Neurosurgical management in children with bleeding diathesis: auditing neurological outcome.

OBJECTIVE The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors' service. METHODS The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients' neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale-Extended (GOS-E Peds) obtained 1 year after the last operation. RESULTS Five patients with a bleeding diathesis who underwent surgery for intracranial hematoma were identified; the diagnosis was hemophilia A in 3 cases, idiopathic thrombocytopenic purpura in 1 case, and severe aplastic anemia in 1 case. Intracerebral hematoma (ICH) (n = 4) and acute subdural hematoma (n = 1) were confirmed on radiological investigations. In 2 of the 4 patients with ICH, the diagnosis of bleeding diathesis was made for the first time on presentation. Four patients (all male) were younger than 2 years; the patient with severe aplastic anemia and spontaneous ICH was 15 years old and female. The duration of symptoms varied from 24 hours to 5 days. Neurological examination at 1 year's follow-up showed complete recovery (GOS-E Peds score of 1) in 3 cases and mild weakness (GOS-E Peds score of 2) in 2 cases. CONCLUSIONS Neurosurgical management of patients with bleeding diathesis should be carried out in a tertiary-care setting with multidisciplinary team management, including members with expertise in neuroimaging and hematology, in addition to neurosurgery. Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.

Outcomes in Patients With Hemophilia and von Willebrand Disease Undergoing Invasive or Surgical Procedures.

Adults with hemophilia A (HA), hemophilia B (HB), and von Willebrand disease (VWD) frequently require surgery and invasive procedures. However, there is variability in perioperative management guidelines. We describe our periprocedural outcomes in this setting. A retrospective chart review from January 2006 to December 2012 of patients with HA, HB, and VWD undergoing surgery or invasive procedures was conducted. Type of procedures, management including the use of continuous factor infusion, and administration of antifibrinolytics were reviewed. Adverse outcomes were defined as acute bleeding (<48 hours), delayed bleeding (≥48 hours), transfusion, inhibitor development, and thrombosis. We identified 59 patients with HA and HB. In all, 24 patients had severe hemophilia and 12 had mild/moderate hemophilia. Twelve patients had inhibitors. There were also 5 female carriers of HA and 6 patients with VWD. There were 34 major surgeries (26 orthopedic, 8 nonorthopedic) and 129 minor surgeries. Continuous infusion was used in 55.9% of major surgeries versus 8.5% of minor surgeries. Antifibrinolytics were administered in 14.7% of major surgeries versus 23.2% of minor surgeries. In all, 4 patients developed acute bleeding and 10 patients developed delayed bleeding. Delayed bleeding occurred in 28.6% of genitourinary procedures and in 16.1% of dental procedures. Five patients acquired an inhibitor and 2 had thrombosis. In conclusion, patients with HA, HB, or VWD had similar rates of adverse outcomes when undergoing minor surgeries or major surgeries. This finding underscores the importance of an interdisciplinary management and procedure-specific guidelines for patients with hemophilia and VWD prior to even minor invasive procedures.

Venomous snakebites in children in southern Croatia.

This retrospective study represents observation of 160 children and adolescents aged up to 18 years that experienced venomous snakebites in southern Croatia and were treated in the Clinical Department of Infectious Diseases in the University Hospital Centre Split from 1979 to 2013. The main purpose of this research was to determine the epidemiological characteristics, clinical presentation, local and general complications, and received treatment. Most bites occurred during warm months, from early May to late August (80%), mostly in May and June. Upper limb bites were more frequent (59%) than lower limb bites (40%). Out of the total number of poisoned children, 24% developed local, and 25% general complications. The most common local complications were haemorrhagic blisters that occurred in 20% children, followed by compartment syndrome presented in 7.5% patients. The most dominated general complication was cranial nerve paresis or paralysis, which was identified in 11.2% patients, whereas shock symptoms were registrated in 7% children. According to severity of poisoning, 9.4% children had minor, 35% mild, 30.6% moderate, and 24.4% had severe clinical manifestation of envenomation. Only one (0.6%) child passed away because of snakebite directly on the neck. All patients received antivenom produced by the Institute of Immunology in Zagreb, tetanus prophylaxis as well, and almost all of them received antibiotics, and a great majority of them also received corticosteroids and antihistamines. Neighter anaphylactic reaction nor serum disease were noticed in our patients after administrating antivenom. A total of 26% children underwent surgical interventions, and incision of haemorrhagic blister was the most common applied surgical treatment, which was preformed in 15.6% patients, while fasciotomy was done in 7.5% subjects. All of our surgically treated patients recovered successfully.

Outcomes of liver resection for haemorrhagic hepatocellular adenoma.

BACKGROUND: Intratumoral bleeding and/or intraperitoneal rupture occurs in up to 20% of patients with hepatocellular adenoma (HCA). Hepatectomy in the presence of haemorrhagic HCA has been associated with increased morbidity and mortality rates. This study evaluates the outcomes of hepatectomy for haemorrhagic HCA at a single institution. METHODS: Between January 1997 and December 2012, 52 consecutive patients underwent liver resection for HCA. Among them, 14 patients were resected for haemorrhagic (H)-HCAs (including 9 cases of intratumoural bleeding and 5 cases of intraperitoneal bleeding) and 38 for non-haemorrhagic (NH)-HCAs. RESULTS: The preoperative characteristics were similar between the two groups except for younger age (p = .001) and shorter duration of hormonal use (p = .001) in (H)-HCAs. There were no mortalities. Intraoperative blood loss, transfusion rate, and postoperative morbidity were comparable between the two groups of patients (p = ns). The length of hospital stay was significantly longer in (H)-HCAs (p = .03). In all the resected H-HCAs, pathology showed central haemorrhagic changes with tumoral cells at the periphery of the lesions. CONCLUSIONS: Liver resection for H- and NH-HCAs can be achieved with no mortality and comparable short-term outcomes.

A severe bleeding diathesis in a 6-year-old girl secondary to a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis.

A 6-year-old girl presented with presumed relapse of childhood immune thrombocytopenia. Investigations revealed deranged coagulation parameters, abnormal small bowel thickening, and splenomegaly. A clinically significant bleeding diathesis emerged which was refractory to most hemostatic interventions. Laparatomy revealed a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis. Splenectomy resulted in complete resolution of the coagulopathy. The diagnosis and management of these conditions is inherently complex and without clear guidance. We discuss our perioperative management of the bleeding diathesis. There is a need for long-term follow-up of the underlying pathologies particularly as potentially useful therapeutic agents have emerged.

Topical Ankaferd hemostat application for the management of oral cavity bleedings in children with hemorrhagic diathesis.

Ankaferd blood stopper (ABS) is a hemostatic agent used topically for controlling bleedings of skin or mucosal surfaces in Turkey. It is currently topically used in bleedings of body injuries, traumas, and minor or major surgical interventions. Here we have evaluated 12 pediatric patients with hemorrhagic diathesis on whom Ankaferd was used for oral bleedings. Topical Ankaferd was administered for hemorrhages of oral cavity during 15 bleeding attacks. ABS administrations successfully stopped the bleedings, except for one patient with oral hemorrhage who did not respond to ABS application. Ankaferd is effective for oral bleedings of children with bleeding diathesis especially when other measures have failed.

Lesiones vasculares abdominales / Abdominal vascular lesions

Están entre las lesiones más letales sufridas por los pacientes politraumatizados, así como también, se encuentran entre las más difíciles y desafiantes para los cirujanos de politrauma. Raramente son aisladas, lo habitual es encontrar múltiples lesiones asociadas, lo que aumenta su gravedad y el tiempo necesario para repararlas, lo que conlleva una morbimortalidad significativa. El manejo correcto implica un diagnóstico y abordaje quirúrgico precoces (AU)

Abdominal vascular lesions are amongst the most lethal lesions suffered by patients with multiple injuries, as well as being among the most difficult and challenging for the surgeon. They are rarely isolated, they are usually found with associated multiple injuries, which increases its seriousness and the time required to repair them and may lead to a significant morbidity and mortality. The correct management involves an early diagnosis and surgical approach (AU)

Management of children with inherited mild bleeding disorders undergoing adenotonsillar procedures.

OBJECTIVE: To evaluate the incidence of perioperative bleeding among children with mild bleeding disorders who underwent adenotonsillar surgery in our hospital and were treated with our perioperative bleeding prophylaxis protocol. METHODS: Retrospective chart review was aimed at determining the perioperative bleeding rate in children with mild bleeding disorders subjected to our prophylactic protocol while undergoing adenotonsillar procedures. Low von Willebrand factor (LVWF), unspecific platelet function disorders (UPFD) and mild factor VII deficiency were considered as mild bleeding disorders. The protocol utilizes intravenous desmopressin, tranexamic acid, NSAID avoidance, and overnight observation. RESULTS: Between 2004 and 2009, 44 children with mild bleeding disorders underwent adenotonsillar procedures in our hospital and were treated with the protocol. One patient (LVWF+UPFD) developed perioperative bleeding (2.3%). CONCLUSIONS: It is possible to obtain low rates of perioperative bleeding in children with mild bleeding disorders undergoing adenotonsillar procedures, provided there is a well-timed diagnosis and an adequate prophylaxis protocol. We believe that further efforts must be directed at preoperative diagnosis of mild bleeding disorders to ensure safer surgeries.

Diagnosis and surgical management of intraspinal hemorrhagic juxtafacet cysts in lumbar spine: experience of eight cases.

Hemorrhagic juxtafacet synovial cysts have rarely been reported in the literature. We reviewed case records of eight patients with hemorrhagic juxtafacet cysts treated surgically. Back pain and acute radicular pain were the presenting features in the all patients. The possible cause for hemorrhage could be identified in only three patients. Six patients had medial facetectomy and hemilaminectom and two patients with stenosis had medial facetectomy and laminectomy. Following surgery there was immediate relief from pain with significant improvement of the Oswestry disability index (ODI). Six patients had an excellent outcome while the remaining two patients had a good outcome. During the follow-up there was no progression of slippage, no segment instability, and no recurrence. Hemilaminectomy or laminectomy with medial facetectomy without fusion might serve as an effective and safe management strategy to treat lumbar hemorrhagic juxtrafacet synovial cysts.

Medical haemostasis in acute hepatocyte injury and experimental liver trauma.

BACKGROUND: The aim of the present study was to test the effectiveness of aprotinin to reduce bleeding in liver resection of guinea pigs with acutely injured hepatocyte using intraperitoneal d(+)Galactosamine. METHODS: Thirty-two guinea pigs were divided equally into four groups. Group 1 was the control group. Group 2 received intraperitoneal D(+)galactosamine. Group 3 received intraperitoneal d(+)galactosamine prior to a standard liver resection. Group 4 received 10.000 KIU/kg aprotinin infusion via jugular catheter in 10 min prior to standard liver resection in pretreated animals with d(+)galactosamine. All of the measurements and surgical interventions were made 24 h after the administration of d(+)galactosamine. Bleeding amounts were recorded in groups 3 and 4 for 1 h by weighing the sponges placed into the abdomen. Liver function tests, histologic, haematologic and fibrinolytic parameters were measured. RESULTS: Hepatocyte injury and hyperfibrinolysis were seen at the end of 24 h after application of d(+)galactosamine in groups 2, 3, and 4. Statistically significant amounts of bleeding from the resected livers were observed in group 3 and 4. In group 4, the bleeding was reduced (P < 0.05) and fibrinolytic parameters were normalized (P < 0.05) with aprotinin infusion. CONCLUSIONS: Significant bleeding diathesis and hyperfibrinolysis occurred in groups 2, 3, and 4, which had hepatocyte injury proved with histopathologic and haematologic tests. Prothrombin time (PT) and partial thromboplastin time (aPTT) in groups 3 and 4 were fivefold higher than that in the control group (P = 0.0001). The bleeding tendency according to high PT and aPTT levels were continued with application of aprotinin while reduction of bleeding was seen. Parenchymatous organ haemorrhage in acute liver failure or hyperfibrinolytic conditions could be reduced significantly with aprotinin without procoagulant effect.

Profound imbalance of pro-fibrinolytic and anti-fibrinolytic factors (tissue plasminogen activator and plasminogen activator inhibitor type 1) and severe bleeding diathesis in a patient with cirrhosis: correction by liver transplantation.

A 49-year-old male with alcoholic cirrhosis suffered several spontaneous, life-threatening, deep muscle bleeding episodes. Laboratory evaluation indicated excessive fibrinolysis with low plasminogen, low alpha2-antiplasmin, undetectable plasminogen activator inhibitor type 1 (PAI-1) activity, high tissue plasminogen activator (t-PA) activity and high t-PA antigen. Treatment with oral anti-fibrinolytic agents prevented further bleeding episodes. Decompensated cirrhosis eventually necessitated orthotopic liver transplantation. Post-operatively, the patient did not require oral anti-fibrinolytic agents, and there were no significant bleeding events. Circulating PAI-1 activity, t-PA activity and antigen normalized by 3 months post transplant. In short, the profound bleeding diathesis, as well as the imbalance in t-PA and PAI-1 levels, corrected after liver transplantation. Recognition of such patients is important, because the bleeding diathesis is an indication rather than a contraindication for orthotopic liver transplantation.

Women and inherited bleeding disorders: menstrual issues.

Von Willebrand, who described a bleeder family from Aland in 1926 in whom the index patient died at the fourth menses, stated, "the trait seems to be seen among women." Menorrhagia is defined objectively as a menstrual blood loss of at least 80 mL. However, even though 5% of women aged 30 to 49 years consult their general practitioner and 12% of gynecologic referrals are for menorrhagia, the diagnosis is difficult. A pictorial bleeding assessment chart (PBAC) has a specificity and sensitivity of more than 80%, with a score of > or = 100 being equivalent to more than 80 mL of blood loss. Using this chart to screen 150 women with menorrhagia who attended a gynecology clinic, an inherited bleeding disorder was diagnosed in 17%. Menorrhagia with onset at the menarche was predictive of an inherited bleeding disorder in 65% of von Willebrand's disease (vWD) and 67% of factor XI (FXI)-deficient patients. A retrospective survey in patients with inherited bleeding disorders using the PBAC showed menorrhagia in 74% of patients with vWD, 57% of carriers of hemophilia A or B, and 59% of FXI-deficient patients, compared with 29% of control individuals. Menorrhagia was the most common symptom in 60% of patients with FVII deficiency studied. Menorrhagia in women with bleeding disorders can be controlled with tranexamic acid with good effect. More recently, a desmopressin acetate (DDAVP) spray has been shown to achieve good FVIII and von Willebrand factor (vWF) levels and is efficacious for women with these deficiencies. The oral contraceptive pill may be useful. Since bleeding disorders are found in a substantial number of women with menorrhagia, it is important that such patients are investigated for these disorders before invasive procedures are done; hysteroscopy and hysterectomy in these patients are associated with a high rate of postoperative bleeding.

[Hysteroscopic endometrium ablation in "high-risk" situations and in hemorrhagic diathesis]. / Hysteroskopische Endometriumablation in "high-risk"-Situationen und bei haemorrhagischer Diathese.

A hysteroscopic endometrial ablation (HEA) under maximal anesthesiologic surveillance was performed on 34 high-risk patients (group I: chronic anticoagulant therapy n = 26; group II: endogenous coagulopathy n = 8) with therapy resistant meno-metrorrhagia to avoid a hysterectomy (HE). Total amenorrhea, or a least hypomenorrhea respectively cyclic spotting could be attained primarily in 22 patients (group I: 19; group II: 3), after a repeat procedure in further 6 patients (4 in group I, 2 in group II). Subjective evaluation of surgical results (overall 22 patients primarily satisfied, 6 secondarily) also differed between the two subgroups (group I: p < 0.01 primarily satisfied; p < 0.05 secondarily satisfied vs. p < 0.05 and p < 0.01 in group II). A HE had to be performed on two patients due to extensive adenomyosis uteri interna (group II). The significantly better results in the anticoagulation group were probably due to the basic illness. Larger groups will, however, be necessary before any conclusions from this difference can be drawn. No surgical or anesthesiological complications occurred. There also were no major postoperative complications (1 endomyometritis, 2 cervical stenoses). Endometrial ablation was found to be a valuable treatment alternative for this specific group of patients with severe coagulopathy, thrombo-embolic or thrombotic disease.

[Hemorrhagic diathesis and oral surgery]. / Diatesi emorragiche e chirurgia odontostomatologica.

Dental and/or periodontal diseases are an important clinical and social problem in patients with hemorrhagic diathesis. The possibility to overcome it successfully depends on the type of hemorrhagic disease and on the organization of hematological and odontostomatological centres: in fact, the best results in the surgery of these patients are obtained through the balanced combination of general and local treatments. The right diagnosis of coagulopathy is the first important goal: it can be obtained through the screening tests of hemostasis and, when necessary, through additional tests. Therapeutic protocols are available for each congenital or acquired hemorrhagic diathesis to prepare the patient for surgery; by so doing the risk of hemorrhagic complications is negligible. A better homogeneity in the approach to these patients in the different centres is highly desirable: the present review can offer a contribute in this respect.

Anesthetic considerations in diffuse bleeding diathesis of uncertain origin.

This is a report of a case of a diffuse bleeding tendency in a pregnant woman who presented for emergency splenectomy with a tentative diagnosis of thrombotic thrombocytopenic purpura. The influence of multiple organ dysfunction in the selection of appropriate monitors and the anesthetic technic in such cases are complex.