PEDIATRIC SURGERY UPDATE ©
VOLUME 33, 2009
PSU Volume 33 No 01 JULY 2009
Benign Mesothelioma
Benign cystic mesothelioma (BCM) also
known as cystic mesothelioma of the peritoneum is a rare abdominal
tumor found in young women during their third decade of life. It
originates from the pelvic retroperitoneum with a predilection for
pelvic visceral serosal surfaces. It a neoplastic tumor with a high
tendency for recurrence not associated to asbestos exposure. The tumor
produce cysts that are usually intraperitoneal in location but also the
chest and pericardium can be affected. Clinically the child presents
with progressive painless abdominal distension. Most reliable method of
preoperative diagnosis is with aspiration and cellular analysis of
peritoneal washing showing abundant mesothelial cells. The role of
immunohistochemistry and electron microscopy in diagnosis can be
important. CT-Scan provides an idea of the extension of the benign
tumor. The differential diagnosis includes a cystic lymphangioma. BCM
shows multiplicity, extensive involvement of the peritoneal and serosal
surfaces without significant organ invasion. Management of BCM is
surgical excision. The cysts appear almost avascular. Multiple
resections are common due to the recurrence rate of the tumor.
Prognosis depends on the rate of recurrence and postoperative
complications from multiple abdominal procedures.
References:
1- Raafat F, Egan M: Benign cystic mesothelioma of the peritoneum: immunohistochemical and
ultrastructural features in a child. Pediatr Pathol. 8(3):321-9, 1988
2- Hanukoglu A, Gewurtz G, Zaidel L, Krispin M, Fried D: Benign cystic
mesothelioma of the peritoneum: the occurrence of an adult entity in a
child. Med Pediatr Oncol. 20(2):169-71, 1992
3- McCullagh M, Keen C, Dykes E: Cystic mesothelioma of the peritoneum:
a rare cause of 'ascites' in children. J Pediatr Surg. 29(9):1205-7,
1994
4- Scattone A, Pennella A, Giardina C, Marinaccio M, Ricco R, Pollice
L, Serio G: Polycystic mesothelioma of the peritoneum. Description of 4
cases. Pathologica. 93(5):549-55, 2001
5- Terry NE, Fowler CL: Benign cystic mesothelioma in a child. J Pediatr Surg. 44(5):e9-11, 2009
Anaplastic Thyroid Cancer
Anaplastic or undifferentiated thyroid
cancer occurs very rarely during young adults and children. Most cases
of thyroid malignancy are well differentiated papillary and follicular
variants. Other times medullary thyroid carcinoma associated with
multiple endocrine neoplasia syndromes. Nevertheless it's good to learn
what is the characteristic of this unusual disease in young adults and
children. Poorly-differentiated thyroid carcinoma is a stage in the
development of anaplastic carcinoma from well-differentiated neoplastic
transformation of follicular epithelium. The BRAFT1799A mutation has
been identified. In patients under 40 years of age, encapsulated
poorly-differentiated tumors are more frequent with a trabecular
histological pattern alone with a much smaller size (30 mm). Pathologic
criteria used in the diagnosis of poorly or undifferentiated thyroid
carcinoma includes (1) presence of a solid/trabecular/insular pattern
of growth, (2) absence of the conventional nuclear features of
papillary carcinoma, and (3) presence of at least one of the following
features: convoluted nuclei; mitotic activity >or =3 x 10 HPF; and
tumor necrosis. Among papillary carcinomas, the frequency of a solid
growth pattern, a criterion for classifying a tumor as poorly
differentiated, was higher in the Belarus region of Russia than that in
Japan. Management of anaplastic thyroid malignancy is total
thyroidectomy, since chemotherapy, radiotherapy and radioiodine are of
no avail.
References:
1- Datta C, Bhattacharyya S, Ghosh A, Ghosh S: Dedifferentiated
papillary carcinoma of thyroid in an adolescent girl--a case report.
Indian J Pathol Microbiol. 48(4):496-7, 2005
2- Volante M, Collini P, Nikiforov YE, Sakamoto A, Kakudo K, Katoh R,
Lloyd RV, LiVolsi VA, Papotti M, Sobrinho-Simoes M, Bussolati G, Rosai
J: Poorly differentiated thyroid carcinoma: the Turin proposal for the
use of uniform diagnostic criteria and an algorithmic diagnostic
approach. Am J Surg Pathol. 31(8):1256-64, 2007
3- Shirahige Y, Ito M, Ashizawa K, Motomura T, Yokoyama N, Namba H,
Fukata S, Yokozawa T, Ishikawa N, Mimura T, Yamashita S, Sekine I, Kuma
K, Ito K, Nagataki S: Childhood thyroid cancer: comparison of Japan and
Belarus. Endocr J. 45(2):203-9, 1998
4- Kumagai A, Namba H, Mitsutake N, Saenko VA, Ohtsuru A, Ito M, Noh
JY, Sugino K, Ito K, Yamashita S: Childhood thyroid carcinoma with
BRAFT1799A mutation shows unique pathological
features of poor differentiation. Oncol Rep. 16(1):123-6, 2006
5- Gétaz EP, Shimaoka K, Rao U: Anaplastic carcinoma of the
thyroid following external irradiation. Cancer. 43(6):2248-53, 1979
Genital Tumors
Tumors occurring in the vulva and
external vaginal orifice are rare to find in female children. In the
area of the vulva the most common tumor is either and hemangioma,
lymphangioma, lipomas, neurofibromatosis and vulvar intraepithelial
neoplasia. Hemangiomas resolve with conservative therapy, while
lymphangiomas or other type of tumor will require surgical excision.
Vulva intraepithelial neoplasia are associated with cases of sexual
abuse and human papilloma viral infections. In the external vaginal
orifice bleeding hemangiomas have been previously reported in children.
Cavernous hemangiomas with brisk and continuous bleeding will require
some form of therapy such as cryosurgery, excision or steroid
injection. Interferon has also been used effectively. It is always
important to study with imaging (MRI) the extension of the perineal
hemangioma. Another lesion of importance in this privilege area is the
vaginal rhabdomyosarcoma which presents with protrusion and a bleeding
mass. Management consists of a biopsy, chemotherapy followed by surgery
if necessary.
References:
1- Breen JL, Bonamo JF, Maxson WS: Genital tract tumors in children. Pediatr Clin North Am. 28(2):355-67, 1981
2- Imai A, Furui T, Tamaya T: Gynecologic tumors and symptoms in
childhood and adolescence; 10-years' experience. Int J Gynaecol Obstet.
45(3):227-34, 1994
3- Martelli H, Oberlin O, Rey A, Godzinski J, Spicer RD, Bouvet N,
Haie-Meder C, Terrier-Lacombe MJ, Sanchez de Toledo J, Spooner D,
Sommelet D, Flamant F, Stevens MC: Conservative treatment for girls
with nonmetastatic rhabdomyosarcoma of the genital tract: A report from
the Study Committee of the International Society of Pediatric
Oncology. J Clin Oncol. 17(7):2117-22, 1999
4- Groff DB: Pelvic neoplasms in children. J Surg Oncol. 77(1):65-71, 2001
5- Monk BJ, Tewari KS: The spectrum and clinical sequelae of human
papillomavirus infection. Gynecol Oncol. 107(2 Suppl 1):S6-13, 2007
PSU Volume 33 No 02 AUGUST 2009
MIS for Children Cancer
Minimal invasive surgical (MIS)
procedures through the use of laparoscopy and thoracoscopy have
evolved slowly in the actual management of children with solid
abdominal tumors and cancer in other sites of the body. The main
indication is biopsy or simply determination of resectability of large
abdominal tumors not amenable to immediate surgical therapy, along with
thoracoscopic biopsy of lung metastasis. Adequate tissue with minimal
surgical trauma can be obtained in most of these children. In the case
of neuroblastoma, thoracoscopy has been also useful in resection of
residual tumors and primary tumors of the posterior mediastinum. The
efficacy of laparoscopic adrenalectomy for metastatic lesions,
benign tumors, and small to medium neuroblastomas (two to 4 cm in
diameter) is well established. Trocar site metastasis can be averted by
retrieving the specimen within an endobag. Ovarian tumors are amenable
to laparoscopic resection following strict protocol observation and
managing of tissue extraction. The length of hospital stay, time
to start postoperative feeding, time to start postoperative
chemotherapy and postop discomfort (postoperative pain,
analgesics requirement, postoperative ileus) is significantly
shorter in the group of patients who undergoes MIS procedures. Role of
MIS in Wilm's tumor, rhabdomyosarcoma and hepatoblastoma is limited to
biopsy and staging. MIS can be used safely and successfully to diagnose
children with suspicious solid neoplasms.
References:
1- Sailhamer E, Jackson CC, Vogel AM, Kang S, Wu Y, Chwals WJ,
Zimmerman BT, Hill CB, Liu DC: Minimally invasive surgery for pediatric
solid neoplasms. Am Surg. 69(7):566-8, 2003
2- Iwanaka T, Arai M, Kawashima H, Kudou S, Fujishiro J, Imaizumi S,
Yamamoto K, Hanada R, Kikuchi A, Aihara T, Kishimoto H: Endosurgical
procedures for pediatric solid tumors. Pediatr Surg Int. 20(1):39-42,
2004
3- Saad DF, Gow KW, Milas Z, Wulkan ML: Laparoscopic adrenalectomy for
neuroblastoma in children: a report of 6 cases. J Pediatr Surg.
40(12):1948-50, 2005
4- Iwanaka T, Kawashima H, Uchida H: The laparoscopic approach of neuroblastoma. Semin Pediatr Surg. 16(4):259-65, 2007
5- Leclair MD, Sarnacki S, Varlet F, Heloury Y: Minimally-invasive surgery in cancer children. Bull Cancer. 94(12):1087-90, 2007
6- Chan KW, Lee KH, Tam YH, Yeung CK: Minimal invasive surgery in
pediatric solid tumors. J Laparoendosc Adv Surg Tech A. 17(6):817-20,
2007
Splenic Trauma: Embolization
The spleen is the most commonly
injured visceral organ in blunt abdominal trauma in both adults and
children. Most children with splenic trauma are managed conservatively
and the need for surgical intervention is very rarely utilized. When
the need for surgery arises splenic perservation is tried by all means.
For such purpose various suture techniques, biomaterials and resorbable
protheses are utilized. Splenic artery embolization (SAE) is another
useful technique in the management of blunt splenic injury. SAE is
performed when patients has the following CT angiographic criteria: (1)
extravasation of contrast material extending beyond or within the
splenic parenchyma, (2) arterial disruption or major arteriovenous
fistula, or both. Major complications occurs in 25% of the SAE-treated
patients and included total splenic infarction, splenic atrophy, and
postprocedure bleeding. Minor complications are more common and
included fever, pleural effusion, and partial splenic infarction.
Proximal splenic artery embolization in children may be a safe
therapeutic alternative to either conservative or surgical management
in spontaneous splenic rupture and even after delayed rupture.
Preservation of splenic tissue with a reduced risk of repeated
hemorrhage can be obtained with proximal splenic artery embolization.
Splenic salvage rate goes beyond the 90% in reported series.
References:
1- Uroz Tristan J, Poenaru D, Martinez Lagares F, Leclerc S, Sanchis
Solera L: Selective splenic artery embolization or use of polyglycolic
acid mesh in children with severe splenic trauma. Eur J Pediatr Surg.
5(5):310-2, 1995
2- Hagiwara A, Yukioka T, Ohta S, Nitatori T, Matsuda H, Shimazaki S:
Nonsurgical management of patients with blunt splenic injury: efficacy
of transcatheter arterial embolization.AJR Am J Roentgenol.
167(1):159-66, 1996
3- Naess PA, Gaarder C, Dormagen JB: Nonoperative management of
pediatric splenic injury with angiographic embolization.J Pediatr Surg.
40(11):e63-4, 2005
4- Wu SC, Chen RJ, Yang AD, Tung CC, Lee KH: Complications associated
with embolization in the treatment of blunt splenic injury.World J
Surg. 32(3):476-82, 2008
5- Raikhlin A, Baerlocher MO, Asch MR, Myers A: Imaging and
transcatheter arterial embolization for traumatic splenic injuries:
review of the literature. Can J Surg. 51(6):464-72, 2008
6- Maurer SV, Denys A, Lutz N: Successful embolization of a delayed
splenic rupture following trauma in a child. J Pediatr Surg.
44(6):E1-4, 2009
Artificial Anal Sphincter
Fecal incontinence is a devastating
social and psychological problem in children and adults. Several
methods to manage this condition includes biofeedback, bowel mechanical
cleansing training, dynamic graciloplasty, permanent stoma, sacral
neuromodulation and implantation of an artificial anal sphincter (AAS).
The success rate of AAS is approximately 75%. The artificial anal
sphincter restores continence to solid stools in almost all severely
incontinent patients, two-thirds of whom achieve practically normal
continence. Infection has been the most serious complication, but a
number of technical complications (cuff broken, rectal erosion,
difficulty in evacuating) related to the device have also occurred and
required revisional procedures in up to 60% of the patients. The late
complications (infection and skin erosion) are the main cause of device
explantation. Although morbidity and the need for revisional surgery
are high, after artificial sphincter implantation anal incontinence and
quality of life improve significantly. Recently, a novel artificial
anal sphincter system with sensor feedback based on transcutaneous
energy transmission was developed.
References:
1- Christiansen J: The artificial anal sphincter. Can J Gastroenterol. 14 Suppl D:152D-154D, 2000
2- Lehur PA, Zerbib F, Neunlist M, Glemain P, Bruley des Varannes S:
Comparison of quality of life and anorectal function after artificial
sphincter implantation. Dis Colon Rectum. 45(4):508-13, 2002
3- Wong WD, Congliosi SM, Spencer MP, Corman ML, Tan P, Opelka FG,
Burnstein M, Nogueras JJ, Bailey HR, Devesa JM, Fry RD, Cagir B,
Birnbaum E, Fleshman JW, Lawrence MA, Buie WD, Heine J, Edelstein PS,
Gregorcyk S, Lehur PA, Michot F, Phang PT, Schoetz DJ, Potenti F, Tsai
JY: The safety and efficacy of the artificial bowel sphincter for fecal
incontinence: results from a multicenter cohort study. Dis Colon
Rectum. 45(9):1139-53, 2002
4- O'Brien PE, Dixon JB, Skinner S, Laurie C, Khera A, Fonda D: A
prospective, randomized, controlled clinical trial of placement of the
artificial bowel sphincter (Acticon Neosphincter) for the control of
fecal incontinence. Dis Colon Rectum. 47(11):1852-60, 2004
5- Tan EK, Vaizey C, Cornish J, Darzi A, Tekkis PP: Surgical strategies
for faecal incontinence--a decision analysis between dynamic
graciloplasty, artificial bowel sphincter and end stoma. Colorectal
Dis. 10(6):577-86, 2008.
6- CarmonaSafioleas M, Andromanakos N, Lygidakis N: Anorectal
incontinence: therapeutic strategy of a complex surgical problem.
Hepatogastroenterology. 55(85):1320-6, 2008
7- Zan P, Yan G, Liu H, Luo N, Zhao Y: Adaptive transcutaneous power
delivery for an artificial anal sphincter system. J Med Eng Technol.
33(2):136-41, 2009
PSU Volume 33 No 03 SEPTEMBER 2009
Waardenburg Syndrome
Waardenburg syndrome (WS) is an
autosomical recessive or dominant trait condition derived from the
neural crests and includes sensorineural hearing loss (bilateral or
unilateral), and pigmentation disorder of the hair, eyes and skin (iris
heterochromia, white forelock, lateral displacement of inner canthi of
the eyes). Four types of the syndrome have been described. Though very
rare, WS can be associated with Hircshsprung's disease
(Shaw-Waardenburg syndrome). Type IV WS is a term used to denote
intestinal aganglionosis associated with the Waardenburg complex,
consisting of hypopigmented spots on the skin, heterochromia irides,
depigmented ocular fundus, telecanthus, and sensory deafness. The
aganglionosis associated with WS is usually total colonic with
extensive involvement of ileus. Clinically these affected babies
present with intestinal obstruction findings such as bilious vomiting,
abdominal distension, and inability to feed orally from the first few
days of life. Mutations in the endothelin-3 endothelin B receptor, and
SOX10 genes have been identified as causative genes of WS. The length
of the aganglionic segment decides the prognosis of these children. The
longer the affected bowel the worse the prognosis even in the face of
restorative surgery (long bowel myectomy-myotomy).
References:
1-Tomiyama H, Shimotake T, Ono S, Kimura O, Tokiwa K, Iwai N:
Relationship between the type of RET/GDNF/NTN or SOX10 gene mutations
and long-term results after surgery for total colonic aganglionosis
with small bowel involvement. J Pediatr Surg. 36(11):1685-8, 2001
2- Gnananayagam EJ, Solomon R, Chandran A, Anbarasi S, Sen S, Moses
PD:Long segment Hirschsprung's disease in the Waardenburg-Shah
syndrome.Pediatr Surg Int. 2003 Aug;19(6):501-3, 2003
3- Moore SW: The contribution of associated congenital anomalies in
understanding Hirschsprung's disease. Pediatr Surg Int. 22(4):305-15,
2006
4- Jan IA, Stroedter L, Haq AU, Din ZU: Association of
Shah-Waardenburgh syndrome: a review of 6 cases. J Pediatr Surg.
43(4):744-7, 2008
5- Espinosa R, Alonso Calderon JL: Neural crest disorders and Hirschsprung's disease. Cir Pediatr. 22(1):25-8, 2009
5- Karaca I, Turk E, Ortac R, Kandirici A: Waardenburg syndrome with
extended aganglionosis: report of 3 new cases. J Pediatr Surg.
44(6):E9-13, 2009
Bishop-Koop Anastomosis
The survival of babies born with
cystic fibrosis and meconium ileus was improved significantly with the
development of intestinal surgical procedures in the second part of the
past century. Such is the case of the temporary Bishop-Koop anastomosis
(BKA), where the proximally dilated bowel is anastomosed end to side to
the distal bowel which in turns is taken out as a stoma. Succus
entericus can then pass from the proximal bowel to the distal bowel all
the way down to the anus or come out through the stoma if there is a
distal functional obstruction. Beside meconium ileus, this anastomosis
has been used in children suffering from various other intestinal
anomalies such as jejuno-ileal atresia, necrotizing enterocolitis,
volvulus, gastroschisis associated with bowel atresia, and apple-peel
syndrome. In such situations the Bishop-Koop anastomosis is a
safer procedure than primary end-to-end or end-to-side anastomosis,
especially for the management of greatly different intestinal
diameters. This occurs in cases of slowly distensible microcolons
associated with the above conditions where the BKA is a temporary
procedure suitable for the colon to grow properly.
References:
1- Kootstra G, Kamann HL, Okken A, Vander Vliet JA, Zwierstra RP,
Slooff MJ, Krom RA, Kuijjer PJ: The Bishop-Koop anastomosis-a find in
pediatric surgery. Neth J Surg. 32(3):92-6, 1980
2- Murshed R, Spitz L, Kiely E, Drake D: Meconium ileus: a ten-year
review of thirty-six patients. Eur J Pediatr Surg. 7(5):275-7, 1997
3- Wit J, Sellin S, Degenhardt P, Scholz M, Mau H: Is the Bishop-Koop
anastomosis in treatment of neonatal ileus still current?. Chirurg.
71(3):307-10, 2000
4- Fleet MS, de la Hunt MN: Intestinal atresia with gastroschisis: a
selective approach to management. J Pediatr Surg. 35(9):1323-5, 2000
5- Kumaran N, Shankar KR, Lloyd DA, Losty PD: Trends in the management
and outcome of jejuno-ileal atresia. Eur J Pediatr Surg. 12(3):163-7,
2002
6- Siman J, Trnka J: Stoma procedures in the congenital digestive tract malformations. Rozhl Chir. 86(7):347-52, 2007
Fetal Rectal Perforation
Perforation of the extraperitoneal
rectal wall in newborns is very rare. Most described cases are term
babies. Fetal distress is not noted at the time of delivery. All
affected babies have a recognizable abnormality at birth mostly seen
during the first five days of birth. The abnormality can be a perineal
or buttock lesion, varying from a minor skin blemish, fistula, swelling
to a rapidly enlarging aerocele with meconium staining of perineal
tissue with subsequent skin rupture or isolated ascites. Diagnosis is
suggested with lateral simple films. Meconium is expressed under
pressure by fetal peristalsis through a defect in the lower rectal wall
into normal tissue planes within the infralevator space to reach a
subcutaneous level and up toward the abdominal cavity. No other
congenital associated anomaly has been described. The position of the
defect at the level of the pelvic floor is consistent with the
watershed between the inferior and middle rectal arteries and might
suggest a local ischemic insult, though the cause remains unknown.
After resuscitation and antibiotherapy, surgery is mandatory. The
procedure consists of fecal diversion achieved by sigmoid colostomy; in
addition the perineal tissues are debrided and wide drainage is
achieved. Prognosis is usually good with normal continence obtained
later in most affected cases. Some cases has developed a rectal
stenosis amenable to dilatation.
References:
1- Mitsudo SM, Boley SJ, Rosenzweig MJ, Campbell DE: Extraperitoneal
pelvic meconium extravasation in a newborn infant. J Pediatr.
103(4):598-600, 1983
2- Davies MR, Cywes S, Rode H: Prenatal perforation of the
extraperitoneal part of the rectum, associated with a developmental
defect of the pelvic floor. Z Kinderchir. 39(4):271-3, 1984
3- Casaccia G, Giorlandino C, Catalano OA, Bagolan P: Prenatal rectal
perforation: an unsuspected cause of isolated ascites. J Perinatol.
26(11):717-9, 2006
4- Sundararajan L, Patel D, Jawaheer G: Antenatal rectal perforation
presenting in the neonate. Pediatr Surg Int. 24(5):601-3, 2008
5- Pitcher GJ, Davies MR, Bowley DM, Numanoglu A, Rode H: Fetal
extraperitoneal rectal perforation: a rare neonatal emergency. J
Pediatr Surg. 2009 Jul;44(7):1405-9
PSU Volume 33 No 04 OCTOBER 2009
US-guided CVC placement
Central venous catheter (CVC)
placement is an integral part in management of many medical and
surgical conditions in children. Percutaneous placement of CVC in the
subclavian or internal jugular vein is a blind procedure using external
anatomical landmarks technique. As such, they carry an inherent risk of
puncturing the artery or lung parenchyma with its attendant
complications associated with variation in venous anatomy and depth of
cannulating needle. Doppler ultrasound (US) guided CVC placement
permits direct visualization and cannulation of the central veins in
the neck (internal jugular vein), specially when placing CVC in very
small babies. US-guided CVC placement has also a reduced rate of
complications and the rate of needle punctures is also reduced.
Reducing the rate of needle punctures reduce the rate of venous
thrombosis. The right internal jugular vein is preferred with this
technique in most patients. Specific training in interventional
radiology is not essential to perform this technique safely and with a
low complication rate, but a learning curve is associated with
dominating this technique. US-guided CVC placement can be done safely
in children of all ages.
References:
1- Verghese ST, McGill WA, Patel RI, Sell JE, Midgley FM, Ruttimann UE:
Ultrasound-guided internal jugular venous cannulation in infants: a
prospective comparison with the traditional palpation method.
Anesthesiology. 91(1):71-7, 1999
2- MacIntyre PA, Samra G, Hatch DJ: Preliminary experience with the
Doppler ultrasound guided vascular access needle in paediatric
patients. Paediatr Anaesth. 10(4):361-5, 2000
3- Asheim P, Mostad U, Aadahl P: Ultrasound-guided central venous
cannulation in infants and children. Acta Anaesthesiol Scand.
46(4):390-2, 2002
4- Machotta A, Kerner S, Höhne C, Kerner T: Ultrasound-guided
central venous cannulation in a very small preterm neonate. Paediatr
Anaesth. 15(4):325-7, 2005
5- Chuan WX, Wei W, Yu L: A randomized-controlled study of ultrasound
prelocation vs anatomical landmark-guided cannulation of the internal
jugular vein in infants and children. Paediatr Anaesth. 15(9):733-8,
2005
6- Arul GS, Lewis N, Bromley P, Bennett J: Ultrasound-guided
percutaneous insertion of Hickman lines in children. Prospective study
of 500 consecutive procedures. J Pediatr Surg. 44(7):1371-6, 2009
Melanoma
Melanoma is very rare in children with
approximately 400 new cases diagnosed yearly in the United States and
2% of all melanoma cases. The incidence of melanoma is increasing
worldwide. With accurate diagnosis the outcome for pediatric melanoma
is good. Factors associated with an increased risk of melanoma includes
white race, female sex, fair complexion, red or blonde hair, light eye
color, tendency to burn with ultraviolet light, dysplastic nevi,
congenital nevi, increase number of benign nevi, family history and
immunosuppression. Early in infancy melanoma arises from transplacental
metastasis, congenital or large nevi. Clinical signs suspicious of
melanoma include increase or change in lesion size or color, bleeding,
irregular border or pigmentation distribution, pruritus or enlarged
regional lymph nodes. Trunk and extremity are the most common location.
Superficial spreading melanoma is the most common histologic variant.
Recurrence is more common in black children and misdiagnosed cases.
Increase awareness with biopsy of suspicious lesions confirms the
diagnosis while establishing depth of tumor. Excision with sentinel
lymph node (SL N) biopsy or complete lymph node dissection is curative
and improves stage-specific survival in pediatric melanoma. The
sentinel node status correlates with primary tumor depth. Pediatric
patients have a higher incidence of SLN metastases than adults yet have
a lower incidence of recurrence.
References:
1- Roaten JB, Partrick DA, Pearlman N, Gonzalez RJ, Gonzalez R,
McCarter MD: Sentinel lymph node biopsy for melanoma and other
melanocytic tumors in adolescents. J Pediatr Surg. 40(1):232-5, 2005
2- Roaten JB, Partrick DA, Bensard D, Pearlman N, Gonzalez R,
Fitzpatrick J, McCarter MD: Survival in sentinel lymph node-positive
pediatric melanoma. J Pediatr Surg. 40(6):988-92, 2005
3- Strouse JJ, Fears TR, Tucker MA, Wayne AS: Pediatric melanoma: risk
factor and survival analysis of the surveillance, epidemiology and end
results database. J Clin Oncol. 23(21):4735-41, 2005
4- Livestro DP, Kaine EM, Michaelson JS, Mihm MC, Haluska FG,
Muzikansky A, Sober AJ, Tanabe KK: Melanoma in the young: differences
and similarities with adult melanoma: a case-matched controlled
analysis. Cancer. 110(3):614-24, 2007
5- Lewis KG: Trends in pediatric melanoma mortality in the United States, 1968 through 2004. Dermatol Surg. 34(2):152-9, 2008
6- Gow KW, Rapkin LB, Olson TA, Durham MM, Wyly B, Shehata BM: Sentinel
lymph node biopsy in the pediatric population. J Pediatr Surg.
43(12):2193-8, 2008
7- Aldrink JH, Selim MA, Diesen DL, Johnson J, Pruitt SK, Tyler DS,
Seigler HF: Pediatric melanoma: a single-institution experience of 150
patients. J Pediatr Surg. 44(8):1514-21, 2009
Rectal Strictures
Rectal strictures in children can
occur after inflammatory bowel disease, trauma, or most commonly
postoperative following a coloanal anastomosis. Factors that contribute
to the formation of a postoperative rectal stricture include ischemia,
leakage and infection, inflammatory response to anastomotic material,
circular stapler size, and fecal contact with the anastomosis. Stapling
devices are associated with a higher rate of postoperative strictures
than handsewn anastomosis. Clinically, the patient with a rectal
stricture after surgery can develop partial or complete bowel
obstruction, frequent bowel movements, lower abdominal fullness, sense
of residual stools followed by anal pain, or the stricture is diagnosed
after preoperative imaging prior to closing a diverting stoma.
Anastomotic colorectal strictures are usually defined as being less
than 10 to 12 mm in diameter and are usually short (< 1 cm in
length). A radiographic assessment of the stricture length must be
done. Depending on the diameter and length of the stricture management
might consist of transanal dilatations (manual or by bougie),
hydrostatic balloon dilatations, microwave coagulation therapy,
transanal incision, excision, or reanastomosis by means of a circular
stapler. Use of steroid injection (Kenalog) has also been utilized.
Postoperative strictures usually respond well to direct
dilatations.
References:
1- Shimada S, Matsuda M, Uno K, Matsuzaki H, Murakami S, Ogawa M: A New
Device for the Treatment of Coloproctostomic Stricture After Double
Stapling Anastomoses. Ann Surg 224:603-608, 1996
2- Suchan KL, Muldner A, Manegold BC: Endoscopic treatment of
postoperative colorectal anastomotic strictures. Surg Endosc 17:
1110-1113, 2003
3- Garcea G, Sutton CD, Lloyd TD, Jameson J, Scott A, Kelly MJ:
Management of benign rectal strictures: a review of present therapeutic
procedures. Dis Colon Rectum. 46(11):1451-60, 2003
4- Pabst M, Giger U, Senn M, Gauer JM, Boldog B, Scweizer W: Transanal
treatment of strictured rectal anastomosis with circular stapler
device: Simple and safe. Dig Surg 24: 12-14, 2007
5- McKee R, Pricolo V: Stapled revision of complete colorectal
anastomotic obstruction. Am Journal Surg 195: 526-527, 2008
6- Lillehei CW, Leichtner A, Bousvaros A, Shamberger RC: Restorative
proctocolectomy and ileal pouch-anal anastomosis in children. Dis Colon
Rectum. 52(9):1645-9, 2009
PSU Volume 33 No 05 NOVEMBER 2009
Esophageal Leiomyoma
Leiomyoma is considered the most
common benign tumor of the esophagus. More common in adults than
children. The mean age of involvement in pediatric patients is 14 years
old with more girls affected than boys. Contrary to the predominant
solitary and localized nodules found in adults, more than 90% of
affected children harbor diffuse lesions. Associated anomalies include
hiatal hernia, Alport's syndrome (nephropathy with hematuria, deafness
and cataract), genital anomalies, esophageal peptic ulcer,
cholelithiasis and rectal prolapse. Children demonstrate symptoms of
slowly progressive dysphagia, weight loss, hematemesis, food impact,
hiccups and choking. Rarely the tumor might affect the tracheobronchial
tree. Diagnostic tests include esophagogram, endoscopy and CT-Scan.
Barium swallow reveals a long-segment stricture and CT-Scan
demonstrates a circumferential mass lesion in the lower esophagus.
Endoscopic ultrasonography has also been used to diagnose the presence
of a subepithelial tumor causing extrinsic compressions of the
oesophagus. Biopsy through the endoscope is hazardous and is not
recommended. Esophageal leiomyoma must be considered in the
differential diagnosis of a mediastinal mass or esophageal stricture.
Management consists of resection with primary or substitute esophageal
reconstruction. Localized lesions may be amenable to enucleation.
Diffuse lesions might need esophageal replacement.
References:
1- Bourque MD, Spigland N, Bensoussan AL, Collin PP, Saguem MH, Brochu
P, Blanchard H, Reinberg O: Esophageal leiomyoma in children: two case
reports and review of the literature. J Pediatr Surg. 24(10):1103-7,
1989
2- Tannuri U, Feferbaun R, Costa RB: Leiomyoma of the esophagus in
children - case report and review of the literature. J Pediatr (Rio J)
70(6):365-70, 1994
3- Levine MS, Buck JL, Pantongrag-Brown L, Buetow PC, Lowry MA,
Sobin LH: Esophageal leiomyomatosis. Radiology. 199(2):533-6, 1996
4- Massicot R, Aubert D, Mboyo A, Destuynder O, Queneau PE: Localized
esophageal leiomyoma and hypertrophic osteoarthropathy. J Pediatr Surg.
32(4):646-7, 1997
5- Lee H, Morgan K, Abramowsky C, Ricketts RR: Leiomyoma at the site of
esophageal atresia repair. J Pediatr Surg. 36(12):1832-3, 2001
6- Gupta V, Lal A, Sinha SK, Nada R, Gupta NM: Leiomyomatosis of the
esophagus: experience over a decade. J Gastrointest Surg. 13(2):206-11,
2009
Meningococcal Sepsis
Meningococcal sepsis is a devastating
condition caused by the bacteria Neisseria Meningitidis. Meningococcal
septicemia can lead to purpura fulminans with subsequent full thickness
skin loss and deep muscle damage causing mutilating amputations of
hands, digits, lower limbs and toes. Children with this condition are
managed with aggressive intravenous fluids, vasoactive drugs,
antibiotics, respiratory support and immunoglobulin therapy. The basic
process in the pathophysiology of this condition that affects the
microvasculature is increased vascular permeability, pathologic
vasoconstriction and vasodilatation, loss of thromboresistance,
intravascular coagulation and myocardial dysfunction. The lower limbs
are predominantly affected. Surgical therapy is typically delayed in
these children due to cardiovascular instability. Tissue loss can be
extensive and difficult to determine at the outset. Surgical procedures
consist of debridement, amputation, skin-grafting and soft-tissue
releasing incisions. Recently, early microsurgical arteriolysis,
freeing affected blood vessels, has proved a reliable method to
decrease the level and reduce the amputation rate observed in these
cases. Children requiring surgery for purpura fulminans achieve
age-appropriate milestones and are primarily limited by their physical
disability related to amputations, scarring and abnormal bone growth.
References:
1- Huang S, Clarke JA: Severe skin loss after meningococcal
septicaemia: complications in treatment. Acta Paediatr. 86(11):1263-6,
1997
2- Leclerc F, Leteurtre S, Cremer R, Fourier C, Sadik A: Do new
strategies in meningococcemia produce better outcomes? Crit Care Med.
28(9 Suppl):S60-3, 2000
3- Wheeler JS, Anderson BJ, De Chalain TM: Surgical interventions in
children with meningococcal purpura fulminans--a review of 117
procedures in 21 children. J Pediatr Surg. 38(4):597-603, 2003
4- Dinh TA, Friedman J, Higuera S: Plastic surgery management in
pediatric meningococcal-induced purpura fulminans. Clin Plast Surg.
32(1):117-21, 2005
5- Numanoglu A, Bickler SW, Rode H, Bosenberg AT: Meningococcal
septicaemia complications involving skin and underlying deeper
tissues--management considerations and outcome. S Afr J Surg.
45(4):142-6, 2007
6- Boeckx WD, Nanhekhan L, Vos GD, Leroy P, Van den Kerckhove E:
Minimizing limb amputations in meningococcal sepsis by early
microsurgical arteriolysis. J Pediatr Surg. 44(8):1625-30, 2009
All-Terrain Vehicles
All-terrain vehicles (ATV) injuries
continue to produce morbidity and mortality in our kids. ATV include,
four wheels vehicles, minibikes, golf carts, and go-karts. Children
lack the physical strength, cognitive abilities, and fine motor skills
to operate ATVs properly. Most cases of injury occur in males in the
ages of 11 to 15 years. Children aged 0 to 5 years are more likely than
older children to have facial injuries, whereas older children are more
likely to sustain lower trunk and leg or foot injuries. Some of the
recommendations that several medical organizations have proposed to
reduce the injury rate include that laws should prohibit the use of ATV
by children younger than 16 years of age, recreational use of ATV
should be limited to people who have license to operate other motor
vehicles, children between ages 16 and 18 should be supervised by a
legal guardian, drivers should complete an approved training course,
and operators should always wear a government-approved helmet, eye
protection and protective clothing.
References:
1- Shults RA, Wiles SD, Vajani M, Helmkamp JC: All-terrain
vehicle-related nonfatal injuries among young riders: United States,
2001-2003. Pediatrics. 116(5):e608-12, 2005
2- Fonseca AH, Ochsner MG, Bromberg WJ, Gantt D: All-terrain vehicle
injuries: are they dangerous? A 6-year experience at a level I trauma
center after legislative regulations expired. Am Surg. 71(11):937-40,
2005
3- Alawi K, Lynch T, Lim R: All-terrain vehicle major injury patterns
in children: a five-year review in Southwestern Ontario. CJEM.
8(4):277-80, 2006
4- Trauma Committee of the Canadian Association of Pediatric Surgeons:
Canadian Association of Pediatric Surgeons' position statement on the
use of all-terrain vehicles by children and youth. J Pediatr Surg.
43(5):938-9, 2008
5- Kellum E, Creek A, Dawkins R, Bernard M, Sawyer JR: Age-related
patterns of injury in children involved in all-terrain vehicle
accidents. J Pediatr Orthop. 28(8):854-8, 2008
6- Burd R: American Pediatric Surgical Association Trauma Committee
position statement on the use of all-terrain vehicles by children and
youth. J Pediatr Surg. 44(8):1638-9, 2009
PSU Volume 33 No 06 DECEMBER 2009
Subdural Hematoma
Subdural hematoma (SDH) is a type of
traumatic brain injury where blood collect within the dura layer of the
brain. Subdural bleeding usually results from tears in veins that cross
the subdural space. Subdural hematoma requiring surgery in children is
unusual. The incidence of SDH in infants is between 12-25 cases per
100,000 children and most detected SDH are due to physical abuse.
Radiographically, these are easily distinguishable from a "lens shaped"
epidural hematoma. Epidural hematoma is more likely to be accidental.
The small unilateral SDH seen with diffuse head injury can be observed.
Isolated SDH associated with neurologic deficit or progressive loss of
consciousness will need surgical evacuation. Almost 60% of these are
associated with a skull fracture. Chronic SDH are encountered in
children that have been abused. SDH in the infant has a different
pattern from that seen in the older child and adult. It is usually a
widespread, bilateral, thin film, unlike the thick, space-occupying and
often unilateral clot seen in older children and adults after trauma.
Healing of SDH is by formation of a granulating membrane which may
confer vulnerability to rebleeding, either spontaneously or after an
otherwise innocuous event. SDH has a particular significance as one of
the features of the triad (together with retinal hemorrhage and
encephalopathy) associated with non-accidental injury (physical child
abuse).
References:
1- Swift DM, McBride L: Chronic subdural hematoma in children. Neurosurg Clin N Am. 11(3):439-46, 2000
2- Jayawant S, Parr J: Outcome following subdural haemorrhages in infancy. Arch Dis Child. 92(4):343-7, 2007
3- Case ME: Inflicted traumatic brain injury in infants and young children. Brain Pathol. 18(4):571-82, 2008
4- Case ME: Accidental traumatic head injury in infants and young children. Brain Pathol. 18(4):583-9, 2008
5- Squier W, Mack J: The neuropathology of infant subdural haemorrhage. Forensic Sci Int. 187(1-3):6-13, 2009
Subglottic Stenosis
Subglottic stenosis refers to stenosis
of the airway inside the cricoid ring. Subglottic stenosis (SGS) can be
congenital or acquired. In either case the child will develop an
inspiratory and expiratory (biphasic) stridor. Subglottic stenosis will
also have a barking cough and can manifest during a viral illness. The
congenital variety of subglottic stenosis is very rare and consists of
a soft tissue thickening of the subglottic area, occasionally involving
the true vocal cords. Minimal laryngeal inflammation precipitates
airway obstruction because the cricoid cartilage is nondistensible.
With time as laryngeal growth occurs, the congenital stenosis improves
needing surgery less common. The acquired variety of SGS in infants is
usually the result of prolonged endotracheal intubation during the
neonatal period due to hyaline membranes disease or after surgical
procedures. More than the duration of intubation is the continuous
movement of the tube from inadequate fixation that produces the chronic
inflammation needed for the stenosis to develop. With better neonatal
airway care the incidence of acquired subglottic stenosis has
significantly decreased. Severe airway obstruction will need
tracheostomy followed by laryngotracheal reconstruction. Anterior or
multiple cricoid splitting with cartilage graft interpositioning is
usually performed. The success rates for these procedures have been
shown to be approximately 90%.
References:
1- Schroeder JW Jr, Holinger LD: Congenital laryngeal stenosis. Otolaryngol Clin North Am. 41(5):865-75, 2008
2- Walner DL, Loewen MS, Kimura RE: Neonatal subglottic stenosis--incidence and trends. Laryngoscope. 111(1):48-51, 2001
3- Sichel JY, Dangoor E, Eliashar R, Halperin D: Management of
congenital laryngeal malformations. Am J Otolaryngol. 21(1):22-30, 2000
4- Cotton RT: Management of subglottic stenosis. Otolaryngol Clin North Am. 33(1):111-30, 2000
5- Fraga JC, Schopf L, Forte V: Thyroid alar cartilage laryngotracheal
reconstruction for severe pediatric subglottic stenosis. J Pediatr
Surg. 36(8):1258-61, 2001
6- Hartnick CJ, Hartley BE, Lacy PD, Liu J, Willging JP, Myer CM 3rd,
Cotton RT: Surgery for pediatric subglottic stenosis: disease-specific
outcomes. Ann Otol Rhinol Laryngol. 110(12):1109-13, 2001
CHARGE Syndrome
CHARGE syndrome is a specific
collection of non-randomly occurring congenital anomalies named for its
six major clinical features. Patients of CHARGE syndrome may be
presented to ophthalmologists due to ocular coloboma. The acronym
CHARGE stands for the major features of this syndrome: Coloboma of the
eye, Heart defects, Atresia of the choanae, Retarded growth and
development, Genital hypoplasia, and Ear anomalies and/or deafness.
CHARGE syndrome occurs in an estimated of one in 8,500 live births.
Individuals with CHARGE syndrome who demonstrated a less extensive
phenotype (less than three major criteria) were more likely to present
with minor cardiovascular malformations, including small atrial or
ventricular septal defects (VSD) or patent ductus arteriosus (PDA).
Mutations in the CHD7 gene (member of the chromodomain helicase DNA
protein family) are detected in more than 75% of patients with CHARGE
syndrome. A significant cause of morbidity is severe feeding
difficulty, including problems with chewing, swallowing, and
gastroesophageal reflux, which are prevalent throughout childhood.
Evaluation of associated defects and proper referral for timely
management may be critical for patients with CHARGE syndrome.
References:
1- Hsueh KF, Yang CS, Lu JH, Hsu WM: Clinical characteristics of CHARGE syndrome. J Chin Med Assoc. 67(10):542-6, 2004
2- Issekutz KA, Graham JM Jr, Prasad C, Smith IM, Blake KD: An
epidemiological analysis of CHARGE syndrome: preliminary results from a
Canadian study. Am J Med Genet A. 133A(3):309-17, 2005
3- Blake KD, Prasad C: CHARGE syndrome. Orphanet J Rare Dis. 7;1:34, 2006
4- Pagon RA, Graham JM Jr, Zonana J, Yong SL: Coloboma, congenital
heart disease, and choanal atresia with multiple anomalies: CHARGE
association. J Pediatr. 99(2):223-7, 1981
5- Davenport SL, Hefner MA, Mitchell JA: The spectrum of clinical
features in CHARGE syndrome. Clin Genet. 29(4):298-310, 1986