PEDIATRIC SURGERY UPDATE ©
VOLUME 22, 2004
Volume 22 No 01 JANUARY 2004
Hashimoto Thyroiditis
Hashimoto thyroiditis (HT) is a chronic lymphocytic autoimmune thyroiditis
seen with some frequency in adolescent females and children. Most common
cause of asymptomatic enlargement of the thyroid gland in children in iodine-sufficient
geographic regions. Thyroid cell damage in HT is caused by antithyroid
antibody-dependent cell-mediated direct toxicity linked to deficiency in
antigen-specific suppressor T lymphocytes. The gland shows lymphocyte infiltration
with follicular cell hyperplasia. Thyroid antibodies are elevated. Radionuclear
scans show absent uptake. Initially the child develops elevated thyroid
hormones (T3 and T4) followed by symptomatic hypothyroidism. Following
the hypothyroid phase there is final recovery in most patients. Indications
for surgery in HT include: 1- firm enlargement of the gland causing tracheal
compression with dyspnea, hoarseness or swallowing difficulties, 2- failure
to respond to suppressive therapy and development of symptomatic hyperthyroid
goiter, and 3- development and enlargement of a solitary thyroid nodule.
The incidence of malignancy in HT is low. Differentiating a hyperplastic
follicular cell nodule from a follicular neoplasm is very difficult using
fine needle aspiration biopsy. Patient with malignant nodules in Hashimoto
glands are most commonly papillary, females, low frequency of extrathyroidal
invasion and nodal metastasis with absent distal metastasis. It is believed
the lymphocytic infiltration of HT causes a form of immune reaction to
control tumor growth and proliferation.
References:
1- Okayasu I, Fujiwara M, Hara Y, Tanaka Y, Rose NR:
Association of chronic lymphocytic thyroiditis and thyroid papillary carcinoma.
A study of surgical cases among Japanese, and white and African Americans.
Cancer 76(11):2312-8, 1995
2- Webb AJ, Brewster S, Newington D: Problems in diagnosis
and management of goitre in childhood and adolescence. Br J Surg 83(11):1586-90,
1996
3- Nguyen GK, Ginsberg J, Crockford PM, Villanueva RR:
Hashimoto's thyroiditis: cytodiagnostic accuracy and pitfalls. Diagn Cytopathol
16(6):531-6, 1997
4- Loh KC, Greenspan FS, Dong F, Miller TR, Yeo PP: Influence
of lymphocytic thyroiditis on the prognostic outcome of patients with papillary
thyroid carcinoma. J Clin Endocrinol Metab 84(2):458-63, 1999
5- Hopwood NJ, Kelch RP: Thyroid masses: approach to
diagnosis and management in childhood and adolescence. Pediatr Rev 14(12):481-7,
1993
6- Lafranchi S: Thyroiditis and acquired hypothyroidism.
Pediatr Ann 21(1):29, 32-9, 1992
7- Strakosch CR: Thyroiditis. Aust N Z J Med 16(1):91-100,
1986
Bile Reflux Gastritis
Alkaline reflux gastritis develops in patients with previous operations
that destroy the integrity of the pylorus as a true sphincter by removing
(antrectomy), bypassing (gastrojejunostomy) or obliterating (pyloroplasty)
the pylorus. It can be seen in children after repair of duodenal atresia.
Symptoms of bile reflux gastritis consist of epigastric pain, bilious vomiting,
anemia, gastrointestinal bleeding and weight loss. Eating increases the
discomfort. Endoscopy with biopsy in the presence of achloridia is
diagnostic. Mainstay of treatment for bile reflux gastritis consists of
histamine 2-receptor blockers, aluminum-containing antacids (to absorb
bile salts) and metoclopramide (improve gastric emptying). Medical management
should be tried for many months. The operation of choice is a Roux-en-y
diversion. If the original operation was vagotomy with pyloroplasty, the
gastric antrum should be removed (to eliminate cephalic and humoral phase
of gastric secretion) and a Roux-en-y gastrojejunostomy constructed.
References:
1- Davidson ED, Hersh T: The surgical treatment of bile
reflux gastritis: a study of 59 patients. Ann Surg 192(2):175-8, 1980
2- Cooperman AM: Postoperative alkaline reflux gastritis.
Surg Clin North Am 56(6):1445-59, 1976
3- Sorgi M, Keighley MR: Alkaline reflux gastritis: assessment
and therapy. Surg Annu 14:153-79, 1982
4- Burden WR, Hodges RP, Hsu M, O'Leary JP: Alkaline
reflux gastritis. Surg Clin North Am 71(1):33-44, 1991
5- Ritchie WP Jr.: Alkaline reflux gastritis. Gastroenterol
Clin North Am 23(2):281-94, 1994
Ovarian Teratoma
Two-third of all malignant tumors of the ovary in children are germ
cell tumors. Overall, teratoma is the most common germ cell tumor. Ovarian
teratomas contain tissue from the three primitive germ cell layers in an
ectopic location and seldom appear before the age of five years. Ovarian
teratomas are classified as mature, immature and malignant. The vast majority
of ovarian teratomas in children are benign, cystic, mature tumors. Plain
abdominal films may show calcifications. Degree of immaturity depends on
cellular differentiation and foci of neuroepithelium. Immature teratoma
can grow into large tumors presenting with ascites, peritoneal implants
and liver metastasis. Also, AFP and HCG levels can be elevated. Survival
in ovarian teratoma is inversely proportional to the grade of immature
elements present and stage of the disease. Mature teratomas are cured with
surgical resection only. Surgery is also curative for most children and
adolescents with resected ovarian immature teratoma of any grade, even
when elevated levels of serum AFP or microscopic foci of yolk sac tumor
are present. Chemotherapy should be reserved for cases with relapse. Upon
resection surgeons must collect peritoneal fluid for cytology, examine
peritoneal surface and liver, perform wedge biopsy of suspicious contralateral
ovarian lesions, omentectomy and lymph node sampling of enlarged retroperitoneal
nodes.
References:
1- Piver MS, Patton T: Ovarian cancer in children. Semin
Surg Oncol 2(3):163-9, 1986
2- Lazar EL, Stolar CJ: Evaluation and management of
pediatric solid ovarian tumors. Semin Pediatr Surg 7(1):29-34, 1998
3- Kobayashi RH, Moore TC: Ovarian teratomas in early
childhood. J Pediatr Surg 13(4):419-22, 1978
4- Chaung JH, Chen L: Ovarian teratoma with gliomatosis
peritonei. J Pediatr Surg 27(5):662-4, 1992
5- Brown MF, Hebra A, McGeehin K, Ross AJ 3rd: Ovarian
masses in children: a review of 91 cases of malignant and benign masses.
J Pediatr Surg 28(7):930-3, 1993
6- Cass DL, Hawkins E, Brandt ML, Chintagumpala M, Bloss
RS, Milewicz AL, Minifee PK, Wesson DE, Nuchtern JG: Surgery for ovarian
masses in infants, children, and adolescents: 102
consecutive patients treated in a 15-year period. J Pediatr
Surg 36(5):693-9, 2001
7- Marina NM, Cushing B, Giller R, Cohen L, Lauer SJ,
Ablin A, Weetman R, Cullen J, Rogers P, Vinocur C, Stolar C, Rescorla F,
Hawkins E, Heifetz S, Rao PV, Krailo M, Castleberry RP: Complete surgical
excision is effective treatment for children with immature teratomas with
or without malignant elements: A Pediatric Oncology Group/Children's Cancer
Group Intergroup Study. J Clin Oncol 17(7):2137-43, 1999
8- Cushing B, Giller R, Ablin A, Cohen L, Cullen J, Hawkins
E, Heifetz SA, Krailo M, Lauer SJ, Marina N, Rao PV, Rescorla F, Vinocur
CD, Weetman RM, Castleberry RP: Surgical resection alone is effective treatment
for ovarian immature teratoma in children and adolescents: a report of
the pediatric oncology group and the children's cancer group. Am J Obstet
Gynecol 181(2):353-8, 1999
Volume 22 No 02 FEBRUARY 2004
Forme Fruste Choledochal Cyst
In 1985, a new variant of choledochal cyst known as forme fruste was
described in the pediatric literature. Forme fruste choledochal cyst (FFCC)
is characterized by minimal dilatation of the extrahepatic bile duct which
does not grows with time. The normal diameter of the common bile duct in
children ranges between two and 6 mm. FFCC is associated with a diameter
above six mm and below 10 mm. Most patients with FFCC have a long common
channel, in which the common bile duct-pancreatic duct junction is away
from the duodenal papilla, with partial obstruction of the terminal common
bile duct. FFCC is associated with fever, jaundice, abdominal pain, recurrent
pancreatitis and altered liver function tests. Histologically FFCC demonstrates
thickened fibrous connective tissue, absent muscular layer with flattened,
ulcerated and dysplastic mucosa. Diagnosis is established with ultrasound.
ERCP or better yet MRCP can help delineate the anatomy and presence of
a long common pancreaticobiliary channel in FFCC. Management consists of
cyst excision and Roux-en-y hepaticojejunostomy. Due to the small size
the anastomosis is technically difficult and should be performed carefully
to avoid stricture and postoperative cholangitis. To maintain ductal anastomosis
patency it is imperative that diseased ductal tissue not be incorporated
in the anastomosis, the circumstance most likely responsible for the high
incidence of anastomotic stricture in choledochal cyst past drainage operations.
References:
1- Lilly JR, Stellin GP, Karrer FM: Forme fruste choledochal
cyst. J Pediatr Surg 20(4):449-51, 1985
2- Okada A: Forme fruste choledochal cyst. J Pediatr
Surg 21(4):383, 1986
3- Okada A, Oguchi Y, Kamata S, et al: Common channel
syndrome - diagnosis with endoscopic retrograde cholangio-pancreatography
and surgical treatment. Surgery 93: 634-642, 1983
4- Ando H, Ito T, Nagaya M, Watanabe Y, Seo T, Kaneko
K: Pancreaticobiliary Maljunction without choledochal cysts in Infants
and Children: Clinical features and surgical therapy. J Pediatr Surg 30(12):
1658-1662, 1995
5- Thomas S, Sen S, Zachariah N, Chacko J, Thomas G:
Choledochal cyst sans cyst--experience with six "forme fruste" cases. Pediatr
Surg Int 18(4):247-51, 2002
6- Shimotakahara A, Yamataka A, Kobayashi H, Okada Y,
Yanai T, Lane GJ, Miyano T: Forme fruste choledochal cyst: long-term follow-up
with special reference to surgical technique. J Pediatr Surg 38(12):1833-6,
2003
Gallbladder Polyps
A polypoid lesion identified in the gallbladder of a child is a very
rare event. It represents an elevated lesion of the mucosal surface of
the gallbladder which in most instances causes parental concern. Fortunately,
most polypoid lesions identified in gallbladders are benign (90%). Histologically
they are either adenomatous, hyperplastic, gastric heterotopia or cholesterol
polyps. The prevalence of such polyps is greater among males and obese
children. Ultrasonography is the image method of choice in diagnosing gallbladders
polyps in children and adults. They are seen as pedunculated or sessile
echogenic lesions attached to the gallbladder wall protruding toward the
lumen and fixed in changed of posture. Gallbladder polyps can be associated
with acalculous cholecystitis. Lesions smaller than 10 mm do not progress
to malignancy or development of stones, and none produces symptoms or complications
of biliary disease. Surgical management of gallbladder polyps is indicated
when the size of the polypoid lesion is above 10 mm in diameter, when associated
with gallstones and when the child has consistent biliary symptoms. Treatment
consists of laparoscopic cholecystectomy. Asymptomatic small polyps (<
10 mm) should be maintained under ultrasonographic surveillance.
References:
1- Yang HL, Sun YG, Wang Z: Polypoid lesions of the gallbladder:
diagnosis and indications for surgery. Br J Surg 79(3):227-9, 1992
2- Barzilai M, Lerner A: Gallbladder polyps in children:
a rare condition. Pediatr Radiol 27(1):54-6, 1997
3- Stringel G, Beneck D, Bostwick HE: Polypoid lesions
of the gallbladder in children. JSLS 1(3):247-9, 1997
4- Csendes A, Burgos AM, Csendes P, Smok G, Rojas J:
Late follow-up of polypoid lesions of the gallbladder smaller than 10 mm.
Ann Surg 234(5):657-60, 2001
5- Kikiros C, Arunachalam P, Lam MH: Adenomatous hyperplastic
polyp of the gall bladder associated with cholelithiasis in a child. Pediatr
Surg Int 19(1-2):118-9, 2003
6- Stringer MD, Ceylan H, Ward K, Wyatt JI: Gallbladder
Polyps in Children - Classification and Management. J Pediatr Surg 38(11):
1680-1684, 2003
Gastrocutaneous Fistula
Gastrocutaneous fistula (GCF) is most commonly identified after removing
long standing gastrostomy tubes in children. Other times is the result
of gastrojejunal tubes and Crohn's disease. After removing a temporary
gastrostomy tube most stomas close between three and six weeks after removal.
Persistence of stomach leakage through the gastrostoma is a nuisance, erodes
the surrounding skin and causes nutritional depletion. GCF does not close
spontaneously when the stoma has been used for a long period of time, when
there is distal obstruction (delayed gastric emptying), foreign body reaction
(silk), epithelization of the tract (multiple granulomas formation), or
associated chronic granulomatous disease (Crohn). Silk suture should be
avoided when constructing surgical gastrostomies. When the tube is in place
for more than nine months before removal the incidence of GCF can be as
high as 45%. Initial non-surgical therapy should include H2-antagonist
therapy and silver nitrate cauterization. If this does not work permanent
management of GCF consists of surgical closure.
References:
1- Kobak GE, McClenathan DT, Schurman SJ: Complications
of removing percutaneous endoscopic gastrostomy tubes in children. J Pediatr
Gastroenterol Nutr 30(4):404-7, 2000
2- Gordon JM, Langer JC: Gastrocutaneous fistula in children
after removal of gastrostomy tube: incidence and predictive factors. J
Pediatr Surg 34(9):1345-6, 1999
3- Aronian JM, Redo SF: Gastrocutaneous fistula after
tube gastrostomy. Incidence in infants and
children. N Y State J Med 74(13):2364-6, 1974
4- Davies BW, Watson AR, Coleman JE, Rance CH: Do gastrostomies
close spontaneously? A review of the fate of gastrostomies following successful
renal transplantation in children. Pediatr Surg Int 17(4):326-8, 2001
Volume 22 No 03 MARCH 2004
Umbilical Granuloma
Persistent umbilical swelling and discharge during the neonatal period
is of serious concern to both parents and physicians. Among umbilical swelling,
the umbilical granuloma is one of the most commonly seen condition in the
pediatric practice. The normal granuloma, a common inflammatory reaction
to the resolving umbilical stump of a newborn should disappear by the 2nd
to 3rd week of life after proper hygiene. Persistent beyond this time will
need some type of therapy. Umbilical granuloma is managed with 75% Sylver
nitrate stick application. Sylver nitrate is not innocuous and when apply
liberally can cause a minor burn of the periumbilical skin area of the
baby. Caution must be observed while applying Sylver nitrate, careful drying
the umbilical exudate to prevent periumbilical spillage, and discussion
with parent that burns may occur but apparently are not serious. Whenever
Sylver nitrate therapy fails and discharge persists, or contains urine
or fecal material, the physician should suspect that the child has either
a patent urachus or omphalomesenteric duct remnant as both conditions resemble
the common umbilical granuloma seen in general practice. Ultrasound studies
of the periumbilical area looking for a cyst, masses or fixed bowel loops
can help determine the presence of such congenital remnants. Management
of the persistent umbilical granuloma is surgical with double ligature,
cauterization of the base or formal umbilical exploration.
References:
1- Campbell J, Beasley SW, McMullin N, Hutson JM: Clinical
diagnosis of umbilical swellings and discharges in children. Med J Aust
145(9): 450-3, 1986
2- Chamberlain JM, Gorman RL, Young GM: Silver nitrate
burns following treatment for umbilical granuloma. Pediatr Emerg Care 8(1):
29-30, 1992
3- Boothroyd AE, Cudmore RE: Ultrasound of the discharging
umbilicus. Pediatr Radiol 26(5): 362-4, 1996
4- Nagar H: Umbilical granuloma: a new approach to an
old problem. Pediatr Surg Int 17(7): 513-4, 2001
5- Lotan G, Klin B, Efrati Y: Double-ligature: a treatment
for pedunculated umbilical granulomas in children. Am Fam Physician 65(10):
2067-8, 2002
Multicystic Dysplastic Kidneys
Multicystic dysplastic kidneys (MCDK) is a severe form of dysplasia
without any regular lobar development or normal calyceal drainage system.
The kidney stroma and size of the cysts can vary. The bigger the cysts
the less stroma. Most cases are unilateral; left side affected more often.
Bilateral disease is usually incompatible with life. MCDK is the most common
form of renal cystic disease and most common entity responsible for an
abdominal mass in infants. Most MCDK are associated with atresia of part
or all of the ipsilateral ureter. Ultrasound is diagnostic of MCDK. Renal
scan studies (DMSA) will not concentrate the contrast material. Retrograde
studies will show an atretic ureter. The differential diagnosis consists
of cystic mesoblastic nephroma which will show some function on excretory
urography or nuclear studies different from MCDK. MCDK does not have a
premalignant potential. The incidence of short term complications of MCDK
is very low. Regional pain caused by the expanding kidney mass is probably
the most absolute indication for nephrectomy in MCDK. Relative indications
consist of reversible hypertension, symptomatic urinary tract infection
and increasing kidney size. Almost 20% of these lesions will regress within
the first three years of life of the child.
References:
1- Hartman GE, Smolik LM, Shochat SJ: The dilemma of
the multicystic dysplastic kidney. Am J Dis Child 140(9): 925-8, 1986
2- Vinocur L, Slovis TL, Perlmutter AD, Watts FB Jr,
Chang CH: Follow-up studies of multicystic dysplastic kidneys. Radiology
167(2): 311-5, 1988
3- Webb NJ, Lewis MA, Bruce J, Gough DC, Ladusans EJ,
Thomson AP, Postlethwaite RJ: Unilateral multicystic dysplastic kidney:
the case for nephrectomy. Arch Dis Child 76(1): 31-4, 1997
4- Perez LM, Naidu SI, Joseph DB: Outcome and cost analysis
of operative versus nonoperative management of neonatal multicystic dysplastic
kidneys. J Urol 160(3 Pt 2): 1207-11, 1998
5- Abidari JM, Park KH, Kennedy WA, Shortliffe LD: Serial
followup of the contralateral renal size in children with multicystic dysplastic
kidney. J Urol 168(4 Pt 2): 1821-5, 2002
Intractable Constipation
Constipation is a common abdominal symptom in childhood. In the majority
of cases no cause is identified and the condition is labeled as idiopathic.
More than 90% of children with idiopathic constipation respond to medical
treatment (bulk diet, laxatives and enemas). Less than 10% develops intractable
constipation. Intractable constipation, not associated to Hirschsprung's
disease, neuromuscular disease or repaired anorectal malformations, that
fails to respond to aggressive medical management is one of the most difficult
conditions to manage in children. Children have duration of symptoms for
a period beyond five years. Intractable constipation produces progressive
fecal retention, fecal incontinence, distension of the rectum and sigmoid
colon with loss of rectal sensory and motor function. Encopresis ensues
when fecal soiling results from the retained fecal material. Idiopathic
constipation is associated with a thickened internal anal sphincter. Colonic
manometry helps differentiate causes of intractable constipation in childhood
showing the length of the abnormal colonic involved segment. Surgical management
for intractable constipation can consist of internal myectomy, placement
of cecostomy or left-colon tubes for antegrade enema cleansing, or resection
of the disease colonic segment when there is severe stasis and luminal
dilatation. Outcomes have thrown mixed results.
References:
1- Pemberton JH, Rath DM, Ilstrup DM: Evaluation and
surgical treatment of severe chronic constipation. Ann Surg 214(4): 403-11,
1991
2- Hosie GP, Spitz L: Idiopathic constipation in childhood
is associated with thickening of the
internal anal sphincter. J Pediatr Surg 32(7): 1041-1043,
1997
3- Villarreal J, Sood M, Zangen T, Flores A, Michel R,
Reddy N, Di Lorenzo C, Hyman PE: Colonic diversion for intractable constipation
in children: colonic manometry helps guide clinical decisions. J Pediatr
Gastroenterol Nutr 33(5): 588-91, 2001
4- Dey R, Ferguson C, Kenny SE, Shankar KR, Coldicutt
P, Baillie CT, Lamont GL,
Lloyd DA, Losty PD, Turnock RR: After the honeymoon--medium-term
outcome of antegrade continence enema procedure. J Pediatr Surg 38(1):
65-8, 2003
5- Churchill BM, De Ugarte DA, Atkinson JB: Left-colon
antegrade continence enema (LACE) procedure for fecal incontinence. J Pediatr
Surg 38(12): 1778-80, 2003
6- Youssef NN, Pensabene L, Barksdale E, Di Lorenzo C:
Is there a role for surgery beyond colonic aganglionosis and anorectal
malformations in children with intractable constipation? J Pediatr Surg
39 (1): 73-77, 2004
Volume 22 No 04 APRIL 2004
Spigelian Hernias
A spigelian hernia is a rare protrusion of peritoneal sac that occurs
through the transversus aponeurosis between the semicircular and lateral
border of the rectus sheath below the level of the umbilicus. Spigelian
hernias are more common in adults than children. The hernia appears as
an intermittent mass in the lower abdominal quadrant and flank seen when
the child exerts an increase abdominal pressure. Bowel or omentum can be
identified within the hernia content Some children manifests intermittent
abdominal pain. Spigelian hernias have been associated with cryptorchidism
and neuroblastoma. Diagnosis depends on finding an unusual mass on the
anterior abdominal wall and palpation of the rim of the hernia defect upon
reduction of the mass. Spontaneous closure has not been reported. Management
of spigelian hernias is straightforward: surgical repair when diagnosed
to avoid incarceration and strangulation. As in most hernia repairs the
defect should be marked prior to anesthesia since it will not be palpable
during abdominal wall relaxation. The internal oblique fascia along with
the transversalis fascia is closed in an overlapping manner followed by
the external oblique fascia preferably with interrupted nonabsorbable sutures.
Recurrence of the defect after surgery is extremely rare.
References:
1- Jarvis PA, Seltzer MH: Pediatric Spigelian hernia:
a case report. J Pediatr Surg 12(4):609-10, 1977
2- Graivier L, Bronsther B, Feins NR, Mestel AL: Pediatric
lateral ventral (spigelian) hernias. South Med J 81(3):325-6, 1988
3- Komura J, Yano H, Uchida M, Shima I: Pediatric spigelian
hernia: reports of three cases. Surg Today 24(12):1081-4, 1994
4- Walton JM, Bass JA: Spigelian hernias in infants:
report of two cases. Can J Surg 38(1):95-7, 1995
5- Al-Salem AH: Congenital spigelian hernia and cryptorchidism:
cause or coincidence? Pediatr Surg Int 16(5-6):433-6, 2000
6- White JJ: Concomitant Spigelian and inguinal hernias
in a neonate. J Pediatr Surg 37(4):659-60, 2002
7- Losanoff JE, Richman BW, Jones JW: Spigelian hernia
in a child: case report and review of the literature. Hernia 6(4):191-3.
Epub 2002 Sep 07, 2002
Pericardial Cysts
Pericardial cysts are benign large, single, spheroids congenital collections
of serous fluid originating in the mediastinum. Histologically they are
made of mesothelial cells. Since they adhere to the native pericardium,
the appearance if these intrathoracic masses in simple chest films are
that of cardiomegaly. Pericardial cysts account for 7% of all mediastinal
masses in children. Most pericardial cysts are asymptomatic, located in
the right cardiophrenic angle and detected incidentally during routine
chest films. Symptoms and serious complications such as dyspnea, cough,
respiratory distress, chest pain and cardiac tamponade can occur the result
of an expanding lesion on vital adjacent structures. The diagnosis of pericardial
cysts can be done prenatally using ultrasonography. Once suspected
the diagnosis is established by noninvasive studies such as echocardiography
and CT scans. Bronchogenic cysts have similar appearance in CT scans. Management
of pericardial cysts is surgical excision whenever possible. The objective
of removal of the lesion is elimination of the tumorous mass, relieve of
symptoms and allowance of histological examination. Approach can be open
or video-assisted thoracoscopic surgery. Prognosis is excellent in most
cases.
References:
1- Bini RM, Nath PH, Ceballos R, Bargeron LM Jr, Kirklin
JK: Pericardial cyst diagnosed by two-dimensional echocardiography and
computed tomography in a newborn. Pediatr Cardiol 8(1):47-50, 1987
2- Abad C, Rey A, Feijoo J, Gonzalez G, Martin-Suarez
J: Pericardial cyst. Surgical resection in two symptomatic cases. J Cardiovasc
Surg (Torino) 37(2):199-202, 1996
3- Bava GL, Magliani L, Bertoli D, Gorrieri PF, Rimini
A, Zaccagnini G, Bertolini A: Complicated pericardial cyst: atypical anatomy
and clinical course. Clin Cardiol 21(11):862-4, 1998
4- Eto A, Arima T, Nagashima A: Pericardial cyst in a
child treated with video-assisted thoracoscopic surgery. Eur J Pediatr
159(12):889-91, 2000
5- Noyes BE, Weber T, Vogler C: Pericardial cysts in
children: surgical or conservative approach? J Pediatr Surg 38(8):1263-5,
2003
Total Urogenital Mobilization
Total urogenital mobilization (TUM) was initially described by Peña
in 1997 to technically ease the surgical management of persistent cloaca.
Specifically cloacas with common channels less than three centimeters in
length managed using the posterior sagittal approach during separation
of the vagina from the urinary tract. TUM, as the word implies, consists
of total dissection and mobilization of the entire urogenital sinus as
a single unit anteriorly, posteriorly and laterally until enough length
is achieved to connect the vaginal edges to the perineum. This innovative
technical approach reduces operating time and improves final cosmetic appearance.
Furthermore, TUM can reduce the incidence of postoperative complications
such as urethrovaginal fistulas, vaginal stricture and acquired vaginal
atresia. Following this initial report the technique has been expanded
to include cases of congenital adrenal hyperplasia with urogenital sinus
(Prader Classification II, III and IV), female bladder exstrophy/epispadia
and penile agenesis. When the urogenital sinus is not associated with a
cloacal deformity, the procedure can be performed perineally. These cases
might need a posterior perineal skin flap to widen the vaginal introitus.
The technique can be combined
with reduction clitoroplasty for the surgical management of girls
with masculinized external genitalia. With adequate urogenital circumferential
mobilization urinary continence can be preserved.
References:
1- Peña A: Total urogenital mobilization--an easier
way to repair cloacas. J Pediatr Surg 32(2):263-7, 1997
2- Ludwikowski B, Oesch Hayward I, Gonzalez R: Total
urogenital sinus mobilization: expanded applications. BJU Int 83(7):820-2,
1999
3- Kropp BP, Cheng EY: Total urogenital complex mobilization
in female patients with exstrophy. J Urol 164(3 Pt 2):1035-9, 2000
4- Jenak R, Ludwikowski B, Gonzalez R: Total urogenital
sinus mobilization: a modified perineal approach for feminizing genitoplasty
and urogenital sinus repair. J Urol 165(6 Pt 2):2347-9, 2001
5- Hamza AF, Soliman HA, Abdel Hay SA, Kabesh AA, Elbehery
MM: Total urogenital sinus mobilization in the repair of cloacal anomalies
and congenital adrenal hyperplasia. J Pediatr Surg 36(11):1656-8, 2001
6- Hamza AF, Soliman HA, Hay SA, Kabesh AA, Soliman SM,
El Behery MM: Total urogenital sinus mobilization in the repair of cloacal
anomalies and congenital adrenal hyperplasia. Saudi Med J 24(5 Suppl):S47,
2003
Volume 22 No 05 MAY 2004
Pyomyositis
Pyomyositis is a purulent infection of skeletal muscle commonly seen
in children who live in tropical countries. For pyomyositis to develop
initial muscle injury followed by bacteremia must coexist. The initial
traumatic event causes a localized cutaneous infection which is the source
of the bacteremia that seeds the injured muscle tissue. Pyomyositis is
more common in males, especially those who participate in strenuous physical
activity. Peak incidence occurs between two and five years of age in children.
Associated conditions (60%) includes diabetes, liver disease and HIV. Clinically,
pyomyositis is accompanied by abscess formation in the suppurative phase
(fluctuance) but may be without a focal fluid collection in the presuppurative
phase (pain, fever, cellulitis, indurated muscle). Most common sites of
abscess formation are the quadriceps and gluteal muscles. Organisms more
commonly associated with pyomyositis are Staphylococcal Aureus which affects
90% of cases and streptococcus species. MRI is the most accurate means
of diagnosing a lesion within muscle determining location and extension
of the lesion. Initial management consists of systemic antibiotics. Surgical
drainage and debridement are of paramount importance in the management
of pyomyositis. All specimens obtained by aspiration or drainage should
be cultured for aerobic and anaerobic bacteria. In immunocompromised patients
progression to the septicemic stage is associated with high morbidity and
mortality.
References:
1- Meehan J, Grose C, Soper RT, Kimura K: Pyomyositis
in an adolescent female athlete. J Pediatr Surg 30(1):127-8, 1995
2- Brook I: Pyomyositis in children, caused by anaerobic
bacteria. J Pediatr Surg 31(3):394-6, 1996
3- Akman I, Ostrov B, Varma BK, Keenan G: Pyomyositis:
report of three patients and review of the literature. Clin Pediatr (Phila)
35(8):397-401, 1996
4- Ameh EA: Pyomyositis in children: analysis of 31 cases.
Ann Trop Paediatr 19(3):263-5, 1999
5- Bibbo C, Patel DV, Mackessy RP, Lin SS, Barricella
RL: Pyomyositis of the leg with early neurologic compromise. Pediatr Emerg
Care 16(5):352-4, 2000
6- Flier S, Dolgin SE, Saphir RL, Shlasko E, Midulla
P: A case confirming the progressive stages of pyomyositis. J Pediatr Surg
38(10):1551-3, 2003
Congenital Tracheal Stenosis
Congenital tracheal stenosis (CTS) is a rare condition seen immediately
after birth or in early infancy that is uniformly life-threatening. Infants
with CTS presents with stridor, respiratory distress, recurrent pulmonary
infections or failure to thrive. Inflammation of the mucosa or mucous accumulation
can easily obstruct the already stenotic airway. The stenosis includes
a short or long segment of circular cartilaginous ring. Diagnosis is established
using bronchoscopy, MRI (assessment of vascular structures and relation
to the stenosis), or CT-scan (good anatomic delineation of the airway).
Each individual malformation is studied using rigid tracheo-broncho- esophagoscopy.
Associated cardiac defects should rule out with echocardiogram. Management
of CTS is surgical. Selection of the type of treatment depends on the patient's
clinical status and the anatomic pattern of the stenosis. Resection of
a short stenosis with anastomosis can be possible with a length that does
not exceed half of the trachea. For longer stenosis the most useful technique
consists of enlargement tracheoplasty with cartilaginous or a pericardial
graft or the more recent and slide-tracheoplasty. The latter technique
is preferable because it preserves native tracheal tissue with fewer postop
complications. For CTS one should always look for other associated thoracic
malformations, such as a pulmonary sling which may compromise the results
of the surgical correction of the tracheal stenosis. Best prognosis is
obtained with simultaneous correction of the respiratory and cardiovascular
malformation. Overall survival of these children is 75%.
References:
1- Lang FJ, Hurni M, Monnier P: Long-segment congenital
tracheal stenosis: treatment by slide-tracheoplasty. J Pediatr Surg 34(8):1216-22,
1999
2- Matute JA, Romero R, Garcia-Casillas MA, de Agustin
JC, Marhuenda C, Berchi FJ, Vazquez J: Surgical approach to funnel-shaped
congenital tracheal stenosis. J Pediatr Surg 36(2):320-3, 2001
3- Grillo HC, Wright CD, Vlahakes GJ, MacGillivray TE:
Management of congenital tracheal stenosis by means of slide tracheoplasty
or resection and reconstruction, with long-term follow-up of growth after
slide tracheoplasty. J Thorac Cardiovasc Surg 123(1):145-52, 2002
4- Backer CL, Mavroudis C, Holinger LD: Repair of congenital
tracheal stenosis. Semin Thorac Cardiovasc Surg Pediatr Card Surg Annu
5:173-86, 2002
5- Rutter MJ, Cotton RT, Azizkhan RG, Manning PB: Slide
tracheoplasty for the management of complete tracheal rings. J Pediatr
Surg 38(6):928-34, 2003
6- Anton-Pacheco JL, Cano I, Garcia A, Martinez A, Cuadros
J, Berchi FJ: Patterns of management of congenital tracheal stenosis. J
Pediatr Surg 38(10):1452-8, 2003
7- Koopman JP, Bogers AJ, Witsenburg M, Lequin MH, Tibboel
D, Hoeve LJ: Slide tracheoplasty for congenital tracheal stenosis. J Pediatr
Surg 39(1):19-23, 2004
Aplasia Cutis Congenita
Congenital absence of skin, better known as Aplasia Cutis Congenita
(ACC) is a rare birth malformation characterized by partial or full-thickness
absence extending through the underlying tissues in a localized manner.
Sites involve in ACC include most commonly the vertex of the scalp region
(85%) followed by truncal and extremity skin areas. In the scalp the lesion
can include the dura with brain exposure. Life threatening hemorrhage from
the sagittal sinus or sepsis may occur if closure is delayed. Most affected
children with ACC are normal. Pathogenesis is not clear. A few are associated
with other malformations such as omphalocele, absence of distal limbs and
cleft deformities. The majority of these lesions are single and less than
two centimeters in diameter. Management of ACC is conservative for small
lesions with excision and primary closure. A larger lesion might need split-thickness
skin grafting or tissue expansion technique for closure.
References:
1- Vinocur CD, Weintraub WH, Wilensky RJ, Coran AG, Dingman
RO: Surgical management of aplasia cutis congenita. Arch Surg 111(10):1160-4,
1976
2- Sargent LA: Aplasia cutis congenita of the scalp.
J Pediatr Surg 25(12):1211-3, 1990
3- Ross DA, Laurie SW, Coombs CJ, Mutimer KL: Aplasia
cutis congenita: failed conservative treatment. Plast Reconstr Surg 95(1):124-9,
1995
4- Casanova D, Amar E, Bardot J, Magalon G: Aplasia cutis
congenita. Report on 5 family cases involving the scalp. Eur J Pediatr
Surg 11(4):280-4, 2001
5- Verhelle NAC, Heymans O, Deleuze JP, Fabre G, Vranckx
JJ, Van den hof B: Abdominal Aplasia Cutis Congenita: Case Report and Review
of the Literature. J Pediatr Sug 39(2): 237-239, 2004
Volume 22 No 06 JUNE 2004
Foveolar Hyperplasia
Idiopathic focal foveolar hyperplasia (FH) is a rare cause of gastric
outlet obstruction in infants. These non-neoplastic polyps are usually
found in adults. Children affected with FH presents early in life with
persistent postprandial vomiting and failure to thrive, signs which are
undistinguishable from hypertrophied pyloric stenosis. The characteristic
histology of faveolar hyperplasia consists of enlarged, tortuous and dilated
gastric pits (foveolas), producing a redundant mucosa that causes partial
obstruction of the antro-pyloric area. Associated is submucosal eosinophilic
inflammatory reaction suggesting an allergic component. Some reports have
suggested cows' milk protein allergy as a key factor. In general, the etiology
of FH is unknown. A few reports have found that foveolar hyperplasia develops
after prostaglandin E infusion, an effect which is dose related, and resolves
with cessation of the drug. Ultrasound of the antro-pyloric canal will
demonstrate a filling defect, polypoidal, redundant lesion with central
echogenic folds without muscular wall thickening. UGIS shows a longitudinal
filling defect. Upper endoscopy reveals polypoidal mucosal hypertrophy
originating from antrum and extending into the duodenal cap. Biopsy establishes
the diagnosis. Management of symptomatic idiopathic focal foveolar
hyperplasia consists of surgical excision of the involved redundant mucosa
with pyloroplasty or pyloromyotomy.
References:
1- Katz ME, Blocker SH, McAlister WH: Focal foveolar
hyperplasia presenting as an antral-pyloric mass in a young infant. Pediatr
Radiol 15(2):136-7, 1985
2- McAlister WH, Katz ME, Perlman JM, Tack ED: Sonography
of focal foveolar hyperplasia causing gastric obstruction in an infant.
Pediatr Radiol 18(1):79-81, 1988
3- Mercado-Deane MG, Burton EM, Brawley AV, Hatley R:
Prostaglandin-induced foveolar hyperplasia simulating pyloric stenosis
in an infant with cyanotic heart disease. Pediatr Radiol 24(1):45-6, 1994
4- Holland AJ, Freeman JK, Le Quesne GW, Khong TY: Idiopathic
focal foveolar hyperplasia in infants. Pediatr Surg Int 12(7):497-500,
1997
5- Master V, Davidson G, Morris L, Martin J, Kennedy
D, Byard R, Freeman J: Focal foveolar cell hyperplasia presenting as recurrent
emesis in a young infant. J Pediatr Gastroenterol Nutr 26(2):222-5, 1998
6- Morinville V, Bernard C, Forget S: Foveolar hyperplasia
secondary to cow's milk protein hypersensitivity presenting with clinical
features of pyloric stenosis. J Pediatr Surg 39(1):E29-31, 2004
Congenital Extremity Gangrene
Being born with arterial or venous occlusion of a distal extremity and
gangrene is a rare event of obscure etiology in newborns. Arterial thrombosis,
emboli, trauma, congenital heart disease, sepsis, dehydration, coagulopathies,
venous occlusion from direct pressure, constrictive bands, compression
by the encircling umbilical cord, and venipuncture are all possible causes
which should be considered in the differential diagnosis of congenital
gangrenous extremity. Unfortunately in most cases the etiology cannot be
established. When gangrene is established at birth surgical amputation,
autoamputation, or some loss of function is usual. Management is in general
supportive, allowing the ischemic area to demarcate and slough. Range-of-motion
exercises and splinting to avoid contracture are helpful in the rehabilitative
phase. In very rare occasions early aggressive systemic thrombolytic therapy
(urokinase) followed by serial soft-tissue debridement and ultimate skin
coverage through cultured epithelial autografts have been reported with
good limb salvage results. Peripheral Ischaemic insults presenting at birth
may be part of a wider spectrum of disorders, both prenatal and perinatal,
attributable to occlusive vascular disruption.
References:
1- Hensinger RN: Gangrene of the newborn. A case report.
J Bone Joint Surg Am 57(1):121-3, 1975
2- Nazer H, Abu Rajab A, Qaryouti S, Tarawneh M, Hamzeh
Y, Arda H, Mustafa M: Neonatal limb gangrene and renal vein thrombosis.
Case report with review of literature. Eur J Pediatr 146(4):429-31,
1987
3- Turnpenny PD, Stahl S, Bowers D, Bingham P: Peripheral
ischaemia and gangrene presenting at birth. Eur J Pediatr 151(8):550-4,
1992
4- Ricciardelli E, Morgan RF, Lin KY: In utero brachial
artery thrombosis: limb salvage with postnatal urokinase infusion. Ann
Plast Surg 34(1):81-3, 1995
5- Carr MM, al-Qattan M, Clarke HM: Extremity gangrene
in utero. J Hand Surg [Br]. 21(5):652-5, 1996
Parotid Hemangioma
Parotid hemangioma (or hemangioendothelioma) is by far the most common
tumor of the parotid gland seen in infants and children. Initially the
infant presents with non-tender swelling of the cheek during the first
weeks of life. The swelling is generally confined to the superficial lobe
of the parotid gland, but it can involve the masseter muscle. With capillary
and bluish involvement of the skin and subcutaneous tissue the diagnosis
is easier to establish. MRI is the investigation of choice because of picture
quality, definition of soft tissues and lack of exposure to ionizing radiation.
MRI allows a definite diagnosis to be made without any invasive procedure
being required. When in doubt a fine-needle biopsy will establish the histologic
nature of the mass. US with Doppler imaging (lobular internal structure,
fine echogenic internal septations, mildly lobulated contour and extremely
high vascularity), and labeled red cell scintigraphy (well-defined area
of intense activity) can also sustain the diagnosis of parotid hemangioma.
Management is conservative since most lesions involute spontaneously. During
involution ulceration and calcification can occur. Medical management (intralesional
injection of steroids, systemic steroids or interferon) is given when the
tumor is large, deforming, ulcerated, or involves nearby structures with
functional consequences. The overall response rate is very high.
References:
1- Tresserra L, Martinez-Mora J, Boix-Ochoa J: Haemangiomas
of the parotid gland in children. J Maxillofac Surg 5(4):238-41, 1977
2- George CD, Ng YY, Hall-Craggs MA, Jones BM: Parotid
haemangioma in infants: MR imaging at 1.5T. Pediatr Radiol 21(7):483-5,
1991
3- Huchzermeyer P, Birchall MA, Kendall B, Bailey CM:
Parotid haemangiomas in childhood: a case for MRI. J Laryngol Otol 108(10):892-5,
1994
4- Roebuck DJ, Ahuja AT: Hemangioendothelioma of the
parotid gland in infants: sonography and correlative MR imaging. AJNR Am
J Neuroradiol 21(1):219-23, 2000
5- Esposito C, Zupi A, Califano L: Surgical therapy of
parotid hemangiomas. Pediatr Surg Int 17(5-6):335-7, 2000
6- Greene AK, Rogers GF, Mulliken JB: Management of parotid
hemangioma in 100 children. Plast Reconstr Surg 113(1):53-60, 2004