VOLUME 47, 2016

PSU Volume 47 No 01 JULY 2016

Paratesticular Rhabdomyosarcoma

Paratesticular rhabdomyosarcoma (Pt-RMS) comprise 10% of all genitourinary RMS tumors and third most common after prostate and bladder in children. Presents before the age of five years or in adolescents as a painless scrotal mass, trauma or bruising. Testicular ultrasound should be the initial diagnostic imaging demonstrating a solid heterogenous extratesticular mass. In Pt-RMS levels of beta-HCG and alpha fetoprotein are not elevated, while LDH might be elevated if there is significant metastatic disease. Thin-cut (5 mm) CT-Scan with IV/po contrast is needed once the diagnosis is established for clinical staging regarding pulmonary, mediastinal and retroperitoneal metastasis. The most common histologic variant is embryonal RMS (80%). TNM staging system is used to stage these tumors based on tumor size, invasiveness, nodal status and presence of distant metastasis. Tumor location also decides a favorable or unfavorable prognosis. Pt-RMS can be either stage I or IV given its location as a favorable primary site. RMS staging is multifactorial and outcome depends on three different classifications: Stage (determine by location, size, presence of regional nodes or metastasis), Group (based on tumor status after resection or biopsy, tumor margin and lymph node disease) and Risk (combination of stage, group and histology). FDG-PET/CT is more sensitive tool in staging an restaging patients with RMS, and also in the assessment of chemotherapy response. Evaluation should include bone marrow, bone scan and lumbar puncture. Multimodal therapy with surgery, chemotherapy and radiotherapy is used to maximize tumor control. Tissue diagnosis is the initial step and suspected Pt-RMS should undergo radical orchiectomy through an inguinal incision with high ligation of the spermatic cord. Scrotal approach is inadequate due to microscopic residual disease needing wide local re-excision of the scrotal scar. Ipsilateral lymph node dissection is controversial; recommended with evidence of enlarged lymph nodes imaging. Survival at 3-years is 95% with multimodal therapy.  

1- Kurzrock EA, Busby JE, Gandour-Edwards R: Paratesticular rhabdomyoma. J Pediatr Surg. 2003 Oct;38(10):1546-7
2- Stevens MC, Rey A, Bouvet N, Ellershaw C, et al: Treatment of nonmetastatic rhabdomyosarcoma in childhood and adolescence: third study of the International Society of Paediatric Oncology--SIOP Malignant Mesenchymal Tumor 89. J Clin Oncol. 2005 Apr 20;23(12):2618-28.
3- Marulaiah M, Gilhotra A, Moore L, Boucaut H, Goh DW: Testicular and paratesticular pathology in children: a 12-year histopathological  review. World J Surg. 2010 May;34(5):969-74.
4- Walterhouse DO, Pappo AS, Meza JL, et al: Shorter-duration therapy using vincristine, dactinomycin, and lower-dose cyclophosphamide with or without radiotherapy for patients with newly diagnosed low-risk rhabdomyosarcoma: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. J Clin Oncol. 2014 Nov 1;32(31):3547-52
5- Dangle PP, Correa A, Tennyson L, Gayed B, Reyes-Magica M, Ost M: Current management of paratesticular rhabdomyosarcoma. Urol Oncol. 2016 Feb;34(2):84-92
6- Seitz G, Fuchs J, Martus P, Klingebiel T, et al: Cooperative Weichteilsarkom Studiengruppe: Outcome, Treatment, and Treatment Failures in Patients Suffering Localized Embryonal Paratesticular Rhabdomyosarcoma. Ann Surg  20(10): 1-8, 2015

Wilms Tumor: Intracaval Extension

Wilms tumor also known as nephroblastoma is the most common malignant renal tumor in children. The survival rate of Wilms tumor has improved significantly over the years to better than 90% long-term survival with the use of surgery, chemotherapy and in a few instances radiotherapy.  Large size of tumor, involvement of adjacent vital structures and intracaval tumor thrombus are universal accepted inoperable criteria. Wilms tumor has a strong tendency to invade blood vessels in the form of tumor thrombus into the renal veins, inferior vena cava and right atrium. Extension of tumor thrombus along to the renal veins into the inferior vena cava occurs in 4-10% of all children, while intraatrial extension occurs in 1-3%. Intracaval extension has been reported to be more common in the right kidney due to a short anatomic renal vein. Most cases with intracaval extension of tumor are asymptomatic and the diagnosis is made during imaging workup (Ultrasound with Doppler, CT-Scan or MRI). Preop identification of intracaval extension is important for surgery, since removal of the tumor can cause significant bleeding and/or tumor embolization with acute cardiac decompensation or arrest after manipulation of the thrombus if it's not adhered to the vessel wall. Staging the extension includes: Level 1-infrahepatic extension < 5 cm, Level 2 - intrahepatic extension > 5 cm and Level 3 - suprahepatic or Level 4 - atrial extension. Identification of intracaval tumor extension should ideally be managed with preoperative chemotherapy prior to resection to reduce the anticipated surgical risks. Preop chemotherapy reduces size of the tumor, dissolute the thrombus and provides easy surgical removal. This approach can avoid but nor eliminate the need of cardio-pulmonary bypass and cavotomy during removal of the tumor. Failure of regression, failure to tolerate chemotherapy or acute tumor rupture may need early resection. Single best predictor of survival is the histologic subtype.   

1- Akyaz C, Emir S, Bayakpamukau N, Atahan L, Caaylar M, Kutluk T, Bayakpamukau M: Cavoatrial tumor extension in children with wilms tumor: a retrospective review of 17 children in a single center. J Pediatr Hematol Oncol. 27(5):267-9, 2005
2- Lall A, Pritchard-Jones K, Walker J, Hutton C, Stevens S, Azmy A, Carachi R: Wilms' tumor with intracaval thrombus in the UK Children's Cancer Study Group UKW3 trial. J Pediatr Surg. 41(2):382-7, 2006
3- Cristofani LM, Duarte RJ, Almeida MT, Odone Filho V, Maksoud JG, Srougi M: Intracaval and intracardiac extension of Wilms' tumor. The influence of preoperative chemotherapy on surgical morbidity. Int Braz J Urol. 33(5):683-9, 2007
4- Guo A, Wei L, Song X, Liu A: Adult wilms tumor with intracaval and intracardiac extension: report of a case and review of literature. J Cancer. 2:132-5, 2011
5- Emir S: Wilms tumor with intravascular tumor thrombus. Transl Pediatr. 3(1):29-33, 2014
6- McMahon S, Carachi R: Wilms' tumor with intravascular extension: A review article. J Indian Assoc Pediatr Surg. 19(4):195-200, 2014

Urticaria Pigmentosa

Urticaria pigmentosa (UP)falls into the classification of disorders known as pediatric maculopapular cutaneous mastocytosis. Most cases occur in white patients with lesions occurring before the age of two years in the majority of cases. Urticaria pigmentosa is associated with mutation in the signaling receptor molecule c-KIT. UP can appear as a generalized maculopapular rash in the trunk and proximal extremities or as a mastocytoma single lesion large tan-orange plaque or nodule. With mechanical irritation of the plaque or nodule histamine, leukotriene and prostaglandin is release from mast cells causing the symptoms of urticaria (Darier's sign). The diffuse form has an indolent benign course. Diagnosis of urticaria pigmentosa is clinical. Biopsy is rarely necessary but is definitive and may be performed in cases where the diagnosis is not certain based on clinical features or the child is having constant irritation of the lesion with frequent signs of urticaria and pain. Histopathological diagnosis is made by observing mast cells showing metachromasia with toluidin blue in full-thickness skin biopsy. Main management of the systemic form of the disease consist of long acting oral H1- antihistamines. Persistently symptomatic mastocytomas or blistered or ulcerated lesions may be treated with high-potency topical glucocorticoids under occlusion or surgical excision if deemed necessary. The prognosis in most cases is excellent.

1- Frieri M, Quershi M: Pediatric Mastocytosis: A Review of the Literature. Pediatr Allergy Immunol Pulmonol. 26(4):175-180, 2013
2- Mir A, Chamlin SL: A 1-year-old boy with persistent, generalized eruption. Urticaria pigmentosa. Pediatr Ann. 43(1):e13-5, 2014
3- Williams KW, Metcalfe DD, Prussin C, Carter MC, Komarow HD: Telangiectasia macularis eruptiva perstans or highly vascularized urticaria pigmentosa? J Allergy Clin Immunol Pract. 2(6):813-5, 2014
4- Ramphul N, Harikrishnan S, Harikumar C, Carmichael AJ: Urticaria pigmentosa masquerading as non-accidental injury. Arch Dis Child. 100(9):850, 2015
5- Hartmann K, Escribano L, Grattan C, et al: Cutaneous manifestations in patients with mastocytosis: Consensus report of the European Competence Network on Mastocytosis; the American Academy of Allergy, Asthma & Immunology; and the European Academy of Allergology and Clinical Immunology. J Allergy Clin Immunol. 37(1):35-45, 2016
6- Zegpi-Trueba MS, Hasban-Acuaa P, Berroeta-Mauriziano D: [Mastocytosis: Case report and literature review]. Rev Chil Pediatr. Nov 2, 2015

PSU Volume 47 No 02 AUGUST

Chylous Mesenteric Cyst

Chylous mesenteric cysts are rare intraabdominal malformations mostly found in male children before the age of fifteen years. They represent a subclassification of lymphangiomas. Chylous mesenteric cysts are rare variant of  mesenteric lesions making up to 9% of all abdominal cysts and approximately 3% of pediatric lymphangiomas. Though sometimes asymptomatic, when chylous cysts obtain a large size they produce symptoms by virtue of size and volume characterized by abdominal pain, increase in abdominal girth, nausea, vomiting, anorexia, diarrhea, constipation, bowel torsion and/or bowel obstruction. They can even rupture and cause chylous peritoneal ascites. Chylous mesenteric cysts arise from the embryonic retroperitoneal lymph sac. Failure to communicate with the lymphatic or venous system, or blockage of the lymphatics as a result of trauma, infection or neoplasm can give rise to a chylous cyst. Collection of chyle in a portion of the mesentery of the small bowel leads to cyst formation with a characteristic milky fluid. The cyst can attain an enormous size without causing significant symptoms. The composition of the fluid includes mostly chylomicrons and lymphocytes. Abdominal ultrasound and CT-Scan are diagnostic of chylous mesenteric cysts. The different surgical approaches used to manage these cysts include marsupialization, sclerotherapy, drainage, enucleation, percutaneous aspiration or excision. By far, the best management of chylous mesenteric cysts is complete surgical excision, which sometimes can include resection of the affected small bowel. Once removed chylous mesenteric cysts rarely recurs maintaining an excellent prognosis. Malignant transformation has been reported in less than 3% of all cases. 

1- Jain SP: Chylous mesenteric and retroperitoneal cysts of developmental origin amongst Ethiopians: report of four cases. Ethiop Med J. 30(4):233-8, 1992
2- Orobitg FJ, Vazquez L, De Franceschini AB, Ramos-Ruiz E: Mesenteric cyst of lymphatic origin: a radiopathological correlation and case report. P R Health Sci J. 13(3):171-4, 1994
3- Cao X, Wu J, Hong W: [Chylous mesenteric cyst in three children]. Zhongguo Dang Dai Er Ke Za Zhi. 10(3):416-7, 2008
4- Kantarci M, Doganay S, Kurtcan S, Gundogdu C, Oral A, Demir B: The Multi-Detector CT findings of giant abdominal lymphangiectasis mimicking a mesenteric cystic mass in a patient with midgut volvulus. Eurasian J Med. 40(2):94-7, 2008
5- Chen HP, Liu WY, Tang YM, Ma BY, Xu B, Yang G, Wang XJ: Chylous mesenteric cysts in children. Surg Today. 41(3):358-62, 2011
6- Guzman L1, Oppenheimer E, Lugo-Vicente H, Correa M: Chylous jejunal cyst causing volvulus in a child: case report and literature review. Bol Asoc Med P R.
105(1):42-7, 2013

Patent Processus Vaginalis

Inguinal hernia repair is probably one of the most commonest procedure performed in children. When a child has a unilateral reducible inguinal hernia, the issue of exploring the contralateral side has brought great debate among surgeons. The presence of a contralateral patent processus vaginalis (PPV) does not means a metachronous inguinal hernia will develop. With the advent of laparoscopy for repair of a unilateral inguinal hernia the issue of finding a contralateral PPV takes relevance. Using laparoscopy during ipsilateral hernia repair an average of 20% of contralateral processus vaginalis are patent. There are two ways to detect a PPV during laparoscopy: transinguinal or transumbilical approach. Transinguinal laparoscopy through the ipsilateral hernia sac during open repair is the much popular approach having a specificity of 99.5% and sensitivity of 99.4%. Transumbilical laparoscopy provides a more direct view on the inspection of the contralateral deep inguinal ring. It uses an additional umbilical incision. Studies suggest the small possibility of development of metachronous inguinal hernia developing following a negative evaluation of a contralateral PPV after using transumbilical laparoscopy. The argument is that the insufflation of the peritoneal cavity can cause the peritoneal veil at the superior aspect of the contralateral PPV to close the orifice in the intersection of the testicular vessels and vas deferens at the deep ring resulting in the false impression of absence of a contralateral PPV. Regardless of the method used to diagnose a contralateral PPV or the possibility or not of developing a contralateral inguinal hernia, most surgeons will perform closure upon diagnosis by laparoscopy. Meta-analysis has found 50% of metachronous hernia develops within one year while 90% do so in five years from the initial surgical procedure. Children at risk for development of a contralateral PPV include those under peritoneal dialysis, ventriculo-peritoneal shunts, ascites or increased intraabdominal pressure.

1- Mortellaro VE, Gasior AC, Knott EM, Shah SR, Ostlie DJ, Holcomb GW 3rd, St, Peter SD: Is there an increased risk of complications with laparoscopy looking for a contralateral patent processus vaginalis? J Laparoendosc Adv Surg Tech A. 22(7):710-2, 2012
2- Tam YH, Wong YS, Chan KW, Pang KK, Tsui SY, Mou JW, Sihoe JD, Lee KH: Simple maneuvers to reduce the incidence of false-negative findings for contralateral patent processus vaginalis during laparoscopic hernia repair in children: a comparative study between 2 cohorts. J Pediatr Surg. 48(4):826-9, 2013
3- Zhong H, Wang F: Contralateral metachronous hernia following negative laparoscopic evaluation for contralateral patent processus vaginalis: a meta-analysis. J Laparoendosc Adv Surg Tech A. 24(2):111-6, 2014
4- Shalaby R, Ismail M, Samaha A, Yehya A, Ibrahem R, Gouda S, Helal A, Alsamahy O: Laparoscopic inguinal hernia repair; experience with 874 children. J Pediatr Surg. 49(3):460-4, 2014
5- Centeno-Wolf N, Mircea L, Sanchez O, Genin B, Lironi A, Chardot C, Birraux J, Wildhaber BE: Long-term outcome of children with patent processus vaginalis incidentally diagnosed by laparoscopy. J Pediatr Surg. 50(11):1898-902, 2015
6- Ahmed H, Youssef MK, Salem EA, Fawzi AM, Desoky EA, Eliwa AM, Sakr AM, Shahin AM: Efficacy of laparoscopically assisted high ligation of patent processus vaginalis in children.  J Pediatr Urol. 12(1):50-55, 2016

Meckel Diverticulitis

Meckel's diverticulum is an out pouching true ileum diverticulum occurring in 2% of the population two feet from the ileocecal valve described as the most common congenital anomaly of the gastrointestinal tract. It occurs in the antimesenteric border of the ileum and can contain two types of ectopic tissue: pancreas or gastric. Meckel's diverticulum can lead to several complications in children such as 1) bleeding, 2) obstruction, and 3) inflammation with gangrene or perforation. Meckel diverticulitis can present with clinical signs suggestive of acute appendicitis such as abdominal pain, distension, tenderness and rebound tenderness. Meckel diverticulitis is more common in adults than children. Inflammation of a Meckel's diverticulum can occur due to the presence of ectopic gastric mucosa with wall ulceration or due to obstruction of the lumen of the diverticulum with vascular involvement. Obstruction can be caused by food, enterolith, foreign body or even parasites. The diverticulum itself may serve as a fulcrum for twisting of the adjacent small bowel with resultant obstruction. During sonography the inflamed Meckel diverticulum can be seen as a tubular hypoechoic structure or a complex mass leading to then wrong diagnosis of appendicitis or intestinal duplication. Routine color Doppler sonography reveals anomalous vessels and signs of inflammation on the wall of the Meckel's diverticulum. Oral contrast CT-Scan findings of an inflamed Meckel include a blind-ending pouch of variable size and mural thickness containing fluid, air, or particulate material with surrounding mesenteric inflammation. The location of the diverticulum can vary between right lower quadrant to the mid abdomen. Laparoscopy is a safe and effective alternative in the management of a complication Meckel diverticulum. Most cases can be managed with simple diverticulectomy without the need for resection and anastomosis.

1- Baldisserotto M, Maffazzoni DR, Dora MD: Sonographic findings of Meckel's diverticulitis in children. AJR Am J Roentgenol. 180(2):425-8, 2003
2- Bennett GL, Birnbaum BA, Balthazar EJ: CT of Meckel's diverticulitis in 11 patients. AJR Am J Roentgenol. 182(3):625-9, 2004
3- Hamada T, Tanaka M, Hashimoto Y, Yamauchi M, Shigeoka N, Nakai K, Suenaga K: Contrast-enhanced sonographic findings of gangrenous meckel diverticulitis. J Ultrasound Med. 25(9):1227-31, 2006
4- Huang CC, Lai MW, Hwang FM, Yeh YC, Chen SY, Kong MS, Lai JY, Chen JC, Ming YC: Diverse presentations in pediatric Meckel's diverticulum: a review of 100 cases. Pediatr Neonatol.55(5):369-75, 2014
5- Alemayehu H, Stringel G, Lo IJ, Golden J, Pandya S, McBride W, Muensterer O: Laparoscopy and complicated meckel diverticulum in children. JSLS. 18(3), 2014
6- Park JS, Lim CW, Park T, Cho JM, Seo JH, Youn HS: Suppurative meckel diiverticulum in a 3-year-old girl presenting with periumbilical cellulitis. Pediatr Gastroenterol Hepatol Nutr. 18(1):66-70, 2015

PSU Volume 47 NO 03 SEPTEMBER 2016

Vascular complications of CVC

Central venous catheters (CVC) are essential for hemodynamic monitoring, rapid volume resuscitation, intravenous drug therapy, chemotherapy, parenteral hyperalimentation and hemodialysis among other uses. Percutaneous punctured of the subclavian,  internal jugular (IJV) or a femoral vein is the main approach to position the catheter tip in the superior/inferior vena cava. Percutaneous puncture of either veins can also accidentally puncture the nearby artery such as the subclavian, carotid or even vertebral artery. The incidence of arterial puncture during IJV cannulation is approximately 6%. As a consequence the patient can develop life threatening hemorrhage, stroke, pseudoaneurysm, arteriovenous fistula, embolism, thrombus, dissection or other compressive manifestation. Pulling a large-bore catheter from an artery and applying pressure is the general acceptable management in patients that are not anticoagulated so long as the artery is accessible to manual compression. If bleeding is not controlled by external pressure, then endovascular or surgical intervention should be considered. Surgical exploration is the safest and most conservative approach to managing arterial misplacement of catheters especially when the catheter enters the artery in a location where external compression may not be effective or if the arterial trauma occurs with a large-caliber catheter. Endovascular treatment appears to be safe for the management of arterial injuries that are difficult to expose surgically, such as those below or behind the clavicle. Stroke is a devastating symptom associated with accidental placement of the catheter in an artery usually the result of injury to a disease artery with embolization of a plaque or due to an embolizing dislodge clot. Use of ultrasound for placement of CVC reduces significantly these iatrogenic complications. Inadvertent arterial puncture rates are significantly lowered by the use of ultrasound. 

1- Parikh S, Narayanan V: Misplaced peripherally inserted central catheter: an unusual cause of stroke. Pediatr Neurol. 30(3):210-2, 2004
2- Shah PM, Babu SC, Goyal A, Mateo RB, Madden RE: Arterial misplacement of large-caliber cannulas during jugular vein catheterization: case for surgical management. J Am Coll Surg. 198(6):939-44, 2004
3-  Guilbert MC, Elkouri S, Bracco D, Corriveau MM, Beaudoin N, Dubois MJ, Bruneau L, Blair JF: Arterial trauma during central venous catheter insertion: Case series, review and proposed algorithm.  J Vasc Surg. 48(4):918-25, 2008
4- Van Vrancken MJ, Guileyardo J: Vertebral artery thrombosis and subsequent stroke following attempted internal jugular central venous catheterization. Proc (Bayl Univ Med Cent). 25(3):240-2, 2012
5- Bechara CF, Barshes NR, Pisimisis G, Kougias P, Lin PH: Management of inadvertent carotid artery sheath insertion during central venous catheter placement. JAMA Surg. 148(11):1063-6, 2013
6- Malbezin S, Gauss T, Smith I, et al: A review of 5434 percutaneous pediatric central venous catheter inserted by anesthesiologists. Pediatr Anest 23: 974-979, 2013


Pneumoscrotum is very rare and refers to the presence of gas within the scrotal sac of males. Pneumoscrotum includes scrotal emphysema as well as pneumatocele. Scrotal emphysema is palpable and shows signs of scrotal swelling and crepitus, while pneumatocele is not palpable because the air present within the tunica vaginalis of the testis. There are three ways to explain the presence of air in the scrotum: subcutaneous or retroperitoneal air that dissects into the Dartos lining of the scrotal wall, local gas production (gas gangrene) or air introduction, and movement of air from the intraperitoneal space into the scrotum. As such the etiology of pneumoscrotum can include pneumomediastinum, tension pneumothorax from thoracic trauma or spontaneous, pneumoperitoneum, Fournier gangrene, bowel perforation from instrumentation such as colonoscopy, spontaneous or sick bowel. Traumatic and iatrogenic causes accounts for most cases of pneumoscrotum. Endoscopic colonic procedures and abdominal endoscopy accounts are the main causes of iatrogenic pneumoscrotum. Newborns present a high incidence of pneumoscrotum due to gastric and bowel perforation. Causes are various:  congestion of the bowel wall secondary to asphyxia or septicemia, trauma coincident with delivery, excessive gastric acidity, direct or indirect mechanical injury from gavage tubes or resuscitation maneuver, congenital mural defects of the gastroenteric tract, meconium stasis, rupture of a diverticulum, and coincident central nervous system abnormalities. Most clinical pneumoscrotum follows a benign course and can be managed conservatively with observation and antibiotics. Nonsurgical treatment is chosen because of the delayed presentation, lack of abdominal and perineal pain and clinical stability of the patient.

1- Fu KI, Sano Y, Kato S, Fujii T, Sugito M, Ono M, Saito N, Kawashima K, Yoshida S, Fujimori T: Pneumoscrotum: a rare manifestation of perforation associated with therapeutic colonoscopy. World J Gastroenterol. 28;11(32):5061-3, 2005
2- Singh S, Thakur M: Pneumoscrotum after colonoscopy. Can J Gastroenterol. 22(4):411-3, 2008
3- Khan YA, Akhtar J: Pneumoscrotum: a rare presentation of gastric perforation in a neonate. APSP J Case Rep. 1(2):15, 2010
4- Lostoridis E, Gkagkalidis K, Varsamis N, Salveridis N, Karageorgiou G, Kampantais S, Tourountzi P, Pouggouras K: Pneumoscrotum as complication of blunt thoracic trauma: a case report. Case Rep Surg. 2013:392869, 2013
5- Mehraeen R, Osia S: A case of pneumoscrotum following spontaneous colonic perforation and mimicking strangulated inguinal hernia. Iran J Pediatr. 24(1):116-7. 2-14
6- Cochetti G, Barillaro F, Cottini E: Pneumoscrotum: report of two different cases and review of the literature. Ther Clin Risk Manag. 11:581-7, 2015

Hyalinizing Trabecular Thyroid Tumor

Hyalinizing trabecular tumor (HTT) is a rare and controversial tumor of the thyroid gland with uncertain malignant potential frequently misdiagnosed and managed as other thyroid neoplasm due to the similar morphology mimicking papillary thyroid carcinoma and medullary thyroid carcinoma.  Some pathologists believe that HTT is a variant of papillary thyroid carcinoma while others believe it to be an independent neoplasm. HTT has a characteristic trabecular growth pattern and hyalinizing stroma. The overwhelming majority of HTT behaves as benign neoplasms. Malignant potential occurs when there is vascular, capsular and/or parenchymal invasion, local recurrence or distant metastasis. The problem aggravates when FNA is utilized since the features of hypercellularity and grooves, pseudoinclusions and hyperchromaticity of the nuclei which are the main diagnostic features of HTT can also be observed in patients with classic papillary carcinoma. US features of HTT are marked hypoechogenicity, absence of calcifications, parallel shape and presence of vascularity. Surgeons should be aware that the preoperative cytological or frozen section diagnosis may not necessarily agree with the final pathological diagnosis due to the overlapping nature between HTT and PTC. Frozen section is not always diagnostic of HTT. This difficulty with the pathologic diagnosis using FNA can result in overtreatment of a universally benign disease. This overtreatment can occur in 44-71% of patients harboring a HTT.  HTT arises in glands that harbor chronic Hashimoto thyroiditis and multinodular goiter. Grossly HTT are well circumscribe or encapsulated with a color ranging from yellow to tan. It is recommended to perform immunohistochemical stains, at least Ki-67 and Cytokeratin-19 reaction to correctly identified HTT. The prognosis of HTT is excellent and management should consist of thyroidectomy of the affected gland lobe alone.

1- Li J, Yang GZ, Gao LX, Yan WX, Jin H, Li L: Hyalinizing trabecular tumor of the thyroid: Case report and review of the literature. Exp Ther Med. 3(6):1015-1017, 2012
2- Howard BE, Gnagi SH, Ocal IT, Hinni ML: Hyalinizing trabecular tumor masquerading as papillary thyroid carcinoma on fine-needle aspiration. ORL J Otorhinolaryngol Relat Spec. 75(6):309-13, 2013
3- Sung SY, Shen HY, Hsieh CB, Duh QY, Su TF, Chan DC, Shih ML: Hyalinizing trabecular tumor of thyroid: does frozen section prevent unnecessarily aggressive operation? Six new cases and a literature review. J Chin Med Assoc. 77(11):573-7, 2014
4- Barsu C, Barsu M: Medico-historical overview and histopathological coments about a hyalinizing trabecular tumor case of thyroid gland. Rom J Morphol Embryol 55(3): 989-992, 2014
5- Riaz S, Bashir H, Jahangir S, Nawaz MK: Hyalinizing trabecular neoplasm of thyroid.  J Ayub Med Coll Abbottabad. 26(3):410-2, 2014
6- Jang H, Park CK, Son EJ, Kim EK, Kwak JY, Moon HJ, Yoon JH: Hyalinizing trabecular tumor of the thyroid: diagnosis of a rare tumor using ultrasonography, cytology, and intraoperative frozen sections. Ultrasonography. 35(2):131-9, 2016

PSU Volume 47 NO 04 OCTOBER 2016

Prepubertal Testicular Tumors

Testicular tumors presenting before puberty in male patients occur very rarely and very distinct from the adult counterpart. Prepubertal testicular tumors are usually of one histologic type. The most common prepubertal testicular tumor is yolk sac tumor followed by teratoma. Median age at surgery is 17 months and most children present with a solid scrotal painless mass. This must be followed by Ultrasound and tumor markers. The most common type of malignant tumor is yolk sac (62%) and the most common benign tumor is a mature teratoma (23%). Yolk sac histology is more common in Asian/Pacific population, as compared with white American where teratoma predominates. In yolk sac tumor the epithelial lining of the cysts and tubercles secretes high concentration of alpha-fetoprotein (AFP), a tumor marker. AFP is both important in the diagnosis and follow-up of such tumors, especially for recurrence after treatment. Elevation of AFP can also occur in benign teratomas and normal infants up to the age of two months. Most children with yolk sac tumors have clinical stage I disease, with age at presentation of 15 months and absence of metastatic disease. Clinical stage I disease is managed with radical orchiectomy using an inguinal approach. Yolk sac tumors metastasized through lymphatics and blood borne to lymphs nodes and lung. Stage II and higher stages are managed with retroperitoneal lymph node dissection, adjuvant chemotherapy and radiotherapy. In cases of teratoma, the age at operation is 12 months, and immature teratomas present earlier in life than matured teratomas. No child with a prepubertal testicular teratoma showed metastatic disease, regardless of the presence or absence of testicular-sparing surgery. It is proposed that any child with ultrasound showing salvageable normal testicular parenchyma and normal AFP should be managed with testis-sparing surgery.     

1- Agarwal PK, Palmer JS: Testicular and paratesticular neoplasms in prepubertal males.  J Urol. 176(3):875-81, 2006
2- Hisamatsu E, Takagi S, Nakagawa Y, Sugita Y, Yoshino K, Ueoka K, Tanikaze S: Prepubertal testicular tumors: a 20-year experience with 40 cases. Int J Urol. 17(11):956-9, 2010
3- Treiyer A, Blanc G, Stark E, Haben B, Treiyer E, Steffens J: Prepubertal testicular tumors: frequently overlooked.  J Pediatr Urol. 3(6):480-3, 2007
4- Nerli RB, Ajay G, Shivangouda P, Pravin P, Reddy M, Pujar VC: Prepubertal testicular tumors: our 10 years experience. Indian J Cancer. 47(3):292-5, 2010
5- Baik K, Kang M, Park K, Choi H: Prepubertal Testicular Tumors in Korea: A Single Surgeon's Experience of More Than 20 Years. Korean J Urol. 54(6):399-403, 2013
6- Akiyama S, Ito K, Kim WJ, Tanaka Y, Yamazaki Y: Prepubertal testicular tumors: a single-center experience of 44 years. J Pediatr Surg. 51(8):1351-4, 2016

Prenatal Diagnosis Esophageal Atresia

Esophageal atresia (EA) with or without tracheoesophageal fistula is the most common congenital anomaly of the esophagus in children. Prenatal diagnosis of EA relies on the indirect findings in routine sonography at 16-20 weeks gestation of a small or non-visualized stomach bubble in conjunction with subsequent maternal polyhydramnios after 24 weeks. These US findings are nonspecific and can be transient in nature creating unnecessary anxiety in the expectant mother and relatives. Two other more direct signs of the US diagnosis of EA include a dilated proximal esophageal pouch (upper pouch sign) and failure to visualize the entire thoracic esophagus. Using all these findings in US the rate of diagnosing  EA prenatally occurs one-third of the cases. Most cases of EA that are diagnosed prenatally are the long-gap esophageal variety without tracheoesophageal fistula which occur with an overall incidence of 8%. The most common variant of EA with distal tracheoesophageal fistula is missed most of the time since it decompresses through the fistulous tract into the stomach and filling it preventing the development of polyhydramnios. Prenatal diagnosis of EA gives us the opportunity for optimal perinatal management with delivery of the fetus in a tertiary care pediatric center with expertise in esophageal surgery.  Fetal MRI and biochemistry of the amniotic fluid can help confirm the diagnosis since gamma glutamyl transpeptidase (GGTP) and alpha-protein (AFP) in amniotic fluid are elevated in EA. The dynamic sequence of MRI during fetal swallowing is needed to increase the diagnostic yield. Since 50% of EA children have an associated malformation these must be looked carefully to correlate with the diagnosis of VACTERL, CHARGE, abnormal karyotype or other associated syndromes dictating prognosis.

1- Garabedian C, Verpillat P, Czerkiewicz I, Langlois C, Muller F, Avni F, Bigot  J, Sfeir R, Vaast P, Coulon C, Subtil D, Houfflin-Debarge V: Does a combination of ultrasound, MRI, and biochemical amniotic fluid analysis improve prenatal diagnosis of esophageal atresia? Prenat Diagn. 34(9):839-42, 2014
2- Ethun CG, Fallon SC, Cassady CI, Mehollin-Ray AR, Olutoye OO, Zamora IJ, Lee TC, Welty SE, Cass DL: Fetal MRI improves diagnostic accuracy in patients referred to a fetal center for suspected esophageal atresia. J Pediatr Surg. 49(5):712-5, 2014
3- Kunisaki SM, Bruch SW, Hirschl RB, Mychaliska GB, Treadwell MC, Coran AG: The diagnosis of fetal esophageal atresia and its implications on perinatal outcome. Pediatr Surg Int. 30(10):971-7., 2014
4- Garabedian C, Sfeir R, Langlois C, et al: Does prenatal diagnosis modify neonatal treatment and early outcome of children with esophageal atresia? Am J Obstet Gynecol. 212(3):340, 2015
5- Spaggiari E, Faure G, Rousseau V, et al: Performance of prenatal diagnosis in esophageal atresia. Prenat Diagn. 35(9):888-93, 2015
6- Bradshaw CJ, Thakkar H, Knutzen L, Marsh R, Pacilli M, Impey L, Lakhoo K: Accuracy of prenatal detection of tracheoesophageal fistula and oesophageal atresia. J Pediatr Surg. 51(8):1268-72, 2016

Aberrant Subclavian Artery

Aberrant subclavian artery, also known as arteria lusoria, is the most common form of aortic arch vascular anomaly. It results from regression of the right 4th aortic arch between the carotid and subclavian arteries. The right subclavian artery usually persists as a branch from the descending aorta distal to the takeoff of the left subclavian artery and coursing posterior to the esophagus, though it can also pass between the esophagus and the trachea or even anterior to the trachea very rarely. Patients with an aberrant subclavian artery can develop symptoms which include dysphagia, cough, stridor, regurgitation, asphyxia induced by feeding, a globus sensation (or lump in the throat), failure to thrive and thoracic pain, though most patients with this anomaly remain asymptomatic throughout their lifetime. Infants can present with respiratory symptoms due to dysphagia and aspiration of food particles. Some asymptomatic patients can elicit symptoms during exercise. Barium swallow imaging shows a characteristic diagonal posterior compression defect at the level of the 3rd and 4th vertebrae which is diagnostic. CT or MRI angiography and transthoracic echocardiogram confirms the diagnosis. Symptomatic children should undergo surgical management. The goal of operative repair is relieving the symptoms and restores circulation. This is done by reimplanting the aberrant subclavian artery into the ascending aorta or the right common carotid artery directly through a right thoracotomy in children. Anatomic variations that are associated with an aberrant subclavian artery include abnormal origin of the right vertebral artery from the aorta or from the right common carotid artery, the presence of a c common carotid trunk, a right-sided thoracic duct and a nonrecurrent laryngeal nerve.   

1- Atay Y, Engin C, Posacioglu H, Ozyurek R, Ozcan C, Yagdi T, Ayik F, Alayunt EA: Surgical approaches to the aberrant right subclavian artery.  Tex Heart Inst J. 33(4):477-81, 2006
2- Jan SL, Lin SJ, Fu YC, Tsai IC, Chan SC, Lin MC: Effect of exercise on asymptomatic children with an isolated aberrant subclavian artery. Acta Cardiol. 65(2):231-7, 2010
3- Shinkawa T, Greenberg SB, Jaquiss RD, Imamura M: Primary translocation of aberrant left subclavian artery for children with symptomatic vascular ring. Ann Thorac Surg. 93(4):1262-5, 2012
4- Derbel B, Saaidi A, Kasraoui R, Chaouch N, Aouini F, Ben Romdhane N, Manaa J: Aberrant right subclavian artery or arteria lusoria: a rare cause of dyspnea in children. Ann Vasc Surg. 26(3):419, 2012
5- Kir M, Saylam GS, Karadas U, Yilmaz N, et al: Vascular rings: presentation, imaging strategies, treatment, and outcome. Pediatr Cardiol. 33(4):607-17, 2012
6- Tashiro J, Malvezzi L, Kasi A, Burnweit CA: Chronic vomiting and recurrent pneumonia in an adolescent female. J Pediatr Surg. 49(11):1683-5, 2014

PSU Volume 47 NO 05 NOVEMBER 2016

Toxoplasma Lymphadenitis

Enlarged lymph nodes are a common problem seen in children of all ages. Lymphadenitis commonly represent a transient response to a benign local or generalized infection. The most common causes of subacute or chronic lymphadenitis in children include cat scratch disease, mycobacterial infection and toxoplasmosis. The diagnostic approach to a child with an adenopathy longer than six weeks includes serological assays, radiological studies (ultrasound), fine needle aspiration and most helpful complete excisional lymph node biopsy to establish a histologic diagnosis of malignancy or infection. Infestation with Toxoplasma gondii occurs frequently in children around the world. In immunocompetent host, toxoplasma primary infection produces little symptoms, is self-limiting and has a favorable prognosis without treatment. Less than 10% of infected children are symptomatic, with lymphadenopathy as the most frequent clinical manifestation. Symptoms associated with toxoplasmosis include asthenia, fever and nonspecific such as headache, myalgia or arthralgia. Acute lymphadenopathy usually occurs in the head and neck region, followed by supraclavicular, and inguinal sites. The lymph node is painless, solitary, not matted, with mild inflammation and do not suppurate. Toxoplasma lymphadenitis is most often diagnosed by lymph node biopsy and/or serological assays. Fine needle aspiration is rarely useful for the diagnosis since it does not permit evaluation of lymph node architecture. A negative Sabin-Feldman dye test in a lymphadenopathy with more than three weeks evolution excludes toxoplasma as an etiologic agent. The Sabin-Feldman dye and IgM-ISAGA tests is positive in most patients with toxoplasmosis within the first three months after infestation. Observation is all needed for single toxoplasma lymphadenitis. Co-trimoxazole (TSM) is a good therapeutic agent for cerebral or ocular toxoplasmosis. 

1- Ridder GJ, Boedeker CC, Lee TK, Sander A: B-mode sonographic criteria for differential diagnosis of cervicofacial lymphadenopathy in cat-scratch disease and toxoplasmosis. Head Neck. 25(4):306-12, 2003
2- Leung AK, Robson WL: Childhood cervical lymphadenopathy. J Pediatr Health Care. 18(1):3-7, 2004
3- Durlach RA, Kaufer F, Carral L, Hirt J: Toxoplasmic lymphadenitis - clinical and serological profile. Clin Microbiol Infect 9: 625-631, 2003
4- Montoya JG, Berry A, Rosso F, Remington JS: The differential agglutination test as a diagnostic aid in cases of toxoplasmic lymphadenitis. J Clin Microbiol. 45(5):1463-8, 2007
5- Alavi SM, Alavi L: Treatment of toxoplasmic lymphadenitis with co-trimoxazole: double-blind,
randomized clinical trial. Int J Infect Dis. 14 Suppl 3:e67-9, 2010
6- Guneratne R, Mendis D, Bandara T, Fernando SD: Guneratne R(1), Mendis D, Bandara T, Fernando SD. BMC Pediatr. 11:44, 2011

Electrocautery Injury

Bovie designed the first surgical diathermy machine in 1928 to facilitate tumor removal and hemostasis in neurosurgery. The electrocautery involves the passage of electrical current through the body to burn the tissue at the active electrode tip. The current (or flow of electrons) excites tissue molecules producing heat. For cutting intracellular water boils, cells explode and tissue divides. At lower temperatures the heat causes dell dying and blood protein is coagulated causing hemostasis. There are two diathermy modes: monopolar and bipolar. In monopolar the current enters the patient through the small area active electrode and exits safely through the large area neutral grounding pad electrode. This circuit can cause unintended high frequency current burn injury to the patient if not used properly. Bipolar diathermy is safer than monopolar as the current passes between the two prongs of the electrode without significant flow through the patient. A neutral electrode is not required. Advantage of bipolar diathermy is reduction of tissue damage. Electrocautery injury can occur in the form of burn, electrocution, operating room fire, smoke inhalation and gene mutation. Iatrogenic cautery burns can occur from direct contact to the active electrode resting on the patient skin, burns at the site of the grounding electrode, burns from electrode heating of pooled solutions such as spirits, and burns outside the operative field due to an alternate grounding source. Most burns occur due to faulty application of the grounding pad failing to have good contact with the patient skin. An electrocautery injury is a medical error that has medicolegal and ethical implications. The improper use of energy devices may increase patient morbidity and mortality.   

1- Sudhindra TV, Joseph A, Hacking CJ, Haray PN: Are surgeons aware of the dangers of diathermy? Ann R Coll Surg Engl 82: 31-32, 2000
2- M. Saaiq, S. Zaib, S. Ahmad: Electrocautery burns: experience with three cases and review of literature. Ann Burns Fire Disasters. 31; 25(4): 203-206. 2012
3- Sankaranarayanan G, Resapu RR, Jones DB, Schwaitzberg S, De S: Common uses and cited complications of energy in surgery. Surg Endosc 27(9): 3056-3072, 2013
4- Kapil Gupta, GV Prem Kumar, Abishek Bansal, Yatin Mehta: Burn injury by displacement of electrocautery plate. Indian J Anaesth. 55(6): 634-635, 2011
5-Ibrahim Alkatout, Thoralf Schollmeyer, Nusrat A. Hawaldar, Nidhi Sharma, Liselotte Mettler: Principles and Safety Measures of Electrosurgery in Laparoscopy. JSLS. 16(1): 130-139, 2012
6-  Guclu Kaan Beriat, Sefik Halit Akmansu, Hande Ezerarslan, Cem Dogan, Unsal Han, Mehmet Saglam, Oytun Okan Senel, Sinan Kocaturk: The comparison of thermal tissue injuries caused by ultrasonic scalpel and electrocautery use in rabbit tongue tissue. Bosn J Basic Med Sci. 12(3): 151-157, 2012

Splenic Cysts Sclerosis

Splenic cysts are rare in children. Most cases (75%)  are labeled secondary or ‘pseudocysts', the result of blunt trauma representing a late manifestation of posttraumatic intrasplenic or subcapsular hematoma formation.  True primary nonparasitic splenic cysts are extremely rare. Splenic cysts can attain large sizes before they cause symptoms. Clinically splenic cysts present with left upper quadrant abdominal pain or gastric fullness depending on their size. Diagnosis is made with abdominal US  or CT-Scan. They should be managed because of chronic symptoms and the risk of rupture. Symptomatic cysts were originally managed with splenectomy. With the advent of spleen-preserving procedures and the use of laparoscopy the standard care has changed to total cystectomy with partial splenectomy or partial decapsulation of the cyst preserving a significant mass of the spleen.  Another alternative management of splenic cysts consists of US or CT-guided percutaneous drainage followed by sclerotherapy through an inlaying catheter. Several sclerosing agents such as alcohol, formalin, phenol, Pantopaque, doxycycline or tetracycline has been utilized as sclerosing agent during these percutaneous procedures. Results have varied with the used of the sclerotic agent. The most common used sclerotic agent is alcohol since is wide available, high efficient and ease to use. Image-guided sclerotherapy works on the principle of protein denaturation, cell death and fibrosis in the wall of the cyst. The main reason for failure using sclerotic agents occurs when they fail to cover completely the surface of the cyst leaving cells along the cyst wall which cause continuation of fluid secretion and cyst recurence. Retreatment of splenic cysts managed with sclerotherapy is more often necessary than after using this technique with renal or liver cysts. Only in 20% will the cyst disappear completely. Complications associated with sclerosis consist of vasovagal reactions, shoulder pain, free intraabdominal bleeding, bleeding into the cyst cavity, chemical peritonitis and cyst infection. Randomized control trial is needed to compare surgery with sclerosis.

1- Singh AK, Shankar S, Gervais DA, Hahn PF, Mueller PR: Image-guided percutaneous splenic interventions. Radiographics. 32(2):523-34, 2012
2- Goktay AY, Secil M, Ozcan MA, Dicle O: Percutaneous treatment of congenital splenic cysts: drainage and sclerotherapy with polidocanol. Cardiovasc Intervent Radiol. 29(3):469-72, 2006
3- Rifai K, Berger D, Potthoff A, Manns MP, Gebel MJ: Fine needle sclerotherapy as a new effective therapeutic approach for nonparasitic splenic cysts: a case series. Dig Liver Dis. 45(7):595-9, 2013
4- Moir C, Guttman F, Jequier S, Sonnino R, Youssef S: Splenic cysts: aspiration, sclerosis, or resection. J Pediatr Surg. 24(7):646-8, 1989
5- Aon R, Guijarro J, Amoros C, Gil J, Bosca MM, Palmero J, Benages A: Congenital splenic cyst treated with percutaneous sclerosis using alcohol. Cardiovasc Intervent Radiol. 2006 Jul-Aug;29(4):691-3, 2006
6- Accinni A, Bertocchini A, Madafferi S, Natali G, Inserra A: Ultrasound-guided percutaneous sclerosis of congenital splenic cysts using ethyl alcohol 96% and minocycline hydrochloride 10%: A pediatric series. J Pediatr Surg. 51(9):1480-4, 2016

PSU Volume 47 No 06 DECEMBER 2016

Undrained Traumatic Hemothorax

Hemothorax refers as blood in the pleural cavity with the pleural fluid hematocrit being 50% or more of the peripheral blood hematocrit. Hemothorax  results after blunt or penetrating trauma to the chest. Spontaneous hemothorax is rare, but can be seen after anticoagulant therapy, pulmonary embolism and pleural malignancy. Emergent management of hemothorax includes management of the associated hemorrhagic shock along with chest tube thoracostomy which in most instances can resolve the problem and expand the compressed lung. Chest tube drainage produces apposition of the pleural surfaces with tamponade of the bleeding vessels, expansion of lung parenchyma and tamponade of lung vessels and drainage of the partially clotted blood. In 5-30% of cases residual hemothorax persists due to clotting of blood within the chest. Up to 40% of these patients will require further surgical intervention for non-resolving, complicated intrapleural collections, empyema or fibrothorax development. A second chest tube is an inadequate alternative in retained hemothorax where initial tube thoracotomy is insufficient. Alternatives of management include open thoracotomy, video-assisted thoracoscopic surgery (VATS), or intrapleural fibrinolysis using streptokinase. Decision making should be based on thoracic CT findings and not simple chest films. VATS is the best available modality for the management of clotted hemothorax as it can clear the chest cavity in 80% of cases avoiding the use of an open thoracotomy. VATS can cause complications in 10% of patients such as transient hypoxemia, arrhythmia, intercostal neuritis, chest wall bleeding or iatrogenic lung injury. Another available alternative that has gained wide world acceptance is intrapleural fibrinolytic therapy using streptokinase or urokinase with a success rate of 90%. The use of intrapleural streptokinase does not cause significant fibrinolysis and is unlikely to cause systemic bleeding. Fibrinolytic agents appear to have a role in managing retained hemothorax with significant clinical and radiological improvement and should be used as initial management of retained hemothorax.    

1- Agarwal R, Aggarwal AN, Gupta D: Intrapleural fibrinolysis in clotted haemothorax. Singapore Med J. 47(11):984-6, 2006
2- Hunt I, Thakar C, Southon R, Bedard EL: Establishing a role for intra-pleural fibrinolysis in managing traumatic haemothoraces. Interact Cardiovasc Thorac Surg. 8(1):129-33, 2009
3- Vassiliu P, Velmahos GC, Toutouzas KG: Timing, safety, and efficacy of thoracoscopic evacuation of undrained post-traumatic hemothorax. Am Surg. 67(12):1165-9, 2001
4- Velmahos GC(1), Demetriades D, Chan L, Tatevossian R, Cornwell EE 3rd, Yassa N, Murray JA, Asensio JA, Berne TV: Predicting the need for thoracoscopic evacuation of residual traumatic
hemothorax: chest radiograph is insufficient.  J Trauma. 46(1):65-70. 1999
5- Kumar S, Rathi V, Rattan A, Chaudhary S, Agarwal N: VATS versus intrapleural streptokinase: A prospective, randomized, controlled clinical trial for optimum treatment of post-traumatic Residual Hemothorax. Injury. 46(9):1749-52, 2015
6- Kimbrell BJ, Yamzon J, Petrone P, Asensio JA, Velmahos GC: Intrapleural thrombolysis for the management of undrained traumatic hemothorax: a prospective observational study. J Trauma. 62(5):1175-8, 2007

Growing Teratoma Syndrome

Ovarian or testicular teratomas are either mature (most commonly), immature or malignant. The immature and malignant teratomas can secrete alpha fetoprotein (AFP) and/or human chorionic gonadotropin (HCG). Immature teratomas are potentially malignant and as such will need chemotherapy to change the features of immaturity into mature teratoma and reduce the level of tumor markers. Teratomas that increase in size during or after chemotherapy as tumor marker levels decrease is known as growing teratoma syndrome (GTS). By definition GTS includes normalization of previously elevated serum tumors markers (AFP or HCG), an increase in tumor size during or after chemotherapy given for non-seminomatous germ cell tumor and an absence of such components other than mature teratoma at resection. GTS is characterized by an absence of malignant germ cell components as the growing tissue is benign. Further chemotherapy is unable to shrink GTS. The radiological features include increased density of mass with well-circumscribed margins, onset of internal calcification with fatty areas and cystic changes. Retroperitoneum is the most common site for GTS. Pathogenesis of development of GTS is either malignant cell differentiation into mature teratoma or selective chemotherapy induced destruction of immature elements. Complete surgical excision of the mass is required to avoid pressure effects and potential malignant transformation to either sarcoma or carcinoma. Pressure effect of the growing tumor includes vascular thrombosis, ureteral obstruction, bowel obstruction, bile duct obstruction and fecal fistula. Malignant transformation to sarcoma, adenocarcinoma or PNET is reported in 3% of cases. Alpha-2-Interferon can control disseminated unresectable GTS by inhibiting tumor angiogenesis mediated by decreased level of vascular endothelial growth factor and basic fibroblast growth factor, but the regression is slow, incomplete and discontinuation results in progression of disease. Prognosis after complete surgical resection is excellent.

1- Hsieh YL, Liu CS: Progression from an immature teratoma with miliary gliomatosis peritonei to
growing teratoma syndrome with nodular gliomatosis peritonei. Pediatr Neonatol. 50(2):78-81, 2009
2- Sengar AR, Kulkarni JN: Growing teratoma syndrome in a post laparoscopic excision of ovarian immature teratoma.  J Gynecol Oncol. 21(2):129-31, 2010
3- Li S, Liu Z, Dong C, Long F, Liu Q, Sun D, Gao Z, Wang L: Growing Teratoma Syndrome Secondary to Ovarian Giant Immature Teratoma in an Adolescent Girl: A Case Report and Literature Review. Medicine (Baltimore). 95(7):e2647, 2016
4- Daher P, Riachy E, Khoury A, Raffoul L, Ghorra C, Rehayem C: Growing teratoma syndrome: first case report in a 4-year-old girl. J Pediatr Adolesc Gynecol. 28(1):e5-7, 2015
5- Zagama L, Pautier P, Duvillard P, Castaigne D, Patte C, Lhomma C: Growing teratoma syndrome after ovarian germ cell tumors. Obstet Gynecol. 108(3 Pt 1):509-14, 2006
6- Tangjitgamol S, Manusirivithaya S, Leelahakorn S, Thawaramara T, Suekwatana P, Sheanakul C: The growing teratoma syndrome: a case report and a review of the literature. Int J Gynecol Cancer. 16 Suppl 1:384-90, 2006
7- Nimkin K, Gupta P, McCauley R, Gilchrist BF, Lessin MS: The growing teratoma syndrome. Pediatr Radiol. 34(3):259-62, 2004

Epiploic Appendagitis

Epiploic appendages are peritoneum-covered fat outpouches protruding from  the serosal antimesenteric border of the taeniae of the large bowel, except in the rectum. Blood supply of the epiploic appendages is derived from a single artery and vein located within the pedicle. Epiploic appendagitis occur when there occurs either torsion and/or infarction of the appendage. Epiploic appendagitis is an uncommon cause of acute abdominal pain in children and adults manifesting most commonly in the fourth or fifth decade of life with male predominance. Mostly epiploic appendagitis involve the sigmoid colon and the pain can be mistaken for diverticulitis. When it involves the cecum it can mimics appendicitis. With the widespread use of CT-Scan in the diagnosis of abdominal pain in children, epiploic appendagitis is commonly diagnosed before operation is undertaken for an acute abdomen. In US the appendagitis shows a noncompressible hyperechoic mass near the colonic wall at the point of maximum tenderness, absence of changes in the colon wall and absence of color flow on Doppler. CT-Scan findings include an oval lesion with attenuation similar to fat surrounded bu a hyperattenuated ring located near but distinct to the colon, inflammatory changes in the surrounding fat and absence of other abnormalities. The presence of a central hyperdense dot thought to represent a thrombosed vein to the epiploic appendix is a specific sign felt to distinguish epiploic appendagitis from omental torsion. MRI findings of epiploic appendagitis include an oval-shaped lesion, usually one to 4 cm in size, with high signal intensity center and low signal intensity rim on T1-weighted images. Obesity seems a risk factor. If the diagnosis of epiploic appendagitis is made preoperative with certain degree of confidence management can be conservative using pain killers. Most children recover in ten days. If the diagnosis is uncertain then laparoscopy has been found to be effective in diagnosis and management of epiploic appendagitis.  

1- Fraser JD, Aguayo P, Leys CM, St Peter SD, Ostlie DJ: Infarction of an epiploic appendage in a pediatric patient. J Pediatr Surg. 44(8):1659-61, 2009
2- Rashid A, Nazir S, Hakim SY, Chalkoo MA: Epiploic appendagitis of caecum: a diagnostic dilemma. Ger Med Sci. 10: 1612-3174, 2012
3- Toprak H, Yildiz S, Kilicarslan R, Bilgin M: Epiploic appendagitis. JBR-BTR. 97(3):174-5, 2014
4- Cho MS, Hwang-Bo S, Choi UY, Kim HS, Hahn SH: A case of epiploic appendagitis with acute gastroenteritis. Pediatr Gastroenterol Hepatol Nutr. 17(4):263-5, 2014
5- Redmond P, Sawaya DE, Miller KH, Nowicki MJ: Epiploic Appendagitis: A Rare Cause of Acute Abdominal Pain in Children. Report of a Case and Review of the Pediatric Literature. Pediatr Emerg Care. 31(10):717-9, 2015
6- Boscarelli A, Frediani S, Ceccanti S, Falconi I, Masselli G, Casciani E, Cozzi DA: Magnetic resonance imaging of epiploic appendagitis in children. J Pediatr Surg.(on line)

Journal Club