Endoscopic repair of bilateral choanal atresia in neonates: anesthetic and surgical implications

Bilateral Choanal Atresia patients are usually in respiratory distress at birth. Such patients [neonates] have unique anesthetic and surgical requirements regarding equipment, intravenous access, fluid and drug therapy, anesthetic dosage, and environmental control. An understanding of the basic differences in physiology, pharmacologic and pharmaco-dynamic responses, and the underlying pathology of the surgical problem is essential for the development of a safe anesthetic/surgical plane. To describe and evaluate the outcome of the anesthetic / endoscopic transeptal approach for the repair of bilateral choanal atresia in neonates. A prospective analysis of a case series of neonates with bilateral choanal atresia who were treated by the endoscopic transeptal approach between July 2003 and December 2005 was undertaken. Endotracheal intubation was performed under adequate plane of general [inhalational/narcotic] anesthesia. Adequate depth of general [inhalational/narcotic] anesthesia with spontaneous and gentelly assisted ventilation were appropriate till the end of surgery. The endoscopic approach was performed using 4-mm 0° telescope. A laterally based trapezoid shaped septal mucosal flap was created on each side using a radiofrequency needle. The flaps were elevated in a swinging door fashion to expose the atretic plate and the vomer. After removal of the posterior bony septum and widening of the choana, the flaps were trimmed and applied to the exposed lateral bony rim of the choana. All patients were stented bilaterally for three to four weeks postoperatively. Neonatal/infant Pain Scale [NIPS] was used to assess the wellbeing and pain status of neonates in the immediate postoperative period. The study group included 14 neonates aged 2 to 25 days at the time of surgery. Follow up ranged from 4 to 36 months with a mean of 17.3 +/- 9.3 months. All neonates had a smooth anesthesia course, with minimal blood loss. Postoperatively, all have 0 to 1 score in the NIPS. Recovery was uneventful in all cases except for one case that developed bleeding in the immediate postoperative period and was controlled under endoscopic guidance. One case died three months following stent removal from uncompensated heart failure due to concomitant ventricular septal defect. An adequate functional nasal breathing and appropriate feeding with sufficient weight gain were maintained during the entire follow-up period. The described anesthetic/endoscopic technique was very adequate. It offered excellent visualization of the choana and allowed maximal widening of the choana while preserving the mucosa along the entire circumference of the newly created choana and hence less risk of postoperative stenosis

Glucose-6-phosphate dehydrogenase [G6PD] deficency in neonates presenting with Jaundice

To determine the frequency of Glucose-6-phosphate dehydrogenase [G6PD] deficiency in infants presenting with jaundice. This retrospective study was conducted in Fazal Rahim Clinical Laboratory Timergara District Dir North West Frontier Province of Pakistan, from 1st January 2004 to 31st December 2004 on 120 neonates. Data regarding sex and age, serum bilirubin total, serum bilirubin direct and G6PD status was extracted using database software designed by the principal author. The inclusion criterion was neonates with high serum bilirubin and G6PD test performed simultaneously. The exclusion criterion was premature babies and incomplete request forms. Microsoft Excel 2000 was used for data analysis Out of 120 patients, 97 [80.8%] were male and 23 [19.2%] were female ranging in age from 3 - 10 days with mean 6.5 days and median 5 days. Thirty two [26.6%] neonates were found G6PD deficient. Among patients with normal G6PD level male to female ratio was 3.63:1. In G6PD deficient patients male to female ratio was 7:1. The serum bilirubin of the G6PD normal patients was 12.8 + 5.0 mg/dl and that of G6PD deficient patients was 13.5 + 6.8 mg/dl. G6PD deficiency is quite high in neonates presenting with jaundice. The diagnosis is simple and if left undetected may cause serious consequences in situations of oxidant stress

Renal ultrasonography in neonates

Ultrasound [US] is a safe and accurate imaging method in the evaluation of urinary tract and its anomalies in neonates. It is helpful in establishing correct diagnosis in the majority of cases and facilitates precise measurement of kidney length. We conducted this work to study the prevalence and types of renal anomalies in Egyptian neonates and to identify their normal renal length. Our study was conducted on 500 Egyptian neonates, at Alexandria University Maternity and Children's Hospitals. Ultrasonographic examination and measurement of both kidneys were done in all neonates. Abnormal renal ultrasonographic findings were found in 15 neonates, with a prevalence of 3%. The abnormalities encountered were severe bilateral hydronephrosis in 8 cases [1.6%], moderate bilateral hydronephrosis in 3 cases [0.6%]. Horseshoe kidney, bilateral hyperechoic renal medulla, polycystic kidney disease [ARPKD], and unilateral renal tumor were detected in equal frequencies of 0.2% each. Further evaluation of the cases with severe hydronephrosis revealed: posterior urethral valve [PUV] in 5 cases, bilateral primary vesicouretral reflux [VUR] in 2 cases, bilateral pelviureteric junction obstruction [PUJO] in one case. Cases with moderate hydronephrosis resolved on follow-up. Sonographic renal appearance in normal neonates was different from that seen in older children. They showed a pattern of increased corticomedullary differentiation with markedly sonolucent medullary pyramids. The mean renal length in normal newborns was 4.7 +/- 0.7cm. There was a positive correlation between renal length and each of body length and weight than with age and head circumference. The high prevalence of renal anomalies in Egyptian neonates justifies early postnatal renal US examination of all neonates; especially those who had abnormal antenatal ultrasonographic findings. Early diagnosis of such anomalies will lead to proper early management and prevention of chronic renal failure

Incidence of extrapulmonary congenital anomalies with neonatal respiratory distress at Sharkia governorate

Congenital anomalies are one of the leading causes of death in infancy. Not all the disorders producing neonatal respiratory distress are primarily diseases of the lung. So the aim of this work is to find out the spot incidence of extrapulmonary congenital anomalies among neonates with respiratory distress at Sharkia Governorate. For this purpose, all neonates who presented with respiratory distress during a period of one year [from January to December, 1998] were admitted to Neonatal Intensive Care Unit, Pediatric Department, Zagazig University Hospital and were the candidate of this study. Their number was 520 newborn infants, all of them were subjected to full clinical history, thorough clinical examination and the following investigations: complete blood count, blood glucose level, serum calcium level, serum electrolyte, arterial blood gases, chest X-ray and the following investigations were done when indicated, upper gastrointestinal tract [GIT] series with contrast, ultrasonography, CT and Echo- cardiography.The results of this study show that, thirty three out of the 520 neonates with respiratory distress [6.3%] had extrapulmonary congenital anomalies [group I] while the remaining 487 neonates [93.7%] had respiratory distress due to other causes [group II]. In group I, the GIT anomalies were the most common extrapulmonary congenital anomalies with respiratory distress 16/33 [48.50%], while in group II, septicemia with or without bronchopneumonia was the most common cause of neonatal respiratory distress 236/481 [48.5%]. As regards some clinical parameters, we have found that, group I was significantly more common with vaginal deliveries, full term neonates, good moro reflexes, low birth weight neonates, and consanguineous marriages. Whereas group II was significantly more common with caesarian section, premature neonates, poor moro reflexes, normal birth weight neonates and history of maternal drug intake extrapulmonary congenital anomalies are not uncommon causes of neonatal respiratory distress. The diagnosis of these anomalies depends primarily on a high index of suspicion, careful clinical evaluation and if required, investigations according to the suspected anomalies

Congenital anomalies in liveborn Saudi infants

The medical records of a total of 58,207 liveborn Saudi infants born in the three major hospitals in the Eastern Province were analysed for having congenital malformation at birth, over a period of five years. Abnormalities were detected in 579 infants, representing an overall incidence of 8.5 per 100 live births. Major anomalies were observed at birth in 448 newborns [6.6 per 1000]; 32.2% of these required surgical correction in the immediate postnatal period. Pattern of anomalies and their significance are presented and discussed

Cyclopia: a case study and review

A neonatal, human female cyclopic specimen, with brain exhibiting lobar holoprosencephaly was examined by detailed anatomical dissection. In addition to the pachygyric appearance of the cerebrum, the diencephalon failed to attain its normal position in relation to the cerebral hemispheres. The ethmoid bone was the focal point of skull malformations. Possible mechanisms for the generation of the malformations are discussed. Cyclopia is a monostrity that combines lack of separation of the eyes with lack of cleavage of the prosencephalon. The resulting undivided telencephion with a single ventricular cavity was called a holosphere[1] in contrast to the normal hemispheres with symmetrical lateral ventricles. The term holoprosencephaly, which is widely used now-a days to indicate impaired midline cleavage of the embryonic forebrain, is commonly associated with various gradations of facial dysmorphism including cyclopia, ethmocephaly and cebocephaly. According to its severity, holoprosencephaly has been graded into alobar, semilobar or lobar[2]. In cyclopia, a blind-ended proboscis usually occurs above the eye. In some instance, the proboscis may be absent[3]. In cyclopia, the anterior cranial base has been described as being shortened, roofing a medially placed orbital cavity. The ethmoid and the middle portion of the sphenoid are said to be absent. Also described as missing are the nasal bone, turbinates, vomer, lacrimal bones and premaxilla[4]. The present study examines in detail the gross anatomical abnormalities in a case of cyclopia. Special attention was made to the brain and eyes