PEDIATRIC SURGERY UPDATE ©
VOLUME 28, 2007
PSU Volume 28 No 01 JANUARY 2007
Idiopathic Scrotal Edema
This is a very uncommon cause of acute scrotal swelling, but considered
the commonest cause of the 'acute scrotum' in prepubertal boys. Idiopathic
scrotal swelling is characterized by edema and erythema of the scrotal
wall, is usually bilateral and can sometimes involve the shaft of the penis.
The swelling and erythema can extend into the abdominal wall and perineum.
Children affected with acute scrotal swelling are four to six years in
age with symptoms present for less than 24 hours at the time of initial
medical evaluation. The cause of the swelling is usually not identified
but can be associated to reaction to an allergen, bug bite, contact dermatitis
or angioneurotic edema. Leukocytosis is absent, urinalysis is usually normal
and urine culture is sterile. Peripheral eosinophilia is present in some
patients. A connection with trauma, periurethral disease, or streptococcal
disease appears unlikely. The differential diagnosis includes torsion of
the testis or one of the testicular appendages, hydrocele, varicocele,
trauma, tumor, idiopathic scrotal edema, and Henoch-Schönlein purpura.
Color Duplex ultrasound of the scrotum will show increase testicular blood
flow and thickening of skin and muscle of the scrotum. Exploration is required
when a normal testis cannot absolutely be identified. Swelling usually
resolves within two to five days. Management consists of bed rest, reassurance,
and oral histamine.
References:
1- Kaplan GW: Acute idiopathic scrotal edema. J Pediatr
Surg. 12(5):647-9, 1977
2- Najmaldin A, Burge DM: Acute idiopathic scrotal oedema:
incidence, manifestations and aetiology. Br J Surg. 74(7):634-5, 1987
3- Rabinowitz R, Hulbert WC Jr: Acute scrotal swelling.
Urol Clin North Am. 22(1):101-5, 1995
4- van Langen AM, Gal S, Hulsmann AR, De Nef JJ: Acute
idiopathic scrotal oedema: four cases and a short review. Eur J Pediatr.
160(7):455-6, 2001
5- Klin B, Lotan G, Efrati Y, Zlotkevich L, Strauss S:
Acute idiopathic scrotal edema in children--revisited. J Pediatr Surg.
37(8):1200-2, 2002
6- Abul F, Al-Sayer H, Arun N: The acute scrotum: a review
of 40 cases. Med Princ Pract. 14(3):177-81, 2005
Epididymitis
Acute inflammation of the epididymis is an infectious process which
usually occurs during adolescent years, very rarely during prepubertal
ages. The infectious process is caused by a distal urethral obstruction,
ectopic ureter entering the seminal vesicles or epididymis, or after instrumentation.
Bacterial or viral organisms are involved in the infectious process. Epididymitis
seems to be more common than acute testicular torsion. Early clinical manifestations
of epididymitis include scrotal edema, pain, erythema, tenderness with
an associated reactive hydrocele. The epididymis turns elongated and exquisitely
tender to palpation. The differential diagnosis includes testicular torsion,
torsion of the testicular appendage or idiopathic scrotal edema. The urinalysis
will demonstrate pyuria with bacteriuria. Leukocytosis is also identified.
Color Doppler ultrasound or testicular scans can determine rapidly if we
are dealing with torsion due to reduced or absent testicular blood flow
in need of urgent surgery. In the event of doubt or absence of imaging
studies the diagnosis of an acute scrotum requires scrotal exploration.
Management of epididymitis includes intravenous antibiotics, pain medication,
scrotal support and bed rest. Further renal ultrasound and excretory urography
are needed after the episode subsides to determine a congenital urologic
anomaly.
References:
1- Weber DM, Rosslein R, Fliegel C: Color Doppler sonography
in the diagnosis of acute scrotum in boys. Eur J Pediatr Surg. 10(4):235-41,
2000
2- McAndrew HF, Pemberton R, Kikiros CS, Gollow I: The
incidence and investigation of acute scrotal problems in children. Pediatr
Surg Int. 18(5-6):435-7, 2002
3- Haecker FM, Hauri-Hohl A, von Schweinitz D: Acute
epididymitis in children: a 4-year retrospective study. Eur J Pediatr Surg.
15(3):180-6, 2005
4- Nickel JC, Teichman JM, Gregoire M, Clark J, Downey
J: Prevalence, diagnosis, characterization, and treatment of prostatitis,
interstitial cystitis, and epididymitis in outpatient urological practice:
the Canadian PIE Study. Urology. 66(5):935-40, 2005
5- Karmazyn B, Steinberg R, Livne P, Kornreich L, Grozovski
S, Schwarz M, Ziv N, Freud E: Duplex sonographic findings in children with
torsion of the testicular appendages: overlap with epididymitis and epididymoorchitis.
J Pediatr Surg. 41(3):500-4, 2006
6- Schalamon J, Ainoedhofer H, Schleef J, Singer G, Haxhija
EQ, Hollwarth ME: Management of acute scrotum in children--the impact of
Doppler ultrasound. J Pediatr Surg. 41(8):1377-80, 2006
Gastrostomy
Facilitating feeding directly to the stomach through a gastrostomy tube
can be a life saving procedure for children. The most common indications
for gastrostomy placement are the permanent or temporary need for enteral
feeding access, the need for gastric decompression, and an access route
to the esophagus for dilatations. The gastrostomy can be done open, laparoscopically
or percutaneously depending on the general health of the child and associated
medical conditions. When a child is referred for gastrostomy a reflux work-up
should be done if the child has clinical history or sign of reflux. Work-up
includes esophagogram and pH analysis. Should the work-up demonstrate reflux
an antireflux procedure is recommended. Complications associated with a
gastrotomy include those associated with the procedure such as bleeding,
leakage with peritonitis, injury to the colon, wound infection, tube malfunction
or migration leading to distal bowel obstruction. With time and use of
the gastrotomy the child can develop a gastrostomy prolapse, granuloma
formation, persistent gastrocutaneous fistula after tube removal, gastrocolic
fistulas, volvulus around a malposition tube and erosion of the gastrotomy
tube through adjacent organs. Once tube feeding is established there is
a positive impact on the lives of the child and family.
References:
1- Gauderer MW Gastrostomy techniques and devices. Surg
Clin North Am. 72(6):1285-98, 1992
2- 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
3- Sleigh G, Brocklehurst P: Gastrostomy feeding in cerebral
palsy: a systematic review. Arch Dis Child. 89(6):534-9, 2004
4- Janik TA, Hendrickson RJ, Janik JS, Landholm AE: Analysis
of factors affecting the spontaneous closure of a gastrocutaneous fistula.
J Pediatr Surg. 39(8):1197-9, 2004
5- Conlon SJ, Janik TA, Janik JS, Hendrickson RJ, Landholm
AE: Gastrostomy revision: incidence and indications. J Pediatr Surg. 39(9):1390-5,
2004
6- Hazel R: The psychosocial impact on parents of tube
feeding their child. Paediatr Nurs. 18(4):19-22, 2006
PSU Volume 28 No 02 FEBRUARY 2007
Gonadoblastoma
Gonadoblastoma is a sex cord gonadal tumor that contains both germ cell
and sex cord stromal elements. It occurs almost exclusively in sexually
abnormally individuals with gonadal dysgenesis and Y-containing cells,
while other cases occur in children with mixed gonadal dysgenesis (mosaic
45XO/46XY). The combination of the Y chromosome with a dysgenetic gonad
is all that is needed for a gonadoblastoma or dysgerminoma to develop.
The tumor is usually quite small and calcifications are common. Almost
40% of all gonadoblastomas are bilateral. The germ cell component may outgrow
the stromal elements and result in the formation of a dysgerminoma. Most
cases will appear in young female adults with history of primary amenorrhea
during teenage years and virilization. Management of gonadoblastoma consists
of removal of both dysgenetic gonads irrespective of the bilaterality of
the lesion. Because these tumors occur in up to 50% of patients with gonadal
dysgenesis early bilateral prophylactic gonadectomy should be performed.
Gonadoblastomas can exhibit either benign or malignant features, though
most cases are benign tumors that have a good prognosis after excision.
Gonadectomy can either be done open or laparoscopically. With the presence
of malignant germ cell elements, chemotherapy will be needed. Other children
at risk to develop gonadoblastoma later in life include those with Turners
and androgen insensitivity syndrome.
References:
1- Olsen MM, Caldamone AA, Jackson CL, Zinn A:
Gonadoblastoma in infancy: indications for early gonadectomy in 46XY gonadal
dysgenesis. J Pediatr Surg. 23(3):270-1, 1988
2- Gibbons B, Tan SY, Yu CC, Cheah E, Tan HL: Risk of
gonadoblastoma in female patients with Y chromosome abnormalities and dysgenetic
gonads. J Paediatr Child Health. 35(2):210-3, 1999
3- Gravholt CH, Fedder J, Naeraa RW, Muller J: Occurrence
of gonadoblastoma in females with Turner syndrome and Y chromosome material:
a population study. J Clin Endocrinol Metab. 85(9):3199-202, 2000
4- Uno T, Kazui T, Muhammad BA: Laparoscopic surgery
for gonadal dysgenesis in children. Surg Laparosc Endosc Percutan Tech.
9(2):151-5, 1999
5- Mazzanti L, Cicognani A, Baldazzi L, Bergamaschi R,
Scarano E, Strocchi S, Nicoletti A, Mencarelli F, Pittalis M, Forabosco
A, Cacciari E: Gonadoblastoma in Turner syndrome and Y-chromosome-derived
material. Am J Med Genet A. 135(2):150-4, 2005
6- Templeman CL, Fallat ME: Bening Ovarian Masses. Semm
Pediatr Surg. 14(2): 93-99, 2005
7- Bianco B, Lipay MV, Melaragno MI, Guedes AD, Verreschi
IT: Detection of hidden Y mosaicism in Turner's syndrome: importance in
the prevention of gonadoblastoma. J Pediatr Endocrinol Metab. 19(9):1113-7,
2006
Encopresis
Encopresis refers to the involuntary loss of formed, semiformed, or
liquid stools into the child's underwear in the presence of constipation
Solid fecal material accumulated in the distal rectum unable to be discharged
appropriately produces seepage of more proximally fecal fluid which escapes
unconsciously into the cloths of the child. It's a very difficult social
and physical problem to manage satisfactorily in the child. Encopresis
is a complex abnormal motility disorder, requiring a multidisciplinary
approach. The most common causes associated with encopresis consist of
slow transit functional constipation, Hirschsprung's disease and anorectal
malformations. Severely constipated children with encopresis in whom outpatient
management has failed frequently require several days of hospitalization,
as well as conventional treatments involving cathartics and enemas.
A balanced electrolyte solution of the nonabsorbable polymer polyethylene
glycol (GoLytely) offers a safe and efficient method for clearing the intestine
in such cases. Children with encopresis have normal functioning internal
sphincter and can acquire normal bowel control using biofeedback therapy
to correct the abnormal defecation dynamics. A continent appendicostomy
(Malone procedure) is a promising treatment that completely cleanses the
colon, increases the child's autonomy, and decreases the chance of soiling
in intractable cases of encopresis with pseudo-incontinence.
References:
1- Ingebo KB, Heyman MB: Polyethylene glycol-electrolyte
solution for intestinal clearance in children with refractory encopresis.
A safe and effective therapeutic program. Am J Dis Child. 142(3):340-2,
1988
2- Bulut M, Tekant G: Encopretic children: experience
with fifty cases. Turk J Pediatr. 33(3):167-72, 1991
3- Loening-Baucke V: Encopresis and soiling. Pediatr
Clin North Am. 43(1):279-98, 1996
4- Iwai N, Iwata G, Kimura O, Yanagihara J: Is a new
biofeedback therapy effective for fecal incontinence in patients who have
anorectal malformations? J Pediatr Surg. 32(11):1626-9, 1997
5- Hutson JM, McNamara J, Gibb S, Shin YM: Slow transit
constipation in children. J Paediatr Child Health. 37(5):426-30, 2001
6- Di Lorenzo C, Benninga MA: Pathophysiology of pediatric
fecal incontinence. Gastroenterology. 126(1 Suppl 1):S33-40, 2004
Torsion Fallopian Tubes
Torsion of a fallopian tube is a very rare event presenting in premenarchal
girls and postmenarchal teenagers. Factors associated with torsion include
abnormally long tube and mesosalpinx, adnexal venous congestion and abnormal
peristalsis. Other times extrinsic factors such as pelvic masses or trauma
are the principal cause of torsion. Torsion is more common in the right
fallopian tube. Diagnosis is difficult due to nonspecific symptoms. Primordial
symptoms consist of sudden abdominal pain, nausea, and vomiting. Other
times the child will develop abdominal tenderness with peritonitis. Pelvic
ultrasound with color Doppler can identify an elongated cystic mass with
variable septs and scattered internal echoes. CT-Scan can demonstrate thickened
of the affected tube with hemorrhage. The gold standard of diagnosis and
management consists of operative laparoscopy. With isolated tubal torsion
the tube can be untwisted. If torsion occurs due to a pelvic mass, the
mass should be excised. If no mass or accompanied ovarian torsion is present,
the isolated tube should be fixed to the peritoneum or cul-de-sac. Unless
a high index of suspicion is maintained for torsion of the fallopian tube
in adolescent females, this disorder may not be detected until after tubal
destruction.
References:
1- Evans JP: Torsion of the normal uterine adnexa in
premenarchal girls. J Pediatr Surg. 13(2):195-6, 1978
2- Hockberger RF, Sternbach G: Torsion of the fallopian
tube. JACEP. 7(8):315-7, 1978
3- Ghossain MA, Buy JN, Bazot M, Haddad S, Guinet C,
Malbec L, Hugol D, Truc JB, Poitout P, Vadrot D: CT in adnexal torsion
with emphasis on tubal findings: correlation with US. J Comput Assist Tomogr.
18(4):619-25, 1994
4- Rizk DE, Lakshminarasimha B, Joshi S: Torsion of the
fallopian tube in an adolescent female: a case report. J Pediatr Adolesc
Gynecol. 15(3):159-61, 2002
5- Perlman S, Hertweck P, Fallat ME: Paratubal and tubal
abnormalities. Semm Pediatr Surg 14(2): 124-134, 2005
6- Pinkert M, Klein Z, Tepper R, Beyth Y: Hydrosalpinx
with adnexal torsion in an adolescent virgin patient--A diagnostic dilemma:
case report and review of the literature. J Pediatr Adolesc Gynecol. 19(4):297-9,
2006
PSU Volume 28 No 03 MARCH 2007
Budd-Chiari Syndrome
Obstruction to the hepatic venous outflow tract is commonly known as
Budd-Chiari Syndrome. The Budd-Chiari syndrome (BCS) in children can be
the result of a congenital or acquired web in the inferior vena cava, a
thrombotic, inflammatory, neoplastic process or an hypercoagulable state
(antithrombin 3 deficiency). Hepatic venous outflow obstruction produces
hepatic dysfunction producing abdominal pain, ascites, jaundice, hepatosplenomegaly,
portal hypertension and cirrhosis. The factors that influence management
of the BCS include the state of hepatic dysfunction, type of presentation
(acute or chronic), how much venous occlusion is present and the presence
of collateral circulation. Pulsed Doppler ultrasound, venography and liver
biopsy are very helpful in diagnosis. Management of BCS in children has
included use of anticoagulation, thrombolytic therapy, angioplasty with
or without stenting, transjugular intrahepatic portosystemic shunts and
surgical portosystemic shunts. This last choice has fewer options in the
face of liver transplantation and does not improve survival. The combination
of thrombolytic therapy and balloon angioplasty is the best option in the
acute setting of BCS or during the first four weeks after development of
the syndrome. Late or chronic presentation with established hepatic cirrhosis
and portal hypertension sequelae is best managed with liver transplantation.
Early diagnosis offers the best possible chance of cure.
References:
1- Singh V, Sinha SK, Nain CK, Bambery P, Kaur U, Verma
S, Chawla YK, Singh K: Budd-Chiari syndrome: our experience of 71 patients.
J Gastroenterol Hepatol. 15(5):550-4, 2000
2- Perello A, Garcia-Pagan JC, Gilabert R, Suarez
Y, Moitinho E, Cervantes F, Reverter JC, Escorsell A, Bosch J, Rodes J:
TIPS is a useful long-term derivative therapy for patients with Budd-Chiari
syndrome uncontrolled by medical therapy. Hepatology. 35(1):132-9, 2002
3- Benesch M, Urban C, Deutschmann H, Hausegger
KA, Hollwarth M: Management of Budd-Chiari syndrome by hepatic vein stenting
after extended right hepatectomy. J Pediatr Surg. 37(11):1640-2, 2002
4- Rossle M, Olschewski M, Siegerstetter V, Berger E,
Kurz K, Grandt D: The Budd-Chiari syndrome: outcome after treatment with
the transjugular intrahepatic portosystemic shunt. Surgery. 135(4):394-403,
2004
5- Yamada T, Tanaka K, Ogura Y, Ko S, Nakajima Y, Takada
Y, Uemoto S: Surgical techniques and long-term outcomes of living donor
liver transplantation for Budd-Chiari syndrome. Am J Transplant. 6(10):2463-9,
2006
6- Cauchi JA, Oliff S, Baumann U, Mirza D, Kelly DA,
Hewitson J, Rode H, McCulloch M, Spearman W, Millar AJ: The Budd-Chiari
syndrome in children: the spectrum of management. J Pediatr Surg. 41(11):1919-23,
2006
Pyoderma Gangrenosum
Pyoderma gangrenosum is a rare, poorly understood, ulcerative skin disorder
that occurs in all age groups. A systemic disorder such as inflammatory
bowel disease (e.g., ulcerative colitis, Crohn's disease), malignancy or
juvenile rheumatoid arthritis is usually associated in almost three-fourth
of cases seen in children. Pyoderma Gangrenosum (PG) often begins as a
small pustule, but results in localized skin destruction and ulceration
which is characterized by an expanding ulceration with undermined violaceous
borders. PG characteristically involves ulceration in the buttocks, thighs
and perianal area while sparing the legs. Lower legs are the most commonly
affected sites in adults. Infants appear to have an unusual distribution
of perianal and genital lesions not often described in other age groups.
The distribution of lesions in children is similar, often involving the
lower extremities, but pyoderma gangrenosum of the head and face appears
to be more common in children. Infants may have ulcers in genital and perianal
areas. An altered immune response could be the origin of PG. Diagnosis
is established by biopsy. Histologically, lymphocytic and/or leukocytoclastic
vasculitis is present in most of the biopsy specimens obtained from the
borders of the lesions. The most frequently prescribed treatment for children
is systemic corticosteroids, which generally are very effective.
References:
1- Graham JA, Hansen KK, Rabinowitz LG, Esterly NB: Pyoderma
gangrenosum in infants and children. Pediatr Dermatol. 11(1):10-7, 1994
2- Dourmishev AL, Miteva I, Schwartz RA: Pyoderma gangrenosum
in childhood. Cutis. 58(4):257-62, 1996
3- von den Driesch P: Pyoderma gangrenosum: a report
of 44 cases with follow-up. Br J Dermatol. 137(6):1000-5, 1997
4- Mlika RB, Riahi I, Fenniche S, Mokni M, Dhaoui MR,
Dess N, Dhahri AB, Mokhtar I: Pyoderma gangrenosum: a report of 21 cases.
Int J Dermatol. 41(2):65-8, 2002
5- Dinulos JG, Darmstadt GL, Len MK, Rutledge JC, Murray
KF: Infantile Crohn disease presenting with diarrhea and pyoderma gangrenosum.
Pediatr Dermatol. 23(1):43-8, 2006
6- Koturoglu G, Vardar F, Ozkinay F, Kurugol Z, Akalin
T, Ozkinay C: Pyoderma gangrenosum in a six-month-old boy. Turk J Pediatr.
48(2):159-61, 2006
Sertoli-Leydig Ovarian Tumors
Sertoli-Leydig cell ovarian tumors are rare androgen producing tumors
causing masculinization in most girls. A few are nonfunctional tumors.
Sertoli-Leydig cell tumors used to be called arrhenoblastoma or androblastomas.
One-third of all Sertoli-Leydig cell tumors (SLCT) occurs in children.
Most SLCT are unilateral. Histologic diagnosis depends on the presence
of heterologous endodermal and mesenchymal components. The androgenic effect
of the tumor causes accelerated somatic growth and amenorrhea in prepubertal
girls. Postpubertal girls develops irregular menstrual cycles, hirsutism
and masculinization. Most affected children usually present with a pelvic
mass. Testosterone and alpha-fetoprotein produced by the tumor are used
as genetic tumor markers. Diagnosis is usually done by ultrasound or CT-Scan
in association with the masculinizing clinical picture. Management consists
of unilateral salpingo-oophorectomy. Poorly differentiated tumors might
need adjuvant chemotherapy and radiotherapy. Prognosis correlates most
meaningfully with the stage and degree of differentiation of the tumor.
High-stage tumors are all clinically malignant.
References:
1- Young RH, Scully RE: Ovarian Sertoli-Leydig cell tumors.
A clinicopathological analysis of 207 cases. Am J Surg Pathol. 9(8):543-69,
1985
2- Talerman A: Ovarian Sertoli-Leydig cell tumor (androblastoma)
with retiform pattern. A clinicopathologic study. Cancer. 60(12):3056-64,
1987
3- Larsen WG, Felmar EA, Wallace ME, Frieder R: Sertoli-Leydig
cell tumor of the ovary: a rare cause of amenorrhea. Obstet Gynecol. 79(5
( Pt 2)):831-3, 1992
4- Lantzsch T, Stoerer S, Lawrenz K, Buchmann J, Strauss
HG, Koelbl H: Sertoli-Leydig cell tumor. Arch Gynecol Obstet. 264(4):206-8,
2001
5- Chen FY, Sheu BC, Lin MC, Chow SN, Lin HH: Sertoli-Leydig
cell tumor of the ovary. J Formos Med Assoc. 103(5):388-91, 2004
6- Schneider DT, Calaminus G, Harms D, Gobel U; German
Maligne Keimzelltumoren Study Group: Ovarian sex cord-stromal tumors in
children and adolescents. J Reprod Med. 50(6):439-46, 2005
PSU Volume 28 No 04 APRIL 2007
Gallstone Ileus
Gallstone ileus is a very rare disorder characterized by mechanical
obstruction of the gastrointestinal tract due to incipient impacted gallstones
that passes through a bilio-enteric fistula. Impaction followed by obstruction
can occur at the ileum, duodenum or stomach (Bouveret's syndrome). Diagnosis
is usually delayed due to lack of specific signs of biliary disease. The
classic triad of Rigler (small bowel obstruction, ectopic gallstones and
air in the biliary tree) is visualized on abdominal plain films in only
one-thirds of cases. Age ranges from 13 to 87 years with most cases seen
in older patients. Most bilio-enteric fistulas are cholecystoduodenal type,
with a few choledochoduodenal. Work-up includes ultrasound, upper gastrointestinal
series with water soluble contrast medium and contrast enhanced computed
tomography (CT). Preoperative diagnosis of gallstone ileus significantly
increases by combining plain film and US findings. Management consists
initially of simple enterotomy (enterolithotomy) which can be done laparoscopically
assisted. This is followed by takedown of the bilioenteric fistula and
cholecystectomy in a later stage procedure if the medical condition of
the patient permits and he continues symptomatic. Some reports encourage
enterolithotomy, repair of the fistula and cholecystectomy in one procedure.
Other workers report that enterolithotomy alone is adequate treatment in
the elderly, and subsequent cholecystectomy is not mandatory. Early diagnosis
and treatment improve the outcome.
References:
1- Kasahara Y, Umemura H, Shiraha S, Kuyama T, Sakata
K, Kubota H: Gallstone ileus. Review of 112 patients in the Japanese literature.
Am J Surg. 140(3):437-40, 1980
2- Lobo DN, Jobling JC, Balfour TW: Gallstone ileus:
diagnostic pitfalls and therapeutic successes. J Clin Gastroenterol. 30(1):72-6,
2000
3- Ripolles T, Miguel-Dasit A, Errando J, Morote V, Gomez-Abril
SA, Richart J: Gallstone ileus: increased diagnostic sensitivity by combining
plain film and ultrasound. Abdom Imaging. 26(4):401-5, 2001
4- Agresta F, Bedin N: Gallstone ileus as a complication
of acute cholecystitis. Laparoscopic diagnosis and treatment. Surg Endosc.
16(11):1637, 2002
5- Doko M, Zovak M, Kopljar M, Glavan E, Ljubicic N,
Hochstadter H: Comparison of surgical treatments of gallstone ileus: preliminary
report. World J Surg. 27(4):400-4, 2003
6- Masannat Y, Masannat Y, Shatnawei A: Gallstone ileus:
a review. Mt Sinai J Med. 73(8):1132-4, 2006
7- Nichol PF, Adzick NS: Gallstone ileus 20 years after
a Kasai procedure using a stapled antireflux
valve. J Pediatr Surg. 42(1):264-6, 2007
Laparoscopic Gastrostomy
During the past 20 years the number of children without associated surgical
pathology needing a feeding gastrostomy has increased considerably. The
most common indications to construct a gastrostomy are a permanent or temporal
need for enteral feeding access, need for gastric decompression or an access
route to the esophagus for controlled dilatations. The gastrostomy can
be constructed using an open, percutaneous or laparoscopic technique. In
addition when the child has previous gastric surgery or surgery in the
upper abdomen, is obese, or an anatomic distortion of the body such as
kyphoscoliosis precluding percutaneous placement of a gastrostomy the procedure
can be done laparoscopically completely or laparoscopic-assisted percutaneously
endoscopic gastrostomy. The lap procedure uses a two-trocars technique,
stomach is insufflated and fasteners (T-anchors) or sutures are passed
through the abdominal wall to fix the stomach. Using seldinger technique
a needle is passed to the stomach followed by a guide wire and progressive
dilatations until finally a properly sized gastrostomy tube is passed and
the balloon inflated. Alternatively, the child can undergo a percutaneous
placement of the gastrostomy under laparoscopic vision to avoid complications
such as colon perforation in unique anatomic distorted children.
References:
1- Kellnar ST, Till H, Bohn R: Laparoscopically assisted
performance of gastrostomy--a simple, safe and minimal invasive technique.
Eur J Pediatr Surg. 9(5):297-8, 1999
2- Tomicic JT, Luks FI, Shalon L, Tracy TF: Laparoscopic
gastrostomy in infants and children. Eur J Pediatr Surg. 12(2):107-10,
2002
3- Zamakhshary M, Jamal M, Blair GK, Murphy JJ, Webber
EM, Skarsgard ED: Laparoscopic vs percutaneous endoscopic gastrostomy tube
insertion: a new pediatric gold standard? J Pediatr Surg. 40(5):859-62,
2005
4- Yu SC, Petty JK, Bensard DD, Partrick DA, Bruny JL,
Hendrickson RJ: Laparoscopic-assisted percutaneous endoscopic gastrostomy
in children and adolescents. JSLS. 9(3):302-4, 2005
5- Backman T, Arnbjornsson E, Berglund Y, Larsson LT:
Video-assisted gastrostomy in infants less than 1 year. Pediatr Surg Int.
22(3):243-6, 2006
6- Kawahara H, Kubota A, Okuyama H, Shimizu Y, Watanabe
T, Tani G, Hiroaki Y, Okada A: One-trocar laparoscopy-aided gastrostomy
in handicapped children. J Pediatr Surg. 41(12):2076-80, 2006
Feeding Jejunostomy
Neurologically impaired children benefit from receiving alimentation
directly into the stomach. On occasion due to gastric emptying dysfunction,
severe retching, or unmanageable recurrent gastroesophageal reflux after
failed fundoplication the need for a feeding alternative arises. Such alternative
could be feeding directly into the jejunum while venting the stomach. Feeding
directly to the jejunum can be done with transpyloric gastrojejunal tube
placement, catheter or needle jejunostomy, transgastric jejunostomy through
a preexisting gastrostomy, or creating an open roux-en-y tube feeding jejunostomy.
The most significant complications are prolapse, leakage and perforation
of the stoma. Feeding does not have to be elemental diet only. Children
with unmanageable seizure activity in need of multiple drug therapy might
not benefit of jejunostomy feeding. The roux-en-y jejunostomy can be performed
laparoendoscopically. The use of gastrojejunostomy tubes can be hampered
by frequent need of tube manipulation, tube brokage, blockaded or dislodgement.
References:
1- DeCou JM, Shorter NA, Karl SR: Feeding Roux-en-Y jejunostomy
in the management of severely neurologically impaired children. J Pediatr
Surg. 28(10):1276-9, 1993
2- Yoshida NR, Webber EM, Gillis DA, Giacomantonio JM:
Roux-en-Y jejunostomy in the pediatric population. J Pediatr Surg. 31(6):791-3,
1996
3- Gilchrist BF, Luks FI, DeLuca FG, Wesselhoeft CW Jr:
A modified feeding Roux-en-Y jejunostomy in the neurologically damaged
child. J Pediatr Surg. 32(4):588-9, 1997
4- Langer JC, Mazziotti MV, Winthrop AL: Roux-en-Y jejunostomy
button in infants. Pediatr Surg Int. 16(1-2):40-2, 2000
5- Neuman HB, Phillips JD: Laparoscopic Roux-en-Y feeding
jejunostomy: a new minimally invasive surgical procedure for permanent
feeding access in children with gastric dysfunction. J Laparoendosc Adv
Surg Tech A. 15(1):71-4, 2005
6- Raval MV, Phillips JD: Optimal enteral feeding in
children with gastric dysfunction: surgical
jejunostomy vs image-guided gastrojejunal tube placement.
J Pediatr Surg. 41(10):1679-82, 2006
PSU Volume 28 No 05 MAY 2007
Portal Hypertension
Portal hypertension (PH) in children is caused by increased portal venous
flow from such conditions as hemangiomas or hepatic arterioportal fistulas,
or by increase resistance to flow from conditions such as portal vein thrombosis,
liver cirrhosis, congenital fibrosis, biliary atresia, neonatal hepatitis
or hepatic vein thrombosis. In children, extrahepatic obstruction due to
portal vein thrombosis is the most common cause. Most common presentation
of PH is upper gastrointestinal bleeding from esophageal or gastric varices,
followed by splenomegaly with hypersplenism. Diagnostic studies include
liver function tests, upper endoscopy, color Doppler US, splenoportography
and MRI. Initial management of PH can entail the use of vasoactive beta
blockers such as propanolol or somatostatin. Bleeding varices can be managed
with banding or sclerotherapy. Children with favorable liver function,
but unfavorable anatomy and continuous variceal bleeding can benefit from
a devascularization procedure. Those with unfavorable liver function and
bleeding can benefit from transjugular intrahepatic portosystemic shunt
(TIPS), though shunt thrombosis is a problem the smaller the kid. Children
with favorable anatomy can benefit from a distal splenorenal (or splenoadrenal)
shunt, or a makeshift shunt such as the Rex shunt between the inferior
mesenteric vein and a branch of the portal vein high in the hepatic hilum
using autologous vein graft. Liver transplantation is the treatment of
choice for children with PH complicating end-stage liver cirrhosis.
References:
1- Karrer FM: Portal hypertension. Semin Pediatr Surg.
1(2):134-44, 1992
2- Maksoud JG, Goncalves ME: Treatment of portal hypertension
in children. World J Surg. 18(2):251-8, 1994
3- Karrer FM, Narkewicz MR: Esophageal varices: current
management in children. Semin Pediatr Surg. 8(4):193-201, 1999
4- Ryckman FC, Alonso MH: Causes and management of portal
hypertension in the pediatric population. Clin Liver Dis. 5(3):789-818,
2001
5- Ling SC: Should children with esophageal varices receive
beta-blockers for the primary prevention of variceal hemorrhage? Can J
Gastroenterol. 19(11):661-6, 2005
6- Schettino GC, Fagundes ED, Roquete ML, Ferreira AR,
Penna FJ: Portal vein thrombosis in children and adolescents. J Pediatr
(Rio J). 82(3):171-8, 2006
Breast Papilloma
Breast juvenile papilloma in children is a rare benign lesion featuring
atypical papillary duct hyperplasia and numerous cysts. They manifest clinically
as a localized, multinodular mass that is usually interpreted as a juvenile
fibroadenoma. Most cases occur in females, though some cases in males have
been reported. Mean age of diagnosis occurs during the late adolescent
years. Left breast is affected slightly more often than the right. Patterns
of menarche, marital history, parity, and use of birth control pills are
not exceptional for women in this age group. No instance is found of maternal
use of estrogens during pregnancy. Family history of breast carcinoma is
seen in one-thirds of all cases of papillomatosis. Juvenile secretory carcinoma
can be associated with papillomatosis. Breast ultrasonography will show
an ill-defined, inhomogeneous mass with numerous small, hypoechoic areas,
but cannot differentiate a fibroadenoma from papilloma. Excisional biopsy
through a periareolar incision will establish the diagnosis. Should a secretory
carcinoma be found wide local excision is warranted. Due to the precancerous
nature of papillomatosis, long-term yearly follow-up is recommended.
References:
1- Rosen PP, Holmes G, Lesser ML, Kinne DW, Beattie EJ:
Juvenile papillomatosis and breast carcinoma. Cancer. 55(6):1345-52, 1985
2- Ferguson TB Jr, McCarty KS Jr, Filston HC: Juvenile
secretory carcinoma and juvenile papillomatosis: diagnosis and treatment.
J Pediatr Surg. 22(7):637-9, 1987
3- Batchelor JS, Farah G, Fisher C: Multiple breast papillomas
in adolescence. J Surg Oncol. 54(1):64-6, 1993
4- Rice HE, Acosta A, Brown RL, Gutierrez C, Alashari
M, Mintequi D, Rodriguez A, Chavarrfa O, Azizkhan RG: Juvenile papillomatosis
of the breast in male infants: two case reports. Pediatr Surg Int. 16(1-2):104-6,
2000
5- Ohlinger R, Schwesinger G, Schimming A, Kohler G,
Frese H: Juvenile papillomatosis (JP) of the female breast (Swiss Cheese
Disease) -- role of breast ultrasonography. Ultraschall Med. 26(1):42-5,
2005
6- Sonmez K, Turkyilmaz Z, Karabulut R, Demirogullari
B, Ozen IO, Moralioglu S, Basaklar AC, Kale N: Surgical breast lesions
in adolescent patients and a review of the literature. Acta Chir Belg.
106(4):400-4, 2006
Pelvic Inflammatory Disease
Acute pelvic inflammatory disease (PID) is a major gynecologic health
problem in the USA, afflicting more than 1 million women each year. PID
continues to be a common diagnosis among adolescent girls presenting with
low abdominal pain. Adolescents have a higher rate of diagnosis of PID
than any other age group. PID is an ascending polymicrobial infection
affecting the upper genital tract. Risk factors associated to PID include
young age, age at first intercourse, multiple sex partners, the presence
of bacterial vaginosis, vaginal douching, the use of an intrauterine contraceptive
device, and a history of a sexually transmitted disease. Classic symptoms
of pain, fever, and a history of high-risk sexual behavior, is easily diagnosed
with a high degree of specificity in PID. Unfortunate, most females with
PID demonstrate atypical symptoms which sometimes mimic appendicitis discovering
the disease during the appendectomy. Abnormal vaginal discharge full of
neutrophils is an indicator of PID, along with a positive vaginal culture
for Chlamydia or Gonorrhea. Management of PID entails the use of broad-spectrum
antibiotics, which represent the cornerstone of therapy and must adequately
cover the polymicrobial spectrum of pathogens implicated in this infection,
which includes Neisseria gonorrhoeae, Chlamydia trachomatis, and specific
cervicovaginal anaerobic and aerobic bacteria. Sequelae associated
with PID includes infertility, ectopic pregnancy, and chronic pelvic pain
syndromes. The sexual partner of the affected patient should also be treated.
References:
1- Soper DE: Surgical considerations in the diagnosis
and treatment of pelvic inflammatory disease. Surg Clin North Am. 71(5):947-62,
1991
2- Quan M: Pelvic inflammatory disease: diagnosis and
management. J Am Board Fam Pract. 7(2):110-23, 1994
3- Blythe MJ: Pelvic inflammatory disease in the adolescent
population. Semin Pediatr Surg. 7(1):43-51, 1998
4- Patel DR: Management of pelvic inflammatory disease
in adolescents. Indian J Pediatr. 71(9):845-7, 2004
5- Banikarim C, Chacko MR: Pelvic inflammatory disease
in adolescents. Semin Pediatr Infect Dis. 16(3):175-80, 2005
6- Song AH, Advincula AP: Adolescent chronic pelvic pain.
J Pediatr Adolesc Gynecol. 18(6):371-7, 2005
PSU Volume 28 No 06 JUNE 2007
Vaginal Bleeding
Vaginal bleeding in the pre-menstrual female infant is cause for concern
both medically and socially. Differential diagnosis of vaginal bleeding
in this age group includes estrogen stimulation, vulvovaginitis, tumors
of the lower genital tract, ovarian tumors, foreign bodies, or trauma.
Transplacental estrogen stimulation can cause self-limited vaginal bleeding
in newborns during the first two weeks of life. Vulvovaginitis is the most
common gynecological infection in children caused by ascending enteric
organisms due to poor hygiene and is managed with systemic antibiotics.
Condylomas can cause painless vaginal bleeding. Tumors of the genital tract
associated with vaginal bleeding include hemangiomas of the vulva, arteriovenous
malformation of the uterus, rhabdomyosarcoma botryoid (the most common
malignant tumor of the low genital tract in young females), endodermal
sinus tumors of the vagina and endometrial carcinomas. Functional
ovarian or adrenal tumors that produce estrogen can be associated with
sexual precocity and vaginal bleeding. Foreign bodies in the vagina
of a small girl produce local inflammation resulting in a foul smelling
discharge which can be serosanguineous. The debris (foreign body) is often
wads of toilet paper. Redundant urethral mucosa may prolapse through
the urethral meatus and present as a friable polypoid lesion. Finally genital
injury is a major cause of vaginal bleeding including those associated
with child sexual abuse.
References:
1- Imai A, Horibe S, Tamaya T: Genital bleeding in premenarcheal
children. Int J Gynaecol Obstet. 49(1):41-5, 1995
2- Merritt DF: Evaluation of vaginal bleeding in the
preadolescent child. Semin Pediatr Surg. 7(1):35-42, 1998
3- Aribarg A, Phupong V: Vaginal bleeding in young children.
Southeast Asian J Trop Med Public Health. 34(1):208-12, 2003
4- Sugar NF, Graham EA: Common gynecologic problems in
prepubertal girls. Pediatr Rev. 27(6):213-23, 2006
5- Striegel AM, Myers JB, Sorensen MD, Furness PD, Koyle
MA: Vaginal discharge and bleeding in girls younger than 6 years. J Urol.
176(6 Pt 1):2632-5, 2006
Vocal Cord Paralysis
Unilateral or bilateral vocal cord paralysis (VCP) in children can be
associated with ventricular septal defects, enlargement of the auricle,
abnormalities of the great vessels, patent ductus arteriosus, or during
operations for division of cervical tracheo-esophageal fistulas and surgery
for congenital heart abnormalities or the neck (thyroidectomy). Most cases
are iatrogenic with the left recurrent laryngeal nerve with a longer anatomic
course being affected more often. VCP is the second most common cause of
neonatal stridor. The baby with unilateral VCP will show a weak, breathy
cry, aspiration and cyanotic attacks with choking during feeding. Bilateral
damage produces abduction of the cords, constant stridor and cyanotic attacks
needing a tracheostomy. Diagnosis is made with direct video-laryngoscopy.
Management strategies should be individualized and focus on maintenance
of a safe and stable airway, acquisition of intelligible speech, and deglutition
without aspiration. Unilateral VCP treatment is conservative including
thickened feeding and anti-reflux measures. Laryngeal incompetence can
be managed with injectable collagen. Irrespective of cause, morbidity associated
with unilateral VCP is minimal. Although tracheotomy is not required, careful
airway observation is important. Should tracheostomy be constructed vocal
cord lateralization procedures with partial arytenoidectomy afford the
highest operation-specific decannulation rate.
References:
1- Benjamin BN, Gray SD, Bailey CM: Neonatal vocal cord
paralysis. Head Neck. 15(2):169-72, 1993
2- Zbar RI, Smith RJ: Vocal fold paralysis in infants
twelve months of age and younger. Otolaryngol Head Neck Surg. 114(1):18-21,
1996
3- de Jong AL, Kuppersmith RB, Sulek M, Friedman EM:
Vocal cord paralysis in infants and children. Otolaryngol Clin North Am.
33(1):131-49, 2000
4- Hartnick CJ, Brigger MT, Willging JP, Cotton RT, Myer
CM 3rd: Surgery for pediatric vocal cord paralysis: a retrospective review.
Ann Otol Rhinol Laryngol. 112(1):1-6, 2003
5- Patel NJ, Kerschner JE, Merati AL: The use of injectable
collagen in the management of pediatric vocal unilateral fold paralysis.
Int J Pediatr Otorhinolaryngol. 67(12):1355-60, 2003
6- Miyamoto RC, Parikh SR, Gellad W, Licameli GR: Bilateral
congenital vocal cord paralysis: a 16-year institutional review. Otolaryngol
Head Neck Surg. 133(2):241-5, 2005
Stings
The insects that inflict more venous stings than any other in children
are the bees and ants. Stings from bees and wasps produce a local tissue
reaction with a wheal and flair. Symptoms develop within twenty minutes
of the sting and include urticaria, syncope and respiratory distress. Most
serious sequelae is anaphylaxis which occur when the child has been previously
inoculated. More than 500 stings are needed to cause death in a child.
Management is local and systemic. The venom can be removed if the event
has less than 20 minutes. Cold compresses will reduce pain associated with
the sting and baking soda helps with the itching. Systemic support includes
airway control, alpha agonists medication, inhaled beta agonist for bronchospasm
and calcium for muscle spasms. Best prophylaxis is reducing exposure. Fire
ants' sting can produce edema, pruritus, erythema, pain and burning with
a characteristic wheal. Wound is cleaned with soap and water. Rarely systemic
management is needed.
References:
1- Stawiski MA: Insect bites and stings. Emerg Med Clin
North Am. 3(4):785-808, 1985
2- Solley GO: Allergy to stinging and biting insects
in Queensland. Med J Aust. 153(11-12):650-4, 1990
3- Schultze-Werninghaus C, Wahn U, Niggemann B: Evaluation
of the risk of anaphylactic reactions by wasp venom-extract challenges
in children. Pediatr Allergy Immunol. 10(2):133-7, 1999
4- Cohen PR: Imported fire ant stings: clinical manifestations
and treatment. Pediatr Dermatol. 9(1):44-8, 1992
5- Nguyen SA, Napoli DC: Natural history of large local
and generalized cutaneous reactions to imported fire ant stings in children.
Ann Allergy Asthma Immunol. 94(3):387-90, 2005