Circumcision and complications in patients with haemophilia in southern part of Turkey: Çukurova experience.

Circumcision is the oldest and most frequent surgical procedure in the world and especially in Turkey as is seen in the other Islamic countries because of religious and traditional pressures. In this study, we aim to report the experience of circumcision at Çukurova University in a total of 76 patients with haemophilia between 1990 and 2011. We retrospectively reviewed medical records of 69 haemophilia patients without inhibitors and seven haemophilia patients with inhibitors who had been circumcised. Before the year 2000, factor concentrates were given before and after circumcision for 6-7 days. After 2000, we used fibrin glue together with factor concentrates for only 3 days. By-passing agents were used for circumcision in haemophilia patients with inhibitors. Twelve of 69 patients without inhibitors were referred to our centre with bleeding after the circumcision before diagnosis of haemophilia. Nine of these twelve patients had severe life threatening bleeding and three of them had moderate bleeding. Sixty-four patients with haemophilia were circumcised in our centre under general anaesthesia except for three patients who were given local anaesthesia. Thirteen of 57 haemophilia patients (22.8%) without inhibitors had seven mild and six moderate bleeding complications. A few patients had significant bleeding, despite adequate factor replacement. Five of seven haemophilia patients with inhibitors had two moderate and three mild bleeding complications. Our experience showed that circumcision for patients with haemophilia should be carefully performed by surgeons together with paediatric haematologist, under appropriate conditions in haemophilia centres which has comprehensive coagulation lab.

Bilateral abducens nerve palsy following ruptured anterior communicating artery aneurysm: report of 2 cases.

Isolated abducens nerve palsies associated with intracranial aneurysms have rarely been reported. Their association with anterior communicating artery (ACoA) is even rarer. Intracisternal clot formation and elevated intracranial pressure has been proposed to be the responsible mechanisms. Herewith, we report two cases of bilateral abducens palsies following ruptured ACoA aneurysms and speculated the possible mechanisms. Opening of Liliequist's membrane provides clinical improvement due to CSF release.

Difficulties in treatment of CSF leakage associated with a temporal meningocele.

Temporal meningocele is a rarely encountered pathology. It is caused by communication between the subarachnoid space of the middle fossa and lateral extension of the sphenoid sinus. Cerebrospinal fluid (CSF) pressures and the hydrostatic pulsatile forces may lead to the development of pitholes on the middle fossa at the sites of arachnoid villi with herniation of dura/arachnoid or brain tissue into the sinus. We describe an adult patient who presented with spontaneous CSF rhinorrhea due to a temporal meningocele. She was first operated on transsphenoidally, but the CSF rhinorrea did not cessate, therefore she was operated transcranially five days after the first operation. There has been no CSF rhinorrhea for three and a half years. Transcranial temporal encephalocele repairment is more effective than transsphenoidal surgery. Recurrent CSF leaks can occur due to both the increased CSF pressure and the insufficient operation technique.

Lumbar epidural hematoma following lumbar puncture: the role of high dose LMWH and late surgery. A case report.

Spinal epidural hematoma (SEH) is a known complication of spinal surgery, but the incidence of post-surgical SEHs that result in neurologic deficits is extremely rare (0.1%). Patients that require multilevel lumbar procedures and/or have a preoperative coagulopathy are at a significantly higher risk of developing an epidural hematoma. The introduction of higher dose of low molecular weight heparin (LMWH) twice daily 30 mg regimen) increased the reported incidence of neuroaxial hematomas. Surgery performed within 8 hours makes good or partial recovery of neurologic function. Our patient was also started on higher dose of LMWH and developed neurological deficits due to a SEH following lumbar puncture. She underwent operation after six days and she had a mild recovery following the operation. Current administration of high doses of LMWH can cause SEH even after a lumbar puncture, which was performed without multiple attempts. Although surgery performed within 8 hours makes good or partial recovery of neurologic function, laminectomy and epidural hematoma evacuation performed after three days can also have successful results.

The effects of fenestration of the interpeduncular cistern membrane arousted to the opening of lamina terminalis in patients with ruptured ACoA aneurysms: a prospective, comparative study.

BACKGROUND: The incidence of chronic hydrocephalus requiring shunt placement is a well-known and common complication of aneurysmal subarachnoid hemorrhage (aSAH). It was suggested that fenestration of the lamina terminalis (LT) during microsurgery for aSAH may be associated with a reduced rate of shunt-dependent chronic hydrocephalus (SDCH). We analyzed that, fenestrations of the LT and Liliequist membrane (LM) would reduce rate of SDCH and improve rate of favorable outcome. METHODS: 145 patients who were analyzed in the study were treated in our department with ruptured anterior communicating artery (ACoA) aneurysms. We compared the rate of shunting and clinical outcome in patients in whom only fenestration of the LT (Group 1) was performed with that in patients in whom fenestrations of both the LT and LM (Group 2) were performed. RESULTS: Chronic hydrocephalus requiring shunting amounted to 9.8% (7 patients) in Group 1 and 4% (3 patients) in Group 2 (p=0.203). Also, there were no differences in the rate of shunt dependent hydrocephalus between the two groups in patients with Fisher's CT grades 3 (p=0.343) and 4 (p=0.667), and HH grades 4 (p=0.306) and 5 (p=0.361). Favorable clinical outcomes were observed with rates of 74.6% in Group 1 and 79.7% in Group 2 (p=0.693). Also there were no differences in the rates of favorable clinical outcome between the two groups in patients with Fisher's CT grades 3-4, HH grades 4-5. CONCLUSIONS: Our study shows that fenestration of the LM coupled with the opening of the LT reduced-relatively-the incidence of SDCH; this however was not significant. This positive effect was particularly noticeable in patients in whom a cisternal "overflow" was observed at surgery when opening the LM. This corresponded to cases with ventricular dilatation and a IVth ventricle with clots.

Effect of temporary clipping on frontal lobe functions in patients with ruptured aneurysm of the anterior communicating artery.

BACKGROUND: After surgery for ruptured anterior communicating artery (ACoA) aneurysm, several patients who have achieved a favorable neurological outcome yet have been observed to suffer from a poor cognitive outcome. The aim of this study was to explore the possible effects of temporary clip applications on frontal lobe functions in the patients with ruptured ACoA aneurysm. METHODS: Forty patients were chosen among a series of cases who underwent an early surgery (within 96 h) after ACoA aneurysm bleeding. All of them were in Hunt-Hess grade 1 or grade 2. Of the 40 patients, temporary clipping was used in 22 patients (group A), whereas it was not used in 18 patients (group B). These two groups were compared with 20 volunteers (group C) without neurologic or psychiatric disorders. RESULTS: The mean duration of temporary vessel occlusion for both A1 was 8.2 +/- 2.9 min (4-15) in group A. Neither clinical nor radiographic strokes were detected. An improvement in frontal lobe function occurred at long term in group B patients. Whereas, cognitive deficits were persisting at long-term follow-up in group A, especially in patients who had temporary clipping duration longer than 9 min. CONCLUSIONS: The results emphasize that the negative effects of temporary vessel occlusion on cognitive changes occur before ischemic damage. Thus, such negative effects of temporary clipping on cognitive functions should not be neglected by surgeons during surgery.

Primary primitive neuro-ectodermal tumor of cauda equina with intracranial seeding.

Primitive neuro-ectodermal tumor (PNET) of the cauda equina is a rare entity. 18 cases have been reported in the literature so far, including 4 cases with intracranial seeding. Moreover parenchymal involvement of brain has never been reported as a form of intracranial seeding from PNET of the cauda equina. A 31 year-old female patient, with PNET of cauda equina showing intracranial seeding 6 months after surgery, is presented in this report. To our knowledge, this is the first adult case of spinal cord PNET with parenchymal involvement of brain. The histopathological, clinical and radiological findings as well as treatment of the patient were evaluated.

Angiographic follow-up after surgical treatment of intracranial aneurysms.

This study was undertaken to evaluate the long-term angiographic outcome of surgically treated aneurysms. In addition, the incidence of recurrent aneurysms, the fate of residual aneurysms and the de novo formation of aneurysms were evaluated. Moreover, morphological conditions such as dilatation, stenosis or irregularity in temporarily clipped vascular segments were analyzed.166 aneurysms were operated on in 136 patients and these 166 aneurysms subsequently underwent late follow-up angiography. Late angiographic follow-up review was obtained at a mean of 46.6+/-11.5 months postsurgery (range 36-85 months). Out of the 7 aneurysms with known residua, 5 residual aneurysms were determined as unchanged, 1 residual aneurysm as spontaneous thrombosis and 1 residual aneurysm as enlarged. No recurrent aneurysm was found, however two de novo aneurysms were found. During the surgery of 85 aneurysms, 137 vascular segments clipped temporarily were evaluated in terms of morphological changes in postoperative and long-term angiograms. No morphological changes were determined in any vascular segments. These anatomical long-term results confirm the long-term efficacy of aneurysm clipping, when perfect. Small residual aneurysms can be followed with periodically performed angiography.

Risk factors and infant outcomes associated with umbilical cord prolapse.

OBJECTIVES: Our goal was to identify risk factors associated with umbilical cord prolapse and to review the perinatal outcome of cases of cord prolapse. METHODS: During the study years 77 cases were identified retrospectively. Associations between cord prolapse and potential risk factors were evaluated by means of the odds ratio. RESULTS: Of the 77 fetuses with umbilical cord prolapse 9.1% had a fetal weight of < 2500 gas compared with 9.4% for fetuses in control group (P > 0.05). The umbilical cord prolapse occurred in association with vertex presentation 66 times (85.7%), breech presentation nine times (11.7%) and transverse presentation two times (2.6%). The occurrence of breech presentation among the control cases was 2.6%, and that of transverse lie was 1.7% (P < 0.01). Case mothers were 1.6 times more likely to be multiparas compared with the control group (P < 0.01). The perinatal mortality rate was 39/1000. CONCLUSIONS: Our study showed that abnormal fetal presentation and multiparity are associated with an increased risk of umbilical cord prolapse. The association of risk of cord prolapse with low birth weight was statistically insignificant (P > 0.05). We suggest that the cesarean delivery decreased the risk of perinatal mortality among cases of cord prolapse.

Complications of closed continuous lumbar drainage of cerebrospinal fluid.

Sixty three patients who had a lumbar subarachnoid catheter placed for closed continuous cerebrospinal fluid drainage and the complications are presented. The drain was successful in achieving the desired goal in 59 patients (93,6%). The complications are mainly divided into 3 groups; A - complications related to alterations in CSF drainage rate, B - complications due to mechanical failure of the catheter, C - infection. The overall complication rate is found to be 44,4%. Overdrainage, pneumocephalus and meningitis are found to be the most severe complications, but most of these complications are reversible with early recognition. Unfortunately one patient died following meningitis and hepatic failure. Lumbar subarachnoid drainage is a safe method unless the development of any neurological findings should prompt rapid discontinuation of lumbar drainage and immediate radiographic evaluation.

A case report of prenatally diagnosed ophthalmo-acromelic syndrome type Waardenburg.

Ophthalmo-acromelic syndrome type Waardenburg is an extremely rare autosomal recessive syndrome comprising eye malformations ranging from true anophthalmia to mild microphthalmia with acromelic malformations. We report a case of ophthalmo-acromelic syndrome type Waardenburg diagnosed prenatally.

Effects of the temporary clipping in aneurysm surgery on the remnant.

The residual aneurysm rate is reported between 3,8% and 21% in the cases followed after intracranial aneurysm surgery. In the formation of the residual aneurysm, the risk factors include such structural characteristics as the size and lobulation of the aneurysm, posterior circulation, para-ophthalmic localisation and intra-operative rupture. The rates and causes of postoperative residual aneurysms were analyzed in 186 intracranial aneurysm of 160 patients, including the possible effects of temporary clipping on the residual rates. The entire series demonstrated a residual rate of 7%. It was found higher in the large lobulating aneurysms and intra-operative rupture. The residual rate considerably decreased to 4,2% in the aneurysms with temporary clipping. The determination of residual aneurysms, identification of any risk factors and elimination of recoverable factors would allow improvement of surgical results in the treatment of the intracranial aneurysms in the future. Moreover we believe that these will be useful in development of the indications for alternative treatment methods.

Inevitable hysterectomy despite conservative surgical management in advanced cervical pregnancy: a case report.

A 35-year-old woman was diagnosed to have cervical pregnancy of 10.2 weeks duration. Methotrexate therapy was not chosen due to the presence of active tuberculosis. Since the patient had gradually increasing vaginal hemorrhage, she was taken to the operating room. A dilatation and curettage could not control the hemorrhage. A laparotomy was performed and the uterine arteries were ligated. A cervical hysterotomy was also performed to evacuate the products of conception. There was active bleeding in the cervix although no products of conception were observed. Two purse string sutures were placed, but the persistence of profuse bleeding despite these measures necessitated total hysterectomy.

Pregnancy complicated by Evan's syndrome.

Evan's syndrome was initially diagnosed in a 26-year-old pregnant patient. Following the introduction of high dose steroid therapy, the patient developed possible disseminated gonococcal infection which was followed by preterm labor and abruptio placentae. A cesarean delivery was performed at the 34th week of pregnancy following platelet infusion. While the infant survived, the mother had delayed postpartum hemorrhage.

Primary hyperparathyroidism in an infant with three parathyroid glands and pulmonary calcinosis.

A 2 month-old male infant presented with severe hypercalcemia due to parathyroid hyperplasia. A total parathyroidectomy and partial heterotopic autotransplantation were carried out. Hypercalcemia recurred two months later. Normocalcemia was re-established after removing one half of the implanted tissue. Despite two separate surgical explorations and several imaging studies, including 99mTc-sestamibi scintigraphy, ultrasonography, and MRI, only three parathyroid glands were found. Severe pulmonary calcinosis has not previously been reported in children with PHPT. In conclusion, developmental variations of the parathyroid glands may be difficult to identify with present imaging techniques. This may pose difficulties in management of PHPT. The present report describes pulmonary calcinosis as a sequela which can cause additional morbidity in these infants.

Spinal canal stenosis at the level of axis.

We describe a rare case of marked segmental stenosis of the axis secondary to developmental hypertrophy of the posterior neural arch causing severe neck pain and headache in the occipital region. The patient made a remarkable recovery following decompressive laminectomy and foraminal decompression.

Bilateral cerebellopontine angle arachnoid cysts: case report.

OBJECTIVE AND IMPORTANCE: A rare case of bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs), accompanied by cerebellar tonsillar displacement toward the foramen magnum, is presented. CLINICAL PRESENTATION: A 45-year-old woman presented with progressive dysphagia, vertigo, and truncal ataxia. Magnetic resonance imaging revealed bilateral CPA ACs and cerebellar tonsillar displacement. INTERVENTION: The right CPA AC was excised via a suboccipital approach. Decompression of the foramen magnum and duraplasty were also performed. CONCLUSION: The case reported here is the first case of bilateral CPA ACs. Decompression of the foramen magnum and excision of the cyst resulted in complete relief of symptoms.

Proof of the patent subcutaneous fibrous tract in children with V-P shunt malfunction.

A connective tissue sheath that forms around the peritoneal catheter of silicone ventriculo-peritoneal (V-P) shunt tubing is quite often observed in children with V-P shunts. However, proof of the passage of cerebrospinal fluid (CSF) through these sheaths has been reported in only one published study to date. We present four cases associated with chronic malfunction of the V-P shunt peritoneal catheter. In these cases, CSF passage through the subcutaneous fibrous tract, which had a pericatheter connective tissue sheath, was demonstrated around the V-P shunt peritoneal catheter. In the first case the patient suffered intermittent headache attacks over a long period of time; abdominal migration of the peritoneal catheter was detected. The second patient, who had been asymptomatic in the follow-up period with an outgrown peritoneal catheter, was admitted with acute hydrocephalus symptoms. A peritoneal catheter disconnection was detected in another patient, who had had multiple shunt revisions previously. In the last case, an obstruction of the peritoneal catheter was detected. The existence of the subcutaneous fibrous tract and its function were demonstrated by radio-opaque shuntogram in two cases and radionuclide shuntogram in the other two cases. In all four cases V-P shunt revision was performed. Within this study, the possible passage of CSF through a fibrous tract in cases of migration, outgrowth, disconnection or obstruction of the peritoneal shunt catheter was demonstrated. In conclusion, patients with shunt malfunction with a well-grown pericatheter fibrous sheath who are either asymptomatic or minimally symptomatic and show no evidence of active ventricular dilatation on their cranial CT scan should not be regarded as having arrested hydrocephalus until radio-opaque or radionuclide shuntogram studies have been done.