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 and teratoma. They usually presents at birth interfering
with mouth closure, swallowing, normal feeding or even causing respiratory
problems. Complications of the cyst include infection, hemorraghe and rupture
leading 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 marsupialization. Multiple cysts 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 of the tongue and
can safely be managed with marsupialization without excision or Sistrunk
procedure. Cysts of foregut origin presents in the neonatal period, can
be found in the anterior part of the dorsum of the tongue needing complete
surgical excision through a sagittal glossal split. Cyst aspiration is
inadequate definitive treatment. A teratoma likewise needs surgical excision
for cure.
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, Bosenberg AT, Chrystal V: Intralingual
cysts of foregut origin. J Pediatr Surg 27(11):1404-6, 1992
4- 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. Congenital absence or 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 is divided into deficiency
of: antibody (most common), T-cell, combined T and B-cell, phagocytic or
complement activity. Most cases of PI are identified in the first year
of life. Primary symptom is recurrent infections with unusual organisms
(low pathogenicity) that is severe, prolonged and resistant to conventional
therapy. T-cell deficiency occurs before the age of six months while antibody
deficiency 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 pus formation or delayed 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 by the
GI tract. The child usually appears chronically ill with growth failure.
Extensive lab testing will establish the diagnosis. Live vaccine should
be avoided. The child with PI who will undergo a surgical procedure needs:
protective isolation, antibiotic prophylaxis, nutritional backup, physiologic
monitoring and selective replacement of defective substance (immunoglobulins)
a/o immunologic enhancing agents use. Bone marrow transplant can sometimes
correct a PI.
References
1- Peerles AG, Stiehm ER: The Immunocompromised Child,
In Fonkalsrud and Krummel "Infections and Immunologic Disorders in Pediatric
Surgery", WB Saunders Co, 1993, pag 77-90
2- Stiehm ER: Human intravenous immunoglobulin in primary
and secondary antibody deficiencies. Pediatr Infect Dis J 16(7):696-707,
1997
3- Pachman LM, Lynch PA, Silver RK, Ozog DL, Poznanski
AK: Primary immunodeficiency disease in children: an update. Curr Probl
Pediatr 19(1):1-64, 1989
4- Primary immunodeficiency diseases. Report prepared
for the WHO by a scientific group on immunodeficiency. Clin Immunol Immunopathol
28(3):450-75, 1983
5- Pappas BE: Primary immunodeficiency disorders in infancy.
Neonatal Netw 18(1):13-22, 1999
Lymphedema
The most common forms of lymphedema in children affect the lower extremity.
Lymphedema in children can be classified into: congenital, precox and tarda.
The underlying pathology is absence (10%) or hypoplasia (90%) of the lymphatic
channels of the lower extremity. Diagnosis of lymphedema is made clinically.
Congenital lymphedema appears early in life, involves more 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 specific therapy
is required in two thirds of cases. Lymphedema precox appears during adolescence,
occur mostly in females, extends to the groin and is associated with yellow
nails (inadequate lymphatic drainage). Lymphedema tarda occurs spontaneously
in middle age people. In unilateral cases a retroperitoneal tumor or venous
obstruction should be rule out. Complications of lymphedema include swelling,
brawny edema, cellulitis and lymphangitis. Management consists of extremity
care, compressive support stocking and leg elevation. In severe cases the
option is excision of excess 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
abnormalities of the lymphatic system: a new clinical classification. Pediatrics
86:988, 1990
3- Levine C: Primary disorders of the lymphatic vessels:
a unified concept. J Pediatr Surg 24:233, 1989
4- Fonkalsrud EW: Congenital Malformations of the Lymphatic
System. 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 the midgut (from the second portion of the duodenum
to mid-transverse colon). On rare occasions volvulus involve only the small
bowel in a segmental fashion affecting a portion of jejunum, ileum or both.
Segmental volvulus can be the result of a predisposing anatomical defects
such as: congenital bands (a persistent omphalomesenteric band or Meckel
diverticulum fixed to the anterior abdominal wall), acquired adhesive bands
after a surgical procedure, abnormal foreshortened mesenteric defect (segmental
or basilar), hanging tumors (mesenteric cyst), intraluminal lesions of
the small bowel (meconium ileus), worms (Ascariasis) and even VP shunts.
Clinically, the child will present with sudden intestinal obstruction (bilious
vomiting, abdominal distension, signs of peritonitis) associated with variable
changes of ischemic bowel (shock, metabolic acidosis and dehydration).
Recognition of volvulus before infarction occurs is imperative for bowel
survival. Management must be prompt and consist of counterclockwise detorsion
of the affected segment, removing the anatomic cause, and depending on
the viability of the affected segment, resection with anastomosis. In broad-base
mesenteric root defects (basilar) pexing of the bowel may be needed.
Due to the segmental nature loss of massive bowel is fortunately rarely
seen.
References
1- Maung M, Saing H: Intestinal volvulus: an experience
in a developing country. 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
29(10):1339-43, 1994
3- Wilkinson TS, Stone HH: Intestinal 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, West
KW: Anomalies of intestinal rotation in childhood: analysis of 447 cases.
Surgery 108(4):710-5, 1990
5- Abrahamson RH: Acute small intestinal volvulus. 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 obstruction. 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 pneumoperitoneum can arise from ruptured pulmonic blebs
(minute rupture in alveoli subjected to the stress of mechanical ventilation)
dissecting retroperitoneally into the abdomen rather than outwardly into
the pleura. This is known as the Macklin effect published originally in
1943. Precise diagnosis of this type of "medical" pneumoperitoneum will
reduce a needless laparotomy in seriously ill infants. Some suggestions
pointing toward this etiology are the presence of interstitial emphysema,
retrocardiac pneumomediastinum, pneumothorax, dissection of air into the
soft tissues of the neck, the 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 mechanical ventilation. Previous
X-ray may note the absence of dilated bowel loops, edema or pneumatosis.
Contrast bowel studies (with water soluble material) and peritoneal paracentesis
is needed to distinguish 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 al: Blunt traumatic pneumomediastinum:
using CT to reveal the Macklin effect.. Am J Roentgenol. 172(1):129-30,
1999
2- Brotman S, et al: The Macklin effect: a cause of pneumoperitoneum.
Md State Med J. 30(9):30, 1981
3- Rosenfeld DL, Cordell CE, Jadeja N: Retrocardiac pneumomediastinum:
radiographic finding and clinical implications. Pediatrics 85(1):92-7,
1990
4- Cohen MD, Schreiner R, Lemons J: Neonatal pneumoperitoneum
without significant adventitious pulmonary air: Use of metrizamide to rule
out perforation of the bowel. Pediatrics 69(5):587-9, 1982
5- Zerella JT, McCullough JY: Pneumoperitoneum in infants
without gastrointestinal perforation. Surgery 89(2):163-7, 1981
6- Summers B: Pneumoperitoneum associated with artificial
ventilation. Br Med J 1:1528-30, 1979
7- Steves M, Ricketts RR: Pneumoperitoneum in the newborn
infant. Am Surg 53(4):226-30, 1987
8- Campbell RE, Boggs TR Jr, Kirkpatrick JA Jr: Early
neonatal pneumoperitoneum from progressive massive tension pneumomediastinum.
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 entrapment of the small 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-arterial midgut
segment associated to normal post-arterial segment rotation results in
a right MH with the small bowel trapped behind the mesentery of the right
and transverse colon. A left MH occurs when the unsupported area of descending
mesocolon between the inferior mesenteric vein and posterior parietal attachment
is ballooned by the small bowel as it migrates to the left abdominal cavity.
Both MH can presents with chronic or acute abdominal obstruction, gangrene
and bowel perforation. UGIS is diagnostic. Management consists of reduction,
repositioning of the bowel and sparing the inferior mesenteric vessels
during the repair of left MH and re-positioning the right colon to the
left side 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 paraduodenal hernia in
childhood: a case report. J Pediatr Surg 24(11):1153-4, 1989
3- Brigham RA, Fallon WF, Saunders JR, Harmon JW: Paraduodenal
hernia: diagnosis and surgical management. Surgery 96(3):498-502, 1984
4- Gagic NM: Right paraduodenal hernia. Can J Surg 25(1):71-2,
1982
5- Azouz EM, Doyon M: Waldeyer's hernia. J Can Assoc
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 indications for adrenalectomy are mostly tumor related: neuroblastoma
(the most common adrenal mass in a child), pheochromocytoma, adenoma, ganglioneuroma,
neurofibroma, fibromas, incidentalomas and lipomas. Benign, well
encapsulated, fibrous, and non-infiltrative lesions within the adrenal
gland lend themselves to laparoscopic removal. Malignant (due to risk of
port metastasis), large, infiltrative or lesions that need lymph node sampling,
have bilateral involvement will need the conventional open approach. In
children the lateral intra-abdominal approach provides a greater working
place instead of the retroperitoneal approach used in adults. Right adrenal
gland removal needs liver mobilization and secure closure of the short
adrenal vein. Left adrenal removal is easier and can be accomplished after
mobilization of spleno-colonic ligaments. Although the lap approach takes
longer, the overall advantages are less pain, less morbidity, shorter hospital
stay and better cosmetic results.
References
1- Lopoo JB, Albanese CT, Jennings RW, Tyrell D, Harrison
MR, Duh QY: Laparoscopic Adrenalectomy in Children. Pediatr Endosurgery
& Innovat Tech 2(3): 107- 110, 1998
2- Yoshimura K, Yoshioka T, Miyake O, Matsumiya K: Comparison
of clinical outcomes of laparoscopic and conventional open adrenalectomy.
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, 1997
4- 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 causing ischemia. The diagnosis 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 segment should
undergo 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 transverse fashion with removal of the appendix for cases
of bowel malrotation associated with or without midgut volvulus. Asymptomatic
malrotation referred for Ladds procedure can be done laparoscopically using
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 of the mesentery root is 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 future diagnostic problems. 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 difficult and dangerous to deal with dilated a/o ischemic bowel.
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: Laparoscopic evaluation
and treatment of intestinal malrotation in infants. Surg Endosc 10(9):936-7,
1996
3- Bass KD, Rothenberg SS, Chang JH: Laparoscopic Ladd's
procedure 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, Strasberg SM, Langer JC: Intestinal
rotation abnormalities without volvulus: the role of laparoscopy. J Am
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 nodules in skin, muscle, bone a/o subcutaneous tissue with particular
predilection for the head and neck region and trunk. Etiology is unknown.
Microscopically, they are well-circumscribed nodules consisting of short
bundles of plump, spindle-shaped cells displaying characteristics intermediate
between fibroblasts and smooth muscle cells with central necrosis and a
prominent vascular pattern. Clinically two types are described: solitary
and multicentric (generalized) form. Most infants (74%) have a solitary
lesions that respond to conservative surgical excision. The multicentric
variant with a poorer prognosis may involve muscle, bone and viscera. Visceral
lesions are associated with significant morbidity and mortality generally
within the first few months of life secondary to obstruction of a vital
organ (bowel and lung), failure to thrive, or infection. Spontaneous regression
has been identified in one-third of the patients after a two-year period
suggesting that 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 congenital multiple
myofibromatosis developing in an infant. Pediatrics 104(1 Pt 1):113-5 ,
1999
2- Beck JC, Devaney KO, Weatherly RA, Koopmann CF Jr,
Lesperance MM: Pediatric myofibromatosis of the head and neck. Arch Otolaryngol
Head Neck Surg 125(1):39-44, 1999
3- Coffin CM, Neilson KA, Ingels S, Frank-Gerszberg R,
Dehner LP: Congenital generalized myofibromatosis: a disseminated angiocentric
myofibromatosis. Pediatr Pathol 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
gonads and retained Müllerian structures. The most common gonosomal
aberration in MGD is 45 X0/46 XY mosaic karyotype. The external genitalia
could be normal looking female and these children will present later in
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 fallopian tubes may also be present. MGD is characterized by
a streak gonad and a contralateral testis (that is typically cryptorchid)
or bilateral streak testes. The testis might show prepubertal tubules lined
by a few spermatogonia and immature Sertoli cells. Female gender assignment
is usually preferred, but male assignment is an alternative in instances
of extreme virilization. Dysgenetic gonads with the presence of a Y chromosome
or a translocated fragment have a significant risk of developing malignant
gonadoblastoma (though seminoma and dysgerminoma can occur). Routine early
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 male, then various types of hypospadias repair can be done,
gonads can be replaced with prostheses, the prepenile scrotum reconstructed
and Müllerian structures removed.
References
1- Hendren WH: Surgical approach to intersex 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, Glassberg KI: Mixed gonadal dysgenesis
and dysgenetic male pseudohermaphroditism. 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 and child. 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 idiopathic (or caused by hypertrophied Peyer patches after
Adenovirus III infestation). Initial management consists of hydrostatic
or air enema reduction. Recurrent intussusception (RI) occurs in up to
10% of cases 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 within six months of the initial episode. With multiple
recurring episodes a search for a pathological lead point (Meckel's diverticulum,
polyp, ectopic gastric mucosa, duplication or lymphoid hyperplasia) is
imperative. RI even 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 perforation, signs of 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 intussusception 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 Today 28(3):301-4,
1998
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
intussusception. Risks and features. Arch Pediatr 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 intussusception in children. J Pediatr
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-effective work-up. Fibrous 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 lesion is most frequently
found in boys, solitary, identified in the subcutis or lower dermis of
the axillary region, upper arm, upper trunk, inguinal region, scalp, scrotum
and perineum. The lesion varies in size, is firm, fixed to the underlying
tissue and not encapsulated. A few cases show overlying skin changes (pigmentation,
hair). Pathologically they show well-defined bundles of dense, uniform,
fibrous connective tissue projecting into fat, primitive mesenchyme and
mature adipose tissue. Diagnosis has been established by fine-needle aspiration
technique rarely. Standard care consists of local excision to establish
a diagnosis. Observation is not a good option given the uncertainty of
missing a malignant lesion. Incomplete removal might result in recurrence
and need of wider local excision. Mutilating surgery is unnecessary. The
prognosis is excellent.
References
1- Lee JT, Girvan DP, Armstrong RF: Fibrous Hamartoma
of Infancy. J Pediatr Surg 23(8): 759-761, 1988
2- Dickey GE, Sotelo-Avila C: Fibrous hamartoma
of infancy: current review. Pediatr Dev Pathol 2(3):236-43, 1999
3- Popek EJ, Montgomery EA, Fourcroy JL: Fibrous hamartoma
of infancy in the genital 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 Pediatr 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 connection between the dome of 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 patent urachus, a
sinus (opening to the navel), a diverticulum (opening to the bladder),
a cyst (both end close but central portion remains open) or alternating
sinus (cyst-like structure can drain to either the bladder or umbilicus).
Neonatal patent urachus can undergo spontaneous involution. Otherwise,
the transitional epithelium is replaced by granulation tissue and the main
symptom of a patent urachus is urine discharge from the umbilicus associated
with cellulitis, pain and swelling. A cyst or diverticulum can present
as a midline, localized, painful abdominal mass associated with fever and
leucocytosis. The diagnosis can be done with ultrasound (cystic process
is shown), cystography (the ventral position of urachal diverticulum and
fistula is seen, and a bladder-neck obstruction diagnosed) or fistulography
(a connection with the bladder is demonstrated). If infected initial management
consists of antibiotics, otherwise cure is accomplished with excision of
the urachal 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, Rohrschneider WK, Darge K, Troger
J: Sonomorphology and involution of the normal urachus in asymptomatic
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 AA, Gauderer M, Hampel N, Wesselhoeft
CW, Elder JS: Infected urachal cysts: a review of 10 cases. J Urol 140(2):375-8,
1988
5- 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 dominant, and may develop in childhood sporadically or associated
with a multiple endocrine neoplasia (MEN) or Familial syndrome. In sporadic
cases the presentation occurs in adolescence as a thyroid nodule. Production
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 surgery due to extensive 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 familial MTC syndromes. This has
permitted early (prophylactic) gland removal in infancy (MEN IIB)
or early childhood (MEN IIA) before biochemical or clinical MCT develops.
MCT metastasizes to local lymph 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, 1994
2- 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 risk
for multiple endocrine neoplasia type 2A. Ann Surg 220(3):237-47, 1994
3- Skinner MA, DeBenedetti MK, Moley JF, Norton JA, Wells
SA Jr: Medullary thyroid carcinoma in children with multiple endocrine
neoplasia types 2A and 2B. J Pediatr Surg 31(1):177-81, 1996
4- La Quaglia MP, Telander RL: Differentiated and
medullary thyroid cancer in childhood and adolescence. Semin Pediatr Surg
6(1):42-9, 1997
5- Skinner MA, Wells SA Jr: Medullary carcinoma of the
thyroid gland and the MEN 2 syndromes. Semin Pediatr Surg 6(3):134-40,
1997
6- Lallier M, St-Vil D, Giroux M, Huot C, Gaboury L:
Prophylactic thyroidectomy for medullary thyroid carcinoma in gene carriers
of MEN2 syndrome. J Pediatr Surg 33(6):846-8, 1998
7- van Heurn LW, Schaap C, Sie G, Haagen AA, Gerver WJ,
Freling G, van Amstel HK, Heineman E: Predictive DNA testing for
multiple endocrine neoplasia 2: a therapeutic challenge of prophylactic
thyroidectomy in very young children. 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 blood shunting during periods of neonatal asphyxia. Secondary
GP is caused by mechanical disruption (excessive distension or instrumentation)
as observed in situations such as: aggressive mask resuscitation, duodenal
atresia, esophageal atresia with TE fistula, volvulus of the stomach, esophageal
intubation, mechanical ventilation and nasogastric tubes (iatrogenic).
Boys are more commonly affected than girls. Sudden onset of abdominal distension,
feeding intolerance, respiratory distress, metabolic acidosis, shock and
hypoactivity within the first week of life is characteristic. Pneumoperitoneum
is seen in plain abdominal films. Most GP occurs along the greater curvature
of the stomach between the smooth muscle layers. With severely sick infants,
temporary peritoneal drainage with lavage removes gas and acid, decompresses
the abdomen improving ventilation (abdominal compartment syndrome), and
grants time to stabilized the sick infant (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, Bensoussan AL,
Blanchard H, Youssef S: Neonatal gastrointestinal perforations. J Pediatr
Surg 27(10):1340-2, 1992
2- Shashikumar VL, Bassuk A, Pilling GP IV, Cresson SL:
Spontaneous gastric rupture in the newborn: a clinical review of nineteen
cases. Ann Surg 182(1):22-5, 1975
3- Rosser SB, Clark CH, Elechi EN: Spontaneous neonatal
gastric perforation. J Pediatr Surg 17(4):390-4, 1982
4- Tan CE, Kiely EM, Agrawal M, Brereton RJ, Spitz L:
Neonatal gastrointestinal perforation. 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, 1981
6- Aviles T, Lugo-Vicente H, Ocasio MT, Guiven A, Pagán
V, Lliteras O, Vázquez 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
subdivided by history and contrast studies into those with a tendency to
constipation (megasigmoid) or diarrhea (a non-dilatated colon running straight
from the splenic flexure to the anus). Bowel management aims to improve
the personal and social burden inherent to this problem promoting independence
in the affected child. The most effective regimen consists of regular and
complete emptying of the colon 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 administered while the child sits in
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 balloon is deflated and the child allowed to evacuate the colonic content.
Leaving the balloon partially inflated encourages the child to expel it
as a biofeedback mechanism allowing some patients to realize they have
some minimal control to be exploited. If the enema program is effective
in a 3 to 6 month period, the child can become a candidate for a Malone
procedure (appendicostomy). Children with constipation and megasigmoid
needs large volume enemas. Likewise, the large megasigmoid can cause overflow
pseudo incontinence that is only helped with sigmoid resection. Those with
diarrhea may need constipating diet and anti-motility drugs. A few children
that continue with incontinence in spite adequate therapy might benefit
from a permanent colostomy.
References
1- Shandling B, Gilmour RF: The Enema Continence Catheter
in Spina Bifida: Successful Bowel Management. J Pediatr Surg 22(3): 271-273,
1987
2- Blair GK, Djonlic K, Fraser GC, Arnold WD, Murohy
JJ, Irwin B: The Bowel Management Tube: An Effective Means for Controlling
Fecal Incontinence. J Pediatr Surg 27(10): 1269-1272, 1992
3- Peña A, Guardino K, Tovilla JM, Levitt MA,
Rodriguez G, Torres R: Bowel Management for Fecal Incontinence in Patients
with Anorectal malformations. J Pediatr Surg 33(1): 133-137, 1998
4- Levitt MA, Soffer SZ, Pena A: Continent appendicostomy
in the bowel management of fecally incontinent children. J Pediatr Surg
32(11):1630-3, 1997
5- Paidas CN: Fecal incontinence in children with anorectal
malformations. Semin Pediatr Surg 6(4):228-34, 1997
6- Gleeson RM: Bowel continence for the child with a
neurogenic 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-RMS arise from the submucous 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 fecal retention, hematuria,
UTI or palpable mass are initial presentation. Embryonal (75%) and botryoid
(25%) histological variant predominates. MRI is recommended as the key
method of diagnosis and follow-up of pelvic RMS. Unless staging demonstrates
a small or dome lesion amenable to surgical resection, initial management
should consist of high dose multiagent chemotherapy (VAC) and low-dose
irradiation (40 Gy). Response could be: 1- complete, needing follow-up
imaging and cystoscopy, 2- partial (< 50%) or none, needing total cystectomy
or anterior exenteration. Survival is hampered by metastasis, local invasion
and prostatic origin of the tumor. These children need aggressive treatment
modality including total cystectomy. Bladder function preservation is 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, Weinstein HJ, Colodny AH, Shamberger
RC, Delorey M, Tarbell N, Cassady JR, Hendren WH: Genitourinary rhabdomyosarcoma
in children. Cancer 15;63(8):1600-6, 1989
3-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 report from the
Intergroup Rhabdomyosarcoma 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: Prognostic factors in bladder and bladder-prostate
rhabdomyosarcoma. J Pediatr Surg 25(10):1066-72, 1990
5- Hicks BA, Hensle TW, Burbige KA, Altman RP: Bladder
management in children with genitourinary sarcoma. J Pediatr Surg 28(8):1019-22,
1993
6- 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 radiotherapy. A report
from the Intergroup Rhabdomyosarcoma Study (IRS) Committee. J Pediatr Hematol
Oncol 17(1):46-52, 1995
7- Regine WF, Fontanesi J, Kumar P, Ayers D, Bowman LC,
Pappo AS, Coffey DH, Avery L, Rao BN, Kun LE: Local tumor control in rhabdomyosarcoma
following low-dose irradiation: comparison of group II and select group
III patients. Int J Radiat Oncol Biol Phys 31(3):485-91, 1995
8- Heyn R, Newton WA, Raney RB, Hamoudi A, Bagwell C,
Vietti T, Wharam M, Gehan E, Maurer HM: Preservation of the bladder in
patients with rhabdomyosarcoma. J Clin Oncol 15(1):69-75, 1997
9- Merguerian PA, Agarwal S, Greenberg M, Bagli DJ, Khoury
AE, McLorie GA: Outcome analysis of rhabdomyosarcoma 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 condition that is very rarely 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, anorectal
trauma, burns, diaper rash, perianal skin abscesses, and bone marrow transplant.
Most affected children are infants. Patients develop abrupt, rapidly progressive
gangrenous infection with fever, perineal pain, swelling and blistering
of the genital area with systemic signs of toxicity. Infecting organisms
comprise both aerobic and anaerobic organisms. Management consists of broad
spectrum antimicrobial therapy, aggressive and frequent surgical debridement,
and if necessary, urinary and colonic diversions to control the infection.
Fortunately pediatric cases can be successfully 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, Bocchini
JA Jr, Fournier's gangrene in children. Urology 35(5):439-41, 1990
2- Martinelli G, Alessandrino EP, Bernasconi P, Caldera
D, Colombo A, Malcovati L, Gaviglio MR, Vignoli GP, Borroni G, Bernasconi
C: Fournier's gangrene: a clinical presentation of necrotizing fasciitis
after bone marrow transplantation. Bone Marrow Transplant 22(10):1023-6,
1998
3- Efem SE: The features and aetiology of Fournier's
gangrene. Postgrad Med J 70(826):568-71, 1994
4- Muelder K: Fournier's gangrene. World J Surg. 7(5):677,
1983
5- Adeyokunnu AA: Fournier's syndrome in infants. A review
of cases from Ibadan, Nigeria. Clin Pediatr (Phila) 22(2):101-3, 1983