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Volume 26 No 01 JANUARY 2006
Supernumerary Nipple
Supernumerary (or accessory) nipple, also known as polythelia, is a congenital developmental abnormality that occurs most commonly over the anterior aspect of the trunk in the pathway of the embryonic milk line extending from the axilla to pubic region. Supernumerary nipple shows all the histologic components observed in the normal nipple including epidermal thickening, pilosebaceous structures, smooth muscles and mammary glands. Incidence of supernumerary nipple is 25 per 1000 live births with a higher prevalence for the left side and male gender. The accessory nipple is a cosmetic defect with the potential to give rise to a neoplasm since any disease process that involves anatomically normal breasts may affect aberrantly located breasts or nipples as well. Due to its atypical appearance and ectopic location, diagnosis of the anomaly may require a high index of suspicion along with histologic verification. Once thought to have an association with renal defects, the presence of supernumerary nipple in asymptomatic children is not an indication to do additional diagnostic studies of the urinary tract. Supernumerary nipple and ectopic breast are different entities. Supernumerary nipples can be identified at birth, whereas ectopic breast tissue becomes noticeable only after hormonal stimulation, usually during puberty, pregnancy or lactation. Management of supernumerary nipple consist of excision for diagnosis, treatment of symptoms, or cosmesis.

References: 1- Mehregan AH: Supernumerary nipple. A histologic study. J Cutan Pathol 8(2):96-104, 1981 2- Johnson CA, Felson B, Jolles H: Polythelia (supernumerary nipple): an update. South Med J 79(9):1106-8, 1986 3- Hersh JH, Bloom AS, Cromer AO, Harrison HL, Weisskopf B: Does a supernumerary nipple/renal field defect exist? Am J Dis Child 141(9):989-91, 1987 4- Velanovich V: Ectopic breast tissue, supernumerary breasts, and supernumerary nipples. South Med J 88(9):903-6, 1995 5- Schmidt H: Supernumerary nipples: prevalence, size, sex and side predilection -- a prospective clinical study. Eur J Pediatr 157(10):821-3, 1998 6- Grotto I, Browner-Elhanan K, Mimouni D, Varsano I, Cohen HA, Mimouni M: Occurrence of supernumerary nipples in children with kidney and urinary tract malformations. Pediatr Dermatol 18(4):291-4, 2001

Gastric Heterotopia
Gastric heterotopia refers to the finding of normal gastric tissue (mucosa) in foreign sites entirely separated from the stomach. The gastric mucosa is of antral type with parietal and chief cells. Heterotopic gastric mucosa has been reported to occur along the gastrointestinal tract mostly associated with bowel duplications, Meckel's diverticulum, within the gallbladder or cystic duct, in omphalomesenteric ducts and in the liver. Alone, intestinal gastric heterotopia usually is found in the jejunum. A few reports call to the attention of gastric heterotopia in the urinary bladder, spinal column, salivary glands, bronchogenic and thyroglossal duct cysts. The presence of gastric mucosa 1/12



produces deep acid ulceration surrounding non-gastric mucosa. This event causes abdominal pain, gastrointestinal bleeding and rarely perforation. Meckel's diverticulum is the most common site of heterotopic gastric mucosa. The heterotopic mucosa in Meckel's diverticulum is not commonly colonized by H. pylori. Repeated abdominal scanning with technetium-99m pertechnetate can help localize the ectopic gastric heterotopia. Other children will need tagged RBC scanning or arteriogram to identify the site of bleeding. Management of gastric heterotopia is resection whenever possible. Simple transverse resection is not recommended for the short Meckel's diverticulum suspicious of harboring heterotopic gastric mucosa.

References: 1- Shim YT, Kim SY: Heterotopic gastric mucosa and pancreatic tissue in the skin of the abdominal wall. J Pediatr Surg 27(12):1539-40, 1992 2- Karakatsanis KG, Chatzipavlidou V, Zafiriadou E, Mavroudis N, Patsiaoura K, Gotzamani-Psarrakos A: Abdominal scanning with technetium-99m pertechnetate localizes ectopic gastric mucosa in the jejunum: case report and review of the literature. Eur J Nucl Med 20(6):547-50, 1993 3- Kong MS, Huang SC, Tzen KY, Lin JN: Repeated technetium-99m pertechnetate scanning for children with obscure gastrointestinal bleeding. J Pediatr Gastroenterol Nutr 18(3):284-7, 1994 4- Varcoe RL, Wong SW, Taylor CF, Newstead GL: Diverticulectomy is inadequate treatment for short Meckel's diverticulum with heterotopic mucosa. ANZ J Surg 74(10):869-72, 2004 5- Bazlul Karim AS, Matiur Rahman MD, Kamal M: Perforation of jejunal duplication lined with ectopic gastric mucosa. J Paediatr Child Health 40(11):649-50, 2004 6- Jimenez JC, Emil S, Steinmetz B, Romansky S, Weller M: Recurrent gastrointestinal tract bleeding secondary to jejunal gastric heterotopia. J Pediatr Surg 40(10): 1654-1657, 2005

Sialadenitis, defined as inflammation of a salivary gland, is a common benign condition seen in children. Sialadenitis can be caused by an acute viral infection (mumps), suppurative bacterial infection (most commonly staphylococcus aureus and Haemophilus influenzae), a chronic recurrent process without determinate cause, or as part of a granulomatous disease process (tuberculosis, sarcoidosis, Sjgren). The parotid gland of male children is most often involved. Clinically the child develops episodic pain, fever and swelling of the parotid gland. Ultrasonography should be the initial imaging study used for the examination of salivary gland lesions in children. In newborns, acute suppurative sialoadenitis is associated with gavage-feeding, prematurity and methicillinresistant Staphylococcus aureus. In the infant, the suppurative process is associated with bacteremia, abscess formation and the need of incision and drainage. Initial management includes systemic antibiotics therapy. Older children develop mumps sialadenitis from improper immunization. HIV infection is an important cause of viral parotitis in children.

References: 1- Pershall KE, Koopmann CF Jr, Coulthard SW: Sialadenitis in children. Int J Pediatr Otorhinolaryngol. 11(2):199-203, 1986 2- Wang SL, Zou ZJ, Yu SF, Zhu JR: Recurrent swelling of parotid glands and Sjgren's syndrome. Int J Oral Maxillofac Surg. 22(6):362-5, 1993 3- Garcia CJ, Flores PA, Arce JD, Chuaqui B, Schwartz DS: Ultrasonography in the study of salivary gland lesions in children. Pediatr Radiol. 28(6):418-25, 1998 4- Fraser GC. Salivary Glands. In: O'Neill JA, Rowe MI, Grosfeld JL, et al., Pediatric Surgery, 5th ed. St. Louis, MO: MosbyYear Book, 1998: 727-735. 5- Brook I: Aerobic and anaerobic microbiology of suppurative sialadenitis. J Med Microbiol. 51(6):526-9, 2002 2/12



6- McAdams RM, Mair EA, Rajnik M: Neonatal suppurative submandibular sialadenitis: case report and literature review. Int J Pediatr Otorhinolaryngol. 69(7):993-7, 2005

Volume 26 No 02 FEBRUARY 2006

Mucocele of the appendix is a rare condition described when a thin-walled dilated appendix filled with mucous (cystic mass) is encountered in the right lower quadrant. Chronic obstruction of the appendiceal lumen is the most common etiological factor for a mucocele to appear. The appendiceal obstruction can be caused by lymph node mucosal hyperplasia or a tumorous mass such as a cystadenoma, adenocarcinoma or carcinoid. An excess of mucous is secreted in the face of proximal obstruction enlarging the appendix lumen. Clinically, the mucocele can present with chronic abdominal pain, an abdominal mass as an incidental finding during surgery or imaging. Most cases are seen in older adults with distinct female predominance. Typical ultrasound findings are a cystic mass with variable internal echogenicity, layered wall (onion skin sign), and calcifications in the wall. CT findings are those of a well-encapsulated cystic mass with a wall of variable thickness. If untreated, mucoceles may rupture producing a potentially fatal entity known as pseudomyxoma peritonei. Management is open surgical resection. Appendectomy is used for simple mucocele or for cystadenoma. Right hemicolectomy is recommended for cystadenocarcinoma. Laparoscopic resection is contraindicated due to possible dissemination of the tumorous mass.

References: 1- Madwed D, Mindelzun R, Jeffrey RB Jr: Mucocele of the appendix: imaging findings. AJR Am J Roentgenol. 159(1):69-72, 1992 2- Soweid AM, Clarkston WK, Andrus CH, Janney CG: Diagnosis and management of appendiceal mucoceles. Dig Dis. 16(3):183-6, 1998 3- Haritopoulos KN, Brown DC, Lewis P, Mansour F, Eltayar AR, Labruzzo C, Hakim NS: Appendiceal mucocoele: a case report and review of the literature. Int Surg. 86(4):259-62, 2001 4- Caspi B, Cassif E, Auslender R, Herman A, Hagay Z, Appelman Z: The onion skin sign: a specific sonographic marker of appendiceal mucocele. J Ultrasound Med. 23(1):117-21, 2004 5- Rampone B, Roviello F, Marrelli D, Pinto E: Giant appendiceal mucocele: report of a case and brief review. World J Gastroenterol. 11(30):4761-3, 2005 6- Blecha MJ, Gupta A, Hoover JD, Madonna MB: Chronic abdominal pain secondary to a mucous cystadenoma of the appendix in a 10-year-old boy. J Pediatr Surg. 40(11):1792-4, 2005

Hemihypertrophy refers to soft tissue and bone overgrowth of one half of the body of the child, others time the upper or lower extremity might be solely affected. Hemihypertrophy can be congenital (idiopathic), or most of time the manifestation of a medical condition such as chronic lymphedema, lymphangiomatous malformations, neurofibromatosis, vascular malformations including the Klippel-Trenaunay-Weber syndrome, macrodystrophia lipomatosa, multiple enchondromatosis, or Maffucci's and Proteus syndrome. The idiopathic congenital variety of hemihypertrophy is a rare condition seen infrequently, but associated with a high malignant 3/12



potential. Diagnosis is always performed at birth or in the weeks following birth. The increase growth rate and temperature changes in the hypertrophied side increases the ipsilateral oncogenic potential. Hemihypertrophy is cosmetically unsightly and the psychological impact can be quite prolonged. Idiopathic hemihypertrophy is sometimes associated with primitive neoplasms of the liver, adrenals and kidneys, mild mental retardation, genitourinary anomalies, as well as with benign organ growth aberrations. Children developing scoliosis can be managed with orthopedic support or limb lengthening procedures. Regular clinical surveillance using yearly ultrasound for abdominal tumors is recommended. Hemihypertrophy is usually not inherited.

References: 1- Beals RK: Hemihypertrophy and hemihypotrophy. Clin Orthop Relat Res. (166):199-203, 1982 2- Levine C: The imaging of body asymmetry and hemihypertrophy. Crit Rev Diagn Imaging. 31(1):1-80, 1990 3- Parker L, Kollin J, Vicario D, Nguyen T: Hemihypertrophy as possible sign of renal cell carcinoma. Urology. 40(3):286-8, 1992 4- Stoll C, Alembik Y, Steib JP, De Saint-Martin A: Twelve cases with hemihypertrophy: etiology and follow up. Genet Couns. 4(2):119-26, 1993 5- Leung AK, Fong JH, Leong AG: Hemihypertrophy. J R Soc Health. 122(1):24-7, 2002 6- Memon MA, Mohanty S, Das K, Garg I, D'Cruz AL: Hemihypertrophy, renal dysplasia and benign nephromegaly. Pediatr Nephrol. 20(6):821-3, 2005

Buried Penis
Buried or hidden penis is a congenital anomaly in which the penile foreskin is not attached normally to the shaft so that the penis appears to be totally absent. The effect produces a pseudomicropenis in an otherwise healthy obese child. This is different to when an obese child has a thick suprapubic subcutaneous fat pad that conceals the penis. Buried penis includes an excessive development of the penile fascia which retracts the penis, insufficient attachment of the penile skin at the base of the penis, often excessive prepubic fat worsening the appearance of the abnormality but not by itself totally explaining it, and a tight phimosis often present. A surgical approach to the buried penis is warranted in most circumstances. There are psychological benefits to both the patients and the parents. The basis for surgical correction is directed at freeing the penile shaft from abnormal dartos attachments, fixing dartos fascia to Buck's fascia to prevent retraction of the penis, and providing adequate shaft skin coverage with the inner preputial skin. The surgical procedure usually brings significant cosmetic and functional improvement to the penis. Additional procedures are rarely needed due to recurrent retraction.

References: 1- Chuang JH: Penoplasty for buried penis. J Pediatr Surg. 30(9):1256-7, 1995 2- Lipszyc E, Pfister C, Liard A, Mitrofanoff P: Surgical treatment of buried penis. Eur J Pediatr Surg. 7(5):292-5, 1997 3- Alter GJ, Ehrlich RM: A new technique for correction of the hidden penis in children and adults. J Urol. 161(2):455-9, 1999 4- Kojima Y, Hayashi Y, Maruyama T, Sasaki S, Mogami T, Ueda K, Kohri K: Correction of completely buried penis using the modified preputial island pedicle flap method. J Pediatr Surg. 34(10):1524-6, 1999 5- Brisson P, Patel H, Chan M, Feins N: Penoplasty for buried penis in children: report of 50 cases. J Pediatr Surg. 36(3):4215, 2001 6- Herndon CD, Casale AJ, Cain MP, Rink RC: Long-term outcome of the surgical treatment of concealed penis. J Urol. 170(4 Pt 2):1695-7, 2003




Volume 26 No 03 MARCH 2006

Situs Inversus
Situs inversus is a defect of lateralization that results in mirror image positioning of abdominal and thoracic viscera. Complete situs inversus is estimated to occur in one per 8000 persons and may be isolated, associated with cardiac or alimentary tract malformations (duodenal obstruction and midgut volvulus), or occur as a component of Kartagener's syndrome, with autosomic recessive inheritance and associated disturbance of ciliary function, causing bronchiectasis, chronic sinusitis and male infertility. Situs inversus can present with dextrocardia, levocardia or partial heterotaxia. Children with situs inversus and no cardiac lesion are older than two months when diagnosed presenting vague symptoms of intermittent vomiting, abdominal pain, constipation, diarrhea or failure to thrive. The anomaly causes a rotational defect which can cause midgut volvulus. Situs inversus can be diagnosed from prenatal ultrasound. An UGIS and barium enema will help diagnosed the intestinal rotation and fixation anomaly. Liver/spleen scans will determine the presence or absence of the spleen. A prophylactic Ladd procedure has proven to be both safe and effective in preventing intestinal volvulus or obstruction from congenital bands. Some workers advocate use of laparoscopy in malrotation associated with situs inversus since it's minimally invasive, accomplish the same surgical act and is associated with fewer postoperative adhesions.

References: 1- Ruben GD, Templeton JM Jr, Ziegler MM: Situs inversus: the complex inducing neonatal intestinal obstruction. J Pediatr Surg. 18(6):751-6, 1983 2- Tryfonas GI, Chaidos C, Avtzoglou PP, Zioutis J, Klokaris A, Papanastasopoulos A: Partial situs inversus: duodenal obstruction in a neonate with isolated levocardia. J Pediatr Surg. 27(12):1584-6, 1992 3- Chang J, Brueckner M, Touloukian RJ: Intestinal rotation and fixation abnormalities in heterotaxia: early detection and management. J Pediatr Surg. 28(10):1281-4, 1993 4- Cheikhelard A, De Lagausie P, Garel C, Maintenant J, Vuillard E, Blot P, Aigrain Y: Situs inversus and bowel malrotation: contribution of prenatal diagnosis and laparoscopy. J Pediatr Surg. 35(8):1217-9, 2000 5- Mordehai J, Cohen Z, Kurzbart E, Mares AJ: Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report. J Pediatr Surg. 37(4):E5, 2002 6- Nawaz A, Matta H, Hamchou M, Jacobez A, Trad O, Al Salem AH: Situs inversus abdominus in association with congenital duodenal obstruction: a report of two cases and review of the literature. Pediatr Surg Int. 21(7):589-92, 2005

Pre-duodenal Portal Vein Revisited

Pre-duodenal portal vein (PDPV) is a rare congenital vascular anomaly which is often symptomless leading to duodenal intestinal obstruction requiring surgical correction. PDPV is frequently associated with other congenital malformations, among which, and in order of frequency, are duodenal stenosis and atresia, abnormalities of the biliary tract (atresia and duplications), annular pancreas, and malrotation (intestinal, situs inversus). The duodenal atresia associated with PDPV is intrinsic and not due to the external pressure of the anomalous vein on the duodenum. PDPV occurs because of persistence of a preduodenal vitelline communicating vein. Most children with PDPV presents with bowel obstruction and two-thirds are detected in the first two weeks of life. Identification of PDPV is rarely made preoperative. Prenatal Ultrasound has demonstrated the unusual position of the portal vein in cases of PDPV. Presence of PDPV complicates any duodenal surgery since the integrity of the vessel must be preserved to avoid portal vein thrombosis. Management consists of duodenoduodenal bypass anastomosis anterior to the portal vein. Another 5/12



alternative is gastrojejunostomy with truncal vagotomy.

References: 1- Esscher T: Preduodenal portal vein--a cause of intestinal obstruction? J Pediatr Surg. 15(5):609-12, 1980 2- Patti G, Marrocco G, Mazzoni G, Catarci A: Esophageal and duodenal atresia with preduodenal common bile duct and portal vein in a newborn. J Pediatr Surg. 20(2):167-8, 1985 3- Fernandes ET, Burton EM, Hixson SD, Hollabaugh RS: Preduodenal portal vein: surgery and radiographic appearance. J Pediatr Surg. 25(12):1270-2, 1990 4- Choi SO, Park WH: Preduodenal portal vein: a cause of prenatally diagnosed duodenal obstruction. J Pediatr Surg. 30(10):1521-2, 1995 5- Mordehai J, Cohen Z, Kurzbart E, Mares AJ: Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: A case report. J Pediatr Surg. 37(4):E5, 2002

Congenital Melanocytic Nevus

Skin nevus found in infants and children can be a source of concern to both parents and physician dealing with pediatric patients. Congenital melanocytic nevus (CMN) is a characteristic pigmented nevus with mild raised borders and a strong skin discoloration caused by melanin deposits. Incidence of CMN is 0.2% with more than 90% small nevi (< 1.5 cm). Congenital melanocytic nevus may cause cosmetic defects and represent a risk of malignant transformation, namely melanoma. The incidence of developing melanoma is associated with location and size of the CMN; those identified in the face and neck or covering large areas of the body (larger than 5 cm) has a higher incidence of malignant transformation. Not always there is a correlation between pre and postoperative diagnosis after prophylactic excision of a nevi. Digital videomicroscopy using polarize light can help increase the diagnostic yield of CMN. A high index of suspicion for cutaneous melanoma is needed by clinicians assessing melanocytic lesions in children and adolescents for early diagnosis taking into consideration change in color, growth rate, and bleeding, For large lesions and those in exposed areas of the body, surgical excision is the treatment of choice. Medium and small lesions can be managed with Ruby laser treatment. Ruby laser treatment does not result in scarring, mutilation, or functional impairment.

References: 1- Kruk-Jeromin J, Lewandowicz E, Rykala J: Surgical treatment of pigmented melanocytic nevi depending upon their size and location. Acta Chir Plast. 41(1):20-4, 1999 2- Schmid-Wendtner MH, Berking C, Baumert J, Schmidt M, Sander CA, Plewig G, Volkenandt M: Cutaneous melanoma in childhood and adolescence: an analysis of 36 patients. J Am Acad Dermatol. 46(6):874-9, 2002 3- Makkar HS, Frieden IJ: Congenital melanocytic nevi: an update for the pediatrician. Curr Opin Pediatr. 14(4):397-403, 2002 4- Noordzij MJ, van den Broecke DG, Alting MC, Kon M: Ruby laser treatment of congenital melanocytic nevi: a review of the literature and report of our own experience. Plast Reconstr Surg. 114(3):660-7, 2004 5- Zaal LH, Mooi WJ, Sillevis Smitt JH, van der Horst CM: Classification of congenital melanocytic naevi and malignant transformation: a review of the literature. Br J Plast Surg. 57(8):707-19, 2004

Volume 26 No 04 APRIL 2006

Transverse Vaginal Septum 6/12



Transverse vaginal septum is a congenital condition of females that can block the passage of vaginal secretions causing primary amenorrhea, hematocolpus and cyclic pelvic pain. Different to imperforate hymen, in a transverse vaginal septum you find a rim of nonbulging hymenal tissue on the vestibular floor with an intravaginal bulging membrane. The septum can be found in the upper, middle or lower vagina varying in thickness. Most common location of the septum is the upper vagina. Histologically, the diagnosis of transverse vaginal septum is made due to the presence of mllerian duct (mesodermal origin) tissue in the septum. Transverse vaginal septum is a defect of vertical fusion during embryogenesis of the vagina. The estimated incidence is one per 30,000 to 84,000 women. It is sometimes associated with genitourinary tract, gastrointestinal tract, musculoskeletal, and cardiac malformations. Physical exam and pelvic ultrasound are diagnostic. Surgical resection is the treatment of choice. The mucosa on either side of the blockage should be mobilize for approximation with interrupted sutures, while the underlying fibrous septum should be excised. Postoperative dilation may be necessary to prevent restenosis and dyspareunia. Patients with a complete transverse septum in the middle or upper vagina are less likely to conceive than patients with a septum in the lower vagina. Prompt diagnosis and surgical correction to drain accumulated blood may preserve fertility possibly through the prevention of endometriosis.

References: 1- Rock JA, Zacur HA, Dlugi AM, Jones HW Jr, TeLinde RW: Pregnancy success following surgical correction of imperforate hymen and complete transverse vaginal septum. Obstet Gynecol. 59(4):448-51, 1982 2- Ahmed S, Morris LL, Atkinson E: Distal mucocolpos and proximal hematocolpos secondary to concurrent imperforate hymen and transverse vaginal septum. J Pediatr Surg. 34(10):1555-6, 1999 3- Quinn T, Erickson V, Knudson MM: Down's syndrome, precocious puberty, and transverse vaginal septum: an unusual cause of abdominal pain. J Pediatr Surg. 36(4):641-3, 2001 4- Rana A, Manandhar B, Amatya A, Baral J, Gurung G, Giri A, Giri K: Mucocolpos due to complete transverse septum in middle third of vagina in a 17-year-old girl. J Obstet Gynaecol Res. 28(2):86-8, 2002 5- Beyth Y, Klein Z, Weinstein S, Tepper R: Thick transverse vaginal septum: expectant management followed by surgery. J Pediatr Adolesc Gynecol. 17(6):379-81, 2004

Septated Vagina
A septate vagina is another congenital condition that occurs from failure of longitudinal fusion of the lower mllerian ducts leaving a variable amount of an asymptomatic longitudinal vaginal septum in the child. Two vaginal canals are created by the septum completely or partially. As time passes the septate vagina goes unnoticed until the adolescent child start to use tampons or engage in sexual activity. The affected patient might complain of menstrual leakage with the use of tampons since menstruation will continue to egress from the other vaginal canal not occluded by the tampon. This is the case with complete duplication of the mllerian system. Reassurance and vaginal hygiene is all that is required in these cases. Physical exams including endoscopy along with pelvic ultrasound are diagnostic. The identification of a duplicated cervix and a vaginal septum is consistent with several uterine malformations, which leads to frequent misdiagnosis and errors in management. On the other hand when the defect is an isolated partial vaginal septum the patient will complain of dyspareunia during sexual intercourse. Management in these cases consists of excision of the thick septa (septectomy) maintaining good hemostasis.

References: 1- Tolete-Velcek F, Hansbrough F, Kugaczewski J, Coren CV, Klotz DH, Price AF, Laungani G, Kottmeier PK: Utero vaginal 7/12



malformations: a trap for the unsuspecting surgeon. J Pediatr Surg. 24(8):736-40, 1989 2- Mills PL, Pergament E: Urorectal septal defects in a female and her offspring. Am J Med Genet. 13;70(3):250-2, 1997 3- Haddad B, Barranger E, Paniel BJ: Blind hemivagina: long-term follow-up and reproductive performance in 42 cases. Hum Reprod. 14(8):1962-4, 1999 4- Patton PE, Novy MJ, Lee DM, Hickok LR: The diagnosis and reproductive outcome after surgical treatment of the complete septate uterus, duplicated cervix and vaginal septum. Am J Obstet Gynecol. 190(6):1669-75, 2004

Direct Inguinal Hernias

Direct inguinal hernias in infants and children are extremely rare comprising less than 2% of all inguinal hernias. Most inguinal hernias in children are of the indirect type where the defect and sac runs along the spermatic cord structures from the internal ring. Direct inguinal hernias occur due to a defect in the transversalis fascia presenting as a bulge medially in the groin. A direct hernia should be suspected when operating for an inguinal hernia and a typical indirect inguinal hernia is not found. Otherwise, most direct inguinal hernias in children are the result of a recurrence after initial repair of an indirect hernia where the inguinal floor was injured during the surgical procedure. During repair of a direct inguinal hernia a defect in the posterior wall of the inguinal canal medial to the epigastric vessels will be identified. The defect is repaired joining the transversalis fascia between the inguinal ligament and the conjoined tendon with interrupted nonabsorbable sutures (Cooper's ligament -McVay- repair). Laparoscopic repair of the common inguinal hernia in the child has reported a higher incidence of direct inguinal hernia.

References: 1- Viidik T, Marshall DG: Direct inguinal hernias in infancy and early childhood. J Pediatr Surg. 15(5):646-7, 1980 2- Gnidec AA, Marshall DG: Incarcerated direct inguinal hernia containing uterus, both ovaries, and fallopian tubes. J Pediatr Surg. 21(11):986, 1986 3- Wright JE, Gill AW: Direct inguinal hernias in the newborn. Aust N Z J Surg. 61(1):78-81, 1991 4- Grosfeld JL, Minnick K, Shedd F, West KW, Rescorla FJ, Vane DW: Inguinal hernia in children: factors affecting recurrence in 62 cases. J Pediatr Surg. 26(3):283-7, 1991 5- Schier F, Montupet P, Esposito C: Laparoscopic inguinal herniorrhaphy in children: a three-center experience with 933 repairs. J Pediatr Surg. 37(3):395-7, 2002 6- Graf JL, Caty MG, Martin DJ, Glick PL: Pediatric hernias. Semin Ultrasound CT MR. 23(2):197-200, 2002 7- Schier F, Klizaite J: Rare inguinal hernia forms in children. Pediatr Surg Int. 20(10):748-52, 2004

Volume 26 No 05 MAY 2006

The thymus remains quite prominent in the anterior mediastinum during the first year of life causing discrepancy between a normal and hyperplastic gland. Involution occurs in response to stress and sepsis. Rebound hyperplasia after involution can be seen after cardiac surgery, major burns and chemotherapy. Thymoma is the most common neoplastic tumor found in the thymus of children and adults. There is a close relationship between myasthenia gravis and thymoma. Most thymic tumors in children are benign, share a low rate of association with myasthenia gravis and a favorable prognosis. Thymomas are considered malignant on the basis of macroscopic and microscopic capsular invasiveness. The most significant predictors of long-term survival of thymoma include complete excision, stage I disease, and lymphocytic histology. Management of 8/12



thymoma entails surgical resection through a median sternotomy. To increase survival a policy of aggressive, complete surgical resection of all thymomas is advice. Thymoma behaves as a rather indolent tumor, with most deaths from causes unrelated to thymoma or its direct treatment. Chemotherapy is reserved for patients with refractory or metastatic disease. Thymomas are moderately radiosensitive but radiation therapy is not an attractive option for children due to sideeffects on developing organs.

References: 1- Ramon y Cajal S, Suster S: Primary thymic epithelial neoplasms in children. Am J Surg Pathol. 15(5):466-74, 1991 2- Kaplinsky C, Mor C, Cohen IJ, Goshen Y, Yaniv I, Tamary H, Jaber L, Stark B, Stern S, Zaizov R: Childhood malignant thymoma: clinical, therapeutic, and immunohistochemical considerations. Pediatr Hematol Oncol. 9(3):261-8, 1992 3- Pescarmona E, Giardini R, Brisigotti M, Callea F, Pisacane A, Baroni CD: Thymoma in childhood: a clinicopathological study of five cases. Histopathology. 21(1):65-8, 1992 4- Wilkins KB, Sheikh E, Green R, Patel M, George S, Takano M, Diener-West M, Welsh J, Howard S, Askin F, Bulkley GB: Clinical and pathologic predictors of survival in patients with thymoma. Ann Surg. 230(4):562-72, 1999 5- Dhall G, Ginsburg HB, Bodenstein L, Fefferman NR, Greco MA, Chang MW, Gardner S: Thymoma in children: report of two cases and review of literature. J Pediatr Hematol Oncol. 26(10):681-5, 2004 6- Rothstein DH, Voss SD, Isakoff M, Puder M: Thymoma in a child: case report and review of the literature. Pediatr Surg Int. 21(7):548-51, 2005

Peritoneal Dialysis
Peritoneal dialysis is the preferred technique of management utilized in almost two-third of children with chronic renal failure. The most common complications of peritoneal dialysis are peritonitis, catheter infection and dialysate leaks. Catheter infection risk is higher among children less than five years of age or with a previous history of infection. Infection occurs because the dialysate causes an alteration in the normal protective mechanism of the peritoneum reducing the number and function of macrophages. In the acute setting the peritoneal catheter can be placed percutaneously into the peritoneum using the Seldinger technique or alternatively the child hemodyalised using a central venous access (Quinton Catheter). In the elective situation the child is taken to the operating room and a peritoneal (Tenckhoff) cannula placed under direct vision into the peritoneum under general anesthesia. The open or laparoscopic technique permits removal of the omentum (omentectomy) to avoid later occlusion of the cannula during passive effusion of the solution. The catheter should go through the muscles (rectus) and point inferiorly to reduce the incidence of catheter infection. Leakage and adequate fluid effusions (at least 80% of the unfused fluid should drain back rapidly) are tested before terminating the surgical procedure. The cannula is ready to be used, but low volumes should be initially utilized to minimized incisional pain and reduce leaks.

References: 1- Warady BA, Sullivan EK, Alexander SR: Lessons from the peritoneal dialysis patient database: a report of the North American Pediatric Renal Transplant Cooperative Study. Kidney Int Suppl. 53:S68-71, 1996 2- Honda M, Iitaka K, Kawaguchi H, Hoshii S, Akashi S, Kohsaka T, Tuzuki K, Yamaoka K, Yoshikawa N, Karashima S, Itoh Y, Hatae K: The Japanese National Registry data on pediatric CAPD patients: a ten-year experience. A report of the Study Group of Pediatric PD Conference. Perit Dial Int. 16(3):269-75, 1996 3- Lewis MA, Smith T, Roberts D: Peritonitis, functional catheter loss and the sitting of the Dacron cuff in chronic peritoneal dialysis catheters in children. Eur J Pediatr Surg. 6(5):285-7, 1996 4- Beanes SR, Kling KM, Fonkalsrud EW, Torres M, Salusky IB, Quinones-Baldrich WJ, Atkinson JB: Surgical aspects of 9/12



dialysis in newborns and infants weighing less than ten kilograms. J Pediatr Surg. 35(11):1543-8, 2000 5- Leblanc M, Ouimet D, Pichette V: Dialysate leaks in peritoneal dialysis. Semin Dial. 14(1):50-4, 2001 6- Daschner M, Gfrorer S, Zachariou Z, Mehls O, Schaefer F: Laparoscopic Tenckhoff catheter implantation in children. Perit Dial Int. 22(1):22-6, 2002 7- Washburn KK, Currier H, Salter KJ, Brandt ML: Surgical technique for peritoneal dialysis catheter placement in the pediatric patient: a North American survey. Adv Perit Dial. 20:218-21, 2004

Myasthenia Gravis
Children constitute 10% of all cases of Myasthenia Gravis (MG) with three individual forms identified: neonatal, genetic or juvenile. The neonatal phase is transient, associated with a newborn whose mothers have MG and the baby recovers completely after several days or weeks. Genetic MG is not associated to a parent with MG with symptoms confined to ptosis and almost no weakness. The juvenile phase of MG is similar to the adult phase occurring after the age of ten. Symptoms include fluctuating weakness and fatigue in the ocular (diplopia), facial (ptosis), bulbar or limb muscles. weakness, fatigability, ptosis and diplopia. The child develops motor weakness, preservation of sensation, coordination and deep tendon reflex. MG is an autoimmune disease in which there is loss of acetylcholine receptors at the neuromuscular junction. Thymic enlargement occurs in patients with MG. MG is best managed: 1) enhancing neuromuscular transmission with cholinesterase inhibitors though the effect is partial with time; 2) using immune suppression with steroids, azathioprine or cyclophosphamide; 3) with short term immune therapy including plasma exchange or intravenous immune globulin; 4) removal of the thymus (thymectomy) if its enlarged or the child has increase medication requirements.

References: 1- Vincent A: Acetylcholine receptors and myasthenia gravis. Clin Endocrinol Metab. 12(1):57-78, 1983 2- Lopate G, Pestronk A: Autoimmune myasthenia gravis. Hosp Pract 15;28(1):109-12, 1993 3- Shillito P, Vincent A, Newsom-Davis J: Congenital myasthenic syndromes. Neuromuscul Disord. 3(3):183-90, 1993 4- Marx A, Wilisch A, Schultz A, Gattenlohner S, Nenninger R, Muller-Hermelink HK: Pathogenesis of myasthenia gravis. Virchows Arch. 430(5):355-64, 1997 5- Anlar B: Juvenile myasthenia: diagnosis and treatment. Paediatr Drugs. 2(3):161-9, 2000 6- Andrews PI: Autoimmune myasthenia gravis in childhood. Semin Neurol. 24(1):101-10, 2004

Volume 26 No 05 MAY 2006

Traumatic Diaphragmatic Hernia
Motor vehicle trauma is the leading cause of an acquired diaphragmatic hernia in a child and adult. The traumatic event can either be penetrating directly injuring the diaphragm, or most commonly blunt abdominal causing a sharp increase in intraabdominal pressure with rupture of the diaphragmatic muscle. The more medial and lateral fibers of the posterior diaphragm arising from the lumbocostal arch and the vertebrocostal trigone are the weakest points of rupture. The 10/12



posterolateral portion is virtually always the area that ruptures with trauma. Diaphragmatic injuries are difficult to diagnose preoperatively and can be missed easily. Traumatic diaphragmatic hernia should be suspected on the basis of an abnormal chest radiograph in the trauma victim with multiple injuries. If diaphragmatic injury is suspected, ultrasound or CT Scan investigation must be performed. Most cases involve the left diaphragm due to the buttressing effect of the liver. The incidence of bowel strangulation is high in traumatic diaphragmatic hernias. In the acute setting, transabdominal repair after palpation of both hemidiaphragms is the procedure of choice because of the high incidence of associated trauma. Injury severity score and hemorrhagic shock upon admission strongly influence the outcome. Delayed presentation can be repaired through the chest.

References: 1- Brown GL, Richardson JD: Traumatic diaphragmatic hernia: a continuing challenge. Ann Thorac Surg. 39(2):170-3, 1985 2- Sukul DM, Kats E, Johannes EJ: Sixty-three cases of traumatic injury of the diaphragm. Injury. 22(4):303-6, 1991 3- Meyers BF, McCabe CJ: Traumatic diaphragmatic hernia. Occult marker of serious injury. Ann Surg. 218(6):783-90, 1993 4- Catasca JV, Siegel MJ: Posttraumatic diaphragmatic herniation: CT findings in two children. Pediatr Radiol. 25(4):262-4, 1995 5- Ramos CT, Koplewitz BZ, Babyn PS, Manson PS, Ein SH: What have we learned about traumatic diaphragmatic hernias in children? J Pediatr Surg. 35(4):601-4, 2000 6- Mihos P, Potaris K, Gakidis J, Paraskevopoulos J, Varvatsoulis P, Gougoutas B, Papadakis G, Lapidakis E: Traumatic rupture of the diaphragm: experience with 65 patients. Injury. 34(3):169-72, 2003

Mediastinal Teratomas
Mediastinal teratomas are rare tumors that originate in the anterior mediastinum and comprise almost one-fifth of all mediastinal masses. Most grow to large size before causing symptoms. Mediastinal teratoma can appear in any age of the child. Primary symptom is respiratory distress caused by airway compression, followed by feeding problems related to dyspnea, coughing, wheezing and chest pain. Most mediastinal teratomas are mature and benign. Teratomas arise from pluripotent cells and are composed of a wide diversity of tissues originating from three germ layers ectoderm, mesoderm and endoderm. Besides an anterior mediastinal mass, plain chest films can show calcifications. CT Scan is the study of choice to demonstrate the extent of the tumor and its relationship with other structures. As with any other suspected teratoma preoperative alpha fetoprotein and human chorionic gonadotropin markers levels should be obtained. Teratomas are classified as mature, immature and malignant. Mature teratomas are predominantly cystic, while malignant teratomas are mainly solid lesions. Immature teratomas have immature tissue with mature elements. Surgical excision through a median sternotomy is the treatment of choice for mediastinal teratomas. Adjuvant chemotherapy is used in immature and malignant teratoma to increase survival.

References: 1- Quillin SP, Siegel MJ: CT features of benign and malignant teratomas in children. J Comput Assist Tomogr. 16(5):722-6, 1992 2- Lakhoo K, Boyle M, Drake DP: Mediastinal teratomas: review of 15 pediatric cases. J Pediatr Surg. 28(9):1161-4, 1993 3- Arai K, Ohta S, Suzuki M, Suzuki H: Primary immature mediastinal teratoma in adulthood. Eur J Surg Oncol. 23(1):64-7, 1997 4- Hiroshima K, Toyozaki T, Iyoda A, Yusa T, Fujisawa T, Ohwada H: Apoptosis and proliferative activity in mature and immature teratomas of the mediastinum. Cancer. 92(7):1798-806, 2001 11/12



5- Koga H, Yamataka A, Kobayashi H, Miyamoto H, Lane GJ, Miyano T: Median sternotomy provides excellent exposure for excising anterior mediastinal tumors in children. Pediatr Surg Int. 21(11):864-7, 2005

Juvenile Secretory Carcinoma

Carcinoma of the breast in a child is a rare pathologic entity, constitutes less than 1% of all breast lesions in this age group. Juvenile secretory carcinoma is an uncommon malignant tumor that can develop in the breasts of both sexes with a mean age of occurrence at 10 years. It's a slow growing tumor that can recur locally and metastasize to the ipsilateral axillary lymph nodes. Juvenile secretory carcinoma component are devoid of estrogen and progesterone receptors. The child develops a painless mass often near the areola in the breast early in life. The lesion is well demarcated and unencapsulated invading the adjacent tissue. Immunohistochemical staining for alpha lactalbumin is present. When in doubt fine needle aspiration biopsy (cytology) can provide a preoperative diagnosis of the lesion. In all cases, the samples are cellular and feature diffuse, prominent, intracytoplasmic vacuoles and secretion in malignant cells with occasional signet-ring like forms. Management consists of simple mastectomy (wide local excision) with sentinel axillary lymph node biopsy, especially in males cases were metastasis to the axilla are more common. Biological behavior seems to be similarly favorable in both sexes.

References: 1- Ferguson TB Jr, McCarty KS Jr, Filston HC: Juvenile secretory carcinoma and juvenile papillomatosis: diagnosis and treatment. J Pediatr Surg. 22(7):637-9, 1987 2- Rosen PP, Cranor ML: Secretory carcinoma of the breast. Arch Pathol Lab Med. 115(2):141-4, 1991 3- Serour F, Gilad A, Kopolovic J, Krispin M: Secretory breast cancer in childhood and adolescence: report of a case and review of the literature. Med Pediatr Oncol. 20(4):341-4, 1992 4- Gupta RK, Kenwright D, Naran S, Lallu S, Fauck R: Fine needle aspiration cytodiagnosis of secretory carcinoma of the breast. Cytopathology. 11(6):496-502, 2000 5- de Bree E, Askoxylakis J, Giannikaki E, Chroniaris N, Sanidas E, Tsiftsis DD: Secretory carcinoma of the male breast. Ann Surg Oncol. 9(7):663-7, 2002 6- Bond SJ, Buchino JJ, Nagaraj HS, McMasters KM: Sentinel lymph node biopsy in juvenile secretory carcinoma. J Pediatr Surg. 39(1):120-1, 2004

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