PEDIATRIC SURGERY UPDATE ©
VOLUME 46, 2016
PSU Volume 46 NO 01 JANUARY 2016
Congenital Paraesophageal Hernia
Congenital paraesophageal hernia (CPH)
is the rarest of all diaphragmatic hernia of childhood. As the name
implies, the patient is born with the defect. Most paraesophageal
hernias are acquired after procedures performed in the GE junction
(antireflux procedures). In CPH the defect can go unnoticed for several
years or present with recurrent chest infections or vague
gastrointestinal symptoms. Left alone CPH can cause high morbidity or
even mortality hence the importance of an early diagnosis. CPH occurs
because of absent or abnormally lax anatomic anchors of the stomach and
gastroesophageal junction associated to a paraesophageal defect. The
fundus is pushed through the defect by increase intraabdominal pressure
entering the thoracic cavity causing lung compression and mediastinal
shift. If this progress the antrum also enters the thorax and
organoaxial volvulus of the stomach occur causing gastric obstruction
and esophageal dilatation. With enlargement of the defect bowel and
omentum can herniate. There is absence of pulmonary hypoplasia.
Clinically PEH can present acutely with incarceration, obstruction,
gangrene, perforation, bleeding, anemia, as well as acute respiratory
complications. Other times the child has chronic chest infections with
intermittent vomiting. Initial work-up should include chest film, upper
gastrointestinal series and abdominal CT-Scan to determine herniated
content and detect complications of affected lungs. Associated
anomalies with CPH include short esophagus and microgastria. Surgical
repair should be done promptly to avoid serious complications. The
procedure can be performed open or laparoscopically and aims to resect
the hernia sac, reduce the herniated content, close the hiatal defect,
anchor the stomach or do a partial antireflux procedure, and check for
associated malformations.
References:
1- Al-Salem AH: Congenital paraesophageal hernia in infancy and childhood. Saudi Med J. 21(2):164-7, 2000
2- Imamoglu M, Cay A, Kosucu P, Ozdemir O, Orhan F, Sapan L, Sarihan H:
Congenital paraesophageal hiatal hernia: pitfalls in the diagnosis and
treatment. J Pediatr Surg. 40(7):1128-33, 2005
3- Jetley NK, Al-Assiri AH, Al Awadi D: Congenital para esophageal
hernia: a 10 year experience from Saudi Arabia. Indian J Pediatr.
76(5):489-93, 2009
4- Priego Jimenez P, Salvador Sanches JL, Angel V, Escrig-Sos J:
Short-term results for laparoscopic repair of large paraesophageal
hiatal hernias with Gore Bio mesh. Int J Surg. 12(8):794-7, 2014
5- Athanasakis H, Tzortzinis A, Tsiaoussis J, Vassilakis JS, Xynos E:
Laparoscopic repair of paraesophageal hernia. Endoscopy. 33(7):590-4,
2001
6- Yousef Y, Lemoine C, St-Vil D, Emil S: Congenital paraesophageal
hernia: The Montreal experience. J Pediatr Surg. 50(9):1462-6, 2015
Intradiploic Dermoid Cysts
Dermoid cysts are congenital benign
neoplasms derived from both ectoderm and mesoderm tissue layers. They
typically occur in childhood during the first or second decade of life.
They are composed of a keratinizing squamous epithelium present
together with dermal derivatives such as hair follicles, smooth muscle,
sweat gland, sebaceous gland and fibroadipose tissue. On-tenth of all
dermoid cysts occurs in the region of the head and neck in locations
such as periorbital, nasal, frontotemporal, submental and suprasternal.
Dermoid cysts of the head and neck are thought to be the congenital
inclusion type. The periorbital region is the most commonly reported
site for dermoid cysts of head and neck, most commonly near the
zygomaticofrontal suture, laterals to the lateral canthus. Some dermoid
cysts exhibit intermittent discharge of sebaceous material or recurrent
local infection. In the temporal bone dermoid cysts can be mistakenly
labels as a first branchial cleft or pouch anomalous cyst. Lesions in
the midline of the cranium are more concerning for intracranial
extension. Dermoid cysts that are located in the nasal bridge or
temporal area, produce recurrent infection or exhibit a draining sinus
should alert the physician toward the presence of intracranial
extension warranting further imaging with CT-Scan or MRI to plan
excision. Intradiploic dermoid cysts are a rare subtype of the
frontotemporal dermoids occurring most commonly at the bregma. They may
appear as a lytic defect in the skull. The overwhelming majority of
periorbital and frontotemporal dermoids are superficially located and
amenable to treatment with simple excision. Complete en bloc
surgical resection with careful follow-up is the treatment of choice
for dermoid cysts.
References:
1- Parag P, Prakash PJ, Zachariah N: Temporal dermoid--an unusual presentation. Pediatr Surg Int. 17(1):77-9, 2001
2- Scolozzi P, Lombardi T, Jaques B: Congenital intracranial
frontotemporal dermoid cyst presenting as a cutaneous fistula. Head
Neck. 27(5):429-32, 2005
3- Pryor SG, Lewis JE, Weaver AL, Orvidas LJ: Pediatric dermoid cysts
of the head and neck. Otolaryngol Head Neck Surg. 132(6):938-42,2005
4- Rinna C, Reale G, Calafati V, Calvani F, Ungari C: Dermoid cyst: unusual localization. J Craniofac Surg. 23(5):e392-4, 2012
5- Vega RA, Hidlay DT, Tye GW, Fuller CE, Rhodes JL: Intradiploic
dermoid cyst of the lateral frontotemporal skull: case report and
review of the literature. Pediatr Neurosurg. 49(4):232-5, 2013
6- Barnard AR, Jones AP, Hodgkinson PD, Jenkins AJ: Beware
frontotemporal dermoids - they may have intracranial extension: a case
of a middle cranial fossa cyst. J Plast Reconstr Aesthet
Surg. 65(7):e185-8, 2012
Esophageal Atresia with Right Aortic Arch
Almost 5% of children with esophageal
atresia (EA) with or without an associated tracheoesophageal fistula
have a right aortic arch (RAA). Preoperative localization of the aortic
arch position previous to repair of EA is of utmost importance to avoid
an extremely difficult right thoracotomy. Preoperative localization of
a RAA using echocardiography correctly identifies the arch position in
two-thirds of patients. The surgeon needs to reliable identify the
position of the aortic arch, the laterality of the descending thoracic
aorta and the nature of any associated congenital heart disease. If an
aortic arch pass over the right mainstem bronchus, it will descend
through the right side of the chest 95% of the time, and there will be
complete absence of any retro-esophageal component to the aorta. Most
will have mirror-image branching of the innominate and subclavian
arteries. Infants with EA with RAA have more vascular rings, cardiac
anomalies and operative complications compared with those with LAA. Up
to 50% of children with EA and RAA have associated cardiac
malformations, especially ventricular and atrial septal defects. RAA is
found in 25% of cases of Tetralogy of Fallot. Should the suspicion of a
RAA in EA arise, further diagnostic evaluation using CT-angio or MRI
will elucidate the exact anatomic variant. When RAA is associated with
EA, the gap length between the esophageal pouches is usually increased.
A left thoracotomy in the face of RAA allows one to gain better
exposure to the esophagus and tracheoesophageal fistula without the
obstruction of the aorta. In addition, owing to the type of vascular
ring that can be encountered the left-sided thoracotomy allows access
to ligate the PDA or manage the vascular ring. Should a RAA be
unexpectedly encountered during a right thoracotomy and the anastomosis
is not technically feasible a delayed left thoracotomy permits more
accurate evaluation of the vascular anatomy.
References:
1- Bowkett B, Beasley SW, Myers NA: The frequency, significance, and
management of a right aortic arch in association with esophageal
atresia. Pediatr Surg Int. 15(1):28-31, 1999
2- Babu R, Pierro A, Spitz L, Drake DP, Kiely EM: The management of
oesophageal atresia in neonates with right-sided aortic arch. J
Pediatr Surg. 35(1):56-8, 2000
3- Kosloske AM, Jewell PF: A right-sided aortic arch in 12 infants with esophageal atresia. J Pediatr Surg. 35(11):1684, 2000
4- Allen SR, Ignacio R, Falcone RA, Alonso MH, Brown RL, Garcia VF,
Inge TH, Ryckman FC, Warner BW, Azizkhan RG, Tiao GM: The effect of a
right-sided aortic arch on outcome in children with esophageal atresia
and tracheoesophageal fistula. J Pediatr Surg. 41(3):479-83, 2006
5- Bicakci U, Tander B, Ariturk E, Rizalar R, Ayyildiz SH, Bernay F:
The right-sided aortic arch in children with esophageal atresia and
tracheo-esophageal fistula: a repair through the right thoracotomy.
Pediatr Surg Int. 25(5):423-5, 2009
6- Wood JA, Carachi R: The right-sided aortic arch in children with
oesophageal atresia and tracheo-oesophageal fistula. Eur J Pediatr
Surg. 22(1):3-7, 2012
7- Paraloni F, Armellini A, Boroni G, Bagolan P, Alberti D: The
management of newborns with esophagea atresia and right aortic arch: A
systematic review or still unsolved problem. J Pediatr Surg 2015
(Article in press).
PSU Volume 46 NO 02 FEBRUARY 2016
Body Stalk Anomaly
Anterior abdominal wall defects are
classified as gastroschisis, omphalocele and body-stalk anomalies. Body
stalk anomaly (BSA) is the most rare and a severe abdominal wall defect
associated with a high incidence of spontaneous abortion. BSA consists
of a large abdominal wall defect in more severe cases of thoracic
organs as well, associated with severe kyphoscoliosis and a very short
or even absent umbilical cord. In addition the upper half of the fetal
body is in the amniotic cavity while the lower half is in the celomic
cavity. This anomaly might also occur in conjunction with neural tube
defects, genitourinary malformations, abnormalities of the chest wall,
intestinal atresia, and craniofacial defects. The most plausible theory
of formation of BSA is that early amnion rupture before obliteration of
the celomic cavity is the underlying mechanism. The rupture results in
the expulsion of the embryo or a part of it into the exocoelomic
cavity. Other alternative theories include an abnormal embryonic
folding resulting in the failure of the celomic cavity to obliterate or
vascular disruption during the first 4-6 weeks of gestation. Most cases
of body stalk syndrome are incompatible with life. Body stalk anomaly
is not associated with chromosomal abnormalities and there is no
increased risk for a recurrence. Levels of alpha-fetoprotein in
maternal serum are very high in BSA. Prenatal ultrasound in the 9th to
10th weeks of pregnancy has detected this diagnosis. It has been
reported that 50% of women with fetuses affected by body stalk anomaly
smoke cigarettes or drink alcohol and 30% of them smoked marijuana.
Continuation of pregnancy in the presence of BSA is associated with
greater maternal risks, in the form of labor complications requiring
cesarean section. Prenatal diagnosis enables prompt knowledge of the
fetus condition permitting appropriate counseling and
management.
References:
1- Daskalakis G, Pilalis A, Papadopoulos D, Antsaklis A: Body stalk
anomaly diagnosed in the 2nd trimester. Fetal Diagn Ther. 18(5):342-4,
2003
2- Hirokawa S, Uotani H, Futatani T, Sasaki Y, Ogawa J, Sakai M,
Tsukada K, Saito S: A case of body stalk anomaly arising in the second
baby of a triplet pregnancy after in-vitro fertilization and embryo
transfer. Pediatr Surg Int. 19(3):223-5, 2003
3- Kanamori Y, Hashizume K, Sugiyama M, Tomonaga T, Takayasu H,
Ishimaru T, Terawaki K, Suzuki K, Goishi K, Takamizawa M: Long-term
survival of a baby with body stalk anomaly: report of a case. Surg
Today. 37(1):30-3, 2007
4- Murphy A, Platt LD: First-trimester diagnosis of body stalk anomaly using 2- and 3-dimensional
sonography. J Ultrasound Med. 30(12):1739-43, 2011
5- Costa ML, Couto E, Furlan E, Zaccaria R, Andrade K, Barini R, Nomura
ML: Body stalk anomaly: adverse maternal outcomes in a series of 21
cases. Prenat Diagn. 32(3):264-7, 2012
6- Kocherla K, Kumari V, Kocherla PR:Prenatal diagnosis of body stalk
complex: A rare entity and review of literature. Prenatal diagnosis of
body stalk complex: A rare entity and review of literature. Indian J
Radiol Imaging. 25(1): 67–70, 2015
Transverse Colonic Volvulus
Around 5% of all intestinal
obstructions are caused by colonic volvulus. Sigmoid colon is the most
common site for volvulus followed by the cecum, transverse and splenic
flexure in order of frequency. Volvulus of the transverse colon is a
very rare event in children. Mean age is ten years. Main reason is that
the broad-based short mesentery normally prevents the transverse colon
from rotating either clockwise or anti-clockwise. Transverse colonic
volvulus (TCV) is more common in Eastern Europe, Scandinavian countries
and Japan. Risk of volvulus is increased by the following factors:
elongation and redundancy of the transverse colon, narrowing of the
mesenteric attachments causing flexures to come together, and absence
or malfixation of the mesenteries. Other mechanical causes include
previous volvulus of transverse or sigmoid, distal colonic obstruction,
adhesions, malposition of the colon after surgery, inflammatory
structures and carcinoma. Chronic constipation, cerebral palsy, mental
retardation, Hirschsprung's disease and high fiber diet is a
characteristic found in children with TCV. Symptoms of TCV
include abdominal pain, vomiting, abdominal distension and tenderness.
Clinically TCV presents acutely and fulminant or subacute progressive
depending on the degree of ischemia. Simple films show distension of
proximal colon, empty distal bowel and two air-fluids levels caused by
double closed loop obstruction. A barium enema might show a "bird beak"
or "ace of spades" at the focus of the twist bowel. CT Scan is
diagnostic. Management consists of manual detorsion, detorsion with
colopexy, resection with primary anastomosis in case of significant
ischemia, or resection with colostomy and/or ileostomy and mucous
fistula in cases of intraoperative hemodynamic instability. Manual
detorsion is associated with a high incidence of recurrence. Resection
with or without primary anastomosis is the treatment of choice to
prevent recurrence.
References:
1- Asabe K, Ushijima H, Bepu R, Shirakusa T: A case of transverse colon
volvulus in a child and a review of the literature in Japan. J
Pediatr Surg. 37(11):1626-8, 2002
2- Sparks DA, Dawood MY, Chase DM, Thomas DJ: Ischemic volvulus of the
transverse colon: A case report and review of literature. Cases J.
1(1):174, 2008
3- Rahbour G, Ayantunde A, Ullah MR, Arshad S, Kerwat R: Transverse
colon volvulus in a 15 year old boy and the review of the literature.
World J Emerg Surg. 5:19, 2010
4- Sage MJ, Younis J, Schwab KE, Galbraith KA: Colopexy as a treatment
option for the management of acute transverse colon volvulus: a case
report. J Med Case Rep. 6:151, 2012
5- Smith C, Cho A, Tsang T: Transverse colonic volvulus in a child:
successful management with decompression and delayed laparoscopic
colopexy. European J Pediatr Surg Rep. 1(1):48-50, 2013
6- Waluza JJ, Aronson DC, Nyirenda D, Zoetmulder FA, Borgstein ES:
Transverse colon volvulus in children: A case series and a review of
the literature. J Pediatr Surg. 50(10):1641-3, 2015
Povidone-Iodine Pleurodesis
Povidone-iodine is an antiseptic
material used routinely to clean surgical sites and as a procedural
disinfectant. It has been utilized as a safe chemical pleurodesis agent
in cases of spontaneous pneumothorax, air leaks, malignant pleural
effusions and chylothorax in children and adults when injected into the
pleural space. The mechanism of action of povidone-iodine appears to be
related to chelation of proteins and enhanced sclerosis. Iodine has
strong oxidative and cytotoxic properties which induce a potent
inflammatory response. In addition the low pH of povidone-iodine may
contribute to the local inflammatory response. When used in
concentrations of 4% iodopovidone produces pronounced pleural adhesion
and thickening mesothelial cell injury. The most significant
complication reported is chest pain. Visual loss resulting from the
breakdown of the diffusion barrier of the retinal pigment epithelium
with concentrations of 10% during thoracoscopic surgery has been
reported. Procedure consists of injecting a saline solution diluted 4%
povidone-iodine into the pleural space at a dose of 2 ml/kg of weight
followed by clamping the chest tube for four hours. Using these low
iodine concentrations, neither renal function nor thyroid function is
impaired both immediately after the procedure or long term. To further
reduce the risk and severity of side effects, 2% povidone–iodine
may be used, as it has experimentally been shown that it is as
effective as 4% concentration. Multicenter randomized studies that
compare the risks and benefit of povidone-iodine should be done before
we label this option as the procedure of choice.
References:
1- Mahmodlou R, Rahimi-Rad MH, Alizadeh H: Efficacy and safety of
iodopovidone pleurodesis through chest tube in spontaneous
pneumothorax. Pneumologia. 60(2):78-80, 2011
2- Arayici S, Simsek GK, Oncel MY, Yilmaz Y, Canpolat FE, Dilmen U:
Povidone-iodine for persistent air leak in an extremely low birth
weight infant. J Pediatr Surg. 48(5):E21-3, 2013
3- Teixeira LR, Vargas FS, Puka J, Acencio MM, Antonangelo L, Terra RM,
Damico FM, Pitta FG, Marchi E: Effectiveness and safety of iodopovidone
in an experimental pleurodesis model. Clinics (Sao Paulo).
68(4):557-62, 2013
4- Ibrahim IM, Dokhan AL, El-Sessy AA, Eltaweel MF: Povidone-iodine
pleurodesis versus talc pleurodesis in preventing recurrence of
malignant pleural effusion. J Cardiothorac Surg. 10:64, 2015
5- Scottoni F, Fusaro F, Conforti A, Morini F, Bagolan P: Pleurodesis
with povidone-iodine for refractory chylothorax in newborns: Personal
experience and literature review. J Pediatr Surg. 50(10):1722-5,
2015
6- Resch B, Freidl T, Reiterer F: Povidone-iodine pleurodesis for
congenital chylothorax of the newborn. Arch Dis Child Fetal Neonatal
Ed. 101(1):87-8, 2016
PSU Volume 46 NO 03 MARCH 2016
Phrygian Cap Gallbladder
The most common congenital anomaly of
the biliary tract found in 2-6% of autopsies is a folded gallbladder,
also known as Phrygian cap gallbladder, since it resembles a bonnet
worn by the ancient Phrygians of Asia Minor. The Phrygian cap occurs
when the fundus of the gallbladder fold on itself. The literature
suggests the Phrygian cap anomaly predispose this segment of the
gallbladder to slow flow or delayed filling and hence lithiasis. High
definition ultrasound and nuclear studies can identify a Phrygian cap.
Other studies that can establish the diagnosis include CT-Scan and
MRCP. Phrygian cap gallbladder should be a differential diagnosis
considered when a mass is found associated with the gallbladder or
liver adjacent to the gallbladder. Phrygian cap anomaly can also
simulate duplication of the gallbladder. Acalculous and calculous
cholecystitis have been associated with Phrygian cap gallbladder. In
many cases with "Phrygian cap" deformity, there is a mucosal fold
created, which partially subdivides the lumen of the organ. Most
patients with Phrygian cap gallbladder are asymptomatic. Gallbladder
with Phrygian cap deformity can lead to misdiagnosis of gallbladder
stones on B-mode ultrasonography. Prophylactic cholecystectomy is not
indicated in patients with Phrygian cap deformity alone as it is of no
pathological significance. Surgery is performed only for patients with
symptoms or complications of gallbladder disease irrespective of
Phrygian cap deformity.
References:
1- Saji S, Tony J, Kumar KS, Thomas V: Phrygian cap in magnetic resonance cholangiogram. Indian J Gastroenterol. 27(5):194, 2008
2- de Csepel J, Carroccio A, Pomp A: Soft-tissue images. "Phrygian cap" gallbladder. Can J Surg. 46(1):50-1, 2003
3- Al-Ashqar M, Maliyakkal AK, Shiwani MH, Anwar S: Acalculous Phrygian cap cholecystitis. BMJ Case Rep. 2013 Oct 4;2013.
4- Rafailidis V, Varelas S, Kotsidis N, Rafailidis D: Two congenital
anomalies in one: an ectopic gallbladder with phrygian cap deformity.
Case Rep Radiol. 2014;2014:246476
5- Kannan NS, Kannan U, Babu CP: Congenital bilobed gallbladder with
phrygian cap presenting as calculus cholecystitis. J Clin Diagn Res.
8(8):ND05-6, 2014
6- van Kamp MJ, Bouman DE, Steenvoorde P, Klaase JM: A phrygian cap. Case Rep Gastroenterol. 17;7(2):347-51, 2013
BI-RADS in Children
Breast masses in children are rare but
cause significant concern in parents. Fortunately most breast masses in
children are benign consisting of fibroadenomas, gynecomastia, cysts
and macromastia. Size, presence of symptoms and growth are the
characteristics that decide upon excision for diagnostic and
therapeutic reasons. To avoid ionizing radiation, the ultrasound has
become the best imaging study to perform in children breast masses. The
breast imaging reporting and data system (Bi-Rads) developed by the
American College of Radiology categorize these lesions based on the
likehood of malignancy stipulating the need of tissue diagnosis in high
classification cases. The risk of malignancy in children is lower than
adults. Correlating Bi-Rads findings with tissue diagnosis it has been
found that breast lesions in children with Bi-Rads classification of 4
where excision is strongly recommended are almost always benign. This
usually occurs with juvenile fibroadenomas in teenage females. Most
breast masses in children are commonly described as oval, hypoechoic
and circumscribed, and almost never classified as calcified, with
angular or spiculated margins or showing posterior shadowing. Should we
follow adult management children with Bi-Rads 4 would undergo
core-needle biopsy, a procedure which is painful and often requires
anesthesia. In such situation excisional biopsy using a periareolar
incision is more practical than core-needle biopsy. Breast masses with
high Bi-Rads classification should be removed because of size, evidence
of growth or persistent symptoms. Bi-Rads classification overstates the
risk of malignancy in the pediatric population invalidating the
treatment algorithm associated with the classification. Short term
imaging follow-up is an acceptable alternative to immediate
biopsy.
References:
1- Kwak JY, Kim EK, Park HL, Kim JY, Oh KK: Application of the breast
imaging reporting and data system final assessment system in sonography
of palpable breast lesions and reconsideration of the modified triple
test. J Ultrasound Med. 2006 Oct;25(10):1255-61.
2- Park YM, Kim EK, Lee JH, Ryu JH, Han SS, Choi SJ, Lee SJ, Yoon HK:
Palpable breast masses with probably benign morphology at sonography:
can biopsy be deferred? Acta Radiol. 2008 Dec;49(10):1104-11
3- Loving VA, DeMartini WB, Eby PR, Gutierrez RL, Peacock S, Lehman CD:
Targeted ultrasound in women younger than 30 years with focal breast
signs or symptoms: outcomes analyses and management implications. AJR
Am J Roentgenol. 2010 Dec;195(6):1472-7.
4- Son EJ, Kim EK, Youk JH, Kim MJ, Kwak JY, Choi SH:
Imaging-histologic discordance after sonographically guided
percutaneous breast biopsy: a prospective observational study.
Ultrasound Med Biol. 2011 Nov;37(11):1771-8
5- Xiao X, Ou B, Yang H, Wu H, Luo B: Breast contrast-enhanced
ultrasound: is a scoring system feasible? A preliminary study in China.
PLoS One. 2014 Aug 18;9(8):e105517
6- Koning JL, Davenport KP, Poole PS, Kruk PG, Grabowski JE: Breast
Imaging-Reporting and Data System (BI-RADS) classification in 51
excised palpable pediatric breast masses. J Pediatr Surg. 2015
Oct;50(10):1746-50.(leading article).
Asymptomatic Malrotation Revisted
Midgut volvulus is a clockwise
rotation of the bowel occurring in children and adults with malrotation
presenting with acute symptoms of ischemia and bowel obstruction. Early
recognition and surgical intervention is mandatory. Most cases occur
during the neonatal period. Not all cases of malrotation ends in midgut
volvulus, hence some patients present with asymptomatic
malrotation if they are found incidentally with imaging studies
compatible with such but complete absence of symptoms. Radiologic
investigation includes upper gastrointestinal series, Doppler
sonography and contrast enhanced CT of the abdomen. UGIS is the test of
choice for diagnosing malrotation. Ultrasound can be falsely negative
and cannot be used to definitively rule out malrotation or volvulus.
The management of asymptomatic or incidentally discovered malrotation
is controversial. Malrotation can be divided into true malrotation with
a narrow mesenteric stalk, nonrotation with a broad mesentery, and
atypical defined as malposition of the ligament of Treitz or duodenum.
Children with a higher incidence of malrotation include those with
congenital heart disease (CHD) and heterotaxy syndrome, abdominal wall
defect and diaphragmatic hernia. It's unusual to perform a Ladd's
procedure for gastroschisis, omphalocele or diaphragmatic hernia as
adhesions from surgery decrease the incidence of volvulus. There is
minimal evidence to support screening in asymptomatic patients with CHD
and heterotaxy syndrome. In this group of children it seems better to
wait for palliation of the heart defect before offering a Ladd's
procedure. Recent data from APSA regarding the need for prophylactic
Ladd's procedure in asymptomatic malrotated children suggest it should
be done for young age, while observation may be appropriate in the
older asymptomatic patient. The laparoscopic approach is safe for
diagnosing and managing asymptomatic malrotation, while there is
minimal evidence to support the laparoscopic approach in cases of known
volvulus or in neonates. Any age, any condition with symptomatic
malrotation should undergo a Ladd's procedure.
References:
1- Dilley AV, Pereira J, Shi EC, Adams S, Kern IB, Currie B, Henry GM:
The radiologist says malrotation: does the surgeon operate? Pediatr
Surg Int. 16(1-2):45-9, 2000
2- Prasil P, Flageole H, Shaw KS, Nguyen LT, Youssef S, Laberge JM:
Should malrotation in children be treated differently according to age?
J Pediatr Surg. 5(5):756-8, 2000
3- Cohen Z, Kleiner O, Finaly R, Mordehai J, Newman N, Kurtzbart E,
Mares AJ: How much of a misnomer is "asymptomatic" intestinal
malrotation? Isr Med Assoc J. 5(3):172-4, 2003
4- Malek MM, Burd RS: The optimal management of malrotation diagnosed
after infancy: a decision analysis. Am J Surg. 191(1):45-51, 2006
5- Lampl B, Levin TL, Berdon WE, Cowles RA: Malrotation and midgut
volvulus: a historical review and current controversies in diagnosis
and management. Pediatr Radiol. 39(4):359-66, 2009
6- Lodwick DL, Minneci PC, Deans KJ: Current surgical management of
intestinal rotational abnormalities. Curr Opin Pediatr. 27(3):383-8,
2015
7- Graziano K, Islam S, Dasgupta R, Lopez ME, Austin M, Chen LE, Goldin
A, Downard CD, Renaud E, Abdullah F: Asymptomatic malrotation:
Diagnosis and surgical management: An American Pediatric Surgical
Association outcomes and evidence based practice committee systematic
review. J Pediatr Surg. 50(10):1783-90, 2015
PSU Volume 46 NO 04 APRIL 2016
Saline Hydrostatic Intussusception Reduction
An intussusception is an invagination
of the proximal bowel into the lumen of the distal bowel. Most
(>90%) intussusception in children are ileocolic, with the least of
them being either ileoileal or colocolic. Swollen Peyer's patches,
enlarged lymph nodes, polyps, Meckel's diverticulum and duplication
cysts are the most common etiological factors associated with
intussusceptions. They occur usually in children between the ages of
six months to three years demonstrating signs/symptoms such as colicky
pain, bilious vomiting, abdominal distension, and currant jelly stools.
The diagnosis of intussusception can be made using an abdominal
ultrasound when demonstrating an oval pseudokidney mass lesion with
central echoes, a sonolucent target lesion, various layers and
concentric rings, fluid, enlarged lymph nodes or other soft tissue or
cystic mass suggestive of a pathological lead point.
Ultrasonographically guided hydrostatic reduction with the use of
normal warm saline is believed to be one of the most promising method
for the non-surgical treatment of pediatric intussusception. It has
been found to be safe, simple, effective, economical, and a less
time-consuming procedure coupled with fewer complications, no radiation
hazard and a minimal hospital stay. It is also associated with less
morbidity since there is no incidence of a pseudo-obstruction as is
seen in the fluoroscopic guided procedure, there is no fluctuation in
the intracolonic pressure and there is minimal chance for chemical
peritonitis as it occurs when using other contrast material. Risk
factors for failure of reduction include localization (left colon),
bloody stools, free peritoneal fluid and fluid trapped in the
intussusceptum. Contraindications for hydrostatic reduction include
absent or scattered vascularity within the lesion in color Doppler
studies, evident signs of perforation or peritonitis, ascites, multiple
intussusception with a pathological lead point or shock. The reduction
success rate using saline hydrostatic reduction monitored by ultrasound
is above 80%.
References:
1- Shehata S, El Kholi N, Sultan A, El Sahwi E: Hydrostatic reduction
of intussusception: barium, air, or saline? Pediatr Surg Int.
16(5-6):380-2, 2000
2- Nayak D, Jagdish S: Ultrasound guided hydrostatic reduction of
intussusception in children by saline enema: our experience.
Indian J Surg. 70(1):8-13, 2008
3- Mensah Y, Glover-Addy H, Etwire V, Appeadu-Mensah W, Twum M:
Ultrasound guided hydrostatic reduction of intussusception in children
at Korle Bu Teaching Hospital: an initial experience. Ghana Med J.
45(3):128-31, 2011
4- Digant SM, Rucha S, Eke D: Ultrasound guided reduction of an
ileocolic intussusception by a hydrostatic method by using normal
saline enema in paediatric patients: a study of 30 cases. J Clin Diagn
Res. 6(10):1722-5, 2012
5- He N, Zhang S, Ye X, Zhu X, Zhao Z, Sui X: Risk factors associated
with failed sonographically guided saline hydrostatic intussusception
reduction in children. J Ultrasound Med. 33(9):1669-75, 2014
6- Flaum V, Schneider A, Gomes Ferreira C, et al: Twenty years'
experience for reduction of ileocolic intussusceptions by saline enema
under sonography control. J Pediatr Surg. 51(1):179-82, 2016
Perforated Peptic Ulcer Disease
Peptic ulcer disease (PUD) is uncommon
in children and rarely suspected as an etiology of abdominal symptoms
until the child develops a complication such as upper gastrointestinal
bleeding, obstruction or perforation. Peptic ulcer disease is
classified as gastric or duodenal depending on location. Children less
than 10 years of age predominantly have duodenal peptic ulcers, while
above that age gastric ulcer predominates. Most duodenal peptic ulcer
disease is associated with Helicobacter Pylori infection of the gastric
antral mucosa. Other causes of PUD results from medications (aspirin,
NSAID, steroids) and severe stress (burns, head trauma, Crohn's
disease). Peptic ulcer perforation occurs in almost 12% of children
with peptic ulcer disease. Children with PUD perforation present with
an acute sudden onset painful abdomen, vomiting, nocturnal awakening
and generalized peritonitis with board like abdomen. Almost 90% of
children will show free air under the diaphragm in plain abdominal
films. CT-Scans are more sensitive in establishing the diagnosis of
free or contained perforation. Laparoscopy can be utilized for
diagnosis and management of perforated PUD. Surgical management of
perforated PUD depends on the severity of the disease and extent of
perforation. Most small perforations can be managed with laparoscopic
simple closure using an omental patch. Laparoscopic omental patch is
the easiest, quickest and safest treatment. In complex or large
perforated duodenal ulcers open definitive procedure such as vagotomy
or gastric resections may be required due to larger spillage of GI
content, more sepsis and instability. Postoperatively these patients
will require eradication of Helicobacter pylori if breath test or
biopsy is positive along with proton pump inhibitors. The rate of PUD
recurrence is very high in untreated H. Pylori infection.
References:
1- Hua MC, Kong MS, Lai MW, Luo CC: Perforated peptic ulcer in
children: a 20-year experience.J Pediatr Gastroenterol Nutr.
45(1):71-4, 2007
2- Yadav RP, Agrawal CS, Gupta RK, Rajbansi S, Bajracharya A, Adhikary
S: Perforated duodenal ulcer in a young child: an uncommon condition.
JNMA J Nepal Med Assoc. 48(174):165-7, 2009
3- Coppolino F, Gatta G, Di Grezia G, Reginelli A, Iacobellis F,
Vallone G, Giganti M, Genovese E: Gastrointestinal perforation:
ultrasonographic diagnosis. Crit Ultrasound J. 5 Suppl 1:S4, 2013
4- Schwartz S, Edden Y, Orkin B, Erlichman M: Perforated peptic ulcer
in an adolescent girl. Pediatr Emerg Care. 28(7):709-11, 2012
5- Yildiz T, Ilce HT, Ceran C, Ilce Z: Simple patch closure for
perforated peptic ulcer in children followed by helicobacter pylori
eradication. Pak J Med Sci. 30(3):493-6, 2014
6- Wong CW, Chung PH, Tam PK, Wong KK: Laparoscopic versus open
operation for perforated peptic ulcer in pediatric patients: A 10-year
experience. J Pediatr Surg. 50(12):2038-40, 2015
OEIS Complex
OEIS complex is a very rare group of
malformations which includes omphalocele, exstrophy of cloaca,
imperforate anus and spinal defect. This constellation has also been
labeled exstrophy of the cloaca, exstrophia splanchnica,
vesicointestinal fissure, and ectopic cloaca. It represents the most
severe form of exstrophy-epispadia complex occurring at a rate of one
in 200-400,000 live births. Most cases are sporadic with no obvious
etiology. OEIS complex may be difficult to diagnose prenatally.
Currently there is no clear distinction how OEIS complex occurs during
development. OEIS complex can also be associated with spina bifida,
genital abnormalities, renal malformations, symphysis pubis diastasis
and limb abnormalities. Exstrophy of the cloaca includes the
persistence and exstrophy of a common cloaca that receives ureters,
ileum and a rudimentary hindgut. Terminal myelocystoceles constitute
approximately 5% of skin-covered lumbosacral masses and are especially
common in patients with cloacal exstrophy or the OEIS complex. Most
patients with OEIS complex have a single umbilical artery. Associated
cardiac defects are rare. Brain is normal and most patients have normal
intelligence. Suspicion of OEIS complex prenatally occurs when the US
reveals nonvisualization of the bladder, infra-umbilical anterior
abdominal wall defect, omphalocele and myelomeningocele. Prenatal MRI
is diagnostic of the complex malformation helping plan surgery and
provide family/genetic counseling before birth. Management of OEIS
complex requires and interdisciplinary surgical approach from pediatric
surgery, pediatric urology, orthopedics and neurosurgery. The driving
factor behind surgical correction is to prevent postrenal obstructive
uropathy. Surgical treatment has resulted in improved survival and
quality of life.
References:
1- Ben-Neriah Z, Withers S, Thomas M, Toi A, Chong K, Pai A, Velscher
L, Vero S, Keating S, Taylor G, Chitayat D: OEIS complex: prenatal
ultrasound and autopsy findings. Ultrasound Obstet Gynecol.
29(2):170-7, 2007
2- Sawaya D, Gearhart JP: Gastrointestinal reconstruction and outcomes
for patients with the OEIS complex. Semin Pediatr Surg. 20(2):123-5,
2011
3- Phillips TM, Salmasi AH, Stec A, Novak TE, Gearhart JP, Mathews RI:
Urological outcomes in the omphalocele exstrophy imperforate anus
spinal defects (OEIS) complex: experience with 80 patients. J
Pediatr Urol. 9(3):353-8, 2013
4-Mandrekar SR, Amoncar S, Banaulikar S, Sawant V, Pinto RG:
Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect
(OEIS Complex) with overlapping features of body stalk anomaly (limb
body wall complex). Indian J Hum Genet. 20(2):195-8, 2014
5- Santoro JD, Chao S, Hsieh MH, Lee HC: Multimodality Renal Failure in a Patient with OEIS Complex. AJP Rep. 5(2):e161-4, 2015
6- Allam ES, Shetty VS, Farmakis SG: Fetal and neonatal presentation of OEIS complex. J Pediatr Surg. 50(12):2155-8, 2015
PSU Volume 46 NO 05 MAY 2016
Congenital Pulmonary Airway Malformations
Congenital pulmonary airway
malformations (CPAM) refer to an unusual lesion of the pulmonary
airways which combines features of hamartoma malformation and
dysplastic proliferation. CPAM includes cystic pulmonary airway
malformations, bronchopulmonary sequestration, bronchogenic cysts,
hybrid lesions and lobar/segmental emphysema causing respiratory
distress in 20-40% of affected babies in the postnatal period. The
remaining cases continue asymptomatic or develop symptoms later in life
such as chest infections. Most CPAM can be detected on the 20-week
antenatal ultrasound increasing the diagnostic yield if MRI is
utilized. Children with symptoms early in life are managed with
surgery. The management of asymptomatic CPAM is a source of controversy
in the literature. CPAM is classified 0 to IV. Type 0 is very rare
described as acinar aplasia or agenesis and incompatible with life.
Type I the most common is primarily macrocystic with large single or
multiple cysts several centimeters in size. Type II is microcystic and
associated with other anomalies. Type III appears more solid or with
very small cysts similar to immature lungs without bronchi. Type IV
originates from the acinus and present with small cysts on the
periphery of the lung lobes. Once a cystic lesion is detected in
antenatal ultrasound, the location, volume, size, macrocystic or
microcystic classification and blood supply should be evaluated. CPAM
volume to head circumference ratio (CVR) greater than 1.6 results in
fetal demise in about 80% of cases without fetal intervention. CVR <
1.6 will often not continue to grow past the 28th week of gestation.
The reasons used to remove asymptomatic lesions in the first year of
life include the rate of empyema, abscess, recurrent pneumonia, air
leak, pneumothorax and malignancy. Almost 25% of asymptomatic children
show histologic evidence of infection. CPAM have a long-term risk of
malignancy. Multiple courses of antenatal betamethasone for high-risk
fetal CPAM often results in favorable short-term outcomes without the
need for open fetal resection.
References:
1- Baird R, Puligandla PS, Laberge JM: Congenital lung malformations:
informing best practice. Semin Pediatr Surg. 23(5):270-7, 2014
2- Derderian SC, Coleman AM, Jeanty C, Lim FY, Shaaban AM, Farrell JA,
Hirose S, MacKenzie TC, Lee H: Favorable outcomes in high-risk
congenital pulmonary airway malformations treated
with multiple courses of maternal betamethasone. J Pediatr Surg. 50(4):515-8, 2015
3- Gajewska-Knapik K, Impey L: Congenital lung lesions: Prenatal
diagnosis and intervention. Semin Pediatr Surg. 24(4):156-9, 2015
4- Singh R, Davenport M: The argument for operative approach to
asymptomatic lung lesions. Semin Pediatr Surg. 24(4):187-95, 2015
5- Macardle CA, Ehrenberg-Buchner S, Smith EA, Dillman JR, Mychaliska
GB, Treadwell MC, Kunisaki SM: Surveillance of fetal lung lesions using
the congenital pulmonary airway malformation volume ratio: Natural
history and outcomes. Prenat Diagn. 2015 Dec 29
6- Durell J, Thakkar H, Gould S, Fowler D, Lakhoo K: Pathology of
asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr
Surg. 51(2):231-5, 2016
Cutaneous Schwannomas
Schwannoma is a benign, expansile
tumor that originates from any nerve sheath in the body that contains
Schwann cell. Schwann cells insulate normal nerve fibers and enhance
propagation of nerve impulses. Schwannomas can occur anywhere in the
body along the course of a nerve such as cranial nerves, spinal nerves
or peripheral nerves. As such most schwannomas appear
intracranially, intraspinal or lying deep within soft-tissue. The most
common schwannoma is the acoustic neuroma along the VIII cranial nerve.
Cutaneous schwannomas present as a solitary deep-seated nodule in the
deep dermis or subcutaneous tissue. Most cutaneous schwannomas are
asymptomatic. Other times they may cause pain, tenderness or
paresthesia depending on size, site and nerve involved. Males and
females are equally affected. Histologically, schwannomas are
encapsulated by perineurium and contain either cellular areas
characterized by uniform spindle cell clustered in stack and arranged
back to back, or a loose myxoid matrix. Immunohistochemistry of
schwannomas reveals positive S100 and collagen type 4, and capsule is
positive for epithelial membrane antigen. Schwannomas enlarge slowly
and follow a benign course with very rare cases of malignant
transformation. The preoperative diagnosis of a cutaneous schwannoma is
seldom done as the nodule is usually confused with an epidermal,
trichilemmal or dermoid cyst. Among the other types of schwannomas,
plexiform variety constitute 5% of the presentation associated with
neurofibromatosis. Few reports of malignant transformation in
schwannomas have been reported in the literature. Management of
schwannomas is surgical excision which is usually curative. Local
recurrence can occur and long-term follow up is needed.
References:
1- Agaram NP, Prakash S, Antonescu CR: Deep-seated plexiform
schwannoma: a pathologic study of 16 cases and comparative
analysis with the superficial variety. Am J Surg Pathol. 29(8):1042-8,
2005
2- Attia EA, Yassin M, Lasheen MA, Salem SA, Khafagy NH: Multiple
isolated cutaneous plexiform schwannomas. Indian J Dermatol
Venereol Leprol. 77(5):594-6, 2011
3- Elwood H, Taube J: Dermal and Subcutaneous Plexiform Soft Tissue Neoplasms. Surg Pathol Clin. 4(3):819-42, 2011
4- Noh S, Do JE, Park JM, Jee H, Oh SH: Cutaneous schwannoma presented
as a pedunculated protruding mass. Ann Dermatol. 23(Suppl 2):S264-6,
2011
5- Mohan Kh, Manjunath H: Cutaneous schwannoma masquerading as
trichilemmal cyst over scalp in a young male. Indian J Dermatol.
58(5):407, 2013
6- Kacerovska D, Michal M, Kazakov DV: Hybrid Epithelioid Schwannoma/Perineurioma. Am J Dermatopathol. 2016 Feb 9.
Enteral Refeeding
Many neonates undergo intestinal
resections for a variety of conditions losing a significant segment of
bowel including the construction of proximal enterotomies with distal
mucous fistulas. They rely on parenteral nutrition for a significant
period of time after surgery. Parenteral nutrition is associated with a
significant risk of catheter-related blood stream infection,
thrombosis, and neonatal cholestasis. The absence of food in the GI
tract produces mucosal and villous atrophy with reduction of enzymes
necessary for digestion and substrate absorption. Promoting early
enteral nutrition is beneficial even in the face of a proximal
enterostomy. The presence of a proximal enterostomy can produce high
stomal losses with associated fluid and electrolytes imbalances,
metabolic acidosis and impaired absorption of nutrition. Refeeding of
stomal losses into the distal mucous fistula has been used to
minimized fluid and electrolytes losses as well as dependence on
parenteral nutrition. Mucous fistula refeeding (MFR) stimulates mucosal
growth and intestinal adaptation preventing atrophy of the distal gut.
Enteral refeeding requires substantial nursing expertise, time and
commitment to the process. MFR has also been associated with distal
perforation with bacterial overgrowth in the stomal output if there is
a delay between collection and refeeding of the stoma effluent.
Fluoroscopy can be used to insert the distal refeeding tube to be
utilized taking care to determine if there is thin intestinal wall
propene to perforation or distal strictures in the case of NEC babies.
Neonates with enteral refeeding have a better gain in body weight than
those without it, regardless of the gestational age. Only the MFR
procedure and birth weight were significant independent predictors of a
good weight gain. Babies using MFR have a high rate of central venous
catheter removal because of nutritional improvement. The longer the
distal bowel to be fed the better the rate of body weight
gain.
References:
1- Hay WW Jr: Strategies for feeding the preterm infant. Neonatology. 94(4):245-54, 2008
2- Morgan J, Bombell S, McGuire W: Early trophic feeding versus enteral
fasting for very preterm or very low birth weight infants. Cochrane
Database Syst Rev. Mar 28;3:CD000504, 2013
3- Haddock CA, Stanger JD, Albersheim SG, Casey LM, Butterworth SA:
Mucous fistula refeeding in neonates with enterostomies. J Pediatr
Surg. 50(5):779-82, 2015
4- Wong KK, Lan LC, Lin SC, Chan AW, Tam PK: Mucous fistula refeeding
in premature neonates with enterostomies. J Pediatr Gastroenterol Nutr.
39(1):43-5, 2004
5- Al-Harbi K, Walton JM, Gardner V, Chessell L, Fitzgerald PG: Mucous
fistula refeeding in neonates with short bowel syndrome. J Pediatr
Surg. 34(7):1100-3, 1999
6- Koike Y, Uchida K, Nagano Y, Matsushita K, Otake K, Inoue M,
Kusunoki M: Enteral refeeding is useful for promoting growth in
neonates with enterostomy before stoma closure. J Pediatr Surg.
51(3):390-4, 2016
PSU Volume 46 NO 06 JUNE 2016
Retained Appendicolith
Appendicoliths are formed by calcium
phosphate, inorganic salts and fecal debris layered and lodged within
the appendix lumen that may cause obstruction, inflammation and
perforation of the organ. They are present in up to 30% of children
with appendicitis. When an appendicolith is found extraluminally, it is
pathognomonic for perforation of the appendix. Rarely an appendicolith
may be retained from a perforated appendix or dropped during
appendectomy. Retained appendicolith is a delayed complication
associated after previous removal of the appendix using the
laparoscopic or open surgical technique. Dropped or retained
appendicolith has been reported to occur more frequently in the setting
of laparoscopic surgery compared to open appendectomy due to failure to
recognize and extract the appendicolith during the procedure
Appendicolith retained in the peritoneal cavity after an appendectomy
may serve as a nidus of infection and lead to abscess formation days to
months after surgery. The median time to presentation of the abscess is
1.5 weeks with a mean of two months, though a few reports demonstrate
presentation to occur several years later. Children present most
commonly with focal abdominal pain, fever and leukocytosis. The most
common finding on contrast-enhanced abdominal CT scans is an abscess
with one or more subcentimeter foci of high attenuation adjacent to the
cecum or the Morrison pouch. Retained appendicolith has been reported
in different sites including the pelvis, gluteal region, hepatorenal
pouch (Morrison) and subhepatic region. Every effort must be made to
identify and retrieve appendicoliths at the initial appendectomy. In
cases of retained appendicoliths the definitive management is surgical
removal of the appendicolith as failure to do so may result in
recurrent intra-abdominal abscesses, wound infection and occasionally
fistula formation. Retrieval of the retained appendicolith can be
performed open, laparoscopically and in a few instances percutaneously.
References:
1- Lee SW, Lim JS, Hyung WJ, Park MS, Kim MJ, Cha SW, Yoo HS, Kim KW:
Laparoscopic ultrasonography for localization of a retained
appendicolith after appendectomy. J Ultrasound Med. 25(10):1361-3, 2006
2- Lapus RM, Baker MD: An uncommon late complication of appendicitis. Pediatr Emerg Care. 26(10):757-8, 2010
3- Maatouk M, Bunni J, Schuijtvlot M: Perihepatic abscess secondary to
retained appendicolith: A rare complication managed laparoscopically. J
Surg Case Rep. 2011(1):6-9, 2011
4- Whalley HJ, Remoundos DD, Webster J, Silva MA: Shortness of breath,
fever and abdominal pain in a 21-year-old student. BMJ Case Rep.
14;2013, 2013
5- Black MT, Ha BY, Kang YS, Garland AM: Perihepatic abscess caused by
dropped Appendicolith following laparoscopic appendectomy: sonographic
findings. J Clin Ultrasound. 41(6):366-9, 2013
6- Betancourt SL, Palacio D, Bisset GS 3rd: The 'wandering appendicolith'. Pediatr Radiol. 45(7):1091-4, 2015
Melanonychia
Melanonychia striata or longitudinal
refers to deposition of pigment in the nail plate from increased
pigment production within the nail matrix. Melanonychia is a benign
condition found rarely in children and adults. The deposition can
involve a single longitudinal line or all the nail. The nail plate is
not normally pigment, irrespective of race. Melanonychia is mainly due
to melanin pigment produced by activating or proliferating melanocytes
in the matrix. The problem arises in the differential diagnosis which
includes lentigo, subungual nevus, atypical melanocytic hyperplasia or
melanoma of the nail matrix in which case a biopsy is in order.
Clinical features of melanonychia that increase concern whether this is
a melanoma includes pigment bands broader that 3 mm, changing
pigmentation or shape, associated nail dystrophy, Hutchinson sign,
bands that are not homogenous in color, blurred lateral borders,
irregular lines that are not parallel on dermoscopy and rapid
evolution. History of such changes is an indication for nail matrix
biopsy. Should the biopsy show high melanocyte count, pagetoid spread,
presence of nuclear atypia and/or predominance of single units of
melanocytes this would then favor the diagnosis of melanoma of the nail
matrix. Melanonychia striata does not progress to melanoma, hence the
majority of cases of melanonychia can be managed conservatively without
needing to remove the nail and affected matrix (nail avulsion or
tangential excision). Follow up is needed in cases that are not backup
by biopsy using dermatoscopic discrimination index evaluating changes
in color variegation over time. The reluctance of biopsy is the high
incidence of developing nail dystrophy as complication of the procedure.
References:
1- Murata Y, Kumano K: Dots and lines: a dermoscopic sign of regression
of longitudinal melanonychia in children. Cutis. 90(6):293-6,
301, 2012
2- Richert B, Andre J: Nail disorders in children: diagnosis and management. Am J Clin Dermatol. 12(2):101-12, 2011
3- Tosti A, Piraccini BM, Cagalli A, Haneke E: In situ melanoma of the
nail unit in children: report of two cases in fair-skinned Caucasian
children. Pediatr Dermatol. 29(1):79-83, 2012
4- Richert B, Theunis A, Norrenberg S, Andre J: Tangential excision of
pigmented nail matrix lesions responsible for longitudinal
melanonychia: evaluation of the technique on a series of 30 patients. J
Am Acad Dermatol. 69(1):96-104, 2013
5- Cooper C, Arva NC, Lee C, Yalamos O, Obregon R, Sholl LM, Wagner A,
Shen L, Guitart J, Gerami P: A clinical, histopathologic, and outcome
study of melanonychia striata in childhood. J Am Acad Dermatol.
72(5):773-9, 2015
6- Koga H, Yoshikawa S, Shinohara T, Le Gal FA, Cortes B, Saida T, Sota
T: Long-term Follow-up of Longitudinal Melanonychia in Children and
Adolescents Using an Objective Discrimination Index. Acta Derm
Venereol. Jan 25, 2016
Central Venous Catheter Repair
Central venous catheters (CVC) are
essential for providing prolonged enteral nutrition, chemotherapy and
medications in critically and chronically ill pediatric patients. In
cases of gut failure or absence they are the primordial way to provide
nutrition for extended period of time. They create the venue for
outpatient management for many ailments in children. As foreign body
inserted into the body they can get infected, thrombosed, dislodge or
suffer mechanical complications such as breakage needing frequent
change in the life of patients using them as life supporting. Implanted
CVC should be avoided in children with neutropenia (absolute neutrophil
count less than 0.5 x 109/L) due to the increase incidence of
bloodstream infection. In pediatric patients the rate of breakage of
the external portion of the catheter is one per 1000 catheter-days.
When breakage occurs the surgeon has the choice of repairing the
catheter or replace it depending on surgical risk and patent available
veins. Repairs are performed using the kits sold by the manufacturers
of most CVC. Repair can be done using glue after cutting the break site
and placing a plastic sheath slide over the new union site to splint
the repair, or without glue with an extension catheter with adaptor or
connector piece lock into place into the original catheter. A break in
the CVC that breaches the lumen is expected to compromise its
sterility. Repair and retention of that catheter could predispose the
patient to a 2-4 fold higher risk of bloodstream infection in the 30
days following repair. Age, underlying diagnosis, immunocompromised
state, parenteral nutrition, catheter type or repair type modified the
effect of catheter break and repair on the risk of bacteremia and
infection. To stir further the controversy, in gut failure children
repairing CVC increases line longevity and preserves the use of limited
vascular access sites avoiding early transplants due to absent vascular
access. Also, in gut failure cases repairing CVC was not associated
with an increase rate of line infection.
References:
1- Moukarzel AA, Haddad I, Ament ME, Buchman AL, Reyen L, Maggioni A,
Baron HI, Vargas J: 230 patient years of experience with home long-term
parenteral nutrition in childhood: natural history and life of central
venous catheters. J Pediatr Surg. 1994 29(10):1323-7, 1994
2- Elihu A, Gollin G: Complications of implanted central venous catheters in neutropenic children. Am Surg. 73(10):1079-82 2007
3- Milbrandt K, Beaudry P, Anderson R, Jones S, Giacomantonio M,
Sigalet D: A multiinstitutional review of central venous line
complications: retained intravascular fragments. J Pediatr Surg.
44(5):972-6, 2009
4- Abu-El-Haija M, Schultz J, Rahhal RM: Effects of 70% ethanol locks
on rates of central line infection, thrombosis, breakage, and
replacement in pediatric intestinal failure. J Pediatr
Gastroenterol Nutr. 58(6):703-8, 2014
5- Lundgren IS, Zhou C, Malone FR, McAfee NG, Gantt S, Zerr DM: Central
venous catheter repair is associated with an increased risk of
bacteremia and central line-associated bloodstream infection in
pediatric patients. Pediatr Infect Dis J. 31(4):337-40, 2012
6- McNiven C, Switzer N, Wood M, Persad R, Hancock M, Forgie S,
Dicken BJ: Central venous catheter repair is not associated with an
increased risk of central line infection or colonization in intestinal
failure pediatric patients. J Pediatr Surg. 51(3):395-7, 2016