal atresia is associated with one-third of cases of CES. To establish
a diagnosis investigation has to include esophagogram (relatively long,
smooth circumferential narrowing), esophagoscopy with biopsy, pH monitoring
and in selected cases manometry. Recognition of the correct etiologic factor
that caused the stricture will pave the way for adequate management. CES
is managed with forceful dilatation or hydrostatic balloon dilatation,
while
resection with anastomosis will be needed for intractable (fibromuscular
hypertrophy) cases and those harboring tracheobronchial rests. Most intractable
cases are due to the presence of tracheobronchial rest.
References:
1- Dominguez R, Zarabi M, Oh KS, Bender TM, Girdany BR:
Congenital oesophageal stenosis. Clin Radiol 36(3):263-6, 1985
2- Shoshany G, Bar-Maor JA: Congenital stenosis of the
esophagus due to tracheobronchial remnants: a missed diagnosis. J Pediatr
Gastroenterol Nutr 5(6):977-9, 1986
3- Kawahara H, Imura K, Yagi M, Kubota A: Clinical characteristics
of congenital esophageal stenosis distal to associated esophageal atresia.
Surgery 129(1):29-38, 2001
4- Shorter NA, Mooney DP, Vaccaro TJ, Sargent SK: Hydrostatic
balloon dilation of congenital esophageal stenoses associated with esophageal
atresia. J Pediatr Surg 35(12):1742-5, 2000
5- Diab N, Daher P, Ghorayeb Z, Korkmaz G: Congenital
esophageal stenosis. Eur J Pediatr Surg 9(3):177-81, 1999
6- Dohil R, Hassall E: Esophageal stenosis in children.
Gastrointest Endosc Clin N Am 8(2):369-90, 1998
7- Olguner M, Ozdemir T, Akgur FM, Aktug T: Congenital
esophageal stenosis owing to tracheobronchial remnants: a case report.
J Pediatr Surg 32(10):1485-7, 1997
8- Sarihan H, Abes M: Congenital esophageal stenosis.
J Cardiovasc Surg 38(4):421-3, 1997
9- Newman B, Bender TM: Esophageal atresia/tracheoesophageal
fistula and associated congenital esophageal stenosis. Pediatr Radiol 27(6):530-4,
1997
10- Murphy SG, Yazbeck S, Russo P: Isolated congenital
esophageal stenosis. J Pediatr Surg 30(8):1238-41, 1995
Limb Body Wall Complex
Limb-body wall complex (LBWC) is a rare, lethal, sporadic congenital
anomaly pattern in body-wall defects. Diagnosis of this entity is based
on finding at least two of the three following characteristics: (1) neural
tube defect (exencephaly/encephalocele and facial clefts), (2) body-wall
disruption (thoraco- and/or abdominoschisis, diaphragmatic hernia, eventration,
cloacal exstrophy, absent external genitalia or abnormal internal genitalia)
and, (3) limb abnormalities (ectrodactyly, phocomelia). Three pathogenic
mechanisms have been proposed: early amnion rupture, vascular disruption
and embryonic dysgenesis. The internal defects are secondarily to vascular
disruption. Cocaine abuse has been associated with LBWC by impairing uteroplacental
fetal blood flow during critical periods of development. The limb defects
are due to mechanical rupture through the amnion in the presence of a persistent
extraembryonic coelom or from adhesions of the amnion to necrotic embryonic
tissue. Diagnosis of LBWC is established with prenatal sonography. LBWC
is usually incompatible with life.
References:
1- Patten RM, Van Allen M, Mack LA, Wilson D, Nyberg
D, Hirsch J, Viamont T: Limb-body wall complex: in utero sonographic diagnosis
of a complicated fetal malformation. AJR Am J Roentgenol 146(5):1019-24,
1986
2- Van Allen MI, Curry C, Gallagher L: Limb body wall
complex: I. Pathogenesis. Am J Med Genet 28(3):529-48, 1987
3- Colpaert C, Bogers J, Hertveldt K, Loquet P, Dumon
J, Willems P: Limb-body wall complex: 4 new cases illustrating the importance
of examining placenta and umbilical cord. Pathol Res Pract 196(11):783-90,
2000
4- Chen CP: Prenatal diagnosis of limb-body wall complex
with craniofacial defects, amniotic bands, adhesions and upper limb deficiency.
Prenat Diagn 21(5):418-9, 2001
Ingrown Toe Nails
Onychocryptosis is the medical term use to describe ingrown toe nails
(ITN). Congenital hyperplasia of the nail bed lead to this condition in
infants and children. Adolescent and athletes are susceptible to
develop ITN. Other conditions associate with ingrown nails are hallux valgus,
claw toes, gout, diabetes, arthritis, or fungus infections of the nails.
Initial management should be conservative inserting cotton wool pledgets
moistened with an antiseptic under the ingrowing nail edge along with oral
antibiotics if there are signs of infection. Insertion of the pledget under
anesthesia improves the results and chronicity does not adversely affect
the outcome though the procedure is time consuming. Other alternatives
are segmental total nail avulsion, nail edge excision, nail matrix phenol
cauterization or wedge resections. Total nail avulsion and nail edge excision
carries the highest recurrence rate. Nail matrix phenolization is an effective
short procedure that can be done under the presence of an infection with
a low recurrence rate. Radical wedge resection is simple, has a low recurrence
rate, leaves patients with an intact, pain-free, cosmetically acceptable
nail-bearing toe, and permits the wearing of normal shoes within a short
period. In children conservative treatment is more promising than in adults.
If unsuccessful the next reliable surgical approach is wedge resection.
Attentions to hygiene and always cutting nails transversely are important
preventing factors.
References:
1- Herold HZ, Baruchin AM, Shmueli G, Daniel D, Naoum
A: Radical wedge resection for ingrown toenail: long-term results. J Dermatol
Surg Oncol 11(5):513-7, 1985
2- Senapati A: Conservative outpatient management of
ingrowing toenails. J R Soc Med 79(6):339-40, 1986
3- Connolly B, Fitzgerald RJ: Pledgets in ingrowing toenails.
Arch Dis Child 63(1):71-2, 1988
4- Grieg JD, Anderson JH, Ireland AJ, Anderson JR: The
surgical treatment of ingrowing toenails. J Bone Joint Surg Br 73(1):131-3,
1991
5- Kimata Y, Uetake M, Tsukada S, Harii K: Follow-up
study of patients treated for ingrown nails with the nail matrix phenolization
method. Plast Reconstr Surg 95(4):719-24, 1995
6- Lazar L, Erez I, Katz S: A conservative treatment
for ingrown toenails in children. Pediatr Surg Int 15(2):121-2, 1999
Volume 17 No 5 NOVEMBER 2001
Fibrosing Pancreatitis
Chronic pancreatitis does occur in young children and is most commonly
caused by hereditary pancreatitis or idiopathic fibrosing pancreatitis.
Fibrosing pancreatitis is a chronic process of unknown etiology characterized
by extensive infiltration of the pancreatic parenchyma by fibrous tissue.
Most reported cases are females. Fibrosing pancreatitis is a rare cause
of intermittent colicky abdominal pain, recurrent vomiting, weight loss,
steatorrhea, pancreatic exocrine and endocrine insufficiency and obstructive
jaundice that can be seen in early childhood. In cases of obstructive jaundice
the sonographic demonstration of a dilated biliary tree and common bile
duct compressed by an enlarged pancreas may be the first suggestion of
this disease process. Diagnosis is suggested by imaging studies (US, CT-Scan,
MRCP or ERCP) revealing diffuse enlargement of the pancreas, predominantly
the head. Open pancreatic biopsy showing acinar cell atrophy and extensive
fibrosis is diagnostic. Cystic fibrosis should be excluded by appropriate
testing. Management should be directed toward complications. Children with
obstructive jaundice should undergo sphincterotomy to drain the common
bile duct obstruction or bilio-enteric bypass.
References:
1- Meneely RL, O'Neill J, Ghishan FK: Fibrosing pancreatitis--an
obscure
causes of painless obstructive jaundice: a case report and review of the
literature. Pediatrics 67(1):136-9, 1981
2- Atkinson GO Jr, Wyly JB, Gay BB Jr, Ball TI, Winn
KJ: Idiopathic fibrosing pancreatitis: a cause of obstructive jaundice
in childhood. Pediatr Radiol 18(1):28-31, 1988
3- Buchta RM, Bell L: Chronic fibrosing pancreatitis
in a 12-year-old female. J Adolesc Health 12(5):395-7, 1991
4- Amerson JL, Ricketts RR: Idiopathic fibrosing pancreatitis:
a rare cause of obstructive jaundice in children. Am Surg 62(4):295-9,
1996
5- Lewin-Smith MR, Dipalma JS, Hoy GR, Colon AR, Garvin
DF: Chronic fibrosing pancreatitis in childhood: report of a case and literature
review. Pediatr Pathol Lab Med 16(4):681-90, 1996
6- Sylvester FA, Shuckett B, Cutz E, Durie PR, Marcon
MA: Management of fibrosing pancreatitis in children presenting with obstructive
jaundice. Gut 43(5):715-20, 1998
Foreign Body Ingestion
Accidental foreign body (FB) ingestion such as - coins, fish bones,
toys plastic parts, jewels, batteries, safety pin, needles, etc. - is a
common problem in children, specially infants and toddlers. Infants usually
swallow button batteries while coins are the most frequently swallowed
objects in children over the age of three years. No child ingests more
than one FB. Management of esophageal FB differs from the rest of the gastrointestinal
tract. Diagnosis is made by either chest-x-ray, barium swallow or esophagoscopy.
They should be suspected when the child develops excessive salivation,
vomiting, respiratory distress, recent-onset asthma, dysphagia and hematemesis.
FB in the esophagus should be removed urgently to avoid erosion and perforation,
specially those lodge in the upper-third of the esophagus. They can be
removed using flexible fiberoptic endoscopy, balloon catheter or bougienage.
Beyond the stomach, foreign body should be managed conservatively. This
means follow-up visits until the FB spontaneously appears in the feces.
In cases of coins ingestion serious complications are extremely rare. There
is no need to x-ray monitor coins or any other metallic FB. More than 85%
of all ingested FB passes spontaneously through the rectum despite nature
or length. Surgery will be needed in less than 2% of all ingested FB. Patients
with previous abdominal surgery are at increased risk. Development of abdominal
pain, distension or profuse bleeding is an indication to remove the FB
surgically.
References:
1- Suita S, Ohgami H, Nagasaki A, Yakabe S: Management
of pediatric patients who have swallowed foreign objects. Am Surg 55(9):585-90,
1989
2- Gilchrist BF, Valerie EP, Nguyen M, Coren C, Klotz
D, Ramenofsky ML: Pearls and perils in the management of prolonged, peculiar,
penetrating esophageal foreign bodies in children. J Pediatr Surg 32(10):1429-31,
1997
3- Hachimi-Idrissi S, Corne L, Vandenplas Y: Management
of ingested foreign bodies in childhood: our experience and review of the
literature. Eur J Emerg Med 5(3):319-23, 1998
4- Calkins CM, Christians KK, Sell LL: Cost analysis
in the management of esophageal coins: endoscopy versus bougienage. J Pediatr
Surg 34(3):412-4, 1999
5- Cheng W, Tam PK: Foreign-body ingestion in children:
experience with 1,265 cases. J Pediatr Surg 34(10):1472-6, 1999
6- Soprano JV, Mandl KD: Four strategies for the management
of esophageal coins in children. Pediatrics 105(1):e5, 2000
Inflammatory Pseudotumor
Inflammatory pseudotumor, better known as inflammatory myofibroblastic
tumor (IMT) is a rare benign solid tumor mimicking a malignant neoplasm
that grows to a large size. Though most commonly encountered in the lung
and mediastinum, IMT has also been identified in the abdomen (liver, pancreas,
stomach, omentum), retroperitoneum, pelvis (bladder), and extremity of
children. Imaging studies suggest a well-circumscribed mass of soft tissue
density producing displacement or invasion of surrounding structures. Needle
biopsy is not considered a reliable diagnostic method. Diagnosis must be
established by histology after surgical excision. Pathologically there
is proliferation of plasma cells, lymphocytes and histiocytes in a benign
looking spindle-shaped stroma (myofibroblasts). Etiology is unknown. Symptoms
at presentation depend on site of development. Total excision of the lesion
is sufficient for accurate treatment in most cases. Recurrence is common
and should also be managed with surgery. Adjuvant therapy has limited application.
References:
1- Brown G, Shaw DG: Inflammatory pseudotumors in children:
CT and ultrasound appearances with histopathological correlation. Clin
Radiol 50(11):782-6, 1995
2- Bonnet JP, Basset T, Dijoux D: Abdominal inflammatory
myofibroblastic tumors in children: report of an appendiceal case and review
of the literature. J Pediatr Surg 31(9):1311-4, 1996
3- Ciftci AO, Akcoren Z, Tanyel FC, Senocak ME, Caglar
M, Hicsonmez A: Inflammatory pseudotumor causing intestinal obstruction:
diagnostic and therapeutic aspects. J Pediatr Surg 33(12):1843-5,
1998
4- Cerfolio RJ, Allen MS, Nascimento AG, Deschamps C,
Trastek VF, Miller DL, Pairolero PC: Inflammatory pseudotumors of the lung.
Ann Thorac Surg 67(4):933-6, 1999
5- Sanders BM, West KW, Gingalewski C, Engum S, Davis
M, Grosfeld JL: Inflammatory pseudotumor of the alimentary tract: clinical
and surgical experience. J Pediatr Surg 36(1):169-73, 2001
6- Karnak I, Senocak ME, Ciftci AO, Caglar M, Bingol-Kologlu
M, Tanyel FC, Buyukpamukcu N: Inflammatory myofibroblastic tumor in children:
diagnosis and treatment. J Pediatr Surg 36(6):908-12, 2001
Volume 17 No 6 DECEMBER 2001
Gastrinoma
Gastrinomas, the cause of the Zollinger-Ellison syndrome, are gastrin
producing tumors that result in fulminant peptic ulceration and diarrhea.
In children, half the cases are associated with malignancy, metastasis
and multicentricity. Epidemiologically 75% gastrinomas are sporadic and
25% associated with MEN type 1. Diagnosis of gastrinoma is confirmed after
documenting fasting serum hypergastrinemia (> 200 pg/ml), markedly elevated
gastric output, a positive secretin-stimulated gastrin analysis or histologic
confirmation of a neuroendocrine tumor. Most tumors are found in the head,
body and tail of the pancreas, followed by duodenum, liver and lymph nodes.
Localization studies include CT-scan, MRCP, octreotide scan (specially
indium-labeled pentetreotide), endoscopic US, and Imamura test (selective
arterial secretin injection test). Radical resection of the gastrinoma
to achieve biochemical cure even when tumor is extrapancreatic and in lymph
nodes is the cornerstone of therapy. Unresectable or metastatic cases are
managed with pharmacologic therapy using proton pump inhibitors and octreotide.
Total gastrectomy is reserved for absolute failure of both medical and
surgical management. Gastrinomas in children are slow growing, indolent,
and compatible with long life. Prognostic factors correlating with long-term
survival are small tumor size, absence of metastatic disease and non-pancreatic
location of primary tumors. Factors that do not affect survival include
age at diagnosis, sex, presence of lymph node metastases, associated multiple
endocrine neoplasia, and method of ulcer treatment.
References:
1- Bonfils S, Landor JH, Mignon M, Hervoir P: Results
of surgical management in 92 consecutive patients with Zollinger-Ellison
syndrome. Ann Surg 194(6):692-7, 1981
2- Wilson SD: The role of surgery in children with the
Zollinger-Ellison syndrome. Surgery 92(4):682-92, 1982
3- Imamura M, Takahashi K, Isobe Y, Hattori Y, Satomura
K, Tobe T: Curative resection of multiple gastrinomas aided by selective
arterial secretin injection test and intraoperative secretin test. Ann
Surg 210(6):710-8, 1989
4- Wilson SD: Zollinger-Ellison syndrome in children:
a 25-year follow-up. Surgery 110(4):696-702, 1991
5- Farley DR, van Heerden JA, Grant CS, Miller LJ, Ilstrup
DM: The Zollinger-Ellison syndrome. A collective surgical experience. Ann
Surg 215(6):561-9, 1992
6- Zimmer T, Stolzel U, Bader M, Koppenhagen K, Hamm
B, Buhr H, Riecken EO, Wiedenmann B: Endoscopic ultrasonography and somatostatin
receptor scintigraphy in the preoperative localization of insulinomas and
gastrinomas. Gut 39(4):562-8, 1996
Epigastric Hernias
Congenital epigastric defects occur anywhere in the linea alba from
the navel to the xiphoid process. They represent almost 5% of all hernias
defect that presents in children. Most epigastric hernias occur in the
midline, are small (15-25 mm), asymptomatic and reducible. Multiple fascial
defects can also be present in 20% of all cases. The defect might arise
congenitally from an abnormally wide orifice of a blood vessel during development
of the linea alba. The bump is the result of a piece of preperitoneal fat
stuck through the fascial defect. Tenderness is an unusual symptom while
growth of the defect occurs with time. Most surgeons recommend repair of
the defect at the time of presentation. Repair is an outpatient procedure
done under general anesthesia with low morbidity and risk of recurrence.
Voluminous epigastric hernia (5-10 cm) with a sac that contains epiploic
appendages or viscera (ileum loops, stomach) has also been rarely reported
in infants.
References:
1- Coats RD, Helikson MA, Burd RS: Presentation and management
of epigastric hernias in children. J Pediatr Surg 35(12):1754-6,
2000
2- Askar OM: Aponeurotic Hernias: Recent observations
upon paraumbilical and epigastric hernias. Surg Clin North Am 64: 315-333,
1984
3- Corsale I, Palladino E: Diagnosis and treatment of
epigastric hernia. Analysis of our experience. Minerva Chir 55(9):607-10,
2000
4- Ameh EA: Giant congenital epigastric hernia. West
Afr J Med 18(2):151-2, 1999
Necrotizing Fasciitis
Necrotizing fasciitis (NF) is a rare, severe, rapidly progressive soft
tissue infection involving the skin, subcutaneous tissue and superficial
fascia. Most cases occur during the newborn period. In newborns NF is most
frequently associated with omphalitis, necrotizing enterocolitis, balanitis,
urachal anomalies and fetal monitoring. In older children NF can be seen
as a postoperative complication (appendectomy, gastrostomy), in neutropenia
after chemotherapy and secondarily to varicella infestation. Most frequent
sites of initial involvement are the abdominal wall, followed by thorax,
back, scalp, and extremity. Infection is usually polymicrobial containing
both gram positive (beta-hemolytic Streptococcus and Staphylococcus aureus)
and gram negative bacteria (E. Coli and Pseudomonas). Important clinical
findings in infants with NF are tachycardia, abnormal white blood cell
count, systemic toxicity, severe edema, and, in older children pain out
of proportion to the apparent degree of infection. The child can also develop
wound crepitation and radiographic evidence of subcutaneous gas. An ominous
sign indicative of the need for immediate radical debridement is the appearance
of a patch of dusky or gangrenous skin. Initial management consists of
antibiotics, intravenously afluids, blood transfusions, calcium replacement
(due to saponification) and general patient support. Neutropenic patients
benefit from granulocyte transfusions. Surgical procedures include extensive
debridement as soon as possible and as needed for continued necrosis, secondary
closure and skin grafting. Mortality ranges from 10 to 60%. Improved survival
requires early diagnosis followed by prompt aggressive surgical debridement.
References:
1- Kosloske AM, Cushing AH, Borden TA, Woodside JR, Klein
MD, Kulasinghe HP, Bailey WC: Cellulitis and necrotizing fasciitis of the
abdominal wall in pediatric patients. J Pediatr Surg 16(3):246-51,
1981
2- Farrell LD, Karl SR, Davis PK, Bellinger MF, Ballantine
TV: Postoperative necrotizing fasciitis in children. Pediatrics 82(6):874-9,
1988
3- Samuel M, Freeman NV, Vaishnav A, Sajwany MJ, Nayar
MP: Necrotizing fasciitis: a serious complication of omphalitis in neonates.
J Pediatr Surg 29(11):1414-6, 1994
4- Murphy JJ, Granger R, Blair GK, Miller GG, Fraser
GC, Magee JF: Necrotizing fasciitis in childhood. J Pediatr Surg
30(8):1131-4, 1995
5- Moss RL, Musemeche CA, Kosloske AM: Necrotizing fasciitis
in children: prompt recognition and aggressive therapy improve survival.
J Pediatr Surg 31(8):1142-6, 1996
6- Hsieh WS, Yang PH, Chao HC, Lai JY: Neonatal necrotizing
fasciitis: a report of three cases and review of the literature. Pediatrics
103(4):e53, 1999
7- Abbott RE, Marcus JR, Few JW, Farkas AM, Jona J: Necrotizing
fasciitis in infancy: an uncommon setting and a prognostic disadvantage.
J Pediatr Surg 34(9):1432-4, 1999