D. M. Burge

Management of antenatally diagnosed pulmonary sequestration associated with congenital cystic adenomatoid malformation

BACKGROUND Sequestration with associated cystic adenomatoid malformation is rare. A study was undertaken to determine whether pulmonary sequestration associated with congenital cystic adenomatoid malformation has a more favourable natural history than that of sequestration without associated cystic adenomatoid malformation. METHODS An outline of the postnatal work up leading to the management of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation diagnosed antenatally as pulmonary malformation is presented and the indications for surgical intervention are discussed. RESULTS In five infants in whom an antenatal ultrasound scan had detected a congenital lung malformation at 18–19 weeks gestation a final diagnosis of extralobar or intralobar pulmonary sequestration with congenital cystic adenomatoid malformation was made postnatally. Postnatal ultrasound and computerised axial tomographic scans confirmed the diagnosis of sequestration by delineating anomalous vascular supply. Cystic changes were also observed in the basal area of the sequestration in all patients. Four children remained asymptomatic and one infant presented at 10 months of age with pneumonia. The mean age at surgical resection was 6.8 months (range 2–10). Histopathological examination confirmed intralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in two patients and extralobar pulmonary sequestration with associated Stocker type 2 congenital cystic adenomatoid malformation in three patients. The mean period of follow up was four years (range 1–8). The children remain well and are developing normally. CONCLUSIONS The importance of seeking an anomalous blood supply in children with congenital lung lesions is emphasised. Pulmonary sequestration and congenital cystic adenomatoid malformation probably share a common embryogenesis despite diverse morphology. The natural history of antenatally diagnosed lung masses is variable. Early postnatal surgical resection of pulmonary sequestration with cystic adenomatoid malformation is recommended. Surgical excision should be conservative, sparing the normal lung parenchyma.

Reflux nephropathy secondary to intrauterine vesicoureteric reflux

In 107 infants with 182 antenatally diagnosed urinary tract anomalies, 24 had either unilateral (12) or bilateral (12) vesicoureteric reflux (VUR). The VUR was more common in boys (male to female ratio, 16:8) and usually severe (grades IV [16], III [10], II [4], and I [6]). Intravenous pyelography showed the changes of atrophic pyelonephritis in 10 refluxing units, and in another two with an associated pelviureteric junctional hydronephrosis. Lateral ectopia of the ureteric orifices was noted in six of these 10 refluxing renal units. Isotopic renography showed a reduction in function in nine of the 14 patients examined, ranging between 9% and 41%. (45% and above was considered within the normal range). Only two patients developed a urinary infection before intravenous pyelography or isotopic renography was performed, suggesting that renal changes noted were primary rather than secondary. Findings support the hypothesis that foetal VUR may be a contributing factor in the causation of atrophic pyelonephritis (foetal reflux nephropathy) observed in these patients.

Fetal Vesicoureteral Reflux: Outcome Following Conservative Postnatal Management

Of 222 infants with a urinary tract abnormality detected antenatally 30 male and 9 female patients (64 renal units) were found to have primary vesicoureteral reflux. Grade of reflux was predominantly severe, with grade III or higher noted in 83% of the patients. Prenatal and postnatal ultrasound failed to detect any abnormality in 29 refluxing units (45%) discovered contralateral to the known abnormal system, although 19 had grade III or higher reflux. Of the 64 refluxing units 8 underwent primary ureteral reimplantation, 12 were lost to followup and 44 were managed conservatively for a mean of 3.3 years. Reflux ceased in 61% of the cases, improved in 14% and remained unchanged in 23%. In only 1 unit did the grade of reflux increase. Documented urinary tract infection occurred in 6 of the 39 reflux patients. Dimercaptosuccinic acid renography performed in 21 infection-free patients demonstrated global reduction in renal parenchyma in 4 units, focal parenchymal defects in 3 and normal function in 14. Conservative postnatal management of fetal vesicoureteral reflux is justified. Global and focal parenchymal changes can occur in the kidneys of infants with reflux despite the absence of urinary tract infection.

The ascending testis : is late orchidopexy due to failure of screening or late ascent?

Abstract It is widely recommended that infant boys with undescended testes (UDT) should be referred for surgical opinion before the age of 18 months. To attempt to identify the reasons why the mean age at orchidopexy in our institution was as high as 5.5 years, a retrospective review of the screening history, examinations, and management of boys over the age of 3 years at the time of orchidopexy was undertaken by reference to community, general practitioner, and hospital records. In 36 children where hospital and community records giving information prior to referral were available, the UDT had previously been documented on at least one occasion as descended in 24 children or retractile in 10. In 1 child there was delayed referral, and in another, there was operative delay. It is concluded that there is either frequent failure of the Child Health Surveillance screening programme, or that late ascent of a testis previously sited in the scrotum is a common occurrence. We recommend further prospective studies to clarify this latter phenomenon.

Is interval appendicectomy justified after successful nonoperative treatment of an appendix mass in children? A systematic review

BACKGROUND/PURPOSE Interval appendicectomy (IA) is commonly performed after successful nonoperative treatment of appendix mass (AM); although, this approach has recently been challenged. We systematically reviewed the pediatric literature with regard to the justification for this practice. METHODS Using a defined search strategy, studies were identified and data were extracted independently by 2 reviewers. Incidences of recurrent appendicitis, complications after IA, and carcinoid tumor were estimated accounting for interstudy heterogeneity. Cost and length of stay of IA were analyzed. RESULTS Three studies (127 cases) reporting routine nonsurgical treatment were identified; all were retrospective. There was marked interstudy heterogeneity and variable follow-up. After successful nonoperative treatment of AM, the risk of recurrent appendicitis is 20.5% (95% confidence interval [CI], 14.3%-28.4%). The incidence of complications after IA (23 studies, n = 1247) is 3.4% (95% CI, 2.2-5.1), and the incidence of carcinoid tumor found at IA (15 studies, n = 955) is 0.9% (95% CI, 0.5-1.8). No reports compared costs. Mean length of stay for IA was 3 days (range, 1-30 days). CONCLUSIONS Prospective studies comparing routine IA with nonoperative treatment without IA in children are lacking. Available data suggest that 80% of children with AM may not need IA. A prospective study to evaluate the natural history of this condition compared with the morbidity and costs of IA is warranted.

Renal agenesis: An acquired condition?

Renal agenesis is a relatively common congenital anomaly, although its etiology is unknown. It is clear that some solitary kidneys are the result of postnatal involution of multicystic dysplastic kidneys. The authors present a series of nine neonates with abnormal prenatal renal ultrasound findings; subsequent postnatal investigations showed an absent kidney. Five children had a prenatal diagnosis of multicystic dysplastic kidney (MDK) and two of hydronephrosis. In five cases (56%) there was a contralateral renal unit anomaly. It appears that MDK, and occasionally hydronephrosis, can involute prenatally, producing the postnatal appearance of renal agenesis.

Meconium plug obstruction

We reviewed the final diagnosis and incidence of bowel pathology in neonates presenting with large bowel obstruction that was relieved by the passage of meconium plugs. A retrospective case-note review was undertaken of all patients with a discharge diagnosis of meconium plug syndrome (MPS), meconium ileus (MI), Hirschsprung’s disease (HD), or small left colon syndrome (SLCS) from January 1996 to April 2002. Of 21 patients with meconium plug obstruction, eight (38%) had HD, nine had MPS, four had SLCS, and none had MI. However, there was considerable clinical and radiological overlap between MPS and SLCS, suggesting that these terms are imprecise. We found a much higher incidence of HD in babies presenting with meconium plug obstruction than has previously been reported. Overlap between MPS and SLCS suggests that these are not specific diagnoses and that current terminology needs to be changed. All babies with meconium plug obstruction should have HD and cystic fibrosis (CF) excluded.

The effect of antenatal diagnosis on the management of small bowel atresia

The aim of this study was to determine whether babies with small bowel atresia (SBA) diagnosed antenatally followed a different course from those diagnosed postnatally. We reviewed the records of neonates admitted to a single institution between 1985 and 2000 with a diagnosis of SBA. Thirty-nine neonates presented with SBA, with antenatal diagnosis (AND) being made in 12 (31%). There was no difference between the AND and postnatal diagnosis (PND) groups in terms of gestational age at birth, but the AND group had a lower mean birth weight. The babies in the AND group were operated on more quickly than those in the PND group. Ten out of 12 (83%) AND patients required parenteral nutrition compared with 12 out of 27 (44%) in the PND group, and the AND group had a significantly longer mean length of stay and spent a longer time on parenteral nutrition than the PND group.

Gastric volvulus and associated gastro-oesophageal reflux.

Between 1984 and 1994, 10 neurologically normal children between 2 and 24 months were diagnosed as having gastric volvulus with associated gastro-oesophageal reflux (GOR). The common features at presentation were episodic colicky abdominal pain, non-bilious vomiting, upper abdominal distension, haematemesis, and failure to thrive. Anterior gastropexy and conservative management of GOR was curative.

Late-onset volvulus without malrotation in preterm infants

PURPOSE The aim of the study was to report a new observation of late-onset volvulus without malrotation (VWM) in preterm infants. METHODS The study used medical note review of infants with VWM identified at a single regional centre between 1996 and 2007. RESULTS Ten patients were identified. Group 1 includes 4 patients (gestation, 28-38 weeks; median, 32) who presented within 4 days of age (range, 1-4; median, 3). Group 2 includes 6 patients (gestation, 25-33 weeks; median, 27), who presented later (range, 22-57 days; median, 45). Characteristics of group 2 patients included recurrent episodes of abdominal distension and bile vomiting (6/6), long-term continuous positive airway pressure requirement (5/6), and sudden, severe deterioration with acute abdominal signs (6/6). Small bowel volvulus was found at laparotomy requiring resection (30%-70% of total small bowel) and either primary anastomosis (4) or stoma formation (2). All babies survived. CONCLUSIONS There appear to be 2 clinical groups with VWM-one presenting within the first few days of life and the other presenting after the first month of life associated with a specific clinical history. This latter group has not been described before.