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.

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.

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


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.

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


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.

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.

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.

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.

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.

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, 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.

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.

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.

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.

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.

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.

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.

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.

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).

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.

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

Journal Club