57

Letter to the Editor

Fibular Hemimelia with Limb Appendage

Sir,
Absence of fibula (fibular hemimelia) is an uncommon
condition. The presentation may vary from hypoplasia to
complete absence of the fibula. We report a case of leftsided fibular hemimelia with ipsilateral shortening of
femur and antero-medial bowing of the tibia. The defect
was associated with a limb appendage arising from the
ankle joint, a finding not reported earlier.
A full-term female baby was delivered vaginally to a
28-year-old second gravida mother at hospital without
any perinatal complications. There was no history of
consanguineous marriage or exposure to k n o w n
teratogens like drugs, industrial chemicals, or radiation.
First pregnancy ended with a spontaneous abortion at 2
months of gestation, 2 years back. There was no family
history of any congenital malformations. The baby's
weight, crown-heel-length and head circumference were
2620g, 46 cm and 31.8 cm, respectively. Examination
revealed shortening of left lower limb, anteromedial
bowing of tibia, calcaneovalgus deformity of the left foot
and shortening of ipsilateral femur by 2 cm. An
appendage was found arising from the anterior aspect of
the left ankle joint, measuring 6.5 cm in length and 2.8 cm
in diameter (Fig 1). It was firm in consistency. There was
no other congenital malformation, facial dysmorphism or
any evidence of developmental dysplasia of hip. X-ray of
the left lower limb showed absence of fibula and bowing
of tibia. The appendage contained four separate bony
structures within its mass (Fig 2), with no evidence of
bony fusion of appendage and the ankle joint.
Hemivertebra was also noted at D6 level. Skeletal survey
of other bones, chest X-ray and ultrasonography of
abdomen were normal. Orthopedic consultation was
taken but the baby was lost to follow-up.
Partial deficiencies of the lower extremity, like tibial

Fig. 1. Photographshowinglimb appendage.

Indian Journal of Pediatrics, Volume 72~December, 2005

and fibular hemimelias, are defined as congenital absence

of a longitudinal element, either pre-axial or post-axial, in
a segment of the lower extremity. Fibula is found to be the
most frequently deficient long bone. 1 The 4 th to 7 th
gestational week has been identified to be most prone to
these defects)

Fig. 2. X-rayshowingfibularhe"muneliaand limbappendage

Three types of fibular hemimelia are described in
literature.3In type I lesion, the mildest type, only one limb
is affected with partial absence of the fibula, with no
bowed tibia and no foot deformity. Functional
impairment is very little and bony defect is visible only on
radiological examination. In type II lesion, the limb is
moderately affected. The lesion is unilateral or sometimes
bilateral, with complete or almost complete absence of the
fibula. Equinovalgus deformity of the foot and anteromedial bowing of the tibia may be associated. The lateral
one or two rays of the foot may be absent and the other
bones of the foot are hypoplastic. There may be tarsal
coalition with development of a ball-and-socket ankle.
There is moderate to marked limb shortening. The
severest, type III lesion, has a bilateral defect and is
associated with proximal femoral deficiency or with
reduction deformities in the upper extremities and with
hemi-vertebrae. Our patient appeared to have a type II
lesion.
The degree of deformity of the foot or knee and the
amount of shortening do not necessarily correspond with
the extent of the fibular dysplasia. Although, in most
cases the foot is in equinovalgus position, there may be
calcaneo-valgus (as in this case) or pure equinus in some
cases.
Management (like lengthening, epiphyseodesis or
amputation) should be based on a careful analysis of
functional, psychological and cosmetic needs of the
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58

Causes of Hydronephrosis in Pyelonephritic Children

REFERENCES

p a t i e n t s . The p r o g n o s i s for h e i g h t , g r o w t h a n d
development of all segments of the limb is based on serial
examination and intervention begun early in life4. Early
surgical t r e a t m e n t and prosthetic rehabilitation yield
excellent results, not only for the short term but also for
the long term s.

1. Achterman C, Kalamchi A. Congenital absence of the fibula. J

Bone Joint Surg (Br) 1979; 61 (B): 133-137.
2. Amstutz HC. Natural history and treatment of congenital
absence of the fibula, l Bone Joint Surg (Am) 1972; 54 (A): 1349.
3. Coventry MB, Johnson EW. Congenital absence of the fibula.
J Bone Joint Surg (Am) 1952; 34 (A): 941-955.
4. Sharr~rd WJW. Major Congenital Malformations of the Limbs.
In PediatricOrthopedics and Fractures. ya edn. Oxford; Blackwell
Scientific Publications, 1993; 1 : 234-242.
5. Bohne WHO, Root L. Hypoplasia of the fibula. Clin Orthop
1977; 125: 107-112.

A s h o k K u m a r 1, Jayant K. G h o s h , Pragya Pant,

S. Basu and B.D. Bhatia

Division of Neonatology, ~Department of Pediatrics

Institute of Medical Sciences, Banaras Hindu University,
Varanasi- 221 005, India.

Causes of Hydronephrosis in Pyelonephritic Children

Sir,
Urinary tract infection (UTI) is one of the most c o m m o n
bacterial infectious diseases a m o n g children. Since 1960,
the use of radiological imaging has been accepted in child
with UTI. 1However, the choice of radiological imaging in
children is controversial. Urinary tract ultrasonography is
s o m e t i m e s p r e f o r m e d for initial u p p e r u r i n a r y tract
imaging because of the absence of radiation. Radiologists
m a y r e p o r t v a r i o u s d e g r e e s of dilation of collecting
system and recommended further investigation to detect
a b n o r m a l i t i e s such as vesicoureteral reflux (VUR) or
u r e t e r o p l e v i c junction obstruction (UPJO). VUR was
r e p o r t e d in 30%-50% of the children with UTI 2, a n d
dilatation of collecting system was reported in about 27%28% of children with UTI. 3q
Based on the data collected from September 1996 to
April 2003, all symptomatic febrile UTI patients referred
to D e p a r t m e n t of Pediatric N e p h r o l o g y of A m i r k o l a
Children Hospital (north of Iran) were enroled in this
study, and causes of urinary tract dilation were evaluated.
UTI was c o n f i r m e d with positive urine culture in the
samples. Any patient with dilation of collecting system
(by ultrasonography) was selected. Then, VCUG (voiding
cystourethrography) was done, and if the patients do not
have VUR, [VP ( I n t r a v e n o u s p y e l o g r a p h y ) or DTPA
(Diethylenetriamine pentaacetate) imaging was
conducted. Statistical analysis was performed using SPSS
version 10 and student t-test was used to compare the P-

values, which is less than 0.05 and considered significant.

346 patients were admited in the hospital with diagnosis
of UTI. 70 (20%) subjects h a d d i l a t i o n of UTI b y
ultrasound. 40 (57%) patients were male, younger than
the female patients (45 months vs 63 months). 29 (41%)
subjects had 6-12-years-old that were more than other
age-groups. A m o n g the a g e - g r o u p b e l o w 2 years, the
numbers of males outnumbered the females (Table 1). 37
(53%) patients had bilateral involvement and 27 (73%) of
them were males. 40 (57%) and 13 (19%) patients had
VUR and UPJO respectively (p<0.05) (Table 1). 26 (65%)
with VUR had bilateral involvement and 22 (55%) of them
w e r e male. VUR w a s the m o s t c o m m o n c a u s e of
hydronehrosis in the patients with pyelonephritis in both
sexes and all age groups, but renal stone was a second
cause of hydronephrosis in males above 6 yr (Table 1).
Ultrasonography is a non-invasive and non-ionizing
radiation form of imaging which could detect dilation of
collecting system. F o r e s m a n et al reported dilation of
k i d n e y in 52 (28%) patients with UTI. 3 Some a u t h o r s
showed the frequency of VUR had no difference between
patients with or without dilatation (39% vs 32%). 5 Also,
the sensitivity and specificity of ultrasound for detection
of VUR were 40% and 76% respectively; positive a n d
negative predictive value of ultrasound for VUR were
32% and 82% respectively?"5Dipietro found ultrasound is
not reliable for detection of VUR in children aged 5 yr or
older? In this study, 20% of patients with UTI had dilation

TABLE1. Cause of Dilation of Collecting System in Children with UTI According to Age and Sex Groups

Causes Sex
Age groups (years)
Less than 2
3-5
6-15

VUR

UPJO

UVJO

Stone

12
3
7
22

5
3
10
18

3
2
2
7

1
2
3
6

3
2

Uretrocel

1
5
6

1
2
3

Total
F
2
2

25
16
29
70

VUR: Vesicoureteral reflux; UPJO: Urerteroplevic junction obstruction, UVJO: Ureterovesical junction, M: males, F: females
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Indian Journal of Pediatrics, Volume 72--December, 2005