PEDIATRIC SURGERY UPDATE ©
VOLUME 10, 1998
Volume 10 No 01 JANUARY 1998
Caroli's Disease
Caroli's Disease (CD) first described in 1958 by Jacques Caroli as communicating
cavernous ectasia of the biliary tree is an uncommon cause of chronic,
often lifethreatening hepatobiliary disease. CD is a rare condition characterized
by non-obstructive saccular or fusiform dilatation of the intrahepatic
bile ducts usually manifested in childhood, thought to be congenital and
presumably of autosomal recessive hereditary character. Caroli described
two types: Type 1- the rare isolated variety characterized by recurring
episodes of cholangitis. The more frequently occurring Type 2 is associated
with congenital hepatic fibrosis, and consequently there are also symptoms
of portal hypertension. Both types may make their first appearance at a
very early age. Diagnosis can be done non-invasively with hepatobiliary
scintigraphy, ultrasound and echo-doppler. The threats of this condition
are: cholestasis, cholangitis, intrahepatic lithiasis, hepatic failure,
and cholangiocarcinoma. Treatment of the localized form includes lobectomy.
In diffuse disease, treatment may be medical with antibiotics and sometimes
bile solvents. In case of failure, transplantation may be entertained.
Therapy using Ursodeoxycholic acid (10 mg/kg/day) is indicated for intrahepatic
stones in Caroli's syndrome. Patients must be followed closely for many
years to ensure that the intrahepatic ducts do not remain dilated and that
cholangitis do not recur.
References
1- Miller WJ, Sechtin AG, Campbell WL, Pieters PC:
Imaging findings in Caroli's disease. AJR Am J Roentgenol 165(2):333-7,
1995
2- Desroches J, Spahr L, Leduc F, Pomier-Layrargues G,
Picard M; Picard D, Chartrand R, Morais J: Noninvasive diagnosis of Caroli
syndrome associated with congenital hepatic fibrosis using hepatobiliary
scintigraphy. Clin Nucl Med 20(6):512-4, 1995
3- Ros E, Navarro S, Bru C, Gilabert R, Bianchi L, Bruguera
M: Ursodeoxycholic acid treatment of primary hepatolithiasis in Caroli's
syndrome. Lancet 14;342(8868):404-6, 1993
Splenosis
Splenosis refers to autotransplantation of individual fragments of splenic
tissue left behind after either operative or traumatic removal of the spleen.
Although rarely symptomatic, splenosis may cause intestinal obstruction
since the splenules link adjacent loops of bowels to each other, kinking
and obstructing them. Tomographic selective splenic scintigraphy with sulphur
colloid and heatdamaged red cells is the most sensitive method to detect
splenosis. Intramural lesions may also be detected in barium studies of
patients after prior trauma or splenectomy. In large inoculum the splenotic
tissue has been found to have the capacity to remove intranuclear inclusion
bodies from circulating red cells, phagocyte old erythrocytes and confer
some immune protection. Patient who undergo emergency splenectomy for trauma
are at a much higher risk of developing splenosis than those splenectomized
due to hematologic conditions. Spilled splenic tissue seeds the peritoneum,
takes root and grow into vascularized splenules. Experimental evidence
(Folkman) suggests that the presence of a large amount of vascularized
spleen inhibits the growth of other splenic tissue in mice, theory why
patients who undergo partial splenectomy, splenography or are merely observed
after splenic rupture have almost no splenosis. Management of splenosis
is expectant.
References
1- Gunes I, Yilmazlar T, Sarikaya I, Akbunar T,
Irgil C: Scintigraphic detection of splenosis: superiority of tomographic
selective spleen scintigraphy. Clin Radiol 49(2):115-7, 1994
2- Soutter AD, Ellenbogen J, Folkman
J: Splenosis is regulated by a circulating factor. J Pediatr Surg 29(8):1076-9,
1994
3- Hathaway JM, Harley RA, Self S, Schiffman G, Virella
G: Immunological function in post-traumatic splenosis. Clin Immunol Immunopathol
74(2):143-50, 1995
4- Marchant LK, Levine MS, Furth E :
Splenic implant in the jejunum: radiographic and pathologic findings. Abdom
Imaging 20(6):518-20, 1995
5- Prasad C, Beck R: Unusual problems in surgery: Splenosis
and intestinal obstruction. Mt Sinai J Med 35: 534-540, 1968
Pancreas Development
The pancreas develops from an anterior and posterior anlage of
the foregut early during gestation (28 days). The anterior bud leads to
the liver and body and tail of the pancreas. The posterior diverticulum
develops into the head of the pancreas. This bud rotates anteriorly and
later fuses to achieve the relationship to the rest of the pancreas. Development
of the pancreas in embryonic life requires a trophic stimulus from the
associated mesenchyme. Under the influence of this mesenchyme the mature
organ develops, being mainly composed of ductal, exocrine and endocrine
cells. Exocrine and ductal pancreas are derived from the endoderm of the
foregut. Recent evidence suggests that the endocrine cells derive also
from the endoderm of the foregut as evidenced by the expression of the
genes responsible for hormonal production. This challenges the theory that
endocrine cells may originate from the neural crest cells (neuroectodermal)
of the embryo reinforced by the enunciation of the amine precursor uptake
decarboxylase (APUD) theory.
References
1- Debas HT: Molecular Insights into the Development of
the Pancreas. Am J Surg 174(3): 227-231, 1997
2- Madsen OD, Jensen J, Blume N, Petersen
HV, Lund K, Karlsen C, Andersen FG, Jensen PB,
Larsson LI, Serup P: Pancreatic development and maturation of the islet
B cell. Studies of pluripotent islet cultures. Eur J Biochem 15;242(3):435-45,
1996
3- Slack JM: Developmental biology of the pancreas.
Development 121(6):1569-80, 1995
Volume 10 No 02 FEBRUARY 1998
Thyroid Nodules: FNAB
The need to differentiate malignant from benign thyroid nodules is the
most challenging predicament in management.Present diagnostic work-up consists
of ultrasonography (US), radionuclear scans (RNS) and fine-needle aspiration
biopsy (FNAB). After reviewing our ten-year experience with twenty-four
pediatric thyroid nodules we found nineteen benign and five malignant lesions.
Benign nodules were soft, movable, solitary and non-tender. Malignant nodules
were found during late adolecence, characterized by localized tenderness,
a multigandular appearance and fixation to adjacent tissues. US and RNS
were of limited utility since malignancy was identified among cystic and
hot nodules respectively. Suppressive thyroid hormone therapy was useless
in the few cases tried. FNAB in eighteen cases did not limit the number
of thyroid resections. It showed that the probability that a malignant
nodule had suspicious or frankly malignant cytology was 60%. The specificity
was 90%. This is the result of a higher number of patients with follicular
cell cytology in the aspirate. No attempts should be made to differentiate
follicular adenoma from carcinoma since capsular and vascular invasion
cannot be adequately assessed by FNAB.The physical exam findings, persistence
of the nodule, progressive growth and cosmetic appearance were the main
indications for surgery. FNAB is a safe procedure that plays a minor role
in the decision to withhold surgery. Its greatest strength is to anticipate
in case of malignancy that a more radical procedure is probably needed.
FNAB, US and RNS should not replace clinical judgement or suspicion as
the most important determinants in management.
References
1- Lugo-Vicente HL: Pediatric Thyroid Nodules: Management
in the era of FNA (submitted for publication).
2- Lugo-Vicente HL: Pediatric Thyroid Nodules: Insights
in Management (in press).
Li-Fraumeni Syndrome
The Li-Fraumeni familial cancer syndrome is manifested by increased
susceptibility of affected relatives to develop a diverse set of
malignancies during early childhood. The major features of the syndrome
include breast cancer, sarcomas of soft tissue and bone, brain tumor, leukemia
and adrenal cortical carcinoma. More than one-half of the cancers overall
and nearly one-third of the breast cancers were diagnosed before 30 years
of age. Among females, breast cancer is the most common. Germline mutations
within a defined region of the p53 gene have been found in families with
the Li-Fraumeni syndrome. Persistence of the mutation in the germline suggests
a defect in DNA repair in the family member first affected. Asymptomatic
carriers of p53 germline mutation needs closed evaluation and follow-up
for early detection and treatment in case neoplasia develops.
References
1- Garber JE, Goldstein AM, Kantor AF, Dreyfus
MG, Fraumeni JF Jr, Li FP: Follow-up study of twenty-four families with
Li-Fraumeni syndrome. Cancer Res 51(22):6094-7, 1991
2- Santibanez-Koref MF, Birch JM, Hartley AL, Jones PH,
Craft AW, Eden T,Crowther D, Kelsey AM, Harris M: p53 germline mutations
in Li-Fraumeni syndrome.Lancet 338(8781):1490-1, 1991
3- Tricker KJ, Prosser J, Condie A, Kelsey AM, Harris
M, Jones PH, Binchy A,Crowther D, et al: Prevalence and diversity
of constitutional mutations in the p53 gene among 21 Li-Fraumeni families.
Cancer Res 54(5):1298-304, 1994
4- Frebourg T, Barbier N, Yan YX, Garber JE, Dreyfus M,
Fraumeni J Jr, Li FP, Friend SH: Germ-line p53 mutations in 15 families
with Li-Fraumeni syndrome. Am J Hum Genet 56(3):608-15, 1995
5- Strauss EA, Hosler MR, Herzog P, Salhany
K, Louie R, Felix CA :Complex replication error causes p53 mutation in
a Li-Fraumeni family. Cancer Res 55(15):3237-41, 1995
Omphalo-Mesenteric Remnants
Before placental circulation is established fetal nourishment occurs
from the yolk sac through the omphalomesenteric duct. By the 5th to 7th
intrauterine week the duct obliterates. Persistence of the duct might give
rise to a wide spectrum of omphalomesenteric remnants (OMR). Most OMR are
in the form of a Meckel's diverticulum toward the intestinal end. Other
less OMR are in the form of a mucosa-lined sinus or blind pouch at the
umbilicus, an umbilical polyp, an intra- or extraperitoneal cyst, a connective
tissue cord, or a well-formed communication between the ileum and the umbilicus.
Pluripotential ectopic tissues (gastric, duodenal, colonic or pancreatic)
might be found within OMR causing further problems (bleeding, perforation,
obstruction, intussusception). Clinically the infant manifests periumbilical
reddening, ulceration, granuloma, fluid discharge (bowel content), or recurrent
umbilical infections. Diagnosis should come to your mind with recurrent
umbilical discharge, non-healing granuloma, cherry-red nodule, or a rosette-like
opening. Differential diagnosis consists of local infection (omphalitis),
urachal remnants, dermoid cysts or vascular malformations. Management is
umbilical exploration and surgical excision after suspicion or radiographic
diagnosis (US, sinogram) is established.
References
1- Moore TC: Omphalomesenteric duct malformations.
Semin Pediatr Surg 5(2):116-23, 1996
2- Mothes W: [Complications caused by remnants of the
omphalomesenteric duct] Zentralbl Chir 115(22):1431-4, 1990
3- Jauniaux E, De Munter C, Vanesse M, Wilkin P, Hustin
J: Embryonic remnants of the umbilical cord: morphologic and clinical aspects.
Hum Pathol 20(5):458-62, 1989
4- Gaisie G, Curnes JT, Scatliff JH, Croom RD, Vanderzalm
T: Neonatal intestinal obstruction from omphalomesenteric duct remnants.
AJR Am J Roentgenol 144(1):109-12, 1985
5- Schärli AF: Vitello-intestinal disorders, In Neill
V. Freeman's Surgery of the Newborn, Churchill Livingstone Ed, UK, 1994,
pag. 243-255
6- Chapter 48: Disorders of the Umbilicus, In Marc I.
Rowe's Essential in Pediatric Surgery, Mosby Publishers, USA, 1995, pag.
441-445
Volume 10 No 03 MARCH 1998
Hirschsprung's Disease
-
Hirschsprung's disease (HD) is the congenital absence of parasympathetic
innervation of the distal intestine. Occurs one in 1000-1500 live births
with a 4:1 male predominance; 96% are term and 4% prematures babies. Symptoms
usually begin at birth with delayed passage of meconium. In some infants,
the presentation is that of complete intestinal obstruction. Others have
few symptoms until several weeks of age, when the classic symptom of constipation
has its onset. Initial evaluation includes an unprepped barium enema (the
first enema should be a barium enema!). The aganglionic rectum appears
of normal caliber or spastic, there is a transition zone and then dilated
colon proximal to the aganglionic segment. Rectal suction biopsy is then
performed and the submucosal plexus is examined for ganglion cells. Difficulty
in interpreting the specimen would require a full-thickness biopsy for
definitive diagnosis. Conventional treatment requires performing a "leveling"
colostomy in the most distal colon with ganglion cells present. Placement
of the colostomy in an area of aganglionosis will lead to persistent obstruction.
Once the child has reached an adequate size and age a formal pull-through
procedure is done. Current preference is for Soave procedure (modified
endorectal pull-through). A tendency toward primary pull-through without
colostomy in early infancy is being reported along with a laparoscopic
version of this procedure with a decrease in morbidity and hospital stay.
-
References
-
1- Brennan LP, Weitzman JJ, Swenson O: Pitfalls in the
Management of Hirschsprung's Disease. J Pediatr Surg. 2(1): 112, 1967
-
2- Pierro A, Fasoli L, Kiely EM, Drake D, Spitz L: Staged
pull-through for rectosigmoid Hirschsprung's disease is not safer than
primary pull-through. J Pediatr Surg 32(3):505-9, 1997
-
3- Martucciello G: Hirschsprung's disease as a neurochristopathy.
Pediatr Surg Int 12(1):2-10, 1997
-
4- Skinner MA: Hirschsprung's disease. Curr Probl Surg
33(5):389-460, 1996
-
5- Teitelbaum DH: Hirschsprung's disease in children.
Curr Opin Pediatr 7(3):316-22, 1995
-
6- Rothenberg S, Chang JH: Laparoscopic pull-through
procedures using the harmonic scalpel in infants and children with Hirschsprung's
disease. J Pediatr Surg 32(6):894-6, 1997
-
7- Georgeson KE, Fuenfer MM, Hardin WD: Primary laparoscopic
pull-through for Hirschsprung's disease in infants and children. J Pediatr
Surg 30(7):1017-21, 1995; discussion 1021-2
-
Asymptomatic Malrotation
-
Contrast studies often done for other reasons may disclose the presence
of a rotational anomaly of the bowel in an asymptomatic child. This trigger
the question whether surgery is needed to reduce the risk of volvulus (midgut
infarction) in the life expectancy of the affected patient. To make a complete
assessment of the rotational anomaly and know the location and existence
of the duodeno-jejunal (Treitz) and ileo-cecal junctions an UGIS with follow-through
and barium enema will be neccesary. If the rotational anomaly shows that
these two junctions are near each other (narrowing of the mesenteric base)
and proximal to the superior mesenteric artery the threat of volvulus becomes
real and prophylactic Ladd's procedure should be offered. Ladd's procedure
can be done laparoscopically. Patients with malrotation more likely have
bands, mesenteric defects, foreshortened dorsal root and redundant leafs.
Doppler color US or CT can tell whether there is an anatomic change in
the position of the superior mesenteric vein that suggests volvulus. Children
with non-rotation associated to a surgical condition (diaphragmatic hernia,
abdominal wall defects, prune belly, etc.) benefits from the adhesions
created during primary repair and seldom develop volvulus. The morbidity
after a Ladds procedure might be significant in some patients.
-
References
-
1- Schey WL, Donaldson JS, Sty JR: Malrotation of Bowel:
Variable Patterns with Different Surgical Considerations. J Pediatr Surg
28(1): 96-101, 1993
-
2- Zerin JM, DiPietro MA: Superior mesenteric vascular
anatomy at US in patients with surgically proved malrotation of the midgut.
Radiology 183(3):693-4, 1992
-
3- Feitz R, Vos A: Malrotation: The Postoperative Period.
J Pediatr Surg 32 (9): 1322-1324, 1997
-
4- Gross E, Chen MK, Lobe TE: Laparoscopic evaluation
and treatment of intestinal malrotation in infants. Surg Endosc 10(9):936-7,
1996
-
5- Chou CK, Mak CW, Hou CC, Chang JM, Tzeng WS: CT of
the mesenteric vascular anatomy. Abdom Imaging 22(5):477-82, 1997
-
6- Long FR, Kramer SS, Markowitz RI, Taylor GE: Radiographic
patterns of intestinal malrotation in children. Radiographics 16(3):547-56,
1996; discussion 556-60
-
7- Maxson RT, Franklin PA, Wagner CW: Malrotation
in the older child: surgical management, treatment, and outcome. Am Surg
61(2):135-8, 1995
-
Heterotaxia Syndrome
-
Helwig is credited with describing the Heterotaxia (Polysplenia) syndrome
in 1929. The syndrome a defect of lateralization commonly thought of as
"bilateral left sidedness" is highly variable in its anatomic expression.
Is a rare congenital disorder characterized by abnormal viscero-vascular
situs with either left or right isomerism that usually coincides with complex
cardiac malformation. Consist of polysplenia, intestinal malrotation, absence
of the inferior vena cava, situs inversus, preduodenal portal vein, abnormalities
of the hepatic artery and cardiac defects. The syndrome frequently involves
visceral disorders such as malposition, malrotation or malfixation of abdominal
organs. Malrotation is the most frequent anomaly encountered and classically
presents with duodenal obstruction during early infancy. Malfixation of
the stomach might produce gastric volvulus. Management in this cases consist
of both Ladd's procedure and gastropexy. The polysplenia syndrome is the
most common extrahepatic anomaly found in association with Biliary Atresia.
In no way the syndrome jeopardized the result of porto enterostomy in these
children.
-
References
-
1- Helwig FC: Multiple spleen combined with other congenital
abnormalities. Arch Pathol 8: 761-767, 1929
-
2- Vazquez J, Lopez Gutierrez JC, Gamez M, Lopez-Santamaria
M, Murcia J, Larrauri J, Diaz MC, Jara P, Tovar JA: Biliary Atresia and
the Polysplenia Syndrome: Its Impact on Final Outcome. J Pediatr Surg 30
(3): 485-487, 1995
-
3- Stewart DE, Steigman CK, Mahoney KJ, Signs MM, Cobb
LM: Obstructive Jaundice Associated with Polysplenia Syndrome in an Older
Child. J Pediatr Surg 27 (12): 1575-1577, 1992
-
4- Nakada K, Kawaguchi F, Wakisaka M, Nakada M, Enami
T, Yamate N: Digestive Tract Disorders Associated with Asplenia/Polysplenia
Syndrome. J Pediatr Surg 32(1): 91-94, 1997
-
5- Oleszczuk-Raschke K, Set PA, von Lengerke HJ, Troger
J: Abdominal sonography in the evaluation of heterotaxy in children. Pediatr
Radiol 25 Suppl 1:S150-6, 1995
VOLUME 10 No 04 APRIL 1998
Mediastinal Cysts
-
Mediastinal cysts identified in children are classified according to
the compartment where they arise as: anterior (extends to the sternum,
thoracic inlet and anterior border of the heart), middle (between anterior
mediastinum and anterior borders of the vertebrae) or posterior mediastinum.
Although usually asymptomatic, they require excision for purpose of diagnosis
and avoidance of symptoms such as chest pain, airway obstruction, hemoptysis
or dysphagia. Diagnosis can be accomplished with the use of CT-Scan, US
and esophagogram. Some of the most common encounter cysts in the mediastinum
are: bronchogenic cysts, neurenteric cysts, pericardial cysts, cystic hygroma,
thymic and dermoid cysts.
-
References
-
1- Mediastinal Masses: Marc Rowe's ‘Essential of Pediatric
Surgery', Mosby Year Book Publishers, 1995, Chapter 31, pag 306-310
-
2- Robie DK, Gursoy MH, Pokorny WJ: Mediastinal Tumors
- Airway Obstruction and Management. Sem Pediatr Surg 3(4): 259-266, 1994
-
3- Strollo DC, Rosado de Christenson ML, Jett JR: Primary
mediastinal tumors. Part 1: tumors of the anterior mediastinum. Chest 112(2):511-22,
1997
-
Bronchogenic Cysts
-
Bronchogenic cysts (BC), first described in 1911, are benign congenital
lesions of the respiratory tract that have the potential to develop complications
creating a dilemma in diagnosis and treatment. BC are commonly located
in the mediastinum (2/3) or lung parenchyma (1/3) arising from anomalous
budding along the primitive tracheobronchial tube (foregut duplication
errors). Other atypical locations are cervical, subcutaneous, paravertebral,
etc. Contain mucoid material lined with ciliated columnar epithelium (bronchial
glands, smooth muscle, cartilage) not communicating with the respiratory
tract. Clinical presentation may range from prenatal diagnosis, asymptomatic
(1/3) lesions identified during routine work-up to symptomatic (2/3) cases.
Infants may show respiratory distress: cough, dyspnea, cyanosis, hemoptysis
or dysphagia. Older children present with chest pain, non-productive cough
or pulmonary infection. Diagnosis relies on chest films and CT-Scan. Bronchoscopy
and barium swallow are not very useful. Infection, hemorrhage, erosion,
malignant potential and expansion mandate surgical management consisting
of thoracotomy with excision of the lesion if mediastinal in location,
and segmentectomy or lobectomy for intraparenchymal cysts. Marsupialization
is associated with recurrence.
-
References
-
1- Cartmill JA, Hughes CF: Bronchogenic cysts: a persistent
dilemma. Aust N Z J Surg 59(3):253-6, 1989
-
2- Di Lorenzo M, Collin PP, Vaillancourt R, Duranceau
A: Bronchogenic cysts. J Pediatr Surg 24(10):988-91, 1989
-
3- Ribet ME, Copin MC, Gosselin B: Bronchogenic cysts
of the mediastinum. J Thorac Cardiovasc Surg 109(5):1003-10, 1995
-
4- dell'Agnola C, Tadini B, Mosca F, Colnaghi M, Wesley
J: Advantages of prenatal diagnosis and early surgery for congenital cystic
disease of the lung. J Perinat Med 24(6):621-31, 1996
-
5- Nobuhara KK, Gorski YC, La Quaglia MP, Shamberger RC:
Bronchogenic cysts and esophageal duplications: common origins and treatment.
J Pediatr Surg 32(10):1408-13, 1997
-
Neurenteric Cysts
-
The rare neurenteric cyst (NC), also call enterogenous or gastrogenous
cysts, is a combination of an endodermal (duplication) cyst of foregut
origin with a spinal canal dysraphism (cleft, hemivertebrae, spina bifida).
NC represent failure of complete separation of the notochord from the foregut
during the 3rd week of embryogenesis. NC are found in the posterior mediastinum,
superior to the carina and to the right side. Symptoms of respiratory distress
become obvious during the first months of life. Those lined with gastric
epithelium might develop hemorrhage, ulceration or erosion. NC are either
tubular or spherical, a minority communicating with the GI tract below
the diaphragm. Respiratory distress, a posterior mediastinal mass and a
thoracic vertebral defect in x-ray suggest the diagnosis. CT, MRI and myelography
(intraspinal component) are precise. Therapy of choice is complete resection.
-
References
-
1- Alrabeeah A, Gillis DA, Giacomantonio M, Lau H: Neurenteric
Cysts - A Spectrum. J Pediatr Surg 23 (8): 752-754, 1988
-
2- Gilchrist BF, Harrison MW, Campbell JR: Neurenteric
Cyst: Current Management. J Pediatr Surg 25 (12): 1231-1233, 1990
-
3- Rizalar R; Demirbilek S; Bernay F;
Gurses N: A case of a mediastinal neurenteric cyst demonstrated by prenatal
ultrasound. Eur J Pediatr Surg 5(3):177-9, 1995
-
5- Bilik R, Ginzberg H, Superina RA: Unconventional Treatment
of neuroenteric Cyst in a Newborn. J Pediatr Surg 30 (1): 115-117, 1995
-
4- Nobuhara KK, Gorski YC, La Quaglia MP, Shamberger RC:
Bronchogenic cysts and esophageal duplications: common origins and treatment.
J Pediatr Surg 32(10):1408-13, 1997
-
Thymic Cysts
-
Thymic cysts are benign lesion that can arise either aberrantly in the
neck (laterally, deep to anterior border of sternocleidomastoid muscle)
or in the anterior mediastinum. Believed to develop from remnants of thymic
tissue that have failed to descend from the ventral wing of the third branchial
pouch into the mediastinum during the 6th to 8th week of fetal life. Most
cases in the neck produce no symptoms and usually appear incidentally between
ages 6 and 8 years as a soft swelling in the anterior neck triangle rarely
invading contiguous structures. Others children might develop respiratory
distress, tracheal compression, swelling and enlargement due to hemorrhage
or infection. Malignant transformation has also been documented. Preoperative
diagnosis is seldom achieved as they are confused with branchial cleft
cysts and cystic hygromas. Complete excision is management of choice.
-
References
-
1- Johnsen NJ, Bretlau P: Cervical thymic cysts. Acta
Otolaryngol (Stockh) 82(1-2):143-6, 1976
-
2- Reiner M, Beck AR: Cervical thymic cysts in children.
Am J Surg 139(5):704-7, 1980
-
3- Graeber GM, Thompson LD, Cohen DJ, Ronnigen LD, Jaffin
J, Zajtchuk R: Cystic lesion of the thymus. An occasionally malignant cervical
and/or anterior mediastinal mass. J Thorac Cardiovasc Surg 87(2):295-300,
1984
-
4- Spigland N, Bensoussan AL, Blanchard H, Russo P: Aberrant
Cervical Thymus in Children: Three Cases Reports and Review of the Literature.
J Pediatr Surg 25(11): 1196-1199, 1990
-
5- Burton EM, Mercado-Deane MG, Howell CG, Hatley R, Pfeifer
EA, Pantazis CG, Chung C, Lorenzo RL: Cervical thymic cysts: CT appearance
of two cases including a persistent thymopharyngeal duct cyst. Pediatr
Radiol 1995;25(5):363-5
Volume 10 No 05 MAY 1998
-
CDH Study Group
-
by: Kevin Lally, MD
-
Repair of congenital diaphragmatic hernia (CDH) has changed from an
emergent to a delayed procedure in the last decade. Lack of a large multi-center
database has hampered progress in the management of CDH making determination
of current standard difficult. The CDH Study Group was formed in 1995 to
collect data from multiple institutions in North America, Europe and Australia.
Participating centers completed a registry form on all live-born infants
with CDH during 1995 and 1996. Demographic information, data about surgical
management and outcome was collected. Sixty-two centers participated, with
461 patients entered. Overall survival was 280 of 442 patients (63%). The
defect was left-sided in 78%, right-sided in 21% and bilateral in 1%. Subcostal
approach was used in 91% of patients, with pleural drainage in 76%. A patch
was used in 51% of the patients, with PTFE being the most commonly used
material (81%). Mean operative time was 102 minutes, with an average blood
loss of 14 cc (0-500 cc). A majority of patients underwent repair between
6 AM and 6 PM (88%). 19% had surgical repair on ECMO at a mean time
of 170 hours into the ECMO course (10 to 593 hours). Mean age at operation
in patients not treated with ECMO was 73 hours (1 to 445 hours). The data
indicates that prosthetic patching of the defect has become common, that
after-hours repair is infrequent and that delayed operative repair has
become the preferred approach in most centers. Furthermore, the mean survival
of 63% indicates that despite decades of individual effort, the CDH problem
is far from solved.
Duodenal Stenosis
-
Congenital partial obstruction of the duodenum can be either intrinsic
(membrane, web or pure) or extrinsic (Ladd's bands, annular pancreas).
A significant group (25-33%) is born with Down's syndrome. This does not
entail a higher risk of early mortality unless associated with cardiac
malformations. Other associated conditions are malrotation (midgut volvulus
is rare due to absent bowel distension and peristalsis), biliary tract
anomalies and Meckel's diverticulum. The diagnosis is suggested in utero
by the double-bubble image on ultrasound. Vomiting is the most frequent
presenting symptom. UGIS is diagnostic, showing a dilated stomach and first
duodenal portion with scanty passage of contrast material distally. Management
varies accordingly to the type of stenosis: Ladd's bands are lysed. Pure
stenosis is opened longitudinally and closed transversely (Heineke-Mickulicz).
Membranous stenosis is resected. Successful endoscopic membranectomy of
duodenal stenosis has been reported. Duodeno-duodenostomy is the procedure
of choice for annular pancreas. Diaphragms can rarely be double. Anastomotic
malfunction requiring prolonged intravenous nutrition and hospitalization
has prompted development of a diamond shape larger stoma. Tapering or plication
of the dilated duodenum is another effective method of improving disturbed
transit. Other complications after surgery are megaduodenum with blind
loop syndrome, biliary reflux, cholestatic jaundice, delayed transit and
bowel obstruction. Early mortality is associated to prematurity and associated
malformations. Long-term follow-up is warranted to identify late problems.
-
References
-
1- Stauffer UG, Irving I: Duodenal Atresia and Stenosis
- Long Term Results. Prog Pediatr Surg 10: 49-63, 1977
-
2- Okamatsu T, Arai K, Yatsuzuka M, et al: Endoscopic
Membranectomy for Congenital Duodenal Stenosis in an Infant. J Pediatr
Surg 24 (4): 367-368, 1989
-
3- Kimura K, Mukohara N, Nishijima E, et al: Diamond Shaped
Anastomosis for Duodenal Atresia: An Experience with 44 patients over 15
years. J Pediatr Surg 25 (9): 977-979, 1990
-
4- Spigland N, Yazbeck S: Complications Associated with
Surgical treatment of Congenital Intrinsic Duodenal Obstruction. J Pediatr
Surg 25 (11): 1127-1130, 1990
-
5- Stringer MD, Brereton RJ, Draje DP, et al: Double Duodenal
Atresia/Stenosis: A Report of Four Cases. J Pediatr Surg 27 (5): 576-580,
1992
-
6- Samuel M, Wheeler RA, Mami AG: Does Duodenal Atresia
and Stenosis Prevent Midgut Volvulus in Malrotation? Eur J Pediatr Surg
7:11-12, 1997
-
7- Takahashi A, Tomomasa T, Suzuki N, et al: The Relationship
Between Disturbed Transit and Dilated Bowel, and Manometric Findings of
Dilated Bowel in Patients with Duodenal Atresia and Stenosis. J Pediatr
Surg 32 (8): 1157-1160, 1997
Bile Peritonitis
-
Bile peritonitis has been reported after conservative management of
blunt hepatic trauma. The source is a major bile duct or peripheral injury.
Diagnosis may be delayed for several days when the child insidiously develops
symptoms of abdominal pain, jaundice and fever. CT-Scan shows the liver
fracture with abdominal effusion. ERCP can rule out the extrahepatic origin
of the problem. Percutaneous drainage with antibiotic has been successful
management with peripheral liver injuries. Most cases explored will need
cholangiography to identify a major extrahepatic bile duct injury.
-
References
-
1- Poli ML, Lefebvre F, Ludot H, Bouche-Pillon MA, Daoud
S, Tiefin G: Nonoperative management of biliary tract fistulas after blunt
abdominal trauma in a child. J Pediatr Surg 30(12):1719-21, 1995
-
2- Berman SS, Mooney EK, Weireter LJ Jr: Late fatal hemorrhage
in pediatric liver trauma. J Pediatr Surg 27(12):1546-8, 1992
-
3- Oldham KT, Guice KS, Ryckman F, Kaufman RA, Martin
LW, Noseworthy J: Blunt liver injury in childhood: evolution of therapy
and current perspective. Surgery 100(3):542-9, 1986
-
4- Barker SL, Fromm D: Bile peritonitis following expectant
management of liver fracture. N Y State J Med 87(10):565-7, 1987
-
VOLUME 10 No 06 JUNE 1998
Imperforate Hymen
-
During infancy an imperforate hymen (IH) might produce a mucocolpos,
and if not corrected by puberty a hematocolpus or hydrometrocolpus. As
the vagina and uterus fills the hymen membrane distends and bulges, protruding
as a globular mass beyond the introitus (yellowish or grayish white).
IH results when mesoderm of the primitive streak abnormally invades the
urogenital portion of the cloacal membrane. Clinically the infant presents
with an abdomino-pelvic mass, constipation, symptoms of urinary retention
and dilatation of the upper urinary tract. Other times amenorrhea during
early adolescence might be uncovered an IH. Differential diagnosis includes:
labial adhesions, vaginal agenesis, vaginal cyst, ectopic ureter, prolapse
urethra and ureterocele. US and MRI are useful diagnostic studies. Diagnosis
can be made prenatally as early as the second trimester of pregnancy. IH
must be corrected by surgery when discover. At the time of surgery the
urethra is inspected and catheterized, the IH incised and the vagina evacuated.
Cut edges are sutured to the vagina mucosa.
-
References
-
1- Congenital Anomalies of the Female Genitalia, In Huffman,
Dewhurst & Capraro "The Gynecology of Childhood and Adolescence", WB
Saunders, 2nd edition, 1981, pag 156-159
-
2- Peterson-Sweeney KL, Stevens J: 13-year-old female
with imperforate hymen. Nurse Pract 21(8):90-4, 1996
-
3- Loscalzo IL, Catapano M, Loscalzo J; Sama A: Imperforate
hymen with bilateral hydronephrosis: an unusual emergency department diagnosis.
J Emerg Med 13(3):337-9, 1995
-
4- Winderl LM, Silverman RK: Prenatal diagnosis of congenital
imperforate hymen. Obstet Gynecol 85(5 Pt 2):857-60, 1995
-
5- Bejanga BI: Hematocolpos with imperforate hymen. Int
Surg 63(2):97-9, 1978
-
6- Reynolds M: Neonatal Disorders of the External Genitalia
and Vagina. Sem Pediatr Surg 7(1): 2-7, 1998
Esophageal Perforation
-
Most esophageal perforation in newborns occurs in the cervical portion
while trying to intubate the trachea (iatrogenic). Injury produced by laryngoscope
blades, pharyngeal suction catheters, nasogastric and endotracheal tubes
are generally unrecognized until the baby develops signs of esophageal
obstruction mimicking esophageal atresia (excessive salivation, cyanotic
spells, and regurgitation upon feeding), radiographic evidence of pharyngeal
perforation (usually in the posterior mediastinum) or a right-sided pneumothorax.
The child most at risk is the small for gestational age or premature baby.
Types of injuries identified: 1) pharyngeal pseudodiverticulum, 2) mucosal
perforation posteriorly and parallel to the esophagus, and 3) intrapleural
perforation. Diagnosis can be strongly suspected from the findings on the
chest x ray or confirmed by performing an esophagogram (‘double esophagus'
sign). Management depends on extent and location of injury. Overall, perforations
of the pharynx and esophagus in neonates can be satisfactorily managed
medically with antibiotics and parenteral nutrition except in cases that
require mediastinal decompression or chest tube placement. Fluoroscopically
placed NG tubes will allow gastric feedings. Key to prevention: use of
soft-tipped suction catheters and nasogastric tubes and careful visualization
of the cords during endotracheal intubation. Metal stylets to direct endotracheal
tubes are dangerous. Perforations in older infants and children are associated
to foreign body, esophageal dilatation, trauma or lye ingestion dealt depending
on their causative factor.
-
References
-
1- Lee SB, Kuhn JP: Esophageal perforation in the neonate.
A review of the literature. Am J Dis Child 130(3):325-9, 1976
-
2- Touloukian RJ, Beardsley GP, Ablow RC, Effmann EL:
Traumatic perforation of the pharynx in the newborn. Pediatrics 59 Suppl(6
Pt 2):1019-22, 1977
-
3- Grunebaum M, Horodniceanu C, Wilunsky E, Reisner S:
Iatrogenic transmural perforation of the oesophagus in the preterm infant.
Clin Radiol 31(3):257-61, 1980
-
4- Mollitt DL, Schullinger JN, Santulli TV: Selective
management of iatrogenic esophageal perforation in the newborn. J Pediatr
Surg 16(6):989-93, 1981
-
5- Johnson DE, Foker J, Munson DP, Nelson A, Athinarayanan
P, Thompson TR: Management of esophageal and pharyngeal perforation in
the newborn infant. Pediatrics 70(4):592-6, 1982
-
6- Blair GK, Filler RM, Theodorescu D: Neonatal pharyngoesophageal
perforation mimicking esophageal atresia: clues to diagnosis. J Pediatr
Surg 22(8):770-4, 1987
-
7- Krasna IH, Rosenfeld D, Benjamin BG, Klein G, Hiatt
M, Hegyi T: Esophageal Perforation in the Neonate: An Emerging Problem
in the Newborn Nursery. J Pediatr Surg 22(8): 784-790, 1987
-
8- Engum SA, Grosfeld JL, West KW, Rescorla FJ, Scherer
LR, Vaughan WG: Improved survival in children with esophageal perforation.
Arch Surg 131(6):604-10; discussion 611, 1996
Dysgerminoma
-
Thought to arise from the germ cells of the sexually indifferent stages
of gonadogenesis, this malignant ovarian tumor is equivalent to the seminoma
of testes in males. Occur most often in adolescents (60% of dysgerminoma
develops during the first two decades of life), as a rapidly growing asymptomatic
heavy solid pelvic mass. Grossly characterized as a smooth surface, nodular,
encapsulated solid tumor. Rarely, they may secrete hormones (gonadotropin,
PTH-like substance) producing sexual precocity or hypercalcemia. Unilateral
salpingo-oophorectomy is adequate surgery if the tumor is unilateral, encapsulated,
mobile, the opposite ovary is normal, there is no ascites and the retroperitoneal
nodes are not enlarged or abnormal. Ascites, bilateral tumors and evidence
of extension are bad prognosis signs and should be managed by total abdominal
hysterectomy and bilateral salpingo-oophorectomy with irradiation. Recurrence
of the tumor during the first two years after treatment is an ominous sign.
Teratomatous or trophoblastic foci heralds a bad prognosis.
-
References
-
1- Tumors of the Sexual Organs, In Altman & Schwartz
‘Malignant Diseases of Infancy, Childhood and Adolescence', WB Saunders,
2nd ed, 1983, pag 494-495
-
2- Ovarian Tumors in Children and Adolescent, In Huffman's
‘The Gynecology of Childhood and Adolescence', WB Saunders, 2nd ed, 1981,
pag 315-317
-
3- Adkins JC: Malignant Ovarian and Other germ Cell Tumors.
In D.M. Hays ‘Pediatric Surgical Oncology', Grune & Stratton, 1986,
pag 127-128
-
4- Anstey A,Gowers L,Vass A,Robson AO: Ovarian dysgerminoma
presenting with hypercalcaemia. Case report and review of the literature.
Br J Obstet Gynaecol 97(7):641-4, 1990
-
5- Wu PC,Huang RL,Lang JH, Huang HF, Lian LJ; Tang MY:
Treatment of malignant ovarian germ cell tumors with preservation of fertility:
a report of 28 cases. Gynecol Oncol 40(1):2-6, 1991
-