PEDIATRIC SURGERY UPDATE ©
VOLUME 37, 2011
PSU Volume 37 No 01 JULY 2011
Necrotizing Pancreatitis
Necrotizing pancreatitis (NP) refers
to a fulminant disease stage of the pancreas that occurs in 20% of all
patients that develop acute pancreatitis increasing morbidity and
mortality significantly. The initial etiology of the pancreatitis in
children is in order of incidence trauma, drug-induced, biliary
disorders (gallstones), infectious, metabolic and congenital. These
conditions can lead to pancreatic autodigestion by enzyme activation.
Symptoms include abdominal pain, vomiting, fever, elevated amylase and
lipase with leukocytosis. Ultrasound typically shows a diffusely
enlarged hypoechogenic pancreas. Contrast-enhanced CT Scan is the gold
standard for diagnosing necrotizing pancreatitis. The affected
portions of the pancreas in NP fails to enhanced due to disruption of
the normal microcirculation. Initial management of NP consists of
intensive medical support and prevention of infection (systemic and po
non-absorbable antibiotics). With persistent biliary obstruction in the
face of pancreatitis, ERCP should be used in combination with
sphincterotomy to relieve the obstruction. Thirty to 70% of patients
with NP develop a local pancreatic infection which triple the
mortality. Infected peripancreatic collection should be percutaneously
aspirated and drained. Surgery with necrosectomy should be delayed as
long as possible and has no proven role in sterile necrosis.
Complications associated with NP include persistent infection,
hemorrhage, pancreatic fistula, duodenal obstruction and pancreatic
insufficiency. The mortality is significant.
References
1- Takeda K, Matsuno S, Sunamura M, Kobari M: Surgical aspects
and management of acute necrotizing pancreatitis: recent results of a
cooperative national survey in Japan. Pancreas. 16(3):316-22, 1998
2- Sakorafas GH, Tsiotou AG: Etiology and pathogenesis of acute
pancreatitis: current concepts. J Clin Gastroenterol. 30(4):343-56, 2000
3- Krueger WA, Unertl KE: Selective decontamination of the digestive tract. Curr Opin Crit Care. 8(2):139-44, 2002
4- Takeda K, Mikami Y, Fukuyama S, Egawa S, Sunamura M, Ishibashi T,
Sato A, Masamune A, Matsuno S: Pancreatic ischemia associated with
vasospasm in the early phase of human acute necrotizing pancreatitis.
Pancreas. 30(1):40-9, 2005
5- Babu BI, Sheen AJ, Lee SH, O'Shea S, Eddleston JM, Siriwardena AK.
Open pancreatic necrosectomy in the multidisciplinary management of
postinflammatory necrosis. Ann Surg. 251(5):783-6, 2010
6- Wittau M, Scheele J, Gölz I, Henne-Bruns D, Isenmann R:
Changing role of surgery in necrotizing pancreatitis: a single-center
experience.Hepatogastroenterology. 57(102-103):1300-4, 2010
Chilaiditi Syndrome
Chilaiditi syndrome refers to the
abdominal symptoms that arise when a piece of bowel interposition
between the liver and the diaphragm. Chilaiditi syndrome is a rare
anomaly which occurs in up to 0.28 percent of the population and a
source of abdominal problems requiring emergency or elective surgery.
Hepatodiaphragmatic interposition of the transverse colon or small
intestine can cause Chilaiditi syndrome. Though usually asymptomatic,
symptoms can range from intermittent abdominal pain, vomiting,
bloating, constipation to acute bowel obstruction. A few children have
developed respiratory distress. The plain chest and abdominal films are
diagnostic. This condition can be mistaken for pneumoperitoneum. US can
help avoid confusion when pneumoperitoneum is suspected. The presence
of hepatodiaphragmatic interposition of the intestine requires no
specific treatment in the absence of symptoms. Volvulus of the
transverse colon, history of prior abdominal surgery and colon
redundancy can be associated with Chilaiditi syndrome. Surgery is
typically reserved for cases of catastrophic colonic volvulus or
perforation because of the syndrome. In cases of severe Chilaiditi
syndrome refractory to medical treatment, a minimally invasive colopexy
should be considered as a possible treatment option.
References:
1- Risaliti A, De Anna D, Terrosu G, Uzzau A, Carcoforo P,
Bresadola F: Chilaiditi's syndrome as a surgical and nonsurgical
problem. Surg Gynecol Obstet. 176(1):55-8, 1993
2- Sato M, Ishida H, Konno K, Hamashima Y, Naganuma H, Komatsuda T,
Ishida J, Watanabe S: Chilaiditi syndrome: sonographic findings. Abdom
Imaging. 25(4):397-9, 2000
3- White JJ, Chavez EP, Souza J: Internal hernia of the transverse
colon-Chilaiditi syndrome in a child. J Pediatr Surg. 37(5):802-4, 2002
4- Barroso Jornet JM, Balaguer A, Escribano J, Pagone F, Domenech J,
del Castillo D: Chilaiditi syndrome associated with transverse colon
volvulus: first report in a paediatric patient and review of the
literature. Eur J Pediatr Surg. 13(6):425-8, 2003
5- Saber AA, Boros MJ: Chilaiditi's syndrome: what should every surgeon know? Am Surg. 71(3):261-3, 2005
6- Keles S, Artac H, Reisli I, Alp H, Koc O: Chilaiditi syndrome as a cause of respiratory distress.
Eur J Pediatr. 165(6):367-9, 2006
7- Blevins WA, Cafasso DE, Fernandez M, Edwards MJ: Minimally invasive
colopexy for pediatric Chilaiditi syndrome. J Pediatr Surg.
46(3):e33-5, 2011
Gastric Electrical Stimulation
Gastric electrical stimulation (GES)
has recently been developed as an alternative in the management of
medically-refractory intractable nausea and gastroparesis associated
with diabetes mellitus, postviral illness, idiopathic or postsurgical.
The technique consists of placing two electrodes in the anterior
seromuscular wall of the stomach hook to a subcutaneously placed
stimulator using either an open or laparoscopic approach. Temporary
percutaneously placed gastric stimulation electrodes using gastroscopy
helps decide whether the patient will be a responder to the use
permanent gastric electrical stimulation. The antiemetic effect of GES
is mainly mediated by vagal afferent pathways. High-frequency GES has
beneficial effects on symptoms in children with diabetic or idiopathic
gastroparesis and severe nausea with vomiting. There is a significant
improvement in symptoms over a prolonged period, and there are no
adverse effects of the GES.
References:
1- Vandenplas Y, Hauser B, Salvatore S: Current pharmacological
treatment of gastroparesis. Expert Opin Pharmacother. 5(11):2251-4, 2004
2- 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
3- Hyman P, Schropp K, Sarosiek I, Forster J, Lin Z, Gertken J,
McCallum R: Feasibility and safety of gastric electrical stimulation
for a child with intractable visceral pain and gastroparesis. J
Pediatr Gastroenterol Nutr. 49(5):635-8, 2009
4-Andersson S, Ringström G, Elfvin A, Simrén M,
Lönroth H, Abrahamsson H: Temporary percutaneous gastric
electrical stimulation: a novel technique tested in patients with
non-established indications for gastric electrical stimulation.
Digestion. 83(1-2):3-12, 2011
5-Elfvin A, Göthberg G, Lönroth H, Saalman R, Abrahamsson H:
Temporary percutaneous and permanent gastric electrical stimulation in
children younger than 3 years with chronic vomiting. J Pediatr Surg.
46(4):655-61, 2011.
PSU Volume 37 NO 02 AUGUST 2011
Neoappendicostomy
The use of the appendix as a conduit
(appendicostomy) for the management of fecal incontinence caused
by anorectal malformations, spina bifida and other diagnosis, has
markedly improved the quality of life of children. Using this approach
the child can regularly irrigate his large bowel and maintain socially
continent for long periods of time. It also creates independence care
as the child learns how to maintain clean using the prograde
enemas regimen. When the appendix is not available due to previous
surgical removal, a neoappendicostomy using a flap of cecum or
ascending colon can be constructed. Other alternatives are using the
sigmoid, transverse or descending colon. The flap must feed from the
mesenteric side of the bowel to avoid ischemia. The neoappendicostomy
created is wrap by a segment of colon creating a valve mechanism
to reduce the leakage rate. Other workers have found the wrap and
fixation unnecessary. The two main complications of the procedure are
stricture formation and leakage. The button cecostomy is another common
alternative for the child with no appendix available. These buttons can
be placed open, laparoscopically, percutaneously or endoscopically.
Complications of the buttons include dislodgement, granulation tissue,
leakage, pain and ulcer formation. Incidental appendectomy should be
discouraged in children with the potential to develop fecal
incontinence in the future.
References:
1- Koivusalo A, Pakarinen M, Rintala RJ: Are cecal wrap and fixation
necessary for antegrade colonic enema appendicostomy? J Pediatr Surg.
41(2):323-6, 2006
2- Kim J, Beasley SW, Maoate K: Appendicostomy stomas and antegrade
colonic irrigation after laparoscopic antegrade continence enema.J
Laparoendosc Adv Surg Tech A. 16(4):400-3, 2006
3- Koivusalo AI, Pakarinen MP, Pauniaho SL, Rintala RJ: Antegrade
continence enema in the treatment of congenital fecal incontinence
beyond childhood. Dis Colon Rectum. 51(11):1605-10, 2008
4-Bowkett BD, Kelly EW: Mucosal colonic tube fistula with antireflux
wrap for antegrade colonic enema. Pediatr Surg Int. 25(6):507-11, 2009
5- Lawal TA, Rangel SJ, Bischoff A, Pena A, Levitt MA:
Laparoscopic-assisted malone appendicostomy in the management of fecal
incontinence in children. J Laparoendosc Adv Surg Tech A. 21(5):455-9,
2011
6- Chatoorgoon K, Pena A, Lawal T, Hamrick M, Louden E,
Levitt MA: Neoappendicostomy in the management of pediatric fecal
incontinence. J Pediatr Surg. 46(6): 1243-1249, 2011
Vascular Malformations Male Genitalia
Vascular malformations involving the
male genitalia can cause considerable stress, adversely impact a child
self-image, cause pain and alter urinary, reproductive and sexual
function. Vascular malformations of male genitalia are more common than
vascular tumors. They are divided in slow and fast flow lesions. The
most common slow-flow vascular malformations affecting this anatomic
area are lymphatics, venous or a combination of both. Fast-flow lesions
are more commonly arteriovenous defects. These malformations clinically
present with swelling, pain, fluid leakage, and infection. Other
specific symptoms include hematuria, chylous reflux, dysuria, bladder
outlet obstruction, erectile dysfunction and ambiguous genitalia.
Diagnosis is made with a combination of physical exam and imaging.
Indications for management of vascular malformations include bleeding,
infection, fluid leakage, dysfunction and cosmetic concern. Management
of vascular malformation includes surgical excision, pharmacotherapy,
sclerotherapy, embolization, or a combination. The most common specific
malformation is the infantile hemangioma. Though treatment is usually
expectant there is place for use of steroid, propanolol and surgical
excision. Lymphatic malformations in the groin and cord can be confused
with an inguinal hernia. Combined slow-flow malformations are usually
associated with soft tissue and skeletal hypertrophy.
References:
1- Go S, Tokiwa K, Imazu M, Higuchi K, Iwai N: Arteriovenous
malformation of the penis in an infant. J Pediatr Surg. 35(7):1130-1,
2000
2- Marrocco-Trischitta MM, Nicodemi EM, Stillo F: Sclerotherapy for
venous malformations of the glans penis. Urology. 57(2):310-3, 2001
3- Morrison SC, Reid JR: Continuing problems with classifications of vascular malformations. Pediatr Radiol. 37(6):609, 2007
4- Papali AC, Alpert SA, Edmondson JD, Maizels M, Yerkes E, Hagerty J,
Chaviano A, Kaplan WE: A review of pediatric glans malformations: a
handy clinical reference. J Urol. 180(4 Suppl):1737-42, 2008
5-Kulungowski AM, Schook CC, Alomari AI, et al: Vascular anomalies of
the male genitalia. J Pediatr Surg. 46(6):1214–1221, 2011
Appendix Diverticulum
Diverticulum of the appendix is a very
rare disorder. Two types have been described. The true or congenital
type in which all layers of the wall of the appendix are found in the
wall of the diverticulum. The lesion is in the antimesenteric border
and may be single or multiple. The other type most commonly found is
the false or acquired diverticulum in which the muscular layer is
absent from the wall. Acquired diverticulum are found on the mesenteric
border and most commonly multiple. Appendix diverticulum can develop
inflammation and perforation. Clinical symptoms range from chronic
right lower quadrant abdominal pain to acute appendicitis. With
inflammation of the diverticulum the most common diagnosis entertained
is acute appendicitis. Even at operation the diagnosis of a
diverticulum causing the inflammation can be missed. The congenital
variety brings problems in the pediatric age, while the acquired form
is more proper of the adult population. The management in all cases is
appendectomy. Asymptomatic diverticulosis of the appendix found
incidentally warrants appendectomy. Children with cystic fibrosis have
a high incidence of appendix diverticulosis.
References:
1- Trollope ML, Lindenauer SM: Diverticulosis of the appendix: a collective review. Dis Colon Rectum. 17(2):200-18, 1974
2- George DH: Diverticulosis of the vermiform appendix in patients with cystic fibrosis. Hum Pathol. 18(1):75-9, 1987
3- Konen O, Edelstein E, Osadchi A, Shapiro M, Rathaus V: Sonographic
appearance of an appendiceal diverticulum. J Clin Ultrasound.
30(1):45-7, 2002
4- Rakovich G: Diverticulosis of the appendix. Dig Surg. 23(1-2):26, 2006
5- Barc RM, Rousset J, Maignien B, Lu M, Prime-Guitton CH, Garcia JF:
Diverticula of the appendix and their complications: value of
sonography (review of 21 cases). J Radiol. 86(3):299-309, 2005
6- Majeski J: Diverticulum of the vermiform appendix is associated with chronic abdominal pain. Am J Surg. 186(2):129-31, 2003
7- Yates LN: Diverticulum of the vermiform appendix. A review of 28 cases. Calif Med. 116(1):9-11, 1972
PSU Volume 37 NO 03 SEPTEMBER 2011
HBO Therapy
Hyperbaric oxygen (HBO) therapy is a
conservative therapy that involves inhaling up to 100% oxygen at a
pressure greater than one atmosphere in a pressurized chamber design
for such purpose. Most typical indications for hyperbaric oxygen
therapy involve the use of hyperbaric pressures above two atmospheres.
Such pressures are needed to manage conditions such as carbon monoxide
poisoning and improve wound healing. HBO therapy for children is safe,
even at pressures above two atmospheres. Some surgical conditions were
concomitant HBO therapy in children has shown favorable results include
crush injury, traumatic ischemia, electrical injuries, compartment
syndrome, clostridial myonecrosis, necrotizing fasciitis,
chemotherapy-induced neutropenia fasciitis, compromised skin graft,
ischemic flaps, and refractory osteomyelitis. In order to see the
favorable effects of HBO therapy, it is better to start the
treatment within the first 24-48 hours following injury. An increased
oxygen concentration during HBO therapy promoted spontaneous wound
healing. The needs of the pediatric patient, especially the critically
ill, require specific skills and equipment inside the hyperbaric
chamber. Close collaboration between the pediatrician and the
hyperbaric medicine physician is essential to ensure adequate care for
infants and children.
References:
1- Waisman D, Shupak A, Weisz G, Melamed Y: Hyperbaric oxygen therapy
in the pediatric patient: the experience of the Israel Naval Medical
Institute. Pediatrics. 102(5):E53, 1998
2- Johnston DL, Waldhausen JH, Park JR: Deep soft tissue infections in
the neutropenic pediatric oncology patient. J Pediatr Hematol Oncol.
23(7):443-7, 2001
3- Rossignol DA, Rossignol LW, James SJ, Melnyk S, Mumper E: The
effects of hyperbaric oxygen therapy on oxidative stress, inflammation,
and symptoms in children with autism: an open-label pilot study. BMC
Pediatr. 16;7:36, 2007
4- Pandey A, Gangopadhyay AN, Upadhyaya VD: Necrotising fasciitis in
children and neonates: current concepts. J Wound Care. 17(1):5-10, 2008
5- Ambiru S, Furuyama N, Aono M, Otsuka H, Suzuki T, Miyazaki M:
Analysis of risk factors associated with complications of hyperbaric
oxygen therapy. J Crit Care. 23(3):295-300, 2008
6- Takac I, Kvolik S, Divkovic D, Kalajdzic-Candrlic J, Puseljic S,
Izakovic S: Conservative surgical management of necrotic tissues
following meningococcal sepsis: case report of a child treated with
hyperbaric oxygen. Undersea Hyperb Med. 37(2):95-9, 2010
Anterior Mediastinal Mass
The differential diagnosis of an
anterior mediastinal mass in a child includes in order of increased
frequency a lymphoma, teratoma, thymoma or suprasternal goiter.
Histopathologic analysis of an anterior mediastinal mass of unknown
origin is essential for treatment decision. An anterior mediastinal
mass can risk airway compromised. General anesthesia should be avoided
in children with tracheal cross sectional area or peak expiratory flow
rate less than 50% of predicted for age and sex, or severe narrowing
and occlusion of a main stem bronchi. Diagnosis can be established in
most patients by open biopsy or image-guided core needle biopsy under
local anesthesia. Other cases might need aspiration of pleural effusion
or bone marrow biopsy for diagnosis. Children that present with
symptoms of dyspnea at rest, orthopnea, respiratory distress, or
stridor are at risk of respiratory collapse. In severe cases when
symptoms of respiratory collapse are present and the diagnosis cannot
be obtained, a short course of steroid therapy can reduce the size of
the mass and improve the child clinically. The Chamberlain procedure
provides excellent access to the antero-superior mediastinum for biopsy
of obscure mediastinal mass lesions in childhood with a low rate of
complications. Should general anesthesia be needed, spontaneous
ventilation is preferred.
References:
1- Shamberger RC: Preanesthetic evaluation of children with anterior mediastinal masses. Semin Pediatr Surg. 8(2):61-8, 1999
2- Lam JC, Chui CH, Jacobsen AS, Tan AM, Joseph VT: When is a
mediastinal mass critical in a child? An analysis of 29 patients.
Pediatr Surg Int. 20(3):180-4, 2004
3- Salas Valverde S, Gamboa Y, Vega S, Barrantes M, Gonzalez M, Zamora
JB: Diagnosis of anterior mediastinal mass lesions using the
Chamberlain procedure in children. Pediatr Surg Int. 24(8):935-7, 2008
4- Perger L, Lee EY, Shamberger RC: Management of children and
adolescents with a critical airway due to compression by an
anterior mediastinal mass. J Pediatr Surg. 43(11):1990-7, 2008
5- Stricker PA, Gurnaney HG, Litman RS: Anesthetic management of
children with an anterior mediastinal mass. J Clin Anesth.
22(3):159-63, 2010
Cleft Lift Procedure
Sacrococcygeal pilonidal sinus disease
is a chronic disorder that recurs even after adequate surgical
procedures. Recurrence and chronicity are attributed to the depth of
the gluteal cleft, moist bacteria-laden conditions within the airless
cleft, incomplete excision of the diseased tissue, midline suture line,
and closure tension. For the past years the cleft lift procedure has
been applied to this disorder as a means of reducing recurrence. This
technique involves excision of the chronic disease with a parasagittal
elliptical resection and primary closure of the defect with a
lateral-based flap, resulting in a tension-free closure that flattens
the natal cleft and shifts the healing point laterally off of the
midline. The flap technique aims at removing all the inflamed deep
tissues, and the creation of a thick fat flap that reduces the depth of
the airless cleft while decreasing the recurrence rate significantly.
The cleft lift procedure does not involve wide excision of the deep
inflamed tissue. The cleft lift procedure is a superior treatment
method of pilonidal disease in adolescents, resulting in improved wound
healing and lower likelihood of recurrent disease. Shaving, pulling
hairs from the pores, and careful cleaning may be enough for earliest
disease. The mystery of unhealed pilonidal disease is solved by
understanding that the shape of the cleft and the conditions generated
within that cleft inhibit epidermal closure and are the reason for the
failure to heal.
References:
1- Bascom J, Bascom T: Failed pilonidal surgery: new paradigm and new operation leading to cures.
Arch Surg. 137(10):1146-50, 2002
2- Bascom J, Bascom T: Utility of the cleft lift procedure in refractory pilonidal disease. Am J Surg. 193(5):606-9, 2007
3- Rushfeldt C, Bernstein A, Norderval S, Revhaug A: Introducing an
asymmetric cleft lift technique as a uniform procedure for pilonidal
sinus surgery. Scand J Surg. 97(1):77-81, 2008
4- Abdelrazeq AS, Rahman M, Botterill ID, Alexander DJ: Short-term and
long-term outcomes of the cleft lift procedure in the management of
nonacute pilonidal disorders. Dis Colon Rectum. 51(7):1100-6, 2008
5- Dudink R, Veldkamp J, Nienhuijs S, Heemskerk J: Secondary healing
versus midline closure and modified bascom natal cleft lift for
pilonidal sinus disease. Scand J Surg. 100(2):110-3, 2011
6- Gendy AS, Glick RD, Hong AR:A comparison of the cleft lift procedure vs wide excision and
packing for the treatment of pilonidal disease in adolescents. J Pediatr Surg. 46(6): 1256-59, 2011
PSU Volumen 37 NO 04 OCTOBER 2011
Ventriculo-Gallbladder Shunts
When dealing with hydrocephalus in
children the preferred method of drainage by neurosurgeons is the use
of ventriculo-peritoneal shunting. Ventriculoperitoneal shunting may
become dysfunctional due to repeated infections, intraabdominal
adhesions, peritonitis, multiple abdominal surgical procedures,
cerebrospinal fluid cysts or loculations, and other anatomic causes.
When the peritoneum is useless as absorptive medium for CSF fluid,
other alternatives previously used include the central venous system
(atrium), pleural cavity, gallbladder, stomach, ureter and even
fallopian tubes. The ventriculo-cholecystic (gallbladder) shunt is an
attractive alternative when the intraperitoneal and intravascular route
is not longer available. Ultrasound and nuclear studies of the
gallbladder should be performed preop to determine absence of sludge or
stones and adequate bile emptying. A fenestrated piece of distal shunt
within the gallbladder lumen connected by a metal device to the
proximal shunt catheter will provide the placement of the purse string
in the gallbladder fundus. The gallbladder remains functional and
revisions free. In several series involving the use of
ventriculo-gallbladder shunts the long-term patency rate is above 75%.
Infection and obstruction are the most common complications.
References:
1- West KW, Turner MK, Vane DW, Boaz J, Kalsbeck J, Grosfeld JL:
Ventricular gallbladder shunts: an alternative procedure in
hydrocephalus. J Pediatr Surg. 22(7):609-12, 1987
2- Stringel G, Turner M, Crase T: Ventriculo-gallbladder shunts in children. Childs Nerv Syst. 9(6):331-3, 1993
3- Ketoff JA, Klein RL, Maukkassa KF: Ventricular cholecystic shunts in children. J Pediatr Surg. 32(2):181-3, 1997
4- Pal K, Jindal V: Ventriculo cholecystic shunt in the management of hydrocephalus. Indian Pediatr. 44(6):435-7, 2007
5- Aldana PR, James HE, Postlethwait RA: Ventriculogallbladder shunts
in pediatric patients. J Neurosurg Pediatr. 1(4):284-7, 2008
6- Girotti ME, Singh RR, Rodgers BM: The ventriculo-gallbladder shunt
in the treatment of refractory hydrocephalus: a review of the current
literature. Am Surg. 75(8):734-7, 2009
Esophageal Lung
Esophageal lung refers to a very rare
congenital bronchopulmonary foregut malformation where there is an
anomalous connection of a main stem bronchus with the esophagus or
stomach instead of the trachea. Esophageal lung anomaly usually occurs
in the right side with no sex predilection. This anomaly arises when
independent collections of cells with respiratory potential arise from
the primitive esophagus caudal to the normal lung bud or when part of
the lung bud originates from the dorsal esophagus instead of the
ventral laryngotracheal tube. Associated anomalies consist of
esophageal atresia, tracheoesophageal fistula, and cardiac defects. In
cases of esophageal atresia, is the distal esophagus where the
esophageal lung is connected through a bronchus arising from it. The
most common clinical presentation is recurrent lower respiratory tract
infections, atelectasis, and bronchiectasis arising from this lung
which is usually hypoplastic and unilobar. Diagnosis is made with
contrast study of the esophagus, trachea and CT Scan. The esophageal
lung receives its blood supply from the pulmonary artery different from
a sequestration where the blood supply comes directly from an aortic
branch. Drainage occurs via the inferior pulmonary veins. Management of
esophageal lung consists of resection of the destroyed hypoplastic lung
with repair of the esophageal communication. In early cases where
the esophageal lung is not destroyed, it has been reimplanted to the
trachea.
References:
1- Leithiser RE Jr, Capitanio MA, Macpherson RI, Wood BP:
"Communicating" bronchopulmonary foregut malformations. AJR Am J
Roentgenol. 1986 Feb;146(2):227-31
2- Srikanth MS, Ford EG, Stanley P, Mahour GH: Communicating
bronchopulmonary foregut malformations: classification and
embryogenesis.J Pediatr Surg. 1992 Jun;27(6):732-6.
3- Jamieson DH, Fisher RM: Communicating bronchopulmonary foregut
malformation associated with esophageal atresia and tracheo-esophageal
fistula. Pediatr Radiol. 1993;23(7):557-8.
4- Michel JL, Revillon Y, Salakos C, De Blic J, Jan D, Beringer A,
Scheinmann P: Successful bronchotracheal reconstruction in esophageal
bronchus: two case reports. J Pediatr Surg. 1997 May;32(5):739-42
5- Tsugawa J, Tsugawa C, Satoh S, Nishijima E, Muraji T, Takamizawa S,
Kanegawa K, Akasaka Y: Communicating bronchopulmonary foregut
malformation: particular emphasis on concomitant congenital
tracheobronchial stenosis.Pediatr Surg Int. 2005 Nov;21(11):932-5
6- Sugandhi N, Sharma P, Agarwala S, Kabra SK, Gupta AK, Gupta DK:
Esophageal lung: presentation, management, and review of literature. J
Pediatr Surg. 2011 Aug;46(8):1634-7
Histiocytosis
Histiocytosis refers to a general term
use for a group of syndromes that involves an abnormal increase in the
number of immune cells known as histiocytes. The three majors type of
histiocytosis are Langerhans's cell (histiocytosis X), malignant
histiocytosis syndrome (T-cell lymphoma), and non-Langerhans's
histiocytosis (hemophagocytic syndrome). Histiocytosis X is the most
common, and is type of autoimmune condition in which the immune cells
attack the body by mistake. Extra-immune cells may form tumors which
affect the bones, the skulls and other areas of the body. Most cases
are children within the ages of one to 15. Up to 50% of patients
with either single or multi-organ manifestation of Langerhans'
cell histiocytosis initially present with cutaneous symptoms. Symptoms
depend on the system affected such as abdominal pain, bone pain,
irritability, fever, swollen lymph nodes, etc. Children above age five
often have bone involvement. Punch-out lesions in bone x-ray are
characteristics. Management of Histiocytosis X consists of
immunosuppression, chemotherapy and radiotherapy. A localized
self-limited cutaneous form of the disease can be managed with complete
surgical excision. Three types of skin lesions usually occur: nodules
(common), scaling, or crusted papules and soft, yellow papular
xanthomas.
References:
1- Chu T: Langerhans cell histiocytosis. Australas J Dermatol. 42(4):237-42, 2001
2- Janka GE, Schneider EM: Modern management of children with
haemophagocytic lymphohistiocytosis. Br J Haematol. 124(1):4-14, 2004
3- Gasent Blesa JM, Alberola Candel V, Solano Vercet C, Laforga Canales
J, Semler C, Pérez AntolÃMR,
RodrÃguez-Galindo C: Langerhans cell histiocytosis. Clin Transl
Oncol. 10(11):688-96, 2008
4- Abla O, Egeler RM, Weitzman S: Langerhans cell histiocytosis:
Current concepts and treatments. Cancer Treat Rev. 36(4):354-9, 2010
5- Querings K, Starz H, Balda BR: Clinical spectrum of cutaneous
Langerhans' cell histiocytosis mimicking various diseases.Clinical
spectrum of cutaneous Langerhans' cell histiocytosis mimicking various
diseases. Acta Derm Venereol. 86(1):39-43, 2006
6- Hussein MR: Skin-limited Langerhans' cell histiocytosis in children. Cancer Invest. 27(5):504-11, 2009
PSU Volume 37 No 05 NOVEMBER 2011
Chest Wall Tumors
Primary chest wall tumors (PCWT) in
children are rare, with the majority being malignant. They originate
from the skeletal chest wall or surrounding soft tissue. Benign chest
wall tumors consist of osteochondroma, lipoblastoma, osteoblastoma bone
cyst and eosinophilic granuloma. Resection provides cure in most cases.
Malignant PCWT includes primitive neuroectodermal tumor (formerly
Ewing's sarcoma), rhabdomyosarcoma, chondrosarcoma, neuroblastoma,
leiomyosarcoma, fibrosarcoma and osteogenic sarcoma. Both leukemia and
lymphoma can present as chest wall masses. Diagnosis of PCWT includes
simple chest film, CT Scan and MRI studies. An initial biopsy by either
core-needle or open is warranted since many of these tumors are
amenable to preoperative adjuvant therapy. Those tumors that respond to
preop chemotherapy will make later chest wall resection less morbid and
more feasible. During resection of the tumor the surgeon should open
one to two interspace higher and away from the primary rib involved.
Negative microscopic margins are required for malignant tumors when
feasible. To avoid paradoxical motion of the rib after resection of two
or more ribs, rigid chest reconstruction with mesh is needed.
Methylmethacrylate cement sandwich between tow layers of polypropylene
mesh produces this effect very well. The mesh should extend over the
edge of the rigid part of the sandwich to allow suturing with
non-absorbable material. Complications include recurrence, respiratory
insufficiency, scoliosis and chest wall deformity. In general prognosis
is good.
References:
1- Dang NC, Siegel SE, Phillips JD: Malignant chest wall tumors in
children and young adults. J Pediatry Surg.34(12):1773-8, 1999
2- Wong KS, Hung IJ, Wang CR, Lien R: Thoracic wall lesions in children.Pediatr Pulmonol. 37(3):257-63, 2004
3- Soyer T, Karnak I, Ciftci AO, Senocak ME, Tanyel FC,
Büyükpamukçu N: The results
of surgical treatment of chest wall tumors in childhood. Pediatry Surg
Int.22(2):135-9, 2006
4- van den Berg H, van Rijn RR, Merks JH: Management of tumors of the
chest wall in childhood: a review. J Pediatry Hematol
Oncol.30(3):214-21, 2008
5- La Quaglia MP: Chest wall tumors in childhood and adolescence. Semin Pediatry Surg. 17(3):173-80, 2008
6- Gonfiotti A, Santini PF, Campanacci D, Innocenti M, Ferrarello S,
Caldarella A, Janni A: Malignant primary chest-wall tumours: techniques
of reconstruction and survival. Eur J Cardiothorac Surg. 38(1):39-45,
2010
Pancreatic Abscess
A pancreatic abscess is a localized
collection of purulent material with little or no necrosis in the
region of the pancreas which is delineated by a wall of collagen and
granulation tissue. It is also known as walled-off pancreatic necrosis
(WOPN). In contra position, an infected pancreatic pseudocyst is a
localized collection of infected fluid in the region of the pancreas,
and like an abscess is also walled off by a membrane of collagen and
granulation tissue. Both conditions are seen as complications after
acute pancreatitis. The child develops abdominal pain, fever and
leukocytosis. Blood cultures are positive. The degree of enzyme
elevations does not correlate with the degree of necrosis. The presence
of air in necrotic tissue in a pseudocyst is specific for infection.
The diagnosis is established with contrast enhanced CT-Scan. Management
consists of systemic antibiotics, bowel rest, gastric suction and
enteral nutrition. Primary drainage is the treatment of choice for a
wall-off pancreatic necrosis. Endoscopic ultrasound with transgastric
drainage is another option. WOPN can lead to several complications such
as fistula formation, recurrent pancreatitis, bowel obstruction or
death. Acute surgery may be needed in cases of perforation, major
bleeding or when the child is not responding to therapy.
References:
1- Papachristou GI, Takahashi N, Chahal P, Sarr MG, Baron TH: Peroral
endoscopic drainage/debridement of walled-off pancreatic necrosis. Ann
Surg. 245(6):943-51, 2007
2- Stamatakos M, Stefanaki C, Kontzoglou K, Stergiopoulos S,
Giannopoulos G, Safioleas M: Walled-off pancreatic necrosis. World J
Gastroenterol. 16(14):1707-12, 2010
3- Cheung MT, Li WH, Kwok PC, Hong JK: Cheung MT, Li WH, Kwok PC, Hong
JK. J Hepatobiliary Pancreat Sci. 17(3):338-44, 2010
4- Gardner TB, Coelho-Prabhu N, Gordon SR, Gelrud A, Maple JT,
Papachristou GI, Freeman ML, Topazian MD, Attam R, Mackenzie TA, Baron
TH: Direct endoscopic necrosectomy for the treatment of walled-off
pancreatic necrosis: results from a multicenter U.S.
series.Gastrointest Endosc. 73(4):718-26, 2011
5- Brun A, Agarwal N, Pitchumoni CS: Fluid collections in and around
the pancreas in acute pancreatitis. J Clin Gastroenterol.
45(7):614-25, 2011
6- Gluck M, Ross A, Irani S, Lin O, Hauptmann E, Siegal J, Fotoohi M,
Crane R, Robinson D, Kozarek RA: Endoscopic and percutaneous drainage
of symptomatic walled-off pancreatic necrosis reduces hospital stay and
radiographic resources. Clin Gastroenterol Hepatol. 8(12):1083-8, 2010
Pyogenic Granuloma
Pyogenic granuloma, also known as
lobular capillary hemangioma, is a common benign acquired vascular
lesion of the skin and mucous membranes in the pediatric age group.
Mean age of presentation is six years with most cases being males. Mean
lesional size is 6.5 cm. Pyogenic granulomas are rapidly developing
vascular nodules characterized by an erythematous, dome-shaped papule
that bleeds easily most commonly located in the head and neck area,
followed by trunk, upper extremity and lower extremity. The etiology of
pyogenic granuloma is unknown, but proposed agents include trauma,
infection, and preceding dermatoses. The preponderance of pyogenic
granulomas occurs in the skin (80%), with the remaining ones in the
oral cavity and conjunctiva. Most lesions are effectively managed with
full-thickness skin excision and linear closure. Another alternative is
shave excision followed by laser photocoagulation or cryotherapy.
Topical Imiquimod 5% cream has also been used. Shaving and cream
therapy has a higher recurrence rate.
References:
1- Patrice SJ, Wiss K, Mulliken JB: Pyogenic granuloma (lobular
capillary hemangioma): a clinicopathologic study of 178 cases. Pediatr
Dermatol. 8(4):267-76, 1991
2- Kirschner RE, Low DW: Treatment of pyogenic granuloma by shave
excision and laser photocoagulation. Plast Reconstr Surg.
104(5):1346-9, 1999
3- Pagliai KA, Cohen BA: Pyogenic granuloma in children. Pediatr Dermatol. 21(1):10-3, 2004
4- Lin RL, Janniger CK: Pyogenic granuloma. Cutis. 74(4):229-33, 2004
5- Giblin AV, Clover AJ, Athanassopoulos A, Budny PG: Pyogenic
granuloma - the quest for optimum treatment: audit of treatment of 408
cases. J Plast Reconstr Aesthet Surg.60(9):1030-5, 2007.
6- Tritton SM, Smith S, Wong LC, Zagarella S, Fischer G: Pyogenic
granuloma in ten children treated with topical imiquimod.Pediatr
Dermatol. 26(3):269-72, 2009
PSU Volume 37 NO 06 DECEMBER 2011
Single Site Laparoscopic Surgery
As technological surgical advances
continue to occur in the field of laparoscopic surgery in search of the
scarless operation, single site umbilical laparoscopic procedures have
emerged. A single multi-port with several holes is introduced through
the navel, instead of several small incisions. The working space in
children is small and space is needed for instrument triangulation and
retraction. Nevertheless, single site umbilical laparoscopic surgery is
performed in children for such procedures as cholecystectomy,
appendectomy, intussusception reduction, splenectomy, nephrectomy,
inguinal hernia repair, fundoplication, gastrostomy, orchiopexy
and pyloromyotomy. Due to lack of triangulation roticulating and curved
instruments have been developed for single port procedures. Most
roticulating instruments are disposable and need a learning curve for
safe use. Alternatives to roticulating instruments are rigid bent
instruments which can be reused and are low cost. The shaft of the
telescope comes longer to avoid clashing with the surgeons hand.
Retraction can be achieved with the used of magnets. This is achieved
with magnetic intracorporeal graspers and an extracorporeal magnet that
is manipulated over the abdominal wall to adjust and control the
instruments. Laparoscopic surgery in children is moving toward
achieving this goal of scarless surgery.
References:
1- Woldrich JM, Holmes N, Palazzi-Churas K, Alagiri M, DeCambre M,
Kaplan G, Chiang G: Comparison of laparoendoscopic single-site,
conventional laparoscopic, and open nephrectomy in a pediatric
population. Urology. 78(1):74-7, 2011
2- Garey CL, Laituri CA, Ostlie DJ, Snyder CL, Andrews WS, Holcomb GW
3rd, St Peter SD: Single-incision laparoscopic surgery in
children: initial single-center experience. J Pediatr Surg.
46(5):904-7, 2011
3- Kawauchi A, Naitoh Y, Miki T: Laparoendoscopic single-site surgery
for pediatric patients in urology. Curr Opin Urol. 21(4):303-8, 2011
4- Ostlie DJ: Single-site umbilical laparoscopic appendectomy. Semin Pediatr Surg. 20(4):196-200, 2011
5- Padilla BE, Dominguez G, Millan C, Martinez-Ferro M: The use of
magnets with single-site umbilical laparoscopic surgery. Semin Pediatr
Surg. 20(4):224-31, 2011
6- Krpata DM, Ponsky TA: Instrumentation and equipment for single-site
umbilical laparoscopic surgery. Semin Pediatr Surg 20(4): 190-195, 2011
Acquired Tracheoesophageal Fistula
Acquired tracheoesophageal fistula
(TEF) is a rare clinical disease encountered in the pediatric age. Most
cases are the result of a benign condition. Complications resulting
from such conditions as prolonged mechanical ventilation, indwelling
tracheal or esophageal stents, granulomatous mediastinal infection,
trauma, iatrogenic injuries, ingested foreign body and caustic
ingestion are the common cause for development of an acquired TEF. The
most common cause of acquired TEF in children is foreign body
impaction. Onset of symptoms can be immediate with respiratory
difficulty, subcutaneous air, pneumothorax or mediastinum. It can be
chronic with coughing, choking, respiratory infections or dysphagia.
Increased tracheal secretions, air leak around the tube cuff,
suctioning of gastric contents through the tracheotomy, abdominal
distention, and air leakage from a nasogastric or gastrostomy tube are
among the signs that should suggest the potential diagnosis of TEF.
Diagnostic evaluation should include chest film, water soluble
esophagogram, bronchoscopy and esophagoscopy. Initial management should
consist of stabilization and prevention of airway contamination by the
fistula. This is followed by gastrostomy and jejunostomy to vent and
feed the child respectively. Reducing inflammation, managing pulmonary
infection and improving nutrition can hasten spontaneous closure in a
4-6 week period. Failure of conservative management requires surgical
intervention. This includes cervical or thoracic closure of the TEF
with interposition muscle if necessary. Other larger fistulas will
require more complex procedures.
References:
1- Rahbar A, Farha SJ: Acquired tracheoesophageal fistula. J Pediatr Surg. 1978 Aug;13(4):375-6
2- Szold A, Udassin R, Seror D, Mogle P, Godfrey S: Acquired
tracheoesophageal fistula in infancy and childhood. J Pediatr
Surg. 26(6):672-5, 1991
3- Birman C, Beckenham E: Acquired tracheo-esophageal fistula in the
pediatric population. Int J Pediatr Otorhinolaryngol. 10;44(2):109-13,
1998
4- ImamoÄŸlu M, Cay A, KoÅŸucu P,
AhmetoÄŸlu A, Sarihan H: Acquired tracheo-esophageal fistulas
caused by button battery lodged in the esophagus. Pediatr Surg Int.
20(4):292-4., 2004
5- Grisel JJ, Richter GT, Casper KA, Thompson DM: Acquired
tracheoesophageal fistula following disc-battery ingestion: can we
watch and wait? Int J Pediatr Otorhinolaryngol. 72(5):699-706, 2008
6- Yalcin S, Ciftci AO, Karnak I, et al: Management of acquired
tracheoesophageal fistula with various clinical presentations. J
Pediatr Surg 46(10): 1887-1892, 2011
Prevention CVC Infections
Central venous catheter (CVC) are an
indispensable tool in the management of acute and chronic diseases in
children. The most common complication of use of CVC is bloodstream
infection. Recent evidence-based studies and metaanalysis have found:
1) chlorhexidine skin prep and impregnated dressing can reduce CVC
colonization and bloodstream infection. Chlorhexidine in neonates and
premature infants can cause skin irritation and systemic absorption. 2)
use of heparin and antibiotic impregnated CVC can decrease CVC
colonization and bloodstream infection. 3) Ethanol and Vancomycin lock
therapy can reduce the incidence of CVC infection. Vancomycin-heparin
lock is associated with asymptomatic hypoglycemia. 4) Benefit of
systemic prophylactic antibiotic at the time of CVC insertion is
unclear.5) With regard to site of insertion, subclavian placement has a
reduced incidence of CVC colonization.6) CVC infection in the setting
of short-term use should undergo catheter removal with systemic
antibiotics for 7-14 days. 7) Patient in long-term CVC use who develop
infection with S. Aureus, gram-negative bacilli, Candida, clinical
deterioration or persistent relapsing bacteremia should undergo
immediate catheter removal and defined course of systemic antibiotics,
except in rare circumstances when no alternate venous access is
available.
References:
1- O'grady NP, Alexander M, Dellinger EP, Gerberding JL, Heard SO, Maki
DG, Masur H, McCormick RD, Mermel LA, Pearson ML, Raad II, Randolph A,
Weinstein RA; Healthcare Infection Control Practices Advisory
Committee: Guidelines for the prevention of intravascular
catheter-related infections. Am J Infect Control. 30(8):476-89, 2002
2- Levy I, Katz J, Solter E, Samra Z, Vidne B, Birk E, Ashkenazi S,
Dagan O: Chlorhexidine-impregnated dressing for prevention of
colonization of central venous catheters in infants and children: a
randomized controlled study. Pediatr Infect Dis J. 24(8):676-9, 2005
3- Gilbert RE, Harden M: Effectiveness of impregnated central venous
catheters for catheter related blood stream infection: a
systematic review. Curr Opin Infect Dis. 21(3):235-45, 2008
4- Mouw E, Chessman K, Lesher A, Tagge E: Use of an ethanol lock to
prevent catheter-related infections in children with short bowel
syndrome. J Pediatr Surg. 43(6):1025-9, 2008
5- O'Grady NP, Alexander M, Burns LA, Dellinger EP, Garland J, Heard
SO, Lipsett PA, Masur H, Mermel LA, Pearson ML, Raad II, Randolph AG,
Rupp ME, Saint S; Healthcare Infection Control Practices Advisory
Committee (HICPAC): Guidelines for the prevention of intravascular
catheter-related infections. Clin Infect Dis. 52(9):e162-93, 2011 and
Summary of recommendations: Guidelines for the Prevention of
Intravascular Catheter-related Infections. Clin Infect Dis.
52(9):1087-99, 2011
6- Huang EY, Chen C, Abdullah F, et al: Strategies for the prevention
of central venous catheter infections: an American Pediatric Surgery
Association Outcomes and Clinical Trials Committee systematic review. J
Pediatr Surg 46(10): 2000-2011, 2011