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Case Reports |
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Ileal Atresia with Dextrocardia in a Case
of Poland’s Syndrome
CS Ravikiran*, Tejaswini A Kamat**, Pradnya S Bendre***,
Gaddi Diwakar*,
S Minakshi*, Alok S Godse* |
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| Poland’s syndrome is a rare constellation
of anomalies involving the chest wall with absence of pectoralis
major and minor as its main component. We recently encountered
an even rare association of ileal atresia and dextrocardia with
Poland’s syndrome which was successfully managed. We have
discussed the embryology, presentation and management with review
of pertinent literature. |
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| Introduction |
Patients of Poland’s syndrome, by definition,
have aplasia or hypoplasia of the sternocostal portion of
the pectoralis major muscle and in addition, at least one
of the other associated lesions of the hand, breast or chest
wall. We present a case of Poland’s syndrome with a
very rare association of ileal atresia and dextrocardia.
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| Discussion |
A first born, 3 days old, full term normal vaginally
delivered male child with a birth weight of 2.5 kg presented
to us with abdominal distension, bilious vomiting and failure
to pass meconium since birth. Patient was referred to us as
a case of left sided congenital diaphragmatic hernia (CDH)
in view of X-ray showing apparent loops of bowel in left thorax
with shift of mediastinum to right side. On admission, patient
was stable with no tachypnoea, cyanosis or respiratory distress.
Left chest wall was deformed and compressed with absent nipple
on the left and a super numeric nipple on the right side. Heart
sounds were best heard on the right side and air entry was
good on the left and equal to that on the right. Abdomen was
distended with palpable loops and per rectal examination revealed
only mucus. Erect X-rays after Ryle’s tube insertion
ruled out CDH and was suggestive of small bowel atresia with
left rib anomalies with dextrocardia.
Patient was resuscitated; a 2D echo confirmed dextrocardia with no intrinsic
cardiac anomalies - patient was explored, ileal atretic segment excised
and end to back ileo-ileal anastomosis completed. Post - op course was
uneventful. Patient was started on orals on POD-5 and discharged on POD-8.
Patient is at present on follow up.Poland’s syndrome is a rare constellation
of symptoms which include aplasia or hypoplasia of the sternocostal portion
of the pectoralis major and variably associated with syndactyly, hypoplasia
or aplasia of the ribs, chest wall depression, athelia or amastia, absence
of axillary hair and limited subcutaneous fat. It has a sporadic occurrence
estimated between 1 in 30,000 live births1 and is rarely familial.
The cause has been attributed to abnormal migration of the embryonic tissues
forming the pectoral muscles to hypoplasia of the subclavian artery and
in utero injuries from attempted abortions but none of these theories have
been uniformly accepted. Children demonstrate remarkable diversity of this
syndrome and the degree of abnormality of the hand, breast or chest wall
can be variable. CT scan has proven helpful in assessing the configuration
of the chest wall and its need for reconstruction. CT scan also evaluates
the extent of muscular involvement.2
All patients with absent ribs or severe ipsilateral concave deformity should
be considered candidates for repair. The vital components of the repair
include correction of the abnormal position and rotation of the sternum
as well as replacement of the aplastic ribs.3
Poland’s syndrome is associated with a second rare syndrome - Mobius
syndrome - involving bilateral or unilateral facial palsy and abducens
oculi palsy and another unusual association with childhood leukaemia has
been reported.4 However, there is no association described with either
gastrointestinal anomalies or cardiac anomalies which makes this case one
of the rarest among the rare cases. |
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Figs. 1a,b : Preoperative X-ray [AP (a) and Lateral (b)] view
showing hyperlucency and left rib anomalies, apparent mediastinal
shift to the right and dilated intestinal loops with multiple
airfluid levels |
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| References |
| 1. |
reire-Maia N, et al. The Poland
syndrome - clinical and geneological data, dermatoglyphic
analysis, and incidence. Hum Hered 1973; 23 : 97. |
| 2. |
Bainbridge LC, Wright AR, et al. Computed
tomography in the preoperative assessment of Poland’s
syndrome. Br J Plast Surg 1999; 44 : 604. |
| 3. |
Haller JA Jr, et al. Early reconstruction
of Poland’s syndrome using autologous rib grafts
combined with a latissimus muscle flap. J Pediatr Surg
1984; 19 : 423. |
| 4. |
Boaz D, Mace JW, et al. Poland’s
syndrome and leukemia. Lancet 1971; 1 : 349. |
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STATINS AND MULTIPLE SCLEROSIS
The authors conclude that these results lend support
to the case for randomised, controlled, clinical trials
to establish the safety and efficacy of statins for multiple
sclerosis. But warn that the current findings should be
interpreted cautiously in view of the small number of patients
and the study design.
Lancet, 2004; 4 : 1570, 1607.
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