Transitional health care for patients with Hirschsprung disease and anorectal malformations.

BACKGROUND: Hirschsprung disease (HD) and anorectal malformations (ARM) are congenital disorders with potentially lifelong consequences. Although follow-up is performed in most pediatric patients, transfer to adult health care is often problematic. This study assesses transitional care with the help of questionnaires in consultation with adult patients. METHODS: This study was conducted in an outpatient clinic of a pediatric surgical center in the Netherlands. All patients born and treated for ARM or HD before 1992 were invited to visit our clinic. Patients completed questionnaires concerning disease-specific functioning and quality of life at an initial visit to in response to which individual treatment plans were modified. Patients were reviewed 1 year later. RESULTS: Twenty-seven patients (17 ARM and 10 HD), mean age 27.9 years (range 17-64 years) of the 168 invited visited the transitional clinic (17%). Passive fecal incontinence was reported by 7/27, other defecatory problems, including urge incontinence and incomplete evacuation in 17/27 and anal or abdominal pain reported by 9/27. Quality of life was lower than a matched population. Only 13/27 returned for repeat assessment at 1 year; however, a further 8 reported that that their problems had resolved. In those attending follow-up, negative thoughts and feelings about their condition had decreased and one more patient was fully continent. There was no change in quality of life, bowel function or pain recorded. Twelve out of thirteen patients reported that they had found the transitional clinic satisfactory. CONCLUSIONS: The transitional outpatient clinic provides care adapted to the needs and wishes of adult HD and ARM patients. It is a novel addition to quality of care of patients with complex congenital disorders.

Bilateral proximal tibia fracture.

A bilateral fracture of the proximal tibia is rare in children. We describe a girl with a bilateral fracture just distal of the epiphyseal plate after minimal trauma.

Clinical and genetic characteristics of meconium ileus in newborns with and without cystic fibrosis.

The present study compares the clinical presentation and diagnostic features of meconium ileus (MI) in newborns with and without cystic fibrosis (CF). A retrospective study of 43 patients treated in the Pediatric Surgical Center of Amsterdam was performed. Twenty-three of the patients (53.5%) were diagnosed as having CF. Complex MI was significantly more frequent in patients without CF, and these patients had lower gestational ages and birth weights than patients with CF. All of the patients with complex MI had homozygous DF508 mutations, whereas the patients with simple MI also had other mutations. None of the patients with other mutations had complex MI. Therefore, we conclude that the clinical entity of MI represents a spectrum of underlying pathologies.

Progressive perinatal bowel obstruction--a rare cause of short-bowel syndrome.

A girl was born after an uneventful pregnancy of 36 weeks. Prenatally, distended bowel loops had been seen on ultrasound. Multiple small-bowel atresia was diagnosed and treated surgically. In the course of the next eleven weeks, previously patent segments of small bowel became obstructed. In 4 separate operative sessions, several segments of jejunum and ileum were resected, leaving 23 cm of ileum with the ileocecal valve in place. On microscopic examination of all resected material, necrosis of the mucosa was found consistent with ischemia. The child survived and tolerated full enteral feeding at the age of 8.5 months. The origin of the progressive obliterating process remains unknown.

[Femoral shaft fracture in children younger than 4 years: shorter hospital stays with the help of at home traction apparatus]. / Femurschachtfractuur bij kinderen jonger dan 4 jaar: kortere ziekenhuisopname met behulp van tractie thuis.

OBJECTIVE: Evaluation of home traction as a treatment as a treatment of femoral shaft fractures in children with the objective to shorten the hospital stay. DESIGN: Retrospective. SETTING: Paediatric Surgical Centre Amsterdam (EKZ/AMC and AZVU), the Netherlands. METHOD: In the period 1991-1995, 18 femoral shaft fractures in children younger then 4 years were treated. In ten of them traction was applied at home (in the other cases the parents refused to co-operate, the home situation was not appropriate, there were additional medical problems or there was a suspicion of child abuse). During follow-up of the group treated at home with traction, angulation, deformity and leg length discrepancy were determined with special attention to complications. The parents' experience of this method was evaluated by telephone (n = 8). RESULTS: The median age of the children was 2.4 years. The mean hospital stay was 7 days (range: 3-12), the mean follow-up 2.4 years (range: 1.0-4.3). Angulation, rotational deformities and leg length discrepancy > 1 cm did not occur. Oedema and pain were seen in 1 patient as a result of incorrect treatment at home. In one patient a compartment syndrome occurred after a switch from traction to a plaster treatment in another hospital. With exception of some small practical and informational problems, parents were very pleased with this method. CONCLUSION: Treatment at home of femoral shaft fractures in children with traction is a simple and effective method which reduces the hospital stay to one week with minimal complications. Good patient selection and instructions of the parents are mandatory.

Clear peritoneal effluent in a child on CCPD with a phlegmonous appendicitis.

Peritonitis as a result of a perorated appendicitis is a rare but life-threatening situation in a patient on peritoneal dialysis (PD). As far as we are aware, the combination of clear dialysate effluent and phlegmonous appendicitis in a patient on PD has not previously been described. We report a 16-year-old girl with acute onset of abdominal pain and vomiting who turned out to have phlegmonous appendicitis, despite having a clear dialysate effluent with normal cell count, and who subsequently developed E coli peritonitis after surgery.

Rupture of the distal tendon of the biceps brachii muscle.

Rupture of the distal tendon of the biceps brachii muscle is easy to diagnose. For functional and cosmetic reasons the tendon to the radial tuberosity should be re-inserted. Two patients had early treatment, and one was operated upon after four weeks, all three with a good result. After treatment consisted of immobilization in a plaster cast with the arm in flexion and full supination for four weeks. Physical therapy is recommended. Complete functional repair and full recovery of strength can be expected in most cases.

Spontaneous rupture of the oesophagus.

Spontaneous rupture of the oesophagus or Boerhaave syndrome is a rare phenomenon which poses a surgical challenge. Without treatment a mortality has been reported of up to 100 per cent. Early diagnosis and treatment are therefore imperative. An aggressive surgical approach is described. Three patients were treated with good results by closure of the rupture, drainage of the mediastinum, a diverting oesophagostomy, gastric drainage and postoperative enteral feeding via a jejunal catheter.

Epiphyseal fractures of the proximal tibia.

Fractures of the proximal tibial epiphysis and apophysis are rare. Data of ten patients seen in the last 17 yr with an epiphyseal (6) or apophyseal (4) fracture of the proximal tibia were reviewed. Three patients with an epiphyseal fracture were treated by open reduction and fixation, the other three by closed reduction. All apophyseal fractures were treated by open reduction and internal fixation. No major complications occurred. The final results were good.

Postoperative intussusception in children.

BACKGROUND: Postoperative intussusception is an uncommon and sometimes forgotten cause of postoperative intestinal obstruction in children. METHODS: Eleven consecutive cases of postoperative intussusception during a period of 16 years (1981-1997) were analysed retrospectively. RESULTS: Symptoms developed after a median period of 5 days after operation. With one exception, postoperative intussusception was not diagnosed before reoperation. In ten of the 11 cases the intussusception was successfully treated with intraoperative manual reduction. CONCLUSION: Postoperative intussusception is a rare but typical complication in the paediatric age group and should be kept in mind.

Giant cysts in diverticular disease of the sigmoid colon.

Two patients with a giant cyst of the sigmoid colon are presented. The literature is reviewed and the clinical features, differential diagnosis and treatment are discussed.