VOLUME 44, 2015

PSU Volume 44 No 01 JANUARY 2015

Testicular Sex Cord-Stromal Tumors

Testicular sex cord-stromal tumors (TSCS) are very rare in children accounting for near 6-8% of all testicular neoplasms in the pediatric age. TSCS main histological types include Leydig cell tumors, Sertoli cell tumor, juvenile granulosa cell tumor and undifferentiated cell tumors. Clinically they present as a painless testicular mass with hormonal manifestation causing isosexual pseudoprecocity or estrogenic manifestation occurring in up to 20% of all cases. TSCT does not have an aggressive behavior being low-grade tumors. Malignancy is defined when the tumor has lymphatic or vascular invasion, high mitotic index and tumor necrosis. Age adjusted AFP should be negative in TSCS, otherwise it's a germ cell tumor. Leydig cell tumors represent the most common histologic type. They produce testosterone, hence precocious puberty with elevated 17-ketosteroid levels occurs. When diagnosed preop, testis-sparing enucleation may be considered because these tumors tend to follow a benign course. Sertoli cell tumors are diagnosed before one year of life, hormonal silent, occasionally producing gynecomastia due to estrogen secretion. The clinical course is usually benign in children less than five and they can also be managed with testis-sparing surgery when diagnosed appropriately. Older children should have a metastatic workup. Metastatic disease requires aggressive surgical and adjuvant therapy. Children with large cell calcifying Sertoli cell tumors are at risk for endocrine syndromes. Granulosa cell tumors occur in neonates and can be associated with ambiguous genitalia. They should be suspected in neonates with scrotal swelling, normal age-adjusted AFP level, positive inhibin and a complex cystic multiseptated hypoechoic mass in the ultrasound of the testis. They can be managed with testis-sparing surgery. Undifferentiated stromal tumors harbor malignant potential and prepubertal and postpubertal males with these tumors should undergo a metastatic evaluation.

1- Acar C, Gurocak S, Sozen S: Current treatment of testicular sex cord-stromal tumors: critical review. Urology. 73(6):1165-71, 2009
2- Schwentner C, Oswald J, Rogatsch H, Mikuz G, Bartsch G, Radmayr C: Stromal testis tumors in infants. a report of two cases. Urology. 62(6):1121, 2003
3- Schultz KA, Schneider DT, Pashankar F, Ross J, Frazier L: Management of ovarian and testicular sex cord-stromal tumors in children and adolescents. J Pediatr Hematol Oncol. 34 Suppl 2:S55-63, 2012
4- Featherstone JM, Fernando HS, Theaker JM, Simmonds PD, Hayes MC, Mead GM: Sex cord stromal testicular tumors: a clinical series--uniformly stage I disease. J Urol. 181(5):2090-6, 2009
5- Goswitz JJ, Pettinato G, Manivel JC: Testicular sex cord-stromal tumors in children: clinicopathologic study of sixteen children with review of the literature. Pediatr Pathol Lab Med. 16(3):451-70, 1996
6- Cecchetto G(1), Alaggio R, Bisogno G, Virgone C, Dall'Igna P, Terenziani M, Boldrini R, D'Onofrio V, Ferrari A, Bernini G: Sex cord-stromal tumors of the testis in children. A clinicopathologic report from the Italian TREP project. J Pediatr Surg. 45(9):1868-73, 2010

Covered Cloacal Exstrophy

Covered cloacal exstrophy (CCE) is a very rare frequently misdiagnosed malformation found within the spectrum of cloacal exstrophy requiring a high index of suspicion for diagnosis. Low implantation of the umbilical cord in association with separated pubic bones and an anorectal malformation (imperforate anus) are the most common sign associated with a covered cloacal exstrophy. Besides the anorectal malformation these patients also have an absent bladder neck and short colon. Most children with CCE are females with a single clitoris. Inspection of the perineum can discover a single large orifice or four perineal orifices very close to each other. Separation of the pubic bone can be seen at the physical exam seen as two mild prominences away from the midline in the pubic area or in simple films. There is a fibrous band between the separate pubic bones. The presence of a large perineal orifice through which there is constant dribbling of urine is another important sign to establish the diagnosis. There is no abdominal wall defect present. The absence of bladder neck associated with a very small bladder will require urinary reconstruction with bladder augmentation and Mitrofanoff to keep dry urine. Those cases with short colon unable to form solid stools will need a permanent stoma reconstruction. If there is evidence of well-formed solid stools, the child can undergo a pull through procedure. During initial stoma creation no piece of colon should be left attached to the urinary tract to take advantage of its water absorptive capacity and avoid urine absorption and associated hyperchloremic acidosis. Reconstruction of the GI tract takes precedence over the urinary reconstruction. Indication for pull-through depends on successful bowel management through the stoma, which depends on the ability to form solid stools. To maximize this potential it is crucial to use all available hindgut for the initial colostomy and avoid use of colon for urologic or genital reconstruction.     

1- *Bischoff A, Levitt MA, Breech L, Pena: Covered cloacal exstrophy--a poorly recognized condition: hints for a correct diagnosis. J Pediatr Surg. 48(12):2389-92, 2013
2- Weiss RE, Garden RJ, Cohen EL, et al. Covered exstrophy with sequestered colonic remnant. J Urol 150:185-7, 1993
3- Komura M, Tsuchida Y, Honna T, et al. Completely covered cloacal exstrophy: recognition of a new clinical sub-entity. Pediatr Surg Int 8:157-61, 1993
4- Oshita M, Okazaki T, Lee KD, et al. Complete covered cloacal exstrophy. Pediatr Surg Int 23:1029-31, 2007
5- Levitt MA(1), Mak GZ, Falcone RA Jr, Pena: Cloacal exstrophy--pull-through or permanent stoma? A review of 53 patients.J Pediatr Surg. 43(1):164-8, 2008
6-Lund DP(1), Hendren WH: Cloacal exstrophy: a 25-year experience with 50 cases.  J Pediatr Surg. 36(1):68-75, 2001
*Best review.

Angiomatoid Fibrous Histiocytoma

Angiomatoid fibrous histiocytoma (AFH) is a soft tissue tumor with intermediate malignant potential that occurs primarily in children, adolescent and young adults very rarely encountered past the age of 40 years. The median age of presentation is 14 years accounting for 0.3% of all soft tissue neoplasms. It develops as a slowly growing nodular, multinodular or cystic mass of the hypodermis or subcutis occurring most commonly in the extremity of the child. Local symptoms such as pain and tenderness are uncommon, but systemic symptoms such as anemia, fever, malaise and weight loss are occasionally encountered suggesting the production of cytokines by the tumor. The diagnosis is rare made preoperatively. Imaging findings of AFH are as nonspecific as its histogenesis. MRI demonstrates multiple internal cystic areas, an enhancing fibrous pseudocapsule which is markedly hypointense on T1- and T2WI, and foci of susceptibility artifacts representing hemosiderin.  The diagnosis of AFH is made based on histopathology and immunohistology. AFH is generally firm and circumscribed. The characteristic microscopic appearance includes distributions of ovoid to spindle cells with bland, vesicular nuclei, lymphoplasmacytic infiltrate with intervening blood-filled cystic spaces, and a fibrous pseudocapsule. Immunohistochemistry variably demonstrates positivity for desmin, CD68 and CD 99. Management of AFH is surgical resection. Wide surgical excision with clear margins and post-excisional monitoring is warranted. Most patients are free of disease after local excision with a minority developing recurrent or less commonly metastatic disease within two years after surgery. Local recurrence can also be managed with radiation therapy.

1- Cernik C, Channaiah D, Trevino J: Angiomatoid fibrous histiocytoma in a six-year-old child. Pediatr Dermatol. 26(5):636-8, 2009
2- Qian X, Hornick JL, Cibas ES, Dal Cin P, Domanski HA: Angiomatoid fibrous histiocytoma a series of five cytologic cases with literature review and emphasis on diagnostic pitfalls. Diagn Cytopathol. 40 Suppl 2:E86-93, 2012
3- Bauer A, Jackson B, Marner E, Gilbertson-Dahdal D: Angiomatoid fibrous histiocytoma: a case report and review of the literature.  J Radiol Case Rep. 6(11):8-15, 2012
4- Kao YC, Lan J, Tai HC, Li CF, Liu KW, Tsai JW, Fang FM, Yu SC, Huang HY: Angiomatoid fibrous histiocytoma: clinicopathological and molecular characterisation with emphasis on variant histomorphology. J Clin Pathol. 67(3):210-5, 2014
5- Kaygusuz EI, Cetiner H, Yorganci C, Celayir A: A case report: angiomatoid fibrous histiocytoma in a 6-year-old male and review of the literature. Fetal Pediatr Pathol. 33(3):145-50, 2014
6- Huerter ME, Hammadeh R, Zhou Q, Riker AI: Recurrent angiomatoid fibrous histiocytoma: a case report and review of the literature. Ochsner J. 14(3):441-4, 2014

PSU Volume 44 No 02 FEBRUARY 2015

Reexpansion Pulmonary Edema

Reinflation of a collapsed lung in a few cases can lead to pulmonary edema of the reexpanded lung.  This complication termed reexpansion pulmonary edema (RPE) may occur after treatment of a lung that has collapsed after pneumothorax, pleural effusion or thoracoscopic resection of a mediastinal tumor in less than 1% of all cases. The clinical presentation of RPE is characterized by a rapid onset of dyspnea and tachypnea with symptoms developing upon one hour of reexpansion of the lung. Simple chest films may reveal interstitial opacities, consolidations with air bronchograms, and fissural inflammation. Risk factors for RPE includes the degree and chronicity of lung collapse (usually greater than 72 hours), great amount of pleural air or fluid, high speed of reexpansion, use of high negative pressure to do so, hypertension, hypoxemia and previous lung disease. Severe RPE can lead to bradycardia, hypotension, cardiopulmonary arrest, and death. RPE occurs most frequently in a chronically collapsed lung, which is then rapidly reinflated using high suction. The endpoint of the reexpansion injury is an increase in permeability of the endovascular cells and increase in hydrostatic pressure, which then leads to the pulmonary edema. Both of them cause fluid and protein overflow into the pulmonary interstitial space and alveoli, leading to pulmonary edema As a form of prevention the use of immediate suction to a chest tube placed for reexpanding a collapse lung after pneumothorax or effusion should be avoided as this is a precipitant factor for development of RPE. It is preferably that the lung reexpand more gradually. Treatment once RPE occurs remains supportive. The cornerstone is positive-pressure mechanical ventilation and utilization of positive end-expiratory pressure (PEEP) to reexpand collapsed alveoli, increase functional residual capacity, and reduce shunting.  Treatment also may include steroids, diuresis and vasopressor support. In the future, the use of agents such as monoclonal antibody to IL-8 or XOD antagonists may be useful for prevention or treatment of RPE.

1- Paksu MS(1), Paksu S, Akgan M, Kalayca AG, Baysal K: Bilateral reexpansion pulmonary edema associated with pleural empyema: a case report. Eur J Pediatr. 170(9):1205-7, 2011
2- Jardine OS: Reexpansion pulmonary edema. Am J Dis Child. 145(10):1092-4, 1991
3- Kira S, Tozawa K, Sato M, Fukunaga T, Suzuki M: Suspected reexpansion pulmonary edema during emergence from general anesthesia in a child with developmental dysplasia of the hip. Paediatr Anaesth. 22(6):591-2, 2012
4- Neustein SM: Reexpansion pulmonary edema. J Cardiothorac Vasc Anesth. 21(6):887-91, 2007
5- Rodrigues AL(1), Lopes CE, Romaneli MT, Fraga Ade M, Pereira RM, Tresoldi AT: Reexpansion pulmonary edema in children. Rev Paul Pediatr. 31(3):411-5, 2013
6- Kira S: Reexpansion pulmonary edema: review of pediatric cases. Paediatr Anaesth. 24(3):249-56, 2014

Turner Syndrome

Turner syndrome (TS), also known as gonadal dysgenesis, is a fairly common chromosomal abnormality occurring in females. It is characterized by short stature, web neck, cubitus valgus, low hairline, short 4th and 5th metacarpals, sexual infantilism, bilateral rudimentary streak gonads and primary amenorrhea among other defects. Fifty percent of TS have sex chromosome monosomy with a 45,X karyotype and the remaining patients have either mosaicism with a 45,X cell line or a structural X anomaly. Girls with typical TS with bilateral streak gonads are not at risk for development of gonadoblastoma in their dysgenetic gonads and gonadectomy is not indicated. In routine cytogenetic analysis the Y chromosome or Y-specific sequence is present in 5 to 10% of patient with TS. A higher incidence of Y-chromosome material has been reported when polymerase chain reaction (PCR) and fluorescent in situ hybridization (FISH) techniques are used in addition to peripheral blood karyotyping. Dysgenetic gonads with the presence of a Y chromosome or translocated fragment have a significant risk of developing germ cell tumors, specifically gonadoblastoma, in their dysgenetic gonads. The risk ranged between 25% and 75% and increases with advancing age. Gonadoblastoma is the most commonly found tumor and considered an in-situ neoplastic lesion with further risk for malignant transformation to a dysgerminoma or another invasive germ cell tumor (such as yolk sac tumor, embryonal cell carcinoma and malignant teratoma). Gonadoblastoma is mostly seen in those TS with a 45,X/46,XY karyotype. Prophylactic gonadectomy is currently recommended in all TS patients with Y-chromosome material. Laparoscopic salpingo-oophorectomy of the streak gonad is the preferred procedure. This approach eliminates the complication of future ectopic tubal pregnancy along with the possibility of tubal malignancy.

1- Mazzanti L, Cicognani A, Baldazzi L, Bergamaschi R, Scarano E, Strocchi S, Nicoletti A, Mencarelli F, Pittalis M, Forabosco A, Cacciari E: Gonadoblastoma in Turner syndrome and Y-chromosome-derived material. Am J Med Genet A. 135(2):150-4, 2005
2- Liu AX, Shi HY, Cai ZJ, Liu A, Zhang D, Huang HF, Jin HM: Increased risk of gonadal malignancy and prophylactic gonadectomy: a study of 102 phenotypic female patients with Y chromosome or Y-derived sequences. Hum Reprod. 29(7):1413-9, 2014
3- Sallai A, Salyom J, Dobos M, et al: Y-chromosome markers in Turner syndrome: Screening of 130 patients. J Endocrinol Invest. 33(4):222-7, 2010
4- Oliveira RM, Verreschi IT, Lipay MV, et al: Y chromosome in Turner syndrome: review of the literature. Sao Paulo Med J. 127(6):373-8 2009
5- Trabs RB, Hoepffner W, Bahligen U, Limbach A, Keller E, Schatz A, Horn LC, Kiess W, Bennek J: Video-assisted gonadectomy in children with Ullrich Turner syndrome or 46,XY gonadal dysgenesis. Eur J Pediatr Surg. 14(3):179-84, 2004
6- Kanakatti Shankar R, Inge TH, Gutmark-Little I, Backeljauw PF: Oophorectomy versus salpingo-oophorectomy in Turner syndrome patients with Y-chromosome material: clinical experience and current practice patterns assessment. J Pediatr Surg. 49(11):1585-8, 2014

Aortoesophageal Fistula

Aortoesophageal fistula (AEF) is a very rare, serious and almost lethal condition in a child if not diagnosed promptly and managed expedite. Foreign body ingestion remains the commonest cause of AEF seen in children. AEF presents with the classic triad of midthoracic pain, a small sentinel hemorrhage followed later by an exsanguinating hemorrhage. Any child presenting with these symptoms should be suspected of having an AEF until proven otherwise. Most children presenting with an AEF will have had a congenital cardiac or vascular anomaly which required surgical correction or have ingested a foreign body such as fish bone, chicken bones or stuck button batteries. The hemodynamic stability of the patient dictates whether further diagnostic investigations are appropriate. Diagnosis can be established using esophagoscopy, CT-angio Scan, conventional arteriography or upper gastrointestinal contrast imaging. Temporary control of the bleeding from an AEF can be obtained using Sengstaken-Blakemore tube or bedside placement of an aortic occlusion balloon until either diagnosis or definitive surgery can be performed. Other temporizing measures to control hemorrhage include endovascular stents, hemoclips at endoscopy, and radiographic embolization. Surgical exploration of the thoracic esophagus and aorta with repair of the fistula, preferably under cardiopulmonary bypass, remains the only hope for cure and survival for children with AEF. In cases of stuck battery in the esophagus, they should undergo emergency endoscopic removal and inspection of the esophageal mucosa. Failure to remove batteries within two hours can lead to esophageal necrosis and aortoesophageal fistulas. The mortality of AEF is extremely high.

1-Stuth EA, Stucke AG, Cohen RD, Jaquiss RD, Kugathasan S, Litwin SB: Successful resuscitation of a child after exsanguination due to aortoesophageal fistula from undiagnosed foreign body. Anesthesiology. 95(4):1025-6, 2001
2- Hill SJ, Zarroug AE, Ricketts RR, Veeraswamy R: Bedside placement of an aortic occlusion balloon to control a ruptured aorto-esophageal fistula in a small child. Ann Vasc Surg. 24(6):822.e7-9, 2010
3- Coates LJ, McNally J, Caputo M, Cusick E: Survival in a 2-year-old boy with hemorrhage secondary to an aortoesophageal fistula. J Pediatr Surg. 46(12):2394-6, 2011
4- Pae SJ, Habte SH, McCloskey JJ, Schwartz AJ: Battery ingestion resulting in an aortoesophageal fistula. Anesthesiology. 117(6):1354, 2012
5- Panda SS, Agarwala S, Kabra SK, Ray R, Sugandhi N, Bhat AS, Lodha R, Joshi P,  Bisoi AK, Arora A, Gupta AK: Aortoesophageal fistula in a child. J Indian Assoc Pediatr Surg. 18(3):124-6, 2013
6-  Krieves MA, Merritt GR, Nichols CS, Schwartz LI, Campbell DN, Bruny JL, Fagan  TE, Thompson ME, Ing RJ: Aortoesophageal fistula and coarctation of the aorta in a 15-year-old child. Semin Cardiothorac Vasc Anesth. 17(4):294-7. 2013

PSU Volume 44 No 03 MARCH 2015

Prune Belly Syndrome

Prune belly syndrome (PBS) is a rare malformation occurring one in 40,000 live births affecting almost exclusively males (95%), and characterized by deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. The protruding hypoplastic abdominal wall looks like a dried prune. The pathogenesis of PBS is nor fully understood. Malformations of the urinary tract in PBS are due to dysplasia of the smooth muscle of the renal pelvis, ureters and prostatic part of the urethra. Three clinical manifestations are characterized in PBS: non-viable oliguric form due to severe kidney dysplasia, a serious form consisting of marked renal dysplasia with megaureters, mega-vesicles and progressive renal failure or the more favorable form with moderate renal dysplasia and different degrees of ureters and bladder enlargement. Diagnosis can be made prenatally and clinically. Orthopedic deformities (hip dysplasia, missing extremity, club feet) are the second most common associated malformation. Anomalies of the GI tract (malrotation, volvulus, atresia) occur in almost 30% of PBS. Those with oligohydramnios develop pulmonary hypoplasia. Prenatal vesicoamniotic shunt for urinary obstruction can prevent pulmonary hypoplasia and renal dysplasia. The associated nephropathy is partly dysplastic and obstructive. Cryptorchidism is present in almost all cases, with favorable histology. Bladder capacity is enlarged with detrusor muscle thickened and presence of vesicoureteral reflux. Ureters are elongated, dilated with inefficient peristalsis. Treatment of PBS encompasses preserving kidney function with temporary urinary diversion and subsequent surgical reconstruction, reimplantation of dilated ureters, orchidopexy and abdominoplasty. Timing of repair is controversial and should be tailored on an individual basis following a conservative approach. Abdominoplasty and orchiopexy have both physiological and improved quality of life benefits. Renal failure is main cause of death.

1- Woods AG, Brandon DH: Prune belly syndrome. A focused physical assessment. Adv Neonatal Care. 7(3):132-43, 2007
2- Tonni G, Ida V, Alessandro V, Bonasoni MP: Prune-belly syndrome: case series and review of the literature regarding early prenatal diagnosis, epidemiology, genetic factors, treatment, and prognosis. Fetal Pediatr Pathol. 31(1):13-24, 2013
3- Hassett S, Smith GH, Holland AJ: Prune belly syndrome. Pediatr Surg Int. 28(3):219-28, 2012
4- Tran S, Grossman E, Barsness KA: Prune belly syndrome, splenic torsion, and malrotation: a case report. J Pediatr Surg. 48(2):e41-3, 2013
5- Zugor V, Schott GE, Labanaris AP: The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease. Pediatr Rep. 4(2):e20, 2012
6- Danes FT, Lopes RI, Oliveira LM, Tavares A, Srougi M: Modified abdominoplasty for patients with the Prune Belly syndrome. Urology. 83(2):451-4, 2014
7- Ekwunife OH, Ugwu JO, Modekwe V: Prune belly syndrome: early management outcome of nine consecutive cases. Niger J Clin Pract. 17(4):425-30, 2014

Poland Syndrome

Poland syndrome (PS) is a spectrum of congenital chest wall deformities sporadic in occurrence characterized  by chest wall hypoplasia. This is caused by absence of the pectoralis major, pectoralis minor, serratus anterior, rectus abdominis and latissimus dorsi muscle. Other associated conditions include athelia or amastia, nipple deformities, limb deformities (syndactylism, brachydactyly), absent axillary hair and limited subcutaneous fat. Severe (complex) cases include thoracic cage anomalies, most frequently involving ribs II–V. The disease may be inherited as an autosomal-dominant trait. Clinical manifestations of PS are extremely variable and rarely are all the features recognized in one individual. The right side is more commonly affected and is present in males 70% of the time. The etiology of Poland syndrome is unknown but might include abnormal migration of the embryonic tissues forming the pectoralis muscle, hypoplasia of the subclavian artery or a traumatic event in utero. Poland syndrome can occur in varying degrees with mild hypoplasia to total aplasia of muscles, ribs and cartilage. Surgical repair varies according to the extent of Poland syndrome, age and sex of the patient. In girls chest wall reconstruction should precede breast reconstruction. In the complex forms chest wall reconstruction has traditionally been advocated with the use of contralateral, autologous rib grafts stabilized with mesh. Recently the vertical expandable prosthetic titanium rib expander has been reported to stabilize the chest wall after rib grafting. Additional stages of reconstruction include expanders, musculocutaneous flaps, breast implants, nipple and areolar reconstruction and fat grafting. For symmetry reasons surgery to the contralateral breast can be considered.

1- Lieber J, Kirschner HJ, Fuchs J: Chest wall repair in Poland syndrome: complex single-stage surgery including Vertical Expandable Prosthetic Titanium Rib stabilization--a case report. J Pediatr Surg. 47(3):e1-5, 2012
2- Stephenson JT, Song K, Avansino JR, Mesher A, Waldhausen JH: Novel titanium constructs for chest wall reconstruction in children.  J Pediatr Surg. 46(5):1005-10, 2011
3- Garg R, Saheer S, Gupta V, Mehra S: Poland sequence: Series of two cases and brief review of the literature. Ann Thorac Med. 7(2):110-2, 2012
4- Dolas SC(1), Poovamma CU(1), Prema M(1), Khandelwal R(1), Pais AV(1), Kaul A(2): Poland's syndrome: a case report with review of literature regarding management. Breast Dis. 34(3):121-5, 2014
5- Chiummariello S, Pica A, Guarro G, Arleo S, Alfano C: Poland syndrome: an algorithm to select the appropriate chest wall surgical reconstructive treatment. Ann Ital Chir. 85(3):237-43, 2014
6- Rodriguez IE, Heare T, Bruny J, Deleyiannis FW: Customized Titanium Implant for Chest Wall Reconstruction in Complex Poland Syndrome. Plast Reconstr Surg Glob Open. 2(2):e112, 2014


Adipose tumors comprise 5% of all soft-tissue neoplasm in children. Two-third are simple lipomas, 30% lipoblastoma and the rest rare liposarcomas. Liposarcomas occur most commonly in the 3rd to 7th decade of life with a slight predominance in males. They are extremely unusual in children younger than 10 years. In children liposarcoma occurs most commonly in the lower extremity, most have tumors> 5 cm at initial presentation and metastatic disease at the time of initial diagnosis is uncommon. Clinically they present as nontender, slow and progressively growing soft-tissue mass. The myxoid variant is the most common histologic variant of liposarcoma in children. Histologic grade is one of the most important predictors of outcome, with low-grade myxoid tumors having significantly improved survival rates compared to the round-cell, pleomorphic, and dedifferentiated subtypes. Complete surgical resection remains the mainstay of local therapy, but adjuvant radiation therapy is effective at controlling microscopic residual disease after surgical resection. Myxoid tumors are radiosensitive and pre-, intra- and postoperative radiation approach have been effective therapy. The role of chemotherapy for treatment of pediatric liposarcoma is not well established except a role in facilitating tumor resection in patients with unresectable disease. The overall prognosis of myxoid liposarcoma is excellent with surgical treatment alone. The pleomorphic subtype portends a poorer prognosis. 

1- Miller GG, Yanchar NL, Magee JF, Blair GK: Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of  the literature. Can J Surg. 41(6):455-8, 1998
2- Chitnis M, Steyn T, Koeppen P, Breckon V, Lazarus C: Differentiation of a benign myxolipoma from a myxoid liposarcoma by tumour karyotyping--a diagnosis missed. Pediatr Surg Int. 18(1):83, 2002
3- ten Heuvel SE, Hoekstra HJ, van Ginkel RJ, Bastiaannet E, Suurmeijer AJ: Clinicopathologic prognostic factors in myxoid liposarcoma: a retrospective study of 49 patients with long-term follow-up. Ann Surg Oncol. 14(1):222-9, 2007
4- Alaggio R, Coffin CM, Weiss SW, Bridge JA, Issakov J, Oliveira AM, Folpe AL: Liposarcomas in young patients: a study of 82 cases occurring in patients younger than 22 years of age. Am J Surg Pathol. 33(5):645-58, 2009
5- Huh WW, Yuen C, Munsell M, Hayes-Jordan A, Lazar AJ, Patel S, Wang WL, Barahmani N, Okcu MF, Hicks J, Debelenko L, Spunt SL: Liposarcoma in children and young adults: a multi-institutional experience. Pediatr Blood Cancer. 15;57(7):1142-6, 2011
6- Schaefer IM, Fletcher CD: Myxoid variant of so-called angiomatoid "malignant fibrous histiocytoma": clinicopathologic characterization in a series of 21 cases. Am J Surg Pathol. 38(6):816-23, 2014

PSU Volume 44 No 04 APRIL 2015

Antibiotic-impregnated Catheters

Central venous catheter (CVC) and peripherally inserted central catheters (PICC) are widely used in intensive and high dependency care to provide venous access for drug delivery, intravenous feeding, monitoring and blood sampling. Nosocomial bloodstream infections are associated with increased morbidity and mortality. CVC and PICC are the main source of nosocomial bloodstream infection in critically-ill children. The main consequences of catheter-related blood stream infections are increased costs due to treatment, testing and prolonged duration of stay. This infection is caused by colonization of the catheter during insertion or following migration of organisms from the patient skin or from the hub or port of the catheter into the intravascular part of the device. This risk is higher in children receiving acute care and parenteral nutrition. The best option for reducing catheter-related blood stream infection are heparin-coated or antibiotic-impregnated central venous catheter. They are impregnated with minocycline and rifampin due to their synergistic action and potential to penetrate bacterial-secreted biofilm. In the acute critical care setting, the introduction of antibiotic-impregnated CVC/PICC are associated with a significant decrease in nosocomial primary gram-positive and gram-negative bacteremia. This reduction of nosocomial bacteremia is associated with a significant decrease in catheter-related infections and cases of nosocomial multidrug- resistant bacteremia as well as a significant decrease in the length of hospital and ICU stay. In children with burns the uses of PICC are a necessity to deliver crystalloids during the phase of resuscitation. Almost 50% of these children develop bacteremia. Antibiotic-impregnated PICC lines are five times more effective in decreasing catheter-related bloodstream infection than maximal sterile barrier alone. They also decrease the need for systemic antibiotic use in ICU. Cost saving using CVC and PICC impregnated-catheters is significant.

1-  Schutze GE: Antimicrobial-impregnated central venous catheters. Pediatr Infect Dis J. 21(1):63-4, 2002
2- Hanna HA, Raad II, Hackett B, Wallace SK, Price KJ, Coyle DE, Parmley CL; M.D. Anderson Catheter Study Group: Antibiotic-impregnated catheters associated with significant decrease in nosocomial and multidrug-resistant bacteremias in critically ill patients. Chest. 124(3):1030-8, 2003
3- Bhutta A, Gilliam C, Honeycutt M, Schexnayder S, Green J, Moss M, Anand KJ: Reduction of bloodstream infections associated with catheters in paediatric intensive care unit: stepwise approach. BMJ. 334(7589):362-5, 2007
4- Gilbert RE, Harden M: Effectiveness of impregnated central venous catheters for catheter related blood stream infection: a systematic review. Curr Opin Infect Dis. 21(3):235-45, 2008
5- Armstrong SD, Thomas W, Neaman KC, Ford RD, Paulson J: The impact of antibiotic impregnated PICC lines on the incidence of bacteremia in a regional burn center. Burns. 39(4):632-5, 2013
6- Baskin KM, Hunnicutt C, Beck ME, Cohen ED, Crowley JJ, Fitz CR: Long-term central venous access in pediatric patients at high risk: conventional versus antibiotic-impregnated catheters. J Vasc Interv Radiol. 25(3):411-8, 2014

Handlebar Hernia

Traumatic abdominal wall hernia is produced by sudden application of a blunt force that is insufficient to penetrate the skin but strong enough to disrupt the muscle and fascia. In combination with a direct blow to the abdominal wall a sudden increase in intra-abdominal pressure may induce disruption of the abdominal musculature and fascia with the skin remaining intact. These traumatic abdominal wall hernias are categorized as Type 1: small defect such as that caused from a bicycle handlebar, Type 2:  larger defect caused by high-energy transfer such as a motor vehicle crash or fall, and Type 3: defects that involve intraabdominal bowel herniation as described in deceleration injuries. Most traumatic abdominal wall hernias in children are Type 1, also called handlebar hernias. They occur in children between the ages of 5 and 14 years, mostly males. A skin contusion or abrasion is identified in most cases. The majority of handlebar hernias involved a lower abdominal wall defect and they seldom are associated with another intraabdominal injury. Diagnosis is made by history and physical examination (tender bulge or swelling). Ultrasonography and CT-Scan are important diagnostic imaging modalities utilized. Definitive management requires surgical repair of the defect to prevent complications such as bowel obstruction, incarceration, or strangulation with resultant bowel ischemia. Repair is made with primary closure of all the tissue layers or using prosthetic material if the defect is large. Whether to do a formal exploratory laparotomy is debatable due to the low incidence of associated intraabdominal injury found in review cases. In the setting of blunt abdominal trauma, the role of diagnostic and therapeutic laparoscopy is emerging as a reasonable initial option in management. Laparoscopy provides evaluation of solid organ, diaphragmatic, small bowel, mesenteric, and anterior abdominal wall injury.

1-  Chen HY, Sheu MH, Tseng LM: Bicycle-handlebar hernia: a rare traumatic abdominal wall hernia. J Chin Med Assoc. 68(6):283-5, 2005
2- Haimovici L, Papafragkou S, Kessler E, Angus G: Handlebar hernia: traumatic abdominal wall hernia with multiple enterotomies. A case report and review of the literature. J Pediatr Surg. 42(3):567-9, 2007
3- Goliath J, Mittal V, McDonough J: Traumatic handlebar hernia: a rare abdominal wall hernia. J Pediatr Surg. 39(10):e20-2, 2004
4- McKinley AJ, Mahomed AA: Laparoscopy in a case of pediatric blunt abdominal trauma. Surg Endosc. 16(2):358, 2002
5- Kubota A, Shono J, Yonekura T, Hoki M, Asano S, Hirooka S, Kosumi T, Kato M, Oyanagi H: Handlebar hernia: case report and review of pediatric cases. Pediatr Surg Int. 15(5-6):411-2, 1999
6- Iinuma Y, Yamazaki Y, Hirose Y, Kinoshita H, Kumagai K, Tanaka T, Miyajima M, Nitta K, Naitoh S, Kobayashi K: A case of a traumatic abdominal wall hernia that could not be identified until exploratory laparoscopy was performed. Pediatr Surg Int. 21(1):54-7, 2005

Fibrin Glue for Pilonidal Sinus Disease

Pilonidal sinus disease (PSD) is caused by hair that penetrates skin and gluteal cleft causing cyst, sinus formation, infection and abscess. Is the most common benign disease causing school lost in adolescent children. The diagnosis is made clinically, seldom needing imaging studies. From time many surgical approaches have been utilized to remove permanently PSD. They include primary excision with closure, radical excision leaving the wound opened, incision of the sinus with curettage, marsupialization, application of phenol, cleft lift procedure, cryosurgery and laser. The lowest recurrence rates have been described for the lateral cleft lift procedure approach. Leaving the wound opened prolongs the healing process unless vacuum-assisted closure therapy is utilized. The gold standard for management of PSD in children is a lateralizing flap procedure. Fibrin glue is a biological adhesive material that is made from human fibrinogen and is being used as a sealant for the treatment of fistulae. It promotes wound healing by enhancing homeostasis and angiogenesis, stimulating macrophages and collagen production at the wound site. The use of fibrin glue to gap the closed space after excision and closure of PSD has been recently found to be very effective management strategy. Fibrin glue promotes hemostasis, sealing and healing speeding patient recovery and reducing pilonidal disease recurrence. This approach is recommended as primary treatment and for recurrence of PSD in children.

1- Lund JN, Leveson SH: Fibrin glue in the treatment of pilonidal sinus: results of a pilot study. Dis Colon Rectum. 48(5):1094-6, 2005
2- Seleem MI, Al-Hashemy AM: Management of pilonidal sinus using fibrin glue: a new concept and preliminary experience. Colorectal Dis. 7(4):319-22, 2005
3- Patti R, Angileri M, Migliore G, Sparancello M, Termine S, Crivello F, Gioa¨FP, Di Vita G: Use of fibrin glue in the treatment of pilonidal sinus disease: a pilot study. G Chir. 27(8-9):331-4, 2006
4- Handmer M: Sticking to the facts: a systematic review of fibrin glue for pilonidal disease. ANZ J Surg. 82(4):221-4, 2012
5- Brown SR: Invited comment on Elsey and Lund: fibrin glue in the treatment of pilonidal sinus: high patient satisfaction and rapid return to normal activities. Tech Coloproctol. 17(1):105-6, 2013
6- Smith CM, Jones A, Dass D, Murthi G, Lindley R: Early experience of the use of fibrin sealant in the management of children with  pilonidal sinus disease.  J Pediatr Surg. 50(2):320-2., 2015

PSU Volume 44 NO 05 MAY 2015


Hemobilia is defined as bleeding into the biliary tract due to a communication between a blood vessel and the bile ducts. Hepatic trauma (iatrogenic and accidental) is the most frequent cause of hemobilia, but it can also occur after inflammation, hepatobiliary tumors, percutaneous liver biopsy and vascular disorders. The proximity of the intrahepatic bile ducts and the hepatic vascular supply accounts for the occasional development of an arteriobiliary or portobiliary fistula and hemobilia. Hemobilia may be major and present with life-threatening hemorrhage or minor and present many weeks after the initial injury. The classic Sandblom triad of jaundice, epigastric pain and upper GI bleeding occurs in one-third of all patients. Arterial bleeding may be so rapid that blood is easily dissolved in bile passing directly into the duodenum appearing as either hematemesis or melena. With slow hemorrhage the blood and bile do not mix and clots obstruct the bile ducts producing colicky abdominal pain and jaundice. Upper GI endoscopy rules out a bleeding source in the esophagus, stomach or duodenum and can detect bleeding from the ampulla of Vater. Other diagnostic studies performed include US or CT-Scan. Hepatic arteriography is the diagnostic and therapeutic modality of choice. Findings at arteriography are usually a pseudoaneurysm. Significant hemobilia seldom ceases spontaneously and usually necessitates surgical or angiographic intervention. Surgical management includes liver suturing or partial hepatic resection for peripheral lesions and ligation of the hepatic artery for more central lesions. Transcatheter selective arterial embolization with microcoils is currently the safest and preferred technique used to manage hemobilia. It is minimally invasive and can be combined with diagnostic arteriography. The risk of hepatic necrosis is minimal with superselective embolization. The complication rate after embolization is low due to its dual vascular supply with the portal vein and hepatic artery except in cases in which the portal vein is thrombosed.  

1- Laopaiboon V, Aphinives C, Pongsuwan P, Pugkem A, Thammaroj J, Puttharuk W: Hepatic artery embolization to control liver hemorrhages by interventional radiologists: experiences from Khon Kaen University. J Med Assoc Thai. 89(3):384-9, 2006
2- Srivastava DN, Sharma S, Pal S, Thulkar S, Seith A, Bandhu S, Pande GK, Sahni  P: Transcatheter arterial embolization in the management of hemobilia. Abdom Imaging. 31(4):439-48, 2006
3- Gupta LB, Puri AS: Management of traumatic hemobilia with embolization. Indian Pediatr. 43(9):825-7, 2006
4- Villarreal DH, Norwood S, McAuley C, Berne JD: Hemobilia and subsequent hemocholecystitis complicating blunt hepatic injury.  J Trauma. 62(6):E18-9, 2007
5- Marynissen T(1), Maleux G, Heye S, Vaninbroukx J, Laleman W, Cassiman D, Verslype C, Van der Merwe S, Van Steenbergen W, Nevens F: Transcatheter arterial embolization for iatrogenic hemobilia is a safe and effective procedure: case series and review of the literature. Eur J Gastroenterol Hepatol. 24(8):905-9, 2012
6- Zaleska-Dorobisz U, Lasecki M, Olchowy C, Ugorski W, Garcarek J, Patkowski D, Kurcz J: Iatrogenic hemobilia in 10-year-old boy. Pol J Radiol. 79:279-82, 2014


Gynecomastia in pubertal boys is a very distressing condition. Development of glandular tissue (ductal hyperplasia) underneath the areola is thought to occur from an imbalance of free testosterone and estrogen as opposed to the hormone bound to sex hormone binding globulin. Certain medications compete with estrogen binding more than testosterone bindings causing free estrogen to be higher thus stimulating glandular growth in pubertal males. Drugs implicated in breast enlargement include spironolactone, marihuana, amphetamines, anabolic steroids, digoxin, Valium, metronidazole, omeprazole, ranitidine, and metoclopramide. Clinical manifestations consist of soft, elastic, nodule-like retroareolar mass that is occasionally associated with pain. Endocrine evaluation of these patients is usually negative and there is no need for ultrasound or imaging studies to diagnose gynecomastia. Pseudogynecomastia is a form of bilateral breast enlargement which occurs from excess chest fat in obese children. It is clinically manifested by increases in volume that are diffuse, non-nodular and symmetrical. The diagnosis is made on clinical findings. Ultrasound reveals the presence of lobular areas of adipose tissue that are homogenously hypoechogenic and separated from one another by thin hyperechoic bands of fibrous tissue. Initial management of pseudogynecomastia is reassurance and weight reduction. With persistent distress excisional surgery for both pseudogynecomastia and gynecomastia with or without added liposuction for contouring of the chest and upper abdomen ensures flat chests and no partial return of breast enlargement.  

1-  Gusenoff JA, Coon D, Rubin JP: Pseudogynecomastia after massive weight loss: detectability of technique, patient satisfaction, and classification. Plast Reconstr Surg. 122(5):1301-11, 2008
2- Castillo PF, Sepulveda C, Troncoso AL, Villaman JJ, Cuadra A: Transumbilical approach for pseudogynecomastia liposuction. Aesthetic Plast Surg. 33(6):832-3, 2009
3- Johnson RE, Murad MH: Gynecomastia: pathophysiology, evaluation, and management. Mayo Clin Proc. 84(11):1010-5, 2009
4- Venkata Ratnam B: How important is "pseudogynecomastia"? Aesthetic Plast Surg. 35(4):668-9, 2011
5- Draghi F, Tarantino CC, Madonia L, Ferrozzi G: Ultrasonography of the male breast. J Ultrasound. 14(3):122-9, 2011
6- Senger JL, Chandran G, Kanthan R: Is routine pathological evaluation of tissue from gynecomastia necessary? A 15-year retrospective pathological and literature review. Can J Plast Surg. 22(2):112-6, 2014

Anorectal Manometry

Anorectal manometry (ARM) is used to investigate children with chronic constipation, fecal incontinence, as a tool to evaluate continent results after surgery and determine if the child is a candidate for biofeedback therapy. ARM is a noninvasive procedure that explains the mechanisms of defecation disorders because of hypertonia, low tone or paradoxical shrinkage of the internal anal sphincter. ARM can study the recto-anal inhibitory reflex (RAIR) which is absent in cases of Hirschsprung's disease. Manometry investigation of anorectum in an awake and compliant child provides valuable information about physiological function including rectal sensations, defecation dynamics and somatic reflexes. Fecal incontinent children will show decrease resting and maximum squeeze pressures along with decrease maximum tolerable rectal volumes and impaired external anal sphincter response to rectal distension. Internal anal sphincter achalasia will show absent RAIR and normal rectal biopsy.  Manometric assessment has been the principal method to obtain objective data of postoperative sphincter function by comparing with normative data for each age group. Absence of RAIR in postoperative patients signifies internal anal sphincter damage or maldevelopment. ARM is difficult to perform in children younger than one year of age. With uncooperative children the use of sedation with chloral hydrate, midazolam or ketamine has been proposed. Ketamine anesthesia does not affect quantitative or qualitative measurements of autonomic anorectal function in children who are investigated for chronic functional constipation and soiling. It can be used reliably in children, who are young and uncooperative for awake study and in those who require additional painful procedures. Ketamine can be used in conjunction with endosonography to elucidate evidence of sphincter damage in the context of iatrogenic injuries, sexual abuse and surgery for anorectal malformations and Hirschsprung's disease.

1- Senel E, Demirbag S, Tiryaki T, Erdogan D, Cetinkursun S, Cakmak O: Postoperative anorectal manometric evaluation of patients with anorectal malformation. Pediatr Int. 49(2):210-4, 2007
2- Noviello C, Cobellis G, Papparella A, Amici G, Martino A: Role of anorectal manometry in children with severe constipation. Colorectal Dis. 11(5):480-4, 2009
3- Kumar S, Al Ramadan S, Gupta V, Helmy S, Debnath P, Alkholy A: Use of anorectal manometry for evaluation of postoperative results of patients with anorectal malformation: a study from Kuwait.  J Pediatr Surg. 45(9):1843-8, 2010
4- Hong J: Clinical applications of gastrointestinal manometry in children. Pediatr Gastroenterol Hepatol Nutr. 17(1):23-30, 2014
5- Mousavi SA, Karami H, Rajabpoor AA: Intractable chronic constipation in children: outcome after anorectal myectomy. Afr J Paediatr Surg. 11(2):147-9, 2014
6- Keshtgar AS, Choudhry MS, Kufeji D, Ward HC, Clayden GS: Anorectal manometry with and without ketamine for evaluation of defecation disorders in children. J Pediatr Surg. 50(3):438-43, 2015

PSU Volume 44 NO 06 JUNE 2015

Anti-NMDA receptor Encephalitis

Anti-N-methyl-D-aspartate (NMDA) receptor encephalitis is a rare autoimmune paraneoplastic syndrome characterized by escalating confusion, amnesia, agitation and paranoid or delusional thoughts. Most patients with anti-NMDA receptor encephalitis have the following characteristics: young females with a median age of 24 years, prominent neuropsychiatry symptoms such as behavioral or personality changes that could progress to seizures, stereotyped movements, autonomic instability or central hypoventilation, harboring of a matured ovarian or mediastinal teratomas, with detectable quantities of serum or cerebrospinal fluid antibodies that interacted with the cell membrane of rat hippocampal neurons in vivo. The teratoma produces autoantibodies to the NMDA Receptor 1 subunit of the NMDA receptor site detectable in serum and cerebrospinal fluid (anti-NMDA receptor immunoglobulin G antibody). Not all patients presenting with NMDA receptor encephalitis are females with ovarian teratomas, but the frequency is so significant that work-up should include ultrasound, CT Scans, and MRI to rule out a causative tumor. Infants and toddlers with such paraneoplastic syndrome lack an associated tumor. Common presenting symptoms of patients with NMDA receptor encephalitis include neuropsychiatric symptoms, seizures, dyskinesias, loss of consciousness, amnesia, and autonomic dysfunction. Management of anti-NMDA receptor encephalitis caused by a teratoma is removal of the tumor and immunotherapy. Removal of the teratoma is associated with decrease in serum and cerebrospinal fluid levels of the pathologic autoantibody with improvement or full recovery. Immunotherapy includes steroids, intravenous immunoglobulin and plasmapheresis. Since most cases present with neuropsychiatry symptoms, recognition by mental health professional is key to early diagnosis.

1-  Lesher AP, Myers TJ, Tecklenburg F, Streck CJ: Anti-N-methyl-D-aspartate receptor encephalitis associated with an ovarian teratoma in an adolescent female. J Pediatr Surg. 45(7):1550-3, 2010
2- Tanyi JL, Marsh EB, Dalmau J, Chu CS: Reversible paraneoplastic encephalitis in three patients with ovarian neoplasms. Acta Obstet Gynecol Scand. 91(5):630-4, 2012
3- Goldberg EM, Titulaer M, de Blank PM, Sievert A, Ryan N: Anti-N-methyl-D-aspartate receptor-mediated encephalitis in infants and toddlers: case report and review of the literature. Pediatr Neurol. 50(2):181-4, 2014
4- Seifi A, Xia BT, Felte RF: Thinking outside the box about young female patients with sudden-onset bizarre behavior: a case of anti-N-methyl-D-aspartate receptor encephalitis. Prim Care Companion CNS Disord. 15(4): 1-2, 2013
5- Acien P, Acien M, Ruiz-Macia E, Martin-Estefania C: Ovarian teratoma-associated anti-NMDAR encephalitis: a systematic review of reported cases. Orphanet J Rare Dis. 9:157-65, 2014
6- Li S, Zhao A: A case of anti-NMDAR encephalitis induced by ovarian teratoma. Cell Biochem Biophys. 71(2):1011-4, 2015

Hereditary Pancreatitis

Hereditary pancreatitis (HP) is a rare etiology of chronic pancreatitis in children and adults. HP is an autosomal dominant inherited disorder with an incomplete penetrance affecting mostly the white population. HP is characterized by a younger age of onset and a longer course of recurrent episodes of acute pancreatitis before reaching pancreatic insufficiency. It also is associated with a high cumulative risk of developing pancreatic ductal carcinoma more commonly seen with a paternal inheritance pattern. HP is correlated to a mutation in the PRSS1 gene located on 7q35 locus identified as R122H. PRSS1 mutation induces an inability of endogenous trypsin inhibitor binding to inactivate intrapancreatic trypsin, leading to pancreatic autolysis. Other gene mutations implicated are SPINK1 and CFTR. Accurate and reproducible genetic testing for PRSS1, SPINK1, and CFTR has improved the efficiency of diagnosis. The diagnosis of HP is usually based on the recognition of recurrent pancreatitis, usually starting in childhood, in two or more members of a family in the absence of other causes for pancreatitis. HP is characterized by acute onset of recurrent epigastric pain associated with nausea, vomiting and abdominal pressure presenting before the age of ten years. Complications such as pancreatic duct stones, duct strictures and pseudocysts are more frequent in children with HP. They are diagnosed using MRCP and ERCP. Late complications include pancreatic insufficiency with steatorrhea and insulin-dependent diabetes. Management of hereditary pancreatitis includes several objectives: pain-control, prevention of recurrence, treatment of exocrine and endocrine dysfunction, management of complications and early detection of pancreatic ductal adenocarcinoma. Endoscopic management consists of biliary/pancreatic sphincterotomy, pancreatic duct stricture dilatation, stent placement and removal of stones from the pancreatic duct. Endotherapy delays development of chronic pancreatitis and pancreatic cancer. Surgical procedures consist in lateral pancreaticojejunostomy, resection of the tail of the pancreas and duodenal sparing pancreatectomy.

1-  DuBay D, Sandler A, Kimura K, Bishop W, Eimen M, Soper R: The modified Puestow procedure for complicated hereditary pancreatitis in children. J Pediatr Surg. 35(2):343-8, 2000
2- Choudari CP, Nickl NJ, Fogel E, Lehman GA, Sherman S: Hereditary pancreatitis: clinical presentation, ERCP findings, and outcome of endoscopic therapy. Gastrointest Endosc. 56(1):66-71, 2002
3- Rebours V, Boutron-Ruault MC, Schnee M, et al: The natural history of hereditary pancreatitis: a national series. Gut. 58(1):97-103, 2009
4- Schmitt F, Le Henaff G, Piloquet H, Leclair MD, David A, Heloury Y, Podevin G: Hereditary pancreatitis in children: surgical implications with special regard to genetic background. J Pediatr Surg. 44(11):2078-82, 2009
5- Lal A, Lal DR: Hereditary pancreatitis. Pediatr Surg Int. 26(12):1193-9, 2010
6- Ceppa EP, Pitt HA, Hunter JL, Leys CM, Zyromski NJ, Rescorla FJ, Sandrasegaran K, Fogel EL, McHenry LW, Watkins JL, Sherman S, Lehman GA: Hereditary pancreatitis: endoscopic and surgical management. J Gastrointest Surg. 17(5):847-56, 2013
7- Kargl S, Kienbauer M, Duba HC, Schafl R, Pumberger W: Therapeutic step-up strategy for management of hereditary pancreatitis in children. J Pediatr Surg. 50(4):511-4, 2015

Parathyroid Carcinoma

Parathyroid carcinoma is an extremely rare cause of primary hyperparathyroidism in the pediatric population with less than ten cases reported in the world literature. Clinical manifestations of parathyroid cancer in children include palpable neck mass, bone pain, weakness, pancreatitis, malaise, polyuria, polydipsia, nausea and vomiting. Most cases report high levels of PTH associated to severe hypercalcemia with total serum calcium greater than 13 mg/dL and a palpable neck mass.  Sestamibi scan corroborated the diagnosis, while CT-Scan provides clues toward tumor resectability. Parathyroid carcinoma is confirmed by histologic examination with findings of trabecular pattern, mitotic figures, capsular and bloods vessel invasion. Management of parathyroid cancer is en bloc removal of the tumor along with the ipsilateral thyroid lobe avoiding rupture of the tumor capsule and spillage of tumor cells. Removal of adjacent enlarged or abnormal lymph nodes is recommended. The tumor metastasize to the lung primarily, followed by bone, liver and brain. Serum calcium and iPTH levels should normalize after surgery, unless unresected tumor or metastatic disease is present. Recurrence and systemic metastases occur in 50% of patients with parathyroid carcinoma. When metastatic disease is present metastasectomy is recommended to reduce hypercalcemia. Chemotherapy, adjuvant radiotherapy and medical management including calcitonin, mithramycin bisphosphonates and NPS R-568 calcimimetic agent may be used for patients with uncontrollable hypercalcemia with unresectable or metastatic disease. Prognosis is poor with mortality caused by severe hypercalcemia when widespread metastatic or unresectable disease is present.       

1-  Rock K, Fattah N, O'Malley D, McDermott E: The management of acute parathyroid crisis secondary to parathyroid carcinoma: a  case report. J Med Case Rep. 4:28, 2010
2- Wang CA, Gaz RD: Natural history of parathyroid carcinoma. Diagnosis, treatment, and results.Am J Surg. 149(4):522-7, 1985
3- Kim YS: Parathyroid carcinoma with lung metastasis in a thirteen-year-old girl. J Korean Surg Soc. 82(6):385-8, 2012
4- Ito Y, Iwase H, Tanaka H, Yuasa H, Kureyama Y, Yamashita H, Toyama T, Kimura M, Kobayashi S: Metachronous primary hyperparathyroidism due to a parathyroid adenoma and a subsequent carcinoma: report of a case. Surg Today. 31(10):895-8, 2001
5- Kebebew E: Parathyroid carcinoma. Curr Treat Options Oncol. 2(4):347-54, 2001
6- Kung B, Winokur R, Cognetti D, O'Hara B, Rosen D: Parathyroid carcinoma: a rare cause of primary hyperparathyroidism. Ear Nose Throat J. 88(9):E10-3, 2009

Journal Club