PEDIATRIC SURGERY UPDATE ©
VOLUME 45, 2015
PSU Volume 45 NO 01 JULY 2015
Transanal Endoscopic Microsurgery
Transanal endoscopic microsurgery
(TEM) refers to a minimally invasive technique using an operating
proctoscope, a laparoscopic camera and modified laparoscopic
instruments to accomplish local removal of low level rectal tumors. The
technique is ideal to use to remove anorectal benign adenomas, early
stage rectal carcinomas, carcinoids of the rectum, for palliative
resection of advanced rectal cancers, and surgically correct rectal
prolapse and rectal diverticular disease. Recently TEM has been used to
resect presacral tumors in adults. In children TEM has been utilized to
resect a presacral rectal duplication cyst. The proctoscope is 4 cm in
diameter, has four operating ports, maintains an airtight seal at the
anus, with a port for CO2 inflow and outflow facilitating smoke
evacuation during cauterization. Due to the restricted space to work
the operating instruments are angulated to improve the operator range
of motion. Most patients who undergo TEM experience short hospital
stay, lower rate of postoperative complications and early return to
routine activities even after resection of very large lesions with full
thickness excision. Anorectal function short of causing decreased anal
sphincter pressures and compliance after surgery seldom causes
permanent damage such as fecal incontinence. Compared with preoperative
levels there is no significant change in anal squeeze pressure after
surgery. Tumors larger than 4 cm caused significant decline in rectal
sensitivity, urge to defecate threshold and maximum tolerated volume.
TEM excision of adenomas results in low adenoma recurrence rates due to
higher negative margin rates. Major complications though low include
perforation, bleeding and fistulas. The need for diversion before the
procedure has been questioned since the oncologic or infectious
morbidity associated with opening the peritoneal cavity is low. The use
of TEM in managing more advanced rectal must be tested within formal
clinical trials before adopting as routine practice.
References:
1- Heidary B, Phang TP(1), Raval MJ(1), Brown CJ(1): Transanal endoscopic microsurgery: a review. Can J Surg. 57(2):127-38, 2014
2- Guerrieri M, Gesuita R, Ghiselli R, Lezoche G, Budassi A, Baldarelli
M: Treatment of rectal cancer by transanal endoscopic microsurgery:
experience with 425 patients. World J Gastroenterol.
28;20(28):9556-63, 2014
3- Cunningham C: Transanal endoscopic microsurgery. Recent Results Cancer Res. 203:31-8, 2014
4- Marks JH, Frenkel JL, Greenleaf CE, D'Andrea AP: Transanal
endoscopic microsurgery with entrance into the peritoneal cavity: is it
safe? Dis Colon Rectum. 57(10):1176-82, 2014
5- Mora Lopez L, Serra Aracil X, Hermoso Bosch J, Rebasa P, Navarro
SotoS: Study of anorectal function after transanal endoscopic
surgery. Int J Surg. 13:142-7, 2015
6-Clancy C, Burke JP, Albert MR, O'Connell PR, Winter DC: Transanal
endoscopic microsurgery versus standard transanal excision for the
removal of rectal neoplasms: a systematic review and meta-analysis. Dis
Colon Rectum. 58(2):254-61, 2015
Fitz-Hugh-Curtis Syndrome
Fitz-Hugh-Curtis
syndrome (FHCS) is a rare complication of pelvic inflammatory disease.
It results from ascending pelvic infection causing liver capsule
inflammation and right upper quadrant pain mostly in reproductive-aged
female with sexual activity characterized by violin-string like
perihepatic adhesions between the liver capsule and diaphragm or
anterior peritoneal surface. The incidence ranges from 4% to 27% in
women with pelvic inflammatory disease. FHCS can be seen in 4% of
adolescent females with pelvic inflammatory disease. Predominant
symptoms are pain in the right upper quadrant, tenderness, fever and
pleuritic right-sided pain. Symptoms can be confused with biliary
disease. On physical exam patients can be positive for cervical motion
tenderness and adnexal tenderness. Neisseria gonorrhoeae and Chlamydia
trachomatis are the primary causative agents with this last one as a
more common causative organism. In women, the inflammation of the liver
capsule has been attributed to the direct bacterial spread from an
infected fallopian tube via the right paracolic gutter. In men,
hematogenous and lymphatic spread to liver has been postulated as the
underlying mechanism of spread. CT scan may show subcapsular fluid
collection, thickening of hepatic capsule in the arterial phase, and
wedging enhancement of the involved liver parenchyma in more than 50%
of patients. Definitive diagnosis needs invasive procedure like
laparoscopy or laparotomy. Most cases of FHCS are managed with
antibiotics against the principal offending bacteria. If symptoms
persist then surgical lysis of adhesions should be considered.
Laparoscopy has both diagnostic and therapeutic benefits.
References:
1- Banikarim C, Chacko MR: Pelvic inflammatory disease in adolescents. Adolesc Med Clin. 15(2):273-85, 2004
2- Hyun JJ, Kim JY, Bak YT, Lee CH, Choi SY: Education and imaging.
Gastrointestinal: Fitz-Hugh-Curtis syndrome. J Gastroenterol Hepatol.
21(9):1493, 2006
3- Risser WL, Risser JM, Benjamins LJ, Feldmann JM: Incidence of
Fitz-Hugh-Curtis syndrome in adolescents who have pelvic inflammatory
disease. J Pediatr Adolesc Gynecol. 20(3):179-80, 2007
4- Hong DG, Choi MH, Chong GO, Yi JH, Seong WJ, Lee YS, Park IS, Cho
YL: Fitz-Hugh-Curtis Syndrome: single centre experiences. J
Obstet Gynaecol. 30(3):277-80, 2010
5- Perricone G: Hepatology: Fitz-Hugh-Curtis syndrome: pelvic
inflammatory disease with perihepatitis. J Gastroenterol Hepatol.
29(11):1853, 2014
6- You JS, Kim MJ, Chung HS, Chung YE, Park I, Chung SP, Kim S, Lee HS:
Clinical features of Fitz-Hugh-Curtis Syndrome in the emergency
department. Yonsei Med J. 53(4):753-8, 2012
Spontaneous Pneumomediastinum
Pneumomediastinum
can have diverse etiology in the pediatric population such as chronic
lung disease (asthma), rapid scuba diver ascent, illicit drug use,
foreign body ingestion and blast injury. There is a significant group
of children with spontaneous pneumomediastinum (SPM) and no apparent
etiology. The SPM develops after alveolar rupture following an acute
rise in intrathoracic pressure. Air escapes from the alveoli to the
interstitium and then tracks along the tracheobronchial tree after a
pressure gradient between the lung periphery and mediastinum.
Clinically the child develops chest pain radiating to neck or back,
shortness of breath, hoarseness, coughing or subcutaneous
emphysema. Asthmatics have a higher incidence of spontaneous
pneumomediastinum caused by inspiratory effort against a blocked airway
(Mueller Maneuver). Weight lifting and sport related are some other
instigating factor. Other times inhalation of illicit drugs is a
predisposing factor. In such situation the workup imposed in patients
with SPM has included CT-Scan, esophagogram, bronchoscopy, serial chest
films, etc., when most cases resolve spontaneously without significant
intervention. Most of this cases can be initially managed with a short
period of observation in the emergency department followed by rest
under the care at home of a responsible guardian. Invasive studies or
esophagography should be performed on an individual and not systematic
basis such as history of emesis, dysphagia/odynophagia, radiographic
findings of pleural effusion or atelectasis, etc. The hospital course
is usually benign in cases of SPM and most patients require only
supportive measures. The risk of recurrence is very low except in cases
with chronic lung disease.
References:
1- Lee CY, Wu CC, Lin CY: Etiologies of spontaneous
pneumomediastinum in children of different ages. Pediatr Neonatol.
50(5):190-5, 2009
2- Chen IC, Tseng CM, Hsu JH, Wu JR, Dai ZK: Spontaneous
pneumomediastinum in adolescents and children. Kaohsiung J Med Sci.
26(2):84-8, 2010
3- Lee CY, Wu CC, Lin CY: Etiologies of spontaneous pneumomediastinum
in children in middle Taiwan. Pediatr Pulmonol. 45(9):869-73, 2010
4- Wong KS, Wu HM, Lai SH, Chiu CY: Spontaneous pneumomediastinum:
analysis of 87 pediatric patients. Pediatr Emerg Care. 29(9):988-91,
2013
5- Bakhos CT, Pupovac SS, Ata A, Fantauzzi JP, Fabian T: Spontaneous
pneumomediastinum: an extensive workup is not required. J Am Coll Surg.
219(4):713-7, 2014
6- Fitzwater JW, Silva NN, Knight CG, Malvezzi L, Ramos-Irrizary C,
Burnweit CA: Management of spontaneous pneumomediastinum in children. J
Pediatr Surg. 50(6): 983-986, 2015
PSU Volume 45 NO 02 AUGUST 2015
ACNES
Anterior Cutaneous Nerve Entrapment
Syndrome (ACNES) is a recent identified cause of chronic abdominal pain
in children and adults. The superficial end branches of intercostal
thoracic nerves twigs are somehow trapped at the level of the rectus
abdominal muscle leading to pain, and in a few occasions
vomiting. The exact pathophysiology of ACNES is unknown, but is
probably related to traction or compression of the anterior portions of
intercostal thoracic nerves, usually involving intercostal thoracic
nerve VIII to XII. Pregnancy, abdominal trauma, and chronic nerve root
compression caused by thoracolumbar or orthopedic conditions are
occasionally identified as predisposing factors. Diagnosis of ACNES is
suspected by a constant nagging localized abdominal pain that increases
during play or sport associated with a positive Carnett sign. Carnett
test is performed as follows: the physician localizes the point of
maximal pain with his index finger, and the child is then asked to lift
the head or upper torso or the legs while the palpating index finger
remains on the painful spot. If this lifting aggravates the pain, its
origin is probably located in the abdominal wall. Hypoesthesia,
hyperesthesia/ algesia or sometimes even allodynia may be found by
comparison to the normal contralateral abdominal side. A positive pinch
test is a sensitive and highly underrated sign reflecting the presence
of a neuropathic pain syndrome such as ACNES. Attenuating the pain
using trigger point local anesthetic infiltration support the
diagnosis. These children undergo extensive laboratory and imaging
workup which are usually negative and expensive. Management
consists of pain medication. If pain persists, US-guided transverse
abdominis plane technique blocking the entrapped nerve using subfascial
lidocaine with steroid infiltration is utilized. With recurrent or
recalcitrant pain an anterior cutaneous neurectomy will be needed. The
neurectomy removes the affected nerve end twigs. This type of surgical
procedure is highly successful in children and adults.
References:
1- Scheltinga MR, Boelens OB, Tjon A Ten WE, Roumen RM: Surgery
for refractory anterior cutaneous nerve entrapment syndrome (ACNES) in
children. J Pediatr Surg. 46(4):699-703, 2011
2- Boelens OB, Scheltinga MR, Houterman S, Roumen RM: Management of
anterior cutaneous nerve entrapment syndrome in a cohort of 139
patients. Ann Surg. 254(6):1054-8, 2011
3- Boelens OB, van Assen T, Houterman S, Scheltinga MR, Roumen RM: A
double-blind, randomized, controlled trial on surgery for chronic
abdominal pain due to anterior cutaneous nerve entrapment syndrome. Ann
Surg. 257(5):845-9, 2013
4- Akhnikh S, de Korte N, de Winter P: Anterior cutaneous nerve
entrapment syndrome (ACNES): the forgotten diagnosis. Eur J Pediatr.
173(4):445-9, 2014
5- Nizamuddin SL, Koury KM, Lau ME, Watt LD, Gulur P: Use of targeted
transversus abdominus plane blocks in pediatric patients with anterior
cutaneous nerve entrapment syndrome. Pain Physician. 17(5):E623-7, 2014
6- Bairdain S, Dinakar P, Mooney DP: Anterior Cutaneous Nerve
Entrapment Syndrome in Children. J Pediatr Surg 50(7): 1177-1179, 2015
POEM
Achalasia is a rare motility disorder
of the esophagus due to absence of peristalsis in the esophageal body
impairing relaxation of the lower esophageal sphincter during
swallowing. The etiology of achalasia is likely to be affected by
various factors causing immune-mediated ganglionitis which results in
degeneration of the myenteric nerve plexus of the esophageal wall.
Achalasia causes regurgitation, dysphagia to solids, retrosternal pain
and weight loss. The diagnosis of achalasia is first suggested in a
barium swallow and corroborated using esophageal manometry studies.
Achalasia is either managed by esophageal balloon dilatation and most
effectively with either open or laparoscopic anterior surgical
esophageal myotomy. Peroral endoscopic myotomy (POEM) is a novel
procedure developed recently which has ameliorated the symptoms of
patients with achalasia. The endoscopic procedure, performed in the
operating room under general anesthesia, consists of entry incision on
the mucosa at the level of the gastroesophageal junction, establishing
a tunnel on the submucosa, performing the myotomy followed by sealing
of the entry incision. The myotomy is primary performed for the
circular muscle fibers or both the circular and longitudinal muscle
fibers. Leak through the mucosal seal can cause peritonitis and/or
mediastinitis. Compared with the surgical approach to achalasia, POEM
has the advantage of minimally invasiveness. POEM can significantly
improve esophageal motility by decreasing upper esophageal sphincter
and lowering esophageal sphincter pressure. POEM has no effect on
esophageal body peristalsis. After POEM incomplete myotomy and
gastroesophageal reflux can become a problem. The role of POEM in the
treatment of other esophageal motor disorders such as diffuse
esophageal spasm, non-relaxing hypertensive LES and nutcracker
esophagus is still debatable. POEM is a safe and effective technique
for treating pediatric achalasia.
References:
1- Allaix ME, Patti MG: Endoscopic Dilatation, Heller Myotomy, and
Peroral Endoscopic Myotomy: Treatment Modalities for Achalasia.
Surg Clin North Am. 95(3):567-578, 2015
2- Zhang Y, Ling-hu E, Zhai Y, Peng L, Wang X: Peroral endoscopic
myotomy plus balloon shaping for achalasia: a preliminary study.
Hepatogastroenterology. 62(137):82-6, 2015
3- Tang X, Gong W, Deng Z, Zhou J, Ren Y, Zhang Q, Chen Z, Jiang B:
Usefulness of peroral endoscopic myotomy for treating achalasia in
children: experience from a single center. Pediatr Surg Int.
31(7):633-8, 2015
4- Kumbhari V, Khashab MA: Peroral endoscopic myotomy. World J Gastrointest Endosc. 7(5):496-509, 2015
5- Yao S, Linghu E: Peroral endoscopic myotomy can improve esophageal motility in patients with
achalasia from a large sample self-control research (66 patients). PLoS One. 10(5):e0125942, 2015
6- Ramchandani M, Reddy DN, Darisetty S, Kotla R, Chavan R, Kalpala R,
Galasso D, Lakhtakia S, Rao GV: Peroral endoscopic myotomy for
Achalasia Cardia: A single center experience of over 200 consecutive
patients: treatment analysis and follow up. Dig Endosc. May 27, 2015
Lymphorrhea
Lymphorrhea is an abnormal flow of
lymph draining externally from disrupted lymphatics vessels or
previously retained within a wound. In children lymphorrhea usually
occurs following a surgical procedure for a lymphatic anomaly,
following cardiothoracic surgery, in penetrating trauma to the neck or
during removal of lymph nodes for diagnostic or therapeutic
purposes. Complications of uncontrolled lymphorrhea can lead to
subcutaneous collection of lymphatic fluid or a draining fistula. High
output lymphatic leakage is associated with increased mortality when
intervention is not initiated in a timely fashion due to critical loss
of fluids, proteins and electrolytes causing lymphocyte and antibody
depletion Most cases of lymphorrhea in children occur after surgery for
lymphangioma or cystic hygroma, a benign condition of the lymphatic
system consisting of cysts of varying size. Initial management of
lymphorrhea consists of diet modification, drainage, pressure
dressings, and reoperation to stop lymphatic drainage. Octeotride, a
somatostatin analog has been used successfully to manage port-operative
lymphorrhea in children after removal of large lymphangioma in the
cervical and axillary region. Midodrine, an oral selective
alpha-adrenergic drug, can affect contraction of smooth muscle and
decrease lymphatic flow. Sclerotherapy using either doxycycline,
ethanol, bleomycin or minocycline has also been successfully used to
manage lymphatic leakage. Surgical exploration to occlude lymphatic
vessels with the use of fibrin sealant has also produce benefit
in selected patients.
References:
1- Suver DW, Perkins JA, Manning SC: Somatostatin treatment of massive
lymphorrhea following excision of a lymphatic malformation.
International J Pediatr Otorhinolaryngology. 68: 845-850, 2004
2- Carcoforo P, Soliani G, Maestroni U: Octeotride in the Treatment of
Lymphorrhea after Axillary Node dissection. A prospective randomize
controlled trial.J Am Coll Surg. 196:365–369, 2003
3- Lemaitre J, Segers B, Lebrun E: [The use of lanreotide in the
management of lymphorrhea after an aortic valve replacement].
Interactive Cardiovascular and Thoracic Surgery 15:762-763, 2012
4- Friedmann MD: Evaluation and Management of Lymhadenopathy in Children. Pediatrics in Review. 29(2): 53-60, 2008
5- Miloundja J, Manfoumbi Ngoma AB, Mba Ella R, Nguema Edzang B,
N'Zouba L: [Cystic cervicofacial lymphangioma in children in Gabon].
Ann Otolaryngol Chir Cervicofac. 124(6):277-84, 2007
6- Cuervo JL, Galli E, Eisele G, Johannes E, Fainboim A, Tonini S,
Joaquin W, Viola B, Nazar M: [Lymphatic malformations:
percutaneus treatment with bleomycin]. Arch Argent Pediatr.
109(5):417-22, 2011
PSU Volume 45 No 03 SEPTEMBER 2015
Subcutaneous Zygomycosis
Subcutaneous zygomycosis, also known
as Entomophthoromycosis, is a rare, sporadic subcutaneous fungal
infection largely restricted to tropical areas of Africa, Asia and
South America. Two clinically distinct form can be found: subcutaneous
zygomycosis, caused by Basidiobolus ranarum and rhinofacial zygomycosis
caused by Conidiobolus coronatus. Neither of these two forms occurs
preferentially in patients with underlying disease nor defective
immunity. These fungi are used as bio-insecticides. Subcutaneous
zygomycosis is characterized by the formation of firm and non-tender
disciform nodule generally appearing on the extremity and trunk mostly
in children. The nodule can enlarge spreading locally. Skin is usually
tethered to the mass and it may be pigmented. Males are more much
frequently affected than females. The mode of infection is by traumatic
implantation through abrasion or pricks. The infection can also be
transmitted by insect bites or by transepidermal inoculation with
contaminated vegetable matter. Subcutaneous zygomycosis can mimic a
soft tissue tumor such as synovial sarcoma or Burkitt's lymphoma.
Histologically an eosinophilic granulation infiltration is found with
broad thin walled infrequently septate hyphal fragments enveloped by
eosinophilic "Splendore-Hoeppli" material. Diagnosis of subcutaneous
zygomycosis is accepted if the fungus is demonstrated in either
histology or culture. Role of surgery in subcutaneous zygomycosis is
limited to diagnostic biopsy. Excision leads to recurrence. The
standard treatment of choice for subcutaneous zygomycosis is oral
potassium iodide or imidazoles in particular itraconazole, ketaconazole
and posaconazole.
References:
1- Sivaraman, Thappa DM, Karthikeyan, Hemanthkumar: Subcutaneous
phycomycosis mimicking synovial sarcoma. Int J Dermatol. 38(12):920-3,
1999
2- Mahamaytakit N, Singalavanija S, Limpongsanurak W: Subcutaneous
zygomycosis in children: 2 case reports. J Med Assoc Thai. 97: Suppl
6:S248-53, 2014
3- Gugnani HC: A review of zygomycosis due to Basidiobolus ranarum. Eur J Epidemiol. 15(10):923-9, 1999
4- Anand M, Deshmukh SD, Pande DP, Naik S, Ghadage DP: Subcutaneous
Zygomycosis Due to Basidiobolus ranarum: A Case Report from Maharastra,
India. J Trop Med. 2010:950390, 2010
5- Thotan SP, Kumar V, Gupta A, Mallya A, Rao S: Subcutaneous
phycomycosis--fungal infection mimicking a soft tissue tumor: a case
report and review of literature. J Trop Pediatr. 56(1):65-6, 2010
6- Raveenthiran V, Mangayarkarasi V, Kousalya M, Viswanathan P,
Dhanalakshmi M, Anandi V: Subcutaneous entomophthoromycosis mimicking
soft-tissue sarcoma in children. J Pediatr Surg. 50(7):1150-5, 2015
Hypertrophic Scar
Hypertrophic scar (HTS) is a benign
hyperproliferative growth of dermal fibroblasts. They occur most
commonly after thermal injury to the skin caused by burns and trauma,
but can also be seen after elective surgical procedures. Almost 15% of
all wounds can develop into a hypertrophic scar. Children with
hypertrophic scars experience marked physical (deformity, restricted
range of motion, pain and pruritus) and psychological (cosmetic
concern) problems. Fibroblasts from hypertrophic scars produce increase
amounts of collagen when compared with normal fibroblasts. The
undesirable physical properties of HTS tissue can be attributed to the
presence of a large amount of extracellular matrix that is of altered
composition and organization, compared to normal dermis or mature scar.
This matrix is the product of a dense population of fibroblasts,
maintained in a hyperactive state by inflammatory cytokines such as
TGF-b and other factors, some of which may be physical in origin. Most
HTS are accepted by the patient so long as they are asymptomatic.
Management of HTS can range from conservative to excision to
intralesional injection therapy or a combination of these. The most
commonly used therapeutic method for HTS and keloids consist of the
steroid triamcinolone acetonide (Kenalog) wound injection. The dosage
varies from 10-40 mg/ml with a treatment interval administered every
4-6 weeks until the scar flattens. Kenalog can suppress vascular
endothelial growth factor, inhibit fibroblast proliferation, inhibit
transforming growth factor and induce scar regression. Varying the
dosage can provide good results with less recurrence and complications.
Results can be improved and scar recurrence reduced when triamcinolone
is combined with other therapies such as 5-Fluorouracil (best results
in terms of rapid response and fewer side-effects), surgery, pulsed-dye
laser, and radiation therapy.
References:
1- Williams FN, Herndon DN, Branski LK: Where we stand with human
hypertrophic and keloid scar models. Exp Dermatol. 23(11):811-2, 2014
2- Wang J, Dodd C, Shankowsky HA, Scott PG, Tredget EE; Wound Healing
Research Group: Deep dermal fibroblasts contribute to hypertrophic
scarring. Lab Invest. 88(12):1278-90, 2008
3- Darougheh A(1), Asilian A, Shariati F: Intralesional triamcinolone
alone or in combination with 5-fluorouracil for the treatment of keloid
and hypertrophic scars. Clin Exp Dermatol. 34(2):219-23, 2009
4- Longaker MT: Regenerative medicine: a surgeon's perspective. J Pediatr Surg. 45(1):11-7, 2010
5- Hayashi T, Furukawa H, Oyama A, Funayama E, Saito A, Murao N,
Yamamoto Y: A new uniform protocol of combined corticosteroid
injections and ointment application reduces recurrence rates after
surgical keloid/hypertrophic scar excision. Dermatol Surg. 38(6):893-7,
2012
6- Perdanasari AT, Lazzeri D, Su W, et al: Recent Development in the
use of Intralesional Injections Keloid Treatment. Archives Plast Surg
41(6): 620-629, 2014
Annular Pancreas: Late Presentation
Annular pancreas (AP) is a congenital
anomaly occurring when a ring of pancreatic tissue encircles the
duodenum partially or completely. Infants born with this anomaly can
develop duodenal obstruction, stenosis, peptic ulcer, obstructive
jaundice, pancreatitis or pancreatic malignancy. Others remain
asymptomatic until later in life. Pathogenesis includes hypertrophy of
both the ventral and dorsal anlage, adhesion of a portion of the
ventral anlage to the duodenum before migration, or fusion of aberrant
pancreatic tissue from the duodenum. The annulus is usually comprised
of a band of pancreatic tissue, which encircles the second portion of
the duodenum. The annulus itself can be complete, partial, intramural,
or extramural. AP can be classified according to where the annular duct
communicates with Wirsung's (Type 1; most common), common bile duct,
papilla or Santorini. Around 50% of all annular pancreas are diagnosed
in infancy due to complete duodenal obstruction. Later in life the
older child or adult develop chronic partial duodenal obstruction
associated with abdominal pain, nausea and postprandial vomiting.
Diagnosis relies on images. UGIS might depict an incomplete duodenal
obstruction. CT-Scan shows that pancreatic tissue encircles the second
portion of the duodenum. ERCP can show the classic features of a
pancreatic duct system encircling the duodenum. MRCP is the best non
invasive study showing the aberrant pancreatic duct circling and
extending to the right of the duodenum. Intraoperative diagnosis
remains the best diagnostic modality for annular pancreas in any age
group. Annular pancreas with duodenal obstruction should be managed
with a duodenal physiological bypass procedure such as
duodenoduodenostomy or duodenojejunostomy depending on the individual
case. Gastrojejunostomy is an alternative option in case of grossly
fibrotic duodenal C-loop, but can lead to marginal ulcers. Local
resection of the annular segment is avoided because of the fear of
development of pancreatic fistula and pancreatitis.
References:
1- Choi J, Kim MJ, Kim JH, Lim JS, Oh YT, Chung JJ, Song SY, Chung JB,
Yoo HS, Lee JT, Kim KW: Annular pancreas: emphasis on magnetic
resonance cholangiopancreatography findings. J Comput Assist Tomogr.
28(4):528-32, 2004
2- Fu PF, Yu JR, Liu XS, Shen QY, Zheng SS: Symptomatic adult annular
pancreas: report of two cases and a review of the literature.
Hepatobiliary Pancreat Dis Int. 4(3):468-71, 2005
3- Zyromski NJ, Sandoval JA, Pitt HA, Ladd AP, Fogel EL, Mattar WE,
Sandrasegaran K, Amrhein DW, Rescorla FJ, Howard TJ, Lillemoe KD,
Grosfeld JL: Annular pancreas: dramatic differences between children
and adults. J Am Coll Surg. 206(5):1019-25, 2008
4- Zilberstein B, Sorbello MP, Orso IR, Cecconello I: Laparoscopic
duodenal-jejunal bypass for the treatment of duodenal obstruction
caused by annular pancreas: description of a surgical technique. Surg
Laparosc Endosc Percutan Tech. 21(2):e60-4, 2011
5- Badiola I, Las Navas E, Sanz A, Estraviz B, Ibanez FJ: Laparoscopic
side-to-side duodenoduodenostomy for adult annular pancreas. Cir Esp.
92(9):630-1, 2014
6- Alahmadi R, Almuhammadi S: Annular pancreas: a cause of gastric
outlet obstruction in a 20-year-old patient. Am J Case Rep.15:437-40,
2014
PSU Volume 45 NO 04 OCTOBER 2015
Duodenal Web
Intrinsic congenital obstruction of
the duodenum is either caused by an atresia, stenosis or web with a
prevalence of 1:6000 live births. Congenital duodenal obstruction is
the result of several embryologic defects in foregut development,
canalization or rotation. Duodenal atresia causes a complete
obstruction producing symptoms after birth with evident radiographic
findings, while true stenosis and webs of the duodenum can have delayed
presentation manifesting later in life. The radiographic signs of
duodenal atresia are the "double bubble" with gaseous distension of the
stomach and proximal duodenum and total absence of intestinal gas
distally. Duodenal webs are the most rare and difficult to diagnose of
the intrinsic obstruction occurring in this portion of the
gastrointestinal tract. They manifest with recurring and progressive
bouts of vomiting, nausea, epigastric discomfort and early satiety.
Plain film shows a double bubble effect with distal air. The
differential diagnosis includes duodenal stenosis, malrotation with
midgut volvulus, pyloric stenosis and gastroesophageal reflux. The
diagnosis of a duodenal web can be made with fiberoptic upper
endoscopy, hypotonic duodenography or an upper gastrointestinal
contrast imaging study. Initial management of any form of intestinal
obstruction must include gastric decompression, fluid resuscitation and
correction of electrolyte abnormalities, most commonly hypochloremic
metabolic alkalosis. There are several varieties of webs: complete
duodenal atresias or imperforate webs, intraluminal imperforate webs
(wind sock webs), and perforated webs with either central or eccentric
apertures. Most duodenal webs are preampullary with a central single
aperture and size ranging from 0.5 to 20 mm. Annular pancreas is the
most common associated condition of a duodenal web. Surgical management
of a duodenal web includes transduodenal web excision with transverse
duodenoplasty or bypass duodenoduodenostomy. Complications of the
procedure include delayed gastric emptying, pancreatitis, wound
infection and duodenal stenosis or leak.
References:
1- Rothenberg SS: Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and
children. J Pediatr Surg. 37(7):1088-9, 2002
2- Melek M, Edirne YE: Two cases of duodenal obstruction due to a congenital web. World J Gastroenterol. 14(8):1305-7, 2008
3- Mahomed A, D'hondt B, Khan K, Butt A: Technical aspects of the
laparoscopic management of a late presenting duodenal web. J
Laparoendosc Adv Surg Tech A. Suppl 1:S175-7, 2009
4-Kshirsagar AY, Sulhyan SR, Vasisth G, Nikam YP: Duodenal stenosis in a child. Afr J Paediatr Surg. 8(1):92-4, 2011
5- Sarin YK, Sharma A, Sinha S, Deshpande VP: Duodenal webs: an experience with 18 patients. J Neonatal Surg. 1(2):20, 2012
6- DiMaio CJ, Kamal N, Hogan CM, Midulla PS: Pediatric therapeutic
endoscopy: endoscopic management of a congenital duodenal web.
Gastrointest Endosc. 80(1):166-7, 2014
Familial Hirschsprung's Disease
A small proportion, probably in the
range between 2% and 9% of children born with Hirschsprung's disease
(HD), have an inherited familial predisposition, as well as occurring
in mono- and dizygotic twins, and a 12% association with chromosomal
anomalies. This subgroup of children with familial HD is usually male
with an apparent incomplete gene penetrance and variable phenotype.
Affected families with HD have 200 times higher risk of
recurrence. The length of the aganglionic segment is one of the most
consistent predictors of HD transmissibility. Long segment and total
colonic aganglionosis is significantly more frequent in familial HD
cases than in the sporadic variety carrying the highest risk of
recurrence. There is an increase female tendency to transmit the
condition to the offspring. The highest recurrence rate occurs in a
male sibling of a female proband with long segment HD. RET gene is the
major gene causing Hirschsprung's disease (HD). RET proto-oncogene and
EDNRB gene variation are identified in 70% of familial HD compared with
30% in the sporadic form. No specific sites on the gene are
consistently identified with variations including mutations, frame
shifts, deletions, and single nucleotide polymorphism in the RET gene.
The combined cumulative effects of the susceptibility loci of RET and
EDNRB genes, probably contribute to long-segment and total colonic
aganglionosis in familial cases. RET intronic variations may influence
gene penetration. Genetic counseling should be offered in these
families and in particular for those patients with long segment and
total colonic aganglionosis.
References:
1- Schiller M, Levy P, Shawa RA, Abu-Dalu K, Gorenstein A, Katz S:
Familial Hirschsprung's disease--a report of 22 affected siblings in
four families. J Pediatr Surg. 25(3):322-5, 1990
2- Russell MB, Russell CA, Fenger K, Niebuhr E: Familial occurrence of Hirschsprung's disease. Clin Genet. 45(5):231-5, 1994
3- Moore SW, Zaahl MG: A review of genetic mutation in familial
Hirschsprung's disease in South Africa: towards genetic counseling. J
Pediatr Surg. 43(2):325-9, 2008
4-Mc Laughlin D, Puri P: Familial hirschsprung's disease: a systematic review. Pediatr Surg Int. 31(8):695-700, 2015
5- Tomuschat C, Puri P: RET gene is a major risk factor for Hirschsprung's disease: a meta-analysis.
Pediatr Surg Int. 31(8):701-10, 2015
6- Moore SW, Zaahl M: Clinical and genetic correlations of familial Hirschsprung's disease. J Pediatr Surg. 50(2):285-8, 2015
Sengstaken-Blakemore Tube
In the 1946, Blakemore and Sengstaken
developed a tube with two balloons to control bleeding from esophageal
or gastric varices. The tube allows independent inflation of the
gastric and esophageal balloon with a distal lumen to aspirate the
stomach. With the routine use of modern endoscopic technique the
Sengstaken-Blakemore tube (SBT) is rarely use today. The tube is passed
down into the esophagus and the gastric balloon is inflated inside the
stomach. Traction is applied to the tube so that the gastric balloon
will compress the gastroesophageal junction and reduce the blood flow
to esophageal or gastric varices. If the use of traction alone cannot
stop the bleeding, the esophageal balloon is also inflated to help stop
the bleeding. The esophageal balloon should not remain inflated for
more than six hours to avoid necrosis. Cooling the SBT has been held to
stiffen it and aid during insertion. In a few children bleeding is so
severe that medical (octreotide, vasopressin) and endoscopic
(sclerotherapy and/or banding) interventions are not feasible, safe or
effective and the SBT is required to control blood loss and allow the
patient to be resuscitated and stabilized. The SBT can be used
effectively in children with bleeding esophagogastric varices from
portal hypertension and in the rare case of an aortoesophageal fistula.
Hemodynamically unstable patients requiring intubation ventilator
support and large volumes of fluids and blood products for catastrophic
upper GI bleeding considered unsuitable for first line management
should undergo SBT insertion. Almost half of those patients are infants
needing a 14 or 16 Fr SBT. In cases of bleeding aortoesophageal fistula
the SBT can be lifesaving providing suitable time for surgical repair.
Complications of SBT insertion include: recurrent bleeding, tube
dislodgement, esophageal rupture, airway compromise, external cardiac
compression, aspiration pneumonitis, gastric mucosal ulceration and
pressure necrosis at lips and cheeks. Pressure necrosis occurs after
more than four days of constant use of the SBT.
References:
1- Jennuvat S, Vithayasai N: Hepatocellular carcinoma in children
presents with massive upper gastrointestinal bleeding: a case report. J
Med Assoc Thai. 94 Suppl 3:S222-5, 2011
2- Pinto-Marques P, Romaozinho JM, Ferreira M, Amaro P, Freitas D:
Esophageal perforation--associated risk with balloon tamponade after
endoscopic therapy. Myth or reality? Hepatogastroenterology.
53(70):536-9, 2006
3- Coates LJ, McNally J, Caputo M, Cusick E: Survival in a 2-year-old
boy with hemorrhage secondary to an aortoesophageal fistula. J Pediatr
Surg. 46(12):2394-6, 2011
4- Singhi S, Jain P, Jayashree M, Lal S: Approach to a child with upper
gastrointestinal bleeding. Indian J Pediatr. 80(4):326-33, 2013
5- Feng AC, Liao CY, Fan HL, Chen TW, Hsieh C: The modified Sugiura
procedure as bridge surgery for liver transplantation: a case report. J
Med Case Rep. 9:50, 2015
6- Jayakumar S, Odulaja A, Patel S, Davenport M, Ade-Ajayi N: Surviving Sengstaken. J Pediatr Surg. 50(7):1142-6, 2015
PSU Volume 45 NO 05 NOVEMBER 2015
Macrophage Activation Syndrome
Macrophage activation syndrome (MAS)
is a severe, potentially fatal condition caused by excessive activation
and development of macrophages and T-cells (mainly CD8+) leading to an
overwhelming inflammatory reaction in the host (cytokine storm). The
main clinical manifestation of macrophage activation syndrome includes
fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, liver
dysfunction and coagulopathy consistent with disseminated intravascular
coagulation. MAS is also associated with extreme hyperferritinemia. A
pathognomonic feature of MAS is the expansion of well-differentiated
macrophages exhibiting hemophagocytic activity typically found in bone
marrow and lymph nodes. MAS is most strongly associated with the
systemic form of juvenile idiopathic arthritis, but it can also occur
in patients with systemic lupus erythematosus, Kawasaki disease
juvenile dermatomyositis, antiphospholipid syndrome and mixed
connective tissue disease. Diagnosis of MAS is difficult due to its
resemblance to sepsis. In a child with persistently active underlying
rheumatologic disease, a fall in the ESR and platelet count,
particularly in a combination with persistently high CRP and increasing
levels of serum D-dimer and ferritin, should raise a suspicion of
impending MAS. Diagnosis of MAS is usually confirmed by the
demonstration of hemophagocytosis in the bone marrow. Treatment options
include a pro-apoptotic chemotherapy. Patients with evidence of
continued or progressive central nervous system involvement after two
weeks of systemic therapy require intrathecal therapy with methotrexate
combined with corticosteroid induction followed by hematopoietic stem
cell transplantation. The role of the surgeon in this rare condition
includes central venous catheter placement and lymph node biopsy as
warranted for the treatment and diagnosis of MAS.
References:
1- Janka GE: Hemophagocytic syndromes. Blood Rev. 21(5):245-53, 2007
2- Grom AA, Mellins ED: Macrophage activation syndrome: advances
towards understanding pathogenesis. Curr Opin Rheumatol. 22(5):561-6,
2010
3- Lin CI, Yu HH, Lee JH, Wang LC, Lin YT, Yang YH, Chiang BL: Clinical
analysis of macrophage activation syndrome in pediatric patients with
autoimmune diseases. Clin Rheumatol. 31(8):1223-30, 2012
4- Schulert GS, Grom AA: Macrophage activation syndrome and
cytokine-directed therapies. Best Pract Res Clin Rheumatol.
28(2):277-92, 2014
5- Lehmberg K, Pink I, Eulenburg C, Beutel K, Maul-Pavicic A, Janka G:
Differentiating macrophage activation syndrome in systemic juvenile
idiopathic arthritis from other forms of hemophagocytica
lymphohistiocytosis. J Pediatr. 162(6):1245-51, 2013
6- Schulert GS, Grom AA: Pathogenesis of macrophage activation syndrome
and potential for cytokine- directed therapies. Annu Rev Med.
66:145-59, 2015
MeSS Score
Well-differentiated thyroid carcinoma
is the third most common solid malignancy in children with a 2% rise in
a year incidence. It is more commonly seen in females in their
adolescent years with the papillary histologic variant and more than
40% of cases having cervical node disease upon presentation. Surgery
(total thyroidectomy) is the mainstay method of management producing
along with the use of postoperative radioiodine therapy an excellent
long term survival in most cases. Many clinical variables have been
utilized to predict prognosis with varying amounts of scientific
certainty such as histological tumor type, age, gender, extent of
primary-site disease, presence or absence of nodal disease, extent of
thyroidectomy, and the use of radioactive iodine ablation. Several
studies have found male gender, increasing primary disease site
extension, and follicular histologic subtype all had negative
prognostic influence on overall survival. The adult prognostic scales
of thyroid carcinoma utilized such as AGES (age, grade, extent of
disease, size), AMES (age, metastasis, extent of disease, size), MACIS
(metastasis, age at presentation, completeness of surgical resection,
invasion, size), and TNM (tumor, node, metastasis) are not
reliable in children. Instead recent evidence has found in children
that the presence of distant metastasis, tumor size and gender are
independent predictors of mortality at the time of diagnosis. Based on
these findings, a final prognostic scale was created and defined as
distant metastasis (Me), larger primary tumor size (S), and male sex
(S) (MeSS) = +5 (if distant metastasis present), +2 (if primary tumor
size > 4 cm), and +3 (if male gender). MeSS score < 2 has a 0%
mortality, 2-7 moderate risk (2.7% mortality) and >7 has high risk
(23% mortality).
References:
1- Hogan AR, Zhuge Y, Perez EA, Koniaris LG, Lew JI, Sola JE: Pediatric
thyroid carcinoma: incidence and outcomes in 1753 patients. J Surg Res.
156(1):167-72, 2009
2- Haigh PI, Urbach DR, Rotstein LE: AMES prognostic index and extent
of thyroidectomy for well-differentiated thyroid cancer in the United
States. Surgery. 136(3):609-16, 2004
3- Cushing SL, Palme CE, Audet N, Eski S, Walfish PG, Freeman JL:
Prognostic factors in well-differentiated thyroid carcinoma.
Laryngoscope. 114(12):2110-5, 2004
4- Shapiro NL, Bhattacharyya N: Population-based outcomes for pediatric thyroid carcinoma. Laryngoscope. 115(2):337-40, 2005
5- Shayota BJ, Pawar SC, Chamberlain RS: MeSS: A novel prognostic scale
specific for pediatric well-differentiated thyroid cancer: a
population-based, SEER outcomes study. Surgery. 154(3):429-35, 2013
6- Palme CE, Waseem Z, Raza SN, Eski S, Walfish P, Freeman JL:
Management and outcome of recurrent well-differentiated thyroid
carcinoma. Arch Otolaryngol Head Neck Surg. 130(7):819-24, 2004
Lymphoplasmacytic Sclerosing Pancreatitis
Lymphoplasmacytic sclerosing
pancreatitis (LPSP) is a very rare cause of chronic pancreatitis
occurring in the pediatric age group that can mimic a malignant
pancreatic lesion. LPSP is an autoimmune from of chronic pancreatitis
found most commonly in elderly men. It occurs in the absence of
gallstone pancreatitis, pancreas divisum, or excess alcohol ingestion.
It is a diffuse fibrosing process of the pancreas considered an
autoimmune condition associated with inflammatory bowel disease,
Sjögren syndrome, primary biliary cirrhosis and atopic conditions.
It has been proposed that an autoimmune mechanism against carbonic
anhydrase II or lactoferrin and a Th1-type immune response may be
involved in LPSP. Histologically it can be classic or intermediate in
appearance. Classic LPSP has lymphoplasmacytic infiltration of the
pancreas, interstitial fibrosis, periductal inflammation, and
periphlebitis, while intermediate LPSP includes patients with at least
two of these histological findings. Clinically the patient with LPSP
presents with weight loss, jaundice, abdominal pain, elevated CEA and
CA-19 levels similar to patients with pancreatic cancer. On imaging
(CT, MRI, ERCP) the mass is usually in the head of the pancreas with a
more diffuse appearance and irregular narrowing of the pancreatic dust
as compared to the more discrete form of mass of an adenocarcinoma.
LPSP produces high serum levels of IgG4, a rare gamma globulin
subclass, that is not elevated in pancreatic cancer. FNA biopsy of the
lesion can bring conflicting results. FNA histopathological
examinations of the pancreas show fibrosis and pronounced infiltration
of cells, mainly lymphocytes and plasmacytes. Initial management of
LPSP should consist of systemic steroid therapy which can cause
remission of the mass to normal level when follow-up with imaging
studies in most cases.
References:
1- Okazaki K, Uchida K, Matsushita M, Takaoka M: How to diagnose
autoimmune pancreatitis by the revised Japanese clinical criteria. J
Gastroenterol. 42 Suppl 18:32-8, 2007
2- Klimstra DS, Adsay NV: Lymphoplasmacytic sclerosing (autoimmune) pancreatitis. Semin Diagn Pathol. 21(4):237-46, 2004
3- Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, Abraham SC, Yeo CJ,
Lillemoe KD, Choti MA, Campbell KA, Schulick RD, Hruban RH, Cameron JL,
Leach SD: Results of pancreaticoduodenectomy for lymphoplasmacytic
sclerosing pancreatitis. Ann Surg. 237(6):853-8, 2003
4- de Castro SM, de Nes LC, Nio CY, Velseboer DC, ten Kate FJ, Busch
OR, van Gulik TM, Gouma DJ: Incidence and characteristics of chronic
and lymphoplasmacytic sclerosing pancreatitis in patients scheduled to
undergo a pancreatoduodenectomy. HPB (Oxford). 12(1):15-21, 2010
5- Wang G, Zhu H, Yuan CX, Gao Y, Li J, Xue DB, Sun B:
Lymphoplasmacytic sclerosing pancreatitis with obstructive jaundice: a
case report and review of the literature. Onkologie. 32(8-9):506-8, 2009
6- Bartholomew SV, Zigman A, Sheppard B: Lymphoplasmacytic sclerosing
pancreatitis presenting as a pancreatic head mass in a child: case
report and management recommendations. J Pediatr Surg. 41(5):e23-5, 2006
PSU Volume 45 No 06 DECEMBER 2015
Hair Tourniquet Syndrome
Hair tourniquet syndrome (HTS) refers
to hair or other thread fiber becoming tightly wrapped
circumferentially around an appendage of the child causing
strangulation and compromised blood flow. Appendages affected in such
syndrome include in order of preference toe, finger, uvula, tongue or
genital structure (penis and clitoris). The third toe is the most
frequent affected digit. Erythema and circumferential cut with distal
edema is typical compromising the distal venous and lymphatic drainage
of the appendage involved (compartment syndrome). Further edema leads
to interruption of arterial blood supply causing ischemic injury,
tissue necrosis and autoamputation. This process can occur over hours
to weeks. It is important to distinguish HTS from congenital
constriction band syndrome (amniotic band syndrome, Streeter's
dysplasia), the latter being a rare congenital condition that is
associated with other musculoskeletal disorders. The typical case is a
5 month old infant. Males and female are equally affected. Hairs are
suppled and stretched easily when wet and contract when dry, and the
circular configuration results in hydrogen-bonds giving a firmer hold.
The high tensile strength of hair makes it an effective tourniquet.
Management consists of release of the strangulation under either local
or general anesthesia urgently. The constricting hair or fiber may be
mechanically removed or depilatory agents may be used. A short
longitudinal incision (peritendinous) placed perpendicular to the
constricting band can be dorsally made in the digit all the way to the
bone to facilitate a definitive release since severe inflammation can
deeply bury the offending agent. The strand of hair will be then be
seen and removed completely. Antibiotic coverage should be provided as
appropriate.
References:
1- Serour F, Gorenstein A: Treatment of the toe tourniquet syndrome in infants. Pediatr Surg Int. 19(8):598-600, 2003
2- Badawy H, Soliman A, Ouf A, Hammad A, Orabi S, Hanno A: Progressive
hair coil penile tourniquet syndrome: multicenter experience with
25 cases. J Pediatr Surg. 45(7):1514-8., 2010
3- O'Gorman A, Ratnapalan S: Hair tourniquet management. Pediatr Emerg Care. 27(3):203-4, 2011
4- Bannier MA, Miedema CJ: Hair tourniquet syndrome. Eur J Pediatr. 172(2):277, 2013
5- Bean JF, Hebal F, Hunter CJ: A single center retrospective review of
hair tourniquet syndrome and a proposed treatment algorithm. J Pediatr
Surg. 50(9):1583-5, 2015
6- Kuiper JW, de Korte N: Hair thread tourniquet syndrome in a toe of an 18 mo old girl. World J Clin Cases. 3(4):368-70, 2015
Blunt Cardiac Injury
Injury to the chest can cause trauma
to the heart. Blunt cardiac injury (BCI) is rare in children.
Mechanisms associated with such trauma include thrusting of the heart
against the chest wall as a direct result of sudden acceleration or
deceleration, compression of the heart between the sternum and the
vertebral column, and a sudden violent increase in intrathoracic
pressures associated with crushing chest or abdominal injuries. Most
cases occur after motor vehicle accidents. Most (95%) BCI is cardiac
contusion followed by valvular dysfunction and ventricular septal
defects. Other sequelae such as hemopericardium, pericardial effusion,
arrhythmias, cardiac aneurysm, and myocardial rupture may only occur
after some delay emphasizing the need for observation and serial
evaluation. Free wall rupture is lethal. Chest pain is present in 50%
of neurologically responsive children. The most useful investigations
in suspected cases of myocardial contusion are serial cardiac
troponins, an initial and repeat ECG, echocardiography and arrhythmia
monitoring. Troponin 1 level is a sensitive and more specific measure
than CK-MB. They become elevated within hours of injury and remain
elevated for 4-7 days. Levels above 8.0 ng/ml are associated with a
fatal outcome. Troponin is also very useful in cases of non-accidental
chest trauma, such as child abuse. In general, patients with myocardial
contusion have a favorable outcome. Normalization of cardiac troponin
can help to risk-stratify patients. VSD after BCI is also very rare,
occurs more commonly in the muscular septum followed by the membranous
portion due to initial damage of the nutrient vessels supplying the
septum. Conduction anomalies, such as RBB and AV block, along with
arrhythmias such as tachycardia and extrasystole or ventricular
fibrillation can occur depending on damage. Angiography is the
gold standard for confirming coronary involvement.
References:
1- Karpas A, Yen K, Sell LL, Frommelt PC: Severe blunt cardiac injury
in an infant: a case of child abuse. J Trauma. 52(4):759-64, 2002
2- Milligan J, Potts JE, Human DG, Sanatani S: The protean
manifestations of blunt cardiac trauma in children. Pediatr Emerg Care.
2005 May;21(5):312-7
3- Huguet M, Tobon-Gomez C, Bijnens BH, Frangi AF, Petit M: Cardiac
injuries in blunt chest trauma. J Cardiovasc Magn Reson. 17;11:35, 2009
4- Bennett BL, Mahabee-Gittens M, Chua MS, Hirsch R: Elevated cardiac
troponin I level in cases of thoracic nonaccidental trauma. Pediatr
Emerg Care. 27(10):941-4, 2011
5- Acker SN, Stewart CL, Roosevelt GE, Partrick DA, Moore EE, Bensard
DD: When is it safe to forgo abdominal CT in blunt-injured children?
Surgery. 158(2):408-12, 2015
6- Skinner DL, den Hollander D, Laing GL, Rodseth RN, Muckart DJ:
Severe blunt thoracic trauma: differences between adults and children
in a level I trauma centre. S Afr Med J. 105(1):47-51, 2015
VATS: Spontaneous Pneumothorax
Spontaneous pneumothorax (SP) in
children is usually primary and caused by rupture of a subpleural bleb
or bulla in adolescent, tall and thin patients. Secondary SP occurs in
children with underlying lung disease such as asthma, cystic fibrosis,
emphysema or connective tissue disorder. The goals of treatment of SP
consist of lung re-expansion and avoidance of recurrence. Initial
management with needle aspiration or tube thoracostomy drainage have a
high failure and recurrent rate. Instillation of chemicals to sclerose
the pleuras through the chest tube have also a significant rate of
recurrence. A wide variety of chemical sclerosing agents have been
introduced into the pleural space to achieve pleural symphysis. The
most popular agents have been talc, tetracycline, and silver nitrate,
which are known to cause aseptic inflammation leading to dense
adhesions. Talc has the lowest recurrence rate of all chemical agents,
while tetracyclines are the most innocuous. During the last decade
video-assisted thoracoscopic surgery (VATS) has replaced open
thoracotomy in the management of spontaneous pneumothorax. Using VATS
the surgeon can resect the offending bleb or bulla and produce chemical
or mechanical pleurodesis with less pain, less respiratory dysfunction,
less hospital stay and better cosmetic results. VATS with blebectomy
can be used during the first SP episode if there is persistent chest
tube air leak, a contralateral SP develops in a known patient or he has
respiratory insufficiency. The two most common complications after VATS
for SP are prolonged air leak and bleeding. Prolonged leak is
associated with an underlying lung disease such as asthma, cystic
fibrosis or emphysema. Postoperative air leak from the staple lines
probably accounts for most of the need for postop chest tube drainage.
Prolonged air leak is managed with continuous chest tube suction and
further chemical pleurodesis. Both factors increase hospital stay.
Bleeding might occur after dense adhesions of previous recurrent
episodes of SP or after intercostal artery injury from the trocars.
Children with SP should avoid activities that put additional strain on
the lungs including scuba diving, airplane flight and playing wind
musical instruments. Late recurrence is related to formation of new
bullae.
References:
1- Ozcan C, McGahren ED, Rodgers BM: Thoracoscopic Treatment of
Spontaneous Pneumothorax in Children. J Pediatr Surg. 38(10):
1459-1464, 2003
2- Bialas RC, Weiner TM, Phillips JD: Video-assisted thoracic surgery
for primary spontaneous pneumothorax in children: is there an optimal
technique? J Pediatr Surg. 43(12):2151-5, 2008
3- Lee LP, Lai MH, Chiu WK, Leung MW, Liu KK, Chan HB: Management of
primary spontaneous pneumothorax in Chinese children. Hong Kong Med J.
16(2):94-100, 2010
4- Seguier-Lipszyc E, Elizur A, Klin B, Vaiman M, Lotan G: Management
of primary spontaneous pneumothorax in children. Clin Pediatr (Phila).
50(9):797-802, 2011
5- Choi SY, Kim YH, Jo KH, Kim CK, Park JK, Cho DG, Jeong SC, Jeon HW,
Park CB: Video-assisted thoracoscopic surgery for primary spontaneous
pneumothorax in children. Pediatr Surg Int. 29(5):505-9, 2013
6- Lopez ME, Fallon SC, Lee TC, Rodriguez JR, Brandt ML, Mazziotti MV:
Management of the pediatric spontaneous pneumothorax: is primary
surgery the treatment of choice? Am J Surg. 208(4):571-6, 2014
7- Noh D, Lee S, Haam SJ, Paik HC, Lee DY: Recurrence of primary
spontaneous pneumothorax in young adults and children. Interact
Cardiovasc Thorac Surg. 21(2):195-9, 2015