Christa Schimke

Severe Pediatric Blunt Trauma—Successful ROTEM-Guided Hemostatic Therapy with Fibrinogen Concentrate and No Administration of Fresh Frozen Plasma or Platelets

Use of allogeneic blood products to treat pediatric trauma may be challenged, particularly in relation to safety. We report successful treatment of a child with severe abdominal and pelvic injuries with preemptive fibrinogen supplementation followed by rotational thromboelastometry (ROTEM)-guided, goal-directed hemostatic therapy. Fibrinogen concentrate was administered (total dose: 2 g), while transfusion of fresh frozen plasma and platelet concentrate was avoided. Activated partial thromboplastin time was prolonged and Quick values were low but ROTEM clotting time values remained normal, therefore, no thrombin-generating drugs were considered necessary. This case shows the potential for hemostatic treatment with coagulation factor concentrates to be applied to pediatric trauma.

Evaluation of urinary and faecal continent stomas

OBJECTIVES To evaluate the outcome and complications of urinary and faecal continent catheterizable stomas. PATIENTS AND METHODS Retrospective record review of all patients operated on in our institution within the last 6 years. RESULTS Fifty-five children received 63 stomas. The mean age at operation was 9.2 years and the mean follow-up period was 2.8 years. The diagnoses were: neurogenic bladder (n=22), bladder or cloacal exstrophy (n=20), anorectal malformation (n=6), persistent cloaca (n=3), Hirschsprungs disease (n=2), posterior urethral valves (n=1) and prune belly syndrome (n=1). There were 35 urinary stomas and 28 faecal stomas. Eight patients received both stomas. Both the appendix and a transverse tubularized intestinal segment (Yang-Monti technique) were used; in some cases a caecal flap was created. In three patients the appendix was divided and used to construct two stomas. The implantation of urinary stomas was into the native bladder in 24 patients and into the intestinal segment of an augmented bladder in 11 cases. The location of the stoma was umbilical in 20 cases, right lower quadrant of the abdomen in 35, neo-umbilicus in five and left lower quadrant in three. When an extra-umbilical location was chosen skin flaps were used. Excellent cosmetic and functional results were achieved in 53 (84.2%) of the 63 stomas, which are continent and easy to catheterize. Two had acceptable results with minor problems. There were eight re-operations (12.6% of stomas, 14.5% of patients). The reasons for the re-operations were difficult catheterization in two, incontinence in three, and mucosal prolapse in three patients in whom skin flaps were not used. Of the eight re-operations, seven were in urinary stomas and one in a faecal stoma. Urinary incontinence was attributed to shortening of the intravesical tunnel. No difference in results was observed between the different techniques used, with the exception that cutaneous anastomosis without skin flaps had to be revised because of prolapse, and two out of three incontinent channels had been constructed with tubularized ileum. CONCLUSIONS Catheterizable continent urinary and faecal stomas achieve the objectives of urinary and faecal continence and independence in most patients with a low complication rate.

Colpo-wrap: a new continence procedure

To present a new surgical method to increase bladder outlet resistance for the treatment of urinary incontinence in girls and women.

Improvement of continence with reoperation in selected patients after surgery for anorectal malformation.

PURPOSE: Fecal incontinence is a serious complication after repair of anorectal malformations. We investigated whether reoperation can improve fecal continence. METHODS: Medical records of 41 patients (40 children and one adult; 26 male and 15 female) who underwent reoperation after previous reconstruction of an anorectal malformation were reviewed for outcomes of bowel function. Type of primary corrective surgery performed, therapeutic measures, results of physical examination and barium enema, and reoperation procedures were evaluated. A questionnaire was administered to assess stool behaviour and level of continence at follow-up three or more years after secondary operation. RESULTS: Secondary operations in males comprised posterior sagittal anorectoplasty (PSARP) in 16 patients, PSARP with antegrade continent enema in one patient, antegrade continent enema alone in 6, anoplasty in one, rectosigmoid resection in 1, and definitive colostomy in 1 patient. Secondary operations in females included PSARP alone in 4 patients, PSARP with total urogenital mobilization in 4, PSARP with vaginoplasty in 2, PSARP with vaginoplasty and antegrade continent enema in 2, and PSARP with vaginourethroplasty in 3. Of 41 patients 18 (44 percent) were continent at follow-up, 21 (51 percent) were clean with use of enemas, diet, or drug therapy. One patient had a definitive colostomy. One died after kidney transplantation. CONCLUSIONS: Surgery is a good option for improving incontinence in selected patients previously operated for anorectal malformations. Posterior sagittal anorectoplasty is advocated to improve bowel control. Antegrade continent enema is a reliable therapeutic option to maintain clean patients with fecal incontinence.

Rotundum psoas hitch: A new method for colpohysteropexy in girls with bladder exstrophy

Female patients with bladder exstrophy frequently suffer from uterine prolapse, which is due to a defective pelvic floor anatomy. Different techniques for the correction of procidentia in this patient population have been described; however, since the numbers of patients are very small and no series with long-term follow-up exists, it is unclear which technique yields superior results. We describe here a new technique for uteropexy in girls and women with bladder exstrophy. In the rotundum psoas hitch procedure (RPH), the round ligaments are detached from the inner inguinal ring and fixed to the psoas muscle on both sides, thereby fixing the uterus in a more cranial and dorsal position. This procedure has been applied successfully in nine patients with a mean follow-up of 23.8 months. In six cases RPH was done prophylactically and in three cases therapeutically to correct an existing uterine prolapse. The mean age at operation was 9.6 years. In all but one case, RPH was combined with continent urinary reconstruction. No postoperative morbidity due to this technique has been described. RPH is an effective means of preventing or correcting procidentia in patients with bladder exstrophy; we emphasize that it should be combined with other abdominal operations, such as continent reconstruction, in girls or women with this condition.

Congenital obstructive distal urethral membrane.

We present a rare case of complete distal urethral obstruction caused by a distal meatal membrane in a newborn boy. A cystic lesion of the distal penis was detected and excised, revealing a hypospadiac urethra. Further examination showed bilateral vesico-ureteral reflux with a dilated posterior urethra and urethral lesion, suggesting posterior urethral valves (PUV); these were confirmed at 2 months and resected endoscopically. At 16 months a left nephrectomy was performed due to impaired renal function on that side; there was no longer reflux on the right side. The obstruction probably did not become severe until late in gestation, but still led to functional loss of one kidney. The PUV do not seem to have been the cause of renal deterioration. The cystic lesion may have been a remnant of the cloacal membrane, but this is difficult to prove histologically.

Laparoscopic Management of Autoamputated Ovary in Newborns: A Report of 2 Cases

Intrauterine autoamputation of the ovary is an extremely rare diagnosis in the pediatric population. The current literature is limited to contradictory recommendations, while a standard management protocol for autoamputated adnexa secondary to intrauterine ovarian torsion is yet to be established. We report 2 cases of auto-amputation of the ovary, leading to a free-floating intra-abdominal cyst in the newborn. Laparoscopic management was successful in both cases.

Sacrococcygeal Teratoma Presenting with Vaginal Discharge and Polyp in an Infant

BACKGROUND Sacrococcygeal teratoma accounts for the most common solid tumor in neonates. Because of improved technology, 50%-70% of cases can be diagnosed antenatally during routine ultrasound screenings. If not diagnosed antenatally, clinical findings at birth are distinct in most cases including a palpable or visible mass. CASE We report an unusual case of a 1-year-old girl who presented with persistent vaginal discharge leading to diagnosis of a mucosal polypoid lesion of the vagina, ultimately revealing a hidden sacrococcygeal teratoma. SUMMARY AND CONCLUSION We suggest thorough investigation of all infants who present with purulent discharge and recurrent vaginal mass; sacrococcygeal teratoma should routinely be considered as a differential diagnosis.

Die Langerhans Zell Histiozytose der Lunge

Die Langerhans Zell Histiozytose (LH) ist eine selten Erkrankung, die durch das Auftreten von differenzierten Zellen des MonozytenMakrophagen Stammes gekennzeichnet ist. Diese Zellen sind S-100 und CD 1a positiv und haben die typischen intrazellulären Birbeck Granulas. Diese Zellen können alle Organe infiltrieren, und je nach deren Ausdehnung wird die LH in vier Hauptmanifestationen unterteilt: AbtLetterer-Siwe, Hand-Schüller-Christian, Hashimoto-Pitzker und eosinophiles Granulom. Eine Lungenbeteiligung tritt bei Kindern selten auf, und noch seltener ist eine isolierte LH ohne Beteiligung anderer Organsysteme.