Adults with corrected oesophageal atresia: is oesophageal function associated with complaints and/or quality of life?

The aim of this study was to evaluate oesophageal function after correction of oesophageal atresia in adults, and to investigate the association between complaints, oesophageal function and quality of life (QoL). Twenty-five adults were included who participated in previous follow-up studies, during which complaints of dysphagia and gastro-oesophageal reflux (GOR), results of upper gastrointestinal endoscopy, oesophageal biopsies and QoL had been collected. Manometry was performed in 20 patients, 24 h pH-measurements were performed in 21 patients. pH-values (sample time 5 s) were calculated using criteria of Johnson and DeMeester. Associations were tested with ANOVA and chi (2)-tests. Ten patients (48%) reported complaints of dysphagia, seven (33%) of GOR. The amplitude of oesophageal contractions was low (<15 mmHg) in four patients (20%). pH-measurements showed pathological reflux in three patients (14%). Patients reporting dysphagia more often had disturbed motility (P = 0.011), and lower scores on the domains "general health perceptions" (SF-36) (P = 0.026), "standardised physical component" (SF-36) (P = 0.013), and "physical well-being" (GIQLI) (0.047). No other associations were found. This study shows a high percentage of oesophageal motility disturbances and a moderate percentage of GOR after correction of oesophageal atresia. Patients reporting dysphagia, whom more often had disturbed motility, seemed to be affected by these symptoms in their QoL.

[Three children with pancreatic (pseudo)cysts as the cause of undefined abdominal pain]. / Drie kinderen met pancreas(pseudo)cysten als oorzaak van onbegrepen buikklachten.

Three children, two boys aged 5 years and one 2-year-old girl, who were referred because of abdominal pain of variable duration, were found to have cystic malformations that arose from the pancreas. In the first boy, a traumatic pseudocyst was found that eventually turned out to have been caused by child abuse. The second boy had pseudocysts complicating chronic pancreatitis of presumably hereditary origin. In the girl, a congenital pancreatic cyst was found. Pancreatic disease, although rare, should be considered in the differential diagnosis of abdominal pain in children.

[Diagnostic image (265). A toddler with progressive abdominal pain after a car accident]. / Diagnose in beeld (265). Een peuter met progressieve buikklachten na een auto-ongeval.

A 2.5-year-old boy who was caught in a car accident had a seatbelt-sign on his right lower abdomen and a jejunal perforation.

Late presentation of a duodenal web in a patient with situs inversus and apple peel jejunal atresia.

A 16-year-old girl presented with signs of proximal intestinal obstruction. In the neonatal period, surgical correction of an apple peel atresia had been performed, and she also had a situs inversus abdominalis. Revision of the anastomosis had been done when she was 3 years old. Contrast studies apparently again showed a stricture of the anastomosis, which was treated by stricturoplasty. Because of persistent obstruction, reexploration was done and revealed a duodenal membrane. Anastomotic strictures are very rare several years after the primary operation, so other causes of obstruction should be sought.

Oesophageal atresia in premature infants: an analysis of morbidity and mortality over a period of 20 years.

AIM: To determine the morbidity and mortality of premature infants born with oesophageal atresia (OA) and to evaluate historical changes in morbidity and mortality over time. METHODS: Retrospective analysis of morbidity and mortality of all patients admitted for OA, with or without tracheo-oesophageal fistula, between 1982 and 2002. RESULTS: The study group consisted of 197 consecutive patients, of whom 55 (28%) were premature and 21 (11%) very premature. Type A atresia was found more often in very premature and premature infants than in those born at term (p = 0.02). Type E atresia was not found in the premature group (p = 0.004). At least one associated congenital anomaly was also present in 121 patients (61%). Postoperative complications developed more often in very premature and premature infants than in those born at term (p < 0.001). Gastro-oesophageal reflux was diagnosed in 32/76 premature infants and in 41/121 term infants (p = 0.001). Mortality among very premature and premature infants was higher than among those born at term (p = 0.003). Withdrawal of treatment was the most frequent cause of death. CONCLUSION: Premature infants with OA have a higher morbidity and mortality than term infants with OA. The complications of prematurity contribute significantly to morbidity and mortality in premature infants with OA. There is no reason to refrain from the standard treatment of OA in premature infants with no severe associated congenital anomalies.

[Diagnostic image (36). Idiopathic scrotal edema]. / Diagnose in beeld (36). Idiopathisch scrotaal oedeem.

In a 5-year-old boy idiopathic scrotal edema was diagnosed, a rare self-limiting disease.

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.

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.

[Rupture of larynx or trachea resulting from injuries sustained at birth]. / Ruptuur van de larynx of de trachea als geboortetrauma.

Four newborn boys developed respiratory insufficiency and pneumothorax, pneumomediastinum or subcutaneous emphysema as the result of a laryngeal or tracheal rupture. These ruptures were due to birth injuries after difficult labour resulting from shoulder dystocia or a large lymphangioma and to a birth weight of at least 4500 g. The three children with shoulder dystocia also had a clavicular fracture, a Horner's syndrome, Erb paralysis or phrenic nerve paresis. Treatment consisted of surgical repair followed by a few days' intubation. The children with a shoulder dystocia recovered well, although in one of them a tracheal stenosis had to be resected a few months later. The child with the lymphangioma died from a bifurcation embolus. In newborns with respiratory insufficiency and pneumomediastinum or subcutaneous emphysema after a difficult delivery an emergency laryngotracheoscopy has to be performed to exclude rupture of larynx or trachea.

[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.

Congenital hemangiopericytoma: two case reports.

Congenital hemangiopericytoma is a rare tumor consisting of capillaries surrounded by pericytes. Only histologically can the tumor be distinguished from other vascular hamartomas. For a long time the congenital type was considered benign; however, metastases have recently been described. A histologic diagnosis of all soft-tissue sarcomas should be made. We describe two patients with congenital hemangiopericytomas in whom the diagnosis was not suspected until after histologic examination. Long-term follow-up is indicated.

Application of the Mitrofanoff principle in children with severe impairment of bladder function.

Treatment of children with severe impairment of bladder function requires a large-volume, low-pressure reservoir combined with a continent, easily catheterizable valve. The Mitrofanoff principle (MP) appears to meet these requirements. Between 1986 and 1993, the MP was applied in 15 children (4 girls) aged 4 to 14 years. The primary diagnosis was bladder exstrophy in 8 (2 girls), neuropathic bladder in 3 (2 girls), urethral valves in 2, and rhabdomyosarcoma (RMS) in 2. In 10 patients bladder augmentation with an intestinal patch was performed in addition to a Mitrofanoff procedure; in 5 a neobladder and continent appendicostomy were made. One boy with RMS died of distant metastases with a well-functioning appendicostomy and adequate renal function. At 2 to 9 years follow-up of the other 14 patients, 12 have a good result defined as: (1) adequate reservoir capacity; (2) continence; (3) normal renal function; and (4) no hydronephrosis. In 1 exstrophy patient with pre-existing impairment of renal function, further deterioration necessitated frequent catheterization and additional medical treatment. In 1 boy with fulgurated urethral valves, spontaneous micturition became subsequently possible, allowing closure of his appendicovesicostomy. Complications occurred in 10 patients, necessitating reintervention in 7. The MP in combination with the creation of an adequate reservoir gives good results in children with severe impairment of bladder function. Careful attention should be given to patient education regarding emptying of the reservoir. Long-term follow-up of renal function is mandatory.

[Green amniotic fluid as initial symptom of high intestinal obstruction in infants]. / Groen vruchtwater als eerste symptoom van hoge-darmobstructie bij het kind.

At the birth of two children the amniotic fluid was green colored. The Apgar scores were good. Because of bilious vomiting and food retention, respectively, an open stomach tube was inserted, out of which bilious stomach contains were drained. The cause of green amniotic fluid was not meconium production or infection with Listeria monocytogenes, but mixing with green bile. At further investigation the children both proved to have a high intestinal obstruction distal of the papilla duodeni major.

Congenital tracheoesophageal fistula: preventing recurrence.

Recurrent tracheoesophageal fistula is a major complication of surgical therapy among children with congenital esophageal atresia and tracheoesophageal fistula. In a consecutive series of 153 patients operated on during a 20-year period in the same institution, only one patient had this complication. The authors believe that adherence to sound surgical principles can lower the risk of fistula recurrence considerably.

Tracheal left mainstem bronchus and obstructive gastric mucosal intussusception associated with esophageal atresia.

A tracheal left mainstem bronchus and gastric outlet obstruction owing to gastric mucosal intussusception occurred in a child who had esophageal atresia and tracheoesophageal fistula. Bronchography and bronchoscopy, indicated because of persisting atelectasis and ventilation dependency, showed a tracheal left mainstem bronchus. The atelectasis disappeared after reimplantation of the left mainstem bronchus into the carina. Feeding problems and recurrent pulmonary infections complicated the postoperative course. Radiographic imaging and esophagogastroscopy showed severe reflux esophagitis and a prepyloric mass that consisted of a gastric mucosal intussusception. Subsequent gastrotomy and resection of the intussuscepted mucosal fold relieved the gastric outlet obstruction. An antireflux procedure was performed simultaneously. The child recovered satisfactorily. It is suggested that gastric mucosal intussusception may be associated with esophageal atresia rather than with the presence of a gastrostomy tube, as has been proposed in the literature.

[Cholecystitis in infants]. / Cholecystitis bij zuigelingen.

In two infants presenting with non-specific abdominal symptoms, three months and four weeks of age, respectively, cholecystitis was suspected on ultrasound examination of the abdomen and confirmed and treated by surgery. In the first patient the cholecystitis was secondary to cholelithiasis. The second patient was shown to have an infected gallbladder due to an anatomical anomaly of the cystic duct. It is concluded that even in young infants, cholecystitis as a complication of congenital or neonatal pathology may underlie abdominal symptoms. Ultrasonography of the abdomen is an important diagnostic aid and cholecystectomy is the treatment of choice.

Long-term anorectal function after Duhamel operation for Hirschsprung's disease.

Long-term anorectal function was assessed in children operated on using Duhamel's technique for Hirschsprung's disease. The files of 75 patients (16 girls and 59 boys) operated on between 1977 and 1991 were reviewed. Questionnaires were analysed on 63 (12 girls and 51 boys) over 2 years of age. The median age at completing the questionnaire was 6.6 years. Forty-one of these 63 patients had aganglionosis of the rectosigmoid, 15 of a long colonic segment, and 7 of the total colon. Of 14 children age 4 or less, 6 had severe constipation, whereas 8 had regular spontaneous defaecation. Ten of the 49 over 4 years of age were continent without constipation, 22 had soiling and/or constipation, and 17 were incontinent, one of whom had a permanent colostomy. There was no correlation between age or sex and anorectal function. Anorectal function in children with long segment was not worse than in those with rectosigmoid aganglionosis, but only 1 of 7 with total colonic aganglionosis was continent. Because many adapt themselves to the handicap, symptoms are often underreported. A detailed questionnaire appears to be a reliable tool for elucidating the real situation. A systematic follow-up of patients with Hirschsprung's disease is proposed to anticipate the complications and to institute proper measures at an early stage.

Reduced perfusion and uptake of Tc-99m HDP in the iliac bone. A rare presentation of pelvic osteomyelitis.

The authors present a case report of a young patient with two osteomyelitic foci. Bone scintigraphy showed a rare combination of a cold and a hot spot. Possible problems in the interpretation of the scintigram in patients with pelvic osteomyelitis are discussed.

Risks and benefits of antireflux operations in neurologically impaired children.

Gastro-oesophageal reflux (GER) in neurologically impaired children often causes feeding problems and complications of oesophagitis and is frequently resistant to medical treatment. Fifty neurologically impaired children underwent anterior gastropexy as anti-reflux operation, combined with gastrostomy in 23, between 1976 and 1992. There was no operative mortality. There were 25 early complications in 14 patients and 9 late complications in 9 patients. Twelve patients needed 17 re-operations for delayed gastric emptying [4], intestinal obstruction [3], para-oesophageal hernia [3], oesophageal stenosis [4], and recurrent GER, revision of gastrostomy, subphrenic abscess (one each). Nine patients died during the follow up period. Death in two children was related to the operation (incarcerated para-oesophageal hernia and blow-out of the stomach). Out of 41 survivors, the operation was judged successful in 35. It is concluded that antireflux operations in neurologically impaired children carry a high risk of complications. Preoperative identification of risk factors is not possible. The improvements in the quality of life achieved in the majority of patients outweigh the risks.