Jiaps April June 09 17.08.09

Editorial Full text online at http://www.jiaps.com

47 J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2

J Indian Assoc Pediatr Surg / Apr-Jun 2009 / Vol 14 / Issue 2 48

Review Article Full text online at http://www.jiaps.com

Anatomical basis for Wilms tumor surgery
R. B. Tröbs
Department of Pediatric Surgery, Catholic Foundation Marien Hospital Herne, Ruhr-University of Bochum, Herne,
Germany

Address for correspondence: Dr. Ralf-Bodo Tröbs, Department of Paediatric Surgery, Catholic Foundation Marien Hospital Herne,
Ruhr-University of Bochum, D-44627 Herne, Widumer Str. 8, Germany. E-mail: ralf-bodo.troebs@marienhospital-herne.de

ABSTRACT
Wilms tumor surgery requires meticulous planning and sophisticated surgical technique. Detailed anatomical
knowledge can facilitate the uneventful performance of tumor nephrectomy and cannot be replaced by
advanced and sophisticated imaging techniques. We can define two main goals for surgery: (1) exact staging
as well (2) safe and complete resection of tumor without spillage. This review aims to review the anatomical
basis for Wilms tumor surgery. It focuses on the surgical anatomy of retroperitoneal space, aorta, vena cava
and their large branches with lymphatics. Types and management of vascular injuries are discussed.

KEY WORDS: Aorta, gerota’s fascia, Wilms tumor, inferior mesenteric artery, superior mesenteric artery, surgery,
vascular injury
DOI: *****

InTRodUcTIon stage. Thus, less overall treatment was required. These
findings were confirmed by the Third Wilms Tumor
The word surgery has Greek roots and means nothing (WT) Study of the United Kingdom Children’s Cancer
more than “handwork.” This handwork is largely based Study Group.[1,2]
on the science of dissection known as “anatomy.” From
this point of view, pediatric surgery is anatomy applied Removal of a large nephroblastoma can still be a
to the living body of a child. The aim of my presentation demanding undertaking. Large trials in WT surgery
is to review the anatomical basis of Wilms tumor (WT) have identified various anatomy-based complications.
surgery. However, institutional series and case reports give
additional important details. Surgical problems in
Treatment of children with a nephroblastoma is an WT surgery can involve the staging and removal of
interdisciplinary undertaking. In the last 40 years, the nephroblastoma itself, as well as hemorrhage
attempts have been made to decrease the morbidity and injuries to vessels and adjacent structures. The
of treatment while maintaining an excellent survival nomenclature of anatomical structures and eponyms
rate. The pediatric surgeon or urologist plays a key used in this paper follows the recommendations of the
role within the team. In particular, the duration and Federative Committee on Anatomical Terminology.[3]
amount of chemotherapy and radiation therapy can be
influenced by the surgical technique. Zones, spaces and planes
We can define two main goals for surgery (1) exact Wilms Tumor is an embryonic renal tumor located
staging and (2) safe and complete resection of the tumor within the retroperitoneal space. Recently, Skandalakis
without spillage. The Société Internationale d’Oncologie wrote “This space is a vast territory lacking any accurate
Pédiatrique (SIOP) investigators pioneered the concept
of pretreatment of renal tumors before nephrectomy.
The SIOP trials showed that pre-treatment resulted
Presentation held at the 34th Annual Conference of the Indian Association
in safer surgery, reduced tumor rupture rates, and
of Pediatric Surgeon, December 5th, Guwahati, Assam
an increased portion of children with a lower tumor

Tröbs: Basis for Wilms tumor surgery

knowledge and accepted map”.[4] clearly shows the existence of three renal compartments
in a child. The ruptured right lower pole is shown
According Farthmann et al. (1989) the retro peritoneum with separated blood collections in the perirenal space
can be divided into three zones and four parts: the (asterisks) as well as in both pararenal spaces (stars).
central zone (containing the aorta, inferior vena cava, The rate of perirenal infiltration of nephroblastomas
pancreas, and duodenum); two lateral zones (the remains unclear. To obtain clear margins, a rim of
kidneys, ureters, and ascending/descending colon), healthy tissue including covering fibrous tissue and
and the pelvic zone (rectosigmoid, iliac vessels, and fat has to be resected with the tumor. Strict orientation
urogenital organs).[4,5] between different retroperitoneal planes is required to
achieve this. Standard textbooks recommend that the
The retro-peritoneal space is defined as the space
tumor plane should be developed outside the perirenal
between the posterior parietal peritoneum and
fascia.[7-10] In other words, resection of the tumor-bearing
the transversalis fascia. This anatomic highway is
kidney during radical uretero-nephrectomy also requires
responsible for the dissemination of pathologic entities
removal of the intact Gerota’s and Zuckerkandl’s fascia
originating in the retro peritoneum, and is comprised
that cover the kidney. This procedure is known as a
of connective tissue layers.
perifascial nephrectomy. The uretero-nephrectomy
Gerota’s fascia is often used as a general term to specimen shown in Figure 3 demonstrates an upper
describe renal fascia. In 1883, Zuckerkandl described pole tumor removed with a sheath of surrounding fat
the posterior renal fascia but did not recognize the and perirenal fascia.
presence of the anterior layer of renal fascia. In his work
Beiträge zur Kenntnis des Befestigungsapparates der At the upper pole of the kidney, a fascial septum
Niere, the Romanian Gerota documented the presence separates the adrenal gland from the kidney.[4] The
of the anterior fascia and clearly assigned Zuckerkandl’s
name to the posterior fascia.[6]

Three compartments of retro peritoneal space are
related to the kidney: the perirenal space as well as the
anterior and posterior pararenal spaces [Figure 1]. The
perirenal space is the home of the kidneys. The renal
fascia, a collagenous connective tissue of mesodermal
origin that envelopes the kidney, is responsible for this
compartmentalization. The kidney is enveloped by the
anterior and posterior laminae of the renal fascia and
fatty tissue inside and outside the fascia. There is some
medial fixation with the adventitial covering of renal
vessels and aorta or inferior vena cava (IVC).[4]
Figure 2: Peri- and pararenal hematoma after traumatic renal rupture
(CT Scan)
For example, Figure 2 depicts a case of traumatic renal
rupture. The distribution of perirenal blood and urine

Figure 1: Peri- and pararenal compartments (ap – Anterior, pp – Figure 3: Nephrectomy specimen. Upper pole tumor covered by
Posterior Pararenal) Gerota’s fascia



adrenal gland for small or lower pole tumor can be another artery is revealed underneath that could be the
spared.[9] However, in many cases, when the tumor is superior mesenteric artery, aorta, or left or right renal
large and adrenal gland is attached it must be removed artery. This artery should not be ligated until its exact
to achieve adequate margins. identity has been established.[20,21] When in doubt,
this can be done by cross-clamping the vessel with a
Vessels – Keys To sUccessfUl sURgeRy vascular clamp. It can be clearly stated that attempts
at early ligation of the hilar vessels cannot be justified
Generally, three types of vessels have to be taken into until the renal vasculature is clearly identified.
account: arteries, veins, and lymphatic vessels. Modern
imaging techniques allow clear outlining of many The vessels most at risk during excision of right renal
aspects of the visceral blood supply. The aorta and its tumors are IVC and contralateral renal vein. In large
branches are quite small in infants and toddlers; thus right-sided tumors, the IVC enters the tumor mass and
they can be mistaken for the renal artery. In a three- is hidden from vision. Under this circumstance, the
year-old child the aorta at the level of the kidneys has a right renal artery is in close proximity to the left renal
diameter of 6 to 7 mm and the renal artery measures 2.8 vein, which can thus be damaged. [20] If unrecognized,
mm.[11] WT surgery is a safe and partially standardized such damage can lead to venous infarction and loss of
procedure. However, major bleeding and vascular renal function.
injuries can occur. The frequency of vascular injuries
has been reported as 1.5% in the NWTS-3 and 4.[12, 13]
Severe hemorrhage occurs at a higher rate. Further, there
aoRTa and ITs BRanches
exists a hidden surgical mortality.[14]
Modern imaging techniques allow noninvasive clear
The vascular pedicle of both kidneys is an important outlining of the visceral blood supply. The angio-MR
anatomical area and should be analyzed. The left renal [Figure 4] demonstrates the arterial branches of the aorta
vein passes in front of the aorta in the majority of in a child with a left-sided WT.
individuals. The right artery reaches the kidney behind
the IVC. However, variations in surgical importance can It shows that the coeliac trunk (Coe T) and SMA
occur. In up to three per cent cases, a retro aortic left originate from the aorta in close proximity to the origins
renal vein has to be taken into account. Circum aortic of the renal arteries. The distance between the origins
veins also occur in a relevant number of patients. [15] of these aortal tributaries can be 1 cm or less.[22]

Ligature and cutting of the large renal vessels is one of For surgical practice it is helpful to arrange the aortic
the most important steps of nephrectomy. Early ligature
of the vein has the theoretical advantage of preventing
hematogenous tumor spread. [8, 16, 17] However, this was
never confirmed by a prospective trial. On the other
hand, primary ligature of the artery is recommended
for prevention of tumor swelling and rupture. [7,10,18]

There is no consensus in literature regarding the
sequence of vessel ligature. Selected international
standard publications recommend early control of the
hilum. However, this is often not feasible with extremely
large tumors; mobilization of the tumor mass must
first occur to allow exposure of the hilar vessels.[16,19]
The current SIOP 2001/German Society of Pediatric
oncology protocol recommends initial ligature of the
artery. When feasible, we follow this recommendation.

During removal of left renal tumors, damage to the aorta,
superior mesenteric artery (SMA), and right renal artery
has been reported to occur. These vessels are in close
proximity to the tumor mass, and if the aorta and IVC
separate by tumor or lymphatic infiltration, they are
threatened during removal of the left kidney. The left Figure 4: Major Branches of Abdominal Aorta (MRI). R RA – Right
renal vein is usually identified first. Once it is divided Renal Artery, L RA - Left Renal Artery



branches in the three planes they occupy [Figure 5]: (1) constitutes an arch from the left transverse colon to the
blood supply to the gastrointestinal tract in front of the sigmoid colon secondary to the marginal artery.[26,27]
aorta; (2) to three paired glands on both sides and (3) to
diaphragm and the four lumbar arteries. [4] VenoUs dRaInage
Iatrogenic injury to the aorta and its branches has The left and right kidneys show a difference in venous
been inconstantly reported. These reports indicate that drainage. In right-sided nephroblastoma with cava
patients with left sided large tumors are at particular risk thrombus, ligature and dissection of the left renal
for this type of injury. Although attempts were always vein is possible. In the majority of patients, sufficient
made to repair the intraoperative vascular injury, three venous collaterals via the phrenic, adrenal, hemiacygos,
of six patients described in literature died as a result of testicular, lumbal and ureteral veins are present
the vessel injury. [23, 24] All these children had left sided [Figure 7].[28] In contrast, the anatomy of the right kidney
nephroblastoma; four cases had injuries to the SMA, does not allow this maneuver.[29]
one had both SMA and coeliac trunk, and one the aorta
injury. It has been reported that after unnoticed ligation In humans, embryogenesis of the renal and post renal
of SMA, though the bowel may initially appear viable, segments of IVC involves the sequential appearance of
full thickness necrosis develops later. When in doubt, a three paired venous channels: the posterior cardinals
Doppler flow study can provide essential information. as well as the sub cardinal and supra cardinal
veins.[30] The development of anastomotic channels
Lacerations of the SMA require surgical repair.[23,25] between these channels and subsequent regression of
Fullen and coworkers attempted to classify SMA segments of this system might be impaired, leading to
injuries, and found that at least injuries proximal of variants like the persistence of the left sub cardinal
the middle colic artery urgently require repair, whereas
those of the distal part can be tolerated without ischemia
or with ischemia of only a small segment of the
bowel.[25] When possible, end-to-end anastomosis of the
vessel with or without venous interposition or end-to-
side anastomosis to the aorta is the procedure of choice.

In contrast to SMA, dissection of the stem of inferior
mesenteric artery (IMA) is commonly tolerated without
disastrous consequences. Generally the marginal artery,
also known as arcade of Riolan arch, (synonymously
marginal artery of Drummond), sufficiently connects
SMA with IMA [Figure 6]. However, only small
collaterals exist at the splenic flexure.[22] In addition,
the ascending arch of the left colic artery (AALCA) is
present in at least two third of cases. This arterial branch
Figure 6: Marginal Arcade and AALCA Connect SMA with IMA.[26,27]

Figure 5: Three Planes of Aortic Branches.[4] Figure 7: Venous Drainage of Right Kidney



vein leading to a high confluence of the large veins. Surg Gynecol Obstet 1992;175:507-14.
13. Ritchey ML, Shamberger RC, Haase G, Horwitz J, Bergemann T,
Other rare anomalies of the IVC include duplication,
Breslow NE. Surgical complications after primari nephrectomy
left-sidedness of the vein and interruption of the IVC for Wilms tumor: report from the National Wilms Tumor Study
with azygos/ hemiazygos continuation.[15,30,31] Group. J Am Coll Surg 2001;192:63-8.
14. Stehr M, Deilmann K, Haas RJ, Dietz HG. Surgical complications
Lack of recognition of retro peritoneal venous anomalies in the treatment of Wilms tumor. Eur J Pediatr Surg 2005;15:414-9.
15. Aljabri B, MacDonald PS, Satin R, Stein LS, Obrand DI, Steinmetz
can have potentially disastrous consequences because OK. Incidence of major venous and renal anomalies relevant to
anomalous venous structures tend to be dilated and aortoiliac surgery as demonstrated by computed tomography. Ann
tortuous. Intraoperative lacerations can cause life- Vasc Surg 2001;15:615-8.
16. Ehrlich RM. Complications of Wilms tumor surgery. Urol Clin
threatening hemorrhage.
North Am 1983;10:399-406.
17. Leape LL, Breslow NE, Bishop HC. The surgical treatment of
lymph nodes and Vessels Wilms tumor: results of the National Wilms Tumor Study. Ann
Surg 1978;187:351-6.
18. Nephroblastom SIOP 2001/GPOH. Therapieoptimierungsstudie.
Surgeons might not be able to tell from gross inspection 19. Shamberger RC. Renal Tumors. In: Carachi R, Grosfeld JL, Azmy
whether or not lymph nodes contain tumors.[32] Random AF, editors. The Surgery of childhood tumors. 2nd ed. Berlin,
sampling of nodes in each area should be performed Heidelberg: Springer; 2008. p. 171-99.
20. Gough DCS. Wilms Tumor. In: Gearhart JP, Rink RC, Mouriquand
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PD, editors. Pediatric urology. Philadelphia, London, New York:
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ascites has been infrequently described after WT renal artery: a risk in the surgical management of massive left-
sided Wilms tumor. J Indian Assoc Pediatr Surg 2007;12:156-7.
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22. Loeweneck H, Feifel G. Bauch. In, Lanz T v, Wachsmuth W, editors.
lymphadenectomy and operative injury to the cisterna Praktische Anatomie. Vol. 2. Berlin, Heidelberg, New York: 1993.
chyli or their major tributaries. Resection of the supra 23. Ritchey ML, Lally KP, Haase GM, Shochat SJ, Kelalis PP. Superior
hilar nodes carries a particular risk of injuring the mesenteric artery injury during nephrectomy for Wilms tumor. J
Pediatr Surg 1992;27:612-5.
cisterna. Meticulous lymphostasis is of paramount 24. Roth H, Weirich A, Ludwig R, Daum R, Zimmermann H. Die
importance in such cases of lymph node removal. Resektion des Nephroblastoms: Probleme und Komplikationen
– Auswertungen zur Nephroblastomstudie SIOP 9/GPOH.
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2. Tröbs RB, Hänsel M, Friedrich T, Bennek J. A 23-year-experience Schlesinger F, et al. Riolan’s arch: confusing, misnomer, and
with malignant renal tumors in infancy and childhood. Eur J obsolete. An literature survey of the connection(s) between the
Pediatr Surg 2001;11:92-8. superior and inferior mesenteric arteries. Am J Surg 2007;193:742-8.
3. Federative Committee on Anatomical Terminology. Terminologica 27. Lange MM, Buunen M, van de Velde CJ, Lange JF. Level of arterial
anatomica. Stuttgart, New York: Thieme; 1998. p. 163-6. ligation in rectal cancer surgery: low preferred over high tie. A
4. Skandalakis JE, Skandalakis LJ, Zoras O. Posterior aspect of the review. Dis Colon Rectum 2008;51:1139-45.
abdominal viscera and retroperitoneum. In, Merlini MP, Martin 28. Giuliani L. Radikale Chirurgie der Nierentumoren. In: Hohenfellner
RF, editors. Multiorgan resection for cancer. Stuttgart, New York: R, editor. Ausgewählte urologische OP-Techniken. Stuttgart, New
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5. Farthmann EH, Kirchner R, Fraedrich G. Organ- und 29. Narasimhan KL, Chowdhary SK, Marwaha RK, Rao KL. Renal
Gefäßverletzungen des zentralen Retroperitoneums. Chirurg failure following unilateral nephrectomy in Wilms tumor. Ind J
1989;60:657-64. Urol 2000;16:157-60.
6. Chesbrough RM, Burkhard TK, Martinez AJ, Burks DD. Gerota 30. Mathews R, Smith PA, Fishman EK, Marshall FF. Anomalies of
versus Zuckerkandl: The renal fascia revisited. Radiology the inferior vena cava and renal veins: embryologic and surgical
1989;173:845-6. considerations. Urology 1999;53:873-80.
7. Sigel A, Chlepas S. Nephroblastoma. In, Eckstein HB, Hohenfellner 31. Zucconi WB, Guelfguat M, Solounias N. Approach to the
R, Williams DI, editors. Surgical pediatric urology. Philadelphia, educational opportunities provided by variant anatomy,
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8. Othersen BH, Hebra A, Tagge EP. Nephroblastoma and other renal Anat 2002;15:165-8.
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et al. Surgical complications after nephrectomy for Wilms tumor.


Review Article Full text online at http://www.jiaps.com

Psychological issues in cleft lip and cleft palate
Avinash De Sousa, Shibani Devare, Jyoti Ghanshani
Consultant Psychiatrist, Address Missing?????

Address for correspondence: Dr. Avinash De Sousa, Carmel, 18, St. Francis Avenue, Off S.V. Road, Santacruz West,
Mumbai 400-054, Maharashtra, India. E-mail: avinashdes999@yahoo.co.uk

ABSTRACT
Vocational and social issues affect rehabilitation and development of patients with cleft lip and cleft palate.
However, psychological problems like lowered self esteem and difficulties in social interaction have also
been noted in them. Not many pediatric reconstructive surgery teams have a psychiatrist on their panel. It
is likely that psychological problems are higher in incidence than literature actually suggests. Hence it is
very essential that such cases are identified by the surgical team to maximize positive outcome of surgery
and rehabilitation. This study discusses psychological issues revolving around cleft lip and cleft palate
along with lacunae in many psychological research studies.

KEY WORDS: Cleft lip, cleft palate, psychological issues
DOI: *****

InTRodUcTIon contribute in the development of psychosocial issues
among individuals with any form of facial anomaly in
Research shows, learning to live with a change in general. Research has shown that attractive children
appearance of one’s face as a result of injury or disease are seen by others as brighter, having more positive
is a difficult task.[1] It is additionally challenging for social behavior and receive more positive treatment
children with congenital craniofacial conditions and than their less attractive counterparts.[4,5] Self perception
their parents to adjust. Evidence shows, amongst the plays a pivotal role in influencing an individual’s
various craniofacial developmental abnormalities, cleft self esteem and psychological adjustment affected by
lip and cleft palate occur most commonl, affecting one cleft lip and palate anomaly.[6-8] Additionally, parental
in 700 live births.[2] The role of physical appearance has influence also shapes ones psychosocial perception.
proved that a healthy physical appearance, regardless The attitudes, expectations and degree of support
of facial or physical characteristics, is considered shown by parents can influence a child’s perception
attractive.[3] Significant literature has shown, in addition of their cleft impairment.[9,10] Parents of children with
to coping with their physical appearance, children clefts may be more tolerant of misbehavior in their
with cleft anomaly in general have to deal with their child and are more likely to spoil their child by being
more superficial psychological issues/ psychosocial overprotective.[4,11] Additionally, peer interaction also
limitations. Existing multispecialty care is primarily plays an important role in maintaining psychosocial
aimed at physical rehabilitation with the psychological limitations. Many children with cleft lip and palate
issues of care often being neglected.[4] This paper may have a less attractive facial appearance or speech
discusses the various psychosocial issues amongst than their peers. A high incidence of teasing over facial
children and adults with cleft lip and cleft palate. appearance is reported among those with cleft lip and
palate.[12-17] A self report research study on determining
oVeRVIew of psychosocIal IssUes In clefT lIp the psychosocial functioning related to cleft lip and
and clefT palaTe palate, showed participants with cleft lip and palate
reported greater behavioral problems; were teased often
Various physiological and sociocultural factors and less happy with their facial appearances.[12]

Sousa, et al.: Psycological issues in cleft lip and palate

The study concludes that having been teased was a are underachievers along with the evidence of
significant predictor of poor psychological functioning behavioral inhibition, concern regarding appearance,
amongst individuals with cleft lip and palate. However, and decreased expectations by teachers and parents.
it found limited evidence to suggest that individuals
may encounter psychosocial problems as a result of psychologIcal facToRs In clefT sURgeRy
having a cleft lip and palate, with overall adjustment
and functioning appearing to be reasonably good. It is evident that with various limitations that
individuals with cleft lip and palate experience, they are
socIal sTIgma and clefT dIsoRdeRs bound to encounter various psychological difficulties.
Moreover, these limitations build up over a period of
A social stigma is created within an individual when time because of the psychological problems faced.
he/she is negatively discriminated by labeling him/her For example, communication disorders in individuals
different from normal. An individual’s thoughts, feelings with cleft lip and palate seem not to result from
and behavior related to their physical appearance makes phonological defects but from psychological problems
their body image attitudes.[18] A negative response from that may influence the entire development of an affected
outsiders, actual or perceived, may adversely affect child.[24] Anxiety and depression have also been reported
self-image.[4,19,20] Also physical attractiveness plays an to be twice as prevalent in adults with cleft lip and
important role in the development and maintenance palate compared with normal controls.[25] Difficulties
of self beliefs. Research indicates that preference are also experienced in relation to behavioral problems
for attractive individuals subsequently influences and satisfaction with facial appearances.[12] Moreover,
self esteem, social competence, and future ratings of these psychological problems can be interrelated.
attractiveness.[3] Moreover, being physically attractive Anxiety, depression, and palpitations were reported
appears to be an advantageous trait regardless of age. about twice as often by subjects with cleft lip and
Physical attractiveness has shown to play a significant palate compared with controls, and these psychological
role in social set ups like developing relationships problems were strongly associated with concerns about
during various stages of life, school, courtships, work appearance, dentition, speech, and desire for further
etc. Social acceptance often depends on one’s physical treatment.[25] Additionally, findings in studies indicated
look. These associations between physical beauty and that psychological and behavioral problems depended
social acceptability indicate the difficulties for cleft lip on the type of cleft deformity. For example, children
and palate affected individuals.[4,9] with only cleft palate only showed greater problems
with parents, reported depression, anxiety, and
edUcaTIon and commUnIcaTIon pRoBlems learning related to speech than children with unilateral
cleft lip and palate or bilateral cleft lip and palate.
Evidence shows that communication problems related The latter two groups showed fewer problems and a
to cleft lip and palate are noticeable at a young age. A greater relationship of problem to facial appearance.[26]
research study on the development of children with These psychological difficulties are not just limited to
cleft lip and palate infants and toddlers, from birth till individuals/children with cleft lip abnormality, but also
the age of three, reveals that toddlers with cleft palate to their parents. Research studies have shown parents to
exhibit ‘at-risk/delayed’ development in the expressive experience mental crisis, based on their own previous
language domain at 36 months.[21] It is also observed that background, coping with present stress etc. in bringing
factors directly affecting the psychological development up a child with cleft lip.[4,27]
of a child born with cleft lip and palate include possible
speech and language disorders, facial disfigurement, saTIsfacTIon and IssUes afTeR sURgeRy
and hearing loss.[22] Research thus makes the association
of communication problems with cleft lip and palate Surgery, being the immediate option of dealing with
evident. To add on to the communication disadvantages certain issues related to disfigurement, is beneficial in
experienced by individuals with cleft lip and palate, it dealing with both physical and psychological issues.
becomes more difficult to deal with emotional issues Surgery usually results in increased self esteem, self
during their academic years. Studies on cleft have confidence and satisfaction with appearance. [28] It
shown relationships between (a) facial appearance and can be used in young patients to improve esthetic
teacher perception, (b) behavioral inhibition and lower appearance, an important factor in the psychological
school achievement, and (c) speech defectiveness and development of adolescents.[29] However, it is necessary
self-esteem.[23] for oneself to develop positive self skills to deal with
the post surgery situations. Any individual with
Research shows that a high percentage of cleft children facial differences, who has fostered these skills, can


achieve acceptance, develop positive social interaction background play a central role in influencing behavior.
skills, demonstrate social competence, and be less It appears that research studies of the effects of cleft
likely to exhibit significant adjustment problems.[30] related conditions on behavior should examine both
Unrealistic, high expectations post surgery may also internalizing and externalizing dimensions of behavior
lead to dissatisfaction, which may further affect an and consider that there may be subgroups of children
individuals self satisfaction.[4] This disappointment showing these different kinds of behavior across
and dissatisfaction can also be experienced by parents. different age levels.[22]
Evidence makes the need for disseminating valuable
information on the pros and cons of surgery essential. conclUsIon
A study on patient satisfaction observed that majority of
the patients expressed satisfaction on the care provided, Identifying the common psychosocial factors related to
30% of the parents expressed a need to make them cleft lip and palate remains a major challenge. Extensive
more involved treatment planning decision with most research data does suggest that psychological and
of them having no or inadequate knowledge on left clip psychosocial factors have an effect on behavior, but
treatment procedures.[31] there is limited evidence to suggest that individuals
experience psychosocial problems as a result of cleft
pRoBlems wITh clef T lIp and pal aTe lip and palate. More research is required to develop a
psychologIcal ReseaRch tool whereby bias in self reporting could be avoided.
Additionally, there is a need to evaluate patient and
Though several research studies have been carried family before surgery and help provide them with
out on various aspects of cleft lip and palate they are relevant information on post and pre surgery issues.
insufficient in providing information. Studies do predict
some amount of difficulties in psychosocial functioning RefeRences
among cleft lip palate individuals, however there is
1. Rankin M, Borah G. Perceived functional impact of abnormal
limited information on the severity and the duration facial appearance. Plast Reconstr Surg 2003;111:2140-6.
of the same. [12] There is no direct evidence of cleft lip 2. Turner SR, Rumsey N, Sandy JR. Psychological aspects of cleft
palate on behavior. Many studies have shown other lip and palate. Eur J Orthodont 1998;20:407-15.
environmental, confounding factors such as teasing, 3. Sawer DB, Magee L. Psychological aspects of reconstructive
and cosmetic plastic surgery: Clinical, empirical and ethical
leading to poor psychological functioning, more so than perspectives. Lippincott Williams and Wilkins; Maryland: 2006.
having a cleft lip and/or palate per se,[22] thus providing p. 3-11.
conflicting evidence when it comes to establishing 4. Turner SR, Thomas PW, Dowell T, Rumsey N, Sandy JR.
whether children and adults with cleft lip and palate Psychological outcomes amongst cleft patients and their families.
Br J Plast Surg 1997;50:1–9.
experience psychological problems as a result of their 5. Dion KK, Berscheid E, Walster E. What is beautiful is good. J Pers
cleft. Soc Psychol 1972;24:285-90.
6. Videbeck R. Self-concept and the reaction of others. Sociometry
Many studies investigating psychological issues of cleft 1960;23:231-9.
7. Fitts WH. The self concept and behavior: Counsellor recordings
lip and palate use self reported data,[4,12] thus indicating and tests. Research Monograph No.1, Nashville, Tenessee; 1972.
a possible error of predisposition to self perception p. 35-45.
Similar self reports by parents and individual with cleft 8. Strauss RP, Broder H, Helms RW. Perception of appearance and
lip and palate have been reviewed to identify the level speech by adolescent patients with cleft lip and palate and their
parents. Cleft Palate Journal 1988;25:335-41.
of satisfaction post surgery.[32] As observed, unrealistic 9. Bull R, Rumsey N. The social psychology of facial disfigurement.
expectations can also play a pivotal role in developing J Cross Cult Psychol 1988;17:99-108.
psychological distress. Research on determining the 10. Lansdown R, Lloyd J, Hunter J. Facial deformity in childhood:
needs of parents as well as patients on cleft lip and severity and psychological adjustment. Child Care Health Dev
1991;17:165-71.
palate would be useful in providing basic information 11. Harper DC, Richman LC. Personality profiles of physically
of cleft lip palate and its characteristics pre and post impaired adolescents. J Clin Psychol 1978;34:636-42.
surgery. 12. Hunt O, Burden D, Hepper P, Johnston C. The psychosocial
effects of cleft lip and palate: a systematic review. Eur J Orthodont
2005;27:274–85.
Additionally, facial growth would change along with
13. Bernstein NR, Kapp K. Adolescents with cleft palate: body-image
age and treatment interventions; longitudinal studies and psychosocial problems. Psychosomatics 1981;22:97–703.
which determine the facial growth changes and the 14. Heller A, Tidmarsh W, Pless IB. The psychosocial functioning
experience of surgery should be examined as possible of young adults born with cleft lip or palate. Clin Pediatr
1981;20:459–65.
factors influencing psychosocial functioning.[4] An
15. Noar JH. Questionnaire survey of attitudes and concerns of
individual’s personality traits i.e. level of confidence patients with cleft lip and palate and their parents. Cleft Palate
and environmental factors like upbringing, family Craniofac J 1991;28:279–84.



16. Noar JH. A questionnaire survey of attitudes and concerns of Norwegian adults who had undergone standardised treatment
three professional groups involved in the cleft palate team. Cleft of complete cleft lip and palate. Part II. Self-reported problems
Palate Craniofac J 1992;29:92–5. and concerns with appearance. Scand J Plast Reconstr Surg Hand
17. Turner SR, Rumsey N, Sandy JR. Psychological aspects of cleft Surg 1995;29:329–36.
lip and palate. Eur J Orthodont 1998;20:407-15. 26. Pillemer FG, Cook KV. The psychosocial adjustment of pediatric
18. Clifford E. The cleft palate experience: new perspectives on craniofacial patients after surgery. Cleft Palate J 1989;26:201-7.
management. Springfield, Illinois: 1978. p. 22-30. 27. McWilliams BJ. Social and psychological problems associated
19. Goffman E. Stigma: Notes on the management of spoiled identity. with cleft palate. Clin Plast Surg 1982;9:317-26.
Prentice-Hall, Englewood Cliffs, New Jersey: 1968. p. 15-7. 28. Lefebvre AM, Munro I. The role of psychiatry in a craniofacial
20. Charon JM. Symbolic interactionism: an introduction, an team. Plast Reconstr Surg 1978;61:564-9.
interpretation, an integration. Prentice Hall, Englewood Cliffs, 29. Rachmiel A, Aizenbud D, Ardekian L, Peled M, Laufer D.
New Jersey: 1979. p. 11-4. Surgically-assisted orthopedic protraction of the maxilla in cleft
21. Neiman GS, Savage HE. Development of infants and toddlers lip and palate patients. Int J Oral Maxillofac Surg 1999;28:9-14.
with clefts from birth to three years. Cleft Palate Craniofac J 30. Kapp-Simon KA. Psychological interventions for adolescents with
1997;34:218–25. cleft lip and cleft palate. Cleft Palate Craniofac J 1995;32:104-8.
22. Richman LC, Eliason M. Psychological characteristics of children 31. Jeffery S, Boorman JG. Patient satisfaction with cleft lip and palate
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personality variables. Cleft Palate J 1982;19:249–57. JL, O’Riordan M, Goldstein JA, Robin NH. What Information Do
23. Richman LC, Millard T. Brief report: cleft lip and palate: Parent of Newborns With Cleft Lip, Palate, or Both Want to Know?
longitudinal behavior and relationships of cleft conditions to Cleft Palate Craniofac J 2000;38: 55–8.
behavior and achievement. J Pediatr Psychol 1997;22:487–94.
24. Rosanowski F, Ulrich E. Phoniatric aspects in cleft lip patients.
Facial Plast Surg 2002;18:197-203. Source of Support: Nil, Conflict of Interest: None declared.
25. Ramstad T, Otten E, Shaw WC. Psychosocial adjustment in

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Date: 10th August 2009 K. L. N. Rao


Original Article Full text online at http://www.jiaps.com

Hirschsprung’s disease diagnosis: Comparison of
immunohistochemical, hematoxilin and eosin staining
Mehrdad Memarzadeh, Ardeshir Talebi, Masod Edalaty, Mehrdad Hosseinpour1,
Nasrin Vahidi2
Departments of Surgery, 1Pathology, Medical School, Isfahan University of Medical Sciences, 1Department of
Surgery, Medical School, Kashan University of Medical Sciences, 2Al-Zahra University Hospital

Address for correspondence: Dr. Mehrdad Hosseinpour, Trauma Research Center, Shahid Beheshti Hospital, Kashan University of
Medical Sciences, Kashan-Iran. E-mail: meh_hosseinpour@yahoo.com

ABSTRACT
Background: The diagnosis of Hirschsprung’s disease (HD) based on the absence of ganglion cells in
hemotoxilin and eosin (H and E) staining has limitations in the diagnosis of immature ganglion cells in
neonates. Methods: In this prospective study, 54 biopsies taken from suspectedHD patients (five mucosal
specimens and 49 full thickness specimens) were studied. In the laboratory, after preparing sections of
paraffin embedded tissues, H and E staining slides were compared with immunohistochemical (IHC) staining
including: S100, NSE, CD117, CD56, Cathepsin D, Vimentin, bcl2, GFAP, Synaptophysin and chromogranin.
Results: The study revealed 30 negative (absence of ganglion cells) cases (55.5%), 17 positive cases (31.04%)
and seven suspected cases (12.9%) ganglion cells on the H and E staining. On IHC staining with CD56 and
Cathepsin D, all of the 17 positive cases detected through H and E, were confirmed for having ganglion cells
and out of 30 cases reported negative on H and E staining, 28(93.3%) were reported negative and two (6.7%)
positive by IHC staining. Of the seven suspected cases H and E staining), IHC staining detectedganglion
cells only in five slides; two remained negative. Conclusions: IHC staining using CD56 and Cathepsin D
improved the accuracy of diagnosis in HD when used in addition to H and E staining technique, especially
for negative or suspicious slides. Among the IHC staining markers, S100, NSE, CD117, Vimentin, bcl2, GFAP,
Synaptophysin and chromogranin were suboptimal in comparison to CD56 and Cathepsin D.

KEY WORDS: Hirschprung Disease, immunohistochemical staining, megacolon
DOI: *****

InTRodUcTIon O n t h e o t h e r h a n d a l t h o u g h AC h E a c t i v i t y
is diagnostically the most useful set of enzyme–
H e m a t ox i l i n a n d E o s i n ( H a n d E ) s t a i n i n g , histochemical reactions, it is not sufficient. AChE stains
Acetylcholinesterase staining (AChE) are commonly the parasympathetic nerve fibers and trunks of fibers
used [1-5] in the diagnosis of Hirschprung’s disease (HD). that increase dramatically in the lamina propria mucosa
However, diagnosis is not possible with H and E every and sub mucous layer, but is not a specific marker for
times, because staining has limitations in the diagnosis ganglion cell.[8] AChE staining requires the experience
of pathologists and in some instances interpretation is
of immature ganglion cells in neonates and the sub
difficult. [9] There are reports of false positive and false
mucosal area in which the ganglion cells are small (three
negative results using this technique.[10]
to five cells per ganglion) and irregularly distributed
and so their identification is difficult and requires high Earlier, the importance of IHC studies has been
expertise.[2,6, 7] Some sections contain only a part of the emphasized in the diagnosis of immature ganglion cells,
cytoplasmic portion of the ganglion cell.The diagnosis hypoganglionosis and other suspicious situations. [11-13]
of those cells as ganglion cells is difficult, because its In this study, we compareIHC staining using neural
staining is very similar to the background of the slide. markers with H and E staining to find out the best

Memarzadeh, et al.: Application of immunohistochemical staining

diagnostic panel for HD. ResUlTs

maTeRIal and meThods In the microscopic study of 54 specimens, H and E
staining revealed absence of ganglion cells (negative)
This is a prospective study conducted in the period in 30 cases (55.5%), presence of cells (positive) in
2001 to 2004. Rectal biopsy specimens from 54 infants 17(31.04%) and suspected presence in seven cases
suspected to be having HD constituted the material (12.9%). In the study of specimens through IHC staining
for the study. There were five mucosal and 49 full with CD56 and Cathepsin D [Figure 1 and 2], all 17 cases
thickness biopsies. The specimens were kept in 10% detected positive through H and E, were confirmed
formalin solution. for having ganglion cells and of the 30 cases reported
negative through H and E staining, 28 (93.3%) were
In the laboratory, after preparing sections of paraffin reportednegative and two (6.7%) positive by IHC. Out
embedded tissues, H and E staining slides were of seven cases suspected of having ganglion cells on
compared with IHC staining including S 100, NSE, H and E staining, we could find ganglion cells in five
CD 117, CD 56, Cathepsin D, Vimentin, BCL2, GFAP, slides while two remained negative [Table 1].
Synaptophysin, chromogranin.
According to our selection criteria, other markers (S100,
The slides for IHC were processed as follows: NSE, CD 117, Vimentin, bcl2, GFAP, Synaptophysin,
• First sections of four µm were obtained and fixed on and chromogranin ) [14] used for IHC staining were
the slides with polyelizine. This was followed by suboptimal in comparison to CD56 and Cathepsin D
antigen retrieval for 10 minutes using heat and citrate [Figure 3].
buffer (pH is equal to six). Then H2O2% 3 and pure
methanol were added for five minutes and sections dIscUssIon
were washed with distilled water. Next, primary
antibody (with negative control) was added for 10 Detection of ganglion cells in H and E sections can be a
minutes and washing was performedSecondary difficult process for the pathologist.[15] The maturation
antibody (biotinylated link) was added for 10 of ganglion cells is incomplete at the time of birth,
minutes and washed Streptavidin – HRP was added especially in the sub mucosal area. [12] Immature
for 10 minutes and washed with P.B.S. After adding ganglion cells may be unipolar or bipolar and can be
substrate chromogen (D.A.B) for 10 minutes, counter mistaken for stromal cells.[12] Sub mucosal ganglion cells
was stained. All the antibodies were from DAKO.- Co
and the slides were scanned for ganglion cells.

The best staining method was appreciated based on the
degree of staining of ganglion cell versus its background
and clear detection of ganglion cells.

Figure 2: IHC staining of Cathepsin D. In CD56 staining the background
is stainable; but ganglion cells are not. (Magnification =10×40)

Table 1: Comparison of Detection of Ganglion Cells in H& E
and IHC staining
H and E H and E H and E
Positive (17) Negative (30) Suspicious (7)
Figure 1: IHC staining of CD56. In Catepsin D staining, the ganglion
cells are stainable; but the background is not Magnification =10×40) IHC Positive 17 2 5
IHC Negative 0 28 2

Author Queries?????
Please Provide High quality Image of Figures 1-3


Figure 3A: IHC staining of (A) Synaptophysin and (B) BCL2. (In Figure 3B: IHC staining of (A) Synaptophysin and (B) BCL2. (In
Synaptophysin staining, the ganglion cells and background are not Synaptophysin staining, the ganglion cells and background are not
stainable well, but in BCL2 staining, the ganglion cells and background stainable well, but in BCL2 staining, the ganglion cells and background
are both stainable. (Magnification 10 × 40) are both stainable. (Magnifications =10 × 40)

are smaller than myenteric plexus ganglion cells,[7] and compare IHC markers with H and E staining to find
pathologists have to prepare between 50 to 400 sections out the best diagnostic panel for detection of ganglion
of H and E stained slides to find ganglion cells.[16] On cells. As shown in figures 1 to 3, ganglion cell detection
the other hand, although AChE staining is the chosen and its staining with CD56 and Cathepsin D were better
technique for some pathologists[12] its diagnosis needs than Bcl2 and Synaptophysin.
experience and its interpretation is difficult in some
instances.[5,9] One of the problems is the interference To conclude, our study shows that IHC markers,
of red blood cell (RBC) acetyl cholinesterase due to including both Cathepsin D and CD56, especially for
hemorrhage in lamina propria.[15] Also, false positive[16] negative or suspicious slides are the best diagnostic
and false negative[15] reactions were reported using this panel for detection of ganglion cells. In Cathepsin
staining technique. D staining, the ganglion cells are stainable, but the
background is not. On the contrary, in CD56 staining
Previous studies have emphasized the importance the background is stainable, but ganglion cells are
of immunohistochemistry in the diagnosis of not, these two methods complement each other. The
HD. [5,12,13,16-18] The obstacles to widespread use of pediatric surgeon was therefore advised to disregard
enzyme-histochemical reactions for HD diagnosis the surgical operation for negative cases. This panel
are linked to technical difficulties in some pathology can detect smaller or immature ganglion cells and
laboratories such as fresh preparing of medium, toxicity also small cytoplasmic portions of those cells. Hence,
of reagents and storage difficulties. Park et al.[15] found the sensitivity and specificity are increased and false
that the main diagnostic pitfall was the interpretation negative and positive results are decreased.
of the enteric nervous plexuses in the transitional zone
and the detection of the indistinct or immature neurons RefeRences
indistinguishable from enteric glial cells or satellite
cells. They showed immunohistochemical study was 1. Gupta M, Beeram MR, Pohl JF, Custer MD. Ileal atresia associated
a very helpful diagnostic adjunct to delineating the with Hirschsprung disease (total colonic aganglionosis). J
Pediatric Surg 2005;40:e5-7.
immature neurons (BCL2), and the size of the enteric 2. Martucciello G, Brizzolara A, Favre A, Lombardi L, Bocciardi
ganglia and neuromuscular innervation (S-100 protein, R, Sanguineti M, et al. Neural crest neuroblasts can colonise
Synaptophysin, and CD56). Another study [19] found aganglionic and ganglionic gut in vivo. Eur J Pediatr Surg
that Synaptophysin-positive synapses distribution 2007;17:34-40.
3. Meijers JH, van der Sanden MP, Tibboel D, van der Kamp AW,
in circular and longitudinal colonic muscles and Luider TM, Molenaar JC. Colonization characteristics of enteric
intermuscular ganglions can reflect functional neural crest cells: Embryological aspects of Hirschprung’s disease.
disturbances of large bowel motility and could be J Pediatr Surg 1992;27:811-4.
helpful in the description of the innervation status of 4. Sukhotnik I, Bernshteyn A, Mogilner JG. The basic biology of
apoptosis and its implications for pediatric surgery. Eur J Pediatr
colonic specimens in HD patients. Surg 2005;15:229-35.
5. Sams VR, Bobrow LG, Happerfield L, Keeling J. Evaluation of
Facing a wide diversity of opinions, we decided to PGP 9.5 in the diagnosis of Hirschsprung’s Disease. J Pathol



1992;168:55-8. 14. Martucciello G, Pini Prato A, Puri P, Holschneider AM, Meier-Ruge
6. Petras R. Hirshsprung’s disease. In: Sternberg, SS. Diagnostic W, Jasonni V, et al. Controversies concerning diagnostic guidelines
surgical pathology, Williams and Wilkins; Philadelphia: 2004. p. for anomalies of the enteric nervous system: A report from the
1390-1. fourth International Symposium on Hirschsprung’s disease and
7. Rosai J. Large Bowel Disease. In: Ackerman’s Surgical Pathology. related neurocristopathies. J Pediatric Surg 2005;40:1527-31.
Philadelphia: Mosby; 2004. p. 777-9. 15. Park SH, Min H, Chi JG, Park KW, Yang HR, Seo JK.
8. Martucciello G. Hirschsprung’s disease, one of the most difficult Immunohistochemical studies of pediatric intestinal pseudo-
diagnosis in pediatric surgery: a review of the problems from obstruction. Am J Surg Pathol 2005;29:1017-24.
clinical practice to the bench. Eur J Pediatr Surg 2008;18:140-9. 16. Ariel I, Vinograd I, Lernau OZ, Nissan S, Rosenmann E. Rectal
9. Chen F, Winston JH 3rd, Jain SK, Frankel WL. Hirschsprung’s mucosal biopsy in aganglionosis and allied conditions. Hum
Disease in a young adult: report of a case and review of the Pathol 1983;14:991-5.
literature. Ann Diagn Pathol 2006;10:347-51. 17. Barshack I, Fridman E, Goldberg I, Chowers Y, Kopolovic J. The
10. Petras R. Hirschsprung’s disease. In: Sternberg, SS. Diagnostic loss of Calretinin expression indicates disease. J Clin Pathol
surgical pathology, Williams and Wilkins; Philadelphia: 2004. p. 2004;57:712-6.
1390-1. 18. Petchasuwan C, Pintong J. Immunohistochemical for intestinal
11. Davis BJ, Ornstein L. High resolution enzyme localisation with ganglion cells and nerve fibers: aid in the diagnosis of
a new diazo reagent, “hexazonium pararosaniline”. J Hist Cyt Hirschsprung’s Disease. J Med Assoc Thai 2000;83:1402-9.
1959;7:297. 19. Dzienis-Koronkiewicz E, Debek W, Chyczewski L. Use of
12. Hall CL, Lampert PW. Immunohistochemistry as an aid in synaptophysin immuno-histochemistry in intestinal motility
the diagnosis of Hirschsprung’s disease. Am J Clin Pathol disorders. Eur J Pediatr Surg 2005;15:392-8.
1985;83:177-81.
13. Mackenzie JM, Dixon MF. An Immunohistochemical study of the
enteric neural plexus in Hirschsprungs’s Disease. Histopathology Source of Support: Nil, Conflict of Interest: None declared.
1987;11:1055-66.

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Flexible ureterorenoscopy and laser lithotripsy in children
Wen-Chan Yeow, Richard Pemberton, Andrew Barker
Department of Pediatric Surgery, Princess Margaret Hospital, Roberts Road, Subiaco WA 6008

Address for correspondence: Dr. Andrew Barker, Princess Margaret Hospital for Children, Roberts Road, Subiaco, WA 6008,
Australia. E-mail: Andrew.Barker@health.wa.gov.au

ABSTRACT
Background: Flexible ureterorenoscopy (FUR) and laser lithotripsy (LL) are techniques used in the
management of upper urinary tract disorders. These techniques, so far established in adults, are now being
used in children as well. We report our experience with 26 cases of pediatric upper urinary tract disorders
treated using these techniques. Methods: In the period from 1997 to 2006, FUR was performed in 26 children
(14 males and 12 females) in the age group of three months to 15 years with a mean age of 8.2 years. Twenty five
were stented prior to undergoing FUR and 24 presented with suspected upper tract stones (17 pelvicalyceal
and seven midureteric). Two cases showed JJ stent migration post-pyeloplasty. Results: Eight cases involved
diagnostic procedures. Six excluded the presence of renal calculi, one had focal medullary sponge kidney,
and one had calcified papillae. There were 15 cases of therapeutic FUR. Of these, 12 had LL with only one
had incomplete stone fragmentation which subsequently passed spontaneously. Other therapeutic procedures
included removal of migrated JJ stents and FUR with the basket removal of a midureteric calculus. Three
cases failed ureterorenoscopy due to technical difficulties. The overall success rate was 88.5% for FUR.
Conclusion: FUR and LL are valuable minimally invasive techniques for the examination and treatment of
pediatric upper urinary tract conditions. Preoperative stenting improves passage of the ureteroscope and
with progressive miniaturization of instruments, the lower weight limit will decrease.

KEY WORDS: Laser lithotripsy, pediatrics, ureteroscopy, urinary calculi
DOI: *****

InTRodUcTIon FUR and laser lithotripsy (LL) have been demonstrated
as safe procedures and to achieve a greater stone-
Flexible ureterorenoscopy (FUR) is a common procedure free rate when compared to ESWL, especially with
performed on adults for many purposes. Paediatric FUR stones greater than one centimeter.[2,3] The minimal
has become more widely practiced primarily due to the invasiveness of FUR, as compared to open surgery, also
availability of smaller caliber ureteroscopes. Its use in allows complete assessment of the urinary tract in cases
the pediatric population has been most beneficial in with suspected upper tract stones.
urolithiasis, though other upper urinary tract conditions
can also be treated. In 2000, van Savage et al. reviewed the management
of distal ureteric calculi in their pediatric population.
Many pediatric cases of urinary calculi occur in the They found that calculi 4mm or greater in size are
Aboriginal population in Australia.[1] Cheah et al. in unlikely to pass and thus will most likely require
their study of 93 Western Australian children found surgical intervention.[4] Our results with pediatric FUR
91.4% of calculi were located in the upper urinary tract and LL are described.
and only 8.6% were in the bladder.[1] Open nephro-
pyelolithotomy or ureterolithotomy, percutaneous meThods
nephrolithotomy or extracorporeal shock wave
lithotripsy (ESWL) would have been the only options The records of all children who had FUR in Western
available to treat these upper tract calculi. Australia from January 1997 to January 2006 were

Yeow, et al.: Running title missing?????

reviewed. The search found 26 children with 12 females bladder was opened for bladder diverticulum repair and
and 14 males. Their ages ranged from three months to ureteric reimplantation. He had suspected urolithiasis on
15 years (mean 8.2 years). There were seven Aboriginal ultrasound. Inspection revealed calcified papillae [Figure 1].
children.
Only eight children underwent FUR [Table 1]. In six, no
Ultrasound showed 24 children with urolithiasis, all calculi were seen in the upper renal tract at time of FUR.
upper tract calculi (17 pelvicalyceal and seven mid- However, three children had matrix stone seen to wash
ureteric). Four children also had intravenous pyelograms out after removal of the JJ stent. Calcified papillae and
(IVP) in their preoperative assessments. They presented focal medullary sponge kidney were diagnosed in two
with symptoms of pain, hematuria, or had recurrent children. Fifteen children had therapeutic procedures,
urinary tract infections. Some were being monitored 13 were for stone disease. One child had a mid ureteric
with serial ultrasounds, which demonstrated persisting calculus, which on FUR was felt to be small enough
and enlarging stone size. Two children underwent FUR to remove with a basket. Twelve underwent LL, with
to remove JJ stents from the ureter. all having successful fragmentation except one, who
on ultrasound during follow up still had three to four
Twenty three of the 24 children with upper tract stone mm fragments. These were treated conservatively and
disease had retrograde pyelogram and JJ stenting passed spontaneously.
performed prior to FUR. The JJ stent enabled ureteric
dilatation for at least two weeks before any attempt Two children had pyeloplasties performed. One child,
was made to pass the ureteroscope. FUR and Holmium: during removal of the JJ stent had the stent snap
YAG (Lumenis VersaPulse Powersuite 20W) LL was within the ureter. The ureteroscope was passed and the
performed in a manner similar to adults. A 7.5Fr Storz proximal part of the stent was located at the level of the
adult flexible cystoscope or a 7.5Fr Olympus flexible
ureteroscope was used. A 9.5/11.5Fr or 10/12Fr Cook
ureteral access sheath was used in all children who had
LL. The ureteroscope was advanced up to the kidney
under fluoroscopy over a guide wire and, during LL, a
second guide wire was left alongside the ureteroscope
for safety. With the ureteroscope in the renal pelvis, each
calyx was individually assessed for calculi. Once the
calculus was located a 200micron laser fiber was used
under direct vision to vaporize the stone to fragments
one mm or smaller in size.

Post LL, the children stayed overnight with a 3F
ureteric catheter draining the upper tract strapped to a
Foley indwelling urinary catheter, which was removed
the next day. The cases that were assessed to have
edematous ureters post procedure had a JJ stent left in Figure 1: Three-Month-Old Child with Preoperative Renal Calculi on
situ which was removed two weeks postoperatively. Ultrasound. Ureterorenoscopy Revealed Calcified Papillae

Table 1: Summary of fexible ureterorenoscopy outcomes
Patients were followed up in the outpatient clinic with
N (subtotal)
urinary tract ultrasounds. The average follow-up period
Therapeutic (15)
was 20 months. The Aboriginal children from the Laser lithotripsy:
northern regions of Western Australia were followed up Successful fragmentation 11
by their community general practitioners and visiting Incomplete fragmentation 1
pediatricians with regular urinary tract ultrasounds. Retrieval JJ stent 2
Ureteroscopic basket removal mid ureteric calculus 1
ResUlTs “Diagnostic” (8)
Negative for calculi 6
Twenty six patients were treated for upper urinary tract Focal medullary sponge kidney 1
disorders; 24 for presumed urolithiasis and the average Calcified papillae 1
stone size was 10.3mm (range three to 21mm). Twenty Failed (successfully converted to open) (3)
five children had JJ stenting before FUR. The child Ureter not accessible 1
not prestented was a three-month-old male who had Narrow calyx infundibulum 1
“open” ureterorenoscopy into his megaureter whilst the Poor vision 1


Yeow, et al.: Running title missing?????

pelvic brim and snared with a basket to successfully without causing ureteric trauma especially at the orifice.
remove the fragment without complications. The
second child who had pyeloplasty had proximal stent In the earlier patients, when experience with FUR
migration and so there was no distal end to grasp at in children was less, all patients had post-operative
cystoscopy. The ureteroscope was passed and the JJ JJ stenting as well. More recently, if there was no
stent was found midureter and removed with a basket significant ureteric edema (assessed at time of
without complications. operation) and minimal ureteroscopic passages, a
ureteric catheter was inserted up to the renal pelvis and
There were three failed FUR cases. In a 2.5 year old child strapped to a bladder catheter overnight. This enabled
with a 12 by 21mm right renal calculus the ureter was too quick, simple removal as a single unit the next morning
edematous for guide wire insertion after her prestented prior to discharge.
JJ stent was removed at the time when ureteroscopy was
attempted. In a 10-year-old male, the ureteroscope was No complications were encountered other than the
unable to be manipulated successfully due to a narrowed failed FUR due to technical or anatomical difficulties
infundibulum of the calyx. The third case was an 11-year- precluding safe completion of the operation. The
old male who originally had calcium oxalate staghorn major complications reported in the literature include
and a lower pole calculus. His LL was abandoned due to stricture formation, ureteric perforation and ureter
reduced vision from the large stone burden being treated avulsion. We have no known long term complications
with lasertripsy. All three children were successfully for the current follow-up period.
converted to open pyelonephrolithotomy. The overall
success rate was 88.5% (23/26). conclUsIon

dIscUssIon FUR and LL are valuable minimally invasive techniques
for management of pediatric upper urinary tract
In our institution, computed tomography (CT) was disorders. Usually only one treatment is required. Calculi
not routinely performed for assessing urolithiasis in can be followed into the renal pelvis if they migrate up
the time period of this paper because of its radiation- the ureter during treatment and hard cysteine stones are
associated cancer risk. Instead, patients first undergo also treatable. FUR is also useful in other conditions such
an ultrasound and occasionally an IVP, if required, to as those requiring renal pelvis examination, biopsy and
confirm the diagnosis. Currently CT is performed more foreign body retrieval. Preoperative stenting improves
often using lower dose targeted non contrast scanning passage of the ureteroscope and with progressive
with helical CT for urolithiasis. miniaturization of the instruments; it will be able to be
performed in smaller children.
All the eight patients who underwent “diagnostic” FUR
were diagnosed as upper tract calculi preoperatively acKnowledgmenT
with calculi ranging from three to five mm. The FURs
were performed to treat the calculi with LL. The surgeon Thanks to Shane La Bianca, Julian Mander, John Stanley and
saw stone fragments when he removed the JJ stent in Rob Thomas for their technical expertise during the initial
three children just prior to FUR. In the six children learning curve.
(where no calculi were seen) we believe the calculi
were dislodged during their procedures. RefeRences

In our series, 25 patients had stenting performed prior 1. Cheah WK, King PA, Tan HL. A review of pediatric cases of urinary
tract calculi. J Pediatr Surg 1994;29:701-5.
to any attempts to pass the ureteroscope. We found this 2. Lam JS, Greene TD, Gupta M. Treatment of proximal ureteral
beneficial as the kidney was relieved of obstruction, calculi: holmium:YAG laser ureterolithotripsy versus
converting an emergency situation to an elective extracorporeal shock wave lithotripsy. J Urol 2002;167:1972-6.
3. Wollin TA, Teichman JM, Rogenes VJ, Razvi HA, Denstedt
operation. It also allowed passive ureteric dilatation.
JD, Grasso M. Holmium: YAG lithotripsy in children. J Urol
However, two general anesthetics are required. 1999;162:1717-20.
4. Van Savage JG, Palanca LG, Andersen RD, Rao GS, Slaughenhoupt
During ureteroscopies, the ureteric orifice can be BL. Treatment of distal ureteral stones in children: similarities to
difficult to intubate and traverse, as it is narrow. In the American urological association guidelines in adults. J Urol
2000;164:1089-93.
Singh et al. the tip (first 2cm, approximately 9Fr) of a 5. Singh A, Shah G, Young J, Sheridan M, Haas G, Upadhyay J.
hydrophillic 14Fr ureteral access sheath is used to dilate Ureteral access sheath fot the management of pediatric renal and
the ureteric orifice.[5] Our access sheaths used were ureteral stones: a single centre experience. J Uro 2006;175:1080-2.
smaller. The benefit with access sheath use is that the
Source of Support: Nil, Conflict of Interest: None declared.
ureteroscope can be passed multiple times, if required,


Is ligation of hernial sac during orchiopexy mandatory?
Nilay Biswas, Nilanjan Mitra, Hiralal Konar, Dipak Ghosh, Sukanta K. Das
Department of Pediatric Surgery, Medical College and Hospital, Kolkata, West Bengal, India

Address for correspondence: Dr. Veena Kumari, LHQ, Medical college & Hospital, 88 College Street, Kolkata-73, WestBengal, India.
E-mail: dr_veenasingh@yahoo.co.in

ABSTRACT
Aim: Traditionally, ligation of hernial sac during orchiopexy is considered mandatory to prevent postoperative
development of hernia. A prospective study was carried out to see if it is actually required based on the
fact that any peritoneal defect closes within 24 hours by metamorphosis of the in situ mesodermal cells.
Methods: Fifty cases of undescended testis, age ranging from eight months to 12 years were enrolled. All
of them underwent standard orchiopexy without ligation of the hernial sac. Results: Follow up of all cases
ranged between 1.5 years to three years. Not a single case was reported with evidence of hernia. Conclusions:
It is unnecessary to ligate the hernial sac during orchiopexy.

KEY WORDS: Ligation of hernial sac, orchiopexy, undescended testis
DOI: *****

InTRodUcTIon the ligation of the hernia sac. No special investigative
workup was done.
The conventional and accepted technique of orchiopexy
recommends that ligation of the hernial sac is mandatory The hernia sac was dealt with after complete mobilization
for prevention of postoperative development of hernia. of the testis through an inguinal incision [Figure 1].
However, it has been seen that during laparoscopic The sac was first opened up, divided and the proximal
orchiopexy there is no difference to simple suturing end of the divided sac was very gently peeled off with
when peritoneum is incised, hernia sac dissected and dissecting forceps as high as possible without damaging
left alone. This may be due to the fact that any peritoneal the cord structures [Figure 2]. It was left as such without
defect closes within 24 hours by metamorphosis of ligation. Standard orchiopexy was then performed by
the in situ mesodermal cells. We report the results of a making subdartos pouch.
study on nonligation of hernia sac during conventional
orchiopexy.
ResUlTs
maTeRIals and meThods All the patients were followed up for 1.5 years to three
years. No inguinal hernia was detected during the
This prospective study was conducted between April
regular follow-up in any child.
2004 and 2007. Fifty children with an age range of eight
months to 12 years with a diagnosis of undescended
testis were included. Of the 50 cases, 38 were unilateral dIscUssIon
and 12 bilateral cases. Of the 38 unilateral undescended
testis, 22 were right-sided and 16 left-sided. In all the Hernia sac has been routinely dissected and meticulously
cases, testis was palpable. Clinically, no cases presented freed from the cord structures and suture ligated
with hernia. After the baseline investigations, informed proximally during inguinal orchiopexy. This is done to
consent of the parents was taken and the procedure achieve adequate length of the cord to bring down the
explained to those parents who were able to understand. testes to the scrotum to its normal position and prevent
All children underwent standard orchiopexy without development hernia postoperatively.

Author Queries?????
Please Provide High quality Image of Figures 1-2
Biswas, et al.: Do we really need to ligate hernial sac during orchiopexy?

Figure 1: Separation and gentle peeling of sac from cord structures Figure 2: Peeling of sac as high as possible

In cases of inguinal hernia in children, Mohta is very thin and tends to tear very easily.
et al. [1] suggested that nonligation of hernia sac during
herniotomy in children has no untoward effect on the Length of testicular vessel: It is found that the most
early complications and recurrence rate. The study important criteria for bringing down the testes in the
was based on the fact that peritoneal defect closes by scrotum is the length of the testicular vessels; in our
metamorphosis of the in situ mesodermal cells. Earlier, procedure extra length of the testicular vessel can be
Shulman et al.[2] showed that ligation of hernia sac in achieved by peeling off the peritoneum as high as
adult herniorrhaphy is a needless step. A prospective possible.
study of laparoscopic inguinal hernia repair in children
by Schier[3] showed that there is no difference to simple No ligation: Accidental ligation of the cord structures
suturing when peritoneum was incised and hernia sac is avoided.
resected. He stressed that an open internal inguinal
ring is not an inguinal hernia.[4] During laparoscopic Our experience suggests that routine ligation of the
orciopexy, Handa et al. [5] showed that closure of hernial sac is not mandatory. This reduces the operative
the internal ring is not necessary. Mobilization of time in all cases and eliminates the possibility of
undescended testes leaves a raw surface which coupled accidental ligation of cord structures.
with the presence of the pulled through spermatic cord
results in effective closure of the internal inguinal ring. RefeRences

In this study we did not ligate the hernia sac during 1. Mohta A, Jain N, Irniraya KP, Saluja SS, Sharma S, Gupta A. Non
ligation of hernial sac during herniotomy: A prospective study.
inguinal orchiopexy. After dissecting the hernia sac free
Pediatr Surg Int 2003;19:451-2.
from the cord, we simply gently peel off the proximal 2. Shulman AG, Amid PK, Lichtenstein IL. Ligation of hernial sac-
cut end of the hernia sac as high as possible. We have A needless step in adult hernioplasty. Int Surg 1993;78:152-3.
performed 50 cases of inguinal orchiopexy with this 3. Schier F. Laparoscopic inguinal hernia repair- A prospective
personal series of 542 children. J Pediatr Surg 2006;41:1081-4.
procedure and followed up for 1.5 to three years. We
4. Schier F. An open internal inguinal ring is not an inguinal hernia.
did not find any complication or untoward effect. So Pediatr Surg Int 2007;23:825.
we conclude that ligation of hernia sac is not necessary 5. Handa R, Kale R, Harjai MM. Laparoscopic orchiopexy: Is closure
in inguinal orhiopexy. We found a few other advantages of the internal ring necessary? J Postgrad Med 2005;51:266-8.
also: Time saving: Several minutes of operating time are
saved as we can avoid the holding of the proximal cut
end of the hernial sac with multiple small haemostatic
Source of Support: Nil, Conflict of Interest: None declared.
forceps and suture ligating it, especially when the sac


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