Management of urethrocutaneous fistula following hypospadias repair.

Urethrocutaneous fistulas complicating hypospadias repair appear a common problem. There appears less data in the literature regarding the risk and management of recurrent fistulas. A retrospective review of urethrocutaneous fistulas complicating hypospadias repair was performed to evaluate their aetiology, management and outcome. Between 1993 and 2003, 1,753 patients had a hypospadias repair at our institution. Overall 123 (7%) boys developed a fistula, although detailed information was available on 117 patients only. Median age was 3.5 years at the time of fistula repair; 13% had anterior, 57% had middle and 30% had posterior hypospadias. The most common primary surgical procedure was a Durham Smith two-stage repair in 29% (n = 34), followed by a Tubularised Incised Plate urethroplasty in 19% (n = 22) and an Onlay in 14% (n = 16). Thirty-one (27%) patients developed a recurrent fistula, 9 (29%) of which recurred following a second repair. None recurred after a third repair. The risk of a recurrent fistula after an initial distal fistula repair was 12.5% and after a posterior fistula was 62% (chi(2) = 15.4, P = 0.001). Use of a stent, suture type, numbers of fistula and closure attempts did not influence the recurrence rate. Undiagnosed distal obstruction was thought to be related to 27 of 117 first fistula repairs (23%) and 4 of 31 second fistula repairs (13%). The risk of recurrent urethrocutaneous fistula was increased in those boys with a posterior fistula, following a simple repair or when there was evidence of distal urethral obstruction.

Evaluation of the NK2 homeobox 1 gene (NKX2-1) as a Hirschsprung's disease locus.

Hirschsprung's disease (HSCR, colonic aganglionosis) is an oligogenic entity that usually requires mutations in RET and other interacting loci. Decreased levels of RET expression may lead to the manifestation of HSCR. We previously showed that RET transcription was decreased due to alteration of the NKX2-1 binding site by two HSCR-associated RET promoter single nucleotide polymorphisms (SNPs). This prompted us to investigate whether DNA alterations in NKX2-1 could play a role in HSCR by affecting the RET-regulatory properties of the NKX2-1 protein. Our initial study on 86 Chinese HSCR patients revealed a Gly322Ser amino acid substitution in the NKX2-1 protein. In this study, we have examined 102 additional Chinese and 70 Caucasian patients and 194 Chinese and 60 Caucasian unselected, unrelated, subjects as controls. The relevance of the DNA changes detected in NKX2-1 by direct sequencing were evaluated using bioinformatics, reporter and binding-assays, mouse neurosphere culture, immunohistochemistry and immunofluorescence techniques. Met3Leu and Pro48Pro were identified in 2 Caucasian and 1 Chinese patients respectively. In vitro analysis showed that Met3Leu reduced the activity of the RET promoter by 100% in the presence of the wild-type or HSCR-associated RET promoter SNP alleles. The apparent binding affinity of the NKX2-1 mutated protein was not decreased. The Met3Leu mutation may affect the interaction of NKX2-1 with its protein partners. The absence of NKX2-1 expression in mouse but not in human gut suggests that the role of NKX2-1 in gut development differs between the two species. NKX2-1 mutations could contribute to HSCR by affecting RET expression through defective interactions with other transcription factors.

Diagnostic accuracy of surgeon-performed focused abdominal sonography (FAST) in blunt paediatric trauma.

AIM: To study the diagnostic accuracy and clinical efficacy of surgeon-performed focused abdominal sonography (FAST) in paediatric blunt abdominal trauma (BAT). MATERIALS AND METHOD: This was a prospective, single blinded study conducted at The Children's Hospital at Westmead Sydney (CHW). All patients with BAT that justified a trauma call activated on presentation to the Emergency Department (ED) had a FAST performed by the Trauma Fellow. The attending surgical team was blinded to the result of the FAST. An independent radiologist reviewed the FAST pictures, and the findings were compared with computerised tomography (CT), ultrasound (US), laparotomy and the clinical outcome of the patient. Sensitivity, specificity and predictive values were calculated. RESULTS: A total of 85 patients (39 M; 26 F) were enrolled in the study between February 2002 and January 2003. The age ranged between 4 months and 16 years. The mean Injury Severity Score (ISS) was 6 (range 1-38). FAST was performed in a mean time of 3 min. Inter-rater agreement was 96%. FAST was positive in nine as confirmed by a CT scan of the abdomen. Three patients underwent laparotomy, two for bowel injuries and one for a Grade III liver laceration. Of the remaining 76, 19 had a CT, which showed evidence of intra-abdominal injury in seven patients. There were two false negative studies resulting in a sensitivity of 81%, specificity of 100%, negative predictive value of 97%, positive predictive value of 100% and an accuracy of 97%. CONCLUSIONS: Surgeon-performed FAST for BAT was safe and accurate with a high specificity. It would seem a potentially valuable tool in the evaluation of paediatric blunt trauma victims for free fluid within the peritoneal cavity.

Blunt traumatic diaphragmatic injuries in children.

Diaphragmatic injuries following blunt trauma are rare. From January 1988 to February 2002 eight children were treated at the Children's Hospital at Westmead for diaphragmatic injury. Male to female ration was 5:3. Motor vehicle crashes were the most common cause. The injury was left-sided in four, right sided in three and central in one. Initial plain radiograph and computerised tomography detected the injury in 50% of cases. Laparotomy, contrast study and autopsy identified the rupture in one each. Associated injuries were present in all cases. Seven children had laparotomy and repair of the diaphragmatic rupture. The commonest site of rupture was posterolateral (37.5%). Diagnosis was delayed in two cases. There were two deaths (25% mortality) in the series, both due to associated injuries. Although rare, diaphragmatic rupture must be considered in any child with thoracoabdominal injury. Diagnosis may be difficult and require extensive investigation. Mortality usually results from associated injuries.

Pancreatic trauma in children.

BACKGROUND/PURPOSE: The aim of this study was to determine the etiology, associated injuries, and outcome of children with pancreatic injuries. METHODS: A retrospective review was conducted of children under 16 years with pancreatic trauma admitted to the Children's Hospital at Westmead (CHW) from January 1983 to September 2002. Deaths were reported to the New South Wales Paediatric Trauma Death Registry (State Registry) from January 1988 to September 2002. RESULTS: Sixty-five cases were identified: 46 patients were admitted to CHW, and 22 deaths were reported to the State Registry (including 3 deaths at CHW). The median age was 6 years (range, 1 to 14 years). Boys accounted for 60% (n = 40) of cases, decreasing to 50% (n = 11) of those that died. Motor vehicle injuries (MVI) were the most common mechanism, accounting for 40% of survivors and 77% of those who died. Children were restrained incorrectly in 48% of all cases and in 67% of those who died. Significantly more children who died had head, chest, and abdominal visceral injuries, compared with those who survived. Death occurred as a result of head injuries in 68%, with only 3 deaths directly attributed to pancreatico-duodenal injuries. CONCLUSIONS: Pancreatic injuries remain uncommon in children. The most frequent mechanism was MVI, with incorrect use of passenger restraints an important contributing factor. Whereas mortality was mainly caused by other injuries, 3 deaths were directly attributable to pancreato-duodenal trauma.

Children under 5 years presenting to paediatricians with near-drowning.

OBJECTIVE: To characterize children aged under 5 years who present to paediatricians following near-drowning and the circumstances surrounding the event, identify high-risk groups and document short-term outcome. METHODOLOGY: Monthly notifications to the Australian Paediatric Surveillance Unit (an active, national surveillance system) between 1994 and 1996. Collection of additional case information from reporting doctors by postal questionnaire. RESULTS: All 169 reported cases of near-drowning were admitted to hospital (mean (SD) stay 6 (17) days) and 15% required intensive care (mean (SD) stay 19 (32) days). The mean (SD) age for near drowning was 26 (13) months and 22% children were aged between 12 and 18 months. Males predominated (1.6:1) and 69 (41%) of episodes occurred in summer (December - February). The majority (82%) of near-drownings occurred in the child's home, usually in a swimming pool or bath. Children who nearly drowned at home were significantly younger than those who nearly drowned in natural waterways or public pools. Neurological damage at discharge following near-drowning was reported in 7%. CONCLUSIONS: Children reported in this national case series represent the severe end of the spectrum of those who nearly drown, as indicated by their presentation to a paediatrician, universal hospitalization and adverse neurological outcome. The home is the site of most near-drownings and males and toddlers were at particular risk. Unimpeded access to pools and lack of supervision were identified as potentially modifiable factors for prevention. The study suggests the need for additional community education regarding the risks of near-drowning and for further research on long-term neuro-developmental outcomes following near-drowning.

Duodenal atresia with vascular rings and subglottic stenosis.

This case report of duodenal atresia associated with a vascular ring and subglottic stenosis raises some interesting issues of management.

Penetrating injuries in children: is there a message?

OBJECTIVES: To determine the frequency, management and outcome of penetrating trauma in children. METHODS: A retrospective review of penetrating injuries in children under 16 years of age admitted to the Children's Hospital at Westmead (CHW), and deaths reported to the New South Wales Paediatric Trauma Death (NPTD) Registry, from January 1988 to December 2000. Patient details, circumstances of trauma, injuries identified, management and outcome were recorded. RESULTS: Thirty-four children were admitted to the CHW with penetrating injuries during the 13-year period. This represented 0.2% of all trauma admissions, but 3% of those children with major trauma. The injury typically involved a male, school-age child that fell onto a sharp object or was assaulted with a knife or firearm by a parent or person known to them. Twenty-five children (75%) required operative intervention for their injuries and 14 survivors (42%) suffered long-term morbidity. Thirty children were reported to the NPTD Registry over the same interval, accounting for 2.3% of all trauma deaths in New South Wales. Of these, a significant minority was injured by falls from a mower or a tractor towing a machine with blades. CONCLUSIONS: Penetrating injuries are uncommon, but cause serious injury in children. There are two clear groups: (i) those dead at the scene or moribund on arrival, in whom prevention must be the main aim; and (ii) those with stable vital signs. Penetrating wounds should be explored in the operating theatre to exclude major injury. Young children should not ride on mowers or tractors.

Congenital lobar emphysema: Like father, like son.

Congenital lobar emphysema (CLE) is an uncommon cause of progressive respiratory distress that typically presents in the first few days of life. There has been a previous report of CLE in a mother and daughter. The authors describe 2 cases involving the right upper and middle lobes in a father and son secondary to relative deficiency of the bronchial cartilage. This provides additional evidence for inherited factors in the etiology of CLE.

Isolated and multisystem hepatic trauma in children: the true role of non-operative management.

We retrospectively reviewed children with hepatic injuries (HI) admitted to our institutions from January 1982 to December 1999. Specific comparison was made of isolated (IHT) and multisystem hepatic trauma (MHT). Over the 18-year period 127 patients were identified with HI, 91 (71%) with MHT and 36 with IHT. The median age was 8 years (range 13 months to 14 years). Motor vehicle injury was the most common mechanism in both groups, but bicycle injuries were more common in IHT ( P<0.001). Shock ( P=0.02), the requirement for blood transfusion ( P<0.001), and operative intervention for the HI ( P=0.001) were all significantly more common in MHT. The distribution of liver injury grades was similar between the two groups. Twenty-two (17.3%) children died and in 18 the HI was the main cause or contributed significantly. There were 19 and 3 deaths in the MHT and IHT groups, respectively, a difference that was not significant. After excluding children with minor HI, 27 (39%) required operative intervention within 24 h of their injury. This suggests that the high success rate for non-operative management of HIs in the literature may have been biased by the inclusion of a significant proportion of subclinical injuries.

Laser Doppler imaging prediction of burn wound outcome in children.

The ability of laser Doppler imaging (LDI) to evaluate burn depth in children was investigated. Fifty-seven patients were prospectively studied over a 10-month period. Each patient was clinically assessed, photographed and independently scanned between 36 and 72 h of the burn. Patients were reviewed until wound healing had occurred within 12 days or skin grafting had been performed. The median age was 1 year and 10 months (range 5 months to 15 years and 8 months). The median body surface area burnt was 7.0% (range 0.5-30%). In 30 patients, the burn did not heal within 12 days, 17 of which were grafted. Clinical examination correctly determined 66% of deep partial or full thickness burns between 36 and 72 h of injury compared to 90% using LDI. The LDI was also more specific; correctly diagnosing 96% of superficial partial thickness burns as opposed to 71% on clinical examination. Moderate degrees of movement did not appear to limit the accuracy of the scan.

Gastroschisis reduction under analgesia in the neonatal unit.

PURPOSE: The authors present their experience of reduction of gastroschisis in infants in the ward with analgesia and without anesthesia. METHODS: Prospective data were collected on 35 infants born with gastroschisis from 1999 to 2001. Ward reduction (WR) was not attempted in infants with obvious gut atresia, perforated or ischemic bowel, or systemic instability. RESULTS: Ward reduction was attempted in 29 of the 35 infants and was successful in 25. There were no deaths or major morbidity in those that underwent ward reduction. Ventilation was required in 2, and line sepsis occurred in 4. Umbilical hernia was seen in 7; all but 1 were treated conservatively. CONCLUSIONS: In gastroschisis, ward reduction under analgesia is safe if strict selection criteria are adhered to. Postreduction ventilation is avoided in the majority.

Noncervicofacial atypical mycobacterial lymphadenitis in childhood.

BACKGROUND/PURPOSE: Atypical mycobacterial (AM) lymphadenitis is common in children but rarely occurs outside the cervicofacial region. The authors report their experience in the diagnosis and management of noncervicofacial AM lymphadenitis. METHODS: A retrospective review was conducted of cases diagnosed at our institution between January 1976 and December 1999, based on positive culture of atypical mycobacteria or consistent histology with supportive skin testing. RESULTS: Thirty-seven patients were identified over the 23-year review period. The median age was 4.3 years (range, 8 months to 13 years and 5 months), with 19 boys and 17 girls. The median duration of symptoms was 4 weeks, and the most commonly affected sites were the inguinal region (n = 17), axilla (n = 8), and lower limb (n = 6). Preceding local trauma was described in 10 patients and a viral illness in 4. Laboratory culture for atypical mycobacteria was positive in 22, and skin testing suggestive in 21 and equivocal in 2. Treatment was by excision in 28 and drainage with or without curettage in 9. At a median follow-up of 19.7 months, disease had recurred in 4 patients, none of whom had been treated initially by excision. CONCLUSIONS: Atypical mycobacterial infection is an uncommon cause of noncervicofacial lymphadenitis in children. It typically presents with a 4-week history of painless regional lymphadenopathy that may follow penetrating trauma. If untreated, the overlying skin becomes involved with a violaceous discoloration, and ulceration may occur. Definitive treatment involves complete surgical excision, preferably before suppuration extending beyond the involved lymph nodes.

HOSE: an objective scoring system for evaluating the results of hypospadias surgery.

OBJECTIVE: To determine the accuracy and utility of a scoring system designed to allow an objective appraisal of the outcome of hypospadias repair, based on evaluating meatal location, meatal shape, urinary stream, straightness of erection, and the presence and complexity of any complicating urethral fistula. PATIENTS AND METHODS: Twenty patients (median age 23 months) were randomly selected and reviewed at a median of 8.9 months after their hypospadias repair. Two paediatric surgeons, a nurse and one of the child's parents independently assessed each patient using the "hypospadias objective scoring evaluation" (HOSE) system. The results were collated and the level of interobserver variation assessed using the weighted kappa test. RESULTS: The mean weighted kappa was 0.66, indicating good agreement among observers. The level of agreement was highest between surgeon and nurse at 0.70, but remained good between surgeon and parent, at 0.65. CONCLUSIONS: Interobserver variation using the HOSE system was minimal, supporting its use as an objective outcome measure after hypospadias surgery, and facilitating an impartial evaluation of operations used in correcting hypospadias.

Facial fractures in children.

OBJECTIVE: To determine the pattern of injury of facial fractures in children, the relative contribution of plain radiography and CT scanning in the diagnosis of these injuries, and factors leading to delayed diagnosis. DESIGN: Retrospective case note review. PARTICIPANTS: All children with facial fractures identified using the trauma and medical record databases at our institution. RESULTS: Forty-six children with 59 facial fractures presented over a 4-year, 2-month period from November 1995 to December 1999. The median age was 10 years, with a range from 1 to 14. There was a 2-to-1 male-to-female sex ratio. A motor vehicle accident (MVA) involving a child as passenger, pedestrian, or cyclist accounted for 63% of cases. In seven of these, the child was either a front seat passenger or inappropriately restrained for their age and size. In all but one case, the presence of a fracture was associated with an overlying laceration, abrasion, or significant soft tissue edema. Initial examination and plain radiologic assessment by a pediatric clinician led to diagnostic delay in nine children. Facial CT was performed in 38 children, and all results were positive. Twenty-six patients required operative intervention for their facial fracture. Associated injuries, particularly of the head and limbs, were present in all but six patients. CONCLUSIONS: Facial fractures were uncommon overall but occurred more frequently in children with major trauma. Plain facial radiographs provided limited additional diagnostic information to careful clinical examination and often fail to detect or clearly define a facial fracture in children. In the correct clinical setting, a facial CT scan allows accurate diagnosis of the injury and can reveal previously unsuspected additional fractures.

Mechanisms for the development of esophageal atresia.

BACKGROUND: There is no universally accepted theory to explain esophageal embryology and the abnormal development that produces esophageal atresia. METHODS: The impact of Adriamycin administration on the pathogenesis of esophageal atresia was studied in the rat model of VATER association, from embryonic day (ED) 10 to ED 13. RESULTS: Tissues in the ED10 Adriamycin-exposed embryos displayed less cell proliferation as shown by the reduced population of MIB-5-labelled cells. Cell apoptosis that is characteristic of the normal ED 12 lateral epithelial folds of the foregut (the prospective site of tracheoesophageal septation) was absent in the foregut of the Adriamycin-exposed embryo. Histologic examination of the ED 11-exposed embryo showed the presence of abnormal notochord that was stretched, split, or tethered to the foregut. This contrasts with the normal embryo in which the notochord was localized in close vicinity of the ventral part of the neural tube and separated from the foregut by ample amount of mesenchyme. The abnormal localization of the notochord was accompanied by the lack of down-regulation of the sonic hedgehog (Shh) activity in the prospective site of future tracheoesophageal separation in the exposed ED 12 embryo. CONCLUSION: The authors proposed that the ectopic location of the notochord leads to the disruption in Shh signalling that may underpin the development of esophageal atresia.

Urethral trauma in children.

We report our 12-year experience in the management of urethral injuries in nine children, six boys and three girls. The most common mechanisms of injury were motor vehicle accidents, followed by straddle injuries. All the injuries in boys involved the anterior urethra, and in girls the proximal or mid-urethra. There were associated injuries in five, including three pelvic fractures. All children were investigated with a retrograde urethrogram. Four were treated non-operatively with insertion of a urethral catheter. Of the remaining five, one had drainage of a penile haematoma, one cystourethroscopy, two insertion of urinary and suprapubic catheters, and one open cystotomy and passage of a guide wire with antegrade passage of a urethral catheter. Complications included one urinary tract infection, one urethral fistula, one urethrovaginal fistula, and two urethral strictures. Final outcome was satisfactory in all nine children.