Risk factors, characteristics and outcomes of necrotizing enterocolitis in late preterm and term infants.

OBJECTIVE: To identify the risk factors, characteristics and outcomes of necrotizing enterocolitis (NEC) at our institution. STUDY DESIGN: A retrospective case-control analysis of the charts of all late preterm and term infants, who had NEC of Bell's stage ≥ II from 1995 to 2009, along with infants of the same gestational age. Thirty-two late preterm infants had NEC meeting criteria and 128 late preterm and term infants were chosen as matched controls. RESULTS: The 32 NEC infants were more likely to have the following characteristics: a culture-proven sepsis (p = 0.0001), be small for their gestational age (p = 0.003), have a congenital heart disease (p = 0.007), and suffer from hypoxic- ischemic encephalopathy (p = 0.04). The presence of hypotension, metabolic acidosis, thrombocytopenia, and pneumoperitoneum was associated with a poor prognosis. Twelve of the 13 (92%) NEC infants who died had a surgical intervention. CONCLUSION: In this study, late preterm and term infants who developed NEC had other underlying clinical diagnoses and had culture-proven sepsis. Mortality rate was high in infants who required surgical intervention, indicating that they were gravely ill from the onset. Thrombocytopenia, hypotension and metabolic acidosis in late preterm and term infant with NEC were associated with poor prognosis.

Extrahepatic biliary atresia in Saudi Arabia: the importance of early diagnosis and referral.

OBJECTIVE: A retrospective analysis of the medical records of children who were managed for extrahepatic biliary atresia (EHBA) over a 6-year period. MATERIALS AND METHODS: Twelve children were managed at King Fahad National Guard Hospital with a diagnosis of extrahepatic biliary atresia from January 1993 through December 1998. The data obtained included age, sex, clinical presentation, age at referral, investigations, management, complications and follow-up. RESULTS: There were 7 males and 5 females with age of onset of jaundice ranging from 3 days to 42 days (mean 10 days). The mean age at referral was 17 weeks (range 6-49 weeks). Kasai Portoenterostomy (PE) was performed in 7 infants and the mean age at surgery was 8 weeks (range 7 to 10.5 weeks). Four children could not undergo Kasai PE because they were more than 20 weeks old and 1 child although 10 weeks old had extensive liver cirrhosis and was considered not suitable for the procedure. One child who had primary liver transplant outside the Kingdom and 4 children who had Kasai PE are still alive. Seven children are lost to follow-up and are probably dead. CONCLUSION: The incidence of EHBA in Saudi Arabia is unknown and the disease is probably rare based on our review. Although jaundice, pale stools and dark urine were observed in early infancy, referral was always late. Public and health professional's awareness of the clinical features of EHBA is important in early identification of infants and early referral. Starting paediatric hepatobiliary centers in Saudi Arabia will improve the management of these cases.

Pediatric thoracic trauma.

OBJECTIVE: A retrospective analysis of the medical records of children up to 12 years of age inclusive, who sustained thoracic injuries during a 6-year period. METHODS: Ninety-one children were treated at King Fahad National Guard Hospital, Riyadh from January 1993 through December 1998. The clinical data included age, sex, mechanism of injury, associated injuries, pediatric trauma score, treatment and mortality. RESULTS: Eighty-seven children (96%) had injuries from blunt trauma and 4 from penetrating injuries. Of the blunt trauma cases, 82 children sustained motor vehicle accident related injuries, 62 as pedestrians and 20 as passengers. Penetrating thoracic injuries occurred in 4 children: 1 stab wound and 3 gunshots. The most frequent thoracic injuries were pulmonary contusion (70), pneumothorax (32), fractured rib (20) and fractured clavicle (18). Extrathoracic injuries included head (45), abdominal (41) and skeletal (26). Thoracotomy was required in only 1 child, laparotomy being necessary in 9 children for intraabdominal injuries. Tube thoracostomy was required in 33 children. Nine children died from motor vehicle accident related fatal head and neck injuries, 8 as pedestrians all with a pediatric trauma score < or = 6. CONCLUSION: Thoracic injuries in children below 12 years of age are usually from motor vehicle accident related blunt trauma. Pulmonary contusion and pneumothorax are the most common thoracic injuries. Most thoracic injuries can be managed either conservatively or by tube thoracostomy. Thoracic trauma in children is an indicator of multisystem injury with head injury being the most common cause of mortality.

Intussusception. A cause of postoperative intestinal obstruction in children.

Intestinal obstruction is a common postoperative complication and usually related to intra-abdominal adhesions. Postoperative intussusception, however, is a rare cause and may be confused with postoperative ileus. With more children undergoing abdominal operations, the incidence of postoperative intussusception should rise but reports indicate that this complication may be either not suspected or overlooked. We report 2 children who developed postoperative intussusception following repair of ruptured urinary bladder in one and appendicectomy in the other. Both had successful manual reduction at laparotomy.

Megacystis-microcolon-intestinal hypoperistalsis syndrome associated with megaesophagus.

Megacystis-microcolon-intestinal hypoperistalsis syndrome is a rare congenital disorder characterized by megacystis and hypoperistalsis of the gastrointestinal tract. About 80 cases have been reported, predominantly in females. We present a female newborn with typical features of the syndrome associated with megaesophagus.

Congenital diaphragmatic hernia: experience with preoperative stabilization and delayed surgery without ECMO and inhaled nitric oxide.

Despite many advances in the management of congenital diaphragmatic hernia (CDH), the condition carries a mortality rate of 40-50% usually consequent to pulmonary hypoplasia and/or persistent pulmonary hypertension. Several centers have reported improved survival with preoperative stabilization and delayed surgery, which is now an accepted method of management. This is a retrospective analysis of all neonates with respiratory distress at birth due to CDH who were treated at our institution with neither extracorporeal membrane oxygenation nor nitric oxide being used. The medical records of all neonates with CDH and respiratory distress at birth who were treated at this institution from August 1, 1992 through March 1, 1997 were reviewed. There were 21 patients, 11 male and 10 female. There were 17 full-term and 4 premature infants; two premature infants at 30 and 34 weeks' gestation were not resuscitated because of severe associated congenital anomalies. Surgery was performed from 5 to 144 hr (mean 45 hr) in 18 infants. One infant died during preoperative stabilization from severe pulmonary hypoplasia and pulmonary hypertension and one infant died postoperatively from the same conditions. Seventeen of 19 infants (89.5%) survived and were discharged home. Three infants (17.6%) who failed to thrive due to severe gastroesophageal reflux (GER) required fundoplication. Eleven infants (64.7%) who had sepsis proven by blood culture responded satisfactorily to appropriate antibiotics. Preoperative stabilization and delayed surgery has been a satisfactory form of management in our series. The significant complication was sepsis, which must be addressed.

Pelviureteric obstruction in children: conventional pyeloplasty is superior to endo-urology.

BACKGROUND: Hydronephrosis secondary to pelviureteric junction (PUJ) obstruction is common in infancy and childhood. Pyeloplasty has until recently been the accepted method of management, but alternative endo-urological techniques have evolved in the last decade. METHODS: Published results of conventional pyeloplasty for primary PUJ obstruction in children were compared with published results of endo-urological procedures. RESULTS: Sixty-six pyeloplasties were performed in 61 children in a 6-year period. During a similar period, 63 primary endo-urological procedures were reported in the literature. The success rate after pyeloplasty was 95.5% compared with 65% after endo-urology. CONCLUSIONS: Conventional pyeloplasty is superior to endo-urology and should remain the gold standard for the treatment of primary PUJ obstruction in children.

Patent omphalomesenteric duct of the vermiform appendix in a neonate: congenital appendicoumbilical fistula.

Umbilical anomalies arise from fetal structures such as the omphalomesenteric duct (OMD) or urachus or from failure of closure of the umbilical fascial ring. Persistence of the OMD may lead to several anomalies including umbilical sinus, umbilical cyst, Meckel's diverticulum, or patent OMD (POMD). A POMD is usually associated with the ileum, but rarely may be with the caecum or appendix. We describe a POMD of the vermiform appendix and discuss the possible pathogenesis and management.

Non-intubated pyeloplasty for pelviureteric junction obstruction in children.

Sixty-one consecutive patients undergoing pyeloplasty (5 bilateral) were reviewed retrospectively; 54 pyeloplasties were non-intubated (NIP) and 12 were intubated. NIPs were managed by an extrarenal wound drain, which was removed after 2-4 days in 44 repairs with minimal or no urinary leakage and after 6-8 days in 10 with significant leakage. Fifty-two were successful after the primary procedure. One patient who developed a urinoma after drain removal required a percutaneous nephrostomy followed by a revision pyeloplasty. A second revision pyeloplasty was necessary for persistent postoperative obstruction. The results of NIP compared favorably with series where intubation was used routinely and were superior to alternative methods of management such as endopyelotomy.

Hepatic foreign body in a child.

The majority of foreign bodies (FBs) swallowed by children are passed spontaneously without event. Perforation of the intestine with migration to the liver is rare. A child with a needle in the right lobe of the liver is reported.

Female bladder exstrophy.

Bladder exstrophy is one of the most challenging congenital urinary tract abnormalities. Apart from the open bladder the patient also has various other abnormalities, including urogenital, musculoskeletal and anorectal defects. The size of the exstrophic bladder varies from patient to patient. In the female the clitoris to bifid and the vagina is anteriorly placed. In this paper various aspects of female bladder exstrophy are reviewed in detail, including incidence, etiology, anatomy and clinical features, together with early, medium-term and long-term surgical management. The aim is to achieve a functional bladder closure, although some patients are better off with a urinary diversion. Considering the complexity of the urogenital problems and the surgical management thereof, all patients require lifelong follow-up.

Spontaneous closure of acquired tracheo-oesophageal fistula.

Acquired tracheo-oesophageal fistulae are uncommon in the paediatric age group. A case of such a fistula secondary to impaction of a button battery is reported below. Prompt management is essential to reduce morbidity and mortality in these cases.

Prenatal diagnosis of posterior urethral valves in siblings.

Posterior urethral valves are an important cause of obstructive uropathy in the newborn. The diagnosis may be suspected on prenatal ultrasonography, the consistent finding being bilateral ureterohydronephrosis together with a thick-walled, persistently full bladder in a male fetus. There may also be variable degrees of oligohydramnios. This paper reports the case of siblings in whom posterior urethral valves were recognized prenatally. The first child progressed well after appropriate surgical management, but the second died within 24 h of birth.

Acute hydrops of the gallbladder in childhood.

Acute hydrops of the gallbladder (AHGB) is a rare paediatric disease being diagnosed with increased frequency due to its association with other illnesses and the availability of ultrasonography. The symptoms and signs of AHGB include abdominal pain, vomiting, abdominal mass and/or tenderness. As these clinical features mimic the more common surgical conditions such as acute appendicitis, intussusception and volvulus, some cases are still diagnosed only at laparotomy. Diagnosis is established by ultrasonography of the abdomen demonstrating normal biliary ducts and a distended gallbladder without calculi or congenital malformation. The aetiology of acute hydrops of the gallbladder is unknown but may be multifactorial. Treatment varies from non-operative management to surgical intervention.

Haemangiomatous polyp of the posterior urethra in a boy.

Urethral haemangiomas are rare and usually involve the entire urethra or are localized to the anterior urethra. Posterior urethral polyps are uncommon. A posterior urethral polyp was removed from a 3-year-old boy which proved to be haemangioma. In the histological classification of posterior urethral polyps, haemangioma should therefore be included.