Small-bowel perforation in very low birth weight neonates treated with high-dose dexamethasone.

INTRODUCTION: Early postnatal treatment with high doses of corticosteroids may be effective in reducing the duration of mechanical ventilation in very low birth weight infants at risk for bronchopulmonary dysplasia. However, serious side effects may occur. MATERIAL AND METHODS: A retrospective study on 5 very low birth weight neonates, mean (+/- SEM): gestational age range 27.5 +/- 2 weeks, mean birth weight (+/- SEM): 836 g +/- 169 referred between April 1997 and October 1998 from a single academic neonatal intensive care unit to our tertiary pediatric surgical center with the diagnosis of intestinal perforation. During the same period, 60 very low birth weight infants have been treated in that unit following a standardized protocol including surfactant for respiratory distress syndrome and high-dose dexamethasone to prevent bronchopulmonary dysplasia. RESULTS: Pneumoperitoneum was diagnosed between 6 to 9 days after birth. Clinically, all babies remained surprisingly stable. An isolated ileal perforation, without sign of necrotizing enterocolitis, was found at laparotomy in each patient. A limited intestinal resection was performed, with primary end-toend anastomosis (3 cases) or with transient ileostomies (2 cases). Surgical outcome was favorable in all patients. CONCLUSION: isolated intestinal perforation may be a complication of the preventive treatment of chronic lung disease with high-dose corticosteroids in very low birth weight infants. Conversely, corticosteroids may reduce the clinical signs and the multiple organ dysfunction associated with an abdominal drama, explaining the very good surgical prognosis of these newborns. Attention must be paid to an insidious pneumoperitoneum.

Gastric outlet obstruction: unusual ultrasonographic findings in the pyloric and antral regions.

The authors present the cases of four infants aged 2-4 months with symptoms suggesting gastric outlet obstruction. All four had US evaluation of the antropyloric region and upper GI series. Three of them underwent endoscopic investigations and three, surgery. US demonstrated hypertrophied pyloric muscle and, above all, antropyloric abnormalities in all cases, namely a solid mass within the pyloric canal, hypertrophy of the pyloric mucosa in two cases and a double juxtapyloric cyst with an ulcer. Differential diagnosis of the sonographic images is discussed in each case.

Midgut malrotation, the reliability of sonographic diagnosis.

We are unaware of any other anatomical study which deals with normal variations in the relationship between the superior mesenteric artery (SMA) and the superior mesenteric vein (SMV). In our investigation in the majority of cases, the SMV lies to the right of the SMA. It has been suggested that ultrasonographic demonstration of a left sided SMV, is characteristic of midgut malrotation so a prospective study was undertaken in order to try to correlate the position of the mesenteric vessels on Ultrasound with the radiological diagnosis of midgut malrotation on upper GI series. 427 children (95% infants) who were planned for upper GI investigation, have had color Doppler U.S. of the mesenteric vessels before the barium study. In 67 cases, the mesenteric vessels were obscured by intestinal gas. Among them, there were 2 cases of malrotation. Three different positions of the mesenteric vessels were found in the 360 remaining cases. 1) A left sided SMV was found in 4% of the cases, all had midgut malrotation on X-rays and at surgery. 2) A mesenteric vein anterior to the SMA was found in 5% of the cases, 28% had midgut malrotation. 3) A normal right sided SMV was found in 91% of the cases. However 3% of these had midgut malrotation. There are anatomical variations in the position of the mesenteric vessels and a normal relationship between SMA and SMV does not exclude the possibility of intestinal malrotation. Nevertheless, ultrasound remains a useful noninvasive screening technique for the diagnosis of midgut malrotation.

Post-necrotising enterocolitis pseudo-obstruction treated with Cisapride.

We report on one patient in whom segmental colic intestinal pseudo-obstruction (IPO) following the surgical treatment of a grade III necrotising enterocolitis (NEC) was responsible for a severe failure to thrive. Further intestinal resection in an already short gut was avoided by using Cisapride, a new intestinal prokinetic agent (1 mg/kg/d in 4 doses, orally), which dramatically improved the symptoms and allowed weight gain and intestinal adaptation. After 6 months, Cisapride was withdrawn. IPO did not recur after 2 years of follow-up, although proximal distention persisted.

Proenkephalin A associated peptides in the autonomic nervous system of the human infant gastrointestinal tract.

The digestive tract of neonates and infants were examined by immunohistochemistry using specific antisera raised against proenkephalin A related peptides. Proenkephalin A, methionine-enkephalin and leucine-enkephalin are observed in nerve fibres in the smooth muscles in the myenteric and submucosal plexuses or in neuronal cell bodies of the myenteric plexus. In these structures synenkephalin has general distribution as methionine-enkephalin but not the same as leucine-enkephalin. Co-localization of synenkephalin and methionine-enkephalin is found in several neurones. These results suggest that proenkephalin A is the precursor-protein in some enkephalinergic neurones of the human gut. A gradient in the density of immunoreactivity is observed and is maximal in the distal small bowel. This gradient contrasts with observations made in rodents where major enkephalin immunoreactivity is observed in the proximal digestive tract. These findings give evidence that proenkephalin A-derived peptides could have effects in the motility of the human gut.

Decrease of vasoactive intestinal peptide, methionine-enkephalin, substance P and increase of neuropeptide Y immunoreactive nerve fibres in aganglionic colon of Hirschsprung's disease.

Ganglionic and aganglionic full-thickness samples, at 4 levels of the colon of 26 infants with Hirschsprung's disease, were studied by immunohistochemistry. In the distal part of the aganglionic bowel, we observe a decrease of substance P and vasoactive intestinal peptide, an absence of methionine-enkephalin and an increase in neuropeptide Y nerve fibres. When detected, substance P and vasointestinal peptide are mainly present in abnormal bundle nerve fibres. In the middle part of the aganglionic bowel, a slight increase in the number of normal nerve fibres containing substance P, methionine-enkephalin and vasoactive intestinal peptide is observed. Some vasoactive intestinal peptide abnormal bundle nerve fibres are detected. They are less numerous than in the distal part. In the proximal ganglionic bowel, the number of vasoactive intestinal peptide, substance P and methionine-enkephalin normal nerve fibres is increased compared to the middle aganglionic segment but is slightly lower than in the normal colon. Again vasoactive intestinal peptide abnormal bundle nerve fibres are present at that level and are also detected in more proximal ganglionic bowel up to the hepatic flexure of the colon. Thus, abnormal distribution of neuropeptides is also found in more proximal ganglionic bowel and not only in the aganglionic segment of bowel usually specific of Hirschsprung's disease.

Falciform ligament: a cause of extrinsic antral obstruction in the neonatal period.

We are describing two cases of neonatal antral obstruction due to the presence of an abnormal falciform ligament. Abdominal palpation revealed a cord in the right hypochondrium. In one case, a barium swallow revealed a very short extrinsic compression of the antrum. Division of the abnormally wide fibrous falciform ligament cured the babies.

Unusual association of upper gastro-intestinal anomalies in a newborn.

We are describing one case of persistent nonbilious post-prandial vomiting in a newborn due to hypertrophic pyloric stenosis, intestinal malrotation and gastric antral diaphragm. Those three anomalies were successively diagnosed as causes of persistent upper gastrointestinal subobstruction.

[Inguinal hernia in infants and children]. / La hernie inguinale chez le nourrisson et l'enfant.

Inguinal hernia is a rather common pathology encountered in children, present in 1 to 3% of full term newborns and in 3 to 5% of premature babies. Between January 1982 and January 1983, we operated upon 134 children with inguinal hernia, more than half of them were under 6 months of age. In most cases, the history of the patient would suffice to make the diagnosis, indeed, in 99 cases, the history would reveal the transient presence of an inguinal mass often absent during the examination at the clinics. In every other case we palpated an inguinal mass. Among these, 15 cases of indirect inguinal hernia were found, 4 were spontaneously reduced by soft manipulations, 10 were reduced under premedication and only one was irreducible. The other cases of irreducible inguinal mass appeared to be hydrocele and cysts of the cord. All our patients were in a good state of health, but one child suffering from a strangulated inguinal hernia causing intestinal obstruction. He was the only one to undergo an emergency operation, the others were operated upon within 24 to 72 hours after reduction of the mass. No relapse was ever observed. We advocate systematic surgery for any diagnosed hernia. The surgical treatment consists in the closing of the peritoneal vaginal duct. This operation is to be performed regardless of the child's age, height or weight and therefore avoiding complications a strangulated irreducible inguinal hernia would bring. Optimal operation conditions are gathered in a paediatric surgery unit where one is used to the anesthesia and to the delicate postoperative supervision of very young children.

[Macrodystrophia lipomatosa. Angiographic aspects (author's transl)]. / Macrodystrophie lipomateuse. Aspect artériographique.

A new case of this unusual entity is reported. The patient, male aged 43 has an hypertrophy of the first and second left fingers. Radiographic examination shows bony overgrowth and marked soft tissue thickening. Arteriography did not show any evidence of angioma or arteriovenous fistula. Histologic examination shows deposits of fat in soft tissue thickening. Macrodystrophia lipomatosa is a rare disease, usually confined to one or two digits of the hand, and sometimes of the foot. A dysembryoplasic aetiology could be sustained, and this entity is very close from others bone or soft-tissue hamartomas.