VOLUME 18, 2002

Volume 18 No 01 JANUARY 2002

Spontaneous Pneumothorax

Most pneumothorax in children are the result of blunt/open chest trauma, mechanical ventilation (barotrauma), bronchial asthma or an infectious pulmonary process. Primary spontaneous pneumothorax (PSP) is rare in children with most cases seen in adolescent males with thin body habitus. Main presenting symptoms consist of chest pain, cough and shortness of breath. Recurrence is high in this older population of children. PSP is usually the result of: 1) a ruptured apical bleb or bullae in three-fourth cases, 2) destructive parenchymal disease (cystic fibrosis, AIDS), or 3) alveolar rupture due to proximal airway obstruction. Initial management consists of oxygen supplementation for small pneumothorax less than 15% with no tension physiology present. Chest tube drainage is needed for medium or large size pneumothorax. Recurrence or persistent pneumothorax is managed with video-assisted thoracoscopic surgery (VATS) by ablating with endoscopic stapling (Endo GIA), suturing or ligating using an endoloop technique the apical bullae followed by pleurodesis. Pleurodesis can be done chemically or surgically.  Chemical pleurodesis is achieved with such agents as talc, tetracycline, bleomycin or quinacrine instillation. Mechanical pleurodesis carries a lower recurrent rate and can be achieved by abrasion or electrocoagulation. Most common complication is persistent air leak. VATS is a fast, cost-effective method of treatment for PSP with less morbidity.

1- Poenaru D, Yazbeck S, Murphy S: Primary spontaneous pneumothorax in children. J Pediatr Surg  29(9):1183-5, 1994
2- Yim AP: Video-assisted thoracoscopic suturing of apical bullae. An alternative to staple resection in the management of primary spontaneous pneumothorax. Surg Endosc  9(9):1013-6, 1995
3- Wilcox DT, Glick PL, Karamanoukian HL, Allen JE, Azizkhan RG: Spontaneous pneumothorax: a single-institution, 12-year experience in patients under 16 years of age. J Pediatr Surg  30(10):1452-4, 1995
4- Bertrand PC, Regnard JF, Spaggiari L, Levi JF, Magdeleinat P, Guibert L, Levasseur P: Immediate and long-term results after surgical treatment of primary spontaneous pneumothorax by VATS. Ann Thorac Surg  61(6):1641-5, 1996
5- Cook CH, Melvin WS, Groner JI, Allen E, King DR: A cost-effective thoracoscopic treatment strategy for pediatric spontaneous pneumothorax. Surg Endosc  13(12):1208-10, 1999
6- Chan P, Clarke P, Daniel FJ, Knight SR, Seevanayagam S: Efficacy study of video-assisted thoracoscopic surgery pleurodesis for spontaneous pneumothorax. Ann Thorac Surg  71(2):452-4, 2001

Gastrointestinal Autonomic Nerve Tumor

Gastrointestinal Autonomic Nerve Tumor (GANT), also known as plexosarcoma, is a very rare recently described aggressive malignant spindle cell tumor that arises from the autonomic myenteric plexus of the bowel wall. Tumor mostly originates in the small intestine and stomach with a clinical course characterize by either local or distant metastases (liver). Histologically GANT is a low-grade, epithelioid or spindle-cell neoplasms that can be distinguished from the other gastrointestinal stromal tumors on the basis of its unique electron microscopy ultrastructural features. Distinction of GANT from other stromal tumors is not possible based on imaging studies. The tumor is solid and cystic, hemorrhagic, often transmural and usually involving omentum, mesentery and retroperitoneum. Signs and symptoms include abdominal pain, mass, gastrointestinal bleeding, perforation and anemia. Tumor can present with volvulus. In children they occur in females during adolescent years (mean age 12 years) mostly involving the stomach. Primary management consists of surgical resection which is curative, leaving chemotherapy for residual or recurrent tumor. Prognosis is good for younger patients.

1- Segal A, Carello S, Caterina P, Papadimitriou JM, Spagnolo DV: Gastrointestinal autonomic nerve tumors: a clinicopathological, immunohistochemical and ultrastructural study of 10 cases. Pathology  26(4):439-47, 1994
2- Kodet R, Snajdauf J, Smelhaus V: Gastrointestinal autonomic nerve tumor: a case report with electron microscopic and immunohistochemical analysis and review of the literature. Pediatr Pathol  14(6):1005-16, 1994
3- Rueda O, Escribano J, Vicente JM, Garcia F, Villeta R: Gastrointestinal autonomic nerve tumors (plexosarcomas). is A radiological diagnosis possible? Eur Radiol  8(3):458-60, 1998
4- Kerr JZ, Hicks MJ, Nuchtern JG, Saldivar V, Heim-Hall J, Shah S, Kelly DR, Cain WS, Chintagumpala MM: Gastrointestinal autonomic nerve tumors in the pediatric population: a report of four cases and a review of the literature. Cancer  85(1):220-30, 1999
5- Lee JR, Joshi V, Griffin JW Jr, Lasota J, Miettinen M: Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular identity with gastrointestinal stromal tumor. Am J Surg Pathol  25(8):979-87, 2001

Allgrove Syndrome

Achalasia, Alacrima & isolated Adrenal Insufficiency is also known as triple-A or Allgrove syndrome. The high degree of consanguinity usually identified supports an autosomal recessive mode of inheritance for this disorder. Alacrima is the earliest and most consistent clinical sign of Allgrove syndrome. All affected children shows esophageal dysmotility even in the absence of symptomatic dysphagia. The adrenal insufficiency is due to adrenocorticotropic hormone (ACTH) resistance. Other associated neurological abnormalities include autonomic, sensory, and upper- and lower-motor neuropathy, deafness, and mental retardation. Genetically the syndrome has been mapped to mutations in chromosome 12q13 region near the type II keratin gene cluster. Though most cases arise in children between the ages of two and eight years, a few cases originate during early adult years. Management for alacrima consists of artificial tears replacement. Adrenal insufficiency will need steroid replacement. Achalasia is treated with a modified Heller esophagomyotomy which can be done open or laparoscopically.  A few neurologically impaired children will need gastrotomy feedings.

1- Moore PS, Couch RM, Perry YS, Shuckett EP, Winter JS: Allgrove syndrome: an autosomal recessive syndrome of ACTH insensitivity, achalasia and alacrima. Clin Endocrinol (Oxf) 34(2):107-14, 1991
2- Stratakis CA, Lin JP, Pras E, Rennert OM, Bourdony CJ, Chan WY: Segregation of Allgrove (triple-A) syndrome in Puerto Rican kindreds with chromosome 12 (12q13) polymorphic markers. Proc Assoc Am Physicians  109(5):478-82, 1997
3- Huebner A, Yoon SJ, Ozkinay F, Hilscher C, Lee H, Clark AJ, Handschug K: Triple A syndrome--clinical aspects and molecular genetics. Endocr Res  26(4):751-9, 2000
4- Sandrini F, Farmakidis C, Kirschner LS, Wu SM, Tullio-Pelet A, Lyonnet S, Metzger DL, Bourdony CJ, Tiosano D, Chan WY, Stratakis CA: Spectrum of Mutations of the AAAS Gene in Allgrove Syndrome: Lack of Mutations in Six Kindreds with Isolated Resistance to Corticotropin. J Clin Endocrinol Metab  86(11):5433-7, 2001

Volume 18 No 02 FEBRUARY 2002

Pancreatic Cysts

Pancreatic cystic lesions are usually inflammatory pseudocyst (90%) or  neoplastic process (10%). Distinguishing between them is essential for appropriate surgical therapy. Non-inflammatory neoplastic cysts in children are very rare ductal lesions with a spectrum of histologic characteristics and favorable outcomes. Histologically they include retention cysts, lymphoepithelial cysts, papillary cystic tumors, benign serous cystadenoma, mucinous tumors and mucinous cystadenocarcinoma. Most reported cases occur in females during adolescent years. Mode of presentation includes mild upper abdominal pain and palpable mass. Clinical, radiographic and intraoperative frozen section are non-reliable methods in distinguishing the different types of pancreatic cysts. Preoperative cyst fluid obtained by US or CT-guided percutaneous aspiration can be analyzed for viscosity (mucoid, viscous, serous), chemical (amylase, lipase), tumor markers (CEA, CA 19-9, CA125) and cytology characteristics. High CEA levels (> 25 ng/ml) indicate that the cyst is either malignant or mucinous (premalignant) type. Higher levels of CA 19-9 suggest pseudocysts and serous cystadenomas. Very high CA 125 levels appear predictive of malignancy. Viscosity above 1.63 suggests mucinous tumors. Amylase and lipase content should be low in true pancreatic cysts. Cytology analysis is insensitive unless positive for tumor cells. When the nature of the pancreatic cyst cannot be definitively establish by the above methods surgical resection is indicated.

1- Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM, Shandling B: A 20-Year Review of Pediatric Pancreatic Tumor. J Pediatr Surg 27 (10): 1315-1317, 1992
2- Lewandrowski KB, Southern JF, Pins MR, Compton CC, Warshaw AL: Cyst Fluid Analysis in the Differential Diagnosis of Pancreatic Cysts. A Comparison of Pseudocysts, Serous Cystadenomas, Mucinous Cystic Neoplasms, and Mucinous Cystadenocarcinoma. Ann Surg 217 (1): 41-47, 1993
3- Sand JA, Hyoty MK, Mattila J, Dragorn JC, Nordback IH: Clinical assessment compared with cyst fluid analysis in the differential diagnosis of cystic lesions in the pancreas. Surgery 119 (3): 275-287, 1996
4- Ky A, Shilyansky J, Gerstle J, Taylor G, Filler RM, Grace N, Superina R: Experience with Papillary and Solid Epithelial Neoplasms of the Pancreas in Children. J Pediatr Surg 33(1): 42-44, 1998
5- Borgne JL, Calan L, Partensky C, et al: Cystadenomas and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999

Mucinous Cystadenoma

Mucinous cystadenoma (MCA) of the pancreas is a benign cystic tumor made of columnar mucin-producing epithelium with a premalignant potential rarely seen in children. Most cases are identified in teenage and young adult female patients. Abdominal pain and mass effect is the most common symptom when MCA develops. Mucinous cystadenomas grow to very large sizes, is often multilocular in imaging studies and can be associated with recurrent pancreatitis (due to cyst and duct communication). CT findings might include large cysts with septa, peripheral calcifications and solid intracystic components. Cysts development has an insidious onset not associated to trauma, a high incidence of biliary tract disease or alcoholism. More than 90% of these slow-growing cysts are located in the body and tail of the pancreas. Aspiration of MCA fluid shows low levels of pancreatic enzymes with high CEA, CA 19-9 tumor markers and M1 mucin antigen levels. Cytology evaluation can reveal mucin-containing cells. Due to premalignant potential of developing a cystadenocarcinoma management of MCA should include complete resection. Enucleation and median pancreatectomy carries a high rate of pancreatic fistula formation. Resection is curative.

1- Logan SE, Voet RL, Tompkins RT: The malignant potential of mucinous cysts of the pancreas. West J Med 136: 157-162, 1982
2- Grosfeld JL: Chapter 12 - Pancreatic Tumors, In Medad Schiller "Pediatric Surgery of the Liver, Pancreas and Spleen", WB Saunder Co, 1991, pag. 165, 186
3- Hammel PR, Forgue-Lafitte ME, Levy P, Voitot H, Vilgrain V, Flejou JF, Molas G, Gespach C, Ruszniewski P, Bernades P, Bara J: Detection of gastric mucins (M1 antigens) in cyst fluid for the diagnosis of cystic lesions of the pancreas.  Int J Cancer 74(3):286-90, 1997
4- Ooi LL, Ho GH, Chew SP, Low CH, Soo KC: Cystic tumours of the pancreas: a diagnostic dilemma. Aust N Z J Surg 68(12):844-6, 1998
5-  Borgne JL, Calan L, Partensky C, et al: Cystadenomas and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999
6- Sperti C, Pasquali C, Ferronato A, Pedrazzoli S: Median pancreatectomy for tumors of the neck and body of the pancreas. J Am Coll Surg. 190(6):715-6, 2000
7- Johnson PRV, Spitz L: Cysts and Tumors of the Pancreas. Semin Pediatr Surg 9(4): 209-215, 2000

Serous Cystadenoma

Serous cystadenoma (SCA) is a benign microcystic tumor of the pancreas composed of cuboidal epithelium very rarely seen in children. Predominantly seen in young females, this cystic tumor is asymptomatic in one-third of the cases. SCA has a microcystic appearance with cysts less than 2 cm in size with loculations most commonly found in the pancreatic body and tail as a single lesion. CT scan shows well-demarcated multilocular cysts with enhancement that might show central calcifications. SCA is associated with another extrapancreatic neoplasm in 20% of cases. Cytology and biochemical analysis of cyst fluid reveal glycogen-rich cells and very low CEA levels (less than five ng/ml). Oligocystic (large cyst) SCA is an even rarer variety that arise in the head of the pancreas usually presenting in infants as a palpable mass needing resection. Should the diagnosis of SCA be well-established conservative management (observation) can be performed with yearly ultrasound study safely. Otherwise, if the nature of the cystic lesion cannot be established, or complications develop (obstructive jaundice, pancreatic duct dilatation or portal hypertension) resection is the next step in management.

1-Johnson PR, Spitz L: Cysts and Tumors of the Pancreas. Semin Pediatr Surg 9(4): 209-215, 2000
2- Borgne JL, Calan L, Partensky C, et al: Cystadenomas and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999
3- Procacci C, Graziani R, Bicego E, Bergamo-Andreis IA, Guarise A, Valdo M, Bogina G, Solarino U, Pistolesi GF: Serous cystadenoma of the pancreas: report of 30 cases with emphasis on the imaging findings. J Comput Assist Tomogr 21(3):373-82, 1997
4- Ooi LL, Ho GH, Chew SP, Low CH, Soo KC: Cystic tumours of the pancreas: a diagnostic dilemma. Aust N Z J Surg 68(12):844-6, 1998

Volume 18 No 03 MARCH 2002


A pneumatocele is a benign air-containing cyst in the lung most commonly the result of Staphylococcus Aureus pneumonia. Other less common bacteria associated are Hemophilus Influenza, Pseudomonas Aeruginosa and Strep Pneumonia.  Pneumatoceles are most commonly seen in young children (almost 50% of cases are less than one-year of age) during the acute phase of the pneumonic process. In a low percentage of cases the pneumatocele can be the result of closed chest trauma. In the infectious pneumonic setting the inflammatory process causes necrosis and liquefaction of the lung parenchyma followed by air leak and subpleural dissection forming a thin-walled cyst. Fever and respiratory distress are the most common symptoms during initial presentation. Diagnosis is established with the help of simple chest-x-ray films. CT-Scan might be needed to differentiate between a congenital lung cyst or cystic adenomatoid malformation. Follow-up films will help determine if the pneumatocele is growing or not in size. Rapidly enlarging pneumatocele may need percutaneous catheter decompression. Surgery is indicated only if the child develops respiratory distress or the pneumatocele ruptures into the pleural space creating a tension pneumothorax, a bronchopulmonary fistula or an empyema. Fortunately most pneumatoceles gradually decrease in size and disappear after the acute pulmonary infection subsides in a period that may range between six weeks and six months.

1- Joosten KF, Hazelzet JA, Tiddens HA, Hazebroek FW, Dzoljic-Danilovic G, Neijens HJ, de Groot R: Staphylococcal pneumonia in childhood: will early surgical intervention lower mortality? Pediatr Pulmonol  20(2):83-8, 1995
2- Wu MH, Tseng YL, Lin MY, Lai WW: Surgical treatment of pediatric lung abscess. Pediatr Surg Int  12(4):293-5, 1997
3- Kogutt MS, Lutrell CA, Puyau FA, Tieman EK: Decompression of pneumatocele in a neonate by percutaneous catheter placement. Pediatr Radiol  29(6):488-9, 1999
4- Seo T, Ando H, Watanabe Y, Harada T, Ito F, Kaneko K, Mimura S: Acute respiratory failure associated with intrathoracic masses in neonates. J Pediatr Surg  34(11):1633-7, 1999
5- Schimpl G, Schneider U: Traumatic pneumatoceles in an infant: case report and review of the literature. Eur J Pediatr Surg 6(2):104-6, 1996


Computer-assisted surgery (robotics) has several advantages: the increase in three-dimensional accuracy, the reproducibility of repeated procedures, the increased precision of movements enhancing the skills of the surgeon and the unique ability to perform surgery over a distance. Two systems are actually in use today: the Da Vinci and Zeus systems. In image-guided procedures, robots use magnetic resonance and computed tomography image data to guide instruments to the treatment site. By increasing the image capabilities the surgeon can integrate and define the pathology, margins of resection, and vasculature during surgery. Experimental studies have showed that robotically reproduced laparoscopic maneuvers such as tying, suturing, dissection, clipping, and cautery, seemed to be as accurate and fast as maneuvers made without robotics.Some of the clinical applications of robotics surgery includes laparoscopic cholecystectomy and fundoplications, entero-enterostomy, cardiac surgery, port-wine laser ablative surgery, and urologic procedures to mention a few.  Image-guided robots can biopsy brain lesions with less damage to adjacent tissue and are routinely used to shape the femur to precisely fit prosthetic hip joint replacements. Robotic systems are also under development for coronary bypass, microsurgical procedures in ophthalmology and surgical training and simulation. Issues of physician acceptance, high costs, performance and safety remain to be addressed.

1- Marescaux J, Smith MK, Folscher D, Jamali F, Malassagne B, Leroy J: Telerobotic laparoscopic cholecystectomy: initial clinical experience with 25 patients. Ann Surg  234(1):1-7, 2001
2- Docimo SG, Moore RG, Adams J, Ben-Chaim J, Kavoussi LR: Early experience with telerobotic surgery in children. J Telemed Telecare  2 Suppl 1:48-50, 1996
3- Sung GT, Gill IS: Robotic laparoscopic surgery: a comparison of the DA Vinci and Zeus systems. Urology  58(6):893-8, 2001
4- Howe RD, Matsuoka Y: Robotics for surgery. Annu Rev Biomed Eng  1:211-40, 1999
5- Lomanto D, Cheah WK, So JB, Goh PM: Robotically assisted laparoscopic cholecystectomy: a pilot study. Arch Surg  136(10):1106-8, 2001
6- Chitwood WR Jr, Nifong LW, Chapman WH, Felger JE, Bailey BM, Ballint T, Mendleson KG, Kim VB, Young JA, Albrecht RA: Robotic surgical training in an academic institution. Ann Surg  234(4):475-84, 2001
7- Hollands CM, Dixey LN, Torma MJ: Technical assessment of porcine enteroenterostomy performed with ZEUS robotic technology. J Pediatr Surg  36(8):1231-3, 2001

Fibromyxoid Sarcoma

Fibromyxoid sarcoma (FS) is a distinctive indolent soft-tissue sarcoma very uncommonly seen in children. Children demonstrate the low grade variety. FS grows slowly and can metastasizes to distant organs, chiefly to the lungs. The tumor has been located in the thigh or inguinal area, in the shoulder, in the axilla-chest wall area, the perineum, the small bowel mesentery, the neck, and the buttocks. Size can vary between 3.5 cm and 25 cm (median 4.5 cm). Histologically the tumor demonstrates contrasting fibrous and myxoid areas, a swirling, whorled growth pattern and bland, deceptively benign-appearing fibroblastic spindle cells; cellularity is low to moderate, mitotic figures are uncommon, and nuclear pleomorphism is usually absent or slight. The rate of local recurrence is very high. Management should consist of aggressive wide local excision if possible since this tumor has been found to be chemotherapy resistant. Adjuvant radiotherapy is an alternative for residual or metastatic tumors.

1- Evans HL: Low-grade fibromyxoid sarcoma. A report of 12 cases. Am J Surg Pathol  17(6):595-600, 1993
2- Fukunaga M, Ushigome S, Fukunaga N: Low-grade fibromyxoid sarcoma. Virchows Arch  429(4-5):301-3, 1996
3- Canpolat C, Evans HL, Corpron C, Andrassy RJ, Chan K, Eifel P, Elidemir O, Raney B: Fibromyxoid sarcoma in a four-year-old child: case report and review of the literature. Med Pediatr Oncol  27(6):561-4, 1996
4- Folpe AL, Lane KL, Paull G, Weiss SW: Low-grade fibromyxoid sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic study of 73 cases supporting their identity and assessing the impact of high-grade areas. Am J Surg Pathol  24(10):1353-60, 2000

Volume 18 No 04 APRIL 2002

Birth Fractures

Injury during birth is not an uncommon event. Birth fracture includes clavicular, skull, nasal, ribs and long bones. Clavicular fractures (CFx) are the commonest birth injury caused by excessive pressure on the shoulder from the symphysis pubis. Cfx occurs in macrosomic infants, in those with shoulder dystocia and is associated with injury to the brachial plexus. In most cases this injury cannot be predicted prior to delivery. Diagnosis is confirmed by x-ray. Management is expectantly with complete recovery in most cases. Skull fractures result from difficult delivery and use of vacuum extractor and can be either linear or depressed. Linear fractures are observed, while depressed fractures might need surgery. Very rarely the underlying brain is damage. Nasal birth trauma can cause dislocation of the cartilages of the septum with deviation of the nose. Management consists of realignment of the septum (Playfair Maneuver) to avoid long-term nasal airway obstruction. Being obligate nasal breather if the injury reduces the airway passages the baby may have feeding problems needing nasogastric support. Ribs fractures occur very rarely after difficult forceps delivery of large babies. Management is expectancy. Long bones fractures (humerus and femur) occurs after difficult breach delivery, twin pregnancy and prematurity. Babies born with spinal dysraphism can sustain femoral fractures during birth. Bandage protection, traction, spica cast or splint support might be needed depending on the fracture and angulation. Cases with birth injuries have longer hospital stay and higher mortality.

1- Perlow JH, Wigton T, Hart J, Strassner HT, Nageotte MP, Wolk BM: Birth trauma. A five-year review of incidence and associated perinatal factors. J Reprod Med 41(10):754-60, 1996
2- Many A, Brenner SH, Yaron Y, Lusky A, Peyser MR, Lessing JB: Prospective study of incidence and predisposing factors for clavicular fracture in the newborn. Acta Obstet Gynecol Scand 75(4):378-81, 1996
3- Walle T, Hartikainen-Sorri AL: Obstetric shoulder injury. Associated risk factors, prediction and prognosis. Acta Obstet Gynecol Scand 72(6):450-4, 1993
4- Hickey K, McKenna P: Skull fracture caused by vacuum extraction. Obstet Gynecol 88(4 Pt 2):671-3, 1996
5- Nadas S, Reinberg O: Obstetric fractures. Eur J Pediatr Surg 2(3):165-8, 1992
6- Hughes CA, Harley EH, Milmoe G, Bala R, Martorella A: Birth trauma in the head and neck. Arch Otolaryngol Head Neck Surg 125(2):193-9, 1999
7- Korantzis A, Cardamakis E, Chelidonis E, Papamihalis T: Nasal septum deformity in the newborn infant during labour. Eur J Obstet Gynecol Reprod Biol 23;44(1):41-6, 1992
8- Salonen IS: Birth fractures of long bones. Ann Chir Gynaecol 80(1):71-3, 1991
9- Bhat BV, Kumar A, Oumachigui A: Bone injuries during delivery. Indian J Pediatr 61(4):401-5, 1994
10- Morris S, Cassidy N, Stephens M, McCormack D, McManus F: Birth-associated femoral fractures: incidence and outcome. J Pediatr Orthop 22(1):27-30, 2002

Spilled Gallstones

Gallbladder rupture with gallstone spillage occurs more frequently after laparoscopic (5%) than open cholecystectomy. It is generally caused by injury with an electric knife during dissection of the gallbladder bed, injury during gallbladder retraction with grasping forceps, injury during gallbladder extraction from the abdomen, and slippage of cystic duct clips (potentially causing bile and stone spillage). Reports describe postoperative instances of small bowel obstruction, intraabdominal, abdominal wall and subcutaneous abscess developing. A concerted effort should be made to remove all stones spilled in the peritoneal cavity. The necrotic, friable gallbladder should be immediately placed in an endoscopic bag upon completion of the dissection, copious lavage of the peritoneal cavity to evacuate spilled bile should follow, and  a therapeutic course of broad-spectrum antibiotic should be instituted. Conversion to laparotomy is not justifiable. Patients can presents weeks to months postoperatively with vague abdominal complaints, fever and leukocytosis. Computed tomography reveals inflammatory foci involving intraperitoneal gallstones. Management might require percutaneous or operative drainage of the infectious collections. All affected patients should be informed to hasten early diagnosis of later complications.

1- Wilton PB, Andy OJ Jr, Peters JJ, Thomas CF, Patel VS, Scott-Conner CE: Laparoscopic cholecystectomy. Leave no (spilled) stone unturned. Surg Endosc  7(6):537-8, 1993
2- Leslie KA, Rankin RN, Duff JH: Lost gallstones during laparoscopic cholecystectomy: are they really benign? Can J Surg  37(3):240-2, 1994
3- Ponce J, Cutshall KE, Hodge MJ, Browder W: The lost laparoscopic stone. Potential for long-term complications. Arch Surg  130(6):666-8, 1995
4- Kimura T, Goto H, Takeuchi Y, Yoshida M, Kobayashi T, Sakuramachi S, Harada Y: Intraabdominal contamination after gallbladder perforation during laparoscopic cholecystectomy and its complications. Surg Endosc  10(9):888-91, 1996
5- Schafer M, Suter C, Klaiber C, Wehrli H, Frei E, Krahenbuhl L: Spilled gallstones after laparoscopic cholecystectomy. A relevant problem? A retrospective analysis of 10,174 laparoscopic cholecystectomies. Surg Endosc  12(4):305-9, 1998
6- Sarli L, Pietra N, Costi R, Grattarola M: Gallbladder perforation during laparoscopic cholecystectomy. World J Surg  23(11):1186-90, 1999
7- Yao CC, Wong HH, Yang CC, Lin CS: Abdominal wall abscess secondary to spilled gallstones: late complication of laparoscopic cholecystectomy and preventive measures. J Laparoendosc Adv Surg Tech A  11(1):47-51, 2001


Pancreatoblastoma (PB) is a juvenile adenocarcinoma of pancreas occurring rarely in the pediatric age group. The tumor presents during early childhood (mean age four years), affects mostly males and is associated with the Beckwith-Wiedemann syndrome. PB is a large malignant tumor composed of epithelial tissue with acinar differentiation. Most cases originate in the head of the pancreas.  Though being a slow growing tumor, local invasion to duodenum, stomach and peritoneum occurs. Children presents with palpable mass, pain and abdominal distension. Rarely do they develop jaundice. AFP and LDH levels can be elevated. One-third of the cases develop metastasis (liver and lung) US and CT-Scan confirms the presence of a solid multilobulated tumor. Percutaneous needle biopsy is diagnostic. Management consists of wide local excision which usually entails pancreaticoduodenectomy. Preop chemotherapy has made unresectable tumor be resectable. Recurrent or residual disease can be managed with radiotherapy a/o chemotherapy. Prognosis is guarded.

1- Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM, Shandling B: A 20-year review of pediatric pancreatic tumors. J Pediatr Surg  27(10):1315-7, 1992
2- Inomata Y, Nishizawa T, Takasan H, Hayakawa T, Tanaka K: Pancreatoblastoma resected by delayed primary operation after effective chemotherapy. J Pediatr Surg  27(12):1570-2, 1992
3- Klimstra DS, Wenig BM, Adair CF, Heffess CS: Pancreatoblastoma. A clinicopathologic study and review of the literature. Am J Surg Pathol  19(12):1371-89, 1995
4- Willnow U, Willberg B, Schwamborn D, Korholz D, Gobel U: Pancreatoblastoma in children. Case report and review of the literature. Eur J Pediatr Surg  6(6):369-72, 1996
5- Chun Y, Kim W, Park K, Lee S, Jung S: Pancreatoblastoma. J Pediatr Surg  32(11):1612-5, 1997
6- Ogawa B, Okinaga K, Obana K, Nakamura K, Hattori T, Ito T, Yanagawa Y, Tanaka F, Imamura T: Pancreatoblastoma treated by delayed operation after effective chemotherapy. J Pediatr Surg  35(11):1663-5, 2000
7- Johnson PRV, Spitz L: Cysts and Tumors of the Pancreas. Seminars Pediatr Surg 9 (4): 209-215, 2000

Volume 18 No 05 MAY 2002

Clostridium Colitis

Clostridium difficile enterocolitis (CDE) is also known as pseudomembranous colitis. CDE can be seen in children before, during or after surgical management for Hirschsprung's disease though most cases are seen preoperatively. This type of enterocolitis can be associated with fever, abdominal pain/distension, leukocytosis and diarrhea (protein losing enteropathy) which can progress to shock, prostration, toxic megacolon, perforation and even death. Clostridium difficile secretes two toxins (A and B) that produce a variety of local and systemic effects leading to depressed intestinal mucin production and cell renewal rendering the bowel susceptible to bacterial invasion and sepsis. Most cases of CDE have received prior antibiotic treatment for approximately one week or more. Other risk factors for CDE are colon procedures, acute functional bowel obstruction and critically-ill patients. Hirschsprung patients are at increase risk of developing CDE due to intestinal stasis, hypersensitivity to bacterial antigens, abnormality of prostaglandin secretion and abnormal mucin production. In long segment Hirschsprung's disease the aganglionic colon might also be involved. Diagnosis of CDE can be established by culture of C. difficile or a fecal positive toxin assay. Endoscopic evidence of pseudomembranous colitis can also suggest the diagnosis. Management of CDE consists of oral (or stomal) vancomycin or metronidazole therapy since it's more reliable. Refractory or chronic cases might need surgical derivation or bowel resection. The morbidity associated with this nosocomial infection is significant needing a high level of suspicion for early diagnosis.

1-Bagwell CE, Langham MR Jr, Mahaffey SM, Talbert JL, Shandling B: Pseudomembranous colitis following resection for Hirschsprung's disease. J Pediatr Surg  27(10):1261-4, 1992
2-Urushihara N, Kohno S, Hasegawa S: Pseudomembranous enterocolitis and hemorrhagic necrotizing enterocolitis in Hirschsprung's disease. Surg Today  24(3):221-4, 1994
3- Kent KC, Rubin MS, Wroblewski L, Hanff PA, Silen W: The impact of Clostridium difficile on a surgical service: a prospective study of 374 patients. Ann Surg  227(2):296-301, 1998
4- Grundfest-Broniatowski S, Quader M, Alexander F, Walsh RM, Lavery I, Milsom J: Clostridium difficile colitis in the critically ill. Dis Colon Rectum  39(6):619-23, 1996
5- Bartlett JG: Clostridium difficile infection: pathophysiology and diagnosis. Semin Gastrointest Dis  8(1):12-21, 1997
6- Teich S, Schisgall RM, Anderson KD: Ischemic enterocolitis as a complication of Hirschsprung's disease. J Pediatr Surg  21(2):143-5, 1986
7- Fekety R, Shah AB: Diagnosis and treatment of Clostridium difficile colitis. JAMA  6;269(1):71-5, 1993
8- Thomas DF, Fernie DS, Bayston R, Spitz L, Nixon HH: Enterocolitis in Hirschsprung's disease: a controlled study of the etiologic role of Clostridium difficile. J Pediatr Surg  21(1):22-5, 1986
9- Hardy SP, Bayston R, Spitz L: Prolonged carriage of Clostridium difficile in Hirschsprung's disease. Arch Dis Child  69(2):221-4, 1993
10- Brearly S, Armstrong GR, Nairn R, Gornall P, Currie AB, Buick RG, Corkery JJ: Pseudomembranous colitis: a lethal complication of Hirschsprung's disease unrelated to antibiotic usage. J Pediatr Surg  22(3):257-9, 1987

Adnexal Torsion

Evaluation of females with acute onset lower quadrant pain should always consider the possibility of ovarian and tube (adnexal) torsion. Ovarian torsion is associated with a mass, cyst, tumors or simply a normal ovary (30%). Cysts and tumors are the leading cause (70%) of torsion due to excessive rotation and ischemia of the adnexa. The right adnexa is more frequently involved than the left in torsion sometimes undistinguishable from acute appendicitis. Nausea, vomiting and pain are more often seen with ovarian torsion.  Adnexal torsion is particular prone during premenarche years (7-10 year old girls). Preoperative diagnosis is very difficult. Ultrasonography using color Doppler signal can establish a high index of suspicion (echogenic mass, edematous ovary, free intraperitoneal fluid, absent blood flow) of torsion. Most cases present too late to be able to save the adnexa. At operation (or laparoscopy) the viable ovary should be untwisted and the necrotic ovary excised without untwisting the pedicle to avoid thromboembolism. Others feel a more conservative approach toward a necrotic ovary can maintain viable tissue with minimal morbidity (fever). Contralateral oophoropexy at the time of oophorectomy is widely recommended, more so if the torsion is associated with a normal idiopathic adnexa.

1- Davis AJ, Feins NR: Subsequent asynchronous torsion of normal adnexa in children. J Pediatr Surg  25(6):687-9, 1990
2- Shun A: Unilateral childhood ovarian loss: an indication for contralateral oophoropexy?  Aust N Z J Surg  60(10):791-4, 1990
3- Mordehai J, Mares AJ, Barki Y, Finaly R, Meizner I: Torsion of uterine adnexa in neonates and children: a report of 20 cases. J Pediatr Surg  26(10):1195-9, 1991
4- Kimura I, Togashi K, Kawakami S, Takakura K, Mori T, Konishi J: Ovarian torsion: CT and MR imaging appearances.  Radiology  190(2):337-41, 1994
5- Meyer JS, Harmon CM, Harty MP, Markowitz RI, Hubbard AM, Bellah RD: Ovarian torsion: clinical and imaging presentation in children. J Pediatr Surg  30(10):1433-6, 1995
6-  Cohen Z, Shinhar D, Kopernik G, Mares AJ: The laparoscopic approach to uterine adnexal torsion in childhood. J Pediatr Surg  31(11):1557-9, 1996
7- Dolgin SE, Lublin M, Shlasko E: Maximizing ovarian salvage when treating idiopathic adnexal torsion. J Pediatr Surg  35(4):624-6, 2000
8- Templeman C, Hertweck SP, Fallat ME: The clinical course of unresected ovarian torsion. J Pediatr Surg  35(9):1385-7, 2000

Rare Branchial Remnant

Third and fourth branchial cleft remnants,  known as complex branchial remnants,  are extremely rare neck anomalies in children. Most branchial cleft anomalies (tags, cysts, fistulas or sinuses) come from faulty second branchial pouch development (supratonsillar fossa). The third pouch develops into the thymus and upper parathyroid glands, while the fourth pouch gives rise to the lower parathyroids and thyroid gland. Third branchial remnants are more common in the left side presenting as unilateral fistulas or sinus tracts that communicates with the pharynx at the level of the thyrohyoid membrane. They can harbor ectopic thyroid or thymus tissue. A recurrent history of neck infection and drainage is commonly obtained. Fourth branchial anomalies communicate with the thyroid gland or pyriform sinus and give rise to thyroiditis. Surgical excision is the treatment of choice for all branchial remnants in the neck after subsiding any episode of infection with antibiotics. Recurrence is rarely seen after complete removal of the lesion.

1- Doi O, Hutson JM, Myers NA, McKelvie PA: Branchial remnants: a review of 58 cases. J Pediatr Surg  23(9):789-92, 1988
2- Moreno Hurtado C, Blesa Sanchez E, Cabrera Garcia R, Nunez Nunez R: [Third and fourth cervical pouches remnants and branchial clefts]. Cir Pediatr  9(4):154-7, 1996
3- De Caluwe D, Hayes R, McDermott M, Corbally MT: Complex branchial fistula: a variant arch anomaly. J Pediatr Surg  36(7):1087-8, 2001
4- Franciosi JP, Sell LL, Conley SF, Bolender DL: Pyriform Sinus Malformations: A Cadaveric representation. J Pediatr Surg 37(3): 533-538, 2002

Volume 18 No 06 JUNE 2002

Meconium Plug Syndrome

One of the most significant sign of large bowel obstruction during the neonatal period is failure to pass meconium during the first day of life. The differential diagnosis to consider includes Hirschsprung's disease, anorectal malformations, meconium plug syndrome, small left colon syndrome, hypoganglionosis, neuronal intestinal dysplasia and megacystis-microcolon-intestinal hypoperistalsis syndrome. Meconium plug syndrome (MPS) was first described by Clatworthy in 1956 as a transient form of distal colonic or rectal obstruction in newborns caused by an inspissated, immobile meconium. The plug is white and chalky and rarely involves the small bowel. Clinical manifestations include progressive abdominal distension, vomiting (sometimes is bilious) and failure to pass meconium during the initial two days of life. Though most cases are idiopathic, MPS has been associated with prematurity, hypotonia, hypermagnesemia (reduces acetylcholine release with subsequent myoneural depression), diabetic mother, Hirschsprung's disease and cystic fibrosis. Colonic contrast study suggests the diagnosis (filling defect) and can be therapeutic in most cases to relieve the obstruction. Gastrografin instillation is highly effective in moving the obstructing long, thick plug, even in tiny premature infants with MPS. Suction rectal biopsy to exclude the diagnosis of Hirschsprung's disease along with cystic fibrosis screening is warranted in all cases of MPS. The diagnosis of MPS is made after all the above causes are excluded. Need for surgery is extremely rare.

1- Loening-Baucke V, Kimura K: Failure to pass meconium: diagnosing neonatal intestinal obstruction. Am Fam Physician 60(7):2043-50, 1999
2- Clatworthy HW, Howard WH, Lloyd J: The meconium plug syndrome. Surgery 39:131-142, 1956
3- Hen J, Dolan TF, Touloukian RJ: Meconium Plug Syndrome associated with Cystic Fibrosis and Hirschsprung's Disease. Pediatrics 66 (3): 466-468, 1980
4- Krasna IH, Rosenfeld D, Salerno P: Is it Necrotizing Enterocolitis, Microcolon of Prematurity, or Delayed Meconium Plug? A Dilemma in the Tiny Premature Infant. J Pediatr Surg 31(6): 855-858, 1996
5- Olsen MM, Luck SR, Lloyd-Still J, Raffensperger JG: The spectrum of meconium disease in infancy. J Pediatr Surg  17(5):479-81, 1982
6- Rosenstein BJ: Cystic fibrosis presenting with the meconium plug syndrome. Am J Dis Child  132(2):167-9, 1978

Granuloma Annulare

Granuloma annulare is a rare, benign, self-limiting, subcutaneous nodule that can appear in the scalp and/or extremity of infants and children. The nodule is painless, non-mobile, characterized by rapid growth usually identified in the occipital region with absence of bone involvement. Mean age at presentation is four years. Lesions are most commonly located about the elbow, knee, and scalp.  The erythrocyte sedimentation rate could be elevated attesting to the inflammatory nature of the lesion.  Otherwise, no ancillary test is specific for this disorder. Excisional biopsy can establish the diagnosis by showing lesions that resembles rheumatoid nodules, consisting of acellular central areas surrounded by palisading histiocytes. Between 20 and 50% of children have local recurrence or distant development of new lesions. Most patients will not progress to any recognized systemic illness or connective tissue disorder. The clinical course is characterized by spontaneous regression. Parental reassurance is warranted.

1- Challa VR, Weidner N, Bell WO, Prichard RW: Granuloma annulare: a rare occipital lesion in infants and children. Surg Neurol  28(3):211-4, 1987
2- Davids JR, Kolman BH, Billman GF, Krous HF: Subcutaneous granuloma annulare: recognition and treatment. J Pediatr Orthop  13(5):582-6, 1993
3- Felner EI, Steinberg JB, Weinberg AG: Subcutaneous granuloma annulare: a review of 47 cases. Pediatrics  100(6):965-7, 1997
4- Trobs RB, Borte M, Voppmann A, Weidenbach H, Thiele J: Granuloma annulare, nodular type--a subcutaneous pseudorheumatoid lesion in children. Eur J Pediatr Surg  7(6):349-52, 1997
5- McDermott MB, Lind AC, Marley EF, Dehner LP: Deep granuloma annulare (pseudorheumatoid nodule) in children: clinicopathologic study of 35 cases. Pediatr Dev Pathol  1(4):300-8, 1998
6- Grogg KL, Nascimento AG:  Subcutaneous granuloma annulare in childhood: clinicopathologic features in 34 cases. Pediatrics  107(3):E42, 2001

Cervical Teratoma

Germ cell tumor (Teratoma)  arising in the neck region of an infant is an unusual lesion encompassing between three and 5% of all teratomas found in children. These lesions are histologically benign, usually large and can cause airway obstruction during birth. Cervical teratomas are true neoplasm composed of foreign tissue to the anatomic site of origin with all three germ layers represented. Neural tissue predominates. Fine calcifications can be seen on simple films. Newborns might require intubation within the first few hours after birth due to respiratory distress. The tumor is firm, frequently mobile, multilobular, cystic and well encapsulated. Occasionally the tumor is incorrectly diagnosed as cystic hygroma. Prenatally this tumor can be associated with polyhydramnios, pulmonary insufficiency and fetal demise. Prenatal diagnosis by ultrasound gives the clinician the opportunity of maintaining the materno-fetal circulation until the airway is properly secured during birth (EXIT procedure). Cesarean section is recommended for all tumors larger than five cm in size. After birth and stabilization management consists of prompt surgical excision. Total excision is essential to avoid local recurrence and malignant degeneration. Postoperative monitoring for recurrences should include Alpha-fetoprotein levels in difficult cases.

1- Gundry SR, Wesley JR, Klein MD, Barr M, Coran AG: Cervical teratomas in the newborn. J Pediatr Surg  18(4):382-6, 1983
2- Jordan RB, Gauderer MW: Cervical teratomas: an analysis. Literature review and proposed classification. J Pediatr Surg  23(6):583-91, 1988
3- Langer JC, Tabb T, Thompson P, Paes BA, Caco CC: Management of prenatally diagnosed tracheal obstruction: access to the airway in utero prior to delivery. Fetal Diagn Ther  7(1):12-6, 1992
4- Azizkhan RG, Haase GM, Applebaum H, Dillon PW, Coran AG, King PA, King DR, Hodge DS:  Diagnosis, management, and outcome of cervicofacial teratomas in neonates: a Childrens Cancer Group study. J Pediatr Surg  30(2):312-6, 1995
5- Larsen ME, Larsen JW, Hamersley SL, McBride TP, Bahadori RS: Successful management of fetal cervical teratoma using the EXIT procedure. J Matern Fetal Med  8(6):295-7, 1999
6- Elmasalme F, Giacomantonio M, Clarke KD, Othman E, Matbouli S: Congenital cervical teratoma in neonates. Case report and review. Eur J Pediatr Surg  10(4):252-7, 2000

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