Biomedical subjects

P Sacher

Publications and source records attributed to P Sacher.

At least 19 recordsLinked to original sources

PubMed2006

Clinically relevant tracheomalacia after repair of esophageal atresia: the role of minimal intra-operative dissection and timing for aortopexy.

BACKGROUND: Tracheomalacia is associated with esophageal atresia (EA), but may go unnoticed from external splinting forces. Intra-operative dissection with fistula division releases external splinting, revealing tracheomalacia only post-operatively. Analysis of surgical technique may disclose an iatrogenic etiology. METHODS: From 1995 - 2004, 44 neonates underwent surgery for EA. All patients underwent pre-, intra- and postoperative bronchoscopy. Operative and bronchoscopic notes were studied for malacia, and extensive dissection of the esophagus and fistula from the trachea. RESULTS: Surgical mortality was 6.8 %. Pre-operative tracheomalacia was diagnosed in 3 patients, who eventually fared well. In 17 other patients, the pre-operative bronchoscopy was negative, but airway obstruction developed post-operatively. Tracheomalacia was documented at the site of the former fistula and surgical release maneuvers. Aortopexy was required in 5 instances, whereas 12 others with malacia were managed conservatively. CONCLUSIONS: Mediastinal connective tissue and the fistula may splint open the marginally diseased airway in patients with EA, the lack of which may disclose previously unknown tracheomalacia after repair. When aggressive release maneuvers have been required, early aortopexy may be preferred to ventilator dependency.

Airway Obstruction↗

PubMed2002

Intestinal pseudoobstruction as a paraneoplastic syndrome in ganglioneuroblastoma.

Intestinal pseudoobstruction may be part of a paraneoplastic syndrome. We report a teenage girl with ganglioneuroblastoma who presented with severe constipation. The intestinal pseudoobstruction was presumed to be due to inflammation of the myenteric plexus with destruction of the ganglion cells caused by antineuronal nuclear antibodies (ANNA or Anti-Hu).

Adolescent↗

PubMed2001

Infantile systemic hyalinosis in siblings: clinical report, biochemical and ultrastructural findings, and review of the literature.

A boy presented at age 3.5 months with joint contractures, restlessness, and pain on handling. His skin was thickened and there were livid-red macular lesions over bony prominences. Infantile systemic hyalinosis (ISH) was diagnosed, a presumably autosomal recessive, progressive, and painful disorder of as yet unknown pathogenesis. Observation over three years confirmed the diagnosis as typical changes, such as nodules on both ears, pearly papules in the perinasal folds and on the neck, fleshy nodules in the perianal region, and gingival hypertrophy, developed. Skin lesions and painful joint contractures progressed in spite of intense physiotherapy, and at age 3, the child had marked motor disability. The central nervous system (CNS) appeared to be intact and the infant showed normal mental development. Radiologic findings included marked generalized osteopenia, osteolytic erosions in the metaphyses of the long bones, and cortical thinning. Electron microscopy of two skin biopsies demonstrated deposition of floccular amorphous substance that was abundant around, and appeared to originate from, small blood vessels in the dermis, partially interfering with collagen fiber formation. Lysosomal inclusions were not seen. Serum acid hyaluronidase activity was within the normal range, and the synthesis of hyaluronic acid and proteoglycans in cultured skin fibroblasts was similar to that of control cells. A younger sister presented at age two months with painful joint contractures and discrete livid-red macules over both malleoli, and showed a similar progression of the disorder over the first year of life. The diagnosis of ISH should be considered in infants and children presenting with painful joint contractures and skin lesions. The pathogenesis of this disabling and disfiguring disorder remains unclear. Our data confirm probable autosomal recessive inheritance, and do not support lysosomal storage, hyaluronidase deficiency, or a primary collagen disorder, but indicate that the amorphous material accumulating in the skin and articular soft tissues may originate from the blood circulation.

Cells, Cultured↗

PubMed2001

[Postappendectomy abscess--the role of fecoliths].

Intraabdominal abscess formation is the most common septical complication after perforated appendicitis. An appendiceal fecalith favours appendicitis and is a high risk for complications like perforation or abscess formation. In a 6 year period 554 patients have been treated operatively for appendicitis in our institution. In 4 patients, 6 to 12 1/2 years, operated for perforated appendicitis, a fecalith was initially overlooked. This unrecognised fecalith at operation led to an intraabdominal abscess 14 days up to 17 months postoperatively. After laparatomy with extraction of the fecalith, drainage of the abscess and antibiotical therapy all patients attained definitive healing. An appendicolith can escape into the abdominal cavity before or during appendectomy, causing complications. An unrecognised fecalith is a rare cause for intraabdominal abscess formation. Nevertheless in patients with an intraabdominal abscess after appendectomy a thorough radiological search for a fecalith by means of plain abdominal radiograph and abdominal ultrasound is mandatory. Surgical removal of the intraabdominal fecalith is the only way to definitive healing.

Abdominal Abscess↗

PubMed2001

[Abscesses after appendectomy due to intraoperative retaining of fecalith].

OBJECTIVE: Fecaliths appear to predispose a patient to acute appendicitis, abscess formation, and perforation. The number of preoperative radiological evaluations is increasing, although children with suspected perforation still undergo surgery immediately. We report and discuss imaging findings and implications in children with acute appendicitis and fecaliths. METHODS: Four children (3 girls, 1 boy; mean age 9 years) underwent surgery for acute appendicitis. Three children underwent sonography and plain radiography before surgery, 1 child was operated without radiological evaluation. After readmission, all 4 children underwent sonography and plain radiography before surgery. RESULTS: In 3 patients a fecalith was diagnosed initially. After uneventful recovery all 4 patients had acute abdominal pain and readmission was necessary. In all 4 patients the escaped fecalith was demonstrated with sonography and plain radiography confirmed surgically. CONCLUSION: Discussion about the role of imaging in acute appendicities has concentrated on the diagnostic yield of cross-section techniques. The importance of demonstrating a fecalith, prompting a more thorough intraoperative search has found little attention. The radiologist should also detect and localize a fecalith and should be aware of retained fecaliths as a cause of abscess formation after appendectomy.

Abdominal Abscess↗

PubMed2001

The release of antidiuretic hormone is appropriate in response to hypovolemia and/or sodium administration in children with severe head injury: a trial of lactated Ringer's solution versus hypertonic saline.

UNLABELLED: We conducted an open, randomized, and prospective study to determine the effect of hypertonic saline on the secretion of antidiuretic hormone (ADH) and aldosterone in children with severe head injury (Glasgow coma scale <8). Thirty-one consecutive patients at a level III pediatric intensive care unit at a children's hospital received either lactated Ringer's solution (Ringer's group, n = 16) or hypertonic saline (Hypertonic Saline group, n = 15) over a 3-day period. Serum ADH levels were significantly larger in the Hypertonic Saline group as compared with the Ringer's group (P = 0.001; analysis of variance) and were correlated to sodium intake (Ringer's group: r = 0.39, R(2) = 0.15, P = 0.02; Hypertonic Saline group: r = 0.42, R(2) = 0.18, P = 0.02) and volume of fluids given IV (Ringer's group: r = 0.38, R(2) = 0.15, P = 0.02; Hypertonic Saline group: r = 0.32, R(2) = 0.1, P = not significant). Correlation of ADH to plasma osmolality was significant if plasma osmolality was >280 mOsm/kg (r = 0.5, R(2) = 0.25, P = 0.06), indicating an osmotic threshold for ADH release. Serum aldosterone levels were larger on the first day than during Days 2 and 3 in both groups and inversely correlated to serum sodium levels only in the Ringer's group (r = -0.55, R(2) = 0.3, P < 0.001). This group received a significantly larger fluid volume on Day 1 (P = 0.05, Mann-Whitney U-test) than did patients in the Hypertonic Saline group, indicating hypovolemia during the first day. Head-injured children have appropriate levels of ADH. They may be hypovolemic during the first day of treatment, especially if they receive lactated Ringer's solution. IMPLICATIONS: In head-injured patients, we recommend fluid restriction to avoid inappropriate secretion of antidiuretic hormone. In a prospective, randomized, and controlled study in 31 children, we were able to show that the antidiuretic hormone levels are appropriate in response to hypovolemia, sodium load, or both.

Adolescent↗

PubMed2000

Posttraumatic dural sinus thrombosis in children.

Posttraumatic dural sinus thrombosis (DST) in children has rarely been described in the literature. Over a period of two years (1994-1996) we treated 131 children (n=131) with minor or severe head injury requiring a cranial computed tomography (CT) scan. DST was found in eight patients (6.1%), five with mild and three with severe cranial trauma. Diagnosis was suspected either because of a skull fracture crossing over a dural sinus or because of a hyperdensity at a dural sinus in the noncontrast CT scan. Enhanced CT scan was used to confirm DST. No specific symptoms related to DST were observed. DST was managed conservatively in all patients and recovery was uneventful. Recanalization of the sinus was documented to occur within three weeks to six months in six children. One patient showed a residual clot after five months and another did not have a follow-up CT scan. Based on our data we conclude that neither surgical nor medical intervention is indicated in traumatic DST in children.

Adolescent↗

PubMed2000

Pitfalls in therapy of upside-down stomach.

The upside-down stomach (UDS) is a special form of gastric organoaxial volvulus in a supradiaphragmatic hernial sac. The authors report five cases treated between 1979 and 1998, and seek to point out possible problems and pitfalls in the surgical management of this uncommon anomaly. Retrospective analysis of these cases shows that brachyesophagus as described is not a problem in UDS, as the esophagus is of normal length. The hiatus, on the other hand, is always very large and needs proper narrowing, which may be achieved through a transabdominal approach more easily than through a transthoracic access. A common esophago-aortal hiatus is often present. In conclusion, the authors recommend a hiatal repair and gastropexy, i.e. fundophrenopexy and corpoventropexy along the esophageal axis to prevent recurrent gastric herniation or torsion. Transabdominal access should be chosen since brachyesophagus is not expected. Beware of a common hiatus when preparing the hiatal crura. An antireflux procedure is not necessary as gastro-esophageal reflux usually resolves spontaneously after hiatal repair and gastropexy.

Digestive System Surgical Procedures↗

PubMed1999

Hirschsprung's disease and intestinal neuronal dysplasia--a frequent association with implications for the postoperative course.

Between 1991 and 1993, 106 newly diagnosed cases of Hirschsprung's disease (HD) were prospectively analyzed for the association of HD and intestinal neuronal dysplasia (IND) at ten pediatric surgical departments in central Europe. Hirschsprung-associated IND (HaIND) was found in 40% of cases. IND was disseminated in one-third and localized in two-thirds of the patients. Initial clinical symptoms were related to the length of the aganglionic segment, but not to the presence of HaIND. An enterostomy performed in 72 cases (67.9%) was located in a segment of pathologically innervated bowel in 50% of all cases, but in 72% of cases of HaIND. The proximal margin of the resected bowel showed pathological innervation in 44% of cases. Supplemental biopsies from the intestine (apart from diagnostic suction biopsies and biopsies at the enterostomy site) led to the first identification or definition of length of associated IND in 17.9% of cases. Postoperatively, the presence of long-segment aganglionosis or associated IND implied a delay in the restoration or normal defecation. Persistent constipation was found in 40% of patients with associated disseminated IND at follow-up at 6 months, compared to 20.6% in patients with isolated HD. These children needed secondary interventions more often than patients with associated localized IND or isolated HD. HaIND thus has clinical implications for the postoperative course if IND is disseminated.

Child↗

PubMed1999

Rectal biopsy for diagnosis of intestinal neuronal dysplasia in children: a prospective multicentre study on interobserver variation and clinical outcome.

BACKGROUND: Intestinal neuronal dysplasia (IND) of the colonic submucous plexus is considered to be a congenital malformation of the enteric nervous system causing symptoms resembling those of Hirschsprung's disease. In contrast with the established diagnosis of aganglionosis using enzyme histochemistry, controversy exists over the diagnostic criteria of IND on rectal biopsies previously defined by a consensus report and the causal relation between morphological findings and clinical symptoms. AIMS: The interobserver variability was prospectively investigated with respect to final diagnoses and several histological features in rectal biopsy specimens from children suspected of having colonic motility disturbances. METHODS: 377 biopsy specimens from 108 children aged 4 days to 15 years were independently coded without knowledge of clinical symptoms by three experienced pathologists for 20 histological features, and a final diagnosis was given for every case. Interobserver variation for the different items and the final diagnosis were analysed using Cohen's kappa statistic. Clinical data at biopsy and outcome after 12 months were related to morphological findings. RESULTS: The three pathologists agreed completely with respect to the diagnosis Hirschsprung's disease (kappa = 1), but in only 14% of the children without aganglionosis. In 15 (17%) of the 87 children without aganglionosis, at least one pathologist judged the case as normal, while another diagnosed IND. kappa values were close to the zero value expected by chance for the diagnoses normal and IND. Young age was related to the presence of several morphological features-for example, acetylcholine esterase staining and presence of giant ganglia. Children with chronic constipation diagnosed as having IND, given no other specific diagnosis by any of the pathologists, were significantly younger (median 8.8 months) and had a higher cure rate after one year (60%) than constipated patients considered by all observers to have no histological abnormalities (median 6.1 years, cure rate 23%). CONCLUSIONS: In contrast with Hirschsprung's disease, there is a high interobserver variation with regard to the different morphological features and final diagnosis of IND, based on the criteria and conditions of the previous consensus report. The high frequency of histological "abnormalities" in young infants suggests that some of the features may represent a normal variant of postnatal development rather than a pathological process. Investigations using more refined and morphometric methods in rectal specimens from infants and children without bowel disease are needed to define the normal range of morphological appearance at different ages. These preliminary data indicate that, with current knowledge, rectal biopsy for diagnostic purposes should only be performed in constipated children for diagnosis of Hirschsprung's disease.

Adolescent↗

PubMed1998

Cystic nephroma: a rare benign renal tumor.

We report two boys aged 1 year and 2 years 2 months, respectively, with cystic nephromas. Both presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the lower pole of the kidney in both cases with thin septa without solid parts. Macroscopically, the surface of the tumor was smooth. Both patients underwent a renal-sparing procedure; histology confirmed the diagnosis of cystic nephroma.

Child, Preschool↗

PubMed1998

Acute appendicitis in neonates: complication or morbus sui generis?

Neonatal appendicitis (NA) is very rare and is associated with a very high perforation rate. Three cases treated at our hospital since 1969 are presented. All of them had a perforated appendix; two represented a complication of an underlying disease, i.e., Hirschsprung's disease and cystic fibrosis, respectively. These diseases should always be ruled out actively in cases of NA. If an underlying disease is not found, NA might be an isolated form of necrotizing enterocolitis limited to the appendix. Histology cannot distinguish between a simple appendicitis and a localized form of NEC, so NA must be regarded as a complication of an underlying disease as well as a morbus sui generis.

Acute Disease↗

PubMed1998

A prospective, randomized, and controlled study of fluid management in children with severe head injury: lactated Ringer's solution versus hypertonic saline.

OBJECTIVES: Resuscitation in severe head injury may be detrimental when given with hypotonic fluids. We evaluated the effects of lactated Ringer's solution (sodium 131 mmol/L, 277 mOsm/L) compared with hypertonic saline (sodium 268 mmol/L, 598 mOsm/L) in severely head-injured children over the first 3 days after injury. DESIGN: An open, randomized, and prospective study. SETTING: A 16-bed pediatric intensive care unit (ICU) (level III) at a university children's hospital. PATIENTS: A total of 35 consecutive children with head injury. INTERVENTIONS: Thirty-two children with Glasgow Coma Scores of <8 were randomly assigned to receive either lactated Ringer's solution (group 1) or hypertonic saline (group 2). Routine care was standardized, and included the following: head positioning at 30 degrees; normothermia (96.8 degrees to 98.6 degrees F [36 degrees to 37 degrees C]); analgesia and sedation with morphine (10 to 30 microg/kg/hr), midazolam (0.2 to 0.3 mg/kg/hr), and phenobarbital; volume-controlled ventilation (PaCO2 of 26.3 to 30 torr [3.5 to 4 kPa]); and optimal oxygenation (PaO2 of 90 to 105 torr [12 to 14 kPa], oxygen saturation of >92%, and hematocrit of >0.30). MEASUREMENTS AND MAIN RESULTS: Mean arterial pressure and intracranial pressure (ICP) were monitored continuously and documented hourly and at every intervention. The means of every 4-hr period were calculated and serum sodium concentrations were measured at the same time. An ICP of 15 mm Hg was treated with a predefined sequence of interventions, and complications were documented. There was no difference with respect to age, male/female ratio, or initial Glasgow Coma Score. In both groups, there was an inverse correlation between serum sodium concentration and ICP (group 1: r = -.13, r2 = .02, p < .03; group 2: r = -.29, r2 = .08, p < .001) that disappeared in group 1 and increased in group 2 (group 1: r = -.08, r2 = .01, NS; group 2: r = -.35, r2 =.12, p < .001). Correlation between serum sodium concentration and cerebral perfusion pressure (CPP) became significant in group 2 after 8 hrs of treatment (r = .2, r2 = .04, p = .002). Over time, ICP and CPP did not significantly differ between the groups. However, to keep ICP at <15 mm Hg, group 2 patients required significantly fewer interventions (p < .02). Group 1 patients received less sodium (8.0 +/- 4.5 vs. 11.5 +/- 5.0 mmol/kg/day, p = .05) and more fluid on day 1 (2850 +/- 1480 vs. 2180 +/- 770 mL/m2, p = .05). They also had a higher frequency of acute respiratory distress syndrome (four vs. 0 patients, p = .1) and more than two complications (six vs. 1 patient, p = .09). Group 2 patients had significantly shorter ICU stay times (11.6 +/- 6.1 vs. 8.0 +/- 2.4 days; p = .04) and shorter mechanical ventilation times (9.5 +/- 6.0 vs. 6.9 +/- 2.2 days; p = .1). The survival rate and duration of hospital stay were similar in both groups. CONCLUSIONS: Treatment of severe head injury with hypertonic saline is superior to that treatment with lactated Ringer's solution. An increase in serum sodium concentrations significantly correlates with lower ICP and higher CPP. Children treated with hypertonic saline require fewer interventions, have fewer complications, and stay a shorter time in the ICU.

Adolescent↗

PubMed1997

An animal model for short-bowel syndrome in piglets to assess the efficiency of bowel-lengthening procedures.

Intestinal lengthening is considered to be one of the most effective surgical interventions to improve the deleterious sequelae of short-bowel syndrome. To assess the efficiency of bowel-lengthening procedures in short-bowel syndrome, an animal model was created in minipigs that show malnutrition, weight loss and small-bowel dilatation. Group 1 consisted of three animals with a 95% distal intestinal resection leaving 15 cm of ileum. Group 2 comprised six animals with a 95% distal intestinal resection leaving only 5 cm of ileum, and group 3 consisted of three animals with a 95% distal intestinal resection leaving 5 cm of ileum and construction of a distal small-bowel stenosis by temporary banding. In group 1 there was a significant increase in length of residual bowel at terminal laparotomy at 10 weeks and a significant difference for protein and potassium. No bowel dilatation occurred and no animal lost weight. Group 2 animals showed a continuous loss of weight after the intestinal resection and significantly different values in animal profile were found at 10 weeks but no significant difference in length or in diameter of the residual bowel. In group 3, all animals demonstrated a significant dilatation of the residual small bowel at 1 week when the stenosis was resected and bowel continuity restored. We conclude that in piglets a 95% distal intestinal resection leaving 5 cm of ileum leads to a short-bowel syndrome with malnutrition and weight loss, but no intestinal dilatation. To provoke an additional intestinal dilatation, creation of a distal stenosis is mandatory. Animals of group 3 represent a suitable model to assess the efficacy of short/bowel lengthening procedures.

Animals↗

PubMed1997

Laparoscopic removal of hydrosalpinx in a girl: case report.

We report on a 12-year-old girl who underwent laparoscopic excision of a left-sided hydrosalpinx. She presented with recurrent lower abdominal pain 8 years after appendectomy and resection of the left ovary. Sonography revealed a large cystic, tubular mass cranial and to the left of the urinary bladder. Laparoscopy demonstrated a left-sided hydrosalpinx which was resected endoscopically using a stapler.

Appendectomy↗

PubMed1994

Lichen sclerosus et atrophicus causing phimosis in boys: a prospective study with 5-year followup after complete circumcision.

We prospectively investigated 100 pediatric patients suffering from phimosis and found a 10% incidence of lichen sclerosus et atrophicus. This condition usually can be diagnosed preoperatively because of its classic manifestation of severe phimosis due to a sclerotic, whitish ring at the tip of the prepuce, which in our cases was accompanied by sclerogenous glanular lesions. To our knowledge our study represents the first evidence that the development of secondary phimosis with no apparent reason in school-age boys is highly suggestive for lichen sclerosus et atrophicus. Complete circumcision is the therapy of choice because it completely removes all affected tissue and it allows spontaneous regression or resolution of glanular lesions. There has been no recurrence after 5 years of followup.

Adolescent↗

PubMed1994

Surgical procedures in colonic strictures after necrotizing enterocolitis.

Between 1982 and 1992, 22 patients were treated with colonic strictures in the course of necrotizing enterocolitis (NEC). Fourteen newborns in whom a primary enterostomy and, when necessary, resection of necrotic bowel was performed developed strictures in the diverted colon. The strictures were detected by colon contrast enema study performed on average 3 months after the first intervention. Eight additional children suffered from an ileus due to primary strictures after conservatively treated NEC, which was surgically managed by enterostomy. Closure of the enterostomy and resection of the stenotic part of the colon was performed thereafter in all 22 children as a single stage procedure. There was no insufficiency of the anastomosis nor any late stricture at follow-up 2.7 years after NEC in our patients. It is concluded therefore that reanastomosis of the enterostomy and resection of an intestinal stricture can be performed as a single stage procedure without any risk after an interval of 3 months between onset of acute NEC and reevaluation. During this interval, a close monitoring and an appropriate management of adequate supplement of electrolytes and bicarbonates is necessary. Most of our babies could be nursed at home and showed a good weight gain during this period, despite the enterostomy.

Anastomosis, Surgical↗

PubMed1994

[Long-term follow-up of traumatic hip fracture in childhood].

Traumatic hip dislocation is a rare emergency situation in childhood. Long-term follow-up (10 1/12 years [10/12-20 years]) of 9 patients treated at our institution between 1971 and 1992 revealed no late sequelae. There was no case of necrosis of the femoral head. One patient had a recurrent dislocation of the hip 17 and 18 years after the first dislocation. For post-reduction treatment we propose bedrest until pain relief and mobilisation with crutches for 4 weeks. Regular clinical and radiographic controls, including bone scan and/or MRI, are mandatory during the first 2 years.

Adolescent↗