PEDIATRIC SURGERY UPDATE ©
VOLUME 27, 2006
PSU Volume 27 No 01 JULY 2006
Utricle Cysts
Incomplete regression of the müllerian duct system in the male
prostatic urethra produces a cystic structure of variable size that will
persist in the midline between the bladder and the rectum known as
utricle cyst. Possibility of persistence of müllerian remnants cyst
is increased in children born with penoscrotal or perineal hypospadia,
undescended testis and unilateral renal agenesis. The presence of an utricle
cyst can cause lower urinary tract irritative or obstructive symptoms,
epididimitis, hematuria, urinary incontinence, oligospermia, constipation,
pyuria or an abdominal mass. Rectal exam can palpate the cystic mass. The
diagnosis is established with cystourethroscopy or voiding cystourethrogram.
Most utricle cysts can be managed medically. The few that enlarge and produce
significant symptoms should be surgically resected. The surgical approach
to the posterior prostatic area of a child urethra can be very difficult
due to the proximity of these lesions to the ejaculatory ducts, pelvic
nerves, rectum, vas deferens and ureters. Transurethral resection of the
cyst roof does not produce good long term results. The suprapubic transvesical
approach has been most widely use in cysts that are large enough to occupy
part of the abdominal cavity. The posterior sagittal approach with lateral
mobilization of the rectum is suited for medium size (grade III) utricle
cysts resection. There is a small incidence of malignancy in prostatic
utricle cysts. Recently laparoscopic resection was reported.
References:
1- Schuhrke TD, Kaplan GW: Prostatic utricle cysts (müllerian
duct cysts). J Urol. 119(6):765-7, 1978
2- Siegel JF, Brock WA, Pena A: Transrectal posterior
sagittal approach to prostatic utricle (müllerian duct cyst). J Urol.
153(3 Pt 1):785-7, 1995
3- Krstic ZD, Smoljanic Z, Micovic Z, Vukadinovic V,
Sretenovic A, Varinac D: Surgical treatment of the Müllerian duct
remnants. J Pediatr Surg. 36(6):870-6, 2001
4- Coppens L, Bonnet P, Andrianne R, de Leval J:
Adult müllerian duct or utricle cyst: clinical significance and therapeutic
management of 65 cases. J Urol. 167(4):1740-4, 2002
5- Willetts IE, Roberts JP, MacKinnon AE: Laparoscopic
excision of a prostatic utricle in a child. Pediatr Surg Int. 19(7):557-8,
2003
Spitz Nevus
The spitz nevus is a skin lesion described during the past century.
The spitz nevus is a benign skin tumor with a symmetric dome-shaped appearance
ranging in color from light tan to dark brown, sometimes black. The most
usual location of a spitz nevus in a child is the head and neck region.
Spitz nevus are usually confused with hemangiomas early in the life of
the child. Spitz nevi do not blanch on pressure, grow rapidly and most
have a size of less than one centimeter in diameter. In some circumstances,
unequivocal distinction between Spitz nevus and melanoma is practically
impossible. Congenital Spitz nevi are true congenital lesions, with histopathologic
features of both acquired Spitz nevus and superficial congenital melanocytic
nevus. Diagnosis is established by biopsy. When the diagnosis is made at
age greater than 10 years, the diameter of the lesion is greater than 10
mm, presence of ulceration, involvement of the subcutaneous fat, and mitotic
activity of at least 6/mm2 carries a higher likelihood of malignant potential
warranting aggressive surgical therapy. Due to the difficulty in diagnosis
and the metastatic potential in certain spitzoid nevi it is recommended
that these lesions be managed by excisional biopsy with a 1- to 2-mm margin
of normal-appearing skin and long-term follow-up. Local recurrence has
been seen in a few cases. Halo degeneration of spitz nevus represents lymphocytic
infiltrate which permeated the full thickness of the nevus.
References:
1- Spatz A, Calonje E, Handfield-Jones S, Barnhill RL:
Spitz tumors in children: a grading system for risk stratification. Arch
Dermatol. 135(3):282-5, 1999
2- Harris MN, Hurwitz RM, Buckel LJ, Gray HR: Congenital
spitz nevus. Dermatol Surg. 26(10):931-5, 2000
3- Clarke B, Essa A, Chetty R: Plexiform spitz nevus.
Int J Surg Pathol. 10(1):69-73, 2002
4- Gurbuz Y, Apaydin R, Muezzinoglu B, Buyukbabani N:
A current dilemma in histopathology: atypical spitz tumor or Spitzoid melanoma?
Pediatr Dermatol. 19(2):99-102, 2002
5- Gelbard SN, Tripp JM, Marghoob AA, Kopf AW, Koenig
KL, Kim JY, Bart RS: Management of Spitz nevi: a survey of dermatologists
in the United States. J Am Acad Dermatol. 47(2):224-30, 2002
6- Dahlstrom JE, Scolyer RA, Thompson JF, Jain S: Spitz
naevus: diagnostic problems and their management implications. Pathology.
36(5):452-7, 2004
Hyperhidrosis
Primary idiopathic hyperhydrosis is the occurrence of perspiration in
excess of that required for body cooling. Secondary hyperhydrosis can be
the result of conditions such as hyperthyroidism, severe obesity, pheochromocytoma
or anxiety. Primary hyperhydrosis can involve the hands, axillae, or trunk.
Sweat is secreted by eccrine glands innervated by sympathetic cholinergic
fibers. Eccrine glands consist of tubules which extend deeply in the dermis
becoming coiled and surrounded by myoepithelial cells. The majority of
children seek help due to palmar hyperhidrosis. The condition can be socially
debilitating causing psychological and emotional upset. Conservative management
consists of antiperspirants, iontophoresis and systemic medication (anticholinergics).
If the condition does not improve with conservative measures, surgical
sympathectomy is indicated. The aim of surgery is to disrupt the sympathetic
supply to palms by destroying relevant ganglions in the upper thoracic
sympathetic chain (T2 and T3). The sympathectomy can be done supraclavicular,
limited posterior thoracotomy, transaxillary thoracotomy or using thoracoscopy.
Results are excellent. The most serious and uncommon complication is Horner's
syndrome.
References:
1- Hehir DJ, Brady MP: Long-term results of limited thoracic
sympathectomy for palmar hyperhidrosis. J Pediatr Surg. 28(7):909-11, 1993
2- Mares AJ, Steiner Z, Cohen Z, Finaly R, Freud E, Mordehai
J: Transaxillary upper thoracic sympathectomy for primary palmar hyperhidrosis
in children and adolescents. J Pediatr Surg. 29(3):382-6, 1994
3- Kao MC, Lee WY, Yip KM, Hsiao YY, Lee YS, Tsai JC:
Palmar hyperhidrosis in children: treatment with video endoscopic laser
sympathectomy. J Pediatr Surg. 29(3):387-91, 1994
4- Cohen Z, Shinar D, Levi I, Mares AJ: Thoracoscopic
upper thoracic sympathectomy for primary palmar hyperhidrosis in children
and adolescents. J Pediatr Surg. 30(3):471-3, 1995
5- Imhof M, Zacherl J, Plas EG, Herbst F, Jakesz R, Fugger
R: Long-term results of 45 thoracoscopic sympathicotomies for primary
hyperhidrosis in children. J Pediatr Surg. 34(12):1839-42, 1999
PSU Volume 27 No 02 AUGUST 2006
Cautery
The development of the cautery in the first part of the past century
made the most significant contribution to surgical patient care. The cautery
was developed by Bovie and Cushing to heat tissue and control bleeding
during operative procedures. The cautery uses electricity to generate heat
and as such follows the path of least resistance always seeking to return
to an electron reservoir like the ground. The most common current use by
electrosurgery generators (cautery) in the operating room is alternating
current (AC). Two most common types of cautery use in the operating room
are the monopolar and bipolar. Monopolar cautery concentrates greater current,
creates more tissue heat, hence produces greater coagulation and charring
than bipolar cautery. Electrosurgical generators can produce current in
three different modes: cut, fulguration and dessication. With minimal settings
in the generator the cut mode can produce a clean tissue cut similarly
to the scalpel with the advantage of minimal surrounding hemostasis. Tissue
fulguration is obtained with the coagulation mode in the generator producing
a greater area of charring than cut. Using either the cut or coagulation
mode in the generator you can produce tissue desiccation. The real issue
of patient burning safety was ameliorated with the use of patient return
electrodes employing a contact quality monitoring split pad system whereby
an interrogation current constantly monitors the quality of the contact
between patient and return electrode.
References:
1- Jackson R: Basic principles of electrosurgery: a review.
Can J Surg. 13(4):354-61, 1970
2- Nduka CC, Super PA, Monson JR, Darzi AW: Cause and
prevention of electrosurgical injuries in laparoscopy. J Am Coll Surg.
179(2):161-70, 1994
3- Ulmer BC: Electrosurgery Self Study Guide. Valleylab
Institute of Clinical Education. 2003
4- Carroll T, Ladner K, Meyers AD: Alternative surgical
dissection techniques. Otolaryngol Clin North Am. 38(2):397-411, 2005
5- Malis LI: Electrosurgery and bipolar technology. Neurosurgery.
58(1 Suppl):ONS1-12, 2006
Pseudomyxoma Peritonei
Pseudomyxoma peritonei (PMP) is a condition characterized by mucinous
ascites and implants throughout the abdominal peritoneal cavity. Most patients
develop pseudomyxoma peritonei after rupture of a low grade mucinous adenocarcinoma
tumor of the appendix. Other less likely pathogenetic sites include ovary
and pancreas. PMP is a disease of MUC2-expressing goblet cells. Diffuse
peritoneal spread occurs in most patients with pseudomyxoma peritonei while
distant metastasis are infrequent. Management of PMP consist of extensive
cytoreductive surgery and peri-operative intraperitoneal radioisotopes
and/or hyperthermic chemotherapy. Adverse predictors of patient survival
included weight loss, abdominal distention, use of systemic chemotherapy,
diffuse disease, and invasion of other organs. Intraperitoneal chemotherapy
and radioisotopes are effective in prolonging the recurrence time of symptomatic
PMP. Prognostic factors for survival included the completeness of cytoreduction,
the histopathological character of the appendix malignancy, and the extent
of previous surgical interventions. Tumor marker CA19.9 is useful in evaluating
therapy and a prognostic factor for predicting recurrent disease in PMP.
References:
1- Sugarbaker PH: Surgical treatment of peritoneal carcinomatosis:
1988 Du Pont lecture. Can J Surg. 32(3):164-70, 1989
2- Gough DB, Donohue JH, Schutt AJ, Gonchoroff N, Goellner
JR, Wilson TO, Naessens JM, O'Brien PC, van Heerden JA: Pseudomyxoma peritonei.
Long-term patient survival with an aggressive regional approach. Ann Surg.
219(2):112-9, 1994
3-Lee KR, Scully RE: Mucinous tumors of the ovary: a
clinicopathologic study of 196 borderline tumors (of intestinal type) and
carcinomas, including an evaluation of 11 cases with 'pseudomyxoma peritonei'.
Am J Surg Pathol. 24(11):1447-64, 2000
4- Sugarbaker PH: Cytoreductive surgery and peri-operative
intraperitoneal chemotherapy as a curative approach to pseudomyxoma peritonei
syndrome. Eur J Surg Oncol 27(3):239-43, 2001
5- Hadi R, Saunders V, Utkina O, Clingan P, Kam P, Links
M, Morris DL: Review of patients with peritoneal malignancy treated with
peritonectomy and heated intraperitoneal chemotherapy. ANZ J Surg.
76(3):156-61, 2006
Vascular Access Complications
Central venous access in children is a necessity for drawing blood,
administering blood products and chemotherapeutic agents, and providing
parenteral nutrition. Access through the various sites such as the internal
and external jugular veins, subclavian and saphenous vein can be plague
of complications. Immediate complications at the time of the procedure
includes failure to achieve successful access, pleural laceration with
pneumothorax development, laceration of the vein with hemothorax, shock,
and extravascular placement of the catheter leading to infection, airway
compression and pericardial tamponade. Unless you do the procedure fluoroscopically,
it is imperative to obtain a chest film immediately after central venous
access to confirm adequate position of the catheter and check for the abovementioned
complications. Complications associated with long term vascular access
include infection (local or systemic bacteremia), occlusion of the catheter,
and chronic erosion of the catheter through the wall of the vessel with
extravasation. Deep venous thrombosis can occur due to nidus deposition
of fibrin. Catheter breakage with embolization is another complication
of long standing access.
References:
1- Bagwell CE, Salzberg AM, Sonnino RE, Haynes JH: Potentially
lethal complications of central venous catheter placement. J Pediatr Surg.
35(5):709-13, 2000
2- Chiang VW, Baskin MN: Uses and complications of central
venous catheters inserted in a pediatric emergency department. Pediatr
Emerg Care. 16(4):230-2, 2000
3- Liossis G, Bardin C, Papageorgiou A: Comparison of
risks from percutaneous central venous catheters and peripheral lines in
infants of extremely low birth weight: a cohort controlled study of
infants < 1000 g. J Matern Fetal Neonatal Med. 13(3):171-4,
2003
4- Radtke WA: Vascular access and management of its complications.
Pediatr Cardiol. 26(2):140-6, 2005
5- Kapadia S, Parakh R, Grover T, Yadav A: Catheter fracture
and cardiac migration of a totally implantable venous device. Indian J
Cancer. 42(3):155-7, 2005
6- Revel-Vilk S: Central venous line-related thrombosis
in children. Acta Haematol. 115(3-4):201-6, 2006
PSU Volume 27 No 03 SEPTEMBER 2006
Split Cord Malformations
Split cord malformations (SCM) refers to a group of malformations where
the spinal cord is split or clefted over a portion of its length. Most
affected children are females with a mean age of seven years. The malformations
have been recognized into two types. Type I consist of double dural sac
malformations (both spinal cord and dural sac are split) associated with
an extradural bony cartilaginous spur interposed between the two thecal
sacs. Type II are single dural sac malformations with a clefted spinal
cord (both hemicord are contained within a common dural sac). Both types
contain distal aberrant nerve roots that exit from the cord and can tethered
the spinal cord. Tethering the cord can produce neurological deterioration
such as sensorimotor deficit, change in bowel or bladder habit and orthopedic
deformities. Associated enteric malformations (posterior mediastinal foregut
cyst) are frequently found with SCM. The enteric cyst can contain stomach,
small or large bowel and even bronchial tissue. The child born with a SCM
usually has a cutaneous stigmata (focal hypertrichosis) under the malformation.
Evaluation of SCM includes MRI with intravenous contrast and/or CT-Scan
myelography. Since neurological deficit increases with age, prophylactic
repair of the SCM with untethering of the spinal cord is recommended before
neurological signs develop.
References:
1- Meller JL, Loeff DS, Reyes HM: A variant of the split
notochord syndrome. J Pediatr Surg. 24(8):733-5, 1989
2- Ersahin Y, Mutluer S, Kocaman S, Demirtas E: Split
spinal cord malformations in children. J Neurosurg. 88(1):57-65, 1998
3- Jindal A, Mahapatra AK: Split cord malformations--a
clinical study of 48 cases. Indian Pediatr. 37(6):603-7, 2000
4- Schijman E: Split spinal cord malformations: report
of 22 cases and review of the literature. Childs Nerv Syst. 19(2):96-103,
2003
5- Mahapatra AK, Gupta DK: Split cord malformations:
a clinical study of 254 patients and a proposal for a new clinical-imaging
classification. J Neurosurg. 103(6 Suppl):531-6, 2005
6- Schropp C, Sorensen N, Collmann H, Krauss J: Cutaneous
lesions in occult spinal dysraphism--correlation with intraspinal findings.
Childs Nerv Syst. 22(2):125-31, 2006
7- Sinha S, Agarwal D, Mahapatra AK: Split cord malformations:
an experience of 203 cases. Childs Nerv Syst. 22(1):3-7, 2006
Segmental Bowel Dilatation
Dilatation of a segment of bowel in newborns and infants is a rare congenital
malformation associated with partial or complete bowel obstruction. The
dilatation is limited to a segment of the small or large intestine with
a three to fourfold increase size and abrupt transition between normal
and dilated bowel. Histology demonstrates that the neuronal enteric plexus
is normal in the affected segment, proximally and distally. The origin
of the dilatation is unknown. Theory states intrauterine extrinsic compression
of the bowel or a primitive neuromuscular dysfunction can explain the dilatation.
Most cases are found in newborn explore for another surgical malformation.
Other will present a bowel complication such as obstruction, perforation
or bleeding. Bleeding due to ulceration of the dilated segment is found
more commonly in infants. Most frequent associated malformation are omphalocele,
bowel atresias and imperforate anus. Contrast studies of the distal and
proximal bowel will uncover the segmental dilatation. The first diagnostic
possibility to consider is aganglionosis (Hirschsprung's disease) and a
suction rectal biopsy is in order. If the symptomatic child has normal
rectal ganglion cells, the next step in management consists of surgical
exploration with histological confirmation. Most segmental dilatation affects
the ileum. Resection of the affected segment with end to end anastomosis
is curative.
References:
1- Irving IM, Lister J: Segmental dilatation of the ileum.
J Pediatr Surg. 12(1):103-12, 1977
2- Ratcliffe J, Tait J, Lisle D, Leditschke JF, Bell
J: Segmental dilatation of the small bowel: report of three cases and literature
review. Radiology. 171(3):827-30, 1989
3- Ngai RL, Chan AK, Lee JP, Mak CK: Segmental colonic
dilatation in a neonate. J Pediatr Surg. 27(4):506-8, 1992
4- Kuint J, Avigad I, Husar M, Linder N, Reichman B:
Segmental dilatation of the ileum: an uncommon cause of neonatal intestinal
obstruction. J Pediatr Surg. 28(12):1637-9, 1993
5- Porreca A, Capobianco A, Terracciano C, D'Onofrio
V: Segmental dilatation of the ileum presenting with acute intestinal bleeding.
J Pediatr Surg. 37(10):1506-8, 2002
6- Brahim MB, Belghith M, Mekki M et al: Segmental Dilatation
of the Intestine. J Pediatr Surg 41(6): 1130-1133, 2006
Sialoblastoma
Sialoblastoma, originally called embryoma, is the most common type of
congenital epithelial tumor of the salivary gland in children. Is an extremely
rare salivary gland neoplasm encountered after the age of five. Sialoblastoma
is locally aggressive with a high recurrence rate. The parotid gland is
most commonly affected. Diagnosis can be suggested by fine needle aspiration
biopsy. Prenatal diagnosis has been reported. The tumor is characterized
by solid nests of epithelial cells intermingled with proliferating ductal
structures lined by a double layer of cells. Sialoblastomas are mitotically
active primitive cell masses with formative ducts and pseudoductular spaces
without acinar differentiation. MRI can help establish the surrounding
anatomy and relationship of the tumor to other vital structures. Sialoblastomas
should be managed with early conservative surgery provided that free margins
are obtained. The patient's prognosis is likely to be determined by the
tumor grade as well as the stage at presentation and the extent of resection.
Adjuvant chemotherapy or radiotherapy has not been effective in control
of recurrence.
References:
1- Harris MD, McKeever P, Robertson JM: Congenital tumours
of the salivary gland: a case report and review. Histopathology. 17(2):155-7,
1990
2- Hsueh C, Gonzalez-Crussi F: Sialoblastoma: a case
report and review of the literature on congenital epithelial tumors of
salivary gland origin. Pediatr Pathol. 12(2):205-14, 1992
3- Som PM, Brandwein M, Silvers AR, Rothschild MA: Sialoblastoma
(embryoma): MR findings of a rare pediatric salivary gland tumor. AJNR
Am J Neuroradiol. 18(5):847-50, 1997
4- Brandwein M, Al-Naeif NS, Manwani D, Som P, Goldfeder
L, Rothschild M, Granowetter L: Sialoblastoma: clinicopathological/immunohistochemical
study. Am J Surg Pathol. 23(3):342-8, 1999
5- Alvarez-Mendoza A, Calderon-Elvir C, Carrasco-Daza
D: Diagnostic and therapeutic approach to sialoblastoma: report of a case.
J Pediatr Surg. 34(12):1875-7, 1999
6- Garrido A, Humphrey G, Squire RS, Nishikawa H: Sialoblastoma.
Br J Plast Surg. 53(8):697-9, 2000
7- Tatlidede S, Karsidag S, Ugurlu K, Sadikoglu B, Tanik
C, Bas L: Sialoblastoma: A congenital epithelial tumor of the salivary
gland. J Pediatr Surg 41(7): 1322-1325, 2006
PSU Volume 27 No 04 OCTOBER 2006
Acute Scrotum
The term acute scrotum refers to signs and symptoms associated with
local inflammation of the scrotum that appears suddenly and usually is
not associated with trauma. Is a common urological emergency. Such signs
and symptoms include scrotal pain, swelling, redness and heat. The
most common causes of acute scrotum in children constitute testicular torsion,
appendix testis torsion, epididymitis, orchitis and pyocele. Testicular
torsion, a surgical emergency, occurs in 15% of all cases and is the most
important condition to diagnose and manage early in order to avoid testicular
loss, fertility problems and medico-legal issues. Testicular loss commences
past the twelve hours of initiation of symptoms. Beyond twenty-four hours
of symptoms testicular loss in the norm. This is the main reason why in
the absence of ancillary studies surgeons immediately explore the
acute scrotum. The two most commonly used preoperative studies are testicular
scan and color Doppler ultrasound. Testicular scans reliable show whether
the testes have vascular flow or not, but are difficult to be obtained
in the middle of the night. Doppler ultrasounds are operator dependant
and when done by experienced physician can help reduce the number of emergency
operations and hospitalization days. Clinical judgement by the surgeon
is probably the most important factor in assessing testicular salvage.
In the face of doubt the next step in management is immediate surgical
scrotal exploration.
References:
1- Dunne PJ, O'Loughlin BS: Testicular torsion: time
is the enemy. Aust N Z J Surg. 70(6):441-2, 2000
2- Weber DM, Rosslein R, Fliegel C: Color Doppler sonography
in the diagnosis of acute scrotum in boys. Eur J Pediatr Surg. 10(4):235-41,
2000
3- McAndrew HF, Pemberton R, Kikiros CS, Gollow I: The
incidence and investigation of acute scrotal problems in children. Pediatr
Surg Int. 18(5-6):435-7, 2002
4- Stehr M, Boehm R: Critical validation of colour Doppler
ultrasound in diagnostics of acute scrotum in children. Eur J Pediatr Surg.
13(6):386-92, 2003
5- Ben-Meir D, Deshpande A, Hutson JM: Re-exploration
of the acute scrotum. BJU Int. 97(2):364-6, 2006
6- Terai A, Yoshimura K, Ichioka K, Ueda N, Utsunomiya
N, Kohei N, Arai Y, Watanabe Y: Dynamic contrast-enhanced subtraction magnetic
resonance imaging in diagnostics of testicular torsion. Urology. 67(6):1278-82,
2006
7- Schalamon J, Ainoedhofer H, Schleef J, Singer G, Haxhija
EQ, Hollwarth ME: Management of acute scrotum in children - the impact
of Doppler ultrasound. J Pediatr Surg. 41(8): 1377-1380, 2006
Pinch Off Syndrome
Implantable central venous catheters constitute a necessity for the
management of long term intravenous nutrition and chemotherapy. Implantable
central venous access devices placed via the subclavian vein may become
obstructed by thrombosis, impingement against a vein wall, or compressed
between the clavicle and first rib. Compression of the catheter between
the clavicle and first rib is known as pinch-off syndrome (POS). Beside
obstruction, pinch-off syndrome can cause fragmentation, fracture or rupture
of the catheter causing embolization of the released fragment of tubing.
Mechanical friction against the catheter has been well established as the
mechanism for most fractures. POS is characterized by intermittent catheter
malfunction in conjunction with radiologic evidence of catheter compression.
Warning signs of POS include difficulty withdrawing blood samples and resistance
to infusion of IV fluids. Catheter transection with migration of the catheter
into the heart or pulmonary artery may be accompanied by the sudden onset
of chest pain, palpitations, and arrhythmias. Electron microscopic scanning
tends to prove that the catheter's rupture is caused by a fatigue process.
Treatment of POS is removal of the catheter. If the tip of the catheter
has embolized, it can usually be retrieved percutaneously with a transvenous
snare. POS can be prevented by using the internal jugular vein for access
rather than the subclavian vein.
References:
1- Hinke DH, Zandt-Stastny DA, Goodman LR, Quebbeman
EJ, Krzywda EA, Andris DA: Pinch-off syndrome: a complication of implantable
subclavian venous access devices. Radiology. 177(2):353-6, 1990
2- Nace CS, Ingle RJ: Central venous catheter "pinch-off"
and fracture: a review of two under-recognized complications. Oncol Nurs
Forum. 20(8):1227-36, 1993
3- Andris DA, Krzywda EA, Schulte W, Ausman R, Quebbeman
EJ: Pinch-off syndrome: a rare etiology for central venous catheter occlusion.
JPEN J Parenter Enteral Nutr. 18(6):531-3, 1994
4- Mercuri M, Distefano M, Crovaro M, Giri S, Sportelli
G, Carrara A, Butti A, Marin AW: Central venous catheter disruption and
embolization: percutaneous retrieval. A case report. Eur Rev Med Pharmacol
Sci. 4(5-6):133-8, 2000
5- Fazeny-Dorner B, Wenzel C, Berzlanovich A, Sunder-Plassmann
G, Greinix H, Marosi C, Muhm M: Central venous catheter pinch-off and fracture:
recognition, prevention and management. Bone Marrow Transplant. 31(10):927-30,
2003
6- Mirza B, Vanek VW, Kupensky DT: Pinch-off syndrome:
case report and collective review of the literature. Am Surg. 70(7):635-44,
2004
7- Sarzo G, Finco C, Zustovich F, Parise P, Savastano
S, Degregori S, Vecchiato M, Meri gliano S: Early rupture of subclavian
vein catheter: a case report and literature review. J Vasc Access. 5(1):39-46,
2004
Falciform Ligament Abscess
The falciform ligament is a parasagittal structure which extends from
the umbilicus to the diaphragm containing the ligamentum teres and remnants
of umbilical veins. It has an intrahepatic course between the quadrate
lobe and the left lobe of the liver communicating with the portal triad.
It is very rare to develop a falciform ligament abscess in children. Falciform
ligaments abscess present with fever, leukocytosis, postprandial fullness
and a right upper quadrant mass. Computerized axial tomography (CAT scan)
is the essential tool in arriving at a correct preoperative diagnosis demonstrating
a cystic cylindrical mass in the anterior abdomen that courses the
falciform ligament while helping define the relationship of the vascular
structures at the porta hepatis. Most cases are associated with infection
from the umbilicus, gallbladder or a concomitant infected ventriculo-peritoneal
shunt. Portal pyemia is another complication associated with falciform
ligament abscess. Management consists of intravenous antibiotics and percutaneous
drainage.
References:
1- Lipinski JK, Vega JM, Cywes S, Cremin BJ: Falciform
ligament abscess in the infant. J Pediatr Surg. 20(5):556-8, 1985
2- Laucks SS 2nd, Ballantine TV, Boal DK: Abscess of
the falciform ligament in a child with a ventriculoperitoneal shunt. J
Pediatr Surg. 21(11):979-80, 1986
3- Sones PJ Jr, Thomas BM, Masand PP: Falciform ligament
abscess: appearance on computed tomography and sonography. AJR Am J Roentgenol.
137(1):161-2, 1981
4- Brock JS, Pachter HL, Schreiber J, Hofstetter SR:
Surgical diseases of the falciform ligament. Am J Gastroenterol. 87(6):757-8,
1992
5- Pratap A, Tiwari A, Anchal N, Agrawal CS, Shreshta
P, Shakya VC: Falciform ligament abscess with portal pyemia in a newborn.
J Pediatr Surg. 41(8):1473-5, 2006
Volume 27 No 05 NOVEMBER 2006
Bowel Angiodysplasia
Intestinal angiodysplasia represent a very rare cause of lower gastrointestinal
bleeding in infants and children, more commonly seen in the elderly patient.
Diagnosis is usually delayed. The angiodysplasia is a small, flat
vascular malformation (vascular dysplasia) composed of congeries of dilated
capillaries, arterioles, and postcapillary venules located most commonly
in the left colon, followed in frequency by the distal ileum. The child
presents with recurrent hematochezia (most commonly) and chronic blood
loss. Mean age at the time of diagnosis is two years. Work-up in search
of a diagnosis includes lower and upper endoscopy, Meckel (technetium)
scintigraphic scan, packed red blood cells scan, magnetic resonance angiography,
CT-scan (rapid venous enhancement during intravenous administration of
contrast material), intraoperative enteroscopy and selective mesenteric
arteriogram. Wireless capsule endoscopy is another tool that has been useful
among children to diagnose intraluminal conditions of the small bowel,
but this method lacks tissue sampling and therapeutic capabilities. Another
rare cause of lower gastrointestinal bleeding is intestinal hemangiomatosis.
Precise preoperative diagnosis and location are a priority before embarking
in a negative exploratory laparoscopy or laparotomy. Surgical resection
remains the definitive therapy for angiodysplasias and hemangiomas of the
bowel since embolization and steroid therapy has produced conflicting results.
References:
1- Maeda M, Yamashiro Y: Diagnostic red blood cell scintigraphy
in GI tract bleeding from an intestinal hemangioma. J Pediatr Gastroenterol
Nutr. 5(6):987-9, 1986
2- Bank ER, Hernandez RJ, Byrne WJ: Gastrointestinal
hemangiomatosis in children: demonstration with CT. Radiology. 165(3):657-8,
1987
3- Park DD, Ricketts RR: Infantile gastrointestinal hemangioma
as a cause of chronic anemia. South Med J. 85(2):201-3, 1992
4- de la Torre Mondragon L, Vargas Gomez MA, Mora Tiscarreno
MA, Ramirez Mayans J: Angiodysplasia of the colon in children. J Pediatr
Surg. 30(1):72-5, 1995
5- Aziz A, Kane TD, Meza MP, Vaughan KG, Hackam DJ: An
unusual cause of rectal bleeding and intestinal obstruction in a child
with peripheral vascular malformations. Pediatr Surg Int. 21(6):491-3.
Epub 2005 Apr 2, 2005
6- Kavin H, Berman J, Martin TL, Feldman A, Forsey-Koukol
K: Successful wireless capsule endoscopy for a 2.5-year-old child: obscure
gastrointestinal bleeding from mixed, juvenile, capillary hemangioma-angiomatosis
of the jejunum. Pediatrics. 117(2):539-43, 2006
Pyogenic Hepatic Abscess
Liver abscess in children can be of bacterial (pyogenic), fungal, parasitic
or viral origin. Rare in frequency and lethal in occurrence, they are usually
associated with immune compromised patients such as those harboring malignancy
and chronic granulomatous disease. Pyogenic abscess are by far the most
common in parasitic non-endemic areas. Pyogenic hepatic abscess (PHA) establishes
by several routes namely biliary, venous, arterial, direct spread or trauma.
Some examples are appendicitis, cholecystitis, umbilical vein catheterization,
bacteremia and hepatic trauma. Most PHA in infants and children occurs
due to systemic bacteremia with staphylococcus aureus as primary infecting
agent, while neonatal PHA occurs through the portal vein with gram negative
organisms. Children with PLA shows nonspecific symptoms such as abdominal
pain and tenderness, fever and hepatomegaly. Labs may show leukocytosis,
elevated liver enzymes and erythrocyte sedimentation rate. Abdominal ultrasound
and CT-Scan are the first line imaging in accurate diagnosis. Management
consists of multiple systemic antibiotics with percutaneous drainage of
macroscopic abscess collections. Multiple small collections will not be
amenable top drainage. Resolution of the PHA can be followed with ultrasound.
Prognosis has improved considerably with time.
References:
1- Raval B, Wan R, Carey L: The spectrum of liver disease
on CT. J Can Assoc Radiol. 30(4):211-5, 1979
2- Clark RA, Towbin R: Abscess drainage with CT and ultrasound
guidance. Radiol Clin North Am. 21(3):445-59, 1983
3- Oleszczuk-Raszke K, Cremin BJ, Fisher RM, Moore SW,
Millar AJ: Ultrasonic features of pyogenic and amoebic hepatic abscesses.
Pediatr Radiol. 19(4):230-3, 1989
4- Kays DW: Pediatric liver cysts and abscesses. Semin
Pediatr Surg. 1(2):107-14, 1992
5- Ferreira MA, Pereira FE, Musso C, Dettogni RV: Pyogenic
liver abscess in children: some observations in the Espirito Santo State,
Brazil. Arq Gastroenterol. 34(1):49-54, 1997
6- Rajak CL, Gupta S, Jain S, Chawla Y, Gulati M, Suri
S: Percutaneous treatment of liver abscesses: needle aspiration versus
catheter drainage. AJR Am J Roentgenol. 170(4):1035-9, 1998
7- Brook I: Intra-abdominal, retroperitoneal, and visceral
abscesses in children. Eur J Pediatr Surg. 14(4):265-73, 2004
Gallbladder Hydrops
Acute distension of the gallbladder in the absence of stones, bacteria,
or congenital malformations is known as gallbladder hydrops. This condition
has a vascular origin such as transient arteritis or ischemia. In older
children hydrops will present with fever, nausea, vomiting, right upper
quadrant mass and abdominal tenderness, while neonates and infants might
show only a palpable mass. Gallbladder hydrops in neonates or infants is
caused by systemic sepsis, while Kawasaki disease (mucocutaneous lymph
node syndrome), scarlet fever, leptospirosis and trauma are the most common
cause of hydrops in older children. The diagnosis of hydrops is established
using abdominal ultrasound demonstrating normal biliary ducts and a distended
gallbladder without calculi or congenital malformation. The treatment of
hydrops is conservative. Management consists of systemic antibiotics and
early enteral feeding to stimulate gallbladder function and decompression.
Should pain and distension persists, open or percutaneous cholecystostomy
may be helpful depending on the medical condition of the child.
References:
1- Kumari S, Lee WJ, Baron MG: Hydrops of the gallbladder
in a child: diagnosis by ultrasonography. Pediatrics. 63(2):295-7, 1979
2- Rumley TO, Rodgers BM: Hydrops of the gallbladder
in children. J Pediatr Surg. 18(2):138-40, 1983
3- Suddleson EA, Reid B, Woolley MM, Takahashi M: Hydrops
of the gallbladder associated with Kawasaki syndrome. J Pediatr Surg. 22(10):956-9,
1987
4- Crankson S, Nazer H, Jacobsson B: Acute hydrops of
the gallbladder in childhood. Eur J Pediatr. 151(5):318-20, 1992
5- Herek O, Yildiran N: Gallbladder hydrops caused by
intraluminal clot in hemobilia: an unusual
complication of hepatic trauma in childhood. J Pediatr
Gastroenterol Nutr. 33(1):92-3, 2001
6- Zulian F, Falcini F, Zancan L, Martini G, Secchieri
S, Luzzatto C, Zacchello F: Acute surgical abdomen as presenting manifestation
of Kawasaki disease. J Pediatr. 142(6):731-5, 2003
Volume 27 No 06 DECEMBER 2006
Phlegmasia Cerulea Dolens
Phlegmasia cerulea dolens (PCD) is a severe form of venous thrombosis
presenting as an edematous, painful, purplish-blue lower extremity. PCD
is characterized by tense swelling of the lower extremity with tenderness
of the thigh over the femoral vein, mottling of the limb, and absent
distal pulses. It's more commonly seen in adults than children.
It is extremely important to recognize this venous thrombosis as opposed
to an arterial insufficiency to avoid gangrene and amputation. Malignant
disease is the most common underlying condition found in phlegmasia cerulea
dolens (PCD). Unsuccessfully managed iliofemoral venous thrombosis can
result in pulmonary embolism, phlegmasia cerulea dolens, and post-thrombotic
syndrome. Other predisposing conditions include diabetes, hypercoagulation
and previous DVT. PCD can also be seen after percutaneous insertion
of a vena caval filter. PCD is evaluated using radionuclide or standard
venography. Several treatment methods are used alone or in combination,
namely intravenous heparin, thrombolytic therapy and venous thrombectomy.
Nongangrenous forms of PCD respond well to systemic anticoagulation. Combination
therapy using venous thrombectomy and heparin are indicated for severe
ischemia, early venous gangrene, or failure of PCD to improve after six
to twelve hours of heparin therapy. Phlegmasia cerulea dolens with venous
gangrene is the lethal form of the entity and respond poorly to established
therapy.
References:
1- Weaver FA, Meacham PW, Adkins RB, Dean RH: Phlegmasia
cerulea dolens: therapeutic considerations. South Med J. 81(3):306-12,
1988
2- Hood DB, Weaver FA, Modrall JG, Yellin AE: Advances
in the treatment of phlegmasia cerulea dolens. Am J Surg. 166(2):206-10,
1993
3- Patel KR, Paidas CN: Phlegmasia cerulea dolens: the
role of non-operative therapy. Cardiovasc Surg. 1(5):518-23, 1993
4- Gutt CN, Oniu T, Wolkener F, Mehrabi A, Mistry S,
Buchler MW: Prophylaxis and treatment of deep vein thrombosis in general
surgery. Am J Surg. 189(1):14-22, 2005
5- Mousa A, Henderson P, Dayal R, Bernheim J, Kent KC,
Faries PL: Endoluminal recanalization in a patient with phlegmasia cerulea
dolens using a multimodality approach. Vascular. 13(5):313-7, 2005
Diaphragmatic Tumors
Tumors arising from the muscle or elements of the diaphragm are very
rare in occurrence. The small published series has shown that the incidence
is similar between boys and girls along with left or right involvement.
Most primary tumors arising from the diaphragm are malignant, with rhabdomyosarcoma
the most commonly encountered followed by sarcomas, yolk sac tumors and
extraosseous Ewing sarcoma. Lymphangiomas and hemangiomas are the most
common benign tumors found in the diaphragm. The clinical presentation
in children varies, with predominantly chest symptoms (chest pain, shortness
of breath, cough, chest asymmetry or hemothorax). Identifying the site
of origin of the tumor to the diaphragm is difficult even after using CT,
MRI and ultrasound. Exploratory laparotomy with biopsy is the best tool
to assign location to the tumor. Management of primary diaphragmatic tumors
encompasses wide local resection with reconstruction, chemotherapy and
radiotherapy. To obtain cure, a tumor free resection margin must be obtain
initially or after chemotherapy shrinkage of the tumor. Reconstruction
of the diaphragm at the time of resection can be accomplished with a muscle
flap or prosthetic graft (PTFE or Goretex).
References:
1- Raney RB, Anderson JR, Andrassy RJ, Crist WM, Donaldson
SS, Maurer HM; Intergroup Rhabdomyosarcoma Study Group: Soft-tissue sarcomas
of the diaphragm: a report from the Intergroup Rhabdomyosarcoma Study Group
from 1972 to 1997. J Pediatr Hematol Oncol. 22(6):510-4, 2000
2- Hagr A, Laberge JM, Nguyen LT, Emil S, Bernard C,
Patenaude Y: Laparoscopic excision of subdiaphragmatic epidermoid cyst:
a case report. J Pediatr Surg. Jun;36(6):E8, 2001
3- Akinci D, Akhan O, Ozmen M, Ozkan OS, Karcaaltincaba
M: Diaphragmatic mesothelial cysts in children: radiologic findings and
percutaneous ethanol sclerotherapy. AJR Am J Roentgenol. 185(4):873-7,
2005
4- Belaabidia B, Sellami S, Benelkhayat R, Elattar H,
Elidrissi Dafali A: Leiomyosarcoma of the diaphragm: a case report and
review of the literature. Cancer Radiother. 10(3):137-41, 2006
5- Cheon JS, You YK, Kim JG, Lee DH, Park K, Ahn CJ:
Diaphragmatic lipoma in a 4-year-old girl: a case report. J Pediatr Surg.
41(1):e37-9, 2006
6- Cada M, Gerstle JT, Traubici J, Ngan BY, Capra ML:
Approach to diagnosis and treatment of pediatric primary tumors of the
diaphragm. J Pediatr Surg 41(10): 1722-1726, 2006
Breast Cysts
Breast cysts are very common in the adolescent female, while not
so common in males. Rapid cyst growth causes pain and a palpable mass in
the breast that brings the child to seek medical help. Other times breast
cysts get infested and are managed as a breast abscess with antibiotics
and drainage. In males solitary, large male breast cysts are extremely
rare. They occur mainly in children up to the age of seven years and should
be removed under surgery. Whenever a breast mass develops the next step
in diagnosis is an ultrasound-mammography studies. This will corroborate
the cystic or solid nature of the cyst along with its size. Radiation exposure
in children using mammography is not necessary or warrant. Management of
breast cysts in females consist of observation or aspiration of the cyst
with cytological exam. The overwhelming majority of breast cyst in children
are benign and will go spontaneously with time. Follow-up breast ultrasound
of the child for persistent of the mass or bloodstained aspirate should
be done.
References:
1- Jones BM, Bradbeer JW: The presentation and progress
of macroscopic breast cysts. Br J Surg. 67(9):669-71, 1980
2- Hamed H, Coady A, Chaudary MA, Fentiman IS: Follow-up
of patients with aspirated breast cysts is necessary. Arch Surg. 124(2):253-5,
1989
3- Devitt JE, To T, Miller AB: Risk of breast cancer
in women with breast cysts. CMAJ. 147(1):45-9, 1992
4- Hindle WH, Arias RD, Florentine B, Whang J: Lack of
utility in clinical practice of cytologic examination of nonbloody cyst
fluid from palpable breast cysts. Am J Obstet Gynecol. 182(6):1300-5, 2000
5- Bock K, Duda VF, Hadji P, Ramaswamy A, Schulz-Wendtland
R, Klose KJ, Wagner U: Pathologic breast conditions in childhood and adolescence:
evaluation by sonographic diagnosis. J Ultrasound Med. 24(10):1347-54,
2005
6- Vargas HI, Vargas MP, Eldrageely K, Gonzalez KD, Burla
ML, Venegas R, Khalkhali I: Outcomes of surgical and sonographic assessment
of breast masses in women younger than 30. Am Surg. 71(9):716-9, 2005