PEDIATRIC SURGERY UPDATE ©
VOLUME 36, 2011
PSU Volume 36 No 01 JANUARY 2011
Laparoscopic Rectopexy
Full-thickness rectal prolapse is a
relatively comm
on and distressing condition in children that
fortunately is usually self-limiting in the life of the affected child
and family. Peak incidence is between one and three years of age.
Medical management improves more than 80% of all cases of rectal
prolapse. Patients are managed for the underlying cause of the prolapse
such as chronic constipation or acute diarrhea, while cystic fibrosis
is rule-out. Failed medical therapy and recurrent prolapse will be
managed with surgery. Before surgical intervention the rectum should be
evaluated with imaging studies (Barium Enema) and flexible endoscopy.
Therapeutic surgical procedures include submucosal injection of
sclerosant, Thiersch cerclage, open abdominal rectopexy with or without
sigmoid resection and perineal rectopexy. Rectopexy alone for
megarectum or megasigmoid is not appropriate and will eventually need
resection of the dilated bowel. With the use of minimally invasive
techniques, laparoscopic suture rectopexy namely, full posterior
mobilization and fixation to the sacrum, has emerged as an alternative
surgical management for recurrent rectal prolapse in children. No
preoperative bowel preparation is required while the procedure can be
done ambulatory with a low morbidity, low recurrence rate and excellent
cosmetic results. Laparoscopic rectopexy has also been found to improve
constipation and fecal incontinence.
References:
1- Demirbas S, Akin ML, Kalemoglu M, Ogün I, Celenk T:
Comparison of laparoscopic and open surgery for total rectal prolapse.
Surg Today. 35(6):446-52, 2005
2- Vijay V, Halbert J, Zissimopoulos A, Siddiqi S, Warren S: Day case
laparoscopic rectopexy is feasible, safe, and cost effective for
selected patients. Surg Endosc. 22(5):1237-40, 2008
3- Byrne CM, Smith SR, Solomon MJ, Young JM, Eyers AA, Young CJ:
Long-term functional outcomes after laparoscopic and open rectopexy for
the treatment of rectal prolapse. Dis Colon Rectum. 51(11):1597-604,
2008
4- Ismail M, Gabr K, Shalaby R: Laparoscopic management of persistent
complete rectal prolapse in children. J Pediatr Surg. 45(3):533-9, 2010
5- Shalaby R, Ismail M, Abdelaziz M, Ibrahem R, Hefny K, Yehya A, Essa
A: Laparoscopic mesh rectopexy for complete rectal prolapse in
children: a new simplified technique. Pediatr Surg Int. 26(8):807-13,
2010
6- Dean Potter D, Bruny JL, Allshouse MJ et al: Laparoscopic suture
rectopexy for full-thickness anorectal prolapse in children: an
effective outpatient procedure. J Pediatr Surg 45(10): 2103-2107, 2010
Renal Leiomyoma
Leiomyomas are characterized as benign
mesenchymal tumor that arise from smooth muscle cells. There most
common location is the uterus and gastrointestinal tract, though they
can rarely arise anywhere smooth muscle cells exist. Wilms tumor is
still the most common renal tumor in children. Renal leiomyoma is
a very rarely found in children. The leiomyoma of kidney can arise from
the renal capsule (most common site), renal pelvis and calyces, or the
renal cortical vasculature. Most cases of renal leiomyoma are found in
adults at a median age of 40 years with female predominance. In
children the rare cases have been found incidentally. When symptomatic
the tumor has enlarged significantly causing pain, a palpable mass and
hematuria. Alport syndrome has been associated with diffuse
leiomyomatosis, including the genitourinary tract. Diagnostic studies
include abdominal US (solid mass) and CT-Scan (well circumscribed
homogenous enhanced mass). When very large they are undistinguishable
from nephroblastomas. Managements consist of total nephrectomy. An
association between Epstein-Barr virus and smooth muscle tumors such as
renal leiomyoma in immunocompromised patients has been recognized
recently.
References:
1-Tawfik OW, Moral LA, Richardson WP, Lee KR: Multicentric bilateral
renal cell carcinomas and a vascular leiomyoma in a child. Pediatr
Pathol. 13(3):289-98, 1993
2- Belis JA, Post GJ, Rochman SC, Milam DF: Genitourinary leiomyomas. Urology. 13(4):424-9, 1979
3- Anker MC, Arnemann J, Neumann K, Ahrens P, Schmidt H,
König R: Alport syndrome with diffuse leiomyomatosis.Am J
Med Genet A. 119A(3):381-5, 2003
4- Dionne JM, Carter JE, Matsell D, MacNeily AE, Morrison KB, de Sa D:
Renal leiomyoma associated with Epstein-Barr virus in a pediatric
transplant patient. Am J Kidney Dis. 46(2):351-5, 2005
5- Gupta A, Chandra N, Sharma A, Husain N, Kureel SN: Renal leiomyoma
in a child: a rare renal tumor. J Pediatr Surg. 45(9):1900-3, 2010
Acquired Undescended Testis
Undescended testes (UT) are recently
categorized into congenital and acquired forms. In the congenital form
the child is born with an undescended testis, while the acquired form
is characterized for later development in life of cryptorchidism. The
acquired form of undescended testes comprises the high rate of
orchidopexies performed later in life in children. Is a condition in
which a previously fully descended testis can no longer be manipulated
into a stable scrotal position. Acquired UT are mostly situated in a
superficial inguinal pouch, of normal size, with a normal attachment of
the gubernaculum, and in half of the cases associated with an open
processus vaginalis They are not associated with epididymal deformities
or abnormal attachment of the gubernaculum. Acquired UT can be
secondary to failure of natural elongation of cord structures in
proportion to body growth due to complete disappearance of the
processus vaginalis. In contrast the congenital variety of UT is
proximal to the external spermatic ring (intracanalicular), a complete
hernial sac is present, and is associated with epididymal deformities
and abnormal attachment of the gubernaculum. Acquired UT has also been
reported in children with cerebral palsy due to spasticity of the
cremasteric muscle. Both forms of UT should be managed by orchiopexy to
avoid the adverse effects on germ cell development and fertility
potential. For congenital UT orchiopexy is recommended at 6-12 months
of age. Postoperative results are better for the acquired form compared
with the congenital variety.
References:
1- Barthold JS, González R: The epidemiology of congenital
cryptorchidism, testicular ascent and orchiopexy. J Urol. 170(6
Pt 1):2396-401, 2003
2- Meijer RW, Hack WW, van der Voort-Doedens LM, Haasnoot K, Bos SD:
Surgical findings in acquired undescended testis. J Pediatr Surg.
39(8):1242-4, 2004
3- Hutson JM, Hasthorpe S: Testicular descent and cryptorchidism: the
state of the art in 2004. J Pediatr Surg. 40(2):297-302, 2005
4- Hutson JM, Hasthorpe S: Abnormalities of testicular descent. Cell Tissue Res. 322(1):155-8, 2005
5- Sijstermans K, Hack WW, Meijer RW, van der Voort-Doedens LM: The
frequency of undescended testis from birth to adulthood: a review. Int
J Androl. 31(1):1-11, 2008
6- Meij-de Vries A, Hack WWM, Heij HA, Meijer RW: Perioperative
Surgical Findings in Congenital and Acquired Undescended Testis. J
Pediatr Surg 45(9): 1874-1881, 2010
PSU Volume 36 NO 02 FEBRUARY 2011
Ethanol Sclerotherapy for Lymphangiomas
Lymphatic malformations are very
difficult lesions to manage and eradicate in children. They constitute
6% of all benign lesions in infants and children. Head and neck are the
more commonly affected regions. Most lesions will appear within the
first two years of life of the child. The cystic lymphatic lesions are
classified as macrocystic (> 1 cm), microcystic (< 1 cm) and
mixed variety. Lymphatic malformations do not have a familial tendency
and they do not become malignant with time. Surgical excision is the
standard method of choice to manage lymphatic lesions that are
localized and do not involve vital surrounding structures. Mutilating
surgery is avoided at all costs. Diagnosis of the anatomic arrangements
that these lymphatic malformations have with vital structure is
obtained with the help of CT-Scan and MRI imaging. For unresectable or
residual lymphatic cystic lesions the use of sclerotherapy has been
advocated. Sclerosing agents include OK-432, Bleomycin, Fibrin glue,
Doxycycline, 50% dextrose, Ethibloc (alcoholic solution of corn
protein) and recently 98% Ethanol. Some agents are very difficult to
obtained due to FDA regulation. Others cause toxic, allergic and
irritant effects to the child. Sterile 98% ethanol produces minimal
side effect, is easy to purchase and has a low cost. Ethanol
sclerotherapy can be affected using CT-guided technology with the child
under sedation or general anesthesia. Th volume of ethanol used for
sclerotherapy should be no more than 1.0 ml/kg of weight.
References:
1- Saba C, Bossi MC, Barletta A: Therapy of subcutaneous cystic
lymphangioma with ultrasound-guided puncture and alcoholization. Radiol
Med. 83(3):270-2, 1992
2- Puig S, Aref H, Brunelle: Double-needle sclerotherapy of
lymphangiomas and venous angiomas in children: a simple technique to
prevent complications. AJR Am J Roentgenol. 180(5):1399-401, 2003
3- Alomari AI, Karian VE, Lord DJ, Padua HM, Burrows PE: Percutaneous
sclerotherapy for lymphatic malformations: a retrospective
analysis of patient-evaluated improvement. J Vasc Interv
Radiol. 17(10):1639-48, 2006
4- Shiels WE 2nd, Kenney BD, Caniano DA, Besner GE: Definitive
percutaneous treatment of lymphatic malformations of the trunk and
extremities. J Pediatr Surg. 43(1):136-9, 2008
5- Shiels WE 2nd, Kang DR, Murakami JW, Hogan MJ, Wiet GJ: Percutaneous
treatment of lymphatic malformations. Otolaryngol Head Neck Surg.
141(2):219-24, 2009
6- Impellizzeri P, Romeo C, Astra Borrut F, et al: Sclerotherapy for
cervical cystic lymphatic malformations in children. Our experience
with computed tomography-guided 98% sterile ethanol insertion and
review of the literature. J Pediatr Surg. 45(12): 2473-2478, 2010
Ogilvie Syndrome
Acute colonic pseudo-obstruction also
known as Ogilvie's syndrome is a massive colonic dilatation associated
with signs and symptoms of colonic obstruction without an evident
mechanical cause. Ogive syndrome is observed predominantly in the
elderly population with few cases reported in children. Predisposing
factors for Ogilvie's syndrome in children includes postoperative
state, trauma, infections, Sickle cell disease, cardiac diseases and
chemotherapy for malignancy. Symptoms include constipation, abdominal
pain, nausea, vomiting and abdominal distension. Diagnosis is suggested
in flat simple abdominal films. Findings on CT-Scan are diagnostic
showing massive colonic dilatation with diameters of eight to 12 cm and
without evidence of overt mechanical obstruction. If left untreated,
this dilatation can lead to colonic perforation and peritonitis in 10%
of children with high mortality rates. Initial management consists of
nasogastric decompression, bowel rest, hydration, electrolyte
correction, along with discontinuation of drugs affecting bowel
motility. If symptoms fail to improve with initial management then
rectal tube or colonoscopy decompression is utilized. Neostigmine, an
acethycholinesterase inhibitor which increases parasympathetic tone,
has been found to be very effective in managing patients with Ogilvie
syndrome. Neostigmine is slowly titrated in increments up to a total of
0.05mg/kg of weight. Also, oral erythromycin therapy has been used to
manage this condition. Surgery will be needed if the child develops
perforation or signs of bowel ischemia.
References:
1- Hyman PE: Chronic intestinal pseudo-obstruction in childhood: progress in diagnosis and
treatment. Scand J Gastroenterol Suppl. 213:39-46, 1995
2- Gmora S, Poenaru D, Tsai E: Neostigmine for the treatment of
pediatric acute colonic pseudo-obstruction. J Pediatr Surg. 37(10):E28,
2002
3- Jiang DP, Li ZZ, Guan SY, Zhang YB: Treatment of pediatric Ogilvie's
syndrome with low-dose erythromycin: a case report. World J
Gastroenterol. 13(13):2002-3, 2007
4- Kim TS, Lee JW, Kim MJ, Park YS, Lee DH, Chung NG, Cho B, Lee S, Kim
HK: Acute colonic pseudo-obstruction in postchemotherapy complication
of brain tumor treated with neostigmine. J Pediatr Hematol Oncol.
29(6):420-2, 2007
5- Khosla A, Ponsky TA: Acute colonic pseudoobstruction in a child with
sickle cell disease treated with neostigmine. J Pediatr Surg.
43(12):2281-4, 2008
6- Lee JW, Bang KW, Jang PS, Chung NG, Cho B, Jeong DC, Kim HK, Im SA,
Lim GY: Neostigmine for the treatment of acute colonic
pseudo-obstruction (ACPO) in pediatric hematologic malignancies. Korean
J Hematol. 45(1):62-5, 2010
Colostomy Closure
Colostomy closure is a common and
important surgical procedure performed in children which carries a
significant risk of morbidity and mortality. Some complication
associated with closure of a colostomy includes wound infection,
anastomotic dehiscence, bleeding, anastomotic stricture, incisional
hernia and death. The periostoma lymphatics are colonized with
bacteria, reason why surgery site infection rises during this
procedure. For colostomy closure most children should be admitted the
day before surgery for mechanical cleansing of the proximal and distal
bowel. Systemic antibiotics during anesthesia induction are
necessary. Infection rate is not affected by the use of oral
antibiotics. Meticulous surgical technique including packing of
proximal stoma, use of plastic drapes for surgical field
immobilization, correct dissection, careful hemostasis avoiding
contamination and performing an anastomosis in well vascularized limbs
are essential to reduce complications. Peritoneal irrigation, fascial
closure in layers, adequate hemostasis and avoidance of dead spaces are
also essential issues to watch for. Postoperative nasogastric tubes are
not necessary. Early feeding is encouraged.
References:
1- Weber TR, Tracy TF Jr, Silen ML, Powell MA: Enterostomy and its closure in newborns. Arch Surg. 130(5):534-7, 1995
2- Sangkhathat S, Patrapinyokul S, Tadyathikom K: Early enteral feeding
after closure of colostomy in pediatric patients. J Pediatr Surg.
38(10):1516-9, 2003
3- Chandramouli B, Srinivasan K, Jagdish S, Ananthakrishnan N:
Morbidity and mortality of colostomy and its closure in children. J
Pediatr Surg. 39(4):596-9, 2004
4- Breckler FD, Rescorla FJ, Billmire DF: Wound infection after
colostomy closure for imperforate anus in children: utility of
preoperative oral antibiotics.J Pediatr Surg. 45(7):1509-13, 2010
5- Bischoff A, Levitt MA, Lawal TA, Pena A: Colostomy closure: how to
avoid complications. Pediatr Surg Int. 26(11):1087-92, 2010
PSU Volume 36 NO 03 MARCH 2011
Intramural Duodenal Hematoma
In children, an intramural duodenal
hematoma is a rare cause of proximal bowel obstruction and most
commonly arises after blunt abdominal trauma. Common mechanisms include
bicycle handlebar injuries, play or athletic injuries, child abuse and
motor vehicle accidents. Other causes of intramural duodenal hematoma
(IDH) are after endoscopic intestinal biopsy and spontaneously in
children suffering from hemophilia, Henoch-Schönlein purpura
and Glanzmann's thrombasthenia. The child develops epigastric abdominal
pain, persistent vomiting with high output bowel obstruction and
abdominal tenderness. Associated traumatic pancreatitis is found in 30%
of cases due to the direct blow or associated with obstruction of the
ampulla of Vater. CT contrast or MRI imaging will allow early and
definite diagnosis. Treatment of IDH is conservative with bowel rest,
nasogastric suction and parenteral nutrition. Ultrasound and UGIS can
be used to follow resolution of the hematoma. Stricture formation,
lief-threatening hemorrhage or perforation is extremely rare sequelae
in children. Surgery drainage may be needed in case of complications or
if prolonged nonsurgical management fails. Scattered report of
effective laparoscopically surgical drainage, internal incision and
drainage by endoscopy and ultrasonically guided aspiration drainage has
been published in the literature. In general the prognosis after
resolution is good.
References:
1- Thoms CA, Ricketts RR: Intramural duodenal hematoma in children:
reappraisal of current management. South Med J. 81(8):985-8, 1988
2- Chittmittrapap S, Chandrakamol B, Chomdej S: Intramural haematoma of
the alimentary tract in children. Br J Surg. 75(8):754-7, 1988
3- Voss M, Bass DH: Traumatic duodenal haematoma in children. Injury. 1994 May;25(4):227-30
4- Lin YC, Chen Y, Yeh SJ: Traumatic intramural hematoma of the
duodenum: report of one case. Acta Paediatr Taiwan. 45(6):343-5, 2004
5- Iuchtman M, Steiner T, Faierman T, Breitgand A, Bartal G:
Post-traumatic intramural duodenal hematoma in children. Isr Med Assoc
J. 8(2):95-7, 2006
6- Banieghbal B, Vermaak C, Beale P: Laparoscopic drainage of a
post-traumatic intramural duodenal hematoma in a child. J Laparoendosc
Adv Surg Tech A. 18(3):469-72, 2008
7- Chien JH, Ho TY, Shih-Peng L, Lee CL, Ou SF: Acquired duodenal obstruction in children. Pediatr Neonatol. 49(5):193-6, 2008
8- Kwon CI, Ko KH, Kim HY, Hong SP, Hwang SG, Park PW, Rim KS: Bowel
obstruction caused by an intramural duodenal hematoma: a case report of
endoscopic incision and drainage. J Korean Med Sci. 24(1):179-83,
2009
Atypical Fibroxanthoma
Atypical fibroxanthoma (AFX) is an
uncommon skin/subcutaneous tissue neoplasm generally found in elderly
Caucasian men with sun-damaged skin. It also rarely appears in
children. AFX arising in children tends to occur on the limb and trunk
areas. AFX typically begins as a solitary firm erythematous nodule that
grows rapidly and may become ulcerated. Most lesions are less than 2
cm. AFX follows a benign clinical course and it rarely metastasize.
Histologically, AFX presents as a dermal nodule composed of haphazardly
arranged spindle cells with multinucleated giant and xanthomatous
histiocytes scattered throughout the tumor. AFX is characterized for
its cellularity, lack of organization, abnormal mitotic figures,
pleomorphism and lymphocytic infiltrate around the edges. Management of
AFX consists of complete excision. Recurrence occurs at a rate of 7%
with the majority occurring within one year of excision. Poorly
circumscribed or irregularly shaped tumors with infiltrative edges on
histology may require larger clinical margins because they account for
the majority of recurrences. AFX has been reported to metastasize to
regional lymph nodes.
References:
1- Mirza B, Weedon D: Atypical fibroxanthoma: a clinicopathological study of 89 cases. Australas J Dermatol. 46(4):235-8, 2005
2- Lum DJ, King AR: Peritoneal metastases from an atypical fibroxanthoma. Am J Surg Pathol. 30(8):1041-6, 2006
3- Melendez MM, Xu X, McClain SA, Huang SI: Atypical fibroxanthoma in a
young woman: An unusual case presentation. Can J Plast
Surg.15(3):169-72, 2007
4- Stefanato CM, Robson A, Calonje JE. The histopathologic spectrum of
regression in atypical fibroxanthoma. J Cutan Pathol. 37(3):310-5, 2010
5- New D, Bahrami S, Malone J, Callen JP: Atypical fibroxanthoma with
regional lymph node metastasis: report of a case and review of
the literature. Arch Dermatol. 146(12):1399-404, 2010
Abdominal Pulmonary Sequestration
A pulmonary sequestration is a piece
of nonfunctioning pulmonary tissue with no communication with the
normal bronchial tree supplied by a systemic artery. Usually in the
thorax (90%) they are classified as intralobar and extralobar. An
extralobar sequestration can also be found in the abdomen of a child.
Intraabdominal pulmonary sequestration is typically located in the left
suprarenal area. The extralobar sequestration has a separate pleural
lining. Most of these extralobar sequestrations are asymptomatic and
appear as an infradiaphragmatic mass. The diagnosis can be suggested
antenatally using ultrasound. Differential diagnosis includes
neuroblastoma, duplication, teratomas and adrenal hemorrhage. MRI,
color Doppler and angio-CT Scan can help define the mass and identify
the feeder blood vessel. Associated conditions include diaphragmatic
hernia and congenital heart disease. Management of abdominal pulmonary
sequestration is surgical excision. Removal can be accomplished either
open or preferably by laparoscopy. Laparoscopic excision has been
demonstrated to be safe and better cosmetically.
References:
1- Gross E, Chen MK, Lobe TE, Nuchtern JG, Rao BN: Infradiaphragmatic
extralobar pulmonary sequestration masquerading as an intra-abdominal,
suprarenal mass. Pediatr Surg Int. 12(7):529-31. 1997
2- Danielson PD, Sherman NJ: Laparoscopic removal of an abdominal
extralobar pulmonary sequestration. J Pediatr Surg.
36(11):1653-5, 2001
3- van der Zee DC, NMa Bax K: Laparoscopic resection of intra-abdominal
extralobar pulmonary sequestration. Pediatr Surg Int. 21(10):841-2,
2005
4- Laje P, Martinez-Ferro M, Grisoni E, Dudgeon D: Intraabdominal
pulmonary sequestration. A case series and review of the literature. J
Pediatr Surg. 41(7):1309-12, 2006
5- Agayev A, Yilmaz S, Cekrezi B, Yekeler E: Extralobar pulmonary
sequestration mimicking neuroblastoma. J Pediatr Surg.
42(9):1627-9, 2007
6- Joyeux L, Mejean N, Rousseau T, Couaillier JF, Piard F, Sapin E:
Ectopic extralobar pulmonary sequestrations in children: interest of
the laparoscopic approach. J Pediatr Surg. 45(11):2269-73, 2010
PSU Volume 36 No 04 APRIL 2011
Malignant Hyperthermia Syndrome
Malignant hyperthermia syndrome (MHS)
is a pharmacogenetic disorder of skeletal muscle presenting as a
hypermetabolic response to potent volatile anesthetic gases such as
halothane, sevoflurane, desflurane, the depolarizing muscle relaxant
succinylcholine and rarely after stress of vigorous exercise and heat.
MHS is inherited in autosomal dominant pattern. A defect in the
ryanodine receptor in chromosome 19. A receptor located in the calcium
channels of the skeletal muscle sarcoplasmic reticulum. Classic signs
of MHS include marked-degree hyperthermia, tachycardia, tachypnea,
increased carbon dioxide production, increase oxygen consumption,
acidosis, muscle rigidity and breakdown (rhabdomyolysis) and
myoglobinuria. Elevations of end-carbon dioxide and temperature are the
initial clues to consider MHS. MHS occur due to uncontrolled release of
myoplasmic calcium which activates skeletal muscle to a hypermetabolic
state causing ATP depletion, compromised muscle membrane integrity and
release of potassium and rhabdomyolysis. Children comprise less than
20% of all cases. Diagnosis is established with the halothane/caffeine
contracture test. Management consists of supportive measures,
temperature control and Dantrolene, a specific antagonist of the
pathophysiologic changes in MHS. The actual incidence of MHS has
increased and the mortality is more than 15% in the Unites States. The
risk of MHS in children undergoing muscle biopsy for suspected
neuromuscular disease is less than 1%.
References:
1- Hall SC: General pediatric emergencies. Malignant hyperthermia syndrome. Anesthesiol Clin North America. 19(2):367-82, 2001
2- Brandom BW: The genetics of malignant hyperthermia. Anesthesiol Clin North America. 23(4):615-9, 2005
3- Flick RP, Gleich SJ, Herr MM, Wedel DJ: The risk of malignant
hyperthermia in children undergoing muscle biopsy for suspected
neuromuscular disorder. Paediatr Anaesth. 17(1):22-7, 2007
4- Larach MG, Gronert GA, Allen GC, Brandom BW, Lehman EB: Clinical
presentation, treatment, and complications of malignant hyperthermia
in North America from 1987 to 2006. Anesth Analg. 110(2):498-507,
2010
5- Sumitani M, Uchida K, Yasunaga H, Horiguchi H, Kusakabe Y, Matsuda
S, Yamada Y: Prevalence of malignant hyperthermia and relationship with
anesthetics in Japan: data from the diagnosis procedure combination
database. Anesthesiology. 114(1):84-90, 2011
Tumor Lysis Syndrome
Tumor lysis syndrome (TLS) refers to a
group of metabolic derangements caused during abrupt and massive
release of cellular components into the blood after rapid lysis of
malignant cells. This event occurs after the child receiving cytotoxic,
cytolitic antibiotics or radiation therapy usually in malignancies such
as leukemias, Burkitt's lymphoma and other tumors with high
proliferative rate, large tumor burden or high sensitivity to
chemotherapy. The release of such components and intracellular
metabolites can lead to hyperuricemia, hyperkalemia, hyperphosphatemia,
hypocalcemia and uremia known as TLS. The precipitation of uric acid
can lead to impaired acute renal failure if not managed adequately. TLS
can occur during an operation. Clinical manifestation includes nausea,
vomiting, diarrhea, anorexia, lethargy, edema, fluid overload,
hematuria, CHF, dysrhythmias, seizures, cramps, tetany and death.
Management may consist of aggressive fluid hydration to enhance urine
flow (considered the most effective strategy), alkalinization of the
urine, blocking the conversion of xanthine and hypoxanthine to uric
acid with allopurinol, and promoting the catabolism of uric acid with
recombinant urate oxidase. Depending on risk stratification, the
treatment above is instituted. Hyperphosphatemia, hyperkalemia and
hypocalcemia should also be treated. The best management of TLS is
prevention.
References:
1- Truini-Pittman L, Rossetto C: Pediatric considerations in tumor lysis syndrome. Semin Oncol Nurs. 18(3 Suppl 3):17-22, 2002
2- Lee MH, Cheng KI, Jang RC, Hsu JH, Dai ZK, Wu JR: Tumour lysis
syndrome developing during an operation. Anaesthesia. 62(1):85-7, 2007
3- Coiffier B, Altman A, Pui CH, Younes A, Cairo MS: Guidelines
for the Management of Pediatric and Adult Tumor Lysis Syndrome: An
Evidenced-Based Review. J Clin Oncol 26(16): 2767-2778, 2008
4- Zonfrillo MR: Management of pediatric tumor lysis syndrome in the
emergency department. Emerg Med Clin North Am. 27(3):497-504, 2009
5- Cairo MS, Coiffier B, Reiter A, Younes A; TLS Expert Panel:
Recommendations for the evaluation of risk and prophylaxis of tumour
lysis syndrome (TLS) in adults and children with malignant diseases: an
expert TLS panel consensus. Br J Haematol. 149(4):578-86, 2010
6- Agrawal AK, Feusner JH: Management of tumour lysis syndrome in
children: what is the evidence for prophylactic rasburicase in
non-hyperleucocytic leukaemia? Br J Haematol. Jan 23, 2011
Branchio-Oto-Renal Syndrome
The Branchio-oto-renal (BOR) syndrome
is a rare autosomal dominant disorder with incomplete penetrance and
extremely variable phenotypic expressivity. Main clinical features are
due to congenital abnormal development of the first and second
branchial arches and urinary tract. Others anomalies include early
hearing impairment, preauricular pits, deformity of pinna, external
auditory canal stenosis, branchial fistula and renal anomalies. Renal
anomalies are always present and consist of agenesis, hypoplasia or
renal dysplasia, ureteropelvic junction obstruction, vesicoureteral
reflux and calyceal diverticula. There also can be bifid kidneys with
double ureters and calyceal anomalies. Renal agenesia and dysplasia are
the causes of end-stage renal disease in these children. Bilateral
renal agenesis is the extreme, leading to a miscarriage or immediate
neonatal death. The syndrome gene maps to chromosome 8q13.3 called the
EYA1 gene. The BOR syndrome should be included in the differential
diagnosis of deafness and chronic renal failures in children.
References:
1- Pierides AM, Athanasiou Y, Demetriou K, Koptides M, Deltas CC. A
family with the branchio-oto-renal syndrome: clinical and genetic
correlations. Nephrol Dial Transplant. 17(6):1014-8, 2002
2- Schiff M, Parchoux B, Cochat P. Ear and kidney malformations with
renal failure in an infant: what is the link? Nephrol Dial Transplant.
18(8):1673-4, 2003
3- Kulkarni ML, Deshmukh S, Kumar A, Kulkarni PM. Branchio-oculo-facial syndrome. Indian J Pediatr. 72(8):701-3, 2005
4- Li Y, Manaligod JM, Weeks DL. EYA1 mutations associated with the
branchio-oto-renal syndrome result in defective otic development in
Xenopus laevis. Biol Cell. 17;102(5):277-92, 2010
5- Morisada N, Rendtorff ND, Nozu K, Morishita T, Miyakawa T, Matsumoto
T, Hisano S, Iijima K, Tranebjaerg L, Shirahata A, Matsuo M, Kusuhara
K. Branchio-oto-renal syndrome caused by partial EYA1 deletion due to
LINE-1 insertion. Pediatr Nephrol. 25(7):1343-8, 2010
PSU Volume 36 No 05 MAY 2011
Ehlers-Danlos Syndrome
Ehlers-Danlos syndrome (EDS), also
known as cutis hyperelastica, is a group of inherited connective tissue
disorders caused by a defect in the synthesis of collagen. Depending on
the individual mutation the severity of the syndrome can vary from mild
to severe. Several types of EDS have been categorized with type 4
considered the most dreadful considering the high propensity of these
individuals to develop life-threatening arterial and digestive
complications. EDS type 4 is an autosomic dominant defect (missense
mutation in the COL3A1 gene) characterize by a fascial acrogeria
appearance (large eyes, small chin, thin nose, lobeless ears), small
stature with slim build, and thin pale translucent skin. Children with
EDS have poor wound healing, hypermobile joints, clotting anomalies,
spontaneous pneumothorax and recurrent hernias. Among the catastrophic
events associated with type 4 EDS we can find arterial dissection or
tear caused by deterioration of congenital fragile tissue leading to
hematoma, false aneurysm and intracavitary bleeding. They are
responsible for the majority of deaths. The next set of complications
are spontaneous and recurrent perforation of the colon associated with
a significant risk of leakage after anastomosis and spontaneous
perforation or bleeding of the uterus. There is no cure for EDS and
management is supportive.
References:
1- Liem MS, van der Graaf Y, Beemer FA, van Vroonhoven TJ: Increased
risk for inguinal hernia in patients with Ehlers-Danlos syndrome.
Surgery. 122(1):114-5, 1997
2- Iglesias JL, Renard T: Diaphragmatic hernia in an 8-year-old with Ehlers-Danlos syndrome. Pediatr Surg Int. 13(8):553-5, 1998
3- Lin IC, Ko SF, Shieh CS, Huang CF, Chien SJ, Liang CD: Recurrent
congenital diaphragmatic hernia in Ehlers-Danlos syndrome. Cardiovasc
Intervent Radiol. 29(5):920-3, 2006
4- Demirogullari B, Karabulut R, Demirtola A, Karabulut B, Gol IH,
Aybay C, Symoens S, Sonmez K, Basaklar AC, Kale N: A novel
mutation in the vascular Ehlers-Danlos syndrome: a case presenting with
colonic perforations. J Pediatr Surg. 41(8):e27-30, 2006
5- Hingorjo MR: Ehlers-danlos syndrome--clinical presentation. J Pak Med Assoc. 58(5):279-81, 2008
6- Behjati S, Knight Y, Borgstein R, Goebells A, Myint F: A
life-threatening complication of Ehlers-Danlos syndrome. Br J Hosp Med
(Lond). 70(6):360, 2009
MALT Lymphoma
Marginal zone mucosa-associated
lymphoid tissue (MALT) lymphomas comprised a group of indolent B-cell
non-Hodgkin lymphomas which are rare to find in pediatric patients. The
gastrointestinal tract is the predominant site for this type of MALT
extranodal non-Hodgkin lymphoma. Almost one-third of the patient with
malignant lymphomas has involvement of the stomach (most commonly),
small intestine and large intestine. Other sites include salivary
gland, tonsils, lungs, thyroid, conjunctiva and even skin. The main
categories for MALT lymphomas are the low- and high-grade B-cell
MALTomas with or without a low grade component. Small centrocyte-like
cells, lymphoepithelial lesions, and reactive lymphoid follicles are
the main specific histopathologic features of low-grade MALTomas. On
the contrary, large high-grade cells, which usually infiltrate in
sheets and between glands without forming lymphoepithelial lesions
characterize the high-grade B-cell MALToma. The high-grade MALTomas
have shown a worse prognosis than low-grade and mixed types MALTomas.
Acquired MALTomas may develop as a reaction to autoimmune disease and
infection. Helicobacter pylori infection predisposes to development of
MALTomas in the stomach. Management of MALTomas includes surgical
resection where anatomically feasible along with adjuvant chemotherapy.
References:
1- Kurugoglu S, Mihmanli I, Celkan T, Aki H, Aksoy H, Korman U:
Radiological features in paediatric primary gastric MALT lymphoma and
association with Helicobacter pylori.Pediatr Radiol. 32(2):82-7, 2002
2- Karabulut R, Sönmez K, Türkyilmaz Z,
Yilmaz Y, Akyürek N, Başaklar AC, Kale N:
Mucosa-associated lymphoid tissue lymphoma in the appendix, a lead
point for intussusception. J Pediatr Surg. 40(5):872-4, 2005
3- Dargent JL, Devalck C, De Mey A, Vandeweyer E, Lespagnard L, Heimann
P: Primary cutaneous marginal zone B-cell lymphoma of MALT type in a
child. Pediatr Dev Pathol. 9(6):468-73, 2006
4- Kojima M, Nakamura N, Shimizu K, Tamaki Y, Itoh H, Nakamura S:
Marginal zone B-Cell lymphoma among primary B-Cell lymphoma of
Waldeyer's ring: histopathologic and immunohistochemical study of 16
tonsillectomy specimens. Int J Surg Pathol. 16(2):164-70, 2008
5- Wang T, Gui W, Shen Q: Primary gastrointestinal non-Hodgkin's
lymphoma: clinicopathological and prognostic analysis.Med Oncol.
27(3):661-6, 2010
6- Marte A, Sabatino MD, Cautiero P, Accardo M, Romano M, Parmeggiani
P: Unexpected finding of laparoscopic appendectomy: appendix MALT
lymphoma in children.Pediatr Surg Int. 24(4):471-3, 2008
Central Precocious Puberty
Central precocious puberty (CPP)
occurs with premature activation of the hypothalamic-pituitary-gonadal
axis. CPP is defined as the onset of secondary characteristics
associated with increased linear growth velocity and accelerated bone
maturation occurring before the age of seven to 8 years in girls, and
nine years in boys. CPP is more common in girls than boys. If left
untreated precocious puberty results in early epiphyseal closure and
short final stature. The most common cause of CPP is idiopathic caused
by early onset of luteinizing hormone, follicle stimulating hormone and
estradiol secretion. The goal of therapy is to restore a prepubertal
state attenuating the deleterious effect of early sex steroid exposure
on physical development, skeletal maturation and ultimate adult height.
This can be achieved with parenteral administration of long-acting
gonadotropin releasing hormone agonists (GnRHa) which has been found to
be effective in retarding progression of secondary sexual
characteristics, preventing menses, slowing bone-age maturation and
improving final height. Since GnRHa monthly injections are painful, a
subcutaneous microporous hydrogel implant that release GnRHa on a daily
basis has been developed and tested efficaciously in suppressing
clinical and laboratory parameters of puberty for one year. The implant
is placed in the inner aspect of the arm under local or general
anesthesia as a minor procedure.
References:
1- Arrigo T, Cisternino M, Galluzzi F, Bertelloni S, Pasquino AM,
Antoniazzi F, Borrelli P, Crisafulli G, Wasniewska M, De Luca F:
Analysis of the factors affecting auxological response to GnRH agonist
treatment and final height outcome in girls with idiopathic
central precocious puberty. Eur J Endocrinol. 141(2):140-4, 1999
2- Hirsch HJ, Gillis D, Strich D, Chertin B, Farkas A, Lindenberg T,
Gelber H, Spitz IM: The histrelin implant: a novel treatment for
central precocious puberty. Pediatrics. 116(6):e798-802, 2005
3- Lazar L, Padoa A, Phillip M: Growth pattern and final height after
cessation of gonadotropin-suppressive therapy in girls with central
sexual precocity. J Clin Endocrinol Metab. 92(9):3483-9, 2007
4- Eugster EA, Clarke W, Kletter GB, Lee PA, Neely EK, Reiter EO,
Saenger P, Shulman D, Silverman L, Flood L, Gray W, Tierney D: Efficacy
and safety of histrelin subdermal implant in children with central
precocious puberty: a multicenter trial.J Clin Endocrinol Metab.
92(5):1697-704, 2007
5- Carel JC, Eugster EA, Rogol A, Ghizzoni L, et al: Consensus
statement on the use of gonadotropin-releasing hormone analogs in
children. Pediatrics. 123(4):e752-62, 2009
6- GÅ‚ab E, Barg E, Wikiera B, Grabowski M,
Noczyńska A: Influence of GnRH analog therapy on body mass
in central precocious puberty. Pediatr Endocrinol Diabetes Metab.
15(1):7-11, 2009
PSU Volume 36 No 06 JUNE 2011
Venous Thromboembolism in Children
Venous thromboembolism (VTE) has a low
risk of developing during childhood. Venous thromboembolism arises from
stasis, vascular endothelial damage or a hypercoagulable state
(Virchow's triad). Surgical risk factors include indwelling venous
catheters, reduced lack of mobility after surgery, and intrinsic
hematologic prothrombotic factors (factor V Leiden, antiphospholipid
antibodies or prothrombin mutations). VTE disease in children is more
common in neonates than older children and appears to affect mostly the
upper limb veins, followed by iliac veins and inferior vena cava
with the main danger being that of pulmonary embolism sometimes
confused with a pneumonic process. Acquired inflammatory conditions,
septicemia, staph infections, HIV are risk factors for VTE since they
induce a hypercoagulable state due to transitory decrease of
anticoagulant protein S and increase in procoagulant Factor VIII
activity. Other risk factors in children are central venous catheter,
blood dyscrasias, obesity and malignancy. Traumatic intimal tears
after pelvic or orthopedic trauma and prolonged surgical procedures can
also predispose to venous thromboembolism. Diagnosis of VTE is made by
noninvasive radiologic imaging. Management of VTE in children in high
risk scenarios or with established diagnosis consist of
low-molecular-weight heparin. Use of plasminogen activators is reserved
for the management of thrombi that threaten life, limb or a specific
organ.
References:
1- Tormene D, Gavasso S, Rossetto V, Simioni P: Thrombosis and
thrombophilia in children: a systematic review. Semin Thromb Hemost.
32(7):724-8, 2006
2- Beardsley DS: Venous thromboembolism in the neonatal period. Venous
thromboembolism in the neonatal period. Semin Perinatol. 31(4):250-3,
2007
3- Goldenberg NA, Bernard TJ: Venous thromboembolism in children. Pediatr Clin North Am. 55(2):305-22, 2008
4- Goldenberg NA, Bernard TJ: Venous thromboembolism in children. Hematol Oncol Clin North Am. 24(1):151-66, 2010
5-Long E, Pitfield AF, Kissoon N: Anticoagulation therapy: indications,
monitoring, and complications. Pediatr Emerg Care. 27(1):55-61, 2011
6- Tshifularo N, Arnold M, Moore SW: Thromboembolism and venous
thrombosis of the deep veins in surgical children-an increasing
challenge? J Pediatr Surg. 46(3):433-6, 2011
Pancreatic Mediastinal Pseudocyst
Most pancreatic pseudocysts are
located in the peripancreatic retroperitoneum. On very rare occasions
active pancreatic secretions from a pancreatitis can gain access to the
mediastinum causing a mediastinal pseudocyst. Access to the posterior
mediastinum can occur through the esophageal hiatus most commonly, and
also through extension through the aortic hiatus or direct erosion
through the diaphragm. Children with pancreatic mediastinal pseudocysts
can present with abdominal pain, anorexia, vomiting, dysphagia,
respiratory distress and dyspnea. The etiology of the pancreatitis in
such children can be a congenital ductal anomaly (pancreas divisum), a
posttraumatic event or idiopathic. Diagnosis is made with CT Scan as it
delineates location, extent and anatomic relationship to the adjacent
mediastinal structures. The cyst contains elevated amylase levels. MRCP
provides images of the pancreatic duct. The decision whether to wait
for spontaneous resolution, cyst maturation or perform immediate
intervention is dictated by the child symptoms. Increasing size,
bleeding, infection, or compression symptoms, invasion or rupture
warrants intervention. Intervention can consist of endoscopic
transmural drainage (transpapillary, transgastric, transesophageal),
internal surgical drainage (cystogastrostomy or cystoenterostomy),
resection procedure or CT-guided percutaneous drainage.
References:
1- Sharma S, Puri S, Chaturvedi P, Kulshreshtha R, Baijal VN:
Mediastinal pancreatic pseudocyst following traumatic rupture of
diaphragm. Pediatr Radiol. 18(4):337, 1988
2- Crombleholme TM, deLorimier AA, Adzick NS, Longaker MT, Harrison MR,
Cox KL, Heyman MB: Mediastinal pancreatic pseudocysts in children. J
Pediatr Surg. 25(8):843-5, 1990
3- Bonnard A, Lagausie P, Malbezin S, Sauvat E, Lemaitre AI, Aigrain Y:
Mediastinal pancreatic pseudocyst in a child. A thoracoscopic approach.
Surg Endosc. 15(7):760, 2001
4- Snajdauf J, Rygl M, Kalousová J, Kucera A,
Petrů O, Pýcha K, Mixa V, Keil R,
HrÃbal Z: Surgical management of major pancreatic injury in
children. Eur J Pediatr Surg. 17(5):317-21, 2007
5- Gupta R, Munoz JC, Garg P, Masri G, Nahman NS Jr, Lambiase LR:
Mediastinal pancreatic pseudocyst--a case report and review of the
literature. MedGenMed. 11;9(2):8, 2007
6- Nuwayhid Z, Kassira N, Neville HL, Casillas J, Sola JE: Percutaneous
retropleural drainage of a posttraumatic pancreatic mediastinal
pseudocyst in a child. J Pediatr Surg. 46(3):585-7, 2011
Abdominal Pain in Sickle Cell Disease
Children with Sickle Cell Disease
(SCD) present fairly commonly with acute abdominal pain. The pain must
be differentiated between ischemia from a vasoocclusive crisis or
another abdominal disease requiring surgery. Specific causes for
abdominal pain in SCD include hepatic crisis, cholelithiasis, splenic
sequestration and pancreatitis. Management consists of oxygenation,
hydration and analgesia. When the child with SCD develops classic
features of an acute surgical abdomen such as vomiting, tender
distended abdomen with rigidity, involuntary guarding and rebound
tenderness, the disease process might require surgery. Ultrasound &
CT Scan imaging helps discover whether the child has bowel obstruction
from infarction, a perforated viscus, appendicitis or cholecystitis.
The incidence of appendicitis has been found to be lower in children
with SCD. Close clinical monitoring is essential. Conservative therapy
is warranted in the large majority of patients with SCD who present
with acute abdominal pain. High pain scores, older age, increased
polymorphonuclear count and homozygous SCD types are associated with
prolonged hospital stay during vasoocclusive crisis. Surgical
consultation is necessary if a surgical cause is suspected or the cause
is not obvious after a thorough evaluation.
References:
1- Rogovik AL, Li Y, Kirby MA, Friedman JN, Goldman RD: Admission and
length of stay due to painful vasoocclusive crisis in children. Am J
Emerg Med. 27(7):797-801, 2009
2- Leung AK, Sigalet DL: Acute abdominal pain in children. Am Fam Physician. 67(11):2321-6, 2003
3- Antal P, Gauderer M, Koshy M, Berman B: Is the incidence of
appendicitis reduced in patients with sickle cell disease? Pediatrics.
101(1):E7, 1998
4- Kudsk KA, Tranbaugh RF, Sheldon GF: Acute surgical illness in patients with sickle cell anemia. Am J Surg. 142(1):113-7, 1981
5- Bonadio WA: Clinical features of abdominal painful crisis in sickle cell anemia. J Pediatr Surg. 25(3):301-2, 1990
6- Ahmed S, Shahid RK, Russo LA: Unusual causes of abdominal pain:
sickle cell anemia. Best Pract Res Clin Gastroenterol. 19(2):297-310,
2005