Professional Documents
Culture Documents
Vol. 12 No. 4
JanMar 2012
Perinatology
Journal of Perinatal and Neonatal Care
Indexed in
EMBASE and CINAHL
Vol. 12 No. 4
JanMar 2012
Perinatology
Journal of Perinatal and Neonatal Care
Editor in chief
Dr Jayashree B Keshav
Bangalore
Editorial Board
Dr DK Guha, Delhi
Prof. Anil Narang, Chandigarh
Dr Duru Shah, Mumbai
Dr Pratap Kumar, Manipal
Dr S Suresh, Chennai
Dr MKC Nair, Trivandrum
Dr SS Agarwal, Lucknow
Dr Evita Fernandes, Hyderabad
Indexed in
EMBASE and CINAHL
General
Information
Perinatology
The Journal
Perinatology (ISSN 0972-2408), one of the
few journals dedicated to the emerging
multidisciplinary field of perinatal medicine,
is published and distributed quarterly (in the
months of January, April, July, and October
every year) at The Himalaya Drug Company.
The journal publishes original research articles,
review articles, brief reports on clinical and
laboratory observations, case reports, and
clinical studies. The journal is indexed in
EMBASE, the Excerpta Medica database,
CINAHL database, and Cumulative Index
to Nursing and Allied Health Literature print
index.
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Perinatology
Contents
Research Articles
Battling with Rising Prevalence of Gestational Diabetes MellitusAre Clinicians in
India on the Right Track?
Hema Divakar, Manyonda IT
133
140
Review Article
Neonatal Continuous Positive Airway Pressure Ventilation
Sankaran K.
144
149
Picture Quiz
153
Self-assessment Quiz
155
157
Short Communications
Prenatal Diagnosis of Apert Syndrome in Second TrimesterA Case Report
Phadke SR, Mandal K, Ranganath P
159
163
165
169
Instructions to Authors
173
EMBLEM
Now
in a
new
look...
Perinatology
Editorial
Dear Friends,
I am glad to present to you the final issue of Volume 12 of Perinatology. By the
time the journal reaches you we will be well into 2012. Let me wish you, on
behalf of the editorial board and publishers, a very successful, peaceful, and
healthy 2012.
The journal, in its new format and style under the new publishers M/s The
Himalaya Drug Company, has successfully completed 1 year. The effort put
in by them for this journal deserves appreciation and applause. We plan to
restructure the editorial board and advisory committees to bring in academic
and experienced professionals who will take the journal to new heights. The
board members will be introduced in subsequent issues with short write-up on
each of them.
At the same time, as readers, your feedback and contributions in the form
of articles, and case reports are very crucial in our efforts to achieve high
standards. Let us all work toward achieving this goal.
Have a wonderful year ahead!
Perinatology
Theme
Research
RESEARCH
Articles
ARTICLES
Abstract
Background: Gestational diabetes mellitus (GDM) is associated
with increased perinatal morbidity with overt diabetes in infants
and mothers. In infants, GDM can append an intrauterine environmental risk factor to increased genetic risk for the development of obesity and/or diabetes.
Objective: To assess knowledge among obstetricians and gynecologists regarding management of GDM, after the diagnosis
has been made.
Materials and Methods: A 25-question online survey (www.
abcofobg.com) was developed to assess providers knowledge,
practices, and attitudes related to the management of GDM.
The final sample consisted of 584 respondents. The data were
analyzed using SPSS software (version 16.0).
*Correspondence
Dr Hema Divakar
Consultant in Obstetrics & Gynecology
Divakars Speciality Hospital,
Bangalore, India
Phone: +91 9900154448
Email: hema_divakar@yahoo.com
133
Research Articles
RESEARCH
Divakar
H, et al. ARTICLES
Rising prevalence of gestational diabetes mellitus
Conclusions: This study raises the challenge of translating the evidence from well-resourced research studies into initiatives that
are affordable and feasible, whether in richer or poorer parts
of the world. Also, results of the study reveal the urgent need to
improve quality of diabetes treatment and care that is accessible and community-based with multi-disciplinary teams and
patient-centered approach.
Key words: GDM management, neonatal management, Ob-gyn
specialist, perinatal morbidity, overt diabetes
Background
Diabetes mellitus, a known chronic illness, has become
a major health problem worldwide. According to the
World Health Organization, diabetes has emerged as an
epidemic affecting 246 million people across the world.
Among those affected, almost 80% of onus exists in
developing countries.1
Although there is no evidence that perinatal mortality
is increased in pregnancies with treated gestational
diabetes (GDM), some studies have shown perinatal
mortality to be increased in untreated GDM.24 Gestational diabetes is associated with increased perinatal
morbidity with overt diabetes (macrosomia, neonatal
hypoglycemia, hyperbilirubinemia, respiratory distress
syndrome) in infants and mothers.5 In infants, GDM
can append an intrauterine environmental risk factor
to increased genetic risk for the development of obesity
and/or diabetes.68 Insulin therapy for GDM is associated with less adiposity in offsprings.9 Asian origin and
especially ethnic Indians are at a higher risk of developing GDM and subsequent type 2 diabetes mellitus
(T2DM).1012 Timely screening of all pregnant women
for glucose intolerance would help to achieve euglycemia and adequate nutrition supply would prevent the
vicious cycle of transmitting glucose intolerance from
one generation to the other.13
This study was conducted to assess knowledge among
obstetricians and gynecologists regarding management
of GDM, after the diagnosis was made.
Results
A total of 584 respondents participated in the survey.
Table 1 shows the variables related to management of
GDM.
a) Glucose monitoringmethod
and frequency in GDM patients
who were on insulin
Overall, 47.62% of doctors advised for self-monitoring
at home on a daily basis using a glucometer venous
sample PLBS on fortnightly visits and 41% resorted to
PLBS estimation at 2-week interval without daily estimates.
Research Articles
RESEARCH
ARTICLES
Divakar H, et al. Rising prevalence of gestational
diabetes
mellitus
Table 1. Awareness on Management of GDM and Before Delivery
Awareness on Management of GDM and before Delivery
All my patients on insulin are followed up by
Home self-monitoring by glucometer
PLBS at regular intervals
Other
When my patients are well controlled, I prefer to
Interfere and deliver
Wait for spontaneous labor
You chose Interfere and Deliver above. At what number of weeks would you do this?
<32
32
33
34
35
36
37
38
39
40
You choose to wait for spontaneous labor above. Until what number of weeks would you wait?
<32
32
33
34
35
36
37
38
39
40
You chose Interfere and Deliver above. What is the preferred mode of delivery?
Elective LSCS
Induction and trial of labor if no obstetric contraindication
When my patients are poorly controlled, I prefer to wait until the following number of weeks
before intervention
Elective LSCS
Induction of labor, if inducible
Morbidity of mother in GDM is more because of
Increased incidence of C-section
Increased incidence of infection
Other
Increased incidence of C-section and infection
Increased incidence of C-section and other
Increased incidence of infection and other
All
N=584
278
243
63
375
209
14
0
14
0
0
152
354
14
35
0
0
0
0
0
42
28
167
28
320
0
138
446
47.62
41.67
10.71
64.29
35.71
2.4
0
2.4
0
0
26.1
60.7
2.4
6
0
0
0
0
0
7.21
4.8
28.6
4.8
54.8
0
23.7
76.3
292
292
279
230
91
112
35
35
35
50
50
47.7
39.4
15.5
19.1
5.95
5.95
5.95
Research Articles
RESEARCH
Divakar
H, et al. ARTICLES
Rising prevalence of gestational diabetes mellitus
segment caesarean section (LSCS) (50%) or by induction of labor (50%). Most of the doctors agreed that the
morbidity of the mother in GDM was due to increased
incidence of C-section (47.7%) and increased incidence
of infection (39.4%).
c) Awareness on neonatal
management
Table 2 shows the variables related to neonatal management.
Neonatal problems
Overall, 85.71%, 84.52%, and 79.76% of doctors
agreed that neonates of mothers with GDM would have
macrosomia, hypoglycemia, and RDS, respectively.
Transfer to NICU
Among the neonates born to mothers with GDM,
57.14% of doctors transferred 1 of 10 infants to NICU
and 33.33% of doctors sent 5 of 10 infants to NICU.
Of the 584 respondents, 70.24% were aware of longterm complications of childhood obesity and adult
onset diabetes.
d) Maternal follow-up
Many doctors (92.68%) advised for 6 weeks of postpartum follow-up and 94.05% of doctors advised for
sugar testing on that visit. Most of the doctors (55.95%)
performed a random blood sugar and only a few
(17.86%) requested for 75 g glucose challenge test in the
postpartum period. Only 70% of doctors spoke to the
follow-up patients about lifestyle change and diet advice
and the need for annual checkup (Figure 1). Majority of
the doctors (92.86%) knew that T2DM was the longterm complication in GDM mothers and 67.86% were
aware of the possibility of GDM in next pregnancy.
e) Multispeciality team
As shown in Figure 2, to an Ob-gyn specialist, facilities
available as a multispeciality team were ultrasonologist
Discussion
This study aimed to assess knowledge of doctors about
management of GDM and follow-up after delivery and
awareness about long-term complications.
Research Articles
RESEARCH
ARTICLES
Divakar H, et al. Rising prevalence of gestational
diabetes
mellitus
Table 2. Awareness About Neonatal and Postpartum Management
Awareness after the Delivery
N=584
Neonates out of GDM have higher incidence
of
Macrosomia
501
Hypoglycemia
494
Hyperglycemia
49
RDS
466
FGR
111
IVH
111
NEC
153
Anemia
28
Hyperbilirubinemia
320
35
Other
In your practice, the average weight of
85.71
84.52
8.33
79.76
19.05
19.05
26.19
4.76
54.76
5.95
21.43
0
7.14
1.19
1.19
30.95
0
2.38
7.14
1.19
19.05
4.76
57.14
33.33
9.52
92.86
2.38
0
%
549
94.05
417
71.43
431
73.81
466
79.76
56
9.52
63
10.71
327
55.95
42
7.14
104
17.86
90
15.48
1.19
396
67.86
542
92.86
63
10.71
14
2.38
63
10.71
410
70.24
417
71.43
146
25
69
11.9
1.19
%
355
60.71
369
63.1
556
95.24
361
61.9
473
80.95
4.76
Research Articles
RESEARCH
Divakar
H, et al. ARTICLES
Rising prevalence of gestational diabetes mellitus
seling and insist on strict monitoring of glucose at home
on a daily basis to regulate the insulin dosage as per the
sugar values, rather than adjusting the doses based on
fortnightly estimates. This is too infrequent for a proper
control.
c) Awareness on neonatal
management
The neonates of mothers with GDM are at risk of all
the complications of infants born to mothers with overt
diabetes, particularly those infants born macrosomic
(birth weight >4 kg).16 Overall, 85.71%, 84.52%, and
d) Postpartum follow-up
Gestational diabetic women require careful follow-up.
An oral glucose tolerance test with 75 g of glucose, using
WHO criteria should be performed at 68 weeks postpartum.18 Although many doctors (92.68%) advised
for 6 weeks of postpartum follow-up and 94.05% of
doctors advised for sugar testing, only 20% asked for
the WHO glucose challenge test and only 70% gave
some advice on lifestyle change and diet (Figure 2).
This is in spite of 92.86% being aware that T2DM
was the long-term complication in GDM mother
and 67.86% being aware that she could be vulnerable to GDM in next pregnancy. Indian women with
GDM have a high risk of developing diabetes (especially T2DM), and metabolic syndrome at a comparatively young age.18,19 They should be made aware of
the symptoms of hyperglycemia and advice should be
given about the importance of healthy eating and exercise patterns. Contraceptive advice and counseling
Research Articles
RESEARCH
ARTICLES
Divakar H, et al. Rising prevalence of gestational
diabetes
mellitus
regarding planning future pregnancies should be given.
Preventive measures against T2DM should start during
intrauterine period and continue throughout life from
early childhood.20 Being strict with counseling at the
postpartum visit with lifestyle modifications would go a
long way in containing the long-term problems, both to
the mother and the child.
3.
4.
5.
Conclusion
6.
It is now recognized that it is the low- and middleincome countries that face the greatest burden of
diabetes. However, many governments and public
health planners still remain largely unaware of the
current magnitude, or, more importantly, the future
potential for increases in diabetes and its serious
complications in their own countries. This study raises
the challenge of translating the evidence from wellresourced research studies into initiatives that are
affordable and feasible, whether in richer or poorer
parts of the world.
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
18.
19.
20.
Acknowledgments
We extend our sincere thanks to Ravishankar and Dr
Peter Pothula, BioQuest for their valuable IT and editorial guidance in the generation of this report.
References
1.
2.
Research Articles
RESEARCH ARTICLES
Homozygosity Mapping of a
Consanguineous South Indian
Keratoconus Family to the
Cytogenetic Region 14q11.2
Anandula Venkata Ramana, Rohit Shetty, Govindasamy
Kumaramanickvel, Ramesh N*
Abstract
Purpose: The objective of this study was to map the gene for
autosomal recessive keratoconus (KC). KC is a corneal thinning
condition that is frequently seen in ophthalmic practice. With
a strong genetic inheritance, KC is a steadily progressive, bilaterally nonsymmetrical, noninflammatory disease. It is an ecstatic
disease, where the paraxial stromal thinning and weakening
causes distortion, leading to conical shape of the cornea. The
disease might start with astigmatism and myopia. KC is a challenging problem and at times, frustrating to treat because of the
progressive nature of the disease.
Methods: In a consanguineous south Indian family, three
affected with KC, were subjected to complete ophthalmic
examination that included corneal topography and collection
of peripheral blood for gene mapping. The DNA was analyzed
using Affymetrix GeneChip SNP 6.0 and genome wide homozygosity mapping analysis.
*Correspondence
Dr Ramesh N
Department of Biotechnology,
JJ College of Arts and Science,
Pudukottai - 622422,
Tamil Nadu, India.
Phone: 04322-260103
Fax: 04322-260103
Email: ramanagene@gmail.com
Research Articles
RESEARCH
ARTICLES
Ramana AV, et al. Homozygosity mapping to the
cytogenic region
14q11.2
Introduction
Methodology
Clinical
Pedigree
Detailed pedigree was taken from the proband, and
peripheral blood was collected from the affected as well
as unaffected members (Figure 1).
Figure 1. Pedigree of a four generation family associated with KC showing autosomal recessive inheritance. The striated symbol indicates members. The
open symbol indicates unaffected members.
Laboratory
Ten milliliters of heparinized blood was drawn from
affected patients and their parents after obtaining
written informed consent from both parents. DNA
was extracted using Machery & Nagel (M&N) Kit
according to the manufacturers instructions. High
throughput genotyping was done using Affymetrix
Gene Chip SNP 6.0. The protocol involves around 250
ng of total genomic DNA incubated with 10 units of
restriction endonuclease. The digested DNA is then
incubated with 0.25 M adaptor and DNA ligase. All
fragments resulting from restriction enzyme digestion, regardless of size, are substrates for adaptor ligation. A generic primer, which recognizes the adaptor
Research Articles
RESEARCH
ARTICLES
Ramana
AV, et al.
Homozygosity mapping to the cytogenic region 14q11.2
Table 1. Protocol for PCR Mix Concentration
Reagents
PCR buffer (10X)
dNTP (2.5 mM each)
MgCl2 (25 mM)
PCR Primer Xba (10 M)
AmpliTaq Gold (5 U/L)
H2O
Volume (L)
10
10
10
7.5
2
50.5
Concentration
250 M
2.5 mM
0.75 M
0.1 U/L
Results
The regions of significant homozygosity in this family
were on chromosomes 12p13.1 and 14q11.2 and
some of the SNPs in this region were rs1544671 and
rs3811259, respectively. The regions of homozygosity in
chromosome 14q11.2 showed a better association than
the regions of homozygosity in chromosome 12 p13.1
(Figure 3).
The likelihood of the KC gene in this south Indian
consanguineous family to be in chromosome 14 is quite
high and is strongly associated with the SNP marker
rs3811259 at the cytogenetic region 14q11.2. Further
study is necessary to identify the disease-causing gene.
10
11
12
13
14
15
16
17 18 19 20 21 22
1.0 max
0.9 max
0.8 max
0.7 max
0.6 max
Research Articles
RESEARCH
ARTICLES
Ramana AV, et al. Homozygosity mapping to the
cytogenic region
14q11.2
Discussion
6.
Despite knowing KC as a clinical entity for a considerable period of time in medical history, identification
of a major causative gene has been elusive for a while.
Genes such as VSX1, SOD1, COL4A3, and COL4A4
have been proposed as candidates based on the identification of disease-causing mutations to explain differential gene expression.14
7.
8.
9.
10.
11.
12.
13.
14.
15.
16.
17.
References
1.
2.
3.
18.
4.
19.
5.
Review
RESEARCH
Article
ARTICLES
*Correspondence
Dr Koravangattu Sankaran
Divisions of Neonatology and
Pediatric Infectious Diseases,
Department of Pediatrics,
University of Saskatchewan, Saskatoon,
Saskatchewan, Canada.
144
Review Article
ARTICLES
Sankaran K.RESEARCH
Neonatal CPAP
ventilation
There are several ways of generating CPAP. These
CPAP systems include a conventional ventilator, an
underwater bubbling system, the infant flow driver,
and Benvenistes device. They work by supplying a
flow of gas that is used to generate the needed pressures. The actual amount of gas that reaches the alveoli
is dependent on the amount of leak in the system.
These leaks could be through the nose and around
the delivery device or through the infants mouth. The
amount of leakage can be quite significant and has been
indirectly measured by assessing esophageal or pharyngeal pressure while on CPAP. The optimal amount
of flow required remains unknown and ultimately
depends on the condition that is being treated, the size
of the infant, and the amount of leak in the system as
described above.
A CPAP system usually consists of a gas flow source,
oxygen blender, humidifier, an inspiratory and expiratory circuit, and a manometer. This system is then
attached to the delivery device of choice. The various
systems differ mainly in the expiratory circuits.
Benvenistes valve
Benvenistes valve, when applied to the nasal delivery
device of choice, vents expired air to the surrounding
atmosphere. This device eliminates the need for expiratory tubing, generates more constant pressure, and
may reduce over-distension of the lungs and consequent pneumothoraces. There have been no studies
comparing this device to other available CPAP systems.
Review Article
RESEARCH
ARTICLES
Sankaran
K. Neonatal
CPAP ventilation
The breathing pattern of the neonate with respiratory
distress is quite disorganized. Some of the observed
patterns in them include increased respiratory rate,
active contraction of the inspiratory muscles during
expiration, active expiratory laryngeal narrowing and
tonic activity of the diaphragm, and intercostal muscles.
Premature infants have compliant chest walls causing
paradoxical breathing where the ribcage collapses
inward with the contraction of the diaphragm.
CPAP improves the breathing pattern in neonates
by increasing functional residual capacity and tidal
volume, thereby decreasing burden of breathing. It
also decreases chest wall compliance which is of added
importance in premature infants. CPAP increases the
mean airway pressure which decreases ventilation
perfusion mismatch. CPAP stents the upper airway,
decreases proximal airway resistance, reduces the physiologic dead space, improves synchrony of thoracoabdominal motion, and improves diaphragmatic function.
CPAP is therefore effective in the treatment of obstructive/mixed apnea of prematurity, respiratory distress
syndrome (RDS), respiratory distress of other etiology,
atelectasis, tracheomalacia, and other types of upper
airway obstruction, thus ensuring successful extubation.
In neonatal intensive care units across the world, CPAP
is being used more commonly as an alternative to intubation and intermittent positive pressure ventilation. By
using CPAP in the spontaneously breathing infant, the
adverse effect of intubation and mechanical ventilation
can be avoided. Intubation is associated with untoward
physiological responses to laryngoscopy such as desaturation, bradycardia, and hypotension with the added
risk of cerebral hemorrhage and neuronal injury in
premature infants. Mechanical ventilation predisposes
to atelectrauma, barotrauma, volutrauma, pneumothorax, inadvertent hyperventilation, and long-term side
effects such as stricture and subglottic stenosis. Nosocomial infections may be minimized by avoiding intubation. CPAP is easy to use, inexpensive and studies have
suggested decreased incidence of chronic lung disease
and mortality.
it has demonstrated effectiveness in resuscitation, avoidance of intubation, and facilitating weaning from the
ventilator. It is particularly attractive in the setting of
developing countries due to its ease of use and low cost.
For infants that are unable to tolerate CPAP alone due
to surfactant deficiency, in and out intubation for the
purpose of surfactant administration and extubation to
CPAP has been proposed and continues to be studied.
The downside to this practice is that intubation and its
untoward effects are inevitable. However, the effects
of short- and long-term ventilation may be avoided.
The use of nebulized surfactant has been suggested
in the literature but is yet to be studied. With more
infants receiving antenatal steroids, and there being
an improvement in antenatal and perinatal care, more
infants are likely able to tolerate CPAP alone. This idea
of CPAP only in premature infants is attractive because
intubation and surfactant administration are traumatic
and potentially destabilizing for them.
Review Article
ARTICLES
Sankaran K.RESEARCH
Neonatal CPAP
ventilation
central, obstructive, and mixed apnea. Central apnea is
still being treated with methylxanthines. Nasal CPAP
continue to be useful in the treatment of obstructive or mixed apnea. In mild cases, stimulation by the
bedside nurse persists. Alternative, noninvasive modes
of ventilation available for treatment of AOP are nasal
intermittent positive pressure ventilation (NIPPV) and
high-flow nasal cannula (12 L/min).
Studies show NIPPV to be as effective, or more so
than NCPAP. NIPPV is a technique of noninvasive
ventilations, in which a higher level of CPAP is delivered at intervals to simulate a ventilator breath. It is
used in conjunction with CPAP to augment its effect.
Two Cochrane reviews show that NIPPV is useful
in preventing failure of extubation and troublesome
apneas. Case reports of gastrointestinal perforation have
limited its widespread use. Reviewers, however, noted
that there have been no reports of this adverse effect
when the simulated breaths or backup rate is synchronized with the patients breaths.
The exact mechanism of action of NIPPV in improving
apnea of prematurity is yet to be elucidated. NIPPV is
not delivered directly into the lungs as in the case of
the intubated infant. In the scenario where the pressure
delivered is not synchronized, it may be delivered at any
point in the respiratory cycle. It is possible that NIPPV
works not merely as a ventilator breath but by altering
the respiratory mechanics and providing its benefit in
this way. More studies are needed to further define its
mode of action.
Limitations of CPAP
Use of CPAP does not come without its problems.
There is risk of pulmonary air leak, obstruction of the
nasal device by secretions, nasal irritation or injury,
and gastric distension. Over-distension of the lungs
can result in increased work of breathing, hypercapnia,
and decreased pulmonary vascular resistance resulting
in decreased cardiac output. CPAP therapy may simply
not work. CPAP is said to have failed when there is
unrelenting apnea, PaCO2>60 mmHg, pH<7.25, and
FiO2>0.6. These numbers are arbitrary and based
on clinical experience. It is important to consider the
Contraindications to CPAP
The following contraindications are observed:
Ventilatory failure
Cardiovascular instability
Upper airway abnormalities (such as choanal
atresia, tracheo-esophageal fistula, diaphragmatic
hernia, and absent nose)
Unrelenting apneas
Review Article
RESEARCH
ARTICLES
Sankaran
K. Neonatal
CPAP ventilation
and oxygen requirements. If the infant has deterioration in clinical status, a blood gas test and chest
x-ray may be indicated. It is important to ensure
that the equipment is working appropriately and
the airway or nasal delivery device have not been
plugged by secretions prior to performing the above
investigations.
There is no available evidence to guide weaning or
discontinuation of CPAP. A trial of discontinuation
of CPAP can be attempted if the oxygen requirements substantially reduce or dyspnea resolves.
Different techniques have been used to get the
infant off CPAP. The CPAP may simply be stopped
and the child observed for deterioration; or low/
high flow oxygen by nasal cannula may be tried as
a more gradual, gentler weaning mode.
Recurrence of dyspnea, troublesome apneas, and
increasing oxygen requirement indicate the need to
recommence CPAP.
An infant that fails CPAP using the parameters
described above (see limitations of CPAP section) is
a candidate for intubation and ventilation.
In summary, CPAP is an effective and gentler mode
of ventilation. It is gaining widespread use in neonatal
intensive care units around the world. Due to its ease
Fetal Medicine
Update
RESEARCH
ARTICLES
From the
Bangalore Fetal Medicine Centre
Abstract
*Correspondence
Dr Radhakrishnan P
Bangalore Fetal Medicine Center,
Richmond Road, Bangalore, India.
Email: drprathima@bangalorefetalmedicine.
com
149
Study Design
All the fetuses that presented or were diagnosed to
have CCAM at Bangalore Fetal Medicine Centre from
2005 to 2010 were included and postnatal follow-up
was performed. This included 20 fetuses from 16 to 24
weeks of gestation.
Results
We had a total number of 20 fetuses presented with
CCAM diagnosed in the second trimester of pregnancy. One among them was a bilateral CCAM, and
the rest 19 fetuses were unilateral. Of the 19 fetuses,
eleven of the cases were right-sided CCAM and 8 were
left-sided CCAM. Eleven fetuses had mixed type of
CCAM, 5 were microcystic, and 4 were macrocystic.
Antenatal and postnatal follow-up was available in 19
out of the 20 fetuses. In one of the fetuses, in which
postnatal outcome was not available, the CCAM had
resolved on ultrasound scan examination at 36 weeks
with no residual changes. However, for the purpose
a
Figure 1. Cross section of fetal thorax showing hyperechogenic area in the left lung with medistinal shift to the
right (a) (highlighted in (b)).
20
Live births
15
4/19 (21.1%)
Fetal hydrops
Terminations
Polyhydramnios
Associated sacral agenesis
Parents opted to terminate
6/15 (40%)
Antenatal resolution
Prematurity-related postnatal
death2
Postnatal resolution
9/15 (60%)
Conclusions
Our results suggest that spontaneous antenatal regression of the CCAM is common and has overall an excela
Although a normal postnatal chest x-ray does not indicate complete regression of CCAM, all the infants who
did not have chest x-ray have remained asymptomatic.
Asymptomatic infants with CCAM may be followed
up nonoperatively with no apparent adverse effects.
The decision and timing of an excision in an asymptomatic patient remains controversial among pediatric
surgeons. A CT scan is much more expensive than a
chest xray, and therefore this was a limiting factor for
not performing a CT scan in all the infants, especially
when they remain asymptomatic.
Discussion
CCAM has first been described by Chin and Tang in
1949. It is a rare hamartoma of the lungs that occurs
from unsystematic spread of tubular bronchioles and
enlarged alveolar tissue. This malformation can spontaneously regress, increase in size, or cause nonimmune
hydrops fetalis. Therefore, the differential diagnosis
should be made when echogenic lesions are seen in
the fetal lung at the prenatal period. About 90% to
95% of CCAMs are unilateral. When bilateral, diagb
Figure 2. Sagittal section of the fetal thorax showing CCAM (a). Color Doppler on the same shows no feeding
vessel from the aorta, which excludes pulmonary sequestration (b).
Summary of Antenatally
Diagnosed CCAM
USS appearances are typicalhyper/cystic
areas in the entire lung or part of the lung with
no feeding vessel from the aorta
About 10% would develop nonimmune
hydrops and then the prognosis is guarded;
hence serial follow-up scans are essential
40% resolve antenatally; the rest nonhydropic
show postnatal resolution
Fetal intervention in macrocystic CCAM
shows improvement in outcome
Postnatal CT scan is more specific in assessing
any residual abnormal lung tissue
If symptomatic, then surgical excision of
abnormal lung tissue may be necessary
In general, parents can be counselled that
prognosis for antenatally diagnosed CCAM is
good and in the ones which develop hydrops,
the mortality will be high. Hence, termination
of pregnancy is not recommended.
nosis of CHAOS (congenital high airway obstruction
syndrome) should be borne in mind. When CCAM
is right sided, diaphragmatic hernia of right side
containing the liver should be considered as a differential diagnosis. This must be confirmed on fetal MRI
(magnetic resonance imaging) as prognosis for the two
conditions is very different. In the absence of hydrops,
CCAM has more than 95% of survival rate. CCAM
with hydrops along with expectant management has a
mortality rate of more than 95%. Macrocystic CCAM
shunting has 65% of survival rate.
Antenatal resolution of CCAM is seen in about 50%
of the cases. Neonatal chest x-ray is reported normal in
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
RESEARCH
Quiz
ARTICLES
Picture Quiz
Can you make the correct diagnosis?
Figure 1
Figure 2
Figure 3
Figure 4
153
Quiz
RESEARCH
Picture
Quiz
ARTICLES
Quiz
RESEARCH
ARTICLES
Self-assessment
Quiz
Self-assessment Quiz
Which of the following statements are true?
1. In females, the urogenital sinus gives
rise to
a. Urethra
b. Bartholins glands
c. Cervix
d. Trigone of the bladder
e. Gartners duct
2. Breech presentation is associated with
a. Prematurity
b. Polyhydramnios
c. Oligohydramnios
d. Hydrocephaly
e. Anencephaly
3. In a pregnant, insulin-requiring diabetic,
the following changes are irreversible
a. Worsening of retinopathy
b. Hypertension
c. Worsening of renal disease
d. Increase in insulin requirements
e. Atherosclerosis in uterine spiral arteries
4. In a pregnant patient with diabetes
mellitus
a. Glycosuria is a reliable sign of poor control
b. The blood glucose should be maintained at
approximately 9 mmol/L
Fundal fibroid
Wrong dates
Placental abruption
Cephalopelvic disproportion
Occipitoposterior position
Pre-eclamptic toxemia
Placental abruption
Amniotic fluid embolism
Hydatidiform mole
Intrauterine fetal death
Quiz
RESEARCH ARTICLES
Self-assessment
Quiz
A grand multipara is in her sixth or subsequent pregnancy. Iron deficiency anemia is common and varicose
veins of the legs tend to worsen with each pregnancy.
The lower ratio of uterine muscle to fibrous tissue
makes unstable lie, malpresentation, malposition, cord
prolapse, uterine rupture, and operative delivery more
likely.
Question 3: A
Although all of these changes take place to a greater or
lesser extent in pregnancy, they are all reversible with
the exception of diabetic proliferative retinopathy. It is
for this reason that known diabetics must be investigated for retinopathy and treated if it is found early in
the pregnancy.
Question 4: C
Because of the increased glomerular filtration rate,
glycosuria is common in pregnancy and is an unreliable measure of blood glucose concentration. Blood
sugar should ideally be kept below 7 mmol/L. Transient
hypoglycemia has little effect on pregnancy. Management is at home; when patients are admitted they
become more difficult to control because of the alteration in their activity levels. The objective is to avoid
iatrogenic prematurity and with good control, delivery
should be after 38 weeks.
Question 6: B, D, E
In a primigravida patient, the fetal head engages from
36 weeks of gestation. In multiparous patients the head
may not engage until labor is commenced. A high head
at term may be found because of wrong dates or for fetal
or maternal reasons. The fetus may be too big (hydrocephaly or cephalopelvic disproportion) or present with
a large diameter (occipitoposterior position). There may
be something else in the pelvis (the placenta, fibroids
in the lower part of the uterus, ovarian masses, full
bladder, and full rectum).
Abstracts from
RESEARCH
ARTICLES
Literature
Neonatology. 2011;99:38-44.
In this prospective study, authors determined the potential relationship of attainment of full gavage feedings
and feeding volume with clinical measures and noninvasive GI tests. In 50 prospectively followed neonates,
authors found the number of GRV >2 mL/kg tended to
decrease with postnatal age (P = .06). Lactase activity
and feeding volume in milliliters per kilogram per
day prior to achieving full feedings were correlated
(P=.007, L = 0.164). There was no correlation between
feeding outcomes and GRV (mL/day), GRV>50%,
GRV>2 mL/kg, small bowel, colonic, or whole bowel
permeability, fecal calprotectin concentration, gastric
emptying, or abdominal distention.
In this prospective case-control study, authors investigated the utility of plasma lactate dehydrogenase
(LDH), alanine aminotransferase (ALT), and aspartate aminotransferase (AST) during the first 12 hours
after birth in predicting hypoxic ischemic encephalopathy (HIE) and adverse neurodevelopment outcome in
newborn term infants with intrapartum signs of fetal
distress. A total of 123 neonates with perinatal asphyxia
including 41 neonates with different degree of HIE
and 123 term control were included in the study. Using
ROC curves, a cut-off level of 1049 U/L for LDH was
the best predictor of HIE (sensitivity 100% and specificity 97%) and for long-term outcome after HIE. If
further studies confirm this finding, this may prove to
be an important tool for diagnostic confirmation and
prognostication of neonates with perinatal asphyxia.
Authors compared transfusion requirements and erythropoietic response in preterms between schedules of
rEPO administration once or three times per week,
using the same weekly dose. A total of 119 preterm
VLBW infants were enrolled in the trial. Infants receive
either subcutaneous rEPO at 250 units/kg per dose,
three times weekly for 6 weeks (Group 1 = 60, n) or
750 units/kg per dose, once weekly for 6 weeks (Group
2, n = 59). Data revealed, incidence of blood transfusion were similar between the two groups; however,
hemoglobin levels were lower at end of study in Group
2 (10.6 1.5 g/dL vs 11.5 1.4 g/dL; P<.003). Finally,
at end of study, reticulocyte counts and serum transferring receptor values increased and serum ferritin values
decreased, without significant differences between the
two groups. Incidence of complications was similar in
both groups. Thus, the once-weekly rEPO schedule for
very low birth weight infants proved as effective as the
three times weekly schedule, in relation to erythropoietic stimulus and transfusion requirement.
157
RESEARCH ARTICLES
In 89 prospectively enrolled infants with respiratory distress [gestational age (GA) 28.1 2.2 weeks]
compared to historical control group, authors evaluated the efficacy of sustained lung inflation (SLI, 25
cm H2O, sustained for 15 s) in improving the respiratory outcome. Data suggest, the SLI group had less
need for (51% vs 76%, P<.0001) and shorter duration of mechanical ventilation (5 11 vs 11 19
days, P = .008), a more frequent occurrence of exclusive nasal continuous airway pressure support (49 vs
24%, P<.0001) and INtubation-SURfactant-Extubation (INSURE) treatment (16% vs 3%, P = .01), less
need for surfactant (45% vs 61%, P = .027) and postnatal steroids (10% vs 25%, P = .01), a shorter duration
of oxygen therapy (21 27 vs 31 31 days, P = .016),
and finally, a lower occurrence of bronchopulmonary
dysplasia in survivors (7% vs 25%, P = .004). Authors
concluded that the application of a sustained lung inflation at birth in preterm infants with respiratory distress
may decrease the need for mechanical ventilation
without inducing evident adverse effects.
Short
RESEARCH ARTICLES
Communications
*Correspondence
Dr Shubha R Phadke,
Professor, Department of Medical Genetics,
Sanjay Gandhi Postgraduate Institute of
Medical Sciences,
Lucknow - 226013, India
Phone: 0522-668004, 668700, 668800, 668900
Extension no. 4325
Email: Subha@sgpgi.ac.in
shubharaphadke@gmail.com
Abstract
Apert syndrome is an autosomal dominant disorder characterized by craniosynostosis, distinctive dysmorphic facial features, limb abnormalities, and developmental delay, resulting
from a dominant mutation in FGFR2 gene. This study reports a
case of Apert syndrome diagnosed prenatally based on the
results of antenatal ultrasonography at 26 weeks of gestation
and molecular testing.
Key words: Apert syndrome, prenatal diagnosis, FGFR2 gene
Introduction
Case Report
159
Short Communications
RESEARCH
Phadke
SR, et al.ARTICLES
Prenatal diagnosis of Apert syndrome
highly placed third ventricle, suggestive of agenesis of
the corpus callosum. In addition, a long and prominent
forehead; a flat facial profile; appearance of mittenhand, suggesting syndactyly of the hands, disorganized
phalanges; and bilateral short halluces were noted in the
fetal ultrasonogram (Figure 1: a, b, c).
b
e
Figure 1. a, b, c: Prenatal ultrasonogram showing the flat face, prominent and long forehead, mitten hands,
syndactyly of the toes, and short and broad great toes.
d, e, f, g: Postmortem examination findings in the fetus: Acrocephaly, flat facial profile, ocular hypertelorism, low
set ears, anteverted nares, and syndactyly of the hands and feet.
Short Communications
RESEARCH
ARTICLES
Phadke SR, et al. Prenatal diagnosis
of Apert
syndrome
and low set ears. Oral cavity examination showed a high
arched palate with a median groove and absent uvula.
There was complete syndactyly of the second, third,
and fourth fingers and partial syndactyly of the fourth
and fifth fingers of both the hands. The halluces were
bilateral, short, and broad, and in both feet there was
syndactyly of the second, third, and fourth toes (Figure
1: d, e, f, and g). The external genitalia were of a normal
male infant. The heart, lungs, gastrointestinal tract,
and genitourinary organs appeared normal. Skeletal
survey revealed bony disorganization of the hands and
feet with syndactyly. A postnatal computerized tomography (CT) scan with three-dimensional reconstruction showed the characteristic facial profile, partially
closed coronal sutures, a wide open sagittal suture, bony
syndactyly, and duplicated hallux bones (Figure 2).
Discussion
Few reports of successful prenatal diagnosis of Apert
syndrome by antenatal ultrasonography have been
published.1 However, basing the diagnosis on the fetal
ultrasonogram alone can be erroneous and molecular
Figure 2. a, b, c: Postnatal CT scan showing the characteristic facial profile, partially closed coronal sutures,
wide open sagittal suture, bony syndactyly, and duplicated hallux bones.
Short Communications
RESEARCH
Phadke
SR, et al.ARTICLES
Prenatal diagnosis of Apert syndrome
testing of the FGFR2 gene should ideally be performed
to confirm the diagnosis.2,3 A sequential diagnostic
approach combining three-dimensional computed
tomography scan and molecular testing in cases wherein
fetal sonography alone is not conclusive, has also
been recommended.4 Quintero-Rivera et al reported
a newborn diagnosed to have Apert syndrome after
birth, whose only abnormality detected in a prenatal
ultrasound was agenesis of the corpus callosum, which
has been detected prenatally in the present case too.
Following the review of the central nervous system
findings of 30 patients with Apert syndrome, it was
suggested that a careful diagnose for hand malformations of Apert syndrome should be carried out in any
fetus detected with agenesis of corpus callosum or mild
ventriculomegaly in the antenatal sonogram.5
Mutation detection prenatally helps not only in confirmation of the diagnosis but also in genetic counseling.
Though most mutations in cases of Apert syndrome
arise de novo, germline mosaicism may give rise to
recurrence in the family, and thereby detection of mutation in the proband helps in early prenatal testing in the
next pregnancy. Two common mutations in the FGFR2
gene, S252W (p.Ser252Trp) and P253R (p.Pro253Arg),
account for 98% of the cases of Apert syndrome, with
the S252W mutation detected in 71% of the cases and
P253R in remaining 26%.6,7 As both these mutations
References
1.
2.
3.
4.
5.
6.
7.
Short Communications
RESEARCH ARTICLES
Abstract
*Correspondence
Dr Somashekhar AR
Associate Professor, Pediatrics,
Department of Pediatrics,
MS Ramaiah Medical College,
Bangalore-560054. India
This study reports a rare case of congenital sternal cleft in association with pectus excavatum. In neonates with cleft sternum, the
sternal bars can be easily approximated by simple sutures due
to flexibility of cartilagenous thorax. If reconstruction is delayed,
increased rigidity of the chest wall and physiologic accommodation of the thoracic organs to the circumference of the chest
render simple approximation impossible, without serious compromise of the heart and lungs.
Key words: Pectus excavatum, sternum/abnormalities, sternum/
surgery
Introduction
Sternal cleft is a very rare midline defect. Incomplete
sternal clefts are more common than the complete ones.
In this challenging anomaly, the underlying mediastinal structures (particularly heart and great vessels)
can be easily injured by external trauma. In addition,
the deformity is cosmetically unpleasant and quite
alarming to young patients and their families.
Although the embryology of the thoracic wall is well
known, the etiology of cleft sternum remains unclear.
Failure of fusion of the lateral sternal bands by an early
disturbance affecting the midline mesodermal structures between the sixth and ninth gestational week is
a possible outcome. However, no familial, teratogenic,
or nutritional factors have been identified as a potential
cause. Cleft sternum is categorized into complete and
incomplete forms depending on the degree of separation
of the lateral sternal bands. Its association with craniofacial hemangiomas and omphalocele is common.
Cleft sternum could be prenatally diagnosed by ultrasonography. During the neonatal period, surgical
correction should be performed where the direct
suturing of the sternal halves is possible and the thorax
can accommodate the thoracic viscera. At an older
age, surgical repair is feasible, but it may require additional measures, such as sliding chondrotomies of the
adjacent costal cartilages and notching of the sternal
bars, to facilitate the approximation. Lung herniation
at the base of the neck should be corrected by uniting
the cervical muscles. The first postoperative day is most
critical owing to acute reduction of the mediastinal
space. Long-term results are satisfactory.
Case Report
A term female neonate, born through normal vaginal
delivery to a 32-year-old gravida 3, para 2 mother, was
noted to have a chest wall abnormality at birth. The
neonate was therefore admitted to neonatal intensive
Short Communications
RESEARCH AR
ARTICLES
Somashekhar
, et al. Conginital sternal cleft with pectus excavatum
care unit. There were no prenatal complications and
the family history was unremarkable. Second trimester
sonographic findings were reportedly normal. The other
siblings of the neonate were normal and the parents had
a nonconsanguineous marriage. There was no history
of drug intake during pregnancy, which would have
an adverse effect on the fetus. The neonate was wellnourished and active with birth weight of 3 kg; head
circumference 34 cm; and length 48 cm. There were no
reports of cyanosis, pallor, or edema.
A defect in the anterior chest wall, extending from the
suprasternal notch to about 1 cm below the xiphoid
process was noted, associated with paradoxical movement of the chest wall during respiration. A blackish
raphe, extending from xiphoid to umbilicus, was
present. A transverse ridge of scar tissue in the region of
hyoid bone was observed and the strap muscles appeared
to be adherent to it. A distinct bulge of the heart was
noted while the baby was crying. Apex beat was localized to the left 4th intercostal space, 1/2 inch medial to
the midclavicular line. Respiratory system was normal
and so was the abdominal examination. X-ray of the
chest showed sternal cleft. A surgical correction for the
sternal cleft was advised and performed after thorough
investigations to rule out other conditions that could
affect the prognosis, post operation.
Discussion
Conclusion
Sternal clefts can be classified into 3 categoriessuperior, inferior, and complete. The superior cleft defect,
which is the most common variant encountered, is
almost always an isolated abnormality but can occasionally be associated with cervicofacial hemangiomas.
The defect is either a U-shaped defect with the cleft
ending at the level of the fourth costal cartilage, or a
V-shaped defect with the sternum cleft at the xiphoid
process. Although the embryologic development of the
sternum has been studied in detail, the etiology of cleft
sternum is unknown.
The superior cleft is the most common variant. Inferior clefts, conversely, are almost always associated with
severe developmental defects, especially ectopia cordis
and pentalogy of Cantrell. The complete form of this
anomaly is the rarest type and usually associated with
the most severe anomalies such as double central incisors, congenital heart defect, neck webbing, bicornuate uterus, and minor anomalies including long face
with hypotelorism and craniofacial hemangiomas. In
neonates, approximation of sternal bars with simple
sutures without the complications of cardiac compression is the preferred procedure.
Some hypotheses include chronic nutritional deficiency, lack of riboflavin during pregnancy, and disruption of the HoxB4 gene.1 Most clefts occur sporadically,
although autosomal recessive familial association has
been reported. Some series have found a skewed sex
References
1.
Short Communications
RESEARCH ARTICLES
Congenital ChylothoraxAn
Evidence-based Approach
Ramesh T,* Vishwanath J, Pejaver RK
Abstract
*Correspondence
Dr Ramesh T
#40C, Roja Illam,
Near Angaleeswari Amman Temple,
Ashok Nagar Extn., Moolapalayam,
Erode, Tamil Nadu, India.
Email: drtr_ero@yahoo.co.in
Points to remember
Case report
A 23-year-old primigravida, who is Rh positive without
any medical illness like hypertension, diabetes mellitus,
heart disease was referred to our hospital in the 3rd
trimester of pregnancy for evaluation of polyhydramnios and isolated left-sided pleural effusion of the fetus
which was detected by routine antenatal ultrasonogram
(USG).
Maternal serology, hepatitis B surface antigen (HBSAg),
Venereal Disease Research Laboratory (VDRL) were
found negative. As the pleural effusion was progressive, elective lower segment cesarean section was done
at 35 weeks of pregnancy after giving steroids. Term
infant (girl) was born with birth weight of 2.8 kg and
Short Communications
RESEARCH
Ramesh
T, et al. ARTICLES
Congenital chylothoraxAn evidence-based approach
an Apgar test scored 4 and 6 at 1 and 5 minutes of age,
respectively. The infant needed resuscitation with bag
and mask ventilation followed by bag and tube ventilation. There were no dysmorphic features or congenital anomalies found. X-ray of the chest of the neonate
was showing gross left-sided pleural effusion with mediastinal shift toward right side. Intercostal drainage
was done and approximately 100 mL of straw colored
fluid was drained. Pleural fluid was sent for analysis.
Indwelling thoracic tube was placed for continuous
drainage of the pleural fluid.
Blood group of the infant was B positive, and peripheral smear study was found to be normal.
Discussion
Initial blood culture revealed no growth. Echocardiogram was done which revealed small PDA (Patent
Ductus Arteriosus) with L to R shunt and small PFO
(Patent Foramen Ovale) with L to R shunt. There was
no pericardial effusion. USG of abdomen and chest was
done which revealed no ascites and no effusion on the
right pleural space. TORCH screening was negative;
high-resolution computed tomography (HRCT) of the
chest was carried out to rule out other causes of isolated
pleural effusion and it was found normal. Chromosomal studies were deferred as there was no features of
dysmorphism.
Pleural fluid analysis was done on day 1 of life which
showed count of 900 cells, 100% lymphocytes, protein
2.7 g%, sugar 55 mg%, fat globules were absent and
no growth was observed on culture. Pleural fluid triglyceride (TGL) level was done on day 5 of life, which
was 489 mg% (N<100 mg%), which confirmed chylothorax. By this time, the infant was on enteral feeds in
increasing quantities and the pleural fluid had turned
milky in appearance. The infant was ventilated for 3
days and then weaned off from ventilator. During the
first week of life, the infant developed hyperkalemia
which was treated with k + free fluid and K-bind. By
the end of first week, the infant lost 400 g of weight
(approximately 20%) which was associated with severe
dehydration and electrolyte abnormalities (hyperkalemia and hyponatremia). Dehydration was corrected
with extra fluid. Twenty percent albumin infusions
were given to build up the nutrition. The infant devel-
Presence of chyle in the pleural space is called chylothorax. Incidence of congenital chylothrax is 1 in 10,000
to 15,000,2 with male female ratio of 2:1.3 It was first
described by Pisek and Linner in 1917.
Etiology
Exact etiology is not known. Congenital malformation
in the thoracic duct,4 hyperextension of spine in intrauterine life which compresses the thoracic duct are the
proposed explanations. Secondary causes include birth
trauma, cardiac surgeries, diaphragmatic repair, like
Noonans, Downs, Turners syndrome, local causes
like pulmonary lymphangiectasias, pulmonary sequestration, hamartoma, infections like viral and bacterial
pneumonias5 (CMV, Adeno, group B streptococcal). In
this case, no specific etiology could be made out.
Clinical Presentation
Usually neonate presents with birth asphyxia due to
large pleural effusion which interferes with expansion
of lung. If the effusion is large, it results in pulmonary hypoplasia and PPHN. The mediastinum will be
shifted to contralateral side and the ipsilateral lung will
have dull note on percussion and breath sounds will be
absent.
Diagnosis
Diagnosis of chylothorax is made by antenatal USG
or by postnatal x-ray chest. It is important to look for
other features of hydrops fetalis like ascite, skin edema.
Chylothorax is usually diagnosed in the presence of
Short Communications
RESEARCH ARTICLES
Ramesh T, et al. Congenital chylothoraxAn evidence-based
approach
plural effusion with triglyceride level >1.1 mmol (>100
mg%) and a cell count >1000 cells/mL with a predominance of lymphocytes6,7(approximately 80%). Presence
or absence of TGL, fat globules in pleural fluid depends
upon the feeding habit of the baby. In neonates, who is
not on enteral feeds, diagnosis of chylothorax is made
by detecting lymphocytes in the pleural fluid. In our
case, initial evaluation shown 900 cells100% cells
lymphocytes and fat globules were absent, whereas on
day 5 (after starting feeds) triglyceride levels were high
(489 mg%).
HRCT chest is the best modality to detect secondary
causes of chylothroax like pulmonary lymphengiectasias, hamartomas, and pulmonary sequestrations. In
our case, HRCT chest was normal. If there is associated ascites, work up should be done for immune and
nonimmune hydrops like karyotyping, TORCH
screening, metabolic work up to rule out storage disorders, Hb electrophoresis, echocardiogram, and ECG.
In our case, extensive work up for nonimmune hydrops
was deferred in view of isolated pleural effusion and
pleural fluid analysis had confirmed chylous nature.
However, TORCH screening, echocardiogram were
done and were normal.
Management
Hydrops fetalis
Chylothorax
Antenatal
It includes thoracocentesis or thoraco amniotic
shunting.8 Indications for thoracoamniotic shunting
include USG evidence of mediastinal shift, inversion
of diaphragm and intrathoracic pressure more than
39 mmHg. The aim is to reduce the complication of
PPHN or reduce the need for mechanical ventilation or
prevent the development of hydrops fetalis.
Postnatal
Conservative
1. It includes mechanical ventilation for respiratory
failure.
2. Continuous thoracostomy tube drainage (or)
repeated thoracentesis: lung expansion tamponading the defect and prevent further fluid
accumulation.
Octreotide infusion
*Supportive measures
Short Communications
RESEARCH
Ramesh
T, et al. ARTICLES
Congenital chylothoraxAn evidence-based approach
3. Dietary modification: Preparations containing
long chain triglycerides (LCT) are absorbed
into the lymphatic system which results in
excess chyle formation and chyle leakage.
Avoiding LCT and adding MCT in diet results
in less chyle formation and leak.9 Examples:
Pregestemil lipil, portagen, or pepti junior.
These preparations should be used at least for
2 weeks. But these preparations are not easily
available in our country. Other option is total
parenteral nutrition.
4. Somatostain analogue: octreotide infusion.
References
1.
2.
3.
4.
5.
6.
7.
8.
9.
10.
11.
12.
13.
10,11
Surgical management
It includes pleurodesis talc, fibrin glue, povidone
iodine,12 plural abrasion, and ligation of the thoracic
duct.13
Complications
Babies with chylothorax may develop dehydration
and metabolic acidosis because of excessive fluid and
sodium loss. They are prone to malnutrition, failure
to thrive, hypoalbuminemia, and infection because of
loss of lymphocytes, immunoglobulins, and albumin
in chyle. In this case, the infant had weight loss, severe
dehydration, and infection which was corrected with
20% albumin, extra fluid and Intravenous (IV) antibiotics.
Short Communications
RESEARCH ARTICLES
*Correspondence
Ahmed A,
Assistant Professor,
Department of Pediatric Surgery,
M.S. Ramaiah Medical College,
Bangalore, India.
Email: draaftab@rediffmail.com
Abstract
A 9-month-old child presented with a swelling in front of the
sternum that was seen since neonatal age. Complete excision
was done. Histopathology confirmed the diagnosis as bronchogenic cyst.
Introduction
Bronchogenic cysts are rare congenital anomalies with
prevalence ranging from 1 in 42,000 to 1 in 68,000.1
Subcutaneous bronchogenic cysts are even rarer, as
less than 70 cases have been reported.2 We report a
case of subcutaneous bronchogenic cyst in front of the
sternum.
Case report
A 9-month-old female child presented with a history of
swelling in front of the sternum which was present since
birth. This swelling had remained quiescent till 2 weeks
before the diagnosis. The 0.5-cm diameter swelling
had sudden increase in size associated with pain. The
infant was treated with antibiotics and analgesics for a
week. Meanwhile, an ultrasound scan showed a cystic
lesion measuring 1 cm in diameter. The swelling had a
Discussion
Bronchogenic cysts are formed due to abnormal development of the ventral foregut.3 During the 5th week
of gestation, the laryngotracheal groove separates the
primitive foregut into ventral and dorsal parts.4 Ventral
Short Communications
RESEARCH
Ahmed
A, et al.ARTICLES
Presternal Bronchogenic Cyst
a
Figure 1. Cyst lined by ciliated columnar epithelium with surrounding seromucinous glands: (a) high power;
(b) low power view.
component forms the lung buds and dorsal component forms the foregut by the 7th week of gestation.
Bronchogenic cysts arise from the lung buds during
this phase. Bronchogenic cysts in subcutaneous tissue
of the anterior chest wall may arise due to migration
of the intra-thoracic bronchogenic cysts or due to the
pinching off effect of the developing sternal bars
during their fusion on the developing lung parenchyma.4 Migration of the sequestered tissue can occur
as the embryo developes. Therefore, bronchogenic
cysts can be found as far as abdominal cavity or superiorly upto the chin, they may be found in the neck
(suprasternal is the most common site in the neck), the
presternal area, or the scapula region.4 But, the most
common extra pulmonary location of bronchogenic
cysts is the mediastinum.4
Bronchogenic cysts which are cutaneous are noticed in
the neonatal age or in early childhood. They are usually
asyptomatic but may develop symptoms of inflammation as in our case. Some may have a fistulous tract
draining mucoid material.4 Rarely, they may develop
into muco-epidermoid carcinoma or melanoma.4,5
Pedunculated bronchogenic cysts have been reported.
Males are four times more affected than females.
Differential diagnosis includes thyroglossal cyst,
branchial cyst, cutaneous ciliated cyst, epidermal inclusion cyst, dermoid cyst, and trichilemmal cyst.2 The
Conclusion
Bronchogenic cysts in skin are rare swellings that are
not suspected before surgery. One should consider
this condition in the differential diagnosis of swell-
Short Communications
RESEARCH
ARTICLES
Ahmed A, et al. Presternal
Bronchogenic
Cyst
ings found in neonates if present in the locations as
mentioned above. Histopathology is required for diagnosis of the condition. Complete excision is necessary to
prevent recurrence, inflammation, or chances of malignant transformation.
2.
3.
4.
References
5.
1.
Bronchopulmonary dysplasia (BPD) results from prematurity and surfactant deficiency with contributing factors such as barotrauma, volutrauma, and oxygen toxicity determined by supportive mechanical ventilation care and infection. Taken together, these factors result in chronic inflammation with
recurring cycles of lung damage and repair that impair alveolarization and vascularization in developing lungs.
Studies show that pulmonary complications continue to exist in early to late childhood. For instance, chronic
airway obstruction typically persists in former premature infants with classic BPD during early childhood and
can be associated to air trapping in infants with new BPD. The airway obstruction is typically fixed (ie, unresponsive to 2-agonists). The mechanism of development of fixed airway obstruction is most likely related to early
life structural changes in the airways. Preterm children with a history of BPD more commonly have abnormal
pulmonary function (ie, significant reduction in FVC, FEV1, and FEF25-75) along with bronchodilator responsiveness as compared to preterm children without BPD and children born term. Several studies have evaluated
the physiological response to exercise in late childhood and overall showed a reduction in performance and a
worsening in respiratory parameters in children with a history of BPD. In addition, a study showed that exerciseinduced bronchospasm occurred in 50% of the BPD group.
Very few studies have assessed pulmonary function of BPD survivors reaching the adult years. They
continue to show different degrees of pulmonary function changes, primarily due to airway obstruction. These complications include chronic pulmonary function impairment, reduced exercise capacity,
and more rapid deterioration of lung function than in normal subjects as adolescents and young
adults. In addition, the diagnosis of asthma and use of asthma inhalers is significantly more prevalent among patients born prematurely than controls. Screening by computer tomography of adult
patients (age 1733 years) showed that 84% of survivors of moderate and severe BPD had emphysema.
Follow-up studies of preterm infants with BPD show that long-term pulmonary complications resulting from
airway structural changes persist into adulthood.
The new BPD in postsurfactant era has different pathophysiological changes and different radiological and clinical presentations. These differences between classic and new BPD may also influence long-term pulmonary
outcomes, with less possible impairment in the latter case. This is an important issue for primary care physicians
who will be involved with the care of these patients.
RESEARCH ARTICLES
Upcoming Events
8th Annual Pediatric Clinical Trials Conference
Date: May 2 to 4, 2012
Neonatal Pharmacology
Dedicated to Pediatrics
Instructions to
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Authors
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Perinatology
Instructions to Authors
Perinatology, one of the few journals dedicated to the emerging
multidisciplinary field of perinatal medicine, is published quarterly.
The journal publishes original articles, brief reports on clinical and
laboratory observations, case reports of substantiate value, invited
editorials, invited papers on recent advances, clinical diagnosis,
announcements of meetings, and summary reports of conferences.
The journal is multidisciplinary and welcomes articles in English from
metabolic specialists, clinical biochemists, obstetricians, neonatologists, pathologists, geneticists, endocrinologists, neurologists, developmental pediatricians, and any other person whose work is related
to metabolic disorders and genetics.
The articles should not exceed 2500 words, with not more than six
figures, tables, and photographs. Brief reports and case reports
should not exceed 1200 words, with not more than two tables and
figures or photographs and six references. Brief accounts of new
observations can also be presented as letters not exceeding 400
words.
Guidelines to be followed while preparing and submitting a manuscript are given below.
MANUSCRIPT CHECKLIST
Typescript and other inputs
Send original (unpublished and not submitted elsewhere)
manuscript along with two photocopies to the address* given
below
Send a softcopy (CD) of the manuscript in MS Word format
Send two sets of illustrations, photographs, and drawings
Line art/graphs/illustrations should be provided in .eps
or .ai format or in a camera-ready form with redundant
areas trimmed
Line drawings can be submitted as photographic prints or
high-quality photocopies. All other illustrations should be
black-and-white photographic prints
Resolution of photographs or their scanned copies should
be 300 or more dpi
If the subjects of the photographs are identifiable, either
their eyes should be masked or their written permission to
use the photograph should be submitted along with the
typescripts
Color illustrations can be used if essential to the article
Provide complete details of the corresponding author,
including address for communication, telephone and fax
numbers, and email address
Keep a copy of the manuscript for your reference
Statements/permissions
Include statements signed by each author on:
Authorship criteria and responsibility
Financial disclosure
Copyright transfer
Include statement signed by corresponding author that written
permission has been obtained from all persons named in the
Acknowledgment
Include research or project support/funding in an acknowledgment
173
Instructions to Authors
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References
Authors cite a reference:
To support their arguments or lay the foundation for their theses
As a source of information or to credit other authors
General Style Points (US Spelling and Style)
Use American English, ize spelling
Numbers under 10 are spelled out, except for measurements
with a unit (8 mmol/L) or age (6-week old), or when in a list with
other numbers (14 dogs, 12 cats, and 9 gerbils)
Use list comma. For example: The bishops of Durham, Canterbury, Bath and Wells, and York were invited. OR I used to
help my mother with the cooking, cleaning, washing and
ironing, and yard work
Use double quotes: Generally, quotes are outside punctuation.
For example: poliomyelitis is commonly referred to as polio.
For range use to in text, for example 40 to 50 mg, and (en
dash) in parenthesis (3547 cm)
Use P value as: P = .05, P<.005, P>.05 (no 0 before period and
P is capital and italics; no space between the symbols (<,>)
and P; rule of exception: insert space before and after the
symbol =)
Closed-up em dash should be used for parenthetical
phrases
Parenthesis style: First priority is parenthesis followed by square
bracket, that is, ([]), (World Health Organization [WHO])
Date: December 5, 2008 (US style)
Foreign words listed in Merriam-Websters online dictionary
should not be italicized and hyphenated (in vitro, in vivo, etc.)
Tables
All tables should be cited in the text and it should be in
sequential order
Table should be cited as Table 1/Tables 1 and 2/Tables 1 to 3 (or
Tables 13, inside parenthesis)
Table caption should be provided and its first part should be in
bold form as given below
For example:
Table 1. Table Caption in Title Case (no full point at the end)
Figures
All figures should be cited in the text and it should be in
sequential order
Figure should be cited as Figure 1/Figures 1 and 2/Figures 1 to 3
(or Figures 13, inside parenthesis)
Figure caption should be provided and its first part should be in
bold form as given below
For example:
Figure 1. Figure caption in sentence case. (Note the full point at the
end.)
Abbreviations
Most abbreviations should be spelled out at first mention (in text);
some very common abbreviations need not be expanded. Abbreviations should not be introduced in titles.
Some notes on the use of abbreviations are given below.
In general, use uppercase letters without period for acronyms
and initialisms such as CHF, GFR, and WBC and also for routes of
administration and dosage schedules such as PO, IV, BID, and
TID. Some abbreviationssuch as PhD and pHdo contain
lowercase letters
Use abbreviations consistently. In general, write out the term or
phrase at first mention, followed by the abbreviation in parentheses; use only the abbreviation thereafter
bacilli
Staphylococcus
staphylococci
Streptococcus
streptococci
Units
Use standard abbreviations for units of measure.
References
All references should be cited in the text, tables, or figures in consecutive numerical order (as presented in the text) by means of
superscript Arabic numerals, outside punctuation.
Article in journals
Typical entry for journal with more than two authors (if more than
two authors, list first author, then et al).
For example:
Hunter DJ, et al. Plasma organochlorine levels and the risk of breast
cancer. N Engl J Med. 1997;337(suppl 1):1253-1258.
Morris LA. The risk revolution: an experimentation of the regulatory
environment [editorial]. J Am Pharm Assoc. 2001;41:S5-S10.
Books
Author name: Same as journal.
Aronoff GR, et al. Drug Prescribing in Renal Failure. Vol. 1. 4th edn.
Philadelphia, PA: American College of Physicians; 1999:39.
Dukes MNG, Aronson JK, eds. Meylers Side Effects of Drugs. 14th
edn. Amsterdam, Netherlands: Elsevier; 2000:xvi-xvii.
Other published material
Electronic media
Perneger TV, et al. Randomized trial of heroin maintenance programme for adults who fail in conventional drug treatments. TBMJT
[serial online]. 1998;317:20-24. Available at: http://www.bmj.com/
cgi/content/full/317/7150/13. Accessed March 4, 2003.
Rennie D, Guyatt G, eds. Users Guides to the Medical Literature:
A Manual for Evidence-Based Clinical Practice [book online].
Chicago, IL: AMA Press; 2002. Available at: http://www.usersguides.
org/textbooks.asp. Accessed February 26, 2004.
Unpublished material
Do not include material that has been submitted for publication but
has not yet been accepted. This material, with its date, should be
noted in the text as unpublished data, as follows:
For example:
These findings have recently been corroborated (Mariman, unpublished data, January 1996).
Printed and published by Dr Pralhad S Patki on behalf of The Himalaya Drug Company, Makali, Bangalore - 562123.
Published at The Himalaya Drug Company, Makali, Bangalore - 562123.
Printed at Sri Sudhindra Offset Process, No. 27/28, DT Street, 8th Cross, Malleshwaram, Bangalore - 560003.
Edited by Dr Ranjan K Pejaver.