PEDIATRIC SURGERY UPDATE ©
VOLUME 18, 2002
Volume 18 No 01 JANUARY 2002
Spontaneous Pneumothorax
Most pneumothorax in children are the result of blunt/open chest trauma,
mechanical ventilation (barotrauma), bronchial asthma or an infectious
pulmonary process. Primary spontaneous pneumothorax (PSP) is rare in children
with most cases seen in adolescent males with thin body habitus. Main presenting
symptoms consist of chest pain, cough and shortness of breath. Recurrence
is high in this older population of children. PSP is usually the result
of: 1) a ruptured apical bleb or bullae in three-fourth cases, 2) destructive
parenchymal disease (cystic fibrosis, AIDS), or 3) alveolar rupture due
to proximal airway obstruction. Initial management consists of oxygen supplementation
for small pneumothorax less than 15% with no tension physiology present.
Chest tube drainage is needed for medium or large size pneumothorax. Recurrence
or persistent pneumothorax is managed with video-assisted thoracoscopic
surgery (VATS) by ablating with endoscopic stapling (Endo GIA), suturing
or ligating using an endoloop technique the apical bullae followed by pleurodesis.
Pleurodesis can be done chemically or surgically. Chemical pleurodesis
is achieved with such agents as talc, tetracycline, bleomycin or quinacrine
instillation. Mechanical pleurodesis carries a lower recurrent rate and
can be achieved by abrasion or electrocoagulation. Most common complication
is persistent air leak. VATS is a fast, cost-effective method of treatment
for PSP with less morbidity.
References:
1- Poenaru D, Yazbeck S, Murphy S: Primary spontaneous
pneumothorax in children. J Pediatr Surg 29(9):1183-5, 1994
2- Yim AP: Video-assisted thoracoscopic suturing of apical
bullae. An alternative to staple resection in the management of primary
spontaneous pneumothorax. Surg Endosc 9(9):1013-6, 1995
3- Wilcox DT, Glick PL, Karamanoukian HL, Allen JE, Azizkhan
RG: Spontaneous pneumothorax: a single-institution, 12-year experience
in patients under 16 years of age. J Pediatr Surg 30(10):1452-4,
1995
4- Bertrand PC, Regnard JF, Spaggiari L, Levi JF, Magdeleinat
P, Guibert L, Levasseur P: Immediate and long-term results after surgical
treatment of primary spontaneous pneumothorax by VATS. Ann Thorac Surg
61(6):1641-5, 1996
5- Cook CH, Melvin WS, Groner JI, Allen E, King DR: A
cost-effective thoracoscopic treatment strategy for pediatric spontaneous
pneumothorax. Surg Endosc 13(12):1208-10, 1999
6- Chan P, Clarke P, Daniel FJ, Knight SR, Seevanayagam
S: Efficacy study of video-assisted thoracoscopic surgery pleurodesis for
spontaneous pneumothorax. Ann Thorac Surg 71(2):452-4, 2001
Gastrointestinal Autonomic Nerve Tumor
Gastrointestinal Autonomic Nerve Tumor (GANT), also known as plexosarcoma,
is a very rare recently described aggressive malignant spindle cell tumor
that arises from the autonomic myenteric plexus of the bowel wall. Tumor
mostly originates in the small intestine and stomach with a clinical course
characterize by either local or distant metastases (liver). Histologically
GANT is a low-grade, epithelioid or spindle-cell neoplasms that can be
distinguished from the other gastrointestinal stromal tumors on the basis
of its unique electron microscopy ultrastructural features. Distinction
of GANT from other stromal tumors is not possible based on imaging studies.
The tumor is solid and cystic, hemorrhagic, often transmural and usually
involving omentum, mesentery and retroperitoneum. Signs and symptoms include
abdominal pain, mass, gastrointestinal bleeding, perforation and anemia.
Tumor can present with volvulus. In children they occur in females during
adolescent years (mean age 12 years) mostly involving the stomach. Primary
management consists of surgical resection which is curative, leaving chemotherapy
for residual or recurrent tumor. Prognosis is good for younger patients.
References:
1- Segal A, Carello S, Caterina P, Papadimitriou JM,
Spagnolo DV: Gastrointestinal autonomic nerve tumors: a clinicopathological,
immunohistochemical and ultrastructural study of 10 cases. Pathology
26(4):439-47, 1994
2- Kodet R, Snajdauf J, Smelhaus V: Gastrointestinal
autonomic nerve tumor: a case report with electron microscopic and immunohistochemical
analysis and review of the literature. Pediatr Pathol 14(6):1005-16,
1994
3- Rueda O, Escribano J, Vicente JM, Garcia F, Villeta
R: Gastrointestinal autonomic nerve tumors (plexosarcomas). is A radiological
diagnosis possible? Eur Radiol 8(3):458-60, 1998
4- Kerr JZ, Hicks MJ, Nuchtern JG, Saldivar V, Heim-Hall
J, Shah S, Kelly DR, Cain WS, Chintagumpala MM: Gastrointestinal autonomic
nerve tumors in the pediatric population: a report of four cases and a
review of the literature. Cancer 85(1):220-30, 1999
5- Lee JR, Joshi V, Griffin JW Jr, Lasota J, Miettinen
M: Gastrointestinal autonomic nerve tumor: immunohistochemical and molecular
identity with gastrointestinal stromal tumor. Am J Surg Pathol 25(8):979-87,
2001
Allgrove Syndrome
Achalasia, Alacrima & isolated Adrenal Insufficiency is also known
as triple-A or Allgrove syndrome. The high degree of consanguinity usually
identified supports an autosomal recessive mode of inheritance for this
disorder. Alacrima is the earliest and most consistent clinical sign of
Allgrove syndrome. All affected children shows esophageal dysmotility even
in the absence of symptomatic dysphagia. The adrenal insufficiency is due
to adrenocorticotropic hormone (ACTH) resistance. Other associated neurological
abnormalities include autonomic, sensory, and upper- and lower-motor neuropathy,
deafness, and mental retardation. Genetically the syndrome has been mapped
to mutations in chromosome 12q13 region near the type II keratin gene cluster.
Though most cases arise in children between the ages of two and eight years,
a few cases originate during early adult years. Management for alacrima
consists of artificial tears replacement. Adrenal insufficiency will need
steroid replacement. Achalasia is treated with a modified Heller esophagomyotomy
which can be done open or laparoscopically. A few neurologically
impaired children will need gastrotomy feedings.
References:
1- Moore PS, Couch RM, Perry YS, Shuckett EP, Winter
JS: Allgrove syndrome: an autosomal recessive syndrome of ACTH insensitivity,
achalasia and alacrima. Clin Endocrinol (Oxf) 34(2):107-14, 1991
2- Stratakis CA, Lin JP, Pras E, Rennert OM, Bourdony
CJ, Chan WY: Segregation of Allgrove (triple-A) syndrome in Puerto Rican
kindreds with chromosome 12 (12q13) polymorphic markers. Proc Assoc Am
Physicians 109(5):478-82, 1997
3- Huebner A, Yoon SJ, Ozkinay F, Hilscher C, Lee H,
Clark AJ, Handschug K: Triple A syndrome--clinical aspects and molecular
genetics. Endocr Res 26(4):751-9, 2000
4- Sandrini F, Farmakidis C, Kirschner LS, Wu SM, Tullio-Pelet
A, Lyonnet S, Metzger DL, Bourdony CJ, Tiosano D, Chan WY, Stratakis CA:
Spectrum of Mutations of the AAAS Gene in Allgrove Syndrome: Lack of Mutations
in Six Kindreds with Isolated Resistance to Corticotropin. J Clin Endocrinol
Metab 86(11):5433-7, 2001
Volume 18 No 02 FEBRUARY 2002
Pancreatic Cysts
Pancreatic cystic lesions are usually inflammatory pseudocyst (90%)
or neoplastic process (10%). Distinguishing between them is essential
for appropriate surgical therapy. Non-inflammatory neoplastic cysts in
children are very rare ductal lesions with a spectrum of histologic characteristics
and favorable outcomes. Histologically they include retention cysts, lymphoepithelial
cysts, papillary cystic tumors, benign serous cystadenoma, mucinous tumors
and mucinous cystadenocarcinoma. Most reported cases occur in females during
adolescent years. Mode of presentation includes mild upper abdominal pain
and palpable mass. Clinical, radiographic and intraoperative frozen section
are non-reliable methods in distinguishing the different types of pancreatic
cysts. Preoperative cyst fluid obtained by US or CT-guided percutaneous
aspiration can be analyzed for viscosity (mucoid, viscous, serous), chemical
(amylase, lipase), tumor markers (CEA, CA 19-9, CA125) and cytology characteristics.
High CEA levels (> 25 ng/ml) indicate that the cyst is either malignant
or mucinous (premalignant) type. Higher levels of CA 19-9 suggest pseudocysts
and serous cystadenomas. Very high CA 125 levels appear predictive of malignancy.
Viscosity above 1.63 suggests mucinous tumors. Amylase and lipase content
should be low in true pancreatic cysts. Cytology analysis is insensitive
unless positive for tumor cells. When the nature of the pancreatic cyst
cannot be definitively establish by the above methods surgical resection
is indicated.
References:
1- Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM,
Shandling B: A 20-Year Review of Pediatric Pancreatic Tumor. J Pediatr
Surg 27 (10): 1315-1317, 1992
2- Lewandrowski KB, Southern JF, Pins MR, Compton CC,
Warshaw AL: Cyst Fluid Analysis in the Differential Diagnosis of Pancreatic
Cysts. A Comparison of Pseudocysts, Serous Cystadenomas, Mucinous Cystic
Neoplasms, and Mucinous Cystadenocarcinoma. Ann Surg 217 (1): 41-47, 1993
3- Sand JA, Hyoty MK, Mattila J, Dragorn JC, Nordback
IH: Clinical assessment compared with cyst fluid analysis in the differential
diagnosis of cystic lesions in the pancreas. Surgery 119 (3): 275-287,
1996
4- Ky A, Shilyansky J, Gerstle J, Taylor G, Filler RM,
Grace N, Superina R: Experience with Papillary and Solid Epithelial Neoplasms
of the Pancreas in Children. J Pediatr Surg 33(1): 42-44, 1998
5- Borgne JL, Calan L, Partensky C, et al: Cystadenomas
and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective
Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999
Mucinous Cystadenoma
Mucinous cystadenoma (MCA) of the pancreas is a benign cystic tumor
made of columnar mucin-producing epithelium with a premalignant potential
rarely seen in children. Most cases are identified in teenage and young
adult female patients. Abdominal pain and mass effect is the most common
symptom when MCA develops. Mucinous cystadenomas grow to very large sizes,
is often multilocular in imaging studies and can be associated with recurrent
pancreatitis (due to cyst and duct communication). CT findings might include
large cysts with septa, peripheral calcifications and solid intracystic
components. Cysts development has an insidious onset not associated to
trauma, a high incidence of biliary tract disease or alcoholism. More than
90% of these slow-growing cysts are located in the body and tail of the
pancreas. Aspiration of MCA fluid shows low levels of pancreatic enzymes
with high CEA, CA 19-9 tumor markers and M1 mucin antigen levels. Cytology
evaluation can reveal mucin-containing cells. Due to premalignant potential
of developing a cystadenocarcinoma management of MCA should include complete
resection. Enucleation and median pancreatectomy carries a high rate of
pancreatic fistula formation. Resection is curative.
References:
1- Logan SE, Voet RL, Tompkins RT: The malignant potential
of mucinous cysts of the pancreas. West J Med 136: 157-162, 1982
2- Grosfeld JL: Chapter 12 - Pancreatic Tumors, In Medad
Schiller "Pediatric Surgery of the Liver, Pancreas and Spleen", WB Saunder
Co, 1991, pag. 165, 186
3- Hammel PR, Forgue-Lafitte ME, Levy P, Voitot H, Vilgrain
V, Flejou JF, Molas G, Gespach C, Ruszniewski P, Bernades P, Bara J: Detection
of gastric mucins (M1 antigens) in cyst fluid for the diagnosis of cystic
lesions of the pancreas. Int J Cancer 74(3):286-90, 1997
4- Ooi LL, Ho GH, Chew SP, Low CH, Soo KC: Cystic tumours
of the pancreas: a diagnostic dilemma. Aust N Z J Surg 68(12):844-6, 1998
5- Borgne JL, Calan L, Partensky C, et al: Cystadenomas
and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective
Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999
6- Sperti C, Pasquali C, Ferronato A, Pedrazzoli S: Median
pancreatectomy for tumors of the neck and body of the pancreas. J Am Coll
Surg. 190(6):715-6, 2000
7- Johnson PRV, Spitz L: Cysts and Tumors of the Pancreas.
Semin Pediatr Surg 9(4): 209-215, 2000
Serous Cystadenoma
Serous cystadenoma (SCA) is a benign microcystic tumor of the pancreas
composed of cuboidal epithelium very rarely seen in children. Predominantly
seen in young females, this cystic tumor is asymptomatic in one-third of
the cases. SCA has a microcystic appearance with cysts less than 2 cm in
size with loculations most commonly found in the pancreatic body and tail
as a single lesion. CT scan shows well-demarcated multilocular cysts with
enhancement that might show central calcifications. SCA is associated with
another extrapancreatic neoplasm in 20% of cases. Cytology and biochemical
analysis of cyst fluid reveal glycogen-rich cells and very low CEA levels
(less than five ng/ml). Oligocystic (large cyst) SCA is an even rarer variety
that arise in the head of the pancreas usually presenting in infants as
a palpable mass needing resection. Should the diagnosis of SCA be well-established
conservative management (observation) can be performed with yearly ultrasound
study safely. Otherwise, if the nature of the cystic lesion cannot be established,
or complications develop (obstructive jaundice, pancreatic duct dilatation
or portal hypertension) resection is the next step in management.
References:
1-Johnson PR, Spitz L: Cysts and Tumors of the Pancreas.
Semin Pediatr Surg 9(4): 209-215, 2000
2- Borgne JL, Calan L, Partensky C, et al: Cystadenomas
and Cystadenocarcinomas of the Pancreas. A Multiinstitutional retrospective
Study of 398 Cases. Ann Surg 230 (2): 152-161, 1999
3- Procacci C, Graziani R, Bicego E, Bergamo-Andreis
IA, Guarise A, Valdo M, Bogina G, Solarino U, Pistolesi GF: Serous cystadenoma
of the pancreas: report of 30 cases with emphasis on the imaging findings.
J Comput Assist Tomogr 21(3):373-82, 1997
4- Ooi LL, Ho GH, Chew SP, Low CH, Soo KC: Cystic tumours
of the pancreas: a diagnostic dilemma. Aust N Z J Surg 68(12):844-6, 1998
Volume 18 No 03 MARCH 2002
Pneumatocele
A pneumatocele is a benign air-containing cyst in the lung most commonly
the result of Staphylococcus Aureus pneumonia. Other less common bacteria
associated are Hemophilus Influenza, Pseudomonas Aeruginosa and Strep Pneumonia.
Pneumatoceles are most commonly seen in young children (almost 50% of cases
are less than one-year of age) during the acute phase of the pneumonic
process. In a low percentage of cases the pneumatocele can be the result
of closed chest trauma. In the infectious pneumonic setting the inflammatory
process causes necrosis and liquefaction of the lung parenchyma followed
by air leak and subpleural dissection forming a thin-walled cyst. Fever
and respiratory distress are the most common symptoms during initial presentation.
Diagnosis is established with the help of simple chest-x-ray films. CT-Scan
might be needed to differentiate between a congenital lung cyst or cystic
adenomatoid malformation. Follow-up films will help determine if the pneumatocele
is growing or not in size. Rapidly enlarging pneumatocele may need percutaneous
catheter decompression. Surgery is indicated only if the child develops
respiratory distress or the pneumatocele ruptures into the pleural space
creating a tension pneumothorax, a bronchopulmonary fistula or an empyema.
Fortunately most pneumatoceles gradually decrease in size and disappear
after the acute pulmonary infection subsides in a period that may range
between six weeks and six months.
References:
1- Joosten KF, Hazelzet JA, Tiddens HA, Hazebroek FW,
Dzoljic-Danilovic G, Neijens HJ, de Groot R: Staphylococcal pneumonia in
childhood: will early surgical intervention lower mortality? Pediatr Pulmonol
20(2):83-8, 1995
2- Wu MH, Tseng YL, Lin MY, Lai WW: Surgical treatment
of pediatric lung abscess. Pediatr Surg Int 12(4):293-5, 1997
3- Kogutt MS, Lutrell CA, Puyau FA, Tieman EK: Decompression
of pneumatocele in a neonate by percutaneous catheter placement. Pediatr
Radiol 29(6):488-9, 1999
4- Seo T, Ando H, Watanabe Y, Harada T, Ito F, Kaneko
K, Mimura S: Acute respiratory failure associated with intrathoracic masses
in neonates. J Pediatr Surg 34(11):1633-7, 1999
5- Schimpl G, Schneider U: Traumatic pneumatoceles in
an infant: case report and review of the literature. Eur J Pediatr Surg
6(2):104-6, 1996
Robotics
Computer-assisted surgery (robotics) has several advantages: the increase
in three-dimensional accuracy, the reproducibility of repeated procedures,
the increased precision of movements enhancing the skills of the surgeon
and the unique ability to perform surgery over a distance. Two systems
are actually in use today: the Da Vinci and Zeus systems. In image-guided
procedures, robots use magnetic resonance and computed tomography image
data to guide instruments to the treatment site. By increasing the image
capabilities the surgeon can integrate and define the pathology, margins
of resection, and vasculature during surgery. Experimental studies have
showed that robotically reproduced laparoscopic maneuvers such as tying,
suturing, dissection, clipping, and cautery, seemed to be as accurate and
fast as maneuvers made without robotics.Some of the clinical applications
of robotics surgery includes laparoscopic cholecystectomy and fundoplications,
entero-enterostomy, cardiac surgery, port-wine laser ablative surgery,
and urologic procedures to mention a few. Image-guided robots can
biopsy brain lesions with less damage to adjacent tissue and are routinely
used to shape the femur to precisely fit prosthetic hip joint replacements.
Robotic systems are also under development for coronary bypass, microsurgical
procedures in ophthalmology and surgical training and simulation. Issues
of physician acceptance, high costs, performance and safety remain to be
addressed.
References:
1- Marescaux J, Smith MK, Folscher D, Jamali F, Malassagne
B, Leroy J: Telerobotic laparoscopic cholecystectomy: initial clinical
experience with 25 patients. Ann Surg 234(1):1-7, 2001
2- Docimo SG, Moore RG, Adams J, Ben-Chaim J, Kavoussi
LR: Early experience with telerobotic surgery in children. J Telemed Telecare
2 Suppl 1:48-50, 1996
3- Sung GT, Gill IS: Robotic laparoscopic surgery: a
comparison of the DA Vinci and Zeus systems. Urology 58(6):893-8,
2001
4- Howe RD, Matsuoka Y: Robotics for surgery. Annu Rev
Biomed Eng 1:211-40, 1999
5- Lomanto D, Cheah WK, So JB, Goh PM: Robotically assisted
laparoscopic cholecystectomy: a pilot study. Arch Surg 136(10):1106-8,
2001
6- Chitwood WR Jr, Nifong LW, Chapman WH, Felger JE,
Bailey BM, Ballint T, Mendleson KG, Kim VB, Young JA, Albrecht RA: Robotic
surgical training in an academic institution. Ann Surg 234(4):475-84,
2001
7- Hollands CM, Dixey LN, Torma MJ: Technical assessment
of porcine enteroenterostomy performed with ZEUS robotic technology. J
Pediatr Surg 36(8):1231-3, 2001
Fibromyxoid Sarcoma
Fibromyxoid sarcoma (FS) is a distinctive indolent soft-tissue sarcoma
very uncommonly seen in children. Children demonstrate the low grade variety.
FS grows slowly and can metastasizes to distant organs, chiefly to the
lungs. The tumor has been located in the thigh or inguinal area, in the
shoulder, in the axilla-chest wall area, the perineum, the small bowel
mesentery, the neck, and the buttocks. Size can vary between 3.5 cm and
25 cm (median 4.5 cm). Histologically the tumor demonstrates contrasting
fibrous and myxoid areas, a swirling, whorled growth pattern and bland,
deceptively benign-appearing fibroblastic spindle cells; cellularity is
low to moderate, mitotic figures are uncommon, and nuclear pleomorphism
is usually absent or slight. The rate of local recurrence is very high.
Management should consist of aggressive wide local excision if possible
since this tumor has been found to be chemotherapy resistant. Adjuvant
radiotherapy is an alternative for residual or metastatic tumors.
References:
1- Evans HL: Low-grade fibromyxoid sarcoma. A report
of 12 cases. Am J Surg Pathol 17(6):595-600, 1993
2- Fukunaga M, Ushigome S, Fukunaga N: Low-grade fibromyxoid
sarcoma. Virchows Arch 429(4-5):301-3, 1996
3- Canpolat C, Evans HL, Corpron C, Andrassy RJ, Chan
K, Eifel P, Elidemir O, Raney B: Fibromyxoid sarcoma in a four-year-old
child: case report and review of the literature. Med Pediatr Oncol
27(6):561-4, 1996
4- Folpe AL, Lane KL, Paull G, Weiss SW: Low-grade fibromyxoid
sarcoma and hyalinizing spindle cell tumor with giant rosettes: a clinicopathologic
study of 73 cases supporting their identity and assessing the impact of
high-grade areas. Am J Surg Pathol 24(10):1353-60, 2000
Volume 18 No 04 APRIL 2002
Birth Fractures
Injury during birth is not an uncommon event. Birth fracture includes
clavicular, skull, nasal, ribs and long bones. Clavicular fractures (CFx)
are the commonest birth injury caused by excessive pressure on the shoulder
from the symphysis pubis. Cfx occurs in macrosomic infants, in those with
shoulder dystocia and is associated with injury to the brachial plexus.
In most cases this injury cannot be predicted prior to delivery. Diagnosis
is confirmed by x-ray. Management is expectantly with complete recovery
in most cases. Skull fractures result from difficult delivery and use of
vacuum extractor and can be either linear or depressed. Linear fractures
are observed, while depressed fractures might need surgery. Very rarely
the underlying brain is damage. Nasal birth trauma can cause dislocation
of the cartilages of the septum with deviation of the nose. Management
consists of realignment of the septum (Playfair Maneuver) to avoid long-term
nasal airway obstruction. Being obligate nasal breather if the injury reduces
the airway passages the baby may have feeding problems needing nasogastric
support. Ribs fractures occur very rarely after difficult forceps delivery
of large babies. Management is expectancy. Long bones fractures (humerus
and femur) occurs after difficult breach delivery, twin pregnancy and prematurity.
Babies born with spinal dysraphism can sustain femoral fractures during
birth. Bandage protection, traction, spica cast or splint support might
be needed depending on the fracture and angulation. Cases with birth injuries
have longer hospital stay and higher mortality.
References:
1- Perlow JH, Wigton T, Hart J, Strassner HT, Nageotte
MP, Wolk BM: Birth trauma. A five-year review of incidence and associated
perinatal factors. J Reprod Med 41(10):754-60, 1996
2- Many A, Brenner SH, Yaron Y, Lusky A, Peyser MR, Lessing
JB: Prospective study of incidence and predisposing factors for clavicular
fracture in the newborn. Acta Obstet Gynecol Scand 75(4):378-81, 1996
3- Walle T, Hartikainen-Sorri AL: Obstetric shoulder
injury. Associated risk factors, prediction and prognosis. Acta Obstet
Gynecol Scand 72(6):450-4, 1993
4- Hickey K, McKenna P: Skull fracture caused by vacuum
extraction. Obstet Gynecol 88(4 Pt 2):671-3, 1996
5- Nadas S, Reinberg O: Obstetric fractures. Eur J Pediatr
Surg 2(3):165-8, 1992
6- Hughes CA, Harley EH, Milmoe G, Bala R, Martorella
A: Birth trauma in the head and neck. Arch Otolaryngol Head Neck Surg 125(2):193-9,
1999
7- Korantzis A, Cardamakis E, Chelidonis E, Papamihalis
T: Nasal septum deformity in the newborn infant during labour. Eur J Obstet
Gynecol Reprod Biol 23;44(1):41-6, 1992
8- Salonen IS: Birth fractures of long bones. Ann Chir
Gynaecol 80(1):71-3, 1991
9- Bhat BV, Kumar A, Oumachigui A: Bone injuries during
delivery. Indian J Pediatr 61(4):401-5, 1994
10- Morris S, Cassidy N, Stephens M, McCormack D, McManus
F: Birth-associated femoral fractures: incidence and outcome. J Pediatr
Orthop 22(1):27-30, 2002
Spilled Gallstones
Gallbladder rupture with gallstone spillage occurs more frequently after
laparoscopic (5%) than open cholecystectomy. It is generally caused by
injury with an electric knife during dissection of the gallbladder bed,
injury during gallbladder retraction with grasping forceps, injury during
gallbladder extraction from the abdomen, and slippage of cystic duct clips
(potentially causing bile and stone spillage). Reports describe postoperative
instances of small bowel obstruction, intraabdominal, abdominal wall and
subcutaneous abscess developing. A concerted effort should be made to remove
all stones spilled in the peritoneal cavity. The necrotic, friable gallbladder
should be immediately placed in an endoscopic bag upon completion of the
dissection, copious lavage of the peritoneal cavity to evacuate spilled
bile should follow, and a therapeutic course of broad-spectrum antibiotic
should be instituted. Conversion to laparotomy is not justifiable. Patients
can presents weeks to months postoperatively with vague abdominal complaints,
fever and leukocytosis. Computed tomography reveals inflammatory foci involving
intraperitoneal gallstones. Management might require percutaneous or operative
drainage of the infectious collections. All affected patients should be
informed to hasten early diagnosis of later complications.
References:
1- Wilton PB, Andy OJ Jr, Peters JJ, Thomas CF, Patel
VS, Scott-Conner CE: Laparoscopic cholecystectomy. Leave no (spilled) stone
unturned. Surg Endosc 7(6):537-8, 1993
2- Leslie KA, Rankin RN, Duff JH: Lost gallstones during
laparoscopic cholecystectomy: are they really benign? Can J Surg
37(3):240-2, 1994
3- Ponce J, Cutshall KE, Hodge MJ, Browder W: The lost
laparoscopic stone. Potential for long-term complications. Arch Surg
130(6):666-8, 1995
4- Kimura T, Goto H, Takeuchi Y, Yoshida M, Kobayashi
T, Sakuramachi S, Harada Y: Intraabdominal contamination after gallbladder
perforation during laparoscopic cholecystectomy and its complications.
Surg Endosc 10(9):888-91, 1996
5- Schafer M, Suter C, Klaiber C, Wehrli H, Frei E, Krahenbuhl
L: Spilled gallstones after laparoscopic cholecystectomy. A relevant problem?
A retrospective analysis of 10,174 laparoscopic cholecystectomies. Surg
Endosc 12(4):305-9, 1998
6- Sarli L, Pietra N, Costi R, Grattarola M: Gallbladder
perforation during laparoscopic cholecystectomy. World J Surg 23(11):1186-90,
1999
7- Yao CC, Wong HH, Yang CC, Lin CS: Abdominal wall abscess
secondary to spilled gallstones: late complication of laparoscopic cholecystectomy
and preventive measures. J Laparoendosc Adv Surg Tech A 11(1):47-51,
2001
Pancreatoblastoma
Pancreatoblastoma (PB) is a juvenile adenocarcinoma of pancreas occurring
rarely in the pediatric age group. The tumor presents during early childhood
(mean age four years), affects mostly males and is associated with the
Beckwith-Wiedemann syndrome. PB is a large malignant tumor composed of
epithelial tissue with acinar differentiation. Most cases originate in
the head of the pancreas. Though being a slow growing tumor, local
invasion to duodenum, stomach and peritoneum occurs. Children presents
with palpable mass, pain and abdominal distension. Rarely do they develop
jaundice. AFP and LDH levels can be elevated. One-third of the cases develop
metastasis (liver and lung) US and CT-Scan confirms the presence of a solid
multilobulated tumor. Percutaneous needle biopsy is diagnostic. Management
consists of wide local excision which usually entails pancreaticoduodenectomy.
Preop chemotherapy has made unresectable tumor be resectable. Recurrent
or residual disease can be managed with radiotherapy a/o chemotherapy.
Prognosis is guarded.
References:
1- Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM,
Shandling B: A 20-year review of pediatric pancreatic tumors. J Pediatr
Surg 27(10):1315-7, 1992
2- Inomata Y, Nishizawa T, Takasan H, Hayakawa T, Tanaka
K: Pancreatoblastoma resected by delayed primary operation after effective
chemotherapy. J Pediatr Surg 27(12):1570-2, 1992
3- Klimstra DS, Wenig BM, Adair CF, Heffess CS: Pancreatoblastoma.
A clinicopathologic study and review of the literature. Am J Surg Pathol
19(12):1371-89, 1995
4- Willnow U, Willberg B, Schwamborn D, Korholz D, Gobel
U: Pancreatoblastoma in children. Case report and review of the literature.
Eur J Pediatr Surg 6(6):369-72, 1996
5- Chun Y, Kim W, Park K, Lee S, Jung S: Pancreatoblastoma.
J Pediatr Surg 32(11):1612-5, 1997
6- Ogawa B, Okinaga K, Obana K, Nakamura K, Hattori T,
Ito T, Yanagawa Y, Tanaka F, Imamura T: Pancreatoblastoma treated by delayed
operation after effective chemotherapy. J Pediatr Surg 35(11):1663-5,
2000
7- Johnson PRV, Spitz L: Cysts and Tumors of the Pancreas.
Seminars Pediatr Surg 9 (4): 209-215, 2000
Volume 18 No 05 MAY 2002
Clostridium Colitis
Clostridium difficile enterocolitis (CDE) is also known as pseudomembranous
colitis. CDE can be seen in children before, during or after surgical management
for Hirschsprung's disease though most cases are seen preoperatively. This
type of enterocolitis can be associated with fever, abdominal pain/distension,
leukocytosis and diarrhea (protein losing enteropathy) which can progress
to shock, prostration, toxic megacolon, perforation and even death. Clostridium
difficile secretes two toxins (A and B) that produce a variety of local
and systemic effects leading to depressed intestinal mucin production and
cell renewal rendering the bowel susceptible to bacterial invasion and
sepsis. Most cases of CDE have received prior antibiotic treatment for
approximately one week or more. Other risk factors for CDE are colon procedures,
acute functional bowel obstruction and critically-ill patients. Hirschsprung
patients are at increase risk of developing CDE due to intestinal stasis,
hypersensitivity to bacterial antigens, abnormality of prostaglandin secretion
and abnormal mucin production. In long segment Hirschsprung's disease the
aganglionic colon might also be involved. Diagnosis of CDE can be established
by culture of C. difficile or a fecal positive toxin assay. Endoscopic
evidence of pseudomembranous colitis can also suggest the diagnosis. Management
of CDE consists of oral (or stomal) vancomycin or metronidazole therapy
since it's more reliable. Refractory or chronic cases might need surgical
derivation or bowel resection. The morbidity associated with this nosocomial
infection is significant needing a high level of suspicion for early diagnosis.
References:
1-Bagwell CE, Langham MR Jr, Mahaffey SM, Talbert JL,
Shandling B: Pseudomembranous colitis following resection for Hirschsprung's
disease. J Pediatr Surg 27(10):1261-4, 1992
2-Urushihara N, Kohno S, Hasegawa S: Pseudomembranous
enterocolitis and hemorrhagic necrotizing enterocolitis in Hirschsprung's
disease. Surg Today 24(3):221-4, 1994
3- Kent KC, Rubin MS, Wroblewski L, Hanff PA, Silen W:
The impact of Clostridium difficile on a surgical service: a prospective
study of 374 patients. Ann Surg 227(2):296-301, 1998
4- Grundfest-Broniatowski S, Quader M, Alexander F, Walsh
RM, Lavery I, Milsom J: Clostridium difficile colitis in the critically
ill. Dis Colon Rectum 39(6):619-23, 1996
5- Bartlett JG: Clostridium difficile infection: pathophysiology
and diagnosis. Semin Gastrointest Dis 8(1):12-21, 1997
6- Teich S, Schisgall RM, Anderson KD: Ischemic enterocolitis
as a complication of Hirschsprung's disease. J Pediatr Surg 21(2):143-5,
1986
7- Fekety R, Shah AB: Diagnosis and treatment of Clostridium
difficile colitis. JAMA 6;269(1):71-5, 1993
8- Thomas DF, Fernie DS, Bayston R, Spitz L, Nixon HH:
Enterocolitis in Hirschsprung's disease: a controlled study of the etiologic
role of Clostridium difficile. J Pediatr Surg 21(1):22-5, 1986
9- Hardy SP, Bayston R, Spitz L: Prolonged carriage of
Clostridium difficile in Hirschsprung's disease. Arch Dis Child 69(2):221-4,
1993
10- Brearly S, Armstrong GR, Nairn R, Gornall P, Currie
AB, Buick RG, Corkery JJ: Pseudomembranous colitis: a lethal complication
of Hirschsprung's disease unrelated to antibiotic usage. J Pediatr Surg
22(3):257-9, 1987
Adnexal Torsion
Evaluation of females with acute onset lower quadrant pain should always
consider the possibility of ovarian and tube (adnexal) torsion. Ovarian
torsion is associated with a mass, cyst, tumors or simply a normal ovary
(30%). Cysts and tumors are the leading cause (70%) of torsion due to excessive
rotation and ischemia of the adnexa. The right adnexa is more frequently
involved than the left in torsion sometimes undistinguishable from acute
appendicitis. Nausea, vomiting and pain are more often seen with ovarian
torsion. Adnexal torsion is particular prone during premenarche years
(7-10 year old girls). Preoperative diagnosis is very difficult. Ultrasonography
using color Doppler signal can establish a high index of suspicion (echogenic
mass, edematous ovary, free intraperitoneal fluid, absent blood flow) of
torsion. Most cases present too late to be able to save the adnexa. At
operation (or laparoscopy) the viable ovary should be untwisted and the
necrotic ovary excised without untwisting the pedicle to avoid thromboembolism.
Others feel a more conservative approach toward a necrotic ovary can maintain
viable tissue with minimal morbidity (fever). Contralateral oophoropexy
at the time of oophorectomy is widely recommended, more so if the torsion
is associated with a normal idiopathic adnexa.
References:
1- Davis AJ, Feins NR: Subsequent asynchronous torsion
of normal adnexa in children. J Pediatr Surg 25(6):687-9, 1990
2- Shun A: Unilateral childhood ovarian loss: an indication
for contralateral oophoropexy? Aust N Z J Surg 60(10):791-4,
1990
3- Mordehai J, Mares AJ, Barki Y, Finaly R, Meizner I:
Torsion of uterine adnexa in neonates and children: a report of 20 cases.
J Pediatr Surg 26(10):1195-9, 1991
4- Kimura I, Togashi K, Kawakami S, Takakura K, Mori
T, Konishi J: Ovarian torsion: CT and MR imaging appearances. Radiology
190(2):337-41, 1994
5- Meyer JS, Harmon CM, Harty MP, Markowitz RI, Hubbard
AM, Bellah RD: Ovarian torsion: clinical and imaging presentation in children.
J Pediatr Surg 30(10):1433-6, 1995
6- Cohen Z, Shinhar D, Kopernik G, Mares AJ: The
laparoscopic approach to uterine adnexal torsion in childhood. J Pediatr
Surg 31(11):1557-9, 1996
7- Dolgin SE, Lublin M, Shlasko E: Maximizing ovarian
salvage when treating idiopathic adnexal torsion. J Pediatr Surg
35(4):624-6, 2000
8- Templeman C, Hertweck SP, Fallat ME: The clinical
course of unresected ovarian torsion. J Pediatr Surg 35(9):1385-7,
2000
Rare Branchial Remnant
Third and fourth branchial cleft remnants, known as complex branchial
remnants, are extremely rare neck anomalies in children. Most branchial
cleft anomalies (tags, cysts, fistulas or sinuses) come from faulty second
branchial pouch development (supratonsillar fossa). The third pouch develops
into the thymus and upper parathyroid glands, while the fourth pouch gives
rise to the lower parathyroids and thyroid gland. Third branchial remnants
are more common in the left side presenting as unilateral fistulas or sinus
tracts that communicates with the pharynx at the level of the thyrohyoid
membrane. They can harbor ectopic thyroid or thymus tissue. A recurrent
history of neck infection and drainage is commonly obtained. Fourth branchial
anomalies communicate with the thyroid gland or pyriform sinus and give
rise to thyroiditis. Surgical excision is the treatment of choice for all
branchial remnants in the neck after subsiding any episode of infection
with antibiotics. Recurrence is rarely seen after complete removal of the
lesion.
References:
1- Doi O, Hutson JM, Myers NA, McKelvie PA: Branchial
remnants: a review of 58 cases. J Pediatr Surg 23(9):789-92, 1988
2- Moreno Hurtado C, Blesa Sanchez E, Cabrera Garcia
R, Nunez Nunez R: [Third and fourth cervical pouches remnants and branchial
clefts]. Cir Pediatr 9(4):154-7, 1996
3- De Caluwe D, Hayes R, McDermott M, Corbally MT: Complex
branchial fistula: a variant arch anomaly. J Pediatr Surg 36(7):1087-8,
2001
4- Franciosi JP, Sell LL, Conley SF, Bolender DL: Pyriform
Sinus Malformations: A Cadaveric representation. J Pediatr Surg 37(3):
533-538, 2002
Volume 18 No 06 JUNE 2002
Meconium Plug Syndrome
One of the most significant sign of large bowel obstruction during the
neonatal period is failure to pass meconium during the first day of life.
The differential diagnosis to consider includes Hirschsprung's disease,
anorectal malformations, meconium plug syndrome, small left colon syndrome,
hypoganglionosis, neuronal intestinal dysplasia and megacystis-microcolon-intestinal
hypoperistalsis syndrome. Meconium plug syndrome (MPS) was first described
by Clatworthy in 1956 as a transient form of distal colonic or rectal obstruction
in newborns caused by an inspissated, immobile meconium. The plug is white
and chalky and rarely involves the small bowel. Clinical manifestations
include progressive abdominal distension, vomiting (sometimes is bilious)
and failure to pass meconium during the initial two days of life. Though
most cases are idiopathic, MPS has been associated with prematurity, hypotonia,
hypermagnesemia (reduces acetylcholine release with subsequent myoneural
depression), diabetic mother, Hirschsprung's disease and cystic fibrosis.
Colonic contrast study suggests the diagnosis (filling defect) and can
be therapeutic in most cases to relieve the obstruction. Gastrografin instillation
is highly effective in moving the obstructing long, thick plug, even in
tiny premature infants with MPS. Suction rectal biopsy to exclude the diagnosis
of Hirschsprung's disease along with cystic fibrosis screening is warranted
in all cases of MPS. The diagnosis of MPS is made after all the above causes
are excluded. Need for surgery is extremely rare.
References:
1- Loening-Baucke V, Kimura K: Failure to pass meconium:
diagnosing neonatal intestinal obstruction. Am Fam Physician 60(7):2043-50,
1999
2- Clatworthy HW, Howard WH, Lloyd J: The meconium plug
syndrome. Surgery 39:131-142, 1956
3- Hen J, Dolan TF, Touloukian RJ: Meconium Plug Syndrome
associated with Cystic Fibrosis and Hirschsprung's Disease. Pediatrics
66 (3): 466-468, 1980
4- Krasna IH, Rosenfeld D, Salerno P: Is it Necrotizing
Enterocolitis, Microcolon of Prematurity, or Delayed Meconium Plug? A Dilemma
in the Tiny Premature Infant. J Pediatr Surg 31(6): 855-858, 1996
5- Olsen MM, Luck SR, Lloyd-Still J, Raffensperger JG:
The spectrum of meconium disease in infancy. J Pediatr Surg 17(5):479-81,
1982
6- Rosenstein BJ: Cystic fibrosis presenting with the
meconium plug syndrome. Am J Dis Child 132(2):167-9, 1978
Granuloma Annulare
Granuloma annulare is a rare, benign, self-limiting, subcutaneous nodule
that can appear in the scalp and/or extremity of infants and children.
The nodule is painless, non-mobile, characterized by rapid growth usually
identified in the occipital region with absence of bone involvement. Mean
age at presentation is four years. Lesions are most commonly located about
the elbow, knee, and scalp. The erythrocyte sedimentation rate could
be elevated attesting to the inflammatory nature of the lesion. Otherwise,
no ancillary test is specific for this disorder. Excisional biopsy can
establish the diagnosis by showing lesions that resembles rheumatoid nodules,
consisting of acellular central areas surrounded by palisading histiocytes.
Between 20 and 50% of children have local recurrence or distant development
of new lesions. Most patients will not progress to any recognized systemic
illness or connective tissue disorder. The clinical course is characterized
by spontaneous regression. Parental reassurance is warranted.
References:
1- Challa VR, Weidner N, Bell WO, Prichard RW: Granuloma
annulare: a rare occipital lesion in infants and children. Surg Neurol
28(3):211-4, 1987
2- Davids JR, Kolman BH, Billman GF, Krous HF: Subcutaneous
granuloma annulare: recognition and treatment. J Pediatr Orthop 13(5):582-6,
1993
3- Felner EI, Steinberg JB, Weinberg AG: Subcutaneous
granuloma annulare: a review of 47 cases. Pediatrics 100(6):965-7,
1997
4- Trobs RB, Borte M, Voppmann A, Weidenbach H, Thiele
J: Granuloma annulare, nodular type--a subcutaneous pseudorheumatoid lesion
in children. Eur J Pediatr Surg 7(6):349-52, 1997
5- McDermott MB, Lind AC, Marley EF, Dehner LP: Deep
granuloma annulare (pseudorheumatoid nodule) in children: clinicopathologic
study of 35 cases. Pediatr Dev Pathol 1(4):300-8, 1998
6- Grogg KL, Nascimento AG: Subcutaneous granuloma
annulare in childhood: clinicopathologic features in 34 cases. Pediatrics
107(3):E42, 2001
Cervical Teratoma
Germ cell tumor (Teratoma) arising in the neck region of an infant
is an unusual lesion encompassing between three and 5% of all teratomas
found in children. These lesions are histologically benign, usually large
and can cause airway obstruction during birth. Cervical teratomas are true
neoplasm composed of foreign tissue to the anatomic site of origin with
all three germ layers represented. Neural tissue predominates. Fine calcifications
can be seen on simple films. Newborns might require intubation within the
first few hours after birth due to respiratory distress. The tumor is firm,
frequently mobile, multilobular, cystic and well encapsulated. Occasionally
the tumor is incorrectly diagnosed as cystic hygroma. Prenatally this tumor
can be associated with polyhydramnios, pulmonary insufficiency and fetal
demise. Prenatal diagnosis by ultrasound gives the clinician the opportunity
of maintaining the materno-fetal circulation until the airway is properly
secured during birth (EXIT procedure). Cesarean section is recommended
for all tumors larger than five cm in size. After birth and stabilization
management consists of prompt surgical excision. Total excision is essential
to avoid local recurrence and malignant degeneration. Postoperative monitoring
for recurrences should include Alpha-fetoprotein levels in difficult cases.
References:
1- Gundry SR, Wesley JR, Klein MD, Barr M, Coran AG:
Cervical teratomas in the newborn. J Pediatr Surg 18(4):382-6, 1983
2- Jordan RB, Gauderer MW: Cervical teratomas: an analysis.
Literature review and proposed classification. J Pediatr Surg 23(6):583-91,
1988
3- Langer JC, Tabb T, Thompson P, Paes BA, Caco CC: Management
of prenatally diagnosed tracheal obstruction: access to the airway in utero
prior to delivery. Fetal Diagn Ther 7(1):12-6, 1992
4- Azizkhan RG, Haase GM, Applebaum H, Dillon PW, Coran
AG, King PA, King DR, Hodge DS: Diagnosis, management, and outcome
of cervicofacial teratomas in neonates: a Childrens Cancer Group study.
J Pediatr Surg 30(2):312-6, 1995
5- Larsen ME, Larsen JW, Hamersley SL, McBride TP, Bahadori
RS: Successful management of fetal cervical teratoma using the EXIT procedure.
J Matern Fetal Med 8(6):295-7, 1999
6- Elmasalme F, Giacomantonio M, Clarke KD, Othman E,
Matbouli S: Congenital cervical teratoma in neonates. Case report and review.
Eur J Pediatr Surg 10(4):252-7, 2000