PEDIATRIC SURGERY UPDATE ©
VOLUME 39, 2012
PSU Volume 39 No 01 JULY 2012
Intrathoracic Kidney
Congenital intrathoracic kidney is a very rare ectopic
anomaly of the kidney. Most ectopic kidneys are found in the pelvic or
lower lumbar region. Delayed closure of the pleuroperitoneal membrane
as it occurs in diaphragmatic hernia permits excessive cranial
migration of the kidney. Other postulates that delayed ingrowth of the
ureter into the metanephros results in prolongation of the ascending
process involving a long ureter and prolonged migration. In either case
the kidney assumes a thoracic position. Intrathoracic kidneys do not
display dysplastic parenchymal architecture and the kidneys are usually
functional. Thoracic kidneys can be classified as thoracic renal
ectopia with closed diaphragm, eventration of the diaphragm,
diaphragmatic hernia (congenital diaphragmatic defects or acquired
hernia such as Bochdalek hernia), and traumatic rupture of the
diaphragm with renal ectopia. Conditions that affect the normal kidney
such as ureteric stones, hydronephrosis, and renal cell carcinoma has
also been reported to occur in intrathoracic kidneys later in life.
Intrathoracic kidney can be found as a unique anomaly or associated
with a diaphragmatic hernia defect, respiratory symptoms and bowel
herniation within the thorax. Diagnosis is confirmed with CT or MR
imaging. When presenting as a unique anomaly not associated to a bowel
herniation or respiratory symptoms they can be managed conservatively
with regular follow-up since normal growth and development of the
affected kidney occurs. Surgical treatment has been reserved for
children with associated bowel herniation or respiratory compromise. In
such situation repair of the diaphragmatic defect and nephropexy is in
order.
References:
1- Ho HF, Liaw SB, Chang PY: Delayed manifestation of congenital
diaphragmatic hernia with intrathoracic kidney: report of one case.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 38(1):61-4, 199
2- Oon PC, Shen HN, Yang PC: Intrathoracic kidney. J Formos Med Assoc. 104(2):120-2, 2005
3- Panda B, Rosenberg V, Cornfeld D, Stiller R: Prenatal
diagnosis of ectopic intrathoracic kidney in a fetus with a left
diaphragmatic hernia. J Clin Ultrasound. 37(1):47-9, 2009
4- Rouanne M, Le Mandat A, Dorgeret S, Philippe-Chomette P, El
Ghoneimi A: A rare case of ectopic intrathoracic kidney in a 1-year-old
child. Urology. 76(1):57-9, 2010
5- Fiaschetti V, Velari L, Gaspari E, Mastrangeli R, Simonetti
G: Adult intra-thoracic kidney: a case report of bochdalek hernia. Case
Report Med. 2010. pii: 975168, 2010
6- Murphy JJ, Altit G, Zerhouni S: The intrathoracic kidney: should we fix it? J Pediatr Surg 47: 970-973, 2012
Port Inversion
Ports connected to central venous catheter are necessary for
a variety of reasons such as management of children who require
chemotherapy, prolonged antibiotic administration, frequent blood
sampling, parenteral nutrition and transfusion of blood and blood
products. Totally implantable access ports improve the quality of life
and have the advantage of not requiring an external dressing.
Ports are inserted as totally implantable devices underneath the
subcutaneous tissue infraclavicularly most commonly or under the
pectoralis fascia. The most common complications of port application
and maintenance include occlusion, vein thrombosis, port pocket
infection, catheter related sepsis, device rotation or dislodgement,
catheter migration, skin necrosis, pocket hematoma and exposed port.
Port inversion occurs when the port completes a 90-degree rotation due
to mobility and the caretaker cannot cannulate the port diaphragm. A
simple lateral chest film will reveal the problem of port inversion.
Port inversion is seen more commonly after subcutaneous placement than
subpectoral fascia placement. Port implantation in the subpectoral
fascia pocket has been found to have a lower rate of skin complications
than the subcutaneous pocket implantation site. Whether to fix
the port with sutures to avoid rotation or inversion is a matter of
debate, since suturing will need more extensive dissection during
removal. Port inversion will need revision.
References:
1- Dillon PA, Foglia RP: Complications associated with an implantable vascular access device. J Pediatr Surg. 41(9):1582-7, 2006
2- Charles HW, Miguel T, Kovacs S,
Gohari A, Arampulikan J, McCann JW: Chest port placement with use of
the single-incision insertion technique. J Vasc Interv Radiol.
20(11):1464-9, 2009
3- McNulty NJ, Perrich KD, Silas AM,
Linville RM, Forauer AR: Implantable subcutaneous venous access
devices: is port fixation necessary? A review of 534 cases. Cardiovasc
Intervent Radiol. 33(4):751-5, 2012
4- Rouzrokh M, Shamsian BS,
KhaleghNejad Tabari A, Mahmoodi M, Kouranlo J, Manafzadeh G, Arzanian
MT, Fallah F, Anoush M, Abdollah Gorji F: Totally implantable
subpectoral vs. subcutaneous port systems in children with malignant
diseases. Arch Iran Med. 12(4):389-94, 2009
Idiopathic Megaduodenum
Idiopathic megaduodenum is a rare congenital condition defined as a
massive dilatation of the duodenum without evidence of mechanical
extraluminal or intraluminal obstruction. The etiology is unknown. It
may be caused by neuromuscular disorder or abnormal gut collagen and
interticium. Duodenal dilatation and stasis may be caused by decreased
peristalsis and accumulation of motilin in the duodenal cavity.
Children with idiopathic megaduodenum (ID) present with recurrent
abdominal distension, nausea vomiting, diarrhea and malnutrition.
Diagnosis is obtained with upper GI series. Management is surgical and
should achieve drainage of the duodenum, reduce the capacity of the
duodenum and cut off the forceful feeding of the duodenum. This can be
accomplished more physiologically with tapering duodenoplasty and
side-to-side gastrojejunostomy, duodenojejunostomy or Roux-en-Y
duodenojejunostomy. A familiar variety of this condition has been
described in the literature. Also, children with chronic intestinal
pseudoobstruction can present with idiopathic megaduodenum.
References:
1- McClenahan JE, Fisher B: Idiopathic megaduodenum; report of a case. Am J Dig Dis. 15(12):414-6, 1848
2- Eaves ER, Schmidt GT: Chronic idiopathic megaduodenum in a family. Aust N Z J Med. 15(1):1-6, 1985
3- Cheng SC, Sanderson CR, Way NJ, Yoong PM: Familial idiopathic megaduodenum. Aust N Z J Surg. 57(11):879-82, 1987
4- Svartsja B, Sulonen H, Leino R: [Megaduodenum as a first sign of chronic idiopathic intestinal
pseudo-obstruction]. Duodecim. 106(7):578-81, 1990
5- Nichol PF, Stoddard E, Lund DP,
Starling JR: Tapering duodenoplasty and Roux-en-Y duodenojejunostomy in
the management of adult megaduodenum. Surgery. 135(2):222-4, 2004
6- Zhang XW, Abudoureyimu A, Zhang
TC, Zhao JR, Fu LB, Lin F, Qiu XH, Chen YJ: Tapering duodenoplasty and
gastrojejunostomy in the management of idiopathic megaduodenum in
children. J Pediatr Surg. 47(5):1038-42, 2012
PSU Volume 39 No 02 AUGUST 2012
Propranolol for Hemangiomas
The most common benign tumor in
infancy is a hemangioma. Most infantile hemangiomas appear during the
first few weeks of life, proliferate during the first two years of life
and involute during childhood. Involution is characterized by changing
from a cellular into a vascular nature with progressive deposition of
perivascular fibrofatty tissue, abundant mast cells and less
endothelial cell proliferation. Management of hemangiomas has included
steroids, laser, cryosurgery, interferon, vincristine and resection.
Recently, propranolol, a pure selective beta-adrenergic antagonist has
been found to hasten involution of infantile hemangiomas. The suggested
dose of management of propranolol is 2 mg/kg/day in three divided dose.
Children receiving this kind of therapy should have a full cardiologic
evaluation pretreatment. The possible mechanism of action for the
effect on involution of hemangiomas by propranolol includes
vasoconstriction (inhibition of vasodilation mediated by adrenaline),
inhibition of angiogenesis (reduced expression of VEGF),
induction of apoptosis of endothelial cells and inhibiting nitric oxide
production. Propranolol is efficient and highly tolerated. The first
noticeable effect is change in color of the hemangioma followed by
softening of the lesion and finally regression of size. Optimal
length of treatment should cover the proliferative phase and last until
maximal improvement has been achieved. The excellent outcome of this
therapy has prompted its recommendation as first line therapy of
hemangiomas. Infants with renal or hepatic dysfunction, cardiovascular
disease, asthma, diabetes or glaucoma should not be managed with
propranolol. Side effects of therapy to monitor include bradycardia,
hypotension and hypoglycemia.
References:
1- Hogeling M, Adams S, Wargon O: A randomized controlled trial of
propranolol for infantile hemangiomas. Pediatrics. 128(2):e259-66, 2011
2- Dai Y, Hou F, Buckmiller L, Fan CY, Saad A, Suen J, Richter GT:
Decreased eNOS protein expression in involuting and propranolol-treated
hemangiomas. Arch Otolaryngol Head Neck Surg. 138(2):177-82, 2012
3- Talaat AA, Elbasiouny MS, Elgendy DS, Elwakil TF: Propranolol
treatment of infantile hemangioma: clinical and radiologic evaluations.
J Pediatr Surg. 47(4):707-14, 2012
4- Hsu TC, Wang JD, Chen CH, Chang TK, Wang TM, Chou CM, Lin HK:
Treatment with propranolol for infantile hemangioma in 13 Taiwanese
newborns and young infants. Pediatr Neonatol. 53(2):125-32, 2012
5- Chim H, Armijo BS, Miller E, Gliniak C, Serret MA, Gosain AK:
Propranolol Induces Regression of Hemangioma Cells Through
HIF-1α-Mediated Inhibition of VEGF-A. Ann Surg. 2012 May
10
6- Chung SH, Park DH, Jung HL, Shim JW, Kim DS, Shim JY, Park MS, Koo
HH: Successful and safe treatment of hemangioma with oral propranolol
in a single institution. Korean J Pediatr. 55(5):164-70, 2012
Vinyl Glove Ingestion
Toddlers, children with pica and some
mentally retarded children introduce objects into their mouth
constantly. They are the group with the higher incidence of ingested
foreign body. Fortunately most ingested foreign body passes through the
gastrointestinal tract without causing problems. Most common foreign
body site of impaction is the distal ileum, followed by pylorus,
duodenal loop, rectosigmoid and anus in that order. Vinyl glove
ingestion has been reported to occur in handicap children causing bowel
obstruction, perforation and fistulization. Due to the inability to
communicate the diagnosis is usually late in mentally retarded
patients. Vinyl glove ingestion produces a bezoar in the stomach or
distal ileum. The problem is that these soft pliable gloves become hard
and irregular and develop sharp cutting edges after ingestion. The
glove hardens creating a barrier to the movement of food or succus
entericus. This produces a bowel obstruction. Management consists of
surgical removal by either open or laparoscopic technique. Endoscopic
removal is not recommended. Vinyl gloves should be removed from the
immediate proximity of mentally retarded patients or patients with
pica. Institution and families caring for mentally retarded children
should monitor the accessibility of vinyl glove or find a safer
substitute.
References:
1- Selcuk H, Unal H, Korkmaz M, Yilmaz U: Subacutely formed bezoar
resulting from accidentally ingested industrial material.J Chin Med
Assoc. 72(4):202-3, 2009
2- Burstein I, Steinberg R, Zer M: Small bowel obstruction and covered
perforation in childhood caused by bizarre bezoars and foreign bodies.
Isr Med Assoc J. 2(2):129-31, 2000
3- Pitiakoudis M, Tsaroucha A, Mimidis K, Constantinidis T,
Anagnostoulis S, Stathopoulos G, Simopoulos C: Esophageal and small
bowel obstruction by occupational bezoar: report of a case. BMC
Gastroenterol. 3:13, 2003
4- Kamal I, Thompson J, Paquette DM: The hazards of vinyl glove
ingestion in the mentally retarded patient with pica: new
implications for surgical management. Can J Surg. 42(3):201-4, 1999
5- Stringel G, Parker M, McCoy E: Vinyl glove ingestion in children: a word of caution. J Pediatr Surg. 47(5):996-8, 2012
Gastrointestinal Basidiobolomycosis
Gastrointestinal Basidiobolomycosis
(GIB) is a very rare but aggressive fungal infection rarely reported in
the literature. The disease is caused by the fungus Basidiobolus
Ranarum, an environmental saprophyte that infects skin, subcutaneous
tissue and rarely the gastrointestinal tract. GIB presents with
nonspecific signs and symptoms such as abdominal pain, mass and fever.
Patient comes from tropical and subtropical countries.
Radiographic findings are consistent with either malignancy or
inflammation. Patients have elevated WBC with eosinophilia. GI biopsy
specimen shows pleomorphic hyphae surrounded by eosinophilic
inflammation (Splendore-Hoeppli phenomenon). Complications include
bowel perforation, obstructive uropathy, esophageal varices, and
duodenobiliary fistula Definite diagnosis requires culture of the
organism. The fungus enters the GI tract through ingestion of
contaminated soil, animal feces or food. Management consists of
resection of affected inflammatory bowel and debridement of involved
tissue followed by more than three months of antifungal therapy with
itraconazole. GIB causes significant morbidity and mortality.
References:
1- Al Jarie A, Al-Mohsen I, Al Jumaah S, Al Hazmi M, Al Zamil F, Al
Zahrani M, Al Modovar E, Al Dayel F, Al Arishii H, Shehrani D, Martins
J, Al Mehaidib A, Rossi L, Olaiyan I, Le Quesne G, Al-Mazrou A:
Pediatric gastrointestinal basidiobolomycosis. Pediatr Infect Dis J.
22(11):1007-14, 2003
2- Nemenqani D, Yaqoob N, Khoja H, Al Saif O, Amra NK, Amr SS:
Gastrointestinal basidiobolomycosis: an unusual fungal infection
mimicking colon cancer. Arch Pathol Lab Med. 133(12):1938-42, 2009
3- El-Shabrawi MH, Kamal NM: Gastrointestinal basidiobolomycosis in
children: an overlooked emerging infection? J Med Microbiol. 60(Pt
7):871-80, 2011
4- El-Shabrawi MH, Kamal NM, Jouini R, Al-Harbi A, Voigt K, Al-Malki T:
Gastrointestinal basidiobolomycosis: an emerging fungal infection
causing bowel perforation in a child. J Med Microbiol. 60(Pt
9):1395-402, 2011
5- Vikram HR, Smilack JD, Leighton JA, Crowell MD, De Petris G:
Emergence of gastrointestinal basidiobolomycosis in the United States,
with a review of worldwide cases. Clin Infect Dis. 54(12):1685-91, 2012
6- Al-Shanafey S, Alrobean F, Hussain IB: Surgical management of
gastrointestinal basidiobolomycosis in pediatric patients. J Pediatr
Surg. 47(5):949-51, 2012
PSU Volume 39 No 03 SEPTEMBER 2012
Median Arcuate Ligament Syndrome
Median arcuate ligament
syndrome (MALS) also known as celiac artery compression is a rare
condition that occurs when the fibrous portion of the diaphragmatic
crura known as the median arcuate ligament compresses the proximal
celiac artery trunk. This compression can also occur with an excessive
amount of sympathetic nerves or ganglions. The compression usually
occurs during expiration. MALS is a diagnosis of exclusion
characterized by weight loss, postprandial abdominal pain, diarrhea,
nausea, vomiting and epigastric bruit. Most cases are female. The
diagnosis can be established using invasive arteriography or
non-invasive with CT angiography, magnetic resonance angiography and
Doppler ultrasound studies. CT-angio demonstrates a focal hook
appearance narrowing in the proximal celiac artery. Doppler studies
shows variations in peak systolic velocity with a marked increase
during expiration and functional geometric changes such as celiac trunk
deflection. Treatment options for MALS include surgical or laparoscopic
division of the median arcuate ligament, celiac ganglion destruction,
endovascular stenting or bypass surgery. Advantages of the laparoscopic
approach are the imaging magnification, fewer postoperative adhesions,
smaller incisions, better cosmesis and shorter hospital stay. Besides
relieving the mechanical effect of the arcuate ligament, the
interruption of somatic or sympathetic fibers in the course of division
may be responsible for the improvement in abdominal pain due to relieve
of vasospasm.
References:
1- Alehan D, Dogan OF: Pediatric surgical image. A rare case: celiac
artery compression syndrome in an asymptomatic child. J Pediatr
Surg. 39(4):645-7, 2004
2- Gander S, Mulder DJ, Jones S, Ricketts JD, Soboleski DA, Justinich
CJ: Recurrent abdominal pain and weight loss in an adolescent: celiac
artery compression syndrome. Can J Gastroenterol. 24(2):91-3, 2010
3- Said SM, Zarroug AE, Gloviczki P, Shields RC: Pediatric median
arcuate ligament syndrome: first report of familial pattern and
transperitoneal laparoscopic release. J Pediatr Surg. 45(12):e17-20,
2010
4- Aschenbach R, Basche S, Vogl TJ: Compression of the celiac trunk
caused by median arcuate ligament in children and adolescent subjects:
evaluation with contrast-enhanced MR angiography and comparison with
Doppler US evaluation. J Vasc Interv Radiol. 22(4):556-61, 2011
5- Ozel A, Toksoy G, Ozdogan O, et al: Ultrasonographic diagnosis of
median arcuate ligament syndrome: a report of two cases. Medical
Ultrasonography 14(2): 154-157, 2012
6- Wani S, Wakde V, Patel R, et al: Laparoscopic release of median arcuate ligament. J Minim Access Surg 8(1): 16-18, 2012
Giant Omphalocele
A giant omphalocele is defined as a defect larger than 10 cm
in length that harbors the liver. Prenatally diagnosed giant
omphaloceles will need cesarean section as route of birth to avoid
fetal liver rupture. Management of giant omphalocele has a high
morbidity and mortality due to the defect size, visceroabdominal
disproportion, and the associated congenital and genetic malformations.
The large size of the defect and small abdominal cavity creates a
situation where primary closure is almost impossible unless some sort
of stage reduction is tailored. Pulmonary hypoplasia, genetic defects
and cardiac malformation are the source of early mortality in these
babies. The pendulum of management of giant omphalocele has moved
toward a more conservative initial management using topical coverage
creams to create granulation tissue and skin on top of the membrane
followed by repair of the ventral hernia much later in life when the
medical condition of the child permits. Silver sulfadiazine (Silvadene)
provides a moist wound healing environment that promotes epithelization
and simultaneously minimizes the risk of invasive infection including
antifungal coverage. Silver toxicity, though rare can include seizures,
peripheral neuropathy, ocular pathology, nephrotic syndrome, raised
liver enzymes, leukopenia and algiria. For smaller size defects the use
of silo, tissue expanders, biologic mesh, vacuum-assisted closure or
component separation technique closure is indicated. Giant omphalocele
is associated with deficits in developmental achievements in most of
the affected infants ranging from mild to profound delays.
References:
1- Pacilli M, Spitz L, Kiely EM, Curry J, Pierro A: Staged repair of
giant omphalocele in the neonatal period. J Pediatr Surg. 40(5):785-8,
2005
2- Kilbride KE, Cooney DR, Custer MD: Vacuum-assisted closure: a new
method for treating patients with giant omphalocele. J Pediatr
Surg. 41(1):212-5, 2006
3- Lee SL, Beyer TD, Kim SS, Waldhausen JH, Healey PJ, Sawin RS,
Ledbetter DJ: Initial nonoperative management and delayed closure for
treatment of giant omphaloceles.J Pediatr Surg. 41(11):1846-9, 2006
4- van Eijck FC, de Blaauw I, Bleichrodt RP, Rieu PN, van der Staak FH,
Wijnen MH, Wijnen RM: Closure of giant omphaloceles by the abdominal
wall component separation technique in infants.J Pediatr Surg.
43(1):246-50, 2008
5- Danzer E, Gerdes M, D'Agostino JA, Bernbaum J, Siegle J, Hoffman C,
Rintoul NE, Liechty KW, Flake AW, Adzick NS, Hedrick HL: Prospective,
interdisciplinary follow-up of children with prenatally diagnosed giant
omphalocele: short-term neurodevelopmental outcome. J Pediatr
Surg. 45(4):718-23, 2010
6- Ein SH, Langer JC: Delayed management of giant omphalocele using
silver sulfadiazine cream: an 18-year experience. J Pediatr Surg.
47(3):494-500, 2012
Congenital Solid Tumors
Congenital solid tumors refer to masses of tissue that have
grown in location diverge from the normal pattern of development and do
not duplicate any normal structure of the body. Though rare prenatal
sonography has increased the rate of diagnosis. Abdominal tumors are
the most common prenatally diagnosed fetal tumors. Half of congenital
solid tumors are diagnosed at birth and two-third during the
first week of life. The most common congenital tumors are extracranial
teratoma, neuroblastoma, soft tissue tumors and brain tumors. The most
frequent benign tumor is teratoma, while the most frequent malignant is
neuroblastoma. Although easier to detect, cervical and mediastinal
tumors have a worse prognosis. Abdominal masses are more difficult to
detect but have a better prognosis. Management should be
conservative as possible with surgery playing a major role due to the
relative benign behavior of congenital cancer and the potential long
term effect of chemotherapy and radiotherapy.
References:
1-Parkes SE, Muir KR, Southern L, Cameron AH, Darbyshire PJ, Stevens
MC: Neonatal tumours: a thirty-year population-based study. Med Pediatr
Oncol. 22(5):309-17, 1994
2- Sbragia L, Paek BW, Feldstein VA, Farrell JA, Harrison MR, Albanese
CT, Farmer DL: Outcome of prenatally diagnosed solid fetal tumors. J
Pediatr Surg. 36(8):1244-7, 2001
3- Albert A, Cruz O, Montaner A, Vela A, et al: [Congenital solid tumors. A thirteen-year review].Cir Pediatr. 17(3):133-6, 2004
4- Mahony R, McParland P: Approaches to the management of antenatally
diagnosed congenital tumours.Pediatr Radiol. 39(11):1173-8, 2009
5- Lopez Almaraz R, Villafruela Alvarez C, Rodriguez Luis J, Domenech
Martinez E: [Neonatal neoplasms: a single-centre experience]. An
Pediatr (Barc). 65(6):529-35, 2006
6- Crocoli A, Bagolan P, Boldrini R, et al: Congenital Askin tumor with
favorable outcome: case report and review of the literature. J Pediatr
Surg 47(7): 1440-44, 2012
PSU Volume 39 NO 04 OCTOBER 2012
Trocar Injury
Injury by a trocar during a
laparoscopic or thoracoscopic procedure is a very serious complication
in surgery. The two most serious complications most likely to result in
death caused during entrance of a trocar are hemorrhage due to vessel
injury and infection due to bowel injury. The rate of trocar related
complication is less than 3%. The average incidence of trocar-related
vascular injury is 0.1%. Major vessel injury is almost invariably
operator error. Most vascular, bowel and local hemorrhage injury are
caused during the initial trocar insertion. There is some blind force
exertion that causes the blunt/sharp injury to the major vessel. The
vessels most frequently involved are the aorta, the iliac arteries, the
mesenteric vessels, and the vena cava. Force require to insert reusable
trocars is twice that for disposable trocars. Shielded trocars might
provide a margin of safety. Trocar use requires considerable training,
practice, skill, manual dexterity, adequate muscular strength,
knowledge of the associated risks, and careful patient selection. In
addition to laparoscopist-related issues (trocar insertion technique,
patient selection, injury recognition and effective intervention), the
lack of standard device designs, a lack of proven-effective fail-safe
features, and failure of patients to report symptoms in a timely manner
may also contribute to morbidity and mortality. Open (Hasson) entrance
or optical access trocars are recommended for patients with prior
abdominal surgery, small children, patients with lower abdomen skin
cannot be adequately stabilized for safe insertion or Veress needle.
References:
1- Montero M, Tellado MG, Rios J, Mendez R, Somoza I, Pais E, Vela D:
Aortic injury during diagnostic pediatric laparoscopy. Surg Endosc.
15(5):519, 2001
2- Schaefer M, Lauper M, Krahenbahl L: Trocar and Veress needle injuries during laparoscopy. Surg Endosc. 15(3):275-80, 2001
3- Thomas MA, Rha KH, Ong AM, Pinto PA, Montgomery RA, Kavoussi LR,
Jarrett TW: Optical access trocar injuries in urological laparoscopic
surgery. J Urol. 170(1):61-3, 2003
4- Opitz I, Gantert W, Giger U, Kocher T, Krahenbal L: Bleeding remains
a major complication during laparoscopic surgery: analysis of the SALTS
database. Langenbecks Arch Surg. 390(2):128-33, 2005
5- Yanke BV, Horowitz M: Safety of the Veress needle in pediatric laparoscopy. J Endourol. 21(7):695-7, 2007
6- Minervini A, Davenport K, Pefanis G, Keeley FX Jr, Timoney AG:
Prospective study comparing the bladeless optical access trocar versus
Hasson open trocar for the establishment of pneumoperitoneum in
laparoscopic renal procedures. Arch Ital Urol Androl. 80(3):95-8, 2008
7- Nakaoka T, Uemura S, Yoshida T, Tanimoto T, Shiokawa C, Harumoto K:
Umbilical center insertion method for initial trocar placement in
pediatric laparoscopic surgery. Osaka City Med J. 56(2):21-6, 2010
Alagille Syndrome
Alagille syndrome is an autosomal
dominant disorder characterized by paucity of intrahepatic bile ducts,
typical facies, congenital cardiac defects (pulmonary stenosis),
posterior embryotoxon of the eye and butterfly vertebra arch defect.
Due to paucity of bile ducts the baby presents with inefficient
bile excretion causing intrahepatic cholestasis, direct
hyperbilirubinemia and hypercholesterinemia. The clinical picture is
sometimes indistinguishable from biliary atresia needing cholangiogram
and liver biopsy for diagnosis. The vast majority of patients present
with jaundice and failure to thrive or cardiovascular symptoms before
six months of age. Mutation in the JAG-1 gene of chromosome 20p12 is
responsible for more than 90% of cases. There is no cure for Alagille
syndrome. The most disturbing manifestation includes pruritus and
xanthomas. Most children with Alagille syndrome can be managed
conservatively with choleretics (ursodeoxycholic acid), nutrition
optimization, fat-soluble vitamin supplementation and medication for
pruritus (cholestyramine, rifampin, naltrexone). Surgery is aimed at
reducing the pruritus consist of partial external biliary diversion
creating a conduit between gallbladder and skin using jejunum,
performing an ileal exclusion end to side ileocolostomy between
proximal ileum and right colon or partial internal biliary diversion
between gallbladder and ascending colon with jejunum. These procedures
do not improve growth and does not prevent progression of disease
toward liver failure. Liver transplant is used for liver failure.
Mortality is approximately 10%, with vascular accidents, cardiac
disease, and liver disease accounting for most deaths.
References:
1-Piccoli DA, Spinner NB: Alagille syndrome and the Jagged1 gene. Semin Liver Dis. 21(4):525-34, 2001
2- Spinner NB, Hutchinson AL, Krantz ID, Kamath BM: Alagille Syndrome.
In: Pagon RA, Bird TD, Dolan CR, Stephens K, Adam MP, editors.
GeneReviews [Internet]. Seattle (WA): University of Washington,
Seattle; 1993-. 2000 May 19.
3- Kamath BM, Loomes KM, Piccoli DA: Medical management of Alagille
syndrome. J Pediatr Gastroenterol Nutr. 50(6):580-6, 2012
4- Subramaniam P, Knisely A, Portmann B, Qureshi SA, Aclimandos WA,
Karani JB, Baker AJ: Diagnosis of Alagille syndrome-25 years of
experience at King's College Hospital. J Pediatr Gastroenterol Nutr.
52(1):84-9, 2011
5- Vajro P, Ferrante L, Paolella G: Alagille syndrome: an overview. Clin Res Hepatol Gastroenterol. 36(3):275-7, 2012
6- Sheflin-Findling S, Arnon R, Lee S, Chu J, Henderling F, Kerkar N,
Iyer K: Partial internal biliary diversion for Alagille syndrome: case
report and review of the literature. J Pediatr Surg. 47(7):1453-6, 2012
Transcutaneous Electrical Stimulation
Transcutaneous electrical stimulation
(TES) therapy is a noninvasive form of electrical stimulation used in
adults to manage painful musculoskeletal conditions and bladder
incontinence. In general electrical stimulation therapy can be
delivered via the direct route (invasive) with electrodes implantation
in the form of sacral nerve stimulation or the indirect route
(noninvasive) with pad electrode placement on the skin surface over the
affected area and paraspinal region. The battery-operated stimulator
makes home treatment a reality. TES increase defecation frequency and
reduce soiling and abdominal pain in constipation. This has prompted
using TES as therapy for slow transit constipation in children. TES
activates sensory nerve fibers in the spinal nerves in the skin,
sensory and motor nerve in the spinal nerves, sympathetic and
parasympathetic nerves, enteric nerves in the bowel wall or pacemaker
cells in the intestine (cells of Cajal), and intestinal muscle cells.
Improvement of constipation has been found in more than two-thirds of
all patients lasting more than two years in half of them. TES have
reduced the number of surgical procedures (appendicostomy, colectomy,
colostomy) done for intractable slow transit constipation improving
their quality of life.
References:
1- Clarke MC, Chase JW, Gibb S, Hutson JM, Southwell BR: Improvement of
quality of life in children with slow transit constipation after
treatment with transcutaneous electrical stimulation. J Pediatr Surg.
44(6):1268-72, 2009
2- Ismail KA, Chase J, Gibb S, Clarke M, Catto-Smith AG, Robertson VJ,
Hutson JM, Southwell BR: Daily transabdominal electrical stimulation at
home increased defecation in children with slow-transit constipation: a
pilot study. J Pediatr Surg. 44(12):2388-92, 2009
3- Yik YI, Clarke MC, Catto-Smith AG, Robertson VJ, Sutcliffe JR, Chase
JW, Gibb S, Cain TM, Cook DJ, Tudball CF, Hutson JM, Southwell BR:
Slow-transit constipation with concurrent upper gastrointestinal
dysmotility and its response to transcutaneous electrical
stimulation. Pediatr Surg Int. 27(7):705-11, 2011
4- Leong LC, Yik YI, Catto-Smith AG, Robertson VJ, Hutson JM, Southwell
BR: Long-term effects of transabdominal electrical stimulation in
treating children with slow-transit constipation. J Pediatr Surg.
46(12):2309-12, 2011
5- Yik YI, Ismail KA, Hutson JM, Southwell BR: Home transcutaneous
electrical stimulation to treat children with slow-transit
constipation. J Pediatr Surg. 47(6):1285-90, 2012
6-Yik YI, Leong LC, Hutson JM, Southwell BR: The impact of
transcutaneous electrical stimulation therapy on appendicostomy
operation rates for children with chronic constipation-a
single-institution experience. J Pediatr Surg. 47(7):1421-6, 2012
PSU Volume 39 No 05 NOVEMBER 2012
Slow Transit Constipation
Chronic idiopathic constipation is a
distressing problem in children associated with poor appetite,
recurrent abdominal pain and irritability. Two types of constipation
have been described: slow transit constipation (STC) associated with
slow nuclear transit study of the colon, and functional fecal retention
(FFR) associated with normal transit but a delay of anorectal release
in the presence of ganglion cells. STC children characteristically
demonstrate delayed passage of the first meconium stool beyond 24
hrs of age, symptoms of severe constipation within a year, or
treatment-resistant encopresis at 2-3 years, irregular bowel motion,
colicky abdominal pain, frequent uncontrollable soiling and softer
stools as compared with FFR children who pass hard, infrequent stools.
Transverse colon elongation is more common in SCT, whereas sigmoid
colon elongation is not more common in FFR.The correlation between STC
and deficiency of substance P, an excitatory neuropeptide, in the
myenteric axons of the bowel wall implies that this substance is
involved in the cause of the dysmotility. Management is different for
both conditions. In FFR, transit as far as the rectosigmoid is normal,
thus once the rectum is emptied (with enemas or rectal
disimpaction) and behavioral modification is instituted the
condition improves. FFR will not improve with colonic resections. With
STC long-term management is warranted These children do not benefit
from high fiber diet, but can be managed with laxatives and/or enemas.
Those with STC that does not improve will need appendicostomy,
colostomy or colonic resection. Quality of life is significant lower in
children with STC as compared with normal children with both physical
and psychosocial functioning score reduced.
References:
1- Wheatley JM, Hutson JM, Chow CW, Oliver M, Hurley MR: Slow-transit
constipation in childhood. J Pediatr Surg. 34(5):829-32, 1999
2- Marshall J, Hutson JM, Anticich N, Stanton MP: Antegrade continence
enemas in the treatment of slow-transit constipation. J Pediatr
Surg. 36(8):1227-30, 2001
3- Shin YM, Southwell BR, Stanton MP, Hutson JM: Signs and symptoms of
slow-transit constipation versus functional retention. J Pediatr Surg.
37(12):1762-5, 2002
4- Cook BJ, Lim E, Cook D, Hughes J, Chow CW, Stanton MP, Bidarkar SS,
Southwell BR, Hutson JM: Radionuclear transit to assess sites of delay
in large bowel transit in children with chronic idiopathic
constipation. J Pediatr Surg. 40(3):478-83, 2005
5- King SK, Sutcliffe JR, Southwell BR, Chait PG, Hutson JM: The
antegrade continence enema successfully treats idiopathic slow-transit
constipation. J Pediatr Surg. 40(12):1935-40, 2005
6- Yik YI, Cook DJ, Veysey DM, Tudball CF, Cain TM, Southwell BR,
Hutson JM: How common is colonic elongation in children with
slow-transit constipation or anorectal retention? J Pediatr Surg.
47(7):1414-20, 2012
Intramuscular Hemangioma
The most common type of deep
soft-tissue hemangioma occurs within the intramuscular compartment.
Most intramuscular hemangiomas (IH) arise within skeletal muscle and
occurs within the lower extremity during the first three decades of
life. There is a slight female predominance. They are benign lesions
histologically classified into capillary, cavernous or mixed types. The
most common symptom is pain in the affected muscle region associated
with a mass, absent cutaneous changes and neurologic symptoms due to
nerve impingement. The most common involved muscle with IH is the
quadriceps femoris muscle. Though US and CT-Scans are routinely
performed, MRI remains as the imaging of preference for diagnosis
(high-signal intensity on T2-weighted images) and extent of local
involvement. In MRI the cavernous type is predominantly lobulated and
the capillary serpiginous with the mixed type being a combination of
both. Angiography can help plan surgical approach for larger lesions
and even use preoperative embolization to reduce blood loss.
Intramuscular cavernous hemangiomas do not undergo spontaneous
regression and may be locally destructive because pressure is exerted
on neighboring structures. Management of IH consists of medical
and surgical modality. A trial of propranolol should be tried in small
children. Surgery is indicated if there is accelerated growth,
intractable pain, functional impairment, local skin necrosis, cosmetic
deformity or suspicion of malignancy. To reduce recurrences, surgery
consists of wide excision whenever possible avoiding permanent
deformity or functional impairment.
References:
1- Saad DF, Shehata BM, Patrick E, Gow KW: Intramuscular hemangioma of
the abdominal wall. J Pediatr Surg. 41(3):601-2, 2006
2-Bella GP, Manivel JC, Thompson RC Jr, Clohisy DR, Cheng EY:
Intramuscular hemangioma: recurrence risk related to surgical margins.
Clin Orthop Relat Res. 459:186-91, 2007
3- Wu JL, Wu CC, Wang SJ, Chen YJ, Huang GS, Wu SS: Imaging strategies
in intramuscular haemangiomas: an analysis of 20 cases. Int Orthop.
31(4):569-75, 2007
4- Melman L, Johnson FE: Intramuscular cavernous hemangioma. Am J Surg. 195(6):816-7, 2008
5- Hashimoto H, Oshika Y, Obara K, Takeshima S, Sato K, Tanaka Y:
Intercostal venous hemangioma presenting as a chest wall tumor. Gen
Thorac Cardiovasc Surg. 57(4):228-30, 2009
6- Ranero-Juarez AG, Rosales-Galindo VM, Lean-Takahashi AM,
Arenas-Guzmn R, Garcia C: Intramuscular hemangiomas of the
extremities: report of six cases. Int J Dermatol. 48(8):875-8, 2009
Branchiogenic Carcinoma
Branchiogenic carcinoma refers to a
squamous cell carcinoma that arises from second branchial cleft
cysts. To be labeled as branchiogenic carcinoma strict diagnostic
criteria must be met: 1- the location of the tumor in the anatomic
region of the branchial cleft cyst or sinus, 2- the histologic
appearance of the tumor is consistent with tissue present in a
branchial vestige, 3- there is no other evidence of another primary
malignancy after exhaustive work-up (including nasopharyngoscopy,
laryngoscopy, bronchoscopy and esophagoscopy), and 4- the histologic
identification of transition from the normal squamous epithelium of the
cyst to carcinoma. The tumor may be papillary within the cyst and will
always be accompanied by a significant lymphoid component. Most cases
of neck cyst harboring squamous cell carcinoma are metastatic disease
from a primary in the head and neck region, and not primary
branchiogenic carcinoma. Management of branchiogenic carcinoma includes
complete surgical removal with modified radical neck dissection.
Postoperative irradiation to the ipsilateral neck is recommended.
Chemotherapy is utilized when invasion to surrounding tissue is present.
References:
1- Girgivan MR, Rechdouni AK, Zeger GD: Squamous Cell Carcinoma Arising
in a Second Branchial Cleft Cyst. Amer J Clin Oncol 27(1): 96-100, 2004
2- Lin YC, Fang SY, Huang RH: Branchiogenic squamous cell carcinoma: a
case report. Oral & Maxillofascial Surg. 33: 209-212, 2004
3- Jereczek-Fossa BA, Casadio C, Jassem J, et al: Branchiogenic
carcinoma - conceptual or true clinico-pathological entity? Cancer
Treatment Reviews 31: 106-114, 2005
4- Saito T, Sato, Usui H, et al: A Case of Squamous Cell Carcinoma
Arising from Branchial Cleft Cyst. Oral Science International 5(2):
135-140, 2008
5- Roche JP, Younes MN, Funkhouser WK, et al: Branchiogenic carcinoma
of a first branchial cleft syct. Otolaryng Head & Neck Surg. 143:
167-168, 2010
5- Banikas V, Kyrgidis A, Koloutsos G, et al: Branchial Cyst Carcinoma
Revisited: Stem Cells, Dormancy and Malignant Transformation. J of
Craniofacial Surg. 22(3): 918-921, 2011
PSU Volume 39 NO 06 DECEMBER 2012
Ovarian Epithelial Tumors
Ovarian epithelial tumors are the
second most common ovarian neoplasm in children after germ cell tumors.
They arise from the epithelial surface of the ovary representing
between 15-20% of all ovarian neoplasms in adolescent females. The
histologic subtype of epithelial ovarian tumors in children includes
serous and mucinous tumors. Adenocarcinoma of the ovary in children is
an extremely rare entity. Epithelial tumors are further
characterized as benign, malignant or of low malignant potential
(borderline tumors). Mean age at diagnosis is 13 years. Most children
present with history of abdominal pain or an asymptomatic pelvic mass.
The most common type of epithelial neoplasm is the benign serous
cystadenoma. Borderline epithelial ovarian tumors are defined as
epithelial neoplasms of varying level of nuclear atypia, that lacks
stromal invasion of the ovary. They are more common in the pediatric
age. Preoperative work-up includes imaging (Ultrasound, CT and MRI) and
tumor markers (AFP, HCG, LDH, CA-125). Epithelial ovarian tumors
frequently cause elevation of the serum level of the CA-125 tumor
antigen. During laparotomy strict staging criteria must be met such as
collection of ascites/washing, peritoneal surface examination, palpable
lymph nodes removal, omentectomy if involved, suspicious contralateral
ovarian biopsy and complete resection of the involved ovary intact with
sparing of the fallopian tube if not involved (ovarian cystectomy vs.
salpingooophorectomy). Fertility preserving surgery is recommended to
preserve ovarian function. There is no benefit in removing clinically
uninvolved tissue like uterus or contralateral ovary. With mucinous
epithelial tumors the appendix should be removed to avoid synchronous
lesions.
References:
1- Morowitz M, Huff D, von Allmen D: Epithelial ovarian tumors in
children: a retrospective analysis. J Pediatr Surg. 38(3):331-5, 2003
2- Young JL Jr, Cheng Wu X, Roffers SD, Howe HL, Correa C, Weinstein R:
Ovarian cancer in children and young adults in the United States,
1992-1997. Cancer. 2003 May 15;97(10 Suppl):2694-700, 2003
3- Borgfeldt C, et al: Fertility-sparing surgery and outcome in fertile women with ovarian borderline
tumors and epithelial invasive ovarian cancer. Eur J Obstet Gynecol Reprod Biol. 134(1):110-4, 2007
4- Stankovic Z, Djuricic S, Djukic M, Jovanovic D, Vasiljevic M:
Epithelial ovarian tumors and CA125 in premenarchal girls. Eur J
Gynaecol Oncol. 2006;27(6):597-9, 2006
5- Aggarwal A, Lucco KL, Lacy J, Kives S, Gerstle JT, Allen L: Ovarian
epithelial tumors of low malignant potential: a case series of 5
adolescent patients. J Pediatr Surg. ;44(10):2023-7, 2009
6- Song T, Choi CH, Lee YY, Kim TJ, Lee JW, Bae DS, Kim BG: Pediatric
borderline ovarian tumors: a retrospective analysis. J Pediatr Surg.
45(10):1955-60, 2010
7- Grapsa D, Kairi-Vassilatou E, Kleanthis C, Dastamani C, Fillipidou
A, Kondi-Pafiti A: Epithelial ovarian tumors in adolescents: a
retrospective pathologic study and a critical review of the
literature. J Pediatr Adolesc Gynecol. 24(6):386-8, 2011
Ovarian Lymphoma
Primary ovarian non-Hodgkin's
lymphomas are unusual and accounts for 0.5% of all non-Hodgkin's
lymphomas and 1.5% of all ovarian malignancies. The diffuse, large
B-cell lymphoma is the most common type of primary ovarian
non-Hodgkin's lymphoma followed by non-endemic Burkitt's lymphoma. It
has been suggested the tumor originates from lymphocytes in the
ovaries, surrounding blood vessels at the hilum and related to the
corpus luteum. Most patient with ovarian lymphoma present with symptoms
of abdominal pain, pelvic mass, ascites and elevation of serum CA-125
antigen. This lymphoma has the fastest doubling time of any tumor and
it can be as low as 24 hrs. Presence of positive staining for
leukocyte common antigen (LCA) in the histological specimen
distinguishes malignant lymphoma from nonlymphoid neoplasm. The MRI
findings include solid mass with low signal intensity on T1 and mildly
high signal intensity in T2. CT Scan is utilized for staging the
disease in the chest, abdomen and pelvis. Bone marrow biopsy is also
mandatory for staging. B-cell lymphoma is positive for CD20 and
BCL-6. Children with localized disease to one ovary are best
managed with unilateral surgical resection (salpingo-oophorectomy)
followed by systemic chemotherapy. Protocol of chemotherapy used in
diffuse large B-cell histology is the standard CHOP regimen. Metastasis
to the ovaries from another primary site lymphoma should also be
considered and they can be solid, cystic and bilateral. Primary ovarian
lymphoma has a good prognosis.
References:
1- Ambulkar I, Nair R: Primary ovarian lymphoma: report of cases
and review of literature. Leuk Lymphoma. 44(5):825-7, 2003
2- Crawshaw J, Sohaib SA, Wotherspoon A, Shepherd JH: Primary
non-Hodgkin's lymphoma of the ovaries: imaging findings. Br J Radiol.
80(956):e155-8, 2007
3- Vang R, Medeiros LJ, Warnke RA, Higgins JP, Deavers MT: Ovarian
non-Hodgkin's lymphoma: a clinicopathologic study of eight primary
cases. Mod Pathol. 14(11):1093-9, 2001
4- Elharroudi T, Ismaili N, Errihani H, Jalil A: Primary lymphoma of the ovary. J Cancer Res Ther. 4(4):195-6, 2008
5- Arnogiannaki N, Grigoriadis C, Zygouris D, Androutsopoulos G,
Derdelis G, Terzakis E: Primary ovarian non-Hodgkin's lymphoma. Eur J
Gynaecol Oncol. 32(4):441-2, 2011
6- Chakrabarti B, Bhaduri B, Barik S, Gupta D, Chakravorty S: Primary
ovarian lymphoma in a child. J Indian Med Assoc. 109(9):679-80,
2011
Diverticulitis in Children
The most common diverticulum in
children causing surgical problems is the Meckel's diverticulum. In
very rare occasion the pediatric patient can develop diverticular
disease of the colon similar to that occurring in the adult. Such
diverticular disease can lead to colonic diverticulitis. Genetic
disorders in pediatric patients can lead to weakening of the colonic
wall and subsequent development of diverticular disease. Diseases with
an early predilection for diverticulitis include cystic fibrosis,
Ehlers-Danlos syndrome, Marfan syndrome and William-Beuren syndrome.
Some of these syndromes have genetic mutations that alter the collagen
or elastin levels within tissue creating diverticula in early age.
Children with the above syndromes and symptoms of constipation or
relapsing or chronic abdominal pain should undergo colonoscopy or
barium study to identify such diverticular disease. Stool softener and
high-fiber diet can help in the prevention of early complicated
diverticular disease.
References:
1- Benya EC, Nussbaum-Blask AR, Selby DM: Colonic diverticulitis
causing partial bowel obstruction in a child with cystic fibrosis.
Pediatr Radiol. 27(12):918-9, 1997
2- Shin JH, Son BH, Kim H: Clinically distinguishing between appendicitis and right-sided colonic
diverticulitis at initial presentation. Yonsei Med J. 30;48(3):511-6, 2007
3- Bogue CO, Mann EH: Imaging findings in right-sided diverticulitis in a child. Pediatr Radiol. 38(10):1125-7, 2008
4- Garcia MA, Kling KM, Newbury RO, Huang JS: Complicated diverticular
disease in a child with williams syndrome. J Pediatr Gastroenterol
Nutr. 48(2):233-6, 2009
5- Santin BJ, Prasad V, Caniano DA: Colonic diverticulitis in
adolescents: an index case and associated syndromes. Pediatr Surg Int.
25(10):901-5, 2009
6-Ignacio RC Jr, Klapheke WP, Stephen T, Bond S: Diverticulitis in a
child with Williams syndrome: a case report and review of the
literature. J Pediatr Surg. 47(9):e33-5, 2012