VOLUME 24, 2005


Volume 24 No 01 JANUARY 2005

Human Tail

The human tails refers to a extremely rare and benign condition where a child is born with a persistent vestigial tail-like cutaneous structure in the lumbosacrococcygeal region. Pathologically, two types of human tails have been recognized: the true and pseudo human tail. The true human tail arises from the most distal remnant of the embryonic tail lacking bone, cartilage, notochord and spinal cord. Contains a central core of mature fatty tissue divided into small lobules by thin fibrous septa with small blood vessels and nerve fibers scattered. The true tail arises by retention of structures found normally in fetal development. It may be as long as 13 cm, can move and contract, and occurs twice as often in males as in females. The pseudotail is a short, stump-like structure. Spina bifida (dysraphism) is the most frequent coexisting anomaly in both anatomical variants (50%). Other associated lesions include tethered cord syndrome, lipomas, teratomas and gliomas. Investigation of children born with human tail appendages should include a thorough neurological examination, plain x-ray films of the lumbo-sacral region and contrast MRI imaging looking for dysraphism and associated lesions. Management consist of surgical excision of the vestigial tail and repair of the dysraphism. Long-term follow-up for possible sequelae after surgery, especially in cases with spinal dysraphism, is necessary.

1- Belzberg AJ, Myles ST, Trevenen CL: The human tail and spinal dysraphism. J Pediatr Surg 26(10):1243-5, 1991
2- Dao AH, Netsky MG: Human tails and pseudotails. Hum Pathol 15(5):449-53, 1984
3- Dubrow TJ, Wackym PA, Lesavoy MA: Detailing the human tail. Ann Plast Surg 20(4):340-4, 1988
4- Lu FL, Wang PJ, Teng RJ, Yau KI: The human tail. Pediatr Neurol 19(3):230-3, 1998
5- Gonul E, Izci Y, Onguru O, Timurkaynak E, Seber N: The human tail associated with intraspinal lipoma: case report. Minim Invasive Neurosurg 43(4):215-8, 2000
6- Noack F, Reusche E, Gembruch U: Prenatal diagnosis of 'true tail' with cartilage content? Fetal Diagn Ther 18(4):226-9, 2003
7- Muthukumar N: The "human tail": a rare cause of tethered cord: a case report. Spine 29(20):E476-8, 2004

Vaginal Prolapse

Vaginal prolapse in the newborn period is a condition reported rarely. It usually occurs during the first few days of life and presents as a tumor mass protruding from the vulva. The genital prolapse can include both the vagina and uterus. Genital prolapse in this age group is associated with a congenital pelvic neuropathy (myelomeningocele and spina bifida oculta), lesions affecting the development of the levator muscles, primary support of the pelvic organs. Though most cases are seen in babies with central nervous system defects, the condition can also be seen in normal newborn babies born with intrauterine growth retardation. Initial management consists of digital manual reduction. Unfortunately, most cases are often resistant to simple reduction. If the prolapse recurs the wearing of a tiny pessary made from a rolled and tied one-inch penrose drain is introduced into the vagina. The pessary can be removed for cleansing and continued to be used for several weeks until the lax tissues adhere laterally. Should the prolapse be severe and repetitive, temporary sewing together the posterior half of the labia minora and majora together can be done. The suture is removed after they have been in place for two weeks. On a few occasions ventral suspension of the uterus has been deemed necessary.

1- Johnson A, Unger SW, Rodgers BM: Uterine prolapse in the neonate. J Pediatr Surg 19(2):210-1, 1984
2- Shuwarger D, Young RL: Management of neonatal genital prolapse: case reports and historic review. Obstet Gynecol 66(3 Suppl):61S-63S, 1985
3- Carpenter SE, Rock JA: Procidentia in the newborn. Int J Gynaecol Obstet 25(2):151-3, 1987
4- Bayatpour M, McCann J, Harris T, Phelps H: Neonatal genital prolapse. Pediatrics 90(3):465-6, 1992
5- Banieghbal B, Fonseca J: Surgical management of uterine prolapse in an infant. Eur J Pediatr Surg 8(2):119-20, 1998
6- McGlone L, Patole S: Neonatal genital prolapse. J Paediatr Child Health 40(3):156-7, 2004


Fibroadenoma is a common benign tumor found in the breast of adolescent girls. It is also considered the most common discrete solid mass found within the adolescent breast tissue. Most girls harboring a fibroadenoma have between thirteen and 16 years of age, the tumor is slow growing, tends to develop in the upper outer quadrant and is more common in African-American race. Though females may develop breast masses early in life, the risk of malignancy is extremely low. The tumor is usually solitary, with an average diameter of two to 4 cm, characterized by rich cellular stroma and prominent glandular epithelium. At physical exam the mass feels like a well-circumscribed movable nodule. Fibroadenomas may be related to an exaggerated local response to the estrogenic effects of puberty. Mammography, due to the inherent radiation risk and dense fibrous tissue, is not recommended for routine screening or routine imaging of breast masses in adolescents. Alternatively, sonography of the breast is diagnostic on most cases. The tumor looks well-circumscribe, hyperechoic and homogenous on ultrasound. Ten percent of cases harbor a giant juvenile fibroadenoma, a large lesion that distorts the normal breast architecture eroding through the skin and areolar complex. Management of fibroadenoma could be observation or cryoablation. Growing, symptomatic or anxiety-producing masses should be managed with excision through a periareolar incision to preserve cosmesis.

1- Simmons PS: Diagnostic considerations in breast disorders of children and adolescents. Obstet Gynecol Clin North Am 19(1):91-102, 1992
2- West KW, Rescorla FJ, Scherer LR 3rd, Grosfeld JL: Diagnosis and treatment of symptomatic breast masses in the pediatric population.  J Pediatr Surg 30(2):182-6, 1995
3- Dehner LP, Hill DA, Deschryver K: Pathology of the breast in children, adolescents, and young adults. Semin Diagn Pathol 16(3):235-47, 1999
4- Elsheikh A, Keramopoulos A, Lazaris D, Ambela C, Louvrou N, Michalas S: Breast tumors during adolescence. Eur J Gynaecol Oncol 21(4):408-10, 2000
5- Onuigbo W: Breast fibroadenoma in teenage females. Turk J Pediatr 45(4):326-8, 2003
6- Kaufman CS, Bachman B, Littrup PJ, Freeman-Gibb LA, White M, Carolin K, Francescatti D, Stocks LH, Smith JS, Henry CA, Bailey L, Harness JK, Simmons R: Cryoablation treatment of benign breast lesions with 12-month follow-up. Am J Surg 188(4):340-8, 2004

Volume 24 No 02 FEBRUARY 2005

Adrenal Incidentaloma

With the advent of potent imaging studies during the eighties a group of adult patients was found with incidentally discovered adrenal masses, hence the term coined of adrenal Incidentaloma. At the time, masses below a size of three centimeters were observed with follow-up studies for spontaneous regression. Most cases resulted in benign non-functioning adenomas which disappeared with time. The situation in children is different. A mass identified in the adrenal gland is cause for concern. In infancy and childhood the most common adrenal mass is the neuroblastoma, a malignant neural crest tumor. Initial diagnosis of an adrenal mass in a child is made with Ultrasound, which is also used to document regression of uncomplicated neonatal adrenal hemorrhage. Further radiological assessment of an adrenal incidentaloma in a child should include CT-Scan and MRI. MRI can accurately distinguish adrenal adenomas from adenocarcinoma, pheochromocytoma and neuroblastomas. Endocrine tests evaluating pituitary-adrenal function (urinary excretion of 17-hydroxycorticosteroids, 17-ketosteroids and catecholamines, plasma concentrations of ACTH, cortisol, DHEAS, androstenedione and testosterone, dexamethasone suppression test and corticotrophin-releasing hormone stimulation test) should be part of the work-up. Should biochemical studies revealed no hormonal related disease (Cushing, hyperaldosteronism, pheochromocytoma, etc.) a histological diagnosis should be obtained by either CT-guided fine  needle biopsy or surgical resection. In the event of no diagnosis, adrenal tumor resection should be done.

1- Caplan RH, Kisken WA, Huiras CM: Incidentally discovered adrenal masses. Minn Med 74(8):23-6, 1991
2- Kasperlik-Zeluska AA, Roslonowska E, Slowinska-Srzednicka J, Migdalska B, Jeske W, Makowska A, Snochowska H: Incidentally discovered adrenal mass (incidentaloma): investigation and management of 208 patients. Clin Endocrinol (Oxf) 46(1):29-37, 1997
3- Angeli A, Osella G, Ali A, Terzolo M: Adrenal incidentaloma: an overview of clinical and epidemiological data from the National Italian Study Group. Horm Res 47(4-6):279-83, 1997
4- Agrons GA, Lonergan GJ, Dickey GE, Perez-Monte JE: Adrenocortical neoplasms in children: radiologic-pathologic correlation. Radiographics 19(4):989-1008, 1999
5- Masiakos PT, Gerstle JT, Cheang T, Viero S, Kim PC, Wales P: Is surgery necessary for incidentally discovered adrenal masses in children? J Pediatr Surg 39(5):754-8, 2004


Spontaneous pneumomediastinum is a self-limited condition rarely seen in infants and children. Alveolar rupture secondary to increased pressure (such as effected by barotrauma) or over distension leads to air dissection along perivascular and peribronchial tissues up to the hilum of the mediastinum and the soft tissue of the neck (Macklin effect). Clinically the child develops dyspnea, sore throat, sudden chest pain radiating to the back or neck, subcutaneous emphysema and Hamman‘s sign. Rarely, the massive subcutaneous emphysema can cause respiratory compromise requiring emergency releasing incisions. Some of the causes of spontaneous pneumomediastinum include asthma (most common cause), mechanical ventilation, intubation, Valsalva, foreign body, trauma, esophageal perforation and drug inhalation. Diagnosis is established with simple chest films (PA and lateral are needed). Management is conservative (bed rest and analgesics). A few cases need mechanical or high frequency oscillatory ventilation. Most children clinically improve during the next three days with resolution of the pneumomediastinum by seven days.

1- Burton EM, Riggs W Jr, Kaufman RA, Houston CS: Pneumomediastinum caused by foreign body aspiration in children. Pediatr Radiol 20(1-2):45-7, 1989
2- Taylor J, Dibbins A, Sobel DB: Neonatal pneumomediastinum: indications for, and complication of, treatment. Crit Care Med 21(2):296-8, 1993
3- McHugh TP: Pneumomediastinum following penetrating oral trauma. Pediatr Emerg Care 13(3):211-3, 1997
4- Miyahara K, Ichihara T, Watanabe T: Successful use of high frequency oscillatory ventilation for pneumomediastinum. Ann Thorac Cardiovasc Surg 5(1):49-51, 1999
5- Damore DT, Dayan PS: Medical causes of pneumomediastinum in children. Clin Pediatr (Phila) 40(2):87-91, 2001
6- Chapdelaine J, Beaunoyer M, Daigneault P, Berube D, Butter A, Ouimet A, St-Vil D: Spontaneous pneumomediastinum: are we overinvestigating? J Pediatr Surg 39(5):681-4, 2004

Adhesive Bond

Adhesive bond refers to a group of cyanoacrylate tissue glue adhesive used as an alternative for skin wound closure, instead of suture. The most extensive use of adhesive bond is for the repair of simple traumatic skin laceration in the emergency room. Advantages of using adhesive bond include rapid achievement of tissue union, need to use or remove suture is eliminated,  application is less painful than suturing, more efficient use of physician time, and cyanoacrylates have a significant antimicrobial effect against gram-positive organisms. Cosmetic appearance is similar to that obtained after suture skin closure. Early wound deshicence is the most common complication of use of tissue adhesive closure, seen in up to 5% of cases. The adhesive bond is a sterile sealed unit terminally sterilized by gamma radiation. When compared, suturing is cheaper than using the adhesive bond. The use of adhesive bonds is an ideal alternative to conventional suturing for the cutaneous closure of low tension lacerations in children with a long-term cosmetic outcome comparable to conventional suturing. Parent satisfaction is greater with the use of adhesive bond.

1- Bruns TB, Simon HK, McLario DJ, Sullivan KM, Wood RJ, Anand KJ: Laceration repair using a tissue adhesive in a children's emergency department. Pediatrics 98(4 Pt 1):673-5, 1996
2- Simon HK, McLario DJ, Bruns TB, Zempsky WT, Wood RJ, Sullivan KM: Long-term appearance of lacerations repaired using a tissue adhesive. Pediatrics 99(2):193-5, 1997
3- Singer AJ, Hollander JE, Valentine SM, Turque TW, McCuskey CF, Quinn JV: Prospective, randomized, controlled trial of tissue adhesive (2-octylcyanoacrylate) vs standard wound closure techniques for laceration repair. Stony Brook Octylcyanoacrylate Study Group. Acad Emerg Med 5(2):94-9, 1998
4- Barnett P, Jarman FC, Goodge J, Silk G, Aickin R: Randomized trial of histoacryl blue tissue adhesive glue versus suturing in the repair of paediatric lacerations. J Paediatr Child Health 34(6):548-50, 1998
5- Amiel GE, Sukhotnik I, Kawar B, Siplovich L: Use of N-butyl-2-cyanoacrylate in elective surgical incisions--long term outcomes.  Am Coll Surg 189(1):21-5, 1999
6- Ong CC, Jacobsen AS, Joseph VT: Comparing wound closure using tissue glue versus subcuticular suture for pediatric surgical incisions: a prospective, randomized trial. Pediatr Surg Int 18(5-6):553-5, 2002
7- van den Ende ED, Vriens PWHE, Allema JH, Breslau PJ: Adhesive bonds or percutaneous absorbable suture for closure of surgical wounds in children. Results of a prospective randomized trial. J Pediatr Surg 39(8): 1249-1251, 2004

Volume 24 No 03 MARCH 2005


Short bowel syndrome is a very serious gastrointestinal disorder characterized by the absence of significant length of bowel capable of normal digestion and absorption. It is estimated that more than 70% of small bowel length must be lost to develop a short bowel syndrome. The three most common causes of short bowel syndrome in the pediatric age are necrotizing enterocolitis, midgut volvulus and gastroschisis. Intestinal adaptation can occur when the neonate is left with more than thirty (30) centimeters of small bowel with an intact ileo-cecal valve. Though the prospect of bowel transplant continues to develop better forms of avoiding acute rejection, still the median survival after transplantation is short (mean of 15 months). Recently, a novel experimental procedure, has attained the attention of surgeons managing this devastating disease complication. The operation is termed serial transverse enteroplasty (STEP) procedure. After short bowel ensues the process of adaptation includes mucosal hyperplasia and bowel dilatation. The STEP procedure is based on the anatomic principle that the blood supply to the bowel comes from the mesenteric border traversing  along the perpendicular long axis of the bowel. Multiple stapler lines are placed perpendicularly alternating the direction of the stapler creating a channel of bowel smaller in diameter and longer in length than the original bowel. Advantages of STEP: easy to do, no anastomosis needed, does not result in intestinal obstruction, mesentery is not jeopardized, the length is almost double, the tapering is customizable, and can be performed in sequence after a successful Bianchi procedure.  STEP could become the lengthening bowel procedure for short bowel syndrome.

1- Kim HB, Fauza D, Garza J, Oh JT, Nurko S, Jaksic T: Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure. J Pediatr Surg 38(3):425-9, 2003
2- Kim HB, Lee PW, Garza J, Duggan C, Fauza D, Jaksic T: erial transverse enteroplasty for short bowel syndrome: a case report. J Pediatr Surg 38(6):881-5, 2003.
3- Tannuri U: Comment on: J Pediatr Surg 38(6):881-5, 2003. J Pediatr Surg 38(3):425-9, 2003. Serial transverse enteroplasty (STEP): a novel bowel lengthening procedure, and serial transverse enteroplasty for short bowel syndrome. J Pediatr Surg 38(12):1845, 2003; author reply 1845-6

Peritoneal Gliomatosis

Peritoneal Gliomatosis (PG) is a rare complication of solid mature or immature ovarian teratomas. Occurs with rupture of the tumor capsule causing multiple mature glial implantation (neural tumor tissue) on the parietal, visceral peritoneum, omentum or space of Douglas. GP is found in childhood, adolescence, as well as in young women. Peritoneal gliomatosis can be considered to be implantation metastases. The nodules may vary in size, but are usually around 3 mm in diameter. Peritoneal gliomatosis is a benign condition. Mean age at time of diagnosis is eleven years. Most cases described are occurs with mature ovarian teratoma. The prognosis depends chiefly on the degree of maturity of the implants. In mature GP, usually no additional chemotherapy is necessary; in immature GP, chemotherapy can induce maturation of the implants. The fate of the glial tissue is still not clear, but the nodule can persist without detectable changes after more than fifteen years of the initial operation, can undergo fibrosis and disappear, or may transform into malignant glial or teratomatous tissue. Close follow-up of these patients is mandatory. The few cases originating from immature teratoma can developed into malignant transformation. Gliomatosis peritonei in extremely rare circunstances has been reported due to transport of glial tissue from the cerebrospinal fluid into the peritoneal cavity via a ventriculo-peritoneal shunt.

1- Truong LD, Jurco S 3rd, McGavran MH: Gliomatosis peritonei. Report of two cases and review of literature. Am J Surg Pathol 6(5):443-9, 1982
2- El Shafie M, Furay RW, Chablani LV: Ovarian teratoma with peritoneal and lymph node metastases of mature glial tissue: a benign condition. J Surg Oncol 27(1):18-22, 1984
3- Harms D, Janig U, Gobel U: Gliomatosis peritonei in childhood and adolescence. Clinicopathological study of 13 cases including immunohistochemical findings. Pathol Res Pract 184(4):422-30, 1989
4- Chaung JH, Chen L: Ovarian teratoma with gliomatosis peritonei. J Pediatr Surg 27(5):662-4, 1992
5- Dadmanesh F, Miller DM, Swenerton KD, Clement PB: Gliomatosis peritonei with malignant transformation. Mod Pathol 10(6):597-601, 1997
6- Hill DA, Dehner LP, White FV, Langer JC: Gliomatosis peritonei as a complication of a ventriculoperitoneal shunt: case report and review of the literature. J Pediatr Surg 35(3):497-9, 2000

Nevus Sebaceous

Nevus sebaceous of Jadassohn is a congenital hamartomatous skin lesion occurring mostly on the scalp, face and neck. At physical exam the lesion is well circunscribe with a yellow-orange smooth plaque appearance. Borders become irregular with puberty and hormonal changes. Malignant transformation has been reported in 10% of cases occurring only after puberty. The nevus sebaceous can transformed into a basal or squamous cell carcinoma. The lesion will not go away spontaneously. It is uncommon for malignancy to develop in a sebaceous nevus before puberty. Due to the risk of malignant transformation and the difficulty in follow-up of this children with time, early complete excision for prophylaxis is recommended in cases of nevus sebaceous. Excision must encompassed clean deep and lateral margins of resection to be effective. Large lesion will benefit from use of tissue expanders.

1- Hagan WE: Nevus sebaceus of Jadassohn: the head and neck manifestations. Laryngoscope 97(8 Pt 1):909-14, 1987
2- Goldstein GD, Whitaker DC, Argenyi ZB, Bardach J: Basal cell carcinoma arising in a sebaceous nevus during childhood. J Am Acad Dermatol 18(2 Pt 2):429-30, 1988
3- Weng CJ, Tsai YC, Chen TJ: Jadassohn's nevus sebaceous of the head and face. Ann Plast Surg 25(2):100-2, 1990
4- Beer GM, Widder W, Cierpka K, Kompatscher P, Meyer VE: Malignant tumors associated with nevus sebaceous: therapeutic consequences. Aesthetic Plast Surg 23(3):224-7, 1999
5- Dunkin CS, Abouzeid M, Sarangapani K: Malignant transformation in congenital sebaceous naevi in childhood. J R Coll Surg Edinb 46(5):303-6, 2001
6- Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S: Should nevus sebaceus of Jadassohn in children be excised? A study of 757 cases, and literature review. J Craniofac Surg 14(5):658-60, 2003

Volume 24 No 04 APRIL 2005


Thalassemia, also known as Mediterranean or Cooley's anemia, is a genetically determined  heterogeneous group of hemoglobinopathies affecting the synthesis of hemoglobin alpha and/or beta-globin chains. This autosomal dominant illness can occur in major (homozygous), intermediate or minor (heterozygous) varieties. A defect in the synthesis of hemoglobin subunits results in the accumulation of intracellular particles in the red blood cell contributing to early destruction by the spleen. The anemia of thalassemia is an inborn error of metabolism causing large amounts of fetal hemoglobin to be produced, instead of adult hemoglobin. With the block in iron metabolism, large deposits of iron occur throughout the body. Heterozygous (minor) thalassemia child is asymptomatic, while the major or intermediate thalassemia child develops severe chronic anemia, jaundice, hepatosplenomegaly, frontal bossing and growth retardation. With continued hemolysis come gallbladder pigment stones (25%), hypersplenism and splenic infarcts. Splenectomy is palliative and indicated for the management of chronically transfused patients in order to increase red blood cell survival and decrease transfusion requirements. Cardiac iron overload is the most frequent cause of death from chronic transfusion therapy. After splenectomy, a high incidence of sepsis and portal vein thrombosis (hypercoagulable state) has been reported. Partial splenectomy reduces transfusion requirements for a limited amount of time due to regrowth of the splenic remnant.

1- Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E: Thalassemia. Hematology (Am Soc Hematol Educ Program). 14-34, 2004
2- Fujita F, Lyass S, Otsuka K, Giordano L, Rosenbaum DL, Khalili TM, Phillips EH: Portal vein thrombosis following splenectomy: identification of risk factors. Am Surg 69(11):951-6, 2003
3- Al-Salem AH, Nasserulla Z: Splenectomy for children with thalassemia. Int Surg 87(4):269-73, 2002
4- al-Salem AH, al-Dabbous I, Bhamidibati P: The role of partial splenectomy in children with thalassemia. Eur J Pediatr Surg 8(6):334-8, 1998
5- Laopodis V, Kritikos E, Rizzoti L, Stefanidis P, Klonaris P, Tzardis P: Laparoscopic splenectomy in beta-thalassemia major patients. Advantages and disadvantages. Surg Endosc 12(7):944-7, 1998
6- Skarsgard E, Doski J, Jaksic T, Wesson D, Shandling B, Ein S, Babyn P, Heiss K, Hu X: Thrombosis of the portal venous system after splenectomy for pediatric hematologic disease. J Pediatr Surg 28(9):1109-12, 1993

Latex Allergy

Allergy to natural rubber latex is an increasing common condition in both children and health care workers. Almost 20 to 40% of children with spina bifida are allergic to latex. Other affected persons are health care workers, latex industry workers, immune compromised individuals, children with bladder exstrophy, anorectal anomalies, and persons with positive risk factors such as multiple surgical procedures. Chemical additives in latex gloves can cause an irritant or allergic contact dermatitis. Latex proteins are responsible for most of the immediate IgE-mediated hypersensitivity allergic reactions. Symptoms range from rhinitis, conjunctivitis and urticaria to intraoperative anaphylaxis and death. Skin prick testing with natural rubber latex and glove tests are safe diagnostic procedure. In children with spina bifida significant and independent risk factors identified for latex sensitization are multiple interventions and higher levels of total serum IgE. The only currently available treatment is complete avoidance of latex. For children with known history of latex allergy premedication with antihistamines and steroids is in order.

1- Birmingham PK, Suresh S: Latex allergy in children: diagnosis and management. Indian J Pediatr 66(5):717-24, 1999
2- Degenhardt P, Golla S, Wahn F, Niggemann B: Latex allergy in pediatric surgery is dependent on repeated operations in the first year of life. J Pediatr Surg 36(10):1535-9, 2001
3- Zucker-Pinchoff B, Stadtmauer GJ: Latex allergy. Mt Sinai J Med 69(1-2):88-95, 2002
4- Cremer R, Kleine-Diepenbruck U, Hering F, Holschneider AM: Reduction of latex sensitization in spina bifida patients by a primary prophylaxis programme (five years experience). Eur J Pediatr Surg 12 Suppl 1:S19-21, 2002
5- Sparta G, Kemper MJ, Gerber AC, Goetschel P, Neuhaus TJ: Latex allergy in children with urological malformation and chronic renal failure. J Urol 171(4):1647-9, 2004
6- Kimata H: Latex allergy in infants younger than 1 year. Clin Exp Allergy 34(12):1910-5, 2004

Hermansky-Pudlak Colitis

Hermansky-Pudlak syndrome is a triad of tyrosine-positive oculocutaneous albinism, platelet dysfunction, and the deposition of an abnormal ceroid-like pigment in the tissues. Ceroid lipofuscin is accumulated in lysosomes organelles. Children with Hermansky-Pudlak syndrome can suffer from pulmonary fibrosis, renal failure, and cardiomyopathy besides other complications. All identified affected patients in the northwest Puerto Rico are homozygous for a 16-bp duplication in exon 15 of a recently cloned gene. This duplication is associated with a broad range of pigmentation and an increased risk of restrictive lung disease in adults. Several families with the syndrome rarely suffer from granulomatous colitis, a unique type of inflammatory bowel disease with clinical features suggestive of idiopathic ulcerative colitis and pathologic features suggestive of Crohn's disease with perineal and perirectal involvement. Diagnosis is established with endoscopy.  Management is similar to cases with inflammatory bowel disease directed toward pathogenetic mechanisms. Corticosteroids, sulphasalazine and the new salicylates, the immunosuppressants azathioprine, 6-MP and, more recently, cyclosporin and metronidazole have become the accepted and standard forms of treatment. Some cases are refractory to medical treatment needing segmental resection of the affected bowel.

1- Sherman A, Genuth L, Hazzi CG, Balthazar EJ, Schinella RA: Perirectal abscess in the Hermansky-Pudlak syndrome. Am J Gastroenterol. 84(5):552-6, 1989
2- Witkop CJ, Nunez Babcock M, Rao GH, Gaudier F, Summers CG, Shanahan F, Harmon KR, Townsend D, Sedano HO, King RA, et al: Albinism and Hermansky-Pudlak syndrome in Puerto Rico. Bol Asoc Med P R. 82(8):333-9, 1990
3- Schinella RA, Greco MA, Cobert BL, Denmark LW, Cox RP: Hermansky-Pudlak syndrome with granulomatous colitis. Ann Intern Med. 92(1):20-3, 1980
4- Gahl WA, Brantly M, Kaiser-Kupfer MI, Iwata F, Hazelwood S, Shotelersuk V, Duffy LF, Kuehl EM, Troendle J, Bernardini I: Genetic defects and clinical characteristics of patients with a form of
oculocutaneous albinism (Hermansky-Pudlak syndrome). N Engl J Med. 338(18):1258-64, 1998
5- Sandberg-Gertzen H, Eid R, Jarnerot G: Hermansky-Pudlak syndrome with colitis and pulmonary fibrosis. Scand J Gastroenterol. 34(10):1055-6, 1999
6- Mahadeo R, Markowitz J, Fisher S, Daum F: Hermansky-Pudlak syndrome with granulomatous colitis in children. J Pediatr. 118(6):904-6, 1991

Volume 24 No 05 MAY 2005

Cardiac Tamponade

Vascular access using central lines is essential in managing acutely ill, chronically disease and cancer pediatric patients. Vascular access can be obtained either through the neck or groin using the external jugular, internal jugular, subclavian or saphenous vein. Potentially lethal complications of central venous catheter placement consist of arrhythmias, pneumothorax, hemothorax, vascula injury and cardiac tamponade. Cardiac tamponade is some very rare complication of venous access. Tamponade may be an acute or late complication and is usually associated with the effusion of intravenous fluid into the pericardium. Most cases occur acutely during intraoperative placement. In either setting symptoms of tamponade includes chest pain, hypotension, increase central venous pressure, low oxygen saturation, bradycardia and cardiac arrest. The perforation can occur in the superior vena cava, atrium, ventricle or pulmonary artery.  Immediate recognition of pericardial tamponade followed by pericardiocentesis are crucial factors in survival. Contrast infusion is valuable in evaluating this complication of central line placement. In children, most central venous access should be performed in the operating room whenever possible. After insertion, position of the catheter in the central venous circulation should be documented by radiographic means on a hard-film copy. Any deviation in the child's hemodynamic stability during placement or afterward should herald the coming of a lethal complication and managed accordingly.

1- Hansbrough JF, Narrod JA, Stiegman GV: Cardiac perforation and tamponade from a malpositioned subclavian dialysis catheter. Nephron 32(4):363-4, 1982
2- Hunt LB, Olshansky B, Hiratzka LF: Cardiac tamponade caused by pulmonary artery perforation after central venous catheterization. JPEN J Parenter Enteral Nutr 8(6):711-3, 1984
3- Krauss D, Schmidt GA: Cardiac tamponade and contralateral hemothorax after subclavian vein
catheterization. Chest 99(2):517-8, 1991
4- Bagwell CE, Salzberg AM, Sonnino RE, Haynes JH: Potentially lethal complications of central venous catheter placement. J Pediatr Surg 35(5):709-13, 2000
5- Shields LB, Hunsaker DM, Hunsaker JC 3rd: Iatrogenic catheter-related cardiac tamponade: a case report of fatal hydropericardium following subcutaneous implantation of a chemotherapeutic injection port. J Forensic Sci 48(2):414-8, 2003

Accessory Splenic Torsion

It is estimated that 10% of the general population carries an accessory spleen. Accessory spleens are situated on the  hilum of the spleen, splenic artery, pancreas, splenocolic ligament, greater omentum, mesenterium, adnexal region and scrotum. Trauma, torsion and hematologic hemolytic conditions affect an accessory spleen. A careful search should be made for accessory spleens, as they should be removed at the time of primary splenectomy to avoid a second operation later in life. Torsion with infarction of an accessory spleen must be considered as a rare cause of acute abdominal pain in childhood. Accessory splenic torsion causes acute diffuse or localized (left upper quadrant) abdominal pain sometimes undistinguishable from that caused by acute appendicitis or intussusception. Most affected children develop an intraperitoneal inflammatory mass.  Preoperative diagnostic imaging is unable to point to the diagnosis. Ultrasound shows a round, hypoechoic, solid mass. CT Scan demonstrates a low-density mass with peripheral enhancement after intravenous contrast medium. MRI can be helpful in the differential diagnosis of infarction by suggesting hemorrhagic necrosis on the T2-weighted images. Diagnosis is corroborated during laparoscopy or laparotomy. Accessory splenectomy is curative.

1- Broker FH, Khettry J, Filler RM, Treves S: Splenic torsion and accessory spleen: a scintigraphic demonstration. J Pediatr Surg 10(6):913-5, 1975
2- Appel MF, Bart JB: The surgical and hematologic significance of accessory spleens. Surg Gynecol Obstet 143(2):191-2, 1976
3- Seo T, Ito T, Watanabe Y, Umeda T: Torsion of an accessory spleen presenting as an acute abdomen with an inflammatory mass. US, CT, and MRI findings. Pediatr Radiol 24(7):532-4, 1994
4- Chateil JF, Arboucalot F, Perel Y, Roy D, Vergnes P, Diard F: Acute torsion of an accessory spleen. J Radiol 77(3):209-11, 1996
5- Valls C, Mones L, Guma A, Lopez-Calonge E: Torsion of a wandering accessory spleen: CT findings. Abdom Imaging 23(2):194-5, 1998
6- Perez Fontan FJ, Soler R, Santos M, Facio I: Accessory spleen torsion: US, CT and MR findings. Eur Radiol 11(3):509-12, 2001
7- Wacha M, Danis J, Wayand W: Laparoscopic resection of an accessory spleen in a patient with chronic lower abdominal pain. Surg Endosc 16(8):1242-3. Epub 2002 May 23, 2002

Sinus Histiocytosis

Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a benign condition that occurs mainly in children, characterized by a protracted course with painless bilateral enlargement of the cervical lymph nodes, fever, leucocytosis, mild anemia, raised erythrocyte sedimentation rate and hypergammaglobulinemia. Extranodal involvement in SHML occurs in the skin, upper respiratory tract, and bone. Diagnosis is confirmed with histologic evidence of involved lymph nodes characterized by an exuberant intrasinusoidal histiocytic proliferation. SHML can be associated with retropharyngeal obstructive symptoms, mediastinal enlargement and orbital enlargement. Prognosis has been found to correlate both with the number of nodal groups and number of extranodal system involvement. Children with SHML may have a variably expressed immunodeficiency that predisposes them to recurrent infections. In general, management is expectant waiting for spontaneous regression. Cytotoxic chemotherapeutic agents have been utilized for life-threatening complications of SHML.

1- Suarez CR, Zeller WP, Silberman S, Rust G, Messmore H: Sinus histiocytosis with massive lymphadenopathy: remission with chemotherapy. Am J Pediatr Hematol Oncol 5(3):235-41, 1983
2- Foucar E, Rosai J, Dorfman R: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity. Semin Diagn Pathol 7(1):19-73, 1990
3- Maennle DL, Grierson HL, Gnarra DG, Weisenburger DD: Sinus histiocytosis with massive lymphadenopathy: a spectrum of disease associated with immune dysfunction. Pediatr Pathol 11(3):399-412, 1991
4- Brau RH, Sosa IJ, Marcial-Seoane MA: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and extranodal involvement of the orbit. P R Health Sci J 14(2):145-9, 1995
5- Moore SW, Schneider JW, Schaaf HS: Diagnostic aspects of cervical lymphadenopathy in children in the developing world: a study of 1,877 surgical specimens. Pediatr Surg Int 19(4):240-4. Epub 2003 Apr 17, 2003

Volume 24 No 06 JUNE 2005

Oral Gastrografin

Gastrografin is a valuable contrast material used by radiologists for studies of the gastrointestinal tract. Is a water-soluble highly osmolar material (1900 mOsm) composed of sodium diatrizoate, meglumine amidotrizoate and a wetting agent (polysorbate 80). Gastrografin has a therapeutic value in cases of partial mechanical bowel obstruction, postoperative ileus, dissolution of barium-impacted ileus and stomal dysfunction after gastric resection. Possible mechanisms of action of Gastrografin come from its hyperosmolality promoting proximal bowel distension, increasing the gradient pressure across the obstructing segments, decreasing bowel edema and enhancing bowel  motility. A dose of 100 ml of Gastrografin in adults, or 20-50 ml in children is injected via a nasogastric tube and supine plain abdominal radiographs are taken at 30 min and four hrs after administration. At this time if contrast passes to the colon a non-operative course is followed. With clear-cut off sign or absence of contrast material in the cecum in the next 24 hours a diagnosis of unrelieved mechanical obstruction is entertained and surgery probably needed. For absolute diagnosis of successful resolution the abdominal pain should disappear, the abdomen should appear flat and soft, the nasogastric output normalized and the child should have another spontaneous bowel action. Omnipaque, an isosmolar water soluble agent  retains its radiographic density in the small bowel better than Gastrografin being a better alternative than Gastrografin in follow-through examinations of intestinal obstruction.

1- Joyce WP, Delaney PV, Gorey TF, Fitzpatrick JM: The value of water-soluble contrast radiology in the management of acute small bowel obstruction. Ann R Coll Surg Engl 74(6):422-5, 1992
2- Assalia A, Schein M, Kopelman D, Hirshberg A, Hashmonai M: Therapeutic effect of oral Gastrografin in adhesive, partial small-bowel obstruction: a prospective randomized trial. Surgery 115(4):433-7, 1994
3- Chung CC, Meng WC, Yu SC, Leung KL, Lau WY, Li AK: A prospective study on the use of water-soluble contrast follow-through radiology in the management of small bowel obstruction. Aust N Z J Surg 66(9):598-601, 1996
4- Chen SC, Lin FY, Lee PH, Yu SC, Wang SM, Chang KJ: Water-soluble contrast study predicts the need for early surgery in adhesive small bowel obstruction. Br J Surg 85(12):1692-4, 1998
5- Blackmon S, Lucius C, Wilson JP, Duncan T, Wilson R, Mason EM, Ramshaw B: The use of water-soluble contrast in evaluating clinically equivocal small bowel obstruction. Am Surg 66(3):238-42, 2000
6- Choi HK, Chu KW, Law WL: Therapeutic value of gastrografin in adhesive small bowel obstruction after unsuccessful conservative treatment: a prospective randomized trial. Ann Surg 236(1):1-6, 2002
7- Biondo S, Pares D, Mora L, Marti Rague J, Kreisler E, Jaurrieta E: Randomized clinical study of Gastrografin administration in patients with adhesive small bowel obstruction. Br J Surg 90(5):542-6, 2003
8- Roadley G, Cranshaw I, Young M, Hill AG: Role of Gastrografin in assigning patients to a non-operative course in adhesive small bowel obstruction. ANZ J Surg 74(10):830-2, 2004

Ectopic Testis

Whenever a child is born with an empty scrotum, the physical examination should include a diligent palpable search for the undescended testis in the inguinal, femoral, perineal or medial thigh areas. Testes palpable in areas away from the normal descent from the retroperitoneum to the scrotum are termed ectopic testis. An ectopic testis is caused by mislocation of the ipsilateral genito-femoral nerve controlled stimulation causing the gubernaculum to migrate to the wrong site because the chemotactic signal is arising from this wrong place. Testes palpable in the inguinal canal or found intra-abdominally are termed undescended. Compared with undescended testes, ectopic testes are extremely rare found most commonly in the perineal ipsilateral area. Other sites include the femoral canal, suprapubic region (at base of the penis), medial thigh, preperitoneal, umbilical, contralateral scrotum or associated with gastroschisis. The perineal testis is particularly subject to trauma. Management is orchiopexy as soon as the diagnosis is established. The most effective route of approach for repair is inguinal allowing replacement of the testis into the corresponding hemiscrotum without difficulty. Other surgeons use a low scrotal approach due to the low incidence of concomitant hernia. Because of the histopathologic features involved, prognosis is better than that associated with cryptorchidism.

1- Murphy DM, Butler MR: Preperitoneal ectopic testis: a case report. J Pediatr Surg 20(1):93-4, 1985
2- Gauderer MW: Gastroschisis and extraabdominal ectopic testis: simultaneous repair. J Pediatr Surg 22(7):657-9, 1987
3- Maidenberg M: A case of an ectopic testis in the perineum. Prog Urol 3(2):268-71, 1993
4- Celayir AC, Sander S, Elicevik M: Timing of surgery in perineal ectopic testes: analysis of 16 cases. Pediatr Surg Int 17(2-3):167-8, 2001
5- Parsons JK, Ferrer F, Docimo SG: The low scrotal approach to the ectopic or ascended testicle: prevalence of a patent processus vaginalis.  J Urol 169(5):1832-3, 2003
6- Hutson JM, Hasthorpe S: Testicular descent and cryptorchidism: the state of the art in 2004. J Pediatr Surg 40(2) :297-302, 2005

Gastrointestinal Stromal Tumor

Gastrointestinal stromal tumor (GIST), previously known as gastric leiomyoblastoma, is an uncommon nonepithelial mesenchymal kit-positive (CD117 antigen) tumor of the gastrointestinal tract. GIST are the most common mesenchymal tumors of the gastrointestinal tract. Cell of origin is the intersticial cell of Cajal. The frequency of malignant GIST is 20-30% of the frequency of all soft-tissue sarcomas, but small benign tumors often found incidentally during unrelated surgery or autopsy are more common. GIST occurs in children, young adults or on a familial basis. Most involved children are girls with symptoms of abdominal pain and anemia. CT-Scan or MRI suggests the diagnosis. Most GIST appears in the stomach (submucosal mass), followed by the intestine and rarely the colon. Metastasis occurs to the liver. Large tumors (> 5 cm) with high mitotic activity are associated with bad prognosis. Management consist of complete surgical resection with prophylactic omentectomy to reduce the recurrence of GIST. GIST have lower survival rate and more resistance to chemotherapy.

1- Oguzkurt P, Akcoren Z, Senocak ME, Caglar M, Buyukpamukcu N: A huge gastric stromal tumor in a 13-year-old girl. Turk J Pediatr 44(1):65-8, 2002
2-  Miettinen M, Majidi M, Lasota J: Pathology and diagnostic criteria of gastrointestinal stromal tumors (GISTs): a review. Eur J Cancer 38 Suppl 5:S39-51, 2002
3- Durham MM, Gow KW, Shehata BM, Katzenstein HM, Lorenzo RL, Ricketts RR: Gastrointestinal stromal tumors arising from the stomach: a report of three children. J Pediatr Surg 39(10):1495-9, 2004
4- Geramizadeh B, Bahador A, Ganjei-Azar P, Asadi A: Neonatal gastrointestinal stromal tumor. Report of a case and review of literature. J Pediatr Surg 40(3):572-4, 2005
5-Prakash S, Sarran L, Socci N, Dematteo RP, Eisenstat J, Greco AM, Maki RG, Wexler LH, Laquaglia MP, Besmer P, Antonescu CR: Gastrointestinal Stromal Tumors in Children and Young Adults: A  Clinicopathologic, Molecular, and Genomic Study of 15 Cases and Review of the Literature. J Pediatr Hematol Oncol 27(4):179-187, 2005

Journal Club