pialJ. Ptdiat. 43, 1115, 1976
P R U N E BELLY S Y N D R O M E IN A 1 3 - Y E A R - O L D BOY* Report of a case with a brief review VEI?RINDE,R SINOII, H. LAL, L. RAMA KrlMAR
of the literature AND ~ARINDER SlNOtt
Patiala
The "prune trolly syndrome" also :~lled " t h e triad syndrome" is charac~erlsed by hypoplasia of the abdominal wall ~attscles, cryptorchidi~m and congenital inomaiies of the urinary tract. Most of the :ases with this clinical entity die during ~arly infancy and only a few survive beyond ;,arly childhood. The cause of death in ~aost of these cases is attributed to the :on/genital urinary tract abnormalities. According to Ruprecht and Teske 1960) 240 cases of the prune belly syndrome aave been described in the literature up to t961. Most of the earlier reports convey ~ery little information about prognosis. A Pew recent reports mention that a small ~rcentage of cases may reach adulthood. A few cases of prune belly syndrome W e been reported ii'om India but only ~j~aong infants (Chopra et al. 1973, Pathak a/.+1974); and to the best of our knowledge there is no report from India so far of a case With this syndrome surviving at 13 years of No. Report of a Case
D.S., a 13-year-old male child, was admitted to the paedlatric ward of the . j ~., ~Rajindra Hospital, Patiala, on 26.5.75 with ~he complaints of abnormality of the anterior abdominal muscles since birth ~late 1, Fig. 1), mild to moderate grade of
~m
the Department of Paediatrics, Government ~ d i c a l College, Patiala. ~eived on December 20, 1975.
fi:ver fi,r one year, and pain in the lower limbs with increasing difficulty white standing and walking of six months' durati,,n. On symptomatic enquiry, there was no hi~.tory of chronic diarrhoea, colicky abdominal pain, dyspnoea, convulsions, visual disturbances or swelling of the joints. Antenatal, natal and postnatal history were noncontributory. There was no history of similar abdominal or skeletal deformities in parents and other members of the family. On examination, the child was markedly undernourish,'d with generalised loss of subcutaneous fat and muscle mass. The ternperamre of the child ranged from 99.5~ to 101.2"+F and the B.P. was 110/60. The head was asymmetrical with bossing of the frontal and parietal regions. The forearms had a lateral curvature with prominence of the wrists. There was lateral bowing of the thighs and anterolateral bowing of the legs wi~k marked knock knee deformities. The child had the pectus carinatum deformity of the chest with prominence of the costochondral junctions. The heart and lungs revealed no abnormality. The scrotum was of small size with few rugae and the pubic hair was scant?- and downy. The testes were undesccnded and the external urethral meatus was normal. With the child lying in bed, tile abdomen was distended with absence of both the recti. The left kidney was palp a n e . The liver and spleen were not pa!pable. The rest of the systemic examination did not show any abnormality.
106
INDIAN
JOURNAl. OP PF,|)IA'I'I~,{r
The child was anaernic will, a lwmoglobln h'vel of 3.5 (;.%. 'l'-,.,I and ,l;ff,.,,.r!tial h : l t c o c y l e cottnls wt.te 5400/r 41% (polymorphs), 5P'7.,, (lymphocyt,.s) respectively. Specific gravity of the urine was 1020; sugar and alb, min were al ...... t, microscopic examination r,:ve;aled r to 8 pus cells I, er high power field. A culture of the urine saml,le produced g~owth of E. toll, sensitive to mandalamine. Uth,a~y excretion of ca~.cium was 200 rag. in 24 hours. Blood urea, serurr, calcium and alkaline pho:,phatase were 53 rng.~ 11.5 mg.~/o and 50 units respcctiv
P R U N E BELLY S Y N D R O M E IN A 1 3 - Y E A R - O L D BOY* Report of a case with a brief review VEI?RINDE,R SINOII, H. LAL, L. RAMA KrlMAR
of the literature AND ~ARINDER SlNOtt
Patiala
The "prune trolly syndrome" also :~lled " t h e triad syndrome" is charac~erlsed by hypoplasia of the abdominal wall ~attscles, cryptorchidi~m and congenital inomaiies of the urinary tract. Most of the :ases with this clinical entity die during ~arly infancy and only a few survive beyond ;,arly childhood. The cause of death in ~aost of these cases is attributed to the :on/genital urinary tract abnormalities. According to Ruprecht and Teske 1960) 240 cases of the prune belly syndrome aave been described in the literature up to t961. Most of the earlier reports convey ~ery little information about prognosis. A Pew recent reports mention that a small ~rcentage of cases may reach adulthood. A few cases of prune belly syndrome W e been reported ii'om India but only ~j~aong infants (Chopra et al. 1973, Pathak a/.+1974); and to the best of our knowledge there is no report from India so far of a case With this syndrome surviving at 13 years of No. Report of a Case
D.S., a 13-year-old male child, was admitted to the paedlatric ward of the . j ~., ~Rajindra Hospital, Patiala, on 26.5.75 with ~he complaints of abnormality of the anterior abdominal muscles since birth ~late 1, Fig. 1), mild to moderate grade of
~m
the Department of Paediatrics, Government ~ d i c a l College, Patiala. ~eived on December 20, 1975.
fi:ver fi,r one year, and pain in the lower limbs with increasing difficulty white standing and walking of six months' durati,,n. On symptomatic enquiry, there was no hi~.tory of chronic diarrhoea, colicky abdominal pain, dyspnoea, convulsions, visual disturbances or swelling of the joints. Antenatal, natal and postnatal history were noncontributory. There was no history of similar abdominal or skeletal deformities in parents and other members of the family. On examination, the child was markedly undernourish,'d with generalised loss of subcutaneous fat and muscle mass. The ternperamre of the child ranged from 99.5~ to 101.2"+F and the B.P. was 110/60. The head was asymmetrical with bossing of the frontal and parietal regions. The forearms had a lateral curvature with prominence of the wrists. There was lateral bowing of the thighs and anterolateral bowing of the legs wi~k marked knock knee deformities. The child had the pectus carinatum deformity of the chest with prominence of the costochondral junctions. The heart and lungs revealed no abnormality. The scrotum was of small size with few rugae and the pubic hair was scant?- and downy. The testes were undesccnded and the external urethral meatus was normal. With the child lying in bed, tile abdomen was distended with absence of both the recti. The left kidney was palp a n e . The liver and spleen were not pa!pable. The rest of the systemic examination did not show any abnormality.
106
INDIAN
JOURNAl. OP PF,|)IA'I'I~,{r
The child was anaernic will, a lwmoglobln h'vel of 3.5 (;.%. 'l'-,.,I and ,l;ff,.,,.r!tial h : l t c o c y l e cottnls wt.te 5400/r 41% (polymorphs), 5P'7.,, (lymphocyt,.s) respectively. Specific gravity of the urine was 1020; sugar and alb, min were al ...... t, microscopic examination r,:ve;aled r to 8 pus cells I, er high power field. A culture of the urine saml,le produced g~owth of E. toll, sensitive to mandalamine. Uth,a~y excretion of ca~.cium was 200 rag. in 24 hours. Blood urea, serurr, calcium and alkaline pho:,phatase were 53 rng.~ 11.5 mg.~/o and 50 units respcctiv
