ArticlePDF Available

NOSOLOGY OF HAND DISEASES IN CHILDREN AND ADOLESCENTS OPERATED IN PROVISIONAL FACILITIES IN A MODEL HOSPITAL - PERNAMBUCO STATE, BRAZIL

Authors:
  • sos Mao Recife
  • Hospital Teknon Barcelona

Abstract and Figures

Unlabelled: To report the frequencies of congenital hand diseases in patients who underwent surgery on a collective mobilization basis at SOS Hand, Recife, Pernambuco, between 2005 and 2009. Methods: Information was collected from 833 children and adolescents who were examined in eight missions. Results: Among the patients, 306 (36.7%) underwent surgery: 240 (78.4%) because of congenital malformation and 66 (21.6%) because of acquired lesions. The most frequent congenital malformations were: syndactyly, 72 cases (30.0%); polydactyly, 30 cases (12.5%); bifid thumb, 19 cases (7.9%); complex hand malformation, 14 cases (5.8%); cleft hand, 13 cases (5.4%); trigger finger, 12 cases (5.0%); camptodactyly, 11 cases (4.6%); and brachysyndactyly, 9 cases (3.7%). The most frequently acquired injuries were: obstetric traumatic lesions, 26 cases (39.4%); hand trauma sequelae, 18 cases (27.3%); cerebral paralysis sequelae, 7 cases (10.6%); electric shock sequelae, 5 cases (7.6%); and burn sequelae, 4 cases (6.1%). Conclusion: The nosology of hand diseases is similar to that of large series of elective surgery, especially regarding congenital deformities. The frequency of acquired hand lesions seems to be higher than the frequency in international series. The collective mobilization system for hand surgery is important for decreasing the need for this activity in public institutions, and it has been shown to be very efficient. The success of the project may provide support for the Brazilian National Health System to enroll hand surgeons in the on-call system, in emergency units.
Content may be subject to copyright.
NOSOLOGY OF HAND DISEASES IN CHILDREN AND
ADOLESCENTS OPERATED IN PROVISIONAL FACILITIES IN
A MODEL HOSPITAL - PERNAMBUCO STATE, BRAZIL
Mauri Cortez1, Rui Ferreira da Silva2, Alain Gilbert3, Carlos Teixeira Brandt4, Philippe Valenti3
Adjunct Professor in the Federal University of Pernambuco; Director of SOS Hand, Recife, PE.1 –
Director of SOS Hand, Recife, PE.2 –
Hand Surgeon, La Chaîne de L’Espoir, Hand Institute, Jouvenet Clinic, 6 Square Jouvenet, Paris.3 –
Titular Professor of Pediatric Surgery, Federal University of Pernambuco.4 –
Work performed at the SOS Hand Institute, Recife, PE.
Correspondence: Rua Minas Gerais 147, Ilha do Leite, 50070-700 Recife, PE. E-mail: mauri@sosmaorecife.com.br
Declaramos inexistência de conflito de interesses neste artigo
ABSTRACT
Objective: To report the frequencies of congenital
hand diseases in patients who underwent surgery on
a collective mobilization basis at SOS Hand, Recife,
Pernambuco, between 2005 and 2009. Methods: Infor-
mation was collected from 833 children and adoles-
cents who were examined in eight missions. Results:
Among the patients, 306 (36.7%) underwent surgery:
240 (78.4%) because of congenital malformation and 66
(21.6%) because of acquired lesions. The most frequent
congenital malformations were: syndactyly, 72 cases
(30.0%); polydactyly, 30 cases (12.5%); bifid thumb,
19 cases (7.9%); complex hand malformation, 14 cas-
es (5.8%); cleft hand, 13 cases (5.4%); trigger finger,
12 cases (5.0%); camptodactyly, 11 cases (4.6%); and
brachysyndactyly, 9 cases (3.7%). The most frequently
acquired injuries were: obstetric traumatic lesions, 26
cases (39.4%); hand trauma sequelae, 18 cases (27.3%);
cerebral paralysis sequelae, 7 cases (10.6%); electric
shock sequelae, 5 cases (7.6%); and burn sequelae, 4
cases (6.1%). Conclusion: The nosology of hand dis-
eases is similar to that of large series of elective sur-
gery, especially regarding congenital deformities. The
frequency of acquired hand lesions seems to be higher
than the frequency in international series. The collective
mobilization system for hand surgery is important for
decreasing the need for this activity in public institu-
tions, and it has been shown to be very efficient. The
success of the project may provide support for the Bra-
zilian National Health System to enroll hand surgeons
in the on-call system, in emergency units.
Keywords Hand Deformities, congenital; Child;
Adolescent
ARTIGO ORIGINAL
Rev Bras Ortop. 2010;45(5):445-52
INTRODUCTION
Although robust epidemiological data as seen in
India(1) is not available in Brazil, it has been observed
that declines in the rates infectious, parasitic and mal-
nutrition-related diseases have been occurring. On the
other hand, there have been relative increases in the
rates of non-communicable diseases, non-transmissible
chronic diseases and diseases of a genetic nature. One of
these sets of illnesses consists of congenital malformations.
The cardiocirculatory system is the most frequent
location for congenital malformations and the second
most frequent location is the musculoskeletal system(2).
The incidence of congenital malformations in the United
States is between 2% and 3% of live births(3,4), and these
malformations may be located in different organs and
systems.
In Brazil, congenital malformations are in second
place among the causes of child mortality and in third
© 2010 Sociedade Brasileira de Ortopedia e Traumatologia. Open access under CC BY-NC-ND license.
446
place among mortality among children under the age of
five years, and are responsible for 10.5% of these cases.
Between 1995 and 1997, death due to malformations ex-
ceeded death due to diarrhea and respiratory infections.
In 1997, cardiovascular abnormalities were responsible
for 39.4% of the deaths due to malformations and ab-
normalities of the central nervous system accounted for
18.8%(5). In Pernambuco, between 1993 and 2003, there
were increases in the coefficients of mortality at early
neonatal, perinatal, neonatal and under-one-year ages,
due to congenital malformations(6).
Although congenital malformations of the hand do
not contribute towards child mortality, they may have
an important effect on the functional capacity, psycho-
logical state and quality of life of young individuals.
Few studies in Brazil have focused on the frequency
and management of such malformations(7-10). In addi-
tion, few Brazilian papers, particularly from surgeons
specializing in hand surgery, have been published in the
international literature(11-13).
In several countries, there has been abundant sci-
entific production on the epidemiological, clinical and
management features of diseases of the hand(14-37). Even
the issues involved in the genesis of these abnormalities
have started to be revealed(34).
Children frequently use their hands to explore the
environment surrounding them, with consequent risks
of trauma or injury. Thus, accidents involving hands oc-
cur frequently, and thermal lesions are the commonest
acquired condition. Obstetric trauma is also a common
condition, and this may lead to paralysis of the brachial
plexus(38-46).
The reorganization of public healthcare actions in
Brazil that started from the creation of the National
Health System (SUS) in 1988 has been strengthening
the role of tertiary care hospitals. Nonetheless, the scar-
city of provision of hospital care at secondary level has
contributed towards the growth of waiting lists for sur-
gical treatment for a variety of conditions of medium
therapeutic complexity, among which hand surgery is
included. Collective mobilization actions with the aim
of carrying out operations on hand disease cases that
require surgical treatment and thereby reducing the wait-
ing lists in public hospitals are rare. Few institutions
conduct such actions, and SOS Hand in Recife, State
of Pernambuco, is one of them. Thus, the aim of the
present study was to report on the nosology of hand
diseases encountered in collective mobilization actions
carried out in this institution, which is a reference point
in this State.
METHODS
A retrospective observational case series study was
conducted, involving young patients who underwent
hand operations in eight collective mobilization actions
at SOS Hand, in Recife, between 2005 and 2009.
The data were gathered from the electronic medical
files that were made available by the institution’s medi-
cal archives service.
The data analyzed were the name, origin, sex, age,
weight and diagnosis.
For the general analysis, all the patients exam-
ined for whom management consisting of expectant
procedures, physiotherapy and surgery was indicated
were included.
The data were input into Excel spreadsheets and were
expressed in terms of absolute and relative frequencies.
The data gathering received prior authorization from
the institution’s director, and the data were used only for
scientific publication.
RESULTS
During the study period, in the eight collective mo-
bilization actions, 833 patients were examined. Among
these, the diagnoses of 306 patients (36.7%) were con-
firmed and these individuals underwent operations.
Nevertheless, for 91 patients (10.9%), although surgery
was indicated, it was not carried out for a variety of rea-
sons, including: non-acceptance by the parents; expec-
tation that good functional results would not obtained;
and presence of diseases that resulted in suspension
of the surgery.
Among the patients operated, 161 (52.6%) were male
and 145 (47.4%) were female. The ages of the patients
operated ranged from six months to 25 years and seven
months, with a mean age of seven years.
Regarding the patients’ origins, the largest propor-
tion 333/833 (40.0%) came from Recife, while 275/833
(33.0%) came from the surrounding metropolitan area
(Figure 1).
For 436 patients, there was no surgical indication at
the time of the collective mobilizations; most of them
were undergoing physiotherapy.
There were 240 cases of congenital malformation
of the hand (78.4%). These cases resulted from abnor-
Rev Bras Ortop. 2010;45(5):445-52
447
thumb, 2/240 cases (0.8%); malformation of the thumb,
2/240 cases (0.8%); and one case (1/240; 0.4%) of each
of the following conditions: finger hypoplasia, finger
aplasia, Alpert syndrome and Polland syndrome.
The frequencies of the acquired conditions of the
hand (66/306; 21.6%) are shown in Table 2.
608 (73.0%)
175 (21.0%)
50 (6.0%)
0
100
200
300
400
500
600
700
Recife and metropolitan
region
*
Other
regions
**
Other
states
***
*Recife: 333; Metropolitan Region: 275.
**Agreste Region: 83; Zona da Mata Region: 67; Sertão Region: 25.
***Paraíba: 20; Alagoas: 15; Ceará: 10; Sergipe: 5.
Figure 1 – Absolute and relative frequencies of regions of origin
of the patients attended during the eight collective mobilizations
AT3/3(ANDIN2ECIFEmalities of embryonic development, and such conditions
were the ones most commonly diagnosed and operated.
The most frequent of these are described in order of
prevalence in Table 1.
Other congenital malformations of the hand were:
hemangioma of the hand, 8/240 cases (3.3%); arthro-
gryposis, 7/240 cases (2.9%); absence or deficiency of
the thumb extensor, 6/240 cases (2.5%); lesion due to
amniotic constriction bands on the fingers, 6/240 cases
(2.5%); agenesis of the thumb, 5/240 cases (2.1%);
triphalangism, 4/240 cases (1.7%); clinodactyly, 4/240
cases (1.7%); macrodactyly, 3/240 cases (1.2%); radi-
oulnar synostosis, 3/240 cases (1.2%); ectodermal dys-
plasia with aplasia of the forearm, 3/240 cases (1.2%);
aplasia of the forearm, 3/240 cases (1.2%); adductus
Other acquired lesions were: sequelae from cerebral
abscess, 2/66 cases (3.0%); scar neuroma, 2/66 cases
(3.0%); acquired syndactyly, 1/66 case (1.5%); and claw
hand due to leprosy, 1/66 case (1.5%).
DISCUSSION
Collective mobilization for hand surgery
The human hand has specialized mechanisms that
enable unique activities. It has great complexity of func-
tions. This high specificity of functions makes it very
sensitive to error during the process of embryogenesis,
which frequently results in congenital abnormalities(34).
These need to be repaired without much delay, so that
children do not develop within environments that tend
to marginalize them.
The fact that there are waiting lists for surgical treat-
ment on children at tertiary-level university reference
hospitals, because of high demand for beds in these
hospitals for treating high-complexity conditions, is a
stimulus towards adopting new programs for surgical
care. Surgery performed on an outpatient basis is one
of the strategies for reducing the length of waiting lists.
One complementary approach has been to implement
collective mobilization actions for a variety of diseases,
including those of the hand. Such actions for hand sur-
gery, with participation by the non-governmental or-
ganization “La Chaine de L’Espoir”, carried out at the
SOS Hand Institute in Recife, has contributed towards
diminishing the waiting lists for hand surgery, thereby
NOSOLOGY OF HAND DISEASES IN CHILDREN AND ADOLESCENTS OPERATED IN PROVISIONAL
FACILITIES IN A MODEL HOSPITAL - PERNAMBUCO STATE, BRAZIL
Rev Bras Ortop. 2010;45(5):445-52
*Simple syndactyly: 59/72 (81.9%); complex syndactyly: 8/72 (11.1%); and syndac-
tyly associated with other malformations 5/72(6.9).
Congenital malformations of the hand N %
Syndactyly* 72 30.0
Polydactyly 30 12.5
Bifid thumb 19 7.9
Complex malformation of the hand 5.8
Cleft hand 13 Trigger finger 12 5.0
Camptodactyly 11 Brachysyndactyly 9 3.7
Table 1 n$ISTRIBUTIONOFFREQUENCIESOFCONGENITALMALFORMATIONSof the hand.
Acquired hand conditions N %
Lesions due to obstetric trauma 26 Sequelae from hand trauma 18 27.3
Sequelae from cerebral palsy 7 10.6
Sequelae from electric shock 5 7.6
Sequelae from burns 6.1
Table 2 n$ISTRIBUTIONOFTHEFREQUENCIESOFTHEACQUIREDHANDconditions.
448
creating a greater possibility for social inclusion of these
patients, who are mostly children.
Emphasis needs to be given to another factor inher-
ent to carrying out collective mobilization actions for
hand surgery. There was an exchange of experiences
between French and Brazilian hand surgeons through
these actions at SOS Hand in Recife, and this contrib-
uted towards achieving the target. Thus, collective mo-
bilization actions may lead to training for specialized
human resources.
Nosology of congenital hand diseases
Syndactyly has appeared as the most common con-
genital malformation of the hand in several series, rep-
resenting 50% of such anomalies. Nonetheless, in other
series, polydactyly has been mentioned as having the
highest incidence. With regard to syndactyly, an analysis
of 7,478,746 births in China found 2,311 cases of this
malformation, which represents an incidence of 3.09
cases per 10,000 live births and stillbirths. In a general
analysis on large series, the incidence of this abnormal-
ity was found to be between 1/2,000 to 1/3,000 live new-
borns. Through stratifying this incidence as syndactyly
alone or syndactyly associated with other malforma-
tions, the rates become 1.32/10,000 and 1.77/10,000,
respectively(22). These data are concordant with the fact
that this congenital malformation was the one most fre-
quently treated in the collective mobilization actions
at SOS Hand in Recife. This probably corresponds to
greater incidence of this malformation in the regions of
origin of the patients included in the present study.
With regard to polydactyly, a study comparing the
incidence of polydactyly in Latin America (ECLAMC)
involving 3,128,957 live and stillborn neonates and
another collaborative study on congenital malforma-
tions in the Spanish Community (ECEMC) involving
1,093,865 live newborns and 7,271 stillbirths found
that the incidence was 150.2/100,000 for ECLAMC and
67.4/100,000 for ECEMC. These rates were higher than
the syndactyly rates(10). It seems that the most accepted
incidence rate is 1/1,000 live newborns(18,19). In line with
the prevalence trends for congenital hand diseases, poly-
dactyly was the second most common malformation in
the present study. This was similar to the findings from
an important surgical series in another country(20). It
needs to be emphasized that the cases of bifid thumb and
triphalangism were stratified as malformations indepen-
dent of polydactyly, whereas in reality they form part of
the wider group of types of polydactyly. In this circum-
stance, the frequency would be 53/240 (22.1%).
It is possible that there may have been a bias towards
relatively low frequency of polydactyly in the present
study, given that simpler cases of this disease might
have been operated by other professionals, such as pe-
diatric surgeons or plastic surgeons, in institutions that
attend cases of lower complexity.
Bifid or double thumb has been considered to be
the most frequent abnormality of the upper extremity,
and it has been reported as the most common form of
polydactyly(15,16,20). In studies based on registers of
congenital abnormalities, this malformation presents
a general prevalence that is estimated to be 2.08 per
10,000 live newborns. In some countries like Bolivia,
the prevalence reaches 3.37 per 10,000 live newborns(22).
It also needs to be considered that, over the eight col-
lective mobilizations, four cases of triphalangism of
the thumb were operated, and that these can also be
considered to be within the spectrum of polydactyly,
which would raise the prevalence of this condition to
53/240 (22.1%).
In the more consistent surgical series, bifid thumb is
represented by a relatively modest number of cases. For
example, in an international review covering 66 years
of surgical experience, the complications from 54 cases
were reviewed, of which 16 underwent simple exci-
sion and 38 underwent reconstructive surgery of greater
complexity(14). Another example that demonstrates the
small number of cases reported in the literature is a
review of 10 years of experience of surgical treatment
of radial polydactyly at a large plastic surgery center in
Brazil, in which the surgical experience of 19 cases of
bifid thumb was analyzed(14). In the present series, the
inclusion of 19 cases operated over the eight collective
mobilizations at SOS Hand, in Recife, over a five-year
period, makes the present study one of the largest case
series on this disease in Brazil and emphasizes not only
the social importance but also the scientific importance
of the collective mobilizations for hand surgery imple-
mented at this institution.
The classifications of congenital malformations of
the hand make it possible to include most of the pa-
tients in specific phenotypes that provide the basis for
nosological classification. However, in some cases, it is
difficult to fit the anatomical details to specific lesions.
Fusions and lack of organization of bone components
may complicate the particular phenotypic features of
Rev Bras Ortop. 2010;45(5):445-52
449
a nosological type. These patients presented a diver-
sity of phenotypes that were superposed within other
diagnoses in the existing classification(35). Under these
circumstances, 14/240 (5.8%) of the cases in the present
series were thus catalogued. Most of them were patients
with syndromes.
Cleft hand is an infrequent malformation, with an
incidence of around one case per 20,000 live newborns.
In some cases, it is associated with malformation of the
feet. In an evaluation on 850,742 live births in Canada,
the incidence was 1/19,784 neonates(36). It seems that
there has been an increase in the prevalence of this
abnormality, given that in Denmark in the 1940s, its
prevalence was one case per 111,777 inhabitants(20). The
diagnosis and treatment of 13 cases of cleft hand in the
present study demonstrates the range of hand pheno-
types that can be observed in collective mobilizations
for surgery. Moreover, these cases provide density of
surgical training and technical refinement for treating
new cases, thereby increasing the credibility the institu-
tion and its professionals.
The thumb is the digit most frequently involved when
trigger finger is diagnosed in children. The congenital
nature of this malformation is still a matter of contro-
versy and the etiology is unknown. This entity, which is
also known as trigger thumb due to development error,
corresponds to a spectrum of abnormalities that result in
loss of extension and abduction of the digit. There are
surgical series reported in the worldwide literature that
include significant numbers of cases(20). For example,
at a hand surgery service in Turkey, a series of surgical
cases involving 47 trigger thumbs in 36 children was
reported(23). In another important series of case reports
from an international hand surgery service in the Neth-
erlands, operations carried out on 27 children over a
five-year period were reported. Of these, 16 were single
abnormalities(37).
Likewise, the attendance provided for 12 chil-
dren with trigger thumb in the present study shows
the range of phenotypes of hand malformations that
are diagnosed and treated in these collective mobili-
zations and, in some manner, mirrors the likely inci-
dence of this malformation among the population of the
State of Pernambuco.
Camptodactyly represents around 1% of congenital
malformations of the hand and most frequently affects
the fifth finger. The anatomical abnormalities of this
condition are generally related to the lumbrical muscles,
superficial digital flexors, superficial subcutaneous tis-
sue and anomalous extensor muscles(31). The 11 cases
(4.6%) that formed part of the present study are a larger
phenotypic representation of this condition than in other
series(20,31). They may constitute a more formal surgi-
cal indication because of the functional abnormality that
this condition causes, or may indicate lower availability
of conservative treatment in the region, thereby increas-
ing the frequency of this condition on waiting lists.
Brachysyndactyly is generally recognized within the
diagnostic context of syndromes such as Polland. Its
incidence is very low, and it may be associated with
other malformations of the musculoskeletal system. The
record of nine cases operated during the collective mo-
bilizations and reported in the present series confirm the
nosological diversity observed in hand surgery carried
out in the form of collective mobilization actions.
The management of eight cases (3.3%) of heman-
gioma of the hand in the present series of surgical con-
ditions of the hand reinforces the idea of nosological
variety of conditions treated in these collective mobi-
lizations , given that this relatively common condition
occurs most frequently in the skin of the head and neck
region (60%) and trunk region (25%). The cases oper-
ated in the present series were of child hemangioma
type, which is the most common type of vascular ab-
normality seen in hands. In this regard, hemangiomas
represent around 5% of the benign tumors located in
the hands.
The diagnosis of arthrogryposis involves a set of
more than 300 diseases in which congenital contrac-
tures are present. The incidence of this abnormality has
been estimated to be one case in every 3,000 to 5,000
live newborns. The point in common, among cases of
this abnormality, is the lack of movement in a normally
formed joint, which results in replacement of the muscle
by fibrous and fatty tissue, thickening of the joint capsule
and ligaments, and stiffness. Although this abnormality
most frequently affects the lower limbs, it may affect
the upper limbs, including the shoulder, elbow, hand and
fingers(44). Most of these patients are treated conserva-
tively, but some cases require surgical intervention. The
inclusion of seven cases (2.9%) of arthrogryposis in the
nosology of the congenital deformities operated in the
collective mobilizations shows not only the importance
of collective mobilization actions for hand surgery but
also the need to expand the availability of reference
centers for hand surgery.
Rev Bras Ortop. 2010;45(5):445-52
NOSOLOGY OF HAND DISEASES IN CHILDREN AND ADOLESCENTS OPERATED IN PROVISIONAL
FACILITIES IN A MODEL HOSPITAL - PERNAMBUCO STATE, BRAZIL
450
The remaining cases, with lower relative frequencies,
represent rare congenital malformations of the hand that
are generally published in the form of case reports or
small case series.
Overall, from analysis on the nosology and man-
agement of the congenital hand diseases treated over
the eight collective mobilizations, it was observed that
the field of knowledge of hand surgery is expanding,
particularly with regard to congenital malformations of
the hand. This has increased surgeons’ knowledge and
has resulted in new treatment methods and a more sci-
entific surgical approach. The collective mobilizations
carried out in partnership with specialists from abroad
contributed towards this successful enterprise.
New technologies such as osteogenic distraction
and transfers of pedicle flaps have been added to the
treatment principles that were well established by the
pioneers of hand surgery. These have made it possible
for hand surgeons to treat new problems and deal with
old problems in new ways. Nevertheless, despite the
growing success, challenges persist for hand surgeons.
Among these are the challenges of constructing joints
and expanding within the field of fetal surgery(45).
Nosology of acquired diseases of the hand
Lesions caused by obstetric trauma that affect the
upper limbs are the diseases most frequently operated.
These represented around 40% of the acquired lesions
that were operated during the eight collective mobili-
zations. Such diseases present very variable incidence,
ranging from 0.42 to 5.1 per 1,000 live newborns(45,46). It
seems that this disease is associated with the type of de-
livery, such that it is seen more frequently in newborns
coming from normal delivery. It should be emphasized
that surgical cases only constitute 25% of the children
with this obstetric complication. Since the great major-
ity of the children operated during the collective mobi-
lizations were from lower socioeconomic classes, it is
possible that because natural delivery is more common
among mother from these classes, the higher representa-
tion in this study results from this reality. On the other
hand, it is important to note that preventive measures
such as indications for cesarean delivery in appropri-
ate situations, particularly for fetuses with estimated
weights greater than 4,500 grams, and avoidance of
“excessive” lateral traction of the fetus at the time of
delivery labor, may diminish the frequency of occur-
rence of this complication(46).
Sequelae from hand trauma occur more frequently
among young adults, and men are more commonly af-
fected. The most common causes are work accidents
through using machines, followed by trauma due to
vehicle accidents. Among children, domestic accidents
and accidents within the children’s environment(38-40),
particularly at school and in traffic, are the most com-
mon causes. Like in the present series, other studies
have shown that accidents with glass receptacles and
falls at home were the most common causes of hand
sequelae. Likewise, as in other series, breakage of the
finger flexor tendons was the most common injury. It is
important to note that the children in the present study
had first been attended at hospital units that did not
specialize in hand treatment.
It should be emphasized that the numbers of traumat-
ic events affecting the upper limbs have been increasing,
with increases in the kinetic energy involved and, con-
sequently, greater complexity of injuries. Traumatic in-
juries to the hand, with loss of skin tissue and exposure
of prime structures, require coverage in order to protect
these structures or to facilitate future reconstruction.
These points provide support for the notion that indi-
viduals with hand lesions should primarily be attended
in reference units. On the other hand, it is important
to mention that preventive measures can be taken in
order to diminish the cost of these interventions, and
that the costs of implementing preventive measures may
be lower than the costs of surgical treatment for such
lesions(41).
Sequelae in the upper limbs resulting from spastic
cerebral palsy are presented by children who have ce-
rebral ischemia. This condition affects around one in
every 500 births. There are no reliable statistics in Bra-
zil, but in developed countries, the prevalence of this
condition has grown with the advent and dissemination
of neonatal intensive care units. It is important to note
that the preventive measures for this condition include
the use of progesterone of corticoids to prolong preg-
nancy, thereby avoiding the birth of premature infants;
limitation of the number of fetuses in pregnancies in-
duced through fertilization; and induced hypothermia
for newborns with encephalopathy due to hypoxia and
cerebral ischemia(46).
Sequelae from electric shocks to the upper limbs
of children generally occur, like in the present series,
in situations of inadequate electrical installations, in
homes or elsewhere, especially on the periphery of ur-
Rev Bras Ortop. 2010;45(5):445-52
451
ban centers(47,48). The attendance provided for five chil-
dren with such conditions during the eight collective
mobilizations attests to the need to prevent such lesions,
which generally comes through education.
Sequelae from burns on hands frequently occur in
developed countries and probably also in Brazil. In the
United States, one third of burn patients are of pedi-
atric age. Involvement of the arm and hand is a com-
mon occurrence(49). It is important to emphasize that
adequate management in specialized units at the time
of the initial attendance improves the functional result
and diminishes the sequelae that give rise to subsequent
surgical interventions, like in the present series.
Other acquired lesions such as sequelae from cere-
bral abscess, scar neuroma, acquired syndactyly and
cleft hand due to leprosy have occurred sporadically in
hand surgery series carried out as collective mobiliza-
tions. In this respect, the present study is novel within
the Brazilian literature.
Attendance for surgically-treated hand disease
through the Brazilian National Health System
With regard to attendance provided for individuals
with surgically-treated hand disease through the Brazil-
ian National Health System (SUS), it needs to be noted
that although the decentralization of SUS activities to
municipalities has achieved a variety of advances (pri-
mary care in all municipalities and increased imple-
mentation of the Family Healthcare Program and Com-
munity Health Agent Program, etc), this movement has
not enabled the same degree of expansion of specialized
services, hospital beds, intensive care, elective surgery
or emergency surgery, including hand surgery.
SUS maintains its provision of these services through
the private sector, and SUS administrators have been
unable to regulate such services adequately, with a few
honorable exceptions. There has not been any expansion
of the capacity to link together several municipalities in
order to integrate and hierarchically organize these ser-
vices and to do this between these services and primary
care. The result has been fragmentation, multiplication
of procedures, inadequate incorporation of technology,
and so on(50). In this regard, professionals specializing
in hand surgery should form an essential part of the
attendance for SUS users, with advisory systems or on-
call presence in emergency units, and as departments
or services in public hospitals dealing with medium
and high-complexity cases, particularly in university
hospitals.
The alternative, in which hand surgery is performed
in private hospitals in a system of collective mobiliza-
tion actions, with interactions with non-governmental
organizations, as described in the present study, shows
the possibility that society can successfully seek to par-
tially replace the State with regard to its constitutional
function of providing healthcare as a right for all Brazilians.
CONCLUSION
The nosology of hand diseases among the patients
operated during the eight humanitarian collective mo-
bilizations included in this study was, in a general man-
ner, similar to that of large elective hand surgery series,
particularly with regard to congenital deformities.
The system of collective mobilization for hand sur-
gery is important for diminishing the lack of such ac-
tivities in public institutions and was shown to be very
efficient. This report on the nosology of hand diseases
that were operated under the collective mobilization
system may be useful for SUS, regarding provision of
professional participation in hospital units, especially in
emergencies, for SUS users.
REFERENCES
Suresh S, Thangavel G, Sujatha J, Indrani S. Methodological issues in set-1.
ting up a surveillance system for birth defects in India. Natl Med J India.
2005;18(5):259-62.
Sípek A, Gregor V, Sípek A Jr, Horácek J, Klaschka J, Skibová J, et al. Birth de-2.
fects in the Czech Republic in 1994-2007. Ceska Gynekol. 2009;74(1);31-44.
Edmonds LD, James LM. Temporal trends in the prevalence of congenital 3.
malformations at birth based on the birth defects monitoring program, United
States, 1979-1987. MMWR CDC Surveill Summ. 1990;39(4):19-23.
Mattos TC, Giuliani R, Hasse HB. Congenital malformations detected in 731 4.
autopsies of children aged 0 to 14 years. Teratology. 1987;35(3):305-7.
Victora CG. Intervenções para reduzir a mortalidade infantil, pré-escolar e 5.
materna no Brasil. Rev Bras Epidemiol. 2001;4(1):3-69.
Arruda TAM, Amorim MM, Souza AS. Mortalidade determinada por anomalias 6.
congênitas em Pernambuco, Brasil, de 1993 a 2003. Rev Assoc Med Bras.
2008;54(2):122-6.
Tuma Júnior P, Arrunategui G, Wada A, Friedhofer H, Ferreira MC. Rectangular 7.
flaps technique for treatment of congenital hand syndactily. Rev Hosp Clin Fac
Med São Paulo. 1999;54(4):107-10.
Boeing M, Paiva LCF, Garcias GL, Roth MGM, Santos IS. Epidemilogia das 8.
polidactilias: um estudo de casos e controles na população de Pelotas-RS. J
Pediatria. 2001;77(2):148-52.
Pardini AG, Santos MA, Freitas AD. Bandas de constrição congênitas. Acta 9.
Ortop Bras 2001;9(2):3-10.
Barboza LE, Prestes Neto R, Fonseca MJA, Santos JBG, Falopas F. Tratamento 10.
cirúrgico das sindactilias congênitas da mão pela técnica de Bauer. Rev Bras
Ortop. 2006;41(3):54-60.
Rev Bras Ortop. 2010;45(5):445-52
NOSOLOGY OF HAND DISEASES IN CHILDREN AND ADOLESCENTS OPERATED IN PROVISIONAL
FACILITIES IN A MODEL HOSPITAL - PERNAMBUCO STATE, BRAZIL
452
Castilla EE, Lugarinho da Fonseca R, da Graca Dutra M, Bermejo E, Cuevas 11.
L, Martínez-Frías ML. Epidemiological analysis of rare polydactylies. Am J Med
Genet. 1996;65(4):295-303.
Castilla EE, Lugarinho R, da Graça Dutra M, Salgado LJ. Associated anomalies 12.
in individuals with polydactyly. Am J Med Genet. 1998;80(5):459-65.
Siqueira MA, Sterodimas A, Boriani F, Pitanguy I. A 10-year experience with 13.
the surgical experience of surgical treatment or radial polydactyly. Ann Ital Chir.
2008;79(6):441-4.
Townsend DJ, Lipp EB Jr, Chun K, Reinker K, Tuch B. Thumb duplication, 14.
66 years’ experience--a review of surgical complications. J Hand Surg Am.
1994;19(6):973-6.
Ozalp T, Coskunol E, Ozdemir O. Thumbs duplication: an analysis of 72 thumbs. 15.
Acta Orthop Traumatol Turc. 2006;40(5):388-91.
Vasseur C, Martinot V, Pellerin P, Herbaux B, Debeugny P. Palmar burns of the 16.
hand in children. 81 cases. Ann Chir Main Memb Super. 1994;13(4):233-9.
Leck I, Lancashire RJ. Birth prevalence of malformations in members of different 17.
ethnic groups and in the offspring of matings between them, in Birmingham,
England. J Epidemiol Community Health. 1995;49(2):171-9.
Kostakoglu N, Kayikcioglu A, Safak T, Ozcan G, Kecik A, Gursu G. Macrodac-18.
tyly: report of eight cases of a rare anomaly. Turk J Pediatr. 1996;38(1):73-9.
de la Torre J, Simpsom RL. Complete digital duplication: a case report and 19.
review of ulnar plydactyly. Ann Plast Surg. 1998;40(1):76-9.
Larsen CF. Demography and social impact. In: Gupta A, Kay SPJ, Scheker 20.
LR. The growing hand: diagnosis and management of the upper extremity in
children. New York: Mosby; 2000. p.121-4.
Orioli IM, Castilla EE. Thumb/hallux duplication and preaxial polydactyly type 21.
I. Am J Med Genet. 1999;82(3):219-24.
Herdem M, Bayram H, Toğrul E, Sarpel Y. Clinical analysis of the trigger thumb 22.
of childhood. Turk J Pediatr. 2003;45(3):237-9.
Dautel G. Camptodactylies. Chir Main. 2003;22(3):115-24.23.
Deunk J, Nicolai JP, Hamburg SM. Long-term results of syndactyly correction: 24.
full-thickness versus split-thickness skin grafts. J Hand Surg. 2003;28(2):125-
30.
Fernández-Vázquez JM, Schenk-Palao J, Fernández-Palomo J, Camacho-25.
Galindo J. Triphalangeal thumb. Cir Cir. 2003;71(6):469-74.
Dai L, Zhou GX, Zhu J, Mao M, Heng ZC. Epidemiological analysis of syndactyly 26.
in Chinese perinatals. Zhonghua Fu Chan Ke Za Zhi. 2004;39(7):436-8.
Abdel-Ghani H, Amro S. Characteristics of patients with hypoplastic thumb: a 27.
prospective study of 51 patients with the results of surgical treatment. J Pediatr
Orthop B. 2004;13(2):127-38.
Sebastin SJ, Puhaindran ME, Lim AY, Lim IJ, Bee WH. The prevalence of 28.
absence of the palmaris longus--a study in a Chinese population and a review
of the literature. J Hand Surg Br. 2005;30(5):525-7.
Velisavljev-Filipović G. Arthrogryposis multiplex congenita – a rare congenital 29.
stiff joints syndrome. Med Pregl. 2006;59(7-8):375-9.
Forrester MB, Merz RD. Rates for specific birth defects among offspring of 30.
Japanese mothers, Hawaii, 1986-2002. Congenit Anom (Kyoto). 2006;46(2):76-
80.
Salazard B, Quilici V, Samson P. Camptodactyly. Chir Main. 2008;27(Suppl 31.
1): S157-64.
Al-Qattan MM, Al-Shanawani B, Al-Thunayan A, Al-Namla A. The clinical fe-32.
atures of ulnar polydactyly in a middle eastern population. J Hand Surg Eur.
2008;33(1):47-52.
Ali M, Jackson T, Rayan GM. Closing wedge osteotomy of abnormal middle 33.
phalanx for clinodactyly. J Hand Surg Am. 2009;34(5):914-8.
Ogino T. Clinical features and teratogenic mechanisms of congenital absence 34.
of digits. Dev Growth Differ. 2007;49(6):523-31.
Elliot AM, Reed MH, Evans JÁ. Central ray deficiency with extensive syndactily: 35.
a dilemma for classification. Genet Couns. 2009;20(1):27-43.
Elliot AM, Reed MH, Chudley AE, Chodirker BN, Evans JA. Clinical and epide-36.
miological findings in patients with central ray deficiency: split hand foot malfor-
mation (SHFM) in Manitoba, Canada. Am J Med Genet A. 2006:140(13):1428-
39.
van Loveren M, van der Biezen JJ. The congenital trigger thumb: is release 37.
of the first annular pulley alone sufficient to resolve the triggering? Ann Plast
Surg. 2007;58(3):335-7.
Choi M, Armstrong MB, Panthaki ZJ. Pediatric hand burns: thermal, electrical, 38.
chemical. J Craniofac Surg. 2009;20(4):1045-8.
Evans-Jones G, Kay SP, Weindling AM, Cranny G, Ward A, Bradshaw A,, 39.
et al. Congenital brachial palsy: Incidence, causes, and outcome in the Uni-
ted Kingdom and Republic of Ireland. Arch Dis Child Fetal Neonatal Ed.
2003;88(3):F185-9.
Andersen J, Watt J, Olson J, Aerde JV. Perinatal brachial plexus palsy. Paediatr 40.
Child Health. 2006;11(2):93-100.
Sahin F, Dalgic Yücel S, Ylmaz F, Ercalik C, Esit N, Kuran B. Characteristics 41.
of pediatric hand injuries followed up in a hand rehabilitation unit. Ulus Travma
Acil Cerrahi Derg. 2008;14(2):139-44.
Claudet I, Toubal K, Carnet C, Rekhroukh H, Zelmat B, Debuisson C, Cahuzac 42.
JP. When doors slam fingers jam! Arch Pediatr. 2007;14(8):958-63.
Ljunberg EM, Carlsson KS, Dahlin LB. Cost per case or total cost? The potential 43.
prevention of hand injuries in young children – retrospective and prospective
studies. BMC Pediatr. 2008;8(28):1-11. Disponível em: http://www.biomedcen-
tral.com/1471-2431/8/28.
Parsch K, Pietrzak S. Congenital multiple arthrogryposis. Orthopade. 44.
2007;36(3):281-90.
McCarroll HR. Congenital anomalies: a 25-year overview. J Hand Surg Am. 45.
2000;25(6):1007-37.
O’Shea TM. Diagnosis, treatment, and preventions of cerebral palsy. Clin Obstet 46.
Gynecol. 2008;51(4):816-28.
Choi M, Armstrong MB, Panthaki ZJ. Pediatric hand burns: thermal, electrical, 47.
chemical. J Craniofac Surg. 2009;20(4):1045-8.
Ogilvie MP, Panthaki ZJ. Electrical burns of the upper extremity in the pediatric 48.
population. J Craniofac Surg. 2008;19(4):1040-6.
Birchenough SA, Gampper TJ, Morgan RF. Special considerations in the 49.
management of pediatric upper extremity and hand burns. J Craniofac Surg.
2008;19(4):933-4.
Assis E, Cruz VS, Trentin EF, Lucio HM, Meira A, Monteiro JCK, Cria SM, 50.
Focesi MR, Cielo CA, Guerra LM, Farias RMS. Regionalização e novos rumos
para o SUS: a experiência de um colegiado regional. Saude Soc. [online].
2009;18(1):17-21.
Rev Bras Ortop. 2010;45(5):445-52
ResearchGate has not been able to resolve any citations for this publication.
Article
Full-text available
Despite all the advances that have occurred in Sistema Único de Saúde (SUS - National Health System) since its implementation, its weaknesses and limits are also remarkable. One of them, in spite of the constitutional guideline for regionalization, is that, until recently, there had been no concrete policy to stimulate integration between municipalities and, consequently, between health regions, which are necessary to ensure integral care. In the last three years, however, the Ministry of Health, with the implementation of the Health Pact in its management dimension, started this process, which has been promising to the development and growth of the national SUS. This study presents the positive experience of implementation of a Regional College in the State of São Paulo, in the region of Campinas, through which managers and technicians of the health departments are becoming empowered subjects of the construction of Health in the region.
Article
Full-text available
A síndrome da banda de constrição congênita é uma patologia rara, de ocorrência ocasional na natureza e não existe predisposição genética. É freqüentemente associada a amputações de dedos ou membros, sindactilia, acrossindactilia (sindactilia fenestrada), mal formações em face, tórax e/ou abdômen. O presente trabalho representa nossa experiência na abordagem e tratamento da Síndrome da Banda de Constrição Congênita. Foram tratados 10 pacientes atendidos em nosso serviço em um período de 20 anos.
Article
A síndrome da banda de constrição congênita é uma patologia rara, de ocorrência ocasional na natureza e não existe predisposição genética. É freqüentemente associada a amputações de dedos ou membros, sindactilia, acrossindactilia (sindactilia fenestrada), mal formações em face, tórax e/ou abdômen. O presente trabalho representa nossa experiência na abordagem e tratamento da Síndrome da Banda de Constrição Congênita. Foram tratados 10 pacientes atendidos em nosso serviço em um período de 20 anos.
Article
Camptodactyly is a permanent, nontraumatic flexion of the proximal interphalangeal joint. The prevalence is around 1% and the little finger is most commonly affected. Two groups may be identified, depending on the age of onset: camptodactyly severe within 2–3 years or beginning in the early teens. Many anatomical abnormalities have been incriminated as the cause of camptodactyly: anomalous lumbrical muscle, short flexor digitorum superficialis, retractile subcutaneous tissue, anomalous extensor muscle. Splinting is always required, with dynamic and/or static splinting of the proximal interphalangeal joint. If improvement is not obtained with splinting, surgery can be proposed. Surgical treatment must correct the soft tissue contracture (flap and skin graft), the tendinous anatomical abnormalities and the joint contracture. Splinting and physiotherapy is necessary after surgery.
Article
AimEpidemiological analysis in a universitary paediatric emergency unit of children admitted after accidental injuries resulting from fingers crushed in a door.
Article
It was recently shown that hand postaxial polydactyly differed from foot postaxial polydactyly. The aim of this work was to test whether thumb and hallux duplication also had different clinical and epidemiological characteristics, depending on limb involvement. We studied 920 newborn infants with first digit duplication, ascertained among 3,444,374 births by the Latin-American Collaborative Study of Congenital Malformations (ECLAMC), from 1967 to 1995. Since biphalangeal thumb duplication or hallux duplication can occur in families with triphalangeal thumb or polysyndactylous propositi, these groups were also analyzed. The 715 isolated (nonsyndromal) cases (prevalence 2.08 per 10,000) were subdivided into five groups: thumb duplication (N = 568; prevalence: 1.65/10,000); hallux duplication (N = 82; prevalence: 0.24); thumb and/or hallux duplication plus syndactyly (polysyndactyly) (N = 37; prevalence: 0.11); triphalangeal thumb (N = 24; prevalence: 0.07), and thumb duplication plus hallux duplication (N = 4; prevalence: 0.01). Both thumb and hallux duplication groups showed a significant excess of males, and right sidedness was also more frequent in both of them, though without statistical significance for hallux duplication. Thumb duplication was more often unilateral (94.7% versus hallux duplication of 81.5%), and its prevalence was higher in Bolivia (3.37/10,000) than in the other 10 Latin-American countries included (1.62/10,000). In a subseries of 405 preaxial polydactylies with matched controls, a logistic regression analysis showed that birth weight and gestational age had an effect on the calculated risk of having an infant with thumb duplication, while first trimester vaginal bleeding had only a borderline effect. None of the polydactyly groups showed abnormal values for twinning, perinatal mortality, ethnicity, maternal education, parental ages, parity, parental subfertility, or consanguinity. There were 70/405 familial cases. Their pedigrees were compatible with autosomal dominant inheritance with a 9% penetrance for thumb duplication and hallux duplication and a 70% penetrance for triphalangeal thumb and polysyndactyly. Inheritance of thumb duplication and probably the untested inheritance of hallux duplication were also compatible with a four-locus multiplicative model. The observed differences in laterality, geographical distribution, birth weight, gestational age, and first trimester vaginal bleeding between thumb duplication and hallux duplication groups suggested that apparent preaxial polydactyly type 1 is a causally heterogeneous group. Am. J. Med. Genet. 82:219–224, 1999. © 1999 Wiley-Liss, Inc.