PEDIATRIC SURGERY UPDATE ©
VOLUME 42, 2014
PSU Volume 42 No 01 JANUARY 2014
Wilms in HSK
Wilms tumor (WT) is regarded as the most common malignant renal
tumor in children. Several anomalies/syndromes are associated with WT,
this includes: aniridia, hemihypertrophy, cryptorchidism, hypospadia,
ectopia, duplication and horseshoe kidney (HSK). HSK is a common renal
anomaly where the right and left kidneys are fused. HSK has a tendency
toward neoplasia such as hypernephroma and WT. WT develops in HSK as a
result of sequestered metanephric blastemas in the isthmus, which is
known to harbor malignant potential. Most cases present with an
asymptomatic abdominal mass. Almost in one-third of children with WT
and HSK the diagnosis is not made preoperatively. Salient features in
diagnosis using CT-Scan of WT in HSK include malrotation of the
kidney, hydronephrosis, and the presence of an isthmus. HSK are
normally situated lower than normal kidneys and have an anomalous blood
supply with 4-6 renal arteries supplying the HSK. The blood supply to
the isthmus may come from the renal artery, aorta WT in HSK is usually
managed as WT in a solitary kidney and benefits from preoperative
chemotherapy since a more planned and conservative procedure can be
performed later, the surgical morbidity associated with tumor spillage
and incomplete resection is reduced and more renal function is
preserved. CT-reconstructed angiography is helpful in planning the
final excision and mapping the blood supply in WT arising from HSK.
During resection if the tumor involves one kidney in HSK the functional
isthmus has to be resected along with the tumor to avoid a urinary
fistula. If the tumor arises from the isthmus, isthmusectomy with
bilateral lower pole nephrectomy is needed. Annual surveillance of
children with HSK looking for WT development is not recommended.
Estimated four-year survival of WT in HSK is above 80%.
References:
1- Lee SH, Bae MH, Choi SH, et al: Wilm's Tumor in a Horseshoe Kidney. Korean J Urol 53: 577-580, 2012
2- Yeluri SV, Duttaroy DD, Ghodgaonkar P, Karanth S: Wilm's Tumor
arising in a horseshoe kidney. Indian J Med Science. 58(2): 72-73, 2004
3- Huang EY, Mascarenhas L, Mahour GH: Wilms' tumor and horseshoe
kidneys: a case report and review of the literature. J Pediatr Surg.
39(2):207-12, 2004
4- Neville H, Ritchey ML, Shamberger RC, Haase G, Perlman S, Yoshioka
T: The occurrence of Wilms tumor in horseshoe kidneys: a report from
the National Wilms Tumor Study Group (NWTSG). J Pediatr Surg.
37(8):1134-7, 2002
5- Campaini C, Kubheka KR: Nephroblastoma in horseshoe kidney. Cent Afr J Med. 48(7-8):92-4, 2002
6- Talpallikar MC, Sawant V, Hirugade S, Borwankar SS, Sanghani H:
Wilms' tumor arising in a horseshoe kidney. Pediatr Surg Int.
17(5-6):465-6 , 2001
7- Kapur VK, Sakalkale RP, Samuel KV, Meisheri IV, Bhagwat AD,
Ramprasad A, Waingankar VS: Association of extrarenal Wilms' tumor with
a horseshoe kidney. J Pediatr Surg. 33(6):935-7, 1998
Epididymal Cysts
Epididymal cysts (EC) are benign lesions diagnosed during
evaluation of scrotal symptoms of pain, fullness or palpable scrotal
masses in children and adolescents. The etiology of epididymal cysts is
probably a congenital anomaly related to hormonal disorders during
embryonic life, the result of maternal exposure to diethylstilbestrol
or part of the testicular dysgenesis syndrome. An association of
epididymal cysts with cryptorchidism, cystic fibrosis and von
Hippel-Lindau disease has also been reported. Average age of appearance
of EC in children is ten to fifteen years. Physical examination is very
important, but not sufficient for the diagnosis and must be completed
by scrotal ultrasonography, which shows an echo-free cystic epididymal
structure. Associated findings in children with EC include hydrocele,
varicocele and testicular microlithiasis. An association of EC with
increased testicular size is also observed. EC occurs anywhere within
the epididymis and does not contain sperm. Cyst size and symptoms play
a role in deciding the choice of management. Some workers suggest
conservative management for cyst smaller than 10 mm, while surgery
should be reserved for cysts larger than 10 mm in diameter, but this
fact dose not take into account the symptoms of the child. Conservative
management of asymptomatic EC with serial ultrasound follow-up is
recommended since most EC involutes with time. Children with
intractable scrotal pain or cyst enlargement should undergo surgery.
Surgical management consists of cyst excision through a scrotal
approach.
References:
1- Jelloul L, Billerey C, Ait Ali Slimane M, Mboyo A, Aubert D:
[Epididymal cysts in adolescents]. Ann Urol (Paris). 33(2):104-8, 1999
2- Homayoon K, Suhre CD, Steinhardt GF: Epididymal cysts in children: natural history. J Urol. 171(3):1274-6, 2004
3- Chillon Sempere FS, Dominguez Hinarejos C, Serrano Durba A, Estornell Moragues F,
Martinez-Verduch M, Garcia Ibarra F: [Epididymal cysts in childhood]. Arch Esp Urol. 58(4):325-8, 2005
4- Posey ZQ, Ahn HJ, Junewick J, Chen JJ, Steinhardt GF: Rate and
associations of epididymal cysts on pediatric scrotal ultrasound. J
Urol. 184(4 Suppl):1739-42, 2010
5- Niedzielski J, Miodek M, Krakas M: Epididymal cysts in childhood -
conservative or surgical approach? Pol Przegl Chir. 84(8):406-10, 2012
6- Erikci V, Hosgor M, Aksoy N, Okur O, Yildiz M, Dursun A, Demircan Y,
Ornek Y, Genisol I: Management of epididymal cysts in childhood.
J Pediatr Surg. 48(10):2153-6, 2013
Genitofemoral Nerve Injury
The genitofemoral nerve (GFN) arises from the L1 and L2 nerve
roots. The genital branches of the GFN consist of both motor and
sensory fibers Motor fibers to the cremasteric muscle provide
thermoregulation for the testes where sensory fibers supply a portion
of the scrotal skin and upper thigh. The genital branch of the GFN
passes through the abdominal inguinal ring and descends behind the
spermatic cord to the scrotum. The femoral branch of the GFN is located
caudad and lateral to the genital branch and travels on the anterior
surface of the external iliac artery under the inguinal ligament to
supply the skin of the mid-anterior thigh. There exists definitive risk
of injuring the GFN during open inguinal hernia repair, appendectomy
and laparoscopic varicocelectomy in children and adults. Mesh grafts,
sutures and scar may injure the GFN during herniorrhaphy. In fact the
constant bulging hernial sac by itself might be a cause of GFN
entrapment and subtle injury before surgery. Injury to the GFN may
present with pain radiating from the lower abdomen to the anterior
thigh and labia majora in women and the scrotum in men. Postsurgical
chronic pain after hernia repair may be caused by injury to the
iliohypogastric, ilioinguinal or genitofemoral nerves. Is sometimes
difficult to identify the specific nerve injury that is giving rise to
a patient's post herniorrhaphy neuritic symptoms because these nerves
are derived from overlapping nerve roots and closely localize in the
area of surgery. Blocking the trunk nerve proximal to the site of
injury by local anesthetic will help identify the nerve involved and
provide temporary relief. Once identified the origin of persistent pain
more permanent invasive treatment can be instituted using surgery,
stimulation or percutaneous pulse radiofrequency.
References:
1- Nahabedian MY, Dellon AL: Outcome of the operative management of
nerve injuries in the ilioinguinal region. J Am Coll Surg.
184(3):265-8, 1997
2- Rosenberger RJ, Loeweneck H, Meyer G: The cutaneous nerves
encountered during laparoscopic repair of inguinal hernia: new
anatomical findings for the surgeon. Surg Endosc. 14(8):731-5, 2000
3- Muensterer OJ: Genitofemoral nerve injury after laparoscopic varicocelectomy in adolescents. J Urol. 180(5):2155-7, 2008
4- Soyer T, Tosun A, Keles I, Inal E, Cesur O, Cakmak M:
Electrophysiologic evaluation of genitofemoral nerve in children with
inguinal hernia repair. J Pediatr Surg. 43(10):1865-8, 2008
5- Parris D, Fischbein N, Mackey S, Carroll I: A novel CT-guided
transpsoas approach to diagnostic genitofemoral nerve block and
ablation. Pain Med. 11(5):785-9, 2010
6- Bischoff JM, Aasvang EK, Kehlet H, Werner MU: Does nerve
identification during open inguinal herniorrhaphy reduce the risk of
nerve damage and persistent pain?Hernia. 16(5):573-7, 2012
PSU Volume 42 NO 02 FEBRUARY 2014
SIRS
SIRS, refers to the Systemic
Inflammatory Response Syndrome, a stress response defined by
abnormalities of temperature, heart rate, respiratory rate and
peripheral WBC. Two or more of the following disturbances make the
diagnosis of SIRS: Core temperature above 38.5 or below 36 C;
tachycardia defined as mean heart rate above two standard deviations
above normal for age or bradycardia defined as mean heart rate less
than the 10th percentile for age; tachypnea defined as mean respiratory
rate above two standard deviations for age; leukocyte count elevated or
depressed for age or 10% immature neutrophils. The pediatric group is
divided in six clinically and physiologically meaningful age groups for
age-specific vital sign and laboratory variables to meet SIRS criteria.
There is a mandatory requirement for abnormality of temperature or
leukocyte count to be present for a diagnosis of SIRS be considered.
Since SIRS plus infection equal sepsis it is crucial to recognize SIRS
in its initial phase to start adequate management. Multiple
initializing triggers SIRS such as infection, trauma and surgery. Most
pediatric cases of SIRS occur in the 2-5 year age group with a median
age of 30 months. Severe sepsis refers to acute organ dysfunction or
tissue hypoperfusion secondary to infection and septic shock is severe
sepsis plus hypotension not reversed with fluid resuscitation. There is
a high risk of developing sepsis in children with SIRS. Early SIRS
diagnosis eventually leads to early goal-therapy for sepsis. Pediatric
appendicitis that presents initially with SIRS have a higher length of
stay and risk incidence of developing intraabdominal abscess. Systemic
organ failure and intestinal dysfunction are strong risk factors for
postoperative SIRS in children. These preexisting conditions lead to
disruption of normal intestinal flora or barrier function predisposing
children to dramatic SIRS episodes after intestinal surgery.
References:
1- Dellinger RP, Levy MM, Rhodes A, et al: Surviving Sepsis Campaign:
International Guidelines for Management of Severe Sepsis and Septic
Shock: 2012. Critical Care Medicine. 41(2): 580-637, 2013
2- Pavare J, Grope I, Gardovska D: Prevalence of systemic inflammatory
response syndrome (SIRS) in hospitalized children: a point prevalence
study. BMC Pediatr. 9:25, 2009
3- Juskewitch JE, Prasad S, Salas CF, Huskins WC: Reliability of the
identification of the systemic inflammatory response syndrome in
critically ill infants and children. Pediatr Crit Care Med.
13(1):e55-7, 2012
4- Wheeler DS, Jeffries HE, Zimmerman JJ, Wong HR, Carcillo JA: Sepsis
in the pediatric cardiac intensive care unit. World J Pediatr Congenit
Heart Surg. 2(3):393-9, 2011
5- Chawla BK, Teitelbaum DH: Profound systemic inflammatory response
syndrome following non-emergent intestinal surgery in children. J
Pediatr Surg. 48(9):1936-40, 2013
6- Raines A, Garwe T, Wicks R, Palmer M, Wood F, Adeseye A, Tuggle D:
Pediatric appendicitis: The prevalence of systemic inflammatory
response syndrome upon presentation and its association with clinical
outcomes. J Pediatr Surg. 48(12):2442-5, 2013
Splenoma
The term splenoma refers to a splenic
hamartoma. Splenoma is a very rare and benign lesion in the pediatric
age. Approximately 80% of splenic hamartomas produce no clinical
symptoms. Splenic hamartomas may be solitary or multiple and are
usually well defined but not encapsulated. There is no predilection for
sex or age. Most patients with splenic hamartomas are asymptomatic and
found as an incidental finding at laparotomy or autopsy. A few splenic
hamartomas have clinical findings. Common clinical findings include
bleeding tendency caused by thrombocytopenia, digestive symptoms as
abdominal pain and loss of appetite from compressive symptoms.
Spontaneous rupture with bleeding has been reported. In children,
hamartoma of the spleen with haematologic abnormalities may be followed
by growth retardation, frequent infections, fever and night
sweating. Histologically splenic hamartomas can be classified as
red pulp hamartomas when they contain sinuses and structures equivalent
to the pulp cords, white hamartomas when they contain mostly lymphoid
tissue, and mixed type when they have a mixture of the above.
Ultrasound characteristic of splenic hamartomas include hyperechoic
nodules with a cystic component. CT-Scan will need intravenous contrast
material to see the difference between the hamartoma seen as a solid
homogenous mass without calcification and the normal splenic tissue.
Radiocolloid scintigraphy demonstrates uptake within the lesion, but
less than that of normal spleen. In MRI imaging the tumor is isointense
relative to normal splenic tissue on T1-weighted and of increased
intensity with T2 weighting. The management of symptomatic splenic
hamartoma is excision by either total or partial splenectomy depending
on the size, age and splenic involvement.
References:
1- Havlik RJ, Touloukian RJ, Markowitz RI, Buckley P: Partial
splenectomy for symptomatic splenic hamartoma. J Pediatr Surg.
25(12):1273-5, 1990
2- Thompson SE, Walsh EA, Cramer BC, Pushpanathan CC, Hollett P, Ingram
L, Price D: Radiological features of a symptomatic splenic hamartoma.
Pediatr Radiol. 26(9):657-60, 1996
3- Hayes TC, Britton HA, Mewborne EB, Troyer DA, Saldivar VA, Ratner
IA: Symptomatic splenic hamartoma: case report and literature review.
Pediatrics. 101(5):E10, 1998
4- Yoshizawa J, Mizuno R, Yoshida T, Kanai M, Kurobe M, Yamazaki Y:
Spontaneous rupture of splenic hamartoma: a case report. J Pediatr
Surg. 34(3):498-9, 1999
5- Abramowsky C, Alvarado C, Wyly JB, Ricketts R: "Hamartoma" of the
spleen (splenoma) in children. Pediatr Dev Pathol. 7(3):231-6, 2004
6- Basso SM, Sulfaro S, Marzano B, Fanti G, Chiara GB, Lumachi F: Incidentally
discovered asymptomatic splenic hamartoma with rapidly expansive
growth: a case report. In Vivo. 2012 Nov-Dec;26(6):1049-52, 2012
Transport Surgical Neonate
Transporting a neonate with a surgical
condition from one institution to another involve pre-transport
intensive care level of resuscitation, stabilization and continuing
transport to ensure the baby arrives in stable condition. Good
communication and coordination between referring and receiving hospital
is of upmost importance. Transport can occur in-utero, by road or air
(helicopter or airline). In-utero is the best mode of transfer to a
facility that has NICU with working pediatric surgeons. High-risk
neonates are more likely to survive when they are delivered in a
perinatal center compared with local delivery followed by transfer.
When using air transport physiologic changes due to altitude and
decreased atmospheric pressure can cause a decrease in oxygen
concentration and expansion of gases causing increased oxygen
requirement and tachypnea. This issue is important in patients with air
trapped in closed cavities such as pneumothorax, pneumoperitoneum,
volvulus and bowel obstruction. Before leaving these cavities must be
drained since gases will expand and cause respiratory distress. Should
there be a possibility the child might require ventilation during
transfer it is safer to electively intubate and ventilate before
leaving the source institution. If the baby is unstable or on high
ventilatory setting, the baby should not be transported. During
transport all tubes should be secured safely, and running IVF's with
medication should be working properly. Each institution should have a
pre-departure checklist with the essential transport equipment and
medication available. In cases of NEC if there is an evident
perforation of the bowel, insertion of a peritoneal drain with or
without lavage with normal saline should be considered to improve
ventilation and acidosis.
References:
1- Tholkes DR: Air transport of the neonate with a congenital diaphragmatic hernia. Aviat Space Environ Med. 57(2):183-5, 1986
2- Cornish JD, Carter JM, Gerstmann DR, Null DM Jr: Extracorporeal
membrane oxygenation as a means of stabilizing and transporting high
risk neonates. ASAIO Trans. 37(4):564-8, 1991
3- Bjerke HS, Barcliff L, Foglia RP: Neonatal survival during a 2,500-mile flight. Hawaii Med J. 1992 Dec;51(12):332-5.
4- Bergman KA, Geven WB, Molendijk A: Referral and transportation for
neonatal extracorporeal membrane oxygenation. Eur J Emerg Med.
9(3):233-7, 2002
5- McAdams RM, Dotzler SA, Pole GL, Kerecman JD: Long-distance air
medical transport of extremely low birth weight infants with
pneumoperitoneum. J Perinatol. 28(5):330-4, 2008
6- Cabrera AG, Prodhan P, Cleves MA, Fiser RT, Schmitz M, Fontenot E,
McKamie W, Chipman C, Jaquiss RD, Imamura M: Interhospital transport of
children requiring extracorporeal membrane oxygenation support for
cardiac dysfunction. Congenit Heart Dis. 6(3):202-8, 2011
PSU Volume 42 No 03 MARCH 2014
Component Separation Technique
The component separation technique
(CST) was initial developed in 1990 to repair primary large ventral
hernias defects without using mesh or prothesis. CST was originally
described as incising the external oblique aponeurosis and releasing
the external oblique from the internal oblique. This release leads to
improved tissue mobility and decreases tension allowing fascial
approximation closure either to be done primarily or intercalating a
mesh when the defect is very large. The CST does not compromise the
innervation and blood supply of the muscles. The procedure is performed
in adults to repair large giant ventral hernias with lost of abdominal
domain. The procedure is rarely used in children. In short the
technique encompass identifying the borders of the hernial defect,
creating bilateral tissue flaps (skin and subcutaneous tissue) until
the external oblique aponeurosis is exposed, bilateral longitudinal
incision of the external oblique fascia one centimeter lateral to the
rectus muscle dividing the external oblique muscle from the internal
oblique muscle until the midline can be approximated with minimal
tension. At this moment of closure the surgeon might use onlay or
underlay biologic mesh to support the midline closure. The mesh can
alleviate further the tension that exists with the hernia repair and
provide a scaffold for cellular growth. In adults with large ventral
defects the risk of recurrence is decreased when the CST closure is
supported by either mesh onlay or underlay. In children, CST can be
utilized for large ventral hernia defects after initial management of
abdominal wall defects such as omphalocele and gastroschisis. The CST
offers the potential for definitive repair as a newborn as well as
avoidance of the morbidity associated with recurrent hernias and
multiple operations. Complications associated with the CST include
wound infection, seroma, hematoma or skin
necrosis.
References:
1- Jernigan TW, Fabian TC, Croce MA, Moore N, Pritchard FE,
Minard G, Bee TK: Staged management of giant abdominal wall defects:
acute and long-term results. Ann Surg. 238(3):349-55, 2003
2- de Vries Reilingh TS, van Goor H, Charbon JA, Rosman C, Hesselink
EJ, van der Wilt GJ, Bleichrodt RP: Repair of giant midline abdominal
wall hernias: "components separation technique"
versus prosthetic repair : interim analysis of a randomized controlled trial. World J Surg. 31(4):756-63, 2007
3- van Eijck FC, de Blaauw I, Bleichrodt RP, Rieu PN, van der Staak FH,
Wijnen MH, Wijnen RM: Closure of giant omphaloceles by the abdominal
wall component separation technique in infants. J Pediatr Surg.
43(1):246-50, 2008
4- Beres A, Christison-Lagay ER, Romao RL, Langer JC: Evaluation of
Surgisis for patch repair of abdominal wall defects in children. J
Pediatr Surg. 47(5):917-9, 2012
5- Heller L, McNichols CH, Ramirez OM: Component separations. Semin Plast Surg. 26(1):25-8, 2012
6- Levy S, Tsao K, Cox CS Jr, Phatak UR, Lally KP, Andrassy RJ:
Component separation for complex congenital abdominal wall defects: not
just for adults anymore. J Pediatr Surg. 48(12):2525-9, 2013
Immune Thrombocytopenic Purpura
Immune thrombocytopenia purpura (ITP)
is an acquired autoimmune disease characterized by platelet destruction
due to antiplatelet autoantibodies. The reticuloendothelial system
engulfs the affected platelets causing early destruction hence
thrombocytopenia. In children is self-limited with a 6-month remission
rate in more than 70% of cases. After six months the condition is
termed chronic ITP and it occurs in 20% of all cases. Clinically mean
age at onset is six years, males and females are equally
affected, and the disease usually follows an infection. Most children
present with simple petechiae and bruising symptoms. Most children with
ITP experience spontaneous resolution without medication. Severe
bleeding is more likely in children with severe thrombocytopenia from
ITP. First-line therapy for symptomatic and/or severely
thrombocytopenic patients include steroids, anti-D antibody, or
intravenous gamma globulin. Second-line therapy is reserved for
those who have severe bleed or are refractory to first-line medical
therapy and include high-dose steroids, rituximab, and other
chemotherapeutics. Laparoscopic splenectomy is a safe, efficacious, and
cost-effective strategy option for children with uncontrolled chronic
ITP or life-threatening hemorrhage complicating acute ITP.
Unfortunately there is no clear prediction which patient will benefit
from splenectomy. Almost 70% of cases respond favorably and
persistently to splenectomy. Steroid-resistant children are most likely
to have complete respond to splenectomy. Older age, longer duration of
ITP, and male gender correlates with complete response.
Splenectomy improves quality of life of children with ITP. Intracranial
hemorrhage is a devastating complication of ITP preceded by headache
and mucosal bleeding which can be precipitate in the event of aspirin
intake, extreme exercise, head trauma or a congenital vascular cerebral
lesion.
References:
1- Vianelli N, Galli M, de Vivo A, Intermesoli T, Giannini B,
Mazzucconi MG, Barbui T, Tura S, Baccaranion M; Gruppo Italiano
per lo Studio delle Malattie Ematologiche dell'Adulto: Efficacy and
safety of splenectomy in immune thrombocytopenic purpura:
long-term results of 402 cases. Haematologica. 90(1):72-7, 2005
2- Kahne T, Blanchette V, Buchanan GR, Ramenghi U, Donato H, Tamminga RY, Rischewski
J, Berchtold W, Imbach P; Intercontinental Childhood ITP Study Group:
Splenectomy in children with idiopathic thrombocytopenic purpura: A
prospective study of 134 children from the Intercontinental Childhood
ITP Study Group. Pediatr Blood Cancer. 49(6):829-34, 2007
3- Psaila B, Petrovic A, Page LK, Menell J, Schonholz M, Bussel JB:
Intracranial hemorrhage (ICH) in children with immune thrombocytopenia
(ITP): study of 40 cases. Blood. 26;114(23):4777-83, 2009
4- Wood JH, Partick DA, Hays T, Ziegler MM: Predicting response to
splenectomy in children with immune thrombocytopenic purpura. J Pediatr
Surg. 45: 140-144, 2010
5- Hollander LL, Leys CM, Weil BR, Rescorla FJ: Predictive value of
response to steroid therapy on response to splenectomy in children with
immune thrombocytopenic purpura. Surgery. 150(4):643-8, 2011
6- Gwilliam NR, Lazar DA, Brandt ML, Mahoney Jr DH, Wesso DE, Mazziotti
MV, Nuchtern JG, Lee TC: An analysis of outcomes and treatment costs
for children undergoing splenectomy for chronic immune thrombocytopenia
purpura. J Pediatr Surg. 47: 1537-1541, 2012
Lipofibromatosis
Lipofibromatosis (LF) is a rare benign
fibrofatty tumor of childhood largely composed of adipose tissue
traversed by bundles of spindle fibroblastic-like cells. Most
lipofibromatosis are found in the distal extremity. Clinically, LF
presents as a slow growing painless mass with a propensity to affect
the hands and feet. The lesion is subcutaneous in location or in the
deep soft tissue with poorly demarcated margins. The lesion usually
measures one to 3 cm, with a median size of 2 cm. LF can infiltrate
adjacent structures like vessels, nerves and muscle. Functional
impairment is very rare. The findings at MRI are nonspecific, but it
can evaluate the extension of the lesion and involvement of adjacent
structures. Hyperintense signal on T1W and T2W images with loss of
signal on fat-saturated images confirm the lipomatous content of the
lesion. Diagnosis can be made easily with distinctive histopathologic
features without an indication of immunohistochemistry. Mitotic rate is
low and there is usually no cellular atypia. Management of LF is
complete surgical excision if at all feasible. Due to its infiltrative
nature, LF has a high rate of recurrence. The following events were
more common in the group with recurrent or persistent disease:
congenital onset, male sex, hand and foot location, incomplete
excision, and mitotic activity in the fibroblastic element.
References:
1- Fetsch JF, Miettinen M, Laskin WB, Michal M, Enzinger FM: A
clinicopathologic study of 45 pediatric soft tissue tumors with an
admixture of adipose tissue and fibroblastic elements, and a proposal
for classification as lipofibromatosis. Am J Surg Pathol.
24(11):1491-500, 2000
2- Sari A, Tunakan M, Bolat B, Cakmakai H, Ozer E: Lipofibromatosis in
a two-year-old girl: a case report. Turk J Pediatr. 49(3):319-21, 2007
3- Taran K, Woszczyk M, Kobos J: Lipofibromatosis presenting as a neck
mass in eight-years old boy--a case report. Pol J Pathol. 59(4):217-20,
2008
4- Costa Dias S, McHugh K, Sebire NJ, Bulstrode N, Glover M, Michalski
A: Lipofibromatosis of the knee in a 19-month-old child. J Pediatr
Surg. 47(5):1028-31, 2012
5- Leuschner I: [Lipofibromatosis in a 6-year-old girl: a case report]. Pathologe. 31(2):150-2, 2010
PSU Volume 42 No 04 APRIL 2014
Long-Gap Esophageal Atresia
Long gap in esophageal
atresia (EA), a major surgical challenge considered when an anatomic
gap of more than three centimeters (or three vertebral bodies) is
identified between the two stumps of the esophagus precluding a safe
anastomosis. Long gap is more commonly seen in pure esophageal atresia
not associated with a tracheoesophageal fistula. Surgeons have also
used this term in cases of EA with distal tracheoesophageal
fistula when an anastomosis is not possible. No single technique to
accomplish this goal is effective in all patients. When confronted with
this situation surgeons should reproduce several alternatives of
management with the central caveat that the baby own esophagus should
be preserved and is far superior to an esophageal replacement
technique. In cases of long gap with pure EA initial management consist
of a gastrostomy for two purposes: enteral feeding and future imaging
studies to determine the esophageal gap. Should the gap be less than 2
cm a primary anastomosis can be accomplished safely. Some of the
commonly used technique for long gap approximation includes proximal
esophageal dilatation with distal gastric bolus feedings in an effort
to exert pressure on both stumps for spontaneous lengthening, a
technique that takes to much time to accomplish its goal. This has
prompted surgeons to developed faster technique such as sequential
proximal pouch elongation (Kimura advancement) and mechanical
stretching of both stumps with sutures (Foker). Still other techniques
when the distal stump is extremely small encompassed isoperistalsis
tubularization of a piece of stomach (Scharli) to include the
esophageal stump, and creating concomitant antireflux valve procedures
(Collis-Nissen). Finally, replacement of the defect with stomach
(gastric transposition), small bowel or colon is a last resort
technique when all other means fails.
References:
1- Stringel G, Lawrence C, McBride W: Repair of long gap esophageal
atresia without anastomosis. J Pediatr Surg. 45(5):872-5, 2010
2- Nakahara Y, Aoyama K, Goto T, Iwamura Y, Takahashi Y, Asai T:
Modified Collis-Nissen procedure for long gap pure esophageal atresia.
J Pediatr Surg. 47(3):462-6, 2012
3- Friedmacher F, Puri P: Delayed primary anastomosis for management of
long-gap esophageal atresia: a meta-analysis of complications and
long-term outcome. Pediatr Surg Int. 28(9):899-906, 2012
4- Nasr A, Langer JC: Mechanical traction techniques for long-gap esophageal atresia: a critical
appraisal. Eur J Pediatr Surg. 23(3):191-7, 2013
5- Miyano G, Okuyama H, Koga H, Okawada M, Doi T, Takahashi T, Nakamura
H, Suda K, Lane GJ, Okazaki T, Yamataka A: Type-A long-gap esophageal
atresia treated by thoracoscopic esophagoesophagostomy after sequential
extrathoracic esophageal elongation (Kimura's technique). Pediatr Surg
Int. 29(11):1171-5, 2013
6- Bairdain S, Ricca R, Riehle K, Zurakowski D, Saites CG, Lien C,
Anderson GF, Wahoff DC, Linden BC: Early results of an objective
feedback-directed system for the staged traction repair of long-gap
esophageal atresia. J Pediatr Surg. 48(10):2027-31, 2013
7- Beasley SW, Skinner AM: Modified Scharli technique for the very long
gap esophageal atresia. J Pediatr Surg. 48(11):2351-3, 2013
Embryonal Liver Sarcoma
Undifferentiated
embryonal sarcoma of the liver (UESL) is an uncommon malignant hepatic
tumor of mesenchymal origin in children. UESL accounts for almost 5% of
all pediatric malignant hepatic tumors occurring mainly between five
and 10 years of age without a gender predilection. Clinical
presentation is typically an abdominal mass that may be accompanied by
pain and systemic symptoms such as fever, weight loss or vomiting. Due
to its high malignant potential UESL often metastasizes to the lung,
peritoneum and pleura resulting in a poor prognosis. The tumor presents
as a large (above 10 cm) solitary solid mass mainly localized in the
right hepatic lobe with a solitary clear boundary. US reveals a mixed
echoic mass containing an irregular anechoic region with multiple small
capsular spaces. CT reveals cystic lesions due to hemorrhage, necrosis
and liquefaction. Diagnosis requires biopsy. Microscopically the tumor
consists of rather loose foci of pleomorphic, stellate or giant cells
with hyperchromatic nuclei, and numerous mitosis. Immunohistochemistry
of the tumor shows positive expression of SMA, a-ACT, desmin, vimentin
and actin, while Alpha fetoprotein, CEA, CA 19-9 and cytokeratin are
negative. Rarely UESL is misdiagnosed as hepatoblastoma. The key
management of UESL is complete surgical resection followed by
postoperative multiagent chemotherapy. Very large tumors can be managed
with preop chemotherapy followed by surgery. Hepatic transplantation is
an effective therapeutic method for UESL patients whose tumor
unresectable or those who have postoperative recurrence of the
tumor. Prognosis has improved significantly over the years.
References:
1- Bisogno G, Pilz T, Perilongo G, Ferrari A, Harms D, Ninfo V, Treuner
J, Carli M:Undifferentiated sarcoma of the liver in childhood: a
curable disease. Cancer. 94(1):252-7, 2002
2- Chowdhary SK, Trehan A, Das A, Marwaha RK, Rao KL: Undifferentiated
embryonal sarcoma in children: beware of the solitary liver cyst.
J Pediatr Surg. 39(1):E9-12, 2004
3- Wei ZG, Tang LF, Chen ZM, Tang HF, Li MJ: Childhood undifferentiated
embryonal liver sarcoma: clinical features and immunohistochemistry
analysis. J Pediatr Surg. 43(10):1912-9, 2008
4- Plant AS, Busuttil RW, Rana A, Nelson SD, Auerbach M, Federman NC: A
single-institution retrospective cases series of childhood
undifferentiated embryonal liver sarcoma (UELS): success of combined
therapy and the use of orthotopic liver transplant. J Pediatr Hematol
Oncol. 35(6):451-5, 2013
5- Gao J, Fei L, Li S, Cui K, Zhang J, Yu F, Zhang B: Undifferentiated
embryonal sarcoma of the liver in a child: A case report and review of
the literature. Oncol Lett. 5(3):739-742, 2013
6- Geel JA, Loveland JA, Pitcher GJ, Beale P, Kotzen J, Poole JE:
Management of undifferentiated embryonal sarcoma of the liver in
children: a case series and management review. S Afr Med J.
103(10):728-31, 2013
7- Ismail H, Dembowska-Baginska B, Broniszczak D, Kalicinski P,
Maruszewski P, Kluge P, Swieszkowska E, Kosciesza A, Lembas A, Perek D:
Treatment of undifferentiated embryonal sarcoma of the liver in
children--single center experience. J Pediatr Surg.
48(11):2202-6, 2013
Trachea Diverticulum
Tracheal diverticulum is a very rare condition with a
congenital or acquired origin. Congenital tracheal diverticulum is even
rarer, smaller, located approximately 4–5 cm below vocal cords or
just above the carina, is more common in males and can be considered as
a supernumerary malformed branch of the trachea. Most reported cases of
tracheal diverticulum are acquired after esophageal or tracheal
surgery, orotracheal intubation or increased intraluminal pressure
through a weak area of tracheal wall. It occurs more often after
surgery for tracheoesophageal fistula when the fistula is not divided
sufficiently close to the trachea. In this case the diverticulum
becomes a remnant of esophageal epithelium created at the time of
repair of the TEF. In either congenital or acquired cases the
diverticulum acts as a reservoir for pooling of secretions that
may spill over into the tracheobronchial tree predisposing
affected patients to cough, dyspnea, stridor and chronic chest
infection. CT-Scan imaging with thin slices or bronchoscopy is
diagnostic. Surgical treatment of congenital tracheal diverticulum is
rarely advocated. Excision of the acquired diverticulum may be needed
in patients with severe respiratory symptoms, either a large
diverticulum and airway compression or a suspected recurrent fistula.
Another less invasive and effective alternative management is
endoscopic carbon dioxide laser fulguration of the tracheal
diverticulum mucosa.
References:
1- Sharma BG: Tracheal diverticulum: a report of 4 cases. Ear Nose Throat J. 88(1):E11, 2009
2- Berlucchi M, Pedruzzi B, Padoan R, Nassif N, Stefini S: Endoscopic
treatment of tracheocele in pediatric patients. Am J Otolaryngol.
31(4):272-5, 2010
3- Gaissert HA, Grillo HC: Complications of the tracheal diverticulum after division of congenital
tracheoesophageal fistula. J Pediatr Surg. 41(4):842-4, 2006
4- Johnson LB, Cotton RT, Rutter MJ: Management of symptomatic tracheal
pouches. Int J Pediatr Otorhinolaryngol. 71(4):527-31, 2007
5- Cheng AT, Gazali N: Acquired tracheal diverticulum following repair
of tracheo-oesophageal fistula: endoscopic management. Int J Pediatr
Otorhinolaryngol. 72(8):1269-74, 2008
6- Shah AR, Lazar EL, Atlas AB: Tracheal diverticula after
tracheoesophageal fistula repair: case series and review of the
literature. J Pediatr Surg. 44(11):2107-11, 2009
PSU Volume 42 No 05 MAY 2014
Gliomatosis Peritonei
Gliomatosis peritonei (GP) refers to a
very rare entity characterized by miliary disseminated implants
composed of mature glial (fibrous astrocytes) tissue throughout the
serosal surface of the abdominal peritoneal cavity including recesses,
cul-de-sac and intestinal surfaces. Though the picture is of an advance
stage of neoplasia, its behavior is almost invariably benign since its
differentiated cells lack proliferative activity. The majority of cases
of GP are associated with an immature ovarian teratoma, though only
rarely with mature teratoma. Almost 25% of immature teratoma can
develop GP. Hypothesis for development of GP includes implantation and
subsequent maturation of neural precursor cells detached from the
primary tumor or peritoneal metaplasia from stem cells induced by
growths factors. Peak incidence of GP is in the second decade with an
average age of 15 years. GP can occur after capsular rupture during
surgery or spontaneously. The overall prognosis of mature GP is
excellent and chemotherapeutic management is rarely necessary except
with high-grade or immature deposits. Implants can grow rapidly. Serum
markers such as CA125 and alpha fetoprotein are elevated in GP.
Histologically GP demonstrates large amounts of well-differentiated
glial tissue with extensive endothelial proliferation of vessels
similar to brain tumors, minimal atypia and only rare mitosis. Surgical
sampling should be as extensive as possible to evaluate the maturity of
the glial deposits to establish a diagnosis of GP. Immature glial
tissue or other teratomatous component heralds metastatic disease and
should be managed aggressively. GP does not adversely affect the
prognosis of ovarian teratoma. A conservative surgical approach without
adjuvant therapy or chemotherapy is recommended.
References:
1- Harms D, et al: Gliomatosis peritonei in childhood and
adolescence. Clinicopathological study of 13 cases including
immunohistochemical findings. Pathol Res Pract. 184(4):422-30, 1989
2- Chaung JH, Chen L: Ovarian teratoma with gliomatosis peritonei. J Pediatr Surg. 27(5):662-4, 1992
3- Hamada Y(1), Tanano A, Sato M, Tsuji M, Sakaida N, Okamura A, Hioki
K: Ovarian teratoma with gliomatosis peritonei: report of two cases.
Surg Today. 28(2):223-6, 1998
4- Hill DA(1), Dehner LP, White FV, Langer JC: Gliomatosis peritonei as
a complication of a ventriculoperitoneal shunt: case report and review
of the literature. J Pediatr Surg. 35(3):497-9, 2000
5- Hsieh YL, Liu CS: Progression from an immature teratoma with miliary
gliomatosis peritonei to growing teratoma syndrome with nodular
gliomatosis peritonei. Pediatr Neonatol. 50(2):78-81, 2009
6- Yoon NR, Lee JW, Kim BG, Bae DS, Sohn I, Sung CO, Song SY:
Gliomatosis peritonei is associated with frequent recurrence, but does
not affect overall survival in patients with ovarian immature teratoma.
Virchows Arch. 461(3):299-304, 2012
Neutropenic Colitis
Neutropenic colitis (NC) refers to a
potentially life-threatening necrotizing inflammation of the cecum and
colon the result of a chemotherapeutic complication of leukemia
treatment, acquired immunodeficiency syndrome or as a complication of
bone marrow transplantation. The clinical triad of neutropenic colitis
includes neutropenia, abdominal pain/tenderness and fever. The
integrity of the bowel wall is compromised due to factors including
mucosal injury by cytotoxic drugs, neutropenia, and impaired host
defense to intestinal organisms. This leaves the bowel vulnerable to
bacterial invasion, necrosis and perforation. Most cases involve the
cecum and ascending colon. It is sometimes difficult to differentiate
NC from appendicitis in the early onset. Critical key points for
diagnosis of NC are neutropenia (absolute neutrophil counts below 500
cells/µL), high fever, bloody stool and aggressive progress.
Diagnostic accuracy is increased with the use of US or CT-Scans when
there is a bowel wall thickening above 3 mm. Factors associated with
development of NC include age greater than 16 years, mucositis, stem
cell transplantation and chemotherapy within the prior two weeks of
symptoms. Initial management should consist of aggressive hemodynamic
support, bowel rest/decompression, supplemental nutrition,
broad-spectrum antibiotic therapy and G- CSF. Measurements of
C-reactive protein in blood may be of benefit when assessing the
clinical course. Indications for surgical intervention include diffuse
peritonitis, pneumoperitoneum, persistent gastrointestinal bleeding and
continued clinical deterioration despite medical therapy. The most
common surgical indication is bowel perforation. Recommended surgical
procedures include hemicolectomy, ileostomy and secondary anastomosis
or drainage. Primary bowel anastomosis is not a good choice due to the
high risk of leakage, intraperitoneal infection, abscess, intestinal
adhesion and obstruction. Most children do not require surgery. Overall
mortality is lower in children (5%).
References:
1- Moran H, Yaniv I, Ashkenazi S, Schwartz M, Fisher S, Levy I:
Risk factors for typhlitis in pediatric patients with cancer. J Pediatr
Hematol Oncol. 31(9):630-4, 2009
2- Sundell N, Bostram H, Edenholm M, Abrahamsson J: Management of
neutropenic enterocolitis in children with cancer. Acta Paediatr.
101(3):308-12, 2012
3- McCarville MB(1), Adelman CS, Li C, Xiong X, Furman WL, Razzouk BI,
Pui CH, Sandlund JT: Typhlitis in childhood cancer. Cancer.
104(2):380-7, 2005
4- Schlatter M(1), Snyder K, Freyer D: Successful nonoperative
management of typhlitis in pediatric oncology patients. J Pediatr
Surg. 37(8):1151-5, 2002
5- Fike FB(1), Mortellaro V, Juang D, St Peter SD, Andrews WS, Snyder
CL: Neutropenic colitis in children. J Surg Res. 170(1):73-6, 2011
6-Li K, Zheng S, Dong K, Gao Y, Wang H, Liu G, Gao J, Xiao X: Diagnosis
and outcome of neutropenic enterocolitis: experience in a single
tertiary pediatric surgical center in China. Pediatr Surg Int.
27(11):1191-5, 2011
MACIS
Thyroid carcinoma is the third most
common solid tumor in children and adolescent. The incidence is rising.
Most cases of thyroid carcinoma are well differentiated papillary or
follicular tumors. As previously stated they are best managed with
total thyroidectomy and postoperative radioiodine therapy in most
cases. There are several scoring systems used to classify thyroid
cancer. MACIS refer to a recently developed prognostic scoring system
utilized for differentiated thyroid carcinoma in children and
adults that has resulted in recognition of low- and high risk patient
categories allowing meaningful comparison of a variety of treatment
approaches. MACIS stand for the presence of Metastasis, Age,
Completeness of resection, local Invasion and tumor Size. MACIS was
designed in 1993 to predict disease specific survival in patients with
differentiated thyroid carcinoma. The cutoff score has been set at a
score above four heralding a poor prognosis for children less than 21
years of age. MACIS score correlates well with the response to initial
therapy in children and is useful in predicting outcome. MACIS
appear the most useful prognostic system taking completeness of
resection into account.
References:
1- Palme CE(1), 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
2- Voutilainen PE(1), Siironen P, Franssila KO, Sivula A, Haapiainen
RK, Haglund CH: AMES, MACIS and TNM prognostic classifications in
papillary thyroid carcinoma. Anticancer Res. 23(5b):4283-8, 2003
3- Powers PA(1), Dinauer CA, Tuttle RM, Francis GL: The MACIS score
predicts the clinical course of papillary thyroid carcinoma in children
and adolescents. J Pediatr Endocrinol Metab. 17(3):339-43, 2004
4- Kjellman P(1), Zedenius J, Lundell G, Bäckdahl M,
Farnebo LO, Hamberger B, Larsson C, Wallin G: Predictors of outcome in
patients with papillary thyroid carcinoma. Eur J Surg Oncol.
32(3):345-52, 2006
5- Jang HW(1), Lee JI, Kim HK, Oh YL, Choi YL, Jin DK, Kim JH, Chung
JH, Kim SW: Identification of a cut-off for the MACIS score to predict
the prognosis of differentiated thyroid carcinoma in children and young
adults. Head Neck. 34(5):696-701, 2012
PSU Volume 42 NO 06 JUNE 2014
Appendicostomy
Appendicostomy refers to a novel
surgical procedure utilized as an antegrade continent enema for the
management of fecal incontinence, soiling, colonic dysmotility and
intractable slow transit constipation in children and adults. The
appendix is brought to the skin, usually through the navel, as a small
stomal conduit, to be catheterized on a daily basis or regularly and
used for antegrade colonic irrigation washouts achieving socially
acceptable fecal continence and cleansing. The appendicostomy procedure
can be done open or laparoscopically. The laparoscopy approach
has less operative trauma, less pain, better cosmetic results and can
be performed as an outpatient procedure. During the procedure a
silastic catheter or a Chait button is inserted. Cecal wrap and
fixation as antireflux measures have been recommended but they
have not eliminated fecal leak or reflux and increase operative
time. The most important variables that predict outcome are
patient compliance, regular use of the irrigations and patient age.
Complications of using the appendix as conduit include painful catheter
insertion related to stomal stenosis since many children avoid regular
prophylactic catheter insertion. This problem can also be overcome
using constantly an indwelling tube or the Chait button. Mucus leak is
common during the first 6-8 weeks. The washout regime can cause
significant pain due to volume or content (senna). Other less common
complications of the appendicostomy include bleeding, granulation
tissue, perforation, infection, embarrassment about the catheter and
fecal leak. The appendicostomy procedure effectively reduces soiling in
more than 80% of children with idiopathic constipation so long as the
parents are motivated to perform the antegrade enemas on a daily basis.
Abdominal pain common in chronic constipation is significantly reduced
in severity and frequency in these patients. In the preschool child the
benefits are earlier cleanness before starting school with less
incidence of stenosis and leakage.
References:
1- Marshall J, Hutson JM, Anticich N, Stanton MP: Antegrade continence
enemas in the treatment of slow-transit constipation. J Pediatr Surg.
36(8):1227-30, 2001
2- Cascio S, Flett ME, De la Hunt M, Barrett AM, Jaffray B: MACE or
caecostomy button for idiopathic constipation in children: a comparison
of complications and outcomes. Pediatr Surg Int. 20(7):484-7, 2004
3- Koivusalo A, Pakarinen M, Rintala RJ: Are cecal wrap and fixation
necessary for antegrade colonic enema appendicostomy? J Pediatr Surg.
41(2):323-6, 2006
4- Kim J, Beasley SW, Maoate K: Appendicostomy stomas and antegrade
colonic irrigation after laparoscopic antegrade continence enema. J
Laparoendosc Adv Surg Tech A. 16(4):400-3, 2006
5- Chatoorgoon K(1), Pena A, Lawal T, Hamrick M, Louden E, Levitt MA:
Neoappendicostomy in the management of pediatric fecal incontinence. J
Pediatr Surg. 46(6):1243-9, 2011
6- Stenstrom P(1), Graneli C, Salo M, Hagelsteen K, Arnbjornsson E:
Appendicostomy in preschool children with anorectal malformation:
successful early bowel management with a high frequency of minor
complications. Biomed Res Int. Volume 2013, Article ID 297084, 8 pages
Fibrous Soft Tissue Tumors
Fibrous soft tissue tumors (FSTT),
also known as fibromatosis or desmoid tumors, refer to a group of
non-neoplastic spindle cell proliferations that behave locally
aggressively but without a propensity to metastasize. They have an
increase propensity for local invasiveness and recurrence. FSTT
comprised almost 12% of all soft tissue tumors in infants and children.
Mean age of presentation is five years. FSTT are firm circumscribed
masses, with a white-tan cut surface, glistening or a whorled
appearance. They rarely have evidence of hemorrhage or necrosis.
Histologically FSTT appears as proliferation of spindle shaped,
well-differentiated fibroblast arranged in fascicle in a background of
mature collagen with a few or none typical mitotic figures. Included in
this group of FSTT are infantile digital fibromatosis, fibromatosis
colli, aggressive fibromatosis, fibrous hamartoma of infancy and
others. Management of FSTT encompasses local surgical excision with a
recommended surgical margin of 1 cm and excision in depth to include
the adjacent normal tissue plane. Though having negative margins at
resection is the ideal goal of therapy, this is sometimes difficult to
achieve if there is going to be sacrifice of structure or function.
Local recurrence has been reported in more than 10% of FSTT due to
inadequate primary excision. Recurrence of FSTT has been associated
with younger age, incomplete resection and specially when involving
digital or extremity lesions. However, even with incomplete excision,
FSTT has a low recurrence rate and carries an excellent prognosis.
Radiotherapy and/or pharmacologic treatment is reserved for patients
with unresectable or progressive disease.
References:
1- Lee JT, Girvan DP, Armstrong RF: Fibrous hamartoma of infancy. J Pediatr Surg. 23(8):759-61, 1988
2- Aldrink JH(1), Nicol K, Wang W, Teich S: Fibrous soft tissue tumors:
factors predictive of recurrence. J Pediatr Surg. 48(1):56-61. 2013
3- Gatibelza ME(1), Vazquez BR, Bereni N, Denis D, Bardot J, Degardin
N:Isolated infantile myofibromatosis of the upper eyelid: uncommon
localization and long-term results after surgical management. J Pediatr
Surg. 47(7):1457-9, 2012
4- Seguier-Lipszyc E(1), Hermann G, Kaplinski C, Lotan G: Fibrous hamartoma of infancy. J Pediatr Surg. 46(4):753-5, 2011
5- Gold JS(1), Antonescu CR, Hajdu C, Ferrone CR, Hussain M, Lewis JJ,
Brennan MF, Coit DG: Clinicopathologic correlates of solitary fibrous
tumors. Cancer. 15;94(4):1057-68, 2002
6- Blakely ML(1), Spurbeck WW, Pappo AS, Pratt CB, Rodriguez-Galindo C,
Santana VM, Merchant TE, Prichard M, Rao BN: The impact of margin
of resection on outcome in pediatric nonrhabdomyosarcoma soft tissue
sarcoma. J Pediatr Surg. 34(5):672-5, 1999
Diaphragmatic Rupture
Diaphragmatic rupture almost always
occurs after a traumatic event, most probably a motor-vehicle accident
followed by falls. Blunt trauma accounts for more than 80% of
diaphragmatic injuries. More than 50% of cases have an associated
injury. Several anatomic factors predisposing to diaphragmatic rupture
include a thinner abdominal wall, more horizontal position of the
diaphragm, ribs more cartilaginous and elastic rupturing at areas of
fixed attachment especially the costal origin which is longer and
weaker. Regardless of the mechanism of injury, the early detection of
an occult diaphragmatic rupture usually depends on a high index of
suspicion. Isolated diaphragmatic injuries do occur in children more
frequently than in adults and diaphragmatic rupture prevails in the
left posterolateral side. The right side is protected by the liver.
Preoperative chest X-ray and CT Scan are diagnostic of most
diaphragmatic ruptures in children. Early management determines the
effectiveness of treatment and avoids the consequence of missed
injuries. Delayed diagnosis can lead to intestinal obstruction and
strangulation of the intestine, sepsis and death. Contributing factor
for delay in diagnosis also is the extent of diaphragmatic
rupture as well as the site of rupture. Short length of diaphragmatic
rupture may not manifest initially but as the extent of rupture
increases, more and more intraabdominal viscera will herniate into the
thoracic cavity as a result of negative intra-thoracic pressure and
create symptoms. Most of the delayed cases are seen in the right side.
Diaphragmatic rupture is usually repaired using a laparotomy in the
acute setting. With delayed diagnosis surgeons prefer a thoracic
approach for repair. Operative strategies should be planned based on
the localization, size of the rupture, associated injuries and
stability of the patient.
References:
1-Al-Salem AH:Traumatic diaphragmatic hernia in children. Pediatr Surg Int. 2012 Jul;28(7):687-91
2- Soundappan SV, Holland AJ, Cass DT, Farrow GB:Blunt traumatic diaphragmatic injuries in children. Injury. 36(1):51-4, 2005
3- Khan TR, Rawat J, Maletha M, Singh S, Rashid KA, Wakhlu A, Kureel
SN: Traumatic diaphragmatic injuries in children: do they really mark
the severity of injury? Our experience. Pediatr Surg Int. 25(7):595-9,
2009
4- Shehata SM, Shabaan BS: Diaphragmatic injuries in children after blunt abdominal trauma. J Pediatr Surg. 41(10):1727-31, 2006
5- Okur MH, Uygun I(2), Arslan MS, Aydogdu B, Turkoglu A, Goya C, Icen
M, Cigdem MK, Onen A, Otcu S: Traumatic diaphragmatic rupture in
children. J Pediatr Surg. 49(3):420-3, 2014
6- Hwang SW, Kim HY, Byun JH: Management of patients with traumatic
rupture of the diaphragm. Korean J Thorac Cardiovasc Surg.
44(5):348-54, 2011