PEDIATRIC SURGERY UPDATE ©
VOLUME 30, 2008
PSU Volume 30 No 01 JANUARY 2008
Esophageal Atresia with Proximal TEF
Esophageal atresia with or without a tracheoesophageal fistula (TEF)
is considered the most common congenital anomaly of the esophagus. Most
cases are either esophageal atresia with distal TEF or pure esophageal
atresia without a fistula. A gasless abdomen with a coiled nasogastric
tube is sufficient evidence to diagnose pure esophageal atresia. Less than
1% of all cases of esophageal atresia have a concomitant proximal TEF.
The presence of such anomaly can be suspected with the proximal esophageal
stump is filled with air, or if a contrast proximal esophagogram is ordered
and the fistulous tract identified. Intraoperatively a proximal TEF can
be identified during bronchoscopy or more commonly while dissecting the
proximal esophageal stump to obtain length for the anastomosis. Esophageal
atresia is initially managed with a feeding gastrostomy to start gastric
feeding and obtain a study to determine the gap that exists between the
proximal and distal esophageal stumps. In the presence of a proximal TEF
causing chronic aspiration of saliva the need for early esophageal continuity
arises. The strategy can consist of ligating the TEF and doing an anastomosis
under tension, bringing the proximal esophageal stump through an extrathoracic
lengthening procedure or utilizing Foker technique of continuous proximal
and distal lengthening with later anastomosis. In either cases the rate
of ischemia, leak and stricture is high.
References:
1- Yun KL, Hartman GE, Shochat SJ: Esophageal atresia
with triple congenital tracheoesophageal fistulae. J Pediatr Surg. 27(12):1527-8,
1992
2- Lessin MS, Wesselhoeft CW, Luks FI, DeLuca FG: Primary
repair of long-gap esophageal atresia by mobilization of the distal esophagus.
Eur J Pediatr Surg. 9(6):369-72, 1999
3- Kimura K, Nishijima E, Tsugawa C, Collins DL, Lazar
EL, Stylianos S, Sandler A, Soper RT: Multistaged extrathoracic esophageal
elongation procedure for long gap esophageal atresia: Experience with 12
patients. J Pediatr Surg. 36(11):1725-7, 2001
4- Katsura S, Shono T, Yamanouchi T, Taguchi T, Suita
S: Esophageal atresia with double tracheoesophageal fistula--a case report
and review of the literature. Eur J Pediatr Surg. 15(5):354-7, 2005
5- Kane TD, Atri P, Potoka DA: Triple fistula: management
of a double tracheoesophageal fistula with a third H-type proximal fistula.
J Pediatr Surg. 42(6):E1-3, 2007
Dermal Sinus Tract
Dermal sinus tracts (DST) represent incomplete disjunction of the neural
tube from cutaneous ectoderm during neurulation leaving a sinus tract of
cutaneous tissue attached to the nervous system. A dermal sinus tract can
occur at the lumbosacral area (most common location) or less likely at
the cranial end of the neural tube, frontally (frontonasal sinus tracts)
or in the occiput (occipital sinus tracts). The child presents with a skin
dimple, a small dermal mass, a cutaneous hemangioma, skin tag or tuft of
hair in these areas. The lumbosacral sinus tract extends through or between
the laminas, penetrates the dura and ascend within the thecal sac to end
on the dorsal aspect of the spinal cord at the second sacral cord level.
A lumbosacral sinus tract can tethered the cord, serve as portal of entry
for bacteria leading to recurrent infections (subdural abscess), result
in an aseptic meningitis or a dermoid tumor can develop from the tract
and compress the spinal cord. Lumbosacral DST can be associated with focal
neurological deficit, neurogenic bladder or orthopedics deformities. The
diagnosis can be established with MRI in most cases. Management is surgical
excision. Simply removing the cutaneous component without addressing the
spinal cord malformation is not sufficient therapy. Poor awareness leads
to delayed management.
References:
1- Matson DD, Jerva MJ: Recurrent meningitis associated
with congenital lumbo-sacral dermal sinus tract. J Neurosurg. 25(3):288-97,
1966
2- Chen CY, Lin KL, Wang HS, Lui TN: Dermoid cyst with
dermal sinus tract complicated with spinal subdural abscess. Pediatr Neurol.
20(2):157-60, 1999
3- Gupta DK, Shastank RR, Mahapatra AK: An unusual presentation
of lumbosacral dermal sinus with CSF leak and meningitis. A case report
and review of the literature. Pediatr Neurosurg. 41(2):98-101, 2005
4- Ramnarayan R, Dominic A, Alapatt J, Buxton N: Congenital
spinal dermal sinuses: poor awareness leads to delayed treatment. Childs
Nerv Syst. 22(10):1220-4. Epub 2006 Mar 23, 2006
5- Holzmann D, Huisman TA, Holzmann P, Stoeckli SJ: Surgical
approaches for nasal dermal sinus cysts. Rhinology. 45(1):31-5, 2007
Molluscum Contagiosum
Molluscum contagiosum is a pox viral infection affecting primarily the
skin of infants, children and adults. It causes firm discrete pearly papules
that measure between one and four millimeters in diameter. The papules
have a characteristic central umbilication with a caseous type of material
containing virus-laden cells. One-third of children have symptoms from,
or secondary reactions to the infection, including pruritus, erythema and,
occasionally, inflammation and pain. Molluscum contagiosum can occur singly
or in clusters anywhere on the body, though the trunk is more commonly
affected. Spread is usual by direct contact, with genital involvement suggesting
the possibility of sexual abuse in the young child. The virus produces
a number of substances that block immune response formation in the infected
host. Molluscum contagiosum is a benign and self limited disease with most
cases resolving within six months to one year irrespective of therapy,
though patients with weakened immune systems have increased difficulty
in clearing lesions. A single, most effective treatment for either infection
has not been defined. Conventional methods attempt to nonspecifically destroy
infected tissue. Immunocompetent children can be managed with imiquimod,
retinoids, and alpha-hydroxy acids. Surgical management, if undertaken,
includes curettage of the central plug, cryosurgery and/or electrodesiccation.
References:
1- Allen AL, Siegfried EC: Management of warts and molluscum
in adolescents. Adolesc Med. 12(2):vi, 229-42, 2001
2- Silverberg N: Pediatric molluscum contagiosum: optimal
treatment strategies. Paediatr Drugs. 5(8):505-12, 2003
3- Tyring SK: Molluscum contagiosum: the importance of
early diagnosis and treatment. Am J Obstet Gynecol. 189(3 Suppl):S12-6,
2003
4- Laxmisha C, Thappa DM, Jaisankar TJ: Clinical profile
of molluscum contagiosum in children versus adults. Dermatol Online J.
9(5):1, 2003
5- Dohil MA, Lin P, Lee J, Lucky AW, Paller AS, Eichenfield
LF: The epidemiology of molluscum contagiosum in children. J Am Acad Dermatol.
54(1):47-54, 2006
6- Hanna D, Hatami A, Powell J, Marcoux D, Maari C, Savard
P, Thibeault H, McCuaig C: A prospective randomized trial comparing the
efficacy and adverse effects of four recognized treatments of molluscum
contagiosum in children. Pediatr Dermatol. 23(6):574-9, 2006
7- Martín-García RF, García ME,
Rosado A: Modified curettage technique for molluscum contagiosum. Pediatr
Dermatol. 24(2):192-4, 2007
PSU Volume 30 No 02 FEBRUARY 2008
Mesh Repair Congenital Diaphragmatic Hernia
Congenital diaphragmatic hernia continues to carry a high mortality
associated with the presence of pulmonary hypoplasia. Repair of the diaphragmatic
defect is usually carried on when the child obtains sufficient hemodynamic
stability to tolerate a major surgical procedure. Surgical repair or closure
of the defect is either carried out primarily or in case that most of the
hemidiaphragm is lacking, using a piece of prosthetic mesh. Primary repair
is performed when there is sufficient diaphragm to approximate with low
tension, carries a low rate of recurrence and avoids the mechanical and
infectious complications associated with implanted prostheses. When the
size of the defect can be known preoperatively a split abdominal wall muscle
flap through a low abdominal incision can be planned. Mesh repair utilizes
several prosthetic materials such as polytetrafluoroethylene (Gore-Tex),
polypropylene (Marlex), lyophilized dura, Surgisis or even small intestinal
submucosa. Composite patch repair using Gore-Tex/Marlex has also been reported.
Overall, recurrence rates after mesh repair is significant greater (almost
50%) than after primary repair. Polypropylene mesh in contact with small
bowel carries a high risk of fistula formation reason why Gore-Tex is preferred.
Vycryl mesh is not a suitable material for repairing these defects.
References:
1- Saltzman DA, Ennis JS, Mehall JR, Jackson RJ, Smith
SD, Wagner CW: Recurrent congenital diaphragmatic hernia: A novel repair.
J Pediatr Surg. 36(12):1768-9, 2001
2- Sydorak RM, Hoffman W, Lee H, Yingling CD, Longaker
M, Chang J, Smith B, Harrison MR, Albanese CT: Reversed latissimus dorsi
muscle flap for repair of recurrent congenital diaphragmatic hernia. J
Pediatr Surg. 38(3):296-300, 2003
3- Scaife ER, Johnson DG, Meyers RL, Johnson SM, Matlak
ME: The split abdominal wall muscle flap--a simple, mesh-free approach
to repair large diaphragmatic hernia. J Pediatr Surg. 38(12):1748-51, 2003
4- Smith MJ, Paran TS, Quinn F, Corbally MT: The SIS
extracellular matrix scaffold-preliminary results of use in congenital
diaphragmatic hernia (CDH) repair. Pediatr Surg Int. 20(11-12):859-62,
2004
5- Grethel EJ, Cortes RA, Wagner AJ, Clifton MS, Lee
H, Farmer DL, Harrison MR, Keller RL, Nobuhara KK: Prosthetic patches for
congenital diaphragmatic hernia repair: Surgisis vs Gore-Tex. J Pediatr
Surg. 41(1):29-33, 2006
6- Riehle KJ, Magnuson DK, Waldhausen JHT: Low recurrence
rate after Gore-Tex/Marlex composite patch repair for posterolateral congenital
diaphragmatic hernia. J Pediatr Surg 42(11): 1841-1844, 2007
Solitary Intestinal Fibromatosis
Congenital solitary intestinal fibromatosis (SIF) is a very rare tumor
that occurs in the newborn and infant period. It belongs to the group of
pediatric fibromatosis. Solitary Intestinal fibromatosis involves the intestinal
wall, can produce bowel obstruction or perforation and usually involves
most frequently the jejunum and ileum. SIF presents most commonly as a
solitary or less commonly as multiple lesions usually confined to
soft tissue and bone. Lesions in the duodenum can cause gastric outlet
obstruction indistinguishable from pyloric stenosis. Most cases consist
of solitary tumors affecting the small or large bowel. Histologic examination
in each case shows a transmural infiltrative spindle cell lesion having
the morphologic features of fibromatosis. The spindle cells have no atypia,
stain positively for Vimentin and CD34 while negative for muscle cell markers.
Ultrastructural studies reveals the tumor to be composed of myofibroblasts.
The tumor most often presents as a polypoid mass protruding into the intestinal
lumen causing obstruction. Symptoms are usually bilious vomiting, abdominal
distension, malabsorption and obstipation. Management consists of wide
local resection of the tumor along with the segment of affected bowel.
Some cases have demonstrated spontaneous regression. Solitary lesions carry
an excellent prognosis after resection, while multiple lesions carry a
worse prognosis.
References:
1- Canioni D, Fekete C, Nezelof C: Solitary intestinal
fibromatosis: a rare cause of neonatal obstruction. Pediatr Pathol. 9(6):719-24,
1989
2- Srigley JR, Mancer K: Solitary intestinal fibromatosis
with perinatal bowel obstruction. Pediatr Pathol. 2(3):249-58, 1984
3- Türken A, Senocak ME, Kotiloglu E, Kale G, Hiçsönmez
A: Solitary intestinal fibromatosis mimicking malabsorption syndromes.
J Pediatr Surg. 30(9):1387-9, 1995
3- Arets HG, Blanco C, Thunnissen FB, Heineman E: Solitary
intestinal fibromatosis as a cause of bile vomiting in a neonate. J Pediatr
Surg. 35(4):643-5, 2000
4- Numanoglu A, Davies J, Millar AJ, Rode H: Congenital
solitary intestinal fibromatosis. Eur J Pediatr Surg. 12(5):337-40, 2002
5- Coulon A, McHeik J, Milin S, Levard G, Levillain P,
Fromont G: Solitary intestinal fibromatosis associated with congenital
ileal atresia. J Pediatr Surg. 42(11):1942-5, 2007
Natural Orifice Transluminal Endoscopic Surgery
Natural orifice transluminal endoscopic surgery (NOTES) is the next
frontier in minimal invasive surgical procedures. NOTES takes advantage
of doing a laparoscopic procedure through natural orifices of our body
such as mouth (trans-gastric), anus (trans-colonic) or vagina totally eliminating
a scar in the abdomen. Theoretically, this approach could reduce postoperative
abdominal wall pain, wound infection, hernia formation, and adhesions.
So far studies in animal models have demonstrated the feasibility of performing
such procedure to remove the gallbladder through either per-oral transgastric
or per-anal transcolonic by perforating such viscera and introducing a
multichannel locking endoscope to introduce the laparoscopic instruments
utilized during actual transperitoneal procedures. The incision done in
the perforated viscera is subsequently closed with endoscopic clips, endoloops,
or a prototype closure device. In contrast to the transgastric method,
a transcolonic approach provides more consistent identification of structures
in the upper abdomen and provides better en face orientation and scope
stability. There is no bleeding or laceration of adjacent organs. Animal
models have been used to demonstrate the possible applications of NOTES,
including transgastric peritoneoscopy, tubal ligation, gastrojejunostomy,
partial hysterectomy, oophorectomy, nephrectomy and transcolonic exploration,
liver biopsy, distal pancreatectomy and cholecystectomy. The first human
report in 2007 was a successful trans-vaginal cholecystectomy.
References:
1- Ko CW, Kalloo AN: Per-oral transgastric abdominal
surgery. Chin J Dig Dis. 2006;7(2):67-70
2- Pai RD, Fong DG, Bundga ME, Odze RD, Rattner DW, Thompson
CC: Transcolonic endoscopic cholecystectomy: a NOTES survival study in
a porcine model (with video). Gastrointest Endosc. 64(3):428-34, 2006
3- Fong DG, Pai RD, Thompson CC: Transcolonic endoscopic
abdominal exploration: a NOTES survival study in a porcine model. Gastrointest
Endosc. 65(2):312-8, 2007
4- Wagh MS, Thompson CC: Surgery insight: natural orifice
transluminal endoscopic surgery--an analysis of work to date. Nat
Clin Pract Gastroenterol Hepatol. 4(7):386-92, 2007
5- Marescaux J, Dallemagne B, Perretta S, Wattiez A,
Mutter D, Coumaros D: Surgery without scars: report of transluminal cholecystectomy
in a human being. Arch Surg. 142(9):823-6, 2007
6- Fritscher-Ravens A, Ghanbari A, Thompson S, Patel
K, Kahle E, Fritscher T, Niemann H, Koehler P, Milla P: Which parameters
might predict complications after natural orifice endoluminal
surgery (NOTES)? Results from a randomized comparison
with open surgical access in pigs. Endoscopy. 39(10):888-92, 2007
PSU Volume 30 No 03 MARCH 2008
Cutaneous Mucinosis
Frequently pediatric surgeons are asked to evaluate a skin disorder
in children. Most cases are evaluated and managed by dermatologists, but
in a few patients due to the nature of the lesion or age an excisional
ir incisional biopsy is required to establish the histologic diagnosis.
Such is the case of juvenile cutaneous mucinosis, a very rare disorder
of skin found in children. Characteristically cutaneous mucinosis is a
nontender hypopigmented plaque-like raised papula which can appear in the
trunk or extremity singly or with several other associated lesions. Cutaneous
mucinoses are a heterogeneous group of diseases in which mucin accumulates
in the skin or within the hair follicle. Mucin is a gelatinous substance
composed of glycosaminoglycans, especially hyaluronic acid and dermatan
sulfate bound to small quantities of chondroitin sulfate and heparin sulfate.
Two groups are identified: the primary cutaneous mucinoses in which the
mucin deposit is a distinctive histopathologic feature that manifests as
a clinically specific lesion, and the diseases associated with histopathologic
mucin deposition as an additional finding (secondary) such as myxedema,
lupus erythematosus or scleroderma. The primary juvenile variety occurs
in children characterized by an early age of onset, the presence
of plaques and nodules in a characteristic distribution, and rapid onset
followed by spontaneous resolution of the lesions within a period of weeks
to months.
References:
1- Rongioletti F, Rebora A: Cutaneous mucinosis. Ann
Dermatol Venereol. 120(1):75-87, 1993
2- Wadee S, Roode H, Schulz EJ: Self-healing juvenile
cutaneous mucinosis in a patient with nephroblastoma. Clin Exp Dermatol.
19(1):90-3, 1994
3- Caputo R, Grimalt R, Gelmetti C: Self-healing juvenile
cutaneous mucinosis. Arch Dermatol. 131(4):459-61, 1995
4- Aydingöz IE, Candan I, Dervent B: Self-healing
juvenile cutaneous mucinosis. Dermatology. 199(1):57-9, 1999
5- Carder KR, Fitzpatrick JE, Weston WL, Morelli JG:
Self-healing juvenile cutaneous mucinosis. Pediatr Dermatol. 20(1):35-9,
2003
6- Nagaraj LV, Fangman W, White WL, Woosley JT, Prose
N, Selim MA, Morrell DS: Self-healing juvenile cutaneous mucinosis: cases
highlighting subcutaneous/fascial involvement. J Am Acad Dermatol. 55(6):1036-43,
2006
Werdnig-Hoffmann Disease
Spinal muscular atrophy is a common autosomal recessive neuromuscular
disorder characterized by degeneration of motor neurons of the spinal cord.
Werdnig-Hoffman disease (WHD), the second most common neuromuscular disease
in childhood, is a type of spinal muscular atrophy. Werdnig-Hoffmann is
subdivided into two groups on the basis of a combination of age of onset,
milestones of development and age of survival. Type I has an acute onset
before six months of life and the progression of disease is severe with
a more uniform poor prognosis and early death. They showed generalized
hypotonia, abnormal respiration and could not sit without support. Type
II onset of the disease is between the age of six and 18 months and
progression is slower. A gene termed 'survival
of motor neuron' (SMN) has been recognized as the disease-causing
gene. SMN encodes a protein located within a novel nuclear structure and
interacts with RNA-binding proteins. Pre- or postnatal diagnosis is made
with this genetic testing. There is no effective therapy for WHD.Management
consists of preventing or treating the complications of severe weakness,
such as restrictive lung disease, poor nutrition, orthopedic deformities,
immobility, and psychosocial problems. Tracheostomy, gastrostomy and fundoplication
with non-invasive mechanical ventilation can help prolong life in WHD.
References:
1- Russman BS, Iannacone ST, Buncher CR, Samaha FJ, White
M, Perkins B, Zimmerman L, Smith C, Burhans K, Barker L: Spinal muscular
atrophy: new thoughts on the pathogenesis and classification schema. J
Child Neurol. 7(4):347-53, 1992
2- Lefebvre S, Burlet P, Liu Q, Bertrandy S, Clermont
O, Munnich A, Dreyfuss G, Melki J: Correlation between severity and SMN
protein level in spinal muscular atrophy. Nat Genet. 16(3):265-9, 1997
3- Iannaccone ST: Spinal muscular atrophy. Semin Neurol.
18(1):19-26, 1998
4- Stipoljev F, Sertic J, Latin V, Rukavina-Stavljenic
A, Kurjak A: Prenatal diagnosis of spinal muscular atrophy type I (Werdnig-
hoffmann) by DNA deletion analysis of cultivated amniocytes. Croat Med
J. 40(3):433-7, 1999
5- Bach JR, Saltstein K, Sinquee D, Weaver B, Komaroff
E: Long-term survival in Werdnig-Hoffmann disease. Am J Phys Med Rehabil.
86(5):339-45, 2007
6- Bach JR: Medical considerations of long-term survival
of Werdnig-Hoffmann disease. Am J Phys Med Rehabil. 86(5):349-55, 2007
Treadmill Injury
Injury is the leading cause of preventable death and disability in childhood
and early adulthood. Most of us have a treadmill, or jogging machine in
our house to stay fit. Treadmills have been found to account for an increase
in the number of injuries in our children. Approximately 25,000 children
with a median age of 2.5 years are injured on exercise equipment each year.
Jogging machines can cause friction burns, abrasions, blunt trauma, or
even amputation. Most of these injuries will require surgical intervention
and rehabilitation. Almost all injuries are friction injury due to contact
with the moving treadmill belts. Friction injuries that convert into a
full thickness burn needing skin grafting surgery. The upper extremity
is more commonly affected than the lower extremity with almost 75% of cases
involving the palmar aspect of the hand. Most common mechanism is when
the machine is in use by an adult and a curious toddler comes toward the
running treadmill. Other cases are the older child who has the height to
reach and activate the machine sustaining injury when they fall. Prevention
modalities include additional manufacture safety features, warning labels,
and parental education. Parent supervision during use of this type of machine
is paramount. Recommendations include limiting children access, facing
the treadmill toward the open room, use a back mirror, and avoiding the
use of headsets while on the treadmill.
References:
1- Attalla MF, al-Baker AA, al-Ekiabi SA: Friction burns
of the hand caused by jogging machines: a potential hazard to children.
Burns. 17(2):170-1, 1991
2- Carman C, Chang B: Treadmill injuries to the upper
extremity in pediatric patients. Ann Plast Surg. 47(1):15-9, 2001
3- Borschel GH, Wolter KG, Cederna PS, Franklin GA: Acute
management of exercise treadmill-associated injuries in children. J Trauma.
55(1):130-4, 2003
4- Maguiña P, Palmieri TL, Greenhalgh DG: Treadmills:
a preventable source of pediatric friction burn injuries. J Burn Care Rehabil.
25(2):201-4, 2004
5- Han T, Han K, Kim J, Lee G, Choi J, Lee J, Jang Y,
Oh S: Pediatric hand injury induced by treadmill. Burns. 31(7):906-9, 2005
6- Wong A, Maze D, La Hei E, Jefferson N, Nicklin S,
Adams S: Pediatric treadmill injuries: a public health issue. J Pediatr
Surg. 42(12):2086-9, 2007
PSU Volume 30 No 04 APRIL 2008
Esophageal Foreign Bodies
The superior esophagus is the narrowest portion of the alimentary tract
of children and the most common site for lodge foreign bodies. Due to the
nature of infants and toddlers to place objects in their mouth, especially
a coin, this represent the most common foreign body identified within the
proximal esophagus. The child will develop cough, stridor, choking, drooling,
pain and inability to swallow with a lodge esophageal foreign body. Complications
secondary to the esophageal foreign body itself include erosion/perforation,
stricture, migration, mediastinitis and airway complications. Since aspiration
and perforation are immediate complications, the impacted foreign body
mandates urgent surgical attention. A simple chest film will delineate
the position of the lodge coin. With other type of non-opaque foreign bodies
an esophagogram will be needed to help visualized the position and type
of obstruction. Rigid esophagoscopy under general anesthesia or flexible
esophagoscopy under sedation is the procedure of choice to remove the foreign
body, though Foley balloon extraction under fluoroscopic control is an
acceptable method of coin extraction with minimal morbidity. Other times
the foreign body can be pushed toward the stomach using esophageal bougienage.
Children younger than one years, those with a widened tracheoesophageal
interface, not a smooth object or more than one week after ingestion seems
to be at highest risk for esophageal edema, failure of balloon extraction
and complications.
References:
1- Ozguner IF, Buyukyavuz BI, Savas C, Yavuz MS, Okutan
H: Clinical experience of removing aerodigestive tract foreign bodies with
rigid endoscopy in children. Pediatr Emerg Care. 20(10):671-3, 2004
2- Waltzman ML, Baskin M, Wypij D, Mooney D, Jones D,
Fleisher G: A randomized clinical trial of the management of esophageal
coins in children. Pediatrics. 116(3):614-9, 2005
3- Little DC, Shah SR, St Peter SD, Calkins CM, Morrow
SE, Murphy JP, Sharp RJ, Andrews WS, Holcomb GW 3rd, Ostlie DJ, Snyder
CL: Esophageal foreign bodies in the pediatric population: our first 500
cases. J Pediatr Surg. 41(5):914-8, 2006
4- Waltzman ML: Management of esophageal coins. Curr
Opin Pediatr. 18(5):571-4, 2006
5- Tokar B, Cevik AA, Ilhan H: Ingested gastrointestinal
foreign bodies: predisposing factors for complications in children having
surgical or endoscopic removal. Pediatr Surg Int. 23(2):135-9, 2007
6- Weissberg D, Refaely Y: Foreign bodies in the esophagus.
Ann Thorac Surg. 84(6):1854-7, 2007
Lipomas
Lipomas are benign tumors accounting for 6% of soft-tissue tumors found
in the pediatric age. They are ubiquitous and can occur anywhere in the
body with a predilection for the trunk. Lipomas develop as painless, gradually
enlarging swellings, soft to palpation and with demarcated borders. These
neoplasms are slow growing and may reach great proportions without producing
significant symptoms depending on location. Mediastinal lipomas produce
respiratory distress, while spinal cord lipomas are associated with significant
neurological symptoms. Ultrasound will uncover the homogenous nature of
the tumor, while CT or MRI will demonstrate the relation of the tumor with
surrounding structures. Infiltration of surrounding structure rather than
displacement suggests a malignant variant known as liposarcoma. Definite
diagnosis can only be obtained by pathologic examination which must differentiate
between lipoma, lipoblastoma, liposarcoma or myxoma. Needle aspiration
biopsy can provide the diagnosis. Management consists of surgical resection
to establish the diagnosis, alleviate symptoms if present and avoid local
recurrence. Endoscopic excision or liposuction of large capsulated lipomas
can be appropriate treatment and effective from a cosmetic point of view.
References:
1- Sakai Y, Okazaki M, Kobayashi S, Ohmori K: Endoscopic
excision of large capsulated lipomas. Br J Plast Surg. 49(4):228-32, 1996
2- Mahomed AA, Beale P, Puri P: Mediastinal lipoma in
children. Pediatr Surg Int. 13(2-3):218-9, 1998
3- de Jong AL, Park A, Taylor G, Forte V: Lipomas of
the head and neck in children. Int J Pediatr Otorhinolaryngol. 43(1):53-60,
1998
4- Ilhan H, Tokar B: Liposuction of a pediatric giant
superficial lipoma. J Pediatr Surg. 37(5):796-8, 2002
5- Inampudi P, Jacobson JA, Fessell DP, Carlos RC, Patel
SV, Delaney-Sathy LO, van Holsbeeck MT: Soft-tissue lipomas: accuracy of
sonography in diagnosis with pathologic correlation. Radiology. 233(3):763-7,
2004
6- Grandbois L, Vade A, Lim-Dunham J, Al-Masri H: MRI
findings of an intermuscular lipoma in a 2-year-old. Pediatr Radiol. 36(9):974-6,
2006
Anal Canal Duplication
Anal canal duplication is a very rare congenital malformation: the most
distal and least common duplication of the digestive tube. It can be confused
with other types of anorectal pathology including hemorrhoids, fistula-in-ano,
and perirectal abscess. Anal canal duplications are usually located posterior
presenting as a one to 2 mm perineal opening just behind the anus in the
midline. The tract runs along the posterior aspect of the anal canal without
communication with the anorectum. Most cases are females. Simple
perineal inspection makes the diagnosis. Older children will present with
localized infection or pruritus. Most children are asymptomatic. Fistulography
reveals a tubular structure or a cystic structure behind the normal anal
canal whose length varies from 10 to 30 mm. Associated malformations include
sacrococcygeal teratomas, dermoid cysts, sacral dysgenesis, hindgut anomalies
and lumbosacral myelomeningocele. Non-invasive preoperative investigations
consisting of pelvic X-ray, US examination, barium enema and fistulography,
are sufficient in most cases; MRI is reserved to evaluate the presence
of associated anomalies. Surgical treatment through a posterior sagittal
approach or simple mucosectomy restores a normal perineal aspect without
sequelae and avoids future complications like those described in other
types of digestive duplications namely infection, ulceration, bleeding,
and malignant changes during later adult life. Histology shows squamous
epithelium on the surface of the fistula and columnar epithelium and goblet
cells in the base, which confirms the diagnosis of an anal-canal duplication.
Prognosis is good after surgery.
References:
1- Jacquier C, Dobremez E, Piolat C, Dyon JF, Nugues
F: Anal canal duplication in infants and children--a series of 6 cases.
Eur J Pediatr Surg. 11(3):186-91, 2001
2- Ochiai K, Umeda T, Murahashi O, Sugitoh T: Anal-canal
duplication in a 6-year-old child. Pediatr Surg Int. 18(2-3):195-7, 2002
3- Choi SO, Park WH: Anal canal duplication in infants.
J Pediatr Surg. 38(5):758-62, 2003
4- Tiryaki T, Senel E, Atayurt H: Anal canal duplication
in children: a new technique. Pediatr Surg Int. 22(6):560-1, 2006
5- Lisi G, Illiceto MT, Rossi C, Broto JM, Jil-Vernet
JM, Lelli Chiesa P: Anal canal duplication: a retrospective analysis of
12 cases from two European pediatric surgical departments. Pediatr Surg
Int. 22(12):967-73, 2006
PSU Volume 30 No 05 MAY 2008
Enterocutaneous Fistulas
Enterocutaneous fistulas (ECF) are serious complications associated
with high morbidity and mortality. Most ECF occurs after surgery or trauma,
while other times Crohn's disease, necrotizing enterocolitis, intra-abdominal
abscess, malignant disease and radiotherapy are the culprits. ECF can be
classified as low output (less than five ml/kg/day), or high output (greater
than five ml/kg/day). Postoperative ECF results from infection and breakdown
of an anastomosis, bowel injury, deserosalization of bowel, suture-lines
defects, tight sutures with ischemic necrosis, injury to mesenteric vessels,
poor hemostasis, adhesive ischemia, volvulus and bowel loop caught in a
fascial suture. Postoperative ECF can be also classified as early (those
that occur within 48 hours after surgery and are associated to a technical
error), and late (occurring 48 hours after the procedure) and associated
with low ischemia time. It is vital to identify the source and route of
the ECF tract by imaging techniques (UGIS, Barium enema, CT Scan or MRI)
and whether the patient has distal obstruction. Management consists of
reducing the septic state by adequate draining, hydration, correction of
electrolyte imbalances, parenteral antibiotics, somatostatin-14 trial,
bowel rest, parenteral nutrition, cutaneous protection, and surgical correction
using resection with anastomosis or bypass procedures if the ECF fails
to respond to conservative measures.
References:
1- Fekete CN, Ricour C, Duhamel JF, Lecoultre C, Pellerin
D: Enterocutaneous fistulas of the small bowel in children (25 cases).
J Pediatr Surg. 13(1):1-4, 1978
2- Lévy E, Frileux P, Cugnenc PH, Honiger J, Ollivier
JM, Parc R: High-output external fistulae of the small bowel: management
with continuous enteral nutrition. Br J Surg. 76(7):676-9, 1989
3- Falconi M, Pederzoli P: The relevance of gastrointestinal
fistulas in clinical practice: a review. Gut 49: iv2-iv10, 2002
4- Gonzalez-Pinto I, Moreno Gonzalez E: Optimizing the
treatment of upper gastrointestinal fistulas. Gut 49: iv21-iv28, 2002
5- Jamil M, Ahmed U, Sobia H: Role of somatostatin analogues
in the management of enterocutaneous fistulae. J Coll Physicians Surg Pak.
14(4):237-40, 2004
6- Ahmad RR, Fawzy SY: Enterocutaneous fistula. Causes
and management. Saudi Med J. 28(9):1408-13, 2007
Pneumothorax
Pneumothorax is the presence of air in the pleural cavity. Results from
either a tear in the visceral or parietal pleura. Pneumothorax can be spontaneous
(primary or secondary), or acquire. The most common cause of primary spontaneous
pneumothorax is rupture of an apical subpleural bleb of the lung, usually
a thin adolescent male who suddenly develops chest pain and shortness of
breath. Secondary spontaneous pneumothorax occurs after hyaline membrane
disease, meconium aspiration, cystic fibrosis, or AIDS. Acquired pneumothorax
is more common than spontaneous usually the result of blunt or penetrating
trauma, iatrogenic after central line placement, thoracentesis, lung biopsy,
barotrauma from mechanical ventilation and laparoscopic procedures. Diagnosis
of pneumothorax is done with simple chest films. Complex cystic lung conditions
will need chest CT scan for diagnosis. The purpose of management is to
evacuate the air in the pleura and expand adequately the lung. Small pneumothorax
(less than 20%) can be managed with observation and oxygen therapy. Tube
thoracostomy is recommended for pneumothorax larger than 20%. The tube
is removed when the lung has expanded completely and the air leak is no
longer present for at least 24 hours. Surgical treatment is indicated using
video assisted thoracic surgery (VATS) when air leaks continues for more
than 72 hours, there is incomplete lung expansion or pneumothorax recurs
after adequate management.
References:
1- Liu HP, Yim AP, Izzat MB, Lin PJ, Chang CH: Thoracoscopic
surgery for spontaneous pneumothorax. World J Surg. 23(11):1133-6, 1999
2- Shaw KS, Prasil P, Nguyen LT, Laberge JM: Pediatric
spontaneous pneumothorax. Semin Pediatr Surg. 12(1):55-61, 2003
3- Ozcan C, McGahren ED, Rodgers BM: Thoracoscopic treatment
of spontaneous pneumothorax in children. J Pediatr Surg. 38(10):1459-64,
2003
4- Choudhary AK, Sellars ME, Wallis C, Cohen G, McHugh
K: Primary spontaneous pneumothorax in children: the role of CT in guiding
management. Clin Radiol. 60(4):508-11, 2005
5- Qureshi FG, Sandulache VC, Richardson W, Ergun O,
Ford HR, Hackam DJ: Primary vs delayed surgery for spontaneous pneumothorax
in children: which is better? J Pediatr Surg. 40(1):166-9, 2005
6- Butterworth SA, Blair GK, LeBlanc JG, Skarsgard ED:
An open and shut case for early VATS treatment of primary spontaneous pneumothorax
in children. Can J Surg. 50(3):171-4, 2007
Patent Ductus Arteriosus
Patent ductus arteriosus (PDA) is a fetal vessel that connects the main
pulmonary trunk with the descending aorta distal to the origin of the left
subclavian artery. Most term infants have the PDA closed by three weeks
of age, while premature takes longer to close. In terms infants, PDA can
lead to death from congestive heart failure (tachypnea, tachycardia, poor
feeding, slow weight gain and recurrent pulmonary infections). In premature,
the PDA can lead to sepsis, renal failure, necrotizing enterocolitis, metabolic
acidosis and death. PDA murmur is systolic. Indications for closure include
congestive heart failure, bacterial endocarditis, failure to close during
the first year of life. In premature the PDA can be medically closed with
pharmacologic indomethacin therapy (prostaglandin inhibition). If medical
therapy fails surgical closure is needed. Surgical closure (division is
preferred for term and older children, while ligation is reserved for premature)
can be done through a left lateral thoracotomy, a medial sternotomy or
from inside the pulmonary artery. Other alternatives include percutaneous
catheter closure or thoracoscopic closure of the PDA. Some postoperative
complications include bleeding, damage to phrenic, vagus or recurrent laryngeal
nerve and chylothorax. In general prognosis is good.
References:
1- Little DC, Pratt TC, Blalock SE, Krauss DR, Cooney
DR, Custer MD: Patent ductus arteriosus in micropreemies and full-term
infants: the relative merits of surgical ligation versus indomethacin
treatment. J Pediatr Surg. 38(3):492-6, 2003
2- Wyllie J: Treatment of patent ductus arteriosus. Semin
Neonatol. 8(6):425-32, 2003
3- Van Overmeire B, Chemtob S: The pharmacologic closure
of the patent ductus arteriosus. Semin Fetal Neonatal Med. 10(2):177-84,
2005
4- Arora R: Transcatheter closure of patent ductus arteriosus.
Expert Rev Cardiovasc Ther. 3(5):865-74, 2005
5- Hermes-DeSantis ER, Clyman RI: Patent ductus arteriosus:
pathophysiology and management. J Perinatol. 26 Suppl 1:S14-8, 2006
6- Herrera C, Holberton J, Davis P: Prolonged versus
short course of indomethacin for the treatment of patent ductus arteriosus
in preterm infants. Cochrane Database Syst Rev. 18;(2):CD003480, 2007
PSU Volume 30 No 06 JUNE 2008
Doxycycline Sclerotherapy
Lymphangiomas or cystic hygromas are congenital lymphatic vessels developmental
anomalies affecting primarily the head and neck region of newborns. They
manifest as multiple lymphatic fluid cysts involving muscle, fascia, blood
vessels and nerves. This anatomic fact makes surgical excision very dangerous
in respect to mutilating complications. Alternatively, sclerotherapy has
progressively become the therapy of choice in many such cases. OK-432,
bleomycin, fibrin glue sealant and recently doxycycline are the most
commonly used sclerosing agents. Lymphangiomas are classified as either
macrocystic, microcystic or mixed. Pretreatment imaging to classify the
lymphangioma is mandatory, and MRI is the modality of choice for the most
definitive evaluation and classification of these cysts. Macrocystic and
mixed lesions respond well to percutaneous sclerotherapy, while microcystic
disease is usually managed with surgical excision. Doxycycline is an inexpensive
and available tetracycline with widespread use as effective sclerosant
in other parts of the body (pleura, pericardium and postoperative lymphoceles).
A recent study confirmed doxycycline efficacy in managing macrocystic and
mixed lymphatic malformations of the head and neck in children without
significant side effects (4). Aspiration and sclerotherapy is done at the
operating room under general anesthesia using ultrasound cyst guidance.
References:
1- Molitch HI, Unger EC, Witte CL, vanSonnenberg E: Percutaneous
sclerotherapy of lymphangiomas. Radiology. 194(2):343-7, 1995
2- Wimmershoff MB, Schreyer AG, Glaessl A, Geissler A,
Hohenleutner U, Feuerbach SS, Landthaler M: Mixed capillary/lymphatic malformation
with coexisting port-wine stain: treatment
utilizing 3D MRI and CT-guided sclerotherapy. Dermatol
Surg. 26(6):584-587, 2000
3- Mabrut JY, Grandjean JP, Henry L, Chappuis JP, Partensky
C, Barth X, Tissot E: Mesenteric and mesocolic cystic lymphangiomas. Diagnostic
and therapeutic management Ann Chir. 127(5):343-9, 2002
4- Nehra D, Jacobson L, Barnes P, Mallory B, Albanese
CT, Sylvester KG: Doxycycline sclerotherapy as primary treatment of head
and neck lymphatic malformations in children. J Pediatr Surg 43(3): 451-460,
2008
Pancreaticobiliary Maljunction
Pancreaticobiliary maljunction (PBM) or common channel is defined as
communication of the common bile duct and pancreatic duct outside the sphincter
of Oddi (main papilla). Etiology of PBM is caused by a disturbance in the
embryonic connections (misarrangement) of the choledochopancreatic duct
system in the early embryo, whereby the terminal bile duct joins with a
branch of the ventral pancreatic duct system, including the main pancreatic
duct. This malformation causes mixing of bile with pancreatic secretions
resulting in choledochal cysts, recurrent pancreatitis, biliary tract carcinoma
and formation of protein plugs. The frequency of gallbladder cancer in
patients with PBM without congenital biliary duct dilatation is very high
making some consider prophylactic cholecystectomy or resection of the extrahepatic
bile duct for carcinogenesis prevention. The diagnostic criteria for PBM
are the radiological and anatomical detection of the extramural location
of the junction of the pancreatic and biliary ducts in the duodenal wall
done by MRCP or ERCP. Clinical features of PBM are intermittent abdominal
pain, with or without elevation of pancreatic enzyme levels; and obstructive
jaundice, with or without acute pancreatitis, while the clinical features
of PBM patients with congenital cystic duct dilatation are primary bile
duct stone and acute cholangitis. Objective in management of PBM is prevention
of the reciprocal reflux of bile and pancreatic juice in the pancreas and
the bile duct system. To achieve these aims the surgical (resection and
bilioenteric reconstruction) or endoscopic approach (papillotomy) is utilized.
References:
1- Funabiki T, Matsubara T, Ochiai M, Marugami Y, Sakurai
Y, Hasegawa S, Imazu H: Surgical strategy for patients with pancreaticobiliary
maljunction without choledocal dilatation. Keio J Med. 46(4):169-72, 1997
2- Matsumoto Y, Fujii H, Itakura J, Matsuda M, Nobukawa
B, Suda K: Recent advances in pancreaticobiliary maljunction. J Hepatobiliary
Pancreat Surg. 9(1):45-54, 2002
3- Hamada Y, Tanano A, Takada K, Watanabe K, Tokuhara
K, Sato M: Magnetic resonance cholangiopancreatography on postoperative
work-up in children with choledochal cysts. Pediatr Surg Int. 20(1):43-6,
2004
4- Ohuchida J, Chijiiwa K, Hiyoshi M, Kobayashi K, Konomi
H, Tanaka M: Long-term results of treatment for pancreaticobiliary maljunction
without bile duct dilatation. Arch Surg. 141(11):1066-70, 2006
5- Ohama K, Ishikawa N: Dilatation of the intrahepatic
bile duct associated with congenital anomalous junction of the cystic duct--a
new disease entity. J Pediatr Surg. 41(12):1996-8, 2006
6- Terui K, Yoshida H, Kouchi K, Hishiki T, Saito T,
Mitsunaga T, Takenouchi A, Tsuyuguchi T, Yamaguchi T, Ohnuma N: Endoscopic
sphincterotomy is a useful preoperative management for refractory pancreatitis
associated with pancreaticobiliary maljunction. J Pediatr Surg. 43(3):495-9,
2008
7- Okada T, Sasaki F, Honda S, Naitou S, Onodera Y, Todo
S: Usefulness of axial planes of helical computed tomography for diagnosis
of pancreaticobiliary maljunction in early infants with negative findings
on magnetic resonance cholangiopancreatography. J Pediatr Surg. 43(3):579-82,
2008
Gastroparesis
Gastroparesis (GP) is a gastric disorder associated to symptoms of gastric
retention or altered gastric emptying without evidence of mechanical obstruction.
GP in children is usually associated with diabetes mellitus, postviral
syndrome or after surgical procedures. Symptoms associated with gastroparesis
include nausea, vomiting, early satiety, postprandial fullness and abdominal
pain. Establishing the diagnosis of GP is difficult and requires UGIS,
gastric scintigraphy, electrogastrography and endoscopy with biopsy.
Initial management consists of dietary modification, prokinetic agents
(cisapride), antiemetic therapy and pain control. More severe symptoms
may need enteral or parenteral nutritional support. Endoscopic injection
of botulinum toxin to the pyloric muscle can help. Surgery therapy might
consist of pyloromyotomy, pyloroplasty (gastric emptying procedure), gastrostomy
tube, jejunal feeding tube or gastrectomy procedures. Recently gastric
electrical stimulation pacemaker placement has improved children with gastroparesis.
References:
1- Sigurdsson L, Flores A, Putnam PE, Hyman PE, Di Lorenzo
C: Postviral gastroparesis: presentation, treatment, and outcome. J Pediatr.
131(5):751-4, 1997
2- Katz S, Lazar L, Erez I, Kaufman Z: Subtotal gastrectomy
in a teenager with gastroparesis. J Pediatr Surg. 34(3):509-11, 1999
3- Michaud L, Guimber D, Carpentier B, Sfeir R, Lambilliotte
A, Mazingue F, Gottrand F, Turck D: Gastrostomy as a decompression technique
in children with chronic gastrointestinal obstruction. J Pediatr Gastroenterol
Nutr. 32(1):82-5, 2001
4- Smith DS, Williams CS, Ferris CD: Diagnosis and treatment
of chronic gastroparesis and chronic intestinal pseudo-obstruction. Gastroenterol
Clin North Am. 32(2):619-58, 2003
5- Chelimsky TC, Chelimsky GG: Autonomic abnormalities
in cyclic vomiting syndrome. J Pediatr Gastroenterol Nutr. 44(3):326-30,
2007
6- Islam S, Vick LR, Runnels MJ, Gosche JR, Abell T:
Gastric electrical stimulation for children with intractable nausea and
gastroparesis. J Pediatr Surg. 43(3):437-42, 2008