CORRESPONDENCE

To the Editor:

Among the lymphatic vessel dysplasias it is interesting to emphasize the lymphatic vessel abnormalities in patients with Noonan’s syndrome, especially at the level of the pelvis. Cutanous oozing and lymphedema between the thighs and on the scrotum may be the clinical findings indicative of a primary iliac lymphangiectasia with reflux to the genitalia peripheral lymphatics. We did report two cases with Noonan’s syndrome inAJR, February 1980 (134:399-401). A boy 10 years of age had on lymphangiography tortuous pelvic channels, distended and poorly filled. There was even opacification of abdominal and lower subcostal pathways. The boy suffered very much from the oozing. Surgery was performed at 17 years of age. The internal iliac lymph nodes were enormous up to the lumbar area. Ligature of the lymphatic vessels

To the Editor:

The paper by Gongaware et al (26:1430-1431, 1991) describes a case of a testis prolapsed through a skin defect in the upper scrotum. The authors’ claim that this is a very rare case only described once previously. I draw to the attention of the Journal readers a similar case with a photo that is almost identical in the Journal of Urology in 1990. This case report was described by Dr Chris Heyns in the Journal of Urology. ’ Dr Heyns cites at least one other case that has not been mentioned by Dr Gongaware. The authors’ mentioned some old theories that might account for this abnormality, but these do not appear convincing. A much more plausible explanation has been proposed by Douglas Stephens,*,’ which is that there is pressure atrophy from the compression of the scrotum by the heel of the baby’s foot. Pressure atrophy or compression of the inguinoscrotal region has been postulated as a cause for delayed testicular descent and we have confirmed this experimentally. If the pressure was sufficient to cause necrosis of the skin the migrating testis and gubernaculum would naturally prolapse through this defect into the amniotic

with bilateral curetage of internal iliac lymph nodes was performed and healed the oozing. A lo-year-old girl had a truncal and facial dysmorphy typical of Noonan’s syndrome. Surgical biopsy gave rise to an intermittent cutaneous chylous fistula, followed later by a recurrent chylometrorhea and a lymphedema of the left labia majora of the vulva. Lymphangiography of the foot showed a generalized lymphatic dysplasia. Our article is not mentioned in the 48 references of the article of R. Hilliard et al. J. C. Hoeffel

Centre Hospitalier Universitaire Nancy, France

cavity. In the photograph given by Dr Gongaware and coauthors’ there is an area around the opening where the skin looks thin and atrophic. This is entirely consistent with an area of pressure atrophy and central necrosis with secondary prolapse of the descending testis. When seen in this light such an abnormality does not appear to be related to other forms of ectopic testis, where the gubernaculum has apparently migrated in an aberrant direction. Other abnormalities of the inguinoscrotal region also appear to be caused by heel pressure in some babies. Chordee, and even occasionally anterior urethral fistula have been described, and fit Stephens’ explanation that these are secondary effects of compression by the feet in utero. This explanation of testicular ectopia, and the principle of fetal compression in general appears to be not widely known. John M. Hutson

Departments of General Surgery and Paediatric Surgery Royal Children’s Hospital Melbourne, Australia

REFERENCES 1. Heyns CF: Exstrophy of the testis. J Ural 144:724-725,199O 2. Cook WR, Stephens FD: Pathoembryology of the urinary tract, in Ring LR (ed): Urologic Surgery in Neonates and Young Infants. Philadelphia, PA, Saunders, 1988, pp l-23

3. Luthra M, Hutson JM, Stephens FD: Effects of external inguinoscrotal compression on descent of the testis in rats. Pediatr Surg Int 4:403-407,1989

To the Editor:

into the thoracic cavity and is associated with very little postoperative morbidity. R.J. Brereton

We would agree with Saucy et al’ that a muscle-sparing lateral thoracotomy has much to recommend it in neonates, although the article did not report on the actual clinical experience in terms of numbers of patients or complications. Since 1982 we have used a similar posterolateral muscle-sparing incision as our standard approach in 51 consecutive neonates with esophageal atresia.*.’ It offers a quick and relatively bloodless route

JournalofPediatric

Surgery, Vol.27, No 9 (September), 1992: pp 1257-1259

Department of Paediatric Surgery Institute of Child Health London, England D. W. Goh

Department of General Surgery Royal Children’s Hospital Melbourne, Australia

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Lymphatic vessel dysplasias.

CORRESPONDENCE To the Editor: Among the lymphatic vessel dysplasias it is interesting to emphasize the lymphatic vessel abnormalities in patients wi...
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