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PEDIATRIC SURGERY UPDATE

VOLUME 13, 1999

Volume 13 No 01 JULY 1999

Tongue Cysts

Cysts in the tongue are rare in children. The differential diagnosis includes: mucoceles (retention cyst), cystic hygroma, thyroglossal cysts, cysts of foregut origin

presents at birth interfering with mouth closure, swallowing, normal feeding or even causing respiratory problems. Complications of the cyst include infection, he

to increase in size and asphyxia, inability to feed and aspiration pneumonia. Mucoceles (ranula) may appear in the base of the tongue and needs excision or mars

can be a cystic hygroma in the tongue that may need partial glossectomy or management with sclerosing substances. Lingual thyroglossals are found in the base o

be managed with marsupialization without excision or Sis trunk procedure. Cysts of foregut origin prese nts in the neonatal period, can be found in the anterior part

needing complete surgical excision through a sagittal glossal split. Cyst aspiration is inadequate definitive treatment. A teratoma likewise needs surgical excision

References

1- Urao M, Teitelbaum DH, Miyano T: Lingual thyroglossal duct cyst: a unique surgical approach. J Pediatr Surg 31(11):1574-6, 1996

2- Surana R, Losty P, Fitzgerald RJ: Heterotopic gastric cyst of the tongue in a newborn. Eur J Pediatr Surg 3(2):110-1, 1993

3- Wiersma R, Hadley GP, Bosenb erg AT, Chryst al V: Intralingual cys ts of foregu t origin. J Pediatr Surg 27(11):1404-6, 19924- Velcek FT, Klotz DH, Hill CH, Ladogana LE: Tongue lesions in children. J Pediatr Surg 14(3):238-46, 1979

Primary Immunodeficiency

The body mounts an immune response by producing antibodies (B cells), inciting cellular division (T cells), cascading complement and producing phagocytosis. Co

deficiency of this response not associated to a secondary illness is known as primary immunodeficiency (PI). PI is very rare (400 new cases/year in the USA), and

antibody (most common), T-cell, combined T and B-cell, phagocytic or complement activity. Most case s of PI are identified in the first year of life. Primary sympt

with unusual organisms (low pathogenicity) that is severe, prolonged and resistant to conventional therapy. T-cell deficiency occurs before the age of six months

presents clinically after this age due to transplacental protection. Phagocytic and complement deficiency is present since birth as the baby might show a lack of p

umbilical cord detachment. After respiratory tract, the skin is the 2nd most common site of infection, i.e., thrush, furuncles, abscess, fistulas and cellulitis followed

usually appears chronically ill with growth failure. Extensive lab tes ting will establish the diagnosis . Live vaccine should be avoided. The child with PI who will und

needs: protective isolation, antibiotic prophylaxis, nutritional backup, physiologic monitoring and selective replacement of defective substance (immunoglobulins)

agents use. Bone marrow transplant can sometimes correct a PI.

References

1- Peerles AG, Stiehm ER: The Immunoco mpromised Child, In Fonkalsru d and Krummel "Infections and Immunologic Disorders in Pediatric Surgery", WB Saund ers Co, 1993, pag 77-90

2- Stiehm ER: Human intraveno us immunoglobulin in primary and seco ndary antibody deficiencies. Pediatr Infect Dis J 16(7):696-707, 19973- Pachman LM, Lynch PA, Silver RK, Ozog DL, Poznans ki AK: Primary immunodeficiency d isease in ch ildren: an updat e. Curr Probl Pediatr 19(1):1-64, 1989

4- Primary immunod eficiency diseases . Report p repared for the WHO by a s cientific group on immunodeficiency. Clin Immunol Immunopatho l 28(3):450-75, 19835- Pappas BE: Primary immunod eficiency disorders in infancy. Neonatal Netw 18(1):13-22, 1999

Lymphedema

The most common forms of lymphedema in children affect the lower e xtremity. Lymphedema in children can be classified into: congenital, precox and tarda. The

absence (10%) or hypoplasia (90%) of the lymphatic channels of the lower extremity. Diagnosis of lymphedema is made clinically. Congenital lymphedema appea

than one extremity, rarely extends above the knee and enlarges at a slower rate than body growth. The swelling becomes less pronounced with age, and no specif

thirds of cases. Lymphedema precox appears during adolescence, occur mostly in females, extends to the groin and is associated with yellow nails (inadequate ly

Lymphedema tarda occurs spontaneously in middle age people. In unilateral cases a retroperitoneal tumor or venous obstruction should be rule out. Complication

swelling, brawny edema, cellulitis and lymphangitis. Management consists of extremity care, compressive support stocking and leg elevation. In severe cases the

skin and subcutaneous lymphatics (modified Kondoleon procedure).

References

1- Fonkalsrud EW: Surgical management of congenital lymphedema in infants and children. Arch Surg 114(10):1133-6, 1979

2- Hilliard RI, McKendry JBJ, Phillips MJ: Congenital ab normalities o f the lymphatic sy stem: a new clinical classification. Pediatrics 86:988, 1990

3- Levine C: Primary diso rders o f the lymphatic v ess els: a un ified co ncept . J Pediatr Surg 24:233, 1989

4- Fonkalsrud EW: Congenital Malformations of th e Lymphatic Sys tem. Semm Pediatr Surg 3(2): 62-69, 1994

Volume 13 No 02 AUGUST 1999

Segmental Volvulus

Volvulus, an acute surgical emergency, refer to clockwise rotation of the bowel causing lymphatic, venous or arterial occlusion. In malrotation the volvulus include

portion of the duodenum to mid-transverse colon). On rare occasions volvulus involve only the small bowel in a se gmental fashion affecting a portion of jejunum, i

volvulus can be the result of a predisposing anatomical defects such as: congenital bands (a persistent omphalomesenteric band or Meckel diverticulum fixed to t

acquired adhesive bands after a s urgical procedure, abnormal foreshortened mesenteric defect (segmental or basilar), hanging tumors (mesenteric cyst), intralum

bowel (meconium ileus), worms (Ascariasis) and even VP shunts. Clinically, the child will present with sudden intestinal obstruction (bilious vomiting, abdominal di

associated with variable changes of ischemic bowel (shock, metabolic acidosis and dehydration). Recognition of volvulus before infarction occurs is imperative for

Management must be prompt and consist of counterclockwise detorsion of the affected segment, removing the anatomic cause, and depending on the viability of t

rese ction with anastomosis. In broad-base mesenteric root defects (basilar) pexing of the bowel may be needed. Due to the segmental nature loss of massive bow

References

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1- Maung M, Saing H: Intes tinal volvulus : an experience in a developing co untry. J Pediatr Surg 30(5):679-81, 1995

2- Black PR, Mueller D, Crow J, Morris RC, Husain AN: Mesenteric defects as a cause of intestinal volvulus without malrotation and as the possible primary etiology of intestinal atresia. J Pediatr Surg 2

3- Wilkinson TS, Stone HH: Intes tinal volvulus without malrotation in a four-month-old infant. Am Surg 33(5):3665-6, 1967

4- Rescorla FJ, Shedd FJ, Grosfeld JL, Vane DW, Wes t KW: Ano malies of intes tinal rotation in childhood: an alysis o f 447 cases. Surgery 108(4):710-5, 1990

5- Abrahamso n RH: Acute small intestinal vo lvulus. Int Surg 52(3):210-4, 1969

6- Medina V, Diaz H, Lorenzo A , Carrillo A, Gonzalez F: Jejuno-ileal volvulus , a rare cause of intestinal o bst ruction. Rev Esp Enferm Dig 87(12):883-4, 1995

Macklin effect

The most common cause of pneumoperitoneum in neonates is perforated necrotizing enterocolitis followed by spontaneous gastric perforation. In rare occasions p

from ruptured pulmonic blebs (minute rupture in alveoli subjecte d to the stress of mechanical ventilation) dissecting retroperitoneally into the abdomen rather tha

This is known as the Macklin effect published originally in 1943. Precise diagnosis of this type of "medical" pneumoperitoneum will reduce a needless laparotom

suggestions pointing toward this e tiology are the prese nce of interstitial emphysema, retrocardiac pneumomediastinum, pneumothorax, disse ction of air into the s

absence of fluid or meconium in the peritoneum and the presence of air in the stomach in a child with severe pulmonary disease (Hyaline membrane disease) on

Previous X-ray may note the abse nce of dilated bowel loops, edema or pneumatosis. Contrast bowel studies (with water soluble material) and peritoneal paracent

bowel perforation from an intrathoracic origin of the air. Evacuation of the air and supportive management is all that is needed.

References

1- Wintermark M, et a l: Blunt traumatic pneu momediastinum: us ing CT to reveal the Macklin effect.. Am J Roen tgeno l. 172(1):129-30, 1999

2- Brotman S, et al: The Macklin effect: a caus e of pn eumoperitoneum. Md State Med J. 30(9):30, 1981

3- Rosenfeld DL, Cordell CE, Jadeja N: Retrocardiac pneumomediastinum: radiograph ic finding and clinical implications . Pediatrics 85(1):92-7, 1990

4- Cohen MD, Schreiner R, Lemons J: Neona tal pneu moperitoneum without s ignificant adven titious p ulmonary air: Use of metrizamide to ru le out p erforation of the b owel. Pediatrics 69(5):587-9, 1982

5- Zerella JT, McCullough JY: Pneumoperitoneum in infants without g astro intest inal perforation. Surgery 89(2):163-7, 1981

6- Summers B: Pneumoperitoneum as soc iated with artificial vent ilation. Br Med J 1:1528-30, 1979

7- Steves M, Ricketts RR: Pneumoperitoneu m in the n ewborn infant . Am Surg 53(4):226-30, 1987

8- Campbell RE, Boggs TR Jr, Kirkpatrick JA Jr: Early neon atal pn eumoperitoneum from prog ress ive mass ive tens ion pn eumomediastinum. Radiology 114(1):121-6, 1975

Mesocolic Hernias

Mesocolic hernias (MH) are rare congenital malformations accounting for one-third of all internal hernias and arising from an error of rotation of the midgut with

intestine beneath the developing colon. Right (Waldeyer's hernia) and left MH are distinct entities varying in embryological origin. Failure of rotation of the pre-

associated to normal post-arterial se gment rotation results in a right MH with the small bowel trapped behind the mesentery of the right and transve rse colon. A l

unsupported area of descending mesocolon between the inferior mesenteric vein and posterior parietal attachment is ballooned by the small bowel as it migrates t

Both MH can presents with chronic or acute abdominal obstruction, gangrene and bowel perforation. UGIS is diagnostic. Management consists of reduction, repo

sparing the inferior mesenteric vesse ls during the repair of left MH and re-positioning the right colon to the left s ide of the abdomen for repair of right MH.

References

1- Khan MA, Lo AY, Vande Maele DM: Paraduodenal hernia. Am Surg 64(12):1218-22, 1998

2- Dengler WC, Reddy PP: Right paradu odena l hernia in childhood : a case report . J Pediatr Surg 24(11):1153-4, 1989

3- Brigham RA, Fallon WF, Saun ders JR, Harmon JW : Paraduodenal hernia: diagnos is and surg ical management. Surgery 96(3):498-502, 1984

4- Gagic NM: Right paradu odena l hernia. Can J Surg 25(1):71-2, 1982

5- Azouz EM, Doyon M: Waldey er's h ernia. J Can A ss oc Radiol 27(2):108-10, 1976

Volumen 13 No 3 SEPTEMBER 1999

Laparoscopic Adrenalectomy

Adrenalectomy is another procedure that has yielded to the advantages of the laparoscopic approach in very specific situations. In the pediatric age the indicatio

mostly tumor related: neuroblastoma (the most common adrenal mass in a child), pheochromocytoma, adenoma, ganglioneuroma, neurofibroma, fibromas, inciden

Benign, well encapsulated, fibrous, and non-infiltrative lesions within the adrenal gland lend themselves to laparoscopic removal. Malignant (due to risk of port

or les ions that nee d lymph node sampling, have bilateral involvement will need the conventional open approach. In children the lateral intra-abdominal approach p

place instead of the retroperitoneal approach used in adults. Right adrenal gland removal needs liver mobilization and secure closure of the short adrenal vein. Le

and can be accomplished after mobilization of spleno-colonic ligaments. Although the lap approach takes longer, the overall advantages are less pain, less morbid

better cosmetic results.

References

1- Lopoo JB, Albanes e CT, Jennings RW, Tyrell D, Harrison MR, Duh QY: Laparoscop ic Adrenalecto my in Children. Ped iatr Endosu rgery & In novat Tech 2(3): 107- 110, 1998

2- Yosh imura K, Yoshioka T, Miyake O, Mat sumiya K: Comparison of clinical outcomes o f laparoscopic and conventional open adren alectomy. J Endourol 12(6):555-9, 1998

3- Linos DA, Stylopoulos N, Boukis M, Souvatzoglou A: Anterior, posterior, or laparoscopic approach for the management of adrenal diseases? Am J Surg 173(2):120-5, 19974- Fernandez-Cruz L, Benarroch G, Torres E, Astudillo E, Saenz A; Taura P: Laparoscopic approach to the adrenal tumors. J Laparoendosc Surg 3(6):541-6, 1993

Laparoscopic Ladd's Procedure

Malrotation of the bowel can be associated with midgut volvulus defined as clockwise rotation of the small bowel around the superior mesenteric vessels axis cau

is done with the help of both an upper contrast study and barium enema. Asymptomatic malrotation with near-point fixation of the duodenojejunal and ileocolic se

prophylactic Ladd's. Ladd's procedure consists of release of congenital duodeno-colic bands, separation of the duodeno-jejunal from the ileo-colic segment in a tr

removal of the appendix for cases of bowel malrotation associated with or without midgut volvulus. Asymptomatic malrotation referred for Ladds procedure can b

a three port technique. Ladd's bands and duodenocolic adhesions are divided exposing the SMA that lies between the duodenum and ascending colon. The base o

widened using sharp dissection leaving the small bowel on the right abdomen and the colon on the left to prevent volvulus. The appendix is removed to avoid futur

Advantages of laparoscopy are: less pain, less ileus, brief hospital stay and better cosmesis. In cases of midgut volvulus laparoscopy is debatable since it may be

deal with dilated a/o ischemic bowel.

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References

1- Lessin MS, Luks FI: Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int 13(2-3):184-5, 1998

2- Gross E, Chen MK, Lobe TE: Laparos copic eva luation and treatment of intest inal malrotation in infant s. Surg Endosc 10(9):936-7, 1996

3- Bass KD, Rothen berg SS, Chan g JH: Laparosco pic Ladd's p rocedure in infants with malrotation. J Pediatr Surg 33(2):279-81, 1998

4- Waldhausen JH, Sawin RS: Laparoscopic Ladd's procedure and assessment of malrotation. J Laparoendosc Surg 6 Suppl 1:S103-5, 1996 5- Mazziotti MV, Strasbe rg SM, Langer JC: Intes tinal rotation ab normalities without volvulus : the role of laparosco py. J A m Coll Surg 185(2):172-6, 1997

Myofibromatosis

Myofibromatosis refers to a rare benign soft tissue tumor seen mostly in newborns and young infants. The child develops firm, discrete, flesh-colored to purple no

a/o subcutaneous tissue with particular predilection for the head and neck region and trunk. Etiology is unknown. Microscopically, they are well-circumscribed no

bundles of plump, spindle-shaped cells displaying characteristics intermediate between fibroblasts and smooth muscle cells with central necrosis and a prominent

two types are described: solitary and multicentric (generalized) form. Most infants (74%) have a solitary lesions that respond to conservative surgical excision. T

poorer prognosis may involve muscle , bone and visce ra. Visceral lesions are associated with significant morbidity and mortality generally within the first few mon

obstruction of a vital organ (bowel and lung), failure to thrive, or infection. Spontaneous regress ion has been identified in one-third of the patients after a two-year

multiple lesions not affecting vital function, resulting in growth anomalies , or demonstrating rapid aggressive growth can be managed conservatively.

References

1- Schrodt BJ, Callen JP: A case of co ngenital multiple myofibromatosis developing in an infant . Pediatrics 104(1 Pt 1):113-5 , 1999

2- Beck JC, Devaney KO, Weatherly RA, Koop mann CF Jr, Lesperance MM: Pediatric myofibromatosis o f the h ead an d neck. Arch Otolaryng ol Head Neck Surg 125(1):39-44, 1999

3- Coffin CM, Neilson KA, Ingels S, Frank-Gerszberg R, Dehner LP: Congenital gen eralized myofibromatos is: a d isseminated angiocen tric myofibromatos is. Pediatr Pat hol Lab Med 15(4):571-87, 1995

4- Salamah MM, Hammoudi SM, Sadi AR: Infantile myofibromatosis. J Pediatr Surg 23(10):975-7, 1988

5- Chung EB, Enzinger FM: Infantile myofibromatosis. Cancer 15;48(8):1807-18, 1981

Volume 13 No 4 OCTOBER 1999

Mixed Gonadal Dysgenesis

Mixed gonadal dysgenesis (MGD) is an intersexual genetic abnormality caused by a defect in the sex chromosomes (gonosomes) associated with dysgenetic gon

structures . The most common gonosomal aberration in MGD is 45 X0/46 XY mosaic karyotype. The exte rnal genitalia could be normal looking female and these

life with primary amenorrhea. Otherwise, it could be ambiguous: clitoromegaly and urogenital sinus to a sizable phallus with hypospadia. A uterus and one or both

present. MGD is characterized by a streak gonad and a contralateral testis (that is typically cryptorchid) or bilateral streak testes. The testis might show prepube

spermatogonia and immature Sertoli cells. Female gender assignment is usually preferred, but male assignment is an alternative in instances of extreme virilizati

the presence of a Y chromosome or a translocated fragment have a s ignificant risk of deve loping malignant gonadoblastoma (though se minoma and dysgerminom

bilateral gonadectomy is advice in MGD. The child to be raised as a female will need clitoral recession and vaginoplasty in early infancy. If it is to be raised as m

hypospadias repair can be done, gonads can be replaced with prostheses, the prepenile scrotum reconstructed and Mllerian structures removed.

References

1- Hendren W H: Surgical approach to inte rsex problems. Semin Pediatr Surg 7(1):8-18, 1998

2- Calabrese F, Valente M: Mixed gonadal dysgenesis: histological and ultrastructural findings in two cases. Int J Gynecol Pathol 15(3):270-5, 1996 3- Federman DD, Donahoe PK: Ambiguous genitalia--etiology, diagnosis, and therapy. Adv Endocrinol Metab 6:91-116, 1995

4- Borer JG, Nitti VW, Glassbe rg KI: Mixed g onada l dys genes is an d dy sgenetic male pseudo hermaphroditism. J Urol 153(4):1267-73, 1995

5- Krasna IH, Lee ML, Smilow P, Sciorra L, Eierman L: Risk of malignancy in bilateral streak gonads: the role of the Y chromosome. J Pediatr Surg 27(11):1376-80, 1992 6- Coran AG, Polley TZ Jr: Surgical management of ambiguous genitalia in the infant an d ch ild. J Pediatr Surg 26(7):812-20, 1991

Recurrent Intussusception

Intussusception, the most common cause of bowel obstruction in infants, occurs mostly in the ileo-cecal area (distal ileum invaginates inside the colon) and it is idi

hypertrophied Peyer patches after Adenovirus III infestation). Initial management consists of hydrostatic or air enema reduction. Recurrent intussusception (RI)

after hydrostatic reduction and to a lesser degree after surgical reduction (3%). Patients with RI have fewer symptoms with a shorter duration. Most RI occurs w

episode. With multiple recurring episodes a search for a pathological lead point (Meckel's diverticulum, polyp, ectopic gastric mucosa, duplication or lymphoid hy

eve n in the face of previous surgery is not a contraindication to try radiological reduction since the success rate is still high and the morbidity low. The child with p

peritonitis, irreducible intussusception or a diagnosed lead point should undergo immediate surgical reduction.

References

1- Daneman A, Alton DJ, Lobo E, Gravett J, Kim P: Patterns of recurrence of intus sus ception in children: a 17-year review. Pediatr Radiol 28(12):913-9, 1998

2- Hasegawa T, Ueda S, Tazuke Y, Monta O, Sakurai T, Takahara N, Tanaka T, Habukawa C: Colonoscopic diagnosis of lymphoid hyperplasia causing recurrent intussusception: report of a case. Surg T

3- Fecteau A, Flageole H, Nguyen LT, Laberge JM, Shaw KS, Guttman FM: Recurrent intussusception: safe use of hydrostatic enema. J Pediatr Surg 31(6):859-61, 1996

4- Champoux AN, Del Beccaro MA, Nazar-Stewart V: Recurrent intussus ception. Risks and features . Arch Ped iatr Adolesc Med 148(5):474-8, 1994

5- Pierro A, Donnell SC, Paraskevopoulou C, Carty H: Indications for laparotomy after hydrostatic reduction for intussusception. J Pediatr Surg 28(9):1154-7, 1993 6- Ein SH: Recurrent intussus ception in children. J Ped iatr Surg 10(5):751-5, 1975

Fibrous Hamartoma

Establishing a diagnosis on clinical or radiological grounds of a subcutaneous mass in an infant or child could be a challenging dilemma, not to mention poorly cost

hamartoma is an uncommon benign, soft tissue, painless nodule of rapid growth usually seen during the first two years of life. Initially described in 1956, this lesio

boys, solitary, identified in the subcutis or lower dermis of the axillary region, upper arm, upper trunk, inguinal region, s calp, scrotum and perineum. The lesion va

the underlying tissue and not encapsulated. A few cases show overlying skin changes (pigmentation, hair). Pathologically they show well-defined bundles of dense,

tissue projecting into fat, primitive mesenchyme and mature adipose tissue. Diagnosis has been established by fine-needle aspiration technique rarely. Standard cto establish a diagnosis. Observation is not a good option given the uncertainty of missing a malignant lesion. Incomplete removal might result in recurrence and

Mutilating surgery is unnecessary. The prognosis is excellent.

References

1- Lee JT, Girvan DP, Armstro ng RF: Fibrous Hamartoma of Infancy. J Pediatr Surg 23(8): 759-761, 1988

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2- Dickey GE, Sotelo-Avila C: Fibrous hamartoma of infancy : current review. Pediatr Dev Pat hol 2(3):236-43, 1999

3- Popek EJ, Montgo mery EA, Fourcroy JL: Fibrous h amartoma of infancy in th e gen ital region: findings in 15 cases. J Urol 152(3):990-3, 1994

4- Albukerk J, Wexler H, Dana M, Silverman J: A case of fibrous hamartoma of infancy . J Ped iatr Surg 14(1):80-2, 1979

5- Jadusingh IH: Fine needle aspiration cytology of fibrous hamartoma of infancy. Acta Cytol 41(4 Suppl):1391-3, 1997

Volume 13 No 5 NOVEMBER 1999

Urachal Remnants

Urachal remnants are uncommon anomalies that present symptoms in infancy or early childhood. The urachus is formed in early embryonic life as a tubular conne

the bladder and the allantoic stalk. This fistulous tract obliterates into a median ligament. Rare persistence of this remnant after birth can lead to a completely pa

(opening to the navel), a diverticulum (opening to the bladder), a cyst (both end close but central portion remains open) or alternating sinus (cys t-like s tructure ca

or umbilicus). Neonatal patent urachus can undergo spontaneous involution. Otherwise, the transitional epithelium is replaced by granulation tissue and the main

is urine discharge from the umbilicus associated with cellulitis, pain and swelling. A cyst or diverticulum can present as a midline, localized, painful abdominal mas

leucocytosis. The diagnosis can be done with ultrasound (cystic process is shown), cystography (the ventral position of urachal diverticulum and fistula is seen, an

diagnosed) or fistulography (a connection with the bladder is demonstrated). If infected initial management consists of antibiotics, otherwise cure is accomplished

remnant with a cuff of bladder (to avoid recurrent infection, stone formation and later development of carcinoma), double layer closure and drainage.

References

1- Zieger B, Sokol B, Rohrsch neider WK, Darge K, Trog er J: Sonomorpho logy and involution o f the n ormal urachus in asy mptomatic newborns . Pediatr Radiol 28(3):156-61, 1998

2- Suita S, Nagasaki A: Urachal remnants. Semin Pediatr Surg 5(2):107-15, 1996

3- Nagasaki A, Handa N; Kawanami T: Diagnosis of urachal anomalies in infancy and childhood by contrast fistulography, ultrasound and CT. Pediatr Radiol 21(5):321-3, 1991

4- Goldman IL, Caldamone A A, Gauderer M, Hampel N, Wess elhoeft CW, Elder JS: Infected u rachal cys ts: a review of 10 cases. J Urol 140(2):375-8, 19885- Newman BM; Karp MP; Jewett TC; Cooney DR: Advances in the management of infected urachal cysts. J Pediatr Surg 21(12):1051-4, 1986

Medullary Thyroid Carcinoma

Medullary thyroid carcinoma (MTC) is a rare, solid, thyroid neoplasm with amyloid stroma that arise from the parafollicular C-cells , is inherited as autosomal do

childhood sporadically or associated with a multiple endocrine neoplasia (MEN) or Familial syndrome. In sporadic cases the presentation occurs in adolescence a

of thyrocalcitonin by MTC and its precursor (C-cell hyperplasia) permits diagnosis and follow-up, though a significant number of these children are not cured by s

disease at diagnosis. DNA testing has found that the RET proto-oncogene mutation is associated with MCT development in kindreds of sporadic cases, MEN or

This has permitted early (prophylactic) gland removal in infancy (MEN IIB) or early childhood (MEN IIA) before biochemical or clinical MCT develops. MCT

nodes followed by lungs, bone and liver. Management consists of total thyroidectomy and central lymph node sampling with general sampling if enlarged.

References

1- Telander RL, Moir CR: Medullary thyroid carcinoma in children. Semin Pediatr Surg 3(3):188-93, 19942- Wells SA Jr, Chi DD, Toshima K, Dehner LP, Coffin CM, Dowton SB, Ivanovich JL, DeBenedetti MK, Dilley WG, Moley JF, et al: Predictive DNA testing and prophylactic thyroidectomy in patients at

type 2A. Ann Surg 220(3):237-47, 1994

3- Skinner MA, DeBenedett i MK, Moley JF, Norton JA, We lls SA Jr: Medu llary th yroid carcinoma in children with multiple endocrine n eoplas ia types 2A and 2B. J Pediatr Surg 31(1):177-81, 1996

4- La Quaglia MP, Telander RL: Differentiated an d medullary thyroid cancer in childhood an d adoles cence. Semin Pediatr Surg 6(1):42-9, 19975- Skinner MA, Wells SA Jr: Medullary carcinoma of t he th yroid gland and t he MEN 2 syn dromes. Semin Pediatr Surg 6(3):134-40, 1997

6- Lallier M, St-Vil D, Giroux M, Huot C, Gaboury L: Prophylactic thyro idectomy for medullary th yroid carcinoma in g ene carriers of MEN2 syndrome. J Pediatr Surg 33(6):846-8, 19987- van Heurn LW, Schaap C, Sie G, Haagen AA, Gerver WJ, Freling G, van A mstel HK, Heineman E: Pred ictive DNA testing fo r multiple endocrine neoplasia 2: a therapeutic challenge of p rophy lactic th

J Pediatr Surg 34(4):568-71, 1999

Gastric Perforation

Gastric perforation (GP) is a rare abdominal catastrophe seen chiefly in premature infants. GP occurs primarily (spontaneously) after selective ischemia with bloo

neonatal asphyxia. Secondary GP is caused by mechanical disruption (excessive distension or instrumentation) as observed in situations such as: aggressive mas

atresia, esophageal atresia with TE fistula, volvulus of the stomach, esophageal intubation, mechanical ventilation and nasogastric tubes (iatrogenic). Boys are m

girls. Sudden onset of abdominal distension, feeding intolerance, respiratory distress, metabolic acidosis, shock and hypoactivity within the first week of life is ch

Pneumoperitoneum is seen in plain abdominal films. Most GP occurs along the greater curvature of the stomach between the smooth muscle layers. With severel

peritoneal drainage with lavage removes gas and acid, decompresses the abdomen improving ventilation (abdominal compartment syndrome), and grants time to s

(improve acidosis, shock and coagulopathy) in preparation for surgical closure of the perforation. GP carries a high mortality rate.

References

1- St-Vil D, LeBouthillier G, Luks FI, Bensou ss an A L, Blanchard H, Yous sef S: Neonatal ga stroint estinal p erforations . J Ped iatr Surg 27(10):1340-2, 1992

2- Shash ikumar VL, Bassuk A, Pilling GP IV, Cresson SL: Spontan eous gas tric rupture in the newborn: a clinical review of nineteen case s. A nn Su rg 182(1):22-5, 1975

3- Rosser SB, Clark CH, Elechi EN: Spontaneo us neon atal gas tric perforation. J Pediatr Su rg 17(4):390-4, 1982

4- Tan CE, Kiely EM, Ag rawal M, Brereton RJ, Spitz L: Neonatal ga stro intestinal p erforation. J Pediatr Surg 24(9):888-92, 1989

5- Houck WS Jr, Griffin JA 3d: Spontaneous linear tears of the stomach in the newborn infant. Ann Surg 193(6):763-8, 19816- Aviles T, Lugo-Vicente H, Ocasio MT, Guiven A, Pagn V, Lliteras O, Vzquez H: Perforated NEC: The role of percutaneous peritoneal drainage (submitted for publication).

Volume 13 No 6 DECEMBER 1999

Bowel Management

Fecal incontinence (FI) could be the outcome after surgery for anorectal malformations (imperforate anus) or Hirschsprung's disease. FI can be further subdividestudies into those with a tendency to constipation (megasigmoid) or diarrhea (a non-dilatated colon running straight from the splenic flexure to the anus). Bowel m

the personal and social burden inherent to this problem promoting independence in the affecte d child. The most effective regimen consists of regular and complet

limiting the episodes of fecal soilage. This is accomplished with the use of a daily enema program, dietary manipulation, laxatives and drugs. The enema is admini

the toilet at a rate of 10-20 cc/kg of weight. To avoid spillage the silastic tube must have a balloon to seal the distal rectum. After enema administration the balloo

allowed to evacuate the colonic content. Leaving the balloon partially inflated encourages the child to expel it as a biofeedback mechanism allowing some patients

minimal control to be exploited. If the e nema program is effe ctive in a 3 to 6 month period, the child can become a candidate for a Malone procedure (appendicost

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constipation and megasigmoid needs large volume enemas. Likewise, the large megasigmoid can cause overflow pseudo incontinence that is only helped with sig

diarrhea may need constipating diet and anti-motility drugs. A few children that continue with incontinence in spite adequate therapy might benefit from a perman

References

1- Shandling B, Gilmour RF: The Enema Continence Catheter in Spina Bifida: Succes sful Bowel Man agement. J Pediatr Surg 22(3): 271-273, 1987

2- Blair GK, Djonlic K, Fras er GC, Arno ld WD, Mu rohy JJ, Irwin B: The Bowel Management Tube: A n Effective Means for Controlling Fecal Inco ntinence. J Pediatr Su rg 27(10): 1269-1272, 1992

3- Pea A, Guardino K, Tov illa JM, Levitt MA , Rodriguez G, Torres R: Bowel Manag ement for Fecal Inco ntinence in Patients with Anorect al malformations. J Ped iatr Surg 33(1): 133-137, 1998

4- Levitt MA, Soffer SZ, Pen a A: Continent appen dicost omy in the b owel management of fecally incont inent ch ildren. J Pediatr Surg 32(11):1630-3, 1997

5- Paidas CN: Fecal incontinence in children with anorec tal malformations . Semin Pediatr Su rg 6(4):228-34, 1997

6- Gleeson RM: Bowel continence for the child with a n eurogen ic bowel. Rehabil Nurs 15(6):319-21, 1990

Bladder Rhabdomyosarcoma

Bladder Rhabdomyosarcoma (B-RMS) is the most common tumor of the lower genitourinary tract in children (mean appearance at five years of age). Most B-R

tissue of the bladder base, trigone and neck infiltrating the prostatic urethra and surrounding pelvic fascia. Only 20% arise from the bladder dome. Urinary or fec

or palpable mass are initial presentation. Embryonal (75%) and botryoid (25%) histological variant predominates. MRI is recommended as the ke y method of di

pelvic RMS. Unless staging demonstrates a small or dome lesion amenable to surgical resection, initial management should consist of high dose multiagent chem

irradiation (40 Gy). Response could be: 1- complete, needing follow-up imaging and cystoscopy, 2- partial (< 50%) or none, needing total cystectomy or anterior e

hampered by metastasis, local invasion and prostatic origin of the tumor. These children need aggressive treatment modality including total cystectomy. Bladder

difficult to achieve due to location of the lesion, radiation cystitis and hemorrhagic cystitis (cyclophosphamide).

References

1- Ragab AH, Heyn R, Tefft M, Hays DN, Newton WA Jr, Beltangady M: Infants younger than 1 year of age with rhabdomyosarcoma. Cancer 58(12):2606-10, 1986

2- Loughlin KR, Retik AB, Weins tein HJ, Colodny AH, Shamberger RC, Delorey M, Tarbell N, Cassady JR, Hend ren W H: Genitourinary rh abdomyos arcoma in children. Cance r 15;63(8):1600-6, 19893-Hays DM, Lawrence W Jr, Crist WM, Wiener E, Raney RB Jr, Ragab A, Tefft M, Webber B, Johnston J, Maurer HM: Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a repo

Rhabdomyos arcoma Study . J Pediatr Surg 25(7):719-23, 1990

4- La Quaglia MP, Ghavimi F, Herr H, Mandell L, Pennenberg D, Hajdu S, Exelby PR: Prognos tic factors in bladder an d bladd er-prost ate rhab domyosarcoma. J Pediatr Surg 25(10):1066-72, 1990

5- Hicks BA, Hensle TW, Burbige KA, Altman RP: Bladder management in children with gen itourinary s arcoma. J Pediatr Surg 28(8):1019-22, 19936- Hays DM, Raney RB, Wharam MD, Wiener E, Lobe TE: Children with vesical rhabdomyosarcoma (RMS) treated by partial cystectomy with neoadjuvant or adjuvant chemotherapy, with or without ra

Intergrou p Rhab domyosarcoma Stud y (IRS) Committee. J Pediatr Hematol Oncol 17(1):46-52, 19957- Regine WF, Fontanes i J, Kumar P, Ayers D, Bowman LC, Pappo AS, Coffey DH, Ave ry L, Rao BN, Kun LE: Local tumor control in rhab domyosarcoma following low-dos e irradiation: comparison of gr

Int J Radiat Oncol Biol Phys 31(3):485-91, 1995

8- Heyn R, Newton WA , Raney RB, Hamoudi A, Bagwell C, Vietti T, Wh aram M, Gehan E, Maurer HM: Preserva tion of t he bladd er in patient s with rhabdomyos arcoma. J Clin Oncol 15(1):69-75, 1997

9- Merguerian PA, Agarwal S, Greenberg M, Bagli DJ, Khoury A E, McLorie GA: Outcome analysis of rhabd omyosarco ma of the lower urinary tract. J Urol 160(3 Pt 2):1191-4, 1998

Fournier's Gangrene

Necrotizing fascitis of the perineum, genitalia (scrotum and penis) and the abdominal wall is commonly known as Fournier's Gangrene (FG). FG is an emergency

seen in the pediatric age. Thrombosis of small arteries in the genital area results in ischemic injury. In children, FG is seen after circumcision, insect bites, anorecrash, perianal skin abscesses, and bone marrow transplant. Most affected children are infants. Patients develop abrupt, rapidly progressive gangrenous infection

swelling and blistering of the genital area with syste mic signs of toxicity. Infecting organisms comprise both aerobic and anaerobic organisms. Management cons

antimicrobial therapy, aggressive and frequent surgical debridement, and if necessary, urinary and colonic diversions to control the infection. Fortunately pediatri

managed with a more conservative surgical approach and have a significantly lower mortality rate than adult cases.

References

1- Adams JR Jr, Mata JA, Venable DD, Culkin DJ, Bocch ini JA Jr, Fou rnier's gan grene in children. Urology 35(5):439-41, 19902- Martinelli G, Alessand rino EP, Bernasconi P, Caldera D, Colombo A, Malcov ati L, Gaviglio MR, Vignoli GP, Borroni G, Bernasco ni C: Fournier's gang rene: a clinical present ation of necrotizing fasciitis

Bone Marrow Transplant 22(10):1023-6, 1998

3- Efem SE: The feat ures and aetiology of Fournier's g angrene . Post grad Med J 70(826):568-71, 1994

4- Muelder K: Fourn ier's gang rene. W orld J Surg . 7(5):677, 1983

5- Adeyo kunnu A A: Fournier's s yndrome in infants . A review of ca ses from Ibadan, Nigeria. Clin Pediatr (Phila) 22(2):101-3, 1983

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PSU Vol 13, 1999