ABSTRACTS 767

GENERAL CONSIDERATIONS

Congenital Malformations in Newborn Babies. Jo~o M. Videirar'.4maral, M.E. Sacramento Monteiro,:.= Nuho Cordeiro Ferreira, and Wilson Lopes. Rev Port Pediatr Special number on neonatology 1977

A retrospective study of 5921 live-born and stillborn infants (5776 and 145, respectively) in a neonatal care unit in the city of Lisbon was made in order to estimate the incidence of congenital malformations (CM), both as regards the total number and the types of malformations diagnosed at birth and classified as major and minor. The incidence of CM major was 2.6%, and that of CM minor was 5.6%. The incidence of major malformations was higher in infants with low birth weights, and that of minor malformations was higher in infants with weights over 2.5 g.

Malformation was one of the more important causes of neonatal mortality; it was found that 10.9% of stillborn infants and 15% of live-born babies who had died were severely malformed.--Antbnio Gentil Martins

Fetal Wastage as a Result of an Alpha-Fetoprotein Screening

Programme; Iv'. B. Gordon, M. J. Kitau, A. T. Letehworth, J. G. Grundzinskas, M. M. Usherwood, and T. Chard. Lancet 1:677-678 (April), 1978

One hundred two women underwent amniocentesis for the measurement of amniotic alpha-fetoprotein following the finding of raised serum alpha-fetoprotein above the 95th percentile at 16-24 wk. The procedure was performed after ultrasonic confirmation of gestational age, placental localization, and absence of multiple pregnancy. As a result, 17.6% (18 patients) underwent termination of pregnancy. Of these 18, 15 fetuses were found to have neural tube defects, 2 had gastrointestinal abnormalities (one with exomphalos and multiple defects, the other with exomphalos alone), and 1 patient had hydrocephalus. Four patients spontaneously aborted after the procedure, one of which was abnormal with exomphalos and neural tube defect.

The authors suggest that in order to reduce the number of patients submitted to amniocentesis, action should be taken only if serum alpha-fetoprotein exceeds 2.5 times the median value at that gestational age; in order to prevent the complications of amniocentesis, the procedure should be performed under direct ultrasonic observation. They suggest no way in which differentiation may be made between gastrointestinal tract abnormalities and neural tube defects on the basis of alpha-fetoprotein.--J. G. Harvey

Adverse EffeCts of Routine Procedures on Preterm Infants.

B. D. Speidel. Lancet 1:864-865 (April), 1978

Using continuous monitoring of arterial oxygen tension in neonates, the author shows that a significant fall in PaO 2 occurs when the sick neonate is handled during nursing observations, blood.taking,or x~ray proced~es. Spontaneous

.. r e tu rne r the PaO 2 usually occurs; but repeated or~sequential procedures may cause a prolonged fall in PaO2 necessitating ventilatory support. The cause of the fall in PaO 2 may be related to transient apnea during handling or to right-to-left shunting of blood through the foramen ovali or ductus arteriosus during crying. The author suggests that a policy of minimal handling should be adopted in all neonatal nurseries.--J. G. Harvey

Cystic Fibrosis Screening in the Newborn. Jeanette R. Crossley, Colleen C. Berryman, and Robert B. Elliott. Lancet 2:1093-1095 (November), 1977

The authors describe a new screening test for cystic fibrosis in the neonatal period based on the pancreatic enzyme trypsin in the stool sample. A colorless substrate b e n z o y l - a r g i n i n e - p - n i t r o a n i l i d e ( B A P N A ) re leases p-nitroaniline when hydrolyzed by trypsin. Stool samples from neonates who lack tryptic activity give negligible color change. The incidence of false positive results was 0.1% and 0.05% on retesting. There were no false negative results. The authors justify the new method in terms of its sensitivity and ease of interpretation and application.--J. G. Harvey

Cimetidine Cholestatic Jaundice in Children. John R. Lilly, David C. Hitch, Norman B. Javitt. J Surg Res 24:384-387 (May), 1978

Five children ages 1-14 yr with peptic ulcer disease were treated with cimetidine. Dosage was calculated at 20-30 mg/kg/day for both oral and intravenous administration. Serum bilirubin, glutamic oxaloacetic transaminase, alkaline phosphatase, and serum bile acid determinations were made prior to therapy, during therapy, and after treatment. A l l patients demonstrated prompt radiographic healing of ulceration.

Elevated serum bile acids were noted in all patients during therapy, but they returned to normal after discontinuation of cimetidine. Serum bile acid is considered a more discriminating index of hepatic excretory function, and hence cholestasis, than serum bilirubin. It is speculated that cimetidine induces a ductular-type cholestasis because of its blocking effect of vagal and secretin receptors.--Richard J. .4 ndrassy

Hepatic Impairment in Infants Induced by Hig h Calorie Infusion.

1I. Hirai, Y. Sanada, T. Fujiwara, et al. J Jpn Soc Pediatr Surg 14:409 420 (April), 1978

Hepatic impairment was encountered in 14 of 19 newborns and 4 of 9 infants who had long-term intravenous hyperalimentation (more than 2 wk).

The authors classified types of hepatic impairment induced by high calorie infusion into three groups: Transient elevation of serum transaminase without elevation of alkaline phosphatase (type I) was seen in 6 newborns and 4 infants. This type of hepatic impairment was considered to be due=to, imbalance of nutrients. Elevation of alkaline phosphatase and direct bilirubin without elevation of serum transaminase ( type II) was seen in 2 newborns. The ballooning of liver cells by overloading of nutrients resulted in compression of bile canaliculi and obstructive jaundice in this group. Marked and prolonged elevation of alkaline phosphatase, direct bilirubin, and serum transaminase (type III) was seen in 6 newborns. T h e etiology of this type of impairment was considered,to be immaturity of hepatocellular enzymes.--H. Suzuki

Effects of Prolonged Parenteral Nutrition In Preoperatory

Treatment of Glycogenosis. J. G. Maksoud, U. Tannuri, .4. L. Mathias, and V. .4. Carvalho Pinto. Rev Assoc Med Bras 23:211-213 (June), 1977

The authors present their experience with 2 children with glycogen s torage disease t rea ted with intravenous

768 ABSTRACTS

hyperalimentation and subsequently submitted to portacaval shunt. Serum lipids, glucose levels, and acid-basic status were restored to normal before the operation. This small experience reinforces similar reports indicating the benefit of this therapy.--Alberto Peha.

A Life-Threatening Complication of the infusion Pump. Arnran Ayalon, Yacov Berlatzky, Haim Anner, and Medad Schiller. Lancet 1:853-854 (April), 1978

The authors report 2 patients in whom serious disturbances of respiration and venous return resulted from extravasation of fluid into the mediastinum during intravenous parenteral nutrition via a central vein. The authors relate this complication to the use of a peristaltic infusion pump and stress that pressures of up to 850 mm Hg can be generated by the pump, which will continue to operate despite extravasation and will cause a rise in pressure in closed compartments. They suggest that the infusion pump be abandoned in neonatal practice.--J. G. Harvey

Zinc and Copper Changes After Neonatal Parenteral

Alimentation. K. N. Sivasubramanian, G. Hox, and Mary Kate Davitt. Lancet 1:508 (March), 1978

The authors report a child with necrotizing enterocolitis who underwent parenteral alimentation with Freamine II and Intralipid for 5 wk. Serum zinc fell from 64 #g/dl to 18 #g/dl; serum copper fell from 62 /Lg/dl to 39 #g/dl. The child developed weeping erythematous skin lesions and showed increasing irritability. Treatment was instituted with zinc sulfate, 100 ug/kg/day, with improvement after 48 hr and disappearance of the dermatologic and neurologic symptoms and signs within 4 days.

The authors stress the necessity of measuring the serum zinc and serum copper and of using supplements of these two metals over long periods of parenteral alimentation.--J. G. Harvey

HEAD AND NECK

The Role of Psychiatry in a Craniofacial Team. A. Lefebvre, and 1. Munro. Plast and Reconstr Surg 61:564-569 (April), 1978

Because surgical and medical advances have not permitted reconstruction of severe congenital facial deformities until recently, psychological changes produced by this correction are not completely known. This study attempts to answer psychological questions involving the importance of the patient's age at surgery, the postoperative results, and contraindications.

Seventy patients and their parents were seen for separate 1-hr preoperative interviews. During these interviews, tests were used to reveal body image, impressions of deformities, coping mechanisms used by the child and parents, and other background information. Postoperatively, the parents and child were again seen separately for an interview, with similar parameters being retested.

The intellectual capacities of these children appeared to be normal. Appreciation of the deformity ranged in degree from grotesque to minimally deformed, apparently related to the self-assurance and confidence enjoyed by the patient. The improvement of the deformity as seen by the child and

parents postoperatively appeared to be related to the degree of expectation and self-assurance of the child. Coping mechanisms used by both child and parents were reviewed, with explanations given.

It was recommended that the surgical intervention be performed at the earliest possible date. No absolute eontraindications to surgery were seen even in the most "maladjusted child." However, it was suggested that children demonstrating psychotic behavior are poor candidates for plastic surgery.

The article is recommended to all physicians and paramedical personnel concerned with the surgical treatment and medical support given to the child with craniofacial anomalies.--A. B. Sokol

Advancement of the Orbits and the Midface In One Piece,

Combined With Frontal Repositioning, for the Correction of

Crouzon's Deformities. F. Ortiz-Monasterio, A. Fuente de/ Campo, and A. Carrillo. Plast and Reconstr Surg 61:507-516 (April), 1978

From a total of 176 orbital and facial mobilizations done in 5 yr, the authors report on 7 patients with Crouzon's deformity. These patients had advancement of the complete orbits, frontal bone, and maxilla, in one piece to achieve correction. All patients had maxillary retrusion, type III malocclusion, and exorbitism. Two patients were adults (35 and 24 yr old) and the other 5 were between the ages of 4 and 6 yr. The authors believe that the best time for this operation is 5 yr of age.

Preoperative studies include the use of cephalograms, dental molds, facial photographs, and exophthalmometry. Preoperative planning includes exact reconstruction and repositioning required so that concurrent bicortical iliac bone grafts and split rib grafts can be taken during the procedure.

The operation is performed under general nasotracheal anesthesia. No tracheostomy nor mannitol has been required. The operation consists of two incisions, one coronal and one intraoral. Through these incisions the entire frontal bone and midface are separated from the skull to allow advancement and correction of the deformity. The advancement is secured by bicortical iliac and split rib grafts. No interdental fixation is required.

An excellent review is given of the evolution of craniofaeial surgery. In addition, the article is accompanied by excellent preoperative and postoperative clinical photographs demonstrating the remarkable results obtained with this procedure. Finally, there is clear operative discussion, and there are diagrams to facilitate an understanding of this operation.--A. B. Sokol

Sialadenitis in Childhood. L. B. Kaben, J. B. Mulliken, and J. E. Murray. Am J. Surg 135:570-576 (April), 1978

A 30-yr retrospective review of 49 patients up to 16 yr of age treated at the Children's Hospital Medical Center, Boston, Mass., suggests four types of sialadenitis differing in etiology, clinical manifestations, treatment required, and prognosis.

Acute suppurative parotitis (18 patients) was the most common form of pediatric sialadenitis, and it commonly occurred in neonates and in children, with fevel", dehydration, or immunosuppression accompanying an