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Endoscopic strip craniectomy: a minimally invasive treatment for early correction of craniosynostosis.

Abstract: Traditionally, surgical correction of craniosynostosis involves calvarial remodeling, large blood losses necessitating transfusions, hospital stays of several days, and less-than-satisfactory results. In this study, outcomes from a minimally invasive technique called endoscopic strip craniectomy, along with a postoperative molding helmet, to correct craniosynostosis in young infants were evaluated. The endoscopic strip craniectomy was performed on 185 patients with clinical signs of craniosynostosis, with the following distribution: 107 sagittal, 42 coronal, 37 metopic, and 7 lambdoid, for a total of 198 sutures. The mean blood loss was 29.4 cc, and only two patients underwent intraoperative blood transfusion. Fourteen patients underwent postoperative blood transfusion; none was life-threatening. There were no deaths, complications, neurological injuries, or infections. All but six patients were discharged on the first postoperative day. A majority of the patients achieved or approached normocephaly, and there were no complications. Neuroscience nurses need to be aware of this technique when they discuss treatment options with the families of infants with craniosynostosis.

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Craniosynostosis, or premature closure of the cranial sutures, occurs in 1:2,100 children (Lajeunie et al., 1995; see Fig. 1). Of those, 40%-60% are sagittal, 20%-30% are coronal, and 10% are metopic synostosis (Hunter & Rudd, 1984; Lajeunie et al., 1995, 1996; Shillito & Matson, 1968). Lambdoid synostosis is rare (Sun & Persing, 1999).

[FIGURE 1 OMITTED]

Treatment for craniosynostosis is surgical, although some mistakenly argue that surgery is not necessary because the resulting deformity is cosmetic. The American Medical Association defines cosmetic surgery as "surgery performed to reshape normal structures of the body in order to improve the patient's appearance and self-esteem. Reconstructive surgery is performed on abnormal structures of the body, caused by congenital defects, developmental abnormalities, trauma, infection, tumors, or disease. It is generally performed to improve function, but may also be done to approximate a normal appearance" (American Medical Association Policies of House of Delegates, 1989, p. A-89). Because craniosynostosis is a congenital defect, surgery to correct it is reconstructive; this is an issue of importance for some insurance companies.

Although the condition was recognized in the days of Galen and Hippocrates (Cohen, 1986), the first craniectomy to remove a stenosed suture was performed by Lane in 1888 to treat microcephaly (Lane, 1892). In 1890, Lannelongue treated sagittal synostosis with bilateral strip craniectomies (Lannelongue, 1890). However, the strip craniectomy was abandoned because results were not that pleasing. Over the years, many calvarial remodeling techniques have been used to correct craniosynostosis. Some of these techniques can last several hours, requiring blood transfusions to correct estimated blood loss percentages from 25% to 500% (Jimenez, 2002). Most patients undergoing these procedures had significant facial swelling and remained hospitalized for up to 1 week. Costs for these procedures currently average about $51,000 at University of Missouri Healthcare.

Because of the problems with calvarial remodeling, a less invasive technique was developed, which combined the old approach of strip craniectomy with modern endoscopic technology. The results were further enhanced through the use of a molding helmet to overcome the dural forces that would otherwise cause the head to revert to its former shape. By contrast, costs for the endoscopic strip craniectomy are $14,000 or less. This article discusses the outcomes and nursing care of patients undergoing endoscopic strip craniectomy for craniosynostosis.

Methods

A total of 185 consecutive patients reported clinical signs of craniosynostosis between May 1996 and February 2002. Each patient underwent an endoscopic strip craniectomy and was fitted postoperatively with a custom-made molding helmet. When the bone containing the stenosed suture is removed, the growing brain pushes the skull out into a round shape with the help of a molding helmet. The stenosed sutures included the sagittal, coronal, metopic, and lambdoid sutures for a total of 198 sutures removed. Some patients had multiple suture involvement including the following suture combinations: right coronal/metopic, left lambdoid/sagittal/left coronal, sagittal/metopic, right coronal/ sagittal/metopic, bicoronal/sagittal, and bilateral lambdoid/sagittal; and three patients had bicoronal synostosis. Ages of the patients ranged from 12 days to 9.5 months; there were 121 males and 64 females.

Sagittal

A total of 107 patients had sagittal synostosis, demonstrating the characteristic scaphocephalic shape and various aspects of frontal bossing, occipital cupping, bitemporal narrowing, and palpable midline sagittal ridge. The mean age was 3.61 months; the oldest, 9.5 months; and the youngest, 12 days. Of these, 78 were male and 29 were female.

Coronal

A total of 42 had coronal synostosis, demonstrating the characteristic anterior plagiocephaly and various aspects of vertical dystopia, nasional deviation, and flattening of the frontal bone on the ipsilateral side of the stenosed suture. Of these, 18 were males and 24 were female. The mean age was 3.83 months; the oldest was 8 months; and the youngest, 3 weeks.

Metopic

A total of 37 patients had metopic synostosis, demonstrating the characteristic trigonocephalic shape and various aspects of bitemporal narrowing and hypotelorism. Of these, 22 were male and 15 were female. The mean age was 3.90 months, the oldest was 7.25 months; and the youngest, 1.25 months.

Lambdoid

Six patients had lambdoid synostosis, demonstrating the characteristic posterior plagiocephaly and various aspects of a trapezoid shape to the head when viewed from above, tilted skull base, and a palpable bony ridge along the stenosed suture. Of these, five were male and one was female. The mean age was 4.32 months; the oldest was 6.25 months; and the youngest, 3 weeks. Lambdoid synostosis is differentiated from positional molding, which demonstrates a parallelogram shaped head when viewed from above, anterior displacement of the ear on the affected side, absence of a bony ridge, level ears, and absence of a tilted skull base (Cartwright, 2002).

Preoperative Preparation

A careful history was taken and physical examination performed, noting any family history of craniosynostosis and assessing for multiple suture involvement. Although radio-diagnostics were not routinely ordered, a computed tomography (CT) scan of the brain with bone windows was obtained if there was any question about multiple suture involvement. Preoperative blood-work was not ordered unless the history revealed pathology that would require this, such as a history of bleeding disorder.

Although blood transfusions were not anticipated, parents were given the opportunity to directly donate a unit of blood prior to surgery. Preoperative photographs were obtained on all patients, and a cephalic index was measured on those with sagittal synostosis.

To determine the cephalic index (CI), a spreading cranial calipers (GPM Instruments, Switzerland) was used to measure the euryon-to-euryon distance divided by the distance from glabella to opisthocranium. This number multiplied by 100 gives the CI. The CI is low when the head is scaphocephalic and increases as the head rounds out to reach normocephaly.

Infants with coronal synostosis were referred to a pediatric ophthalmologist for evaluation of vertical dystopia. A genetics referral was made if there was a family history of craniosynostosis or the parents wanted to know the likelihood of this occurring in future pregnancies.

Although 90% of patients came to the preoperative visit as a result of reading the Web site (www. muhealth.org/~neuromedicine), parents who had not seen it were given the address. Some parents found it helpful to talk with other parents who have had this surgery at the hospital, and most families were very willing to share their experiences. Permission was obtained before they were put in touch with each other.

Preoperative feeding instructions included no solids or formula 6 hours prior to surgery, but the infants could have Pedialyte (Ross) or breast milk up until 2 hours before surgery. Parents were allowed to be with their infants until the anesthesiologist took them to the operating room. Parents received a beeper the morning of surgery and were told they would be called when the surgery actually started and when it was completed. To minimize discomfort for the infant (and parents), no intravenous (IV) access was started until the infant was asleep in the operating room.

Intraoperative Procedure

Once the infant was asleep and had received mask induction anesthesia, the W was started. A blood sample for type and screen and hematocrit was drawn, and the endotracheal tube placed. To minimize postoperative pain, an acetaminophen suppository (40 mg/kg) was given. Once the infant was asleep, cephalic measurements were obtained on all infants and intercanthal distance was obtained on the infants with metopic synostosis.

After the infant had been positioned, prepped, and draped, a 2- to 3-cm incision was made in the scalp through which the stenosed suture was to be removed. Only a small amount of hair along the incision line was removed and later given to the parents for their baby book. For sagittal synostosis, a midline transverse incision was made behind the anterior fontanel and anterior to the lambda. For coronal synostosis, a 2-cm incision was made at the junction of the coronal suture and the superior temporal line. For metopic synostosis, a 2- to 3-cm transverse incision was made just behind the hairline. To remove the stenosed lambdoid suture, incisions were made lateral to the lambda and medial to the asterion.

Both 30[degrees] and 0[degrees] rigid endoscopes were used to visualize structures beneath the scalp and aid in dissection and detection of bleeding. In all cases, a strip of bone with the stenosed suture was removed. For sagittal suture synostosis, wedge and barrel stave osteotomies were made bilaterally to allow for biparietal and bitemporal expansion. A mixture of thrombin (for hemostasis) and 0.25% marcaine (for pain control) was sprayed on the dura before closure. The skin was approximated with absorbable sutures. Liquid skin adhesive and paper skin closures were applied to the closed incision. A postoperative hematocrit was drawn just before the infant was awakened.

Postoperative Care

As the infant proceeded to the postanesthesia care unit (PACU), the surgeons and nurses talked with the family, and then the parents were taken into the recovery room to see their infant. In the PACU, parents could hold their infant and offer Pedialyte or breast milk. After about an hour or when the vital signs were stable, the infant was transferred to the pediatric unit. Infants with a history of prematurity or any respiratory distress were transferred to the pediatric intensive care unit for observation. All patients were monitored by using a continuous pulse-oximeter and cardiac/apnea monitor.

Infants alternated between sleep and mild to moderate fussiness for approximately the first 8 hours after surgery. Pain was controlled by alternating acetaminophen (15 mg/kg every 6 hours) and ibuprofen (10 mg/kg every 6 hours) orally, supplementing with IV nalbuphine (0.05-0.2 mg/kg every 2-4 hours) for breakthrough pain. Diet was advanced as tolerated, and the IV was saline locked when the infant was taking oral fluids well, usually in just a few hours after surgery.

In addition to the "boggy" appearance of the scalp around the craniectomy site due to the marcaine/thrombin spray, swelling usually reached its peak on the first postoperative day. Elevating the head helped to decrease this swelling. Infants with sagittal synostosis were positioned with the occiput on the mattress to promote an increase in the biparietal and bitemporal diameter until the helmet was worn.

The hair was washed on the second postoperative day, and the paper skin closures were removed. No other incision care was needed. Parents often needed to be reassured that it was "safe" for the infant to have a strip of bone missing, especially those with older siblings. The dura is tough like shoe leather and will protect the brain until the helmet is worn. Because reossification of new bone comes from the dura and not the bone itself, parents were informed that they would begin to feel "islands" of bone on the dura as the skull reossifies. This is particularly easy to feel on the sagittal craniectomy sites because of the wide strip of bone removed. Parents were told that they should care for their infant as usual and no special precautions needed to be taken. The infant was discharged the first postoperative day if he or she was taking oral fluids well without emesis, vital signs were stable, the postoperative hematocrit drawn earlier that morning was above 17%, and the infant's behavior was back to baseline.

Molding Helmet

The mold for the custom-made polypropylene molding helmet was made 3-4 days after surgery, during which time most of the swelling had subsided (Fig. 2). The finished helmet was fitted on the infant's head by the orthofist, and any further adjustments were made. The parents received detailed instructions on helmet care, how to check for pressure points, how to determine whether the helmet is too small, and when to call with problems (Table 1). The helmet was worn almost continuously for approximately 1 year to overcome the propensity of the calvaria to revert to its preoperative shape as the bone reossifies. The helmet was removed only to check for pressure points, to bathe the infant, and to clean the helmet. The helmet is round with space in the parietal/temporal areas to correct scaphocephaly, space in the bitemporal areas to correct trigonocephaly, and space on the ipsilateral side of the stenosed suture for lambdoid and coronal synostosis. An average of 2 to 4 helmets were needed at a cost of $789 per helmet. Helmets needed to be changed more frequently the first 6 months of age because of the rapid head growth that occurs during that time.

[FIGURE 2 OMITTED]

Follow-up

Parents returned to the cranio-facial and orthotics clinics 4-6 weeks after surgery. The CI was measured and graphed, and pictures were taken so parents could compare these with the preoperative pictures and CI. Other visits were scheduled approximately every 2-3 months or when the helmet became too small. Parents were encouraged to bring their infants in for yearly follow-up, even after they were out of the helmet. An annual craniofacial Christmas party was held in the hospital lobby, complete with Santa Claus and presents for all the children. This party facilitated follow-up in clinic the day before and allowed parents to talk with other parents who have gone through a similar experience.

Results

Sagittal

The estimated blood loss ranged from 5 to 150 cc with a mean of 30.51 cc (Table 2). Two patients received intraoperative blood transfusions of 160 cc and 75 cc, respectively. Ten patients received a postoperative blood transfusion ranging from 50 to 230 cc. However, 6 of these transfusions occurred in the first 14 cases. None of these was associated with life-threatening conditions.

The preoperative hematocrit ranged from 21.6% to 57.9% with a mean of 32.5%. The immediate postoperative hematocrit ranged from 10% to 42% with a mean of 27.3%. The hematocrit on the first postoperative day ranged between 13% and 36.5% for a mean of 23.6%.

The size of the strip of bone removed varied with age. Larger strips of bone were removed from the younger infants, because their age allowed for a longer period for reossification. The size of the strip ranged from 0.5 to 8 cm in width and 1 cm to 15 cm in length, for a mean of 5.3 cm in width and 10.1 cm in length. Operating time ranged between 30 and 150 minutes, including multiple sutures, with a mean of 60.39 minutes.

All but 5 of the 107 patients were discharged on the first postoperative day. Four of those were discharged on the second postoperative day at the parents' request. One infant with a history of prematurity had an apneic spell that necessitated intubation and ventilation. This infant was discharged on the third postoperative day without adverse sequelae due to this episode.

Three infants with sagittal synostosis who underwent endoscopic strip craniectomy are shown pre- and postoperatively in Fig 3-5.

[FIGURES 3-5 OMITTED]

Coronal Synostosis

The estimated blood loss ranged from 5 to 60 cc, with a mean of 19.7 cc (Table 2). None of these patients received a blood transfusion. The preoperative hematocrit ranged from 25% to 46% with a mean of 32.9%. The immediate postoperative hematocrit ranged from 24% to 38% with a mean of 29.69%. The hematocrit on the first postoperative day ranged from 20% to 38% with a mean of 27.9%.

The width of the strip of bone removed ranged between 0.4 and 2 cm with a mean of 0.72 cm. The length of the strip ranged from 5 to 13 cm with a mean of 10.16 cm. Operating time ranged from 25 to 150 minutes for a mean of 56.83 minutes. All patients were discharged on the first postoperative day except for 1 patient who was discharged on the second postoperative day.

An infant with coronal synostosis who underwent endoscopic strip craniectomy is shown pre- and postoperatively in Fig 6.

[FIGURE 6 OMITTED]

Metopic Synostosis

The estimated blood loss ranged from 10 to 150 cc for a mean of 33.7 cc (Table 2). None of these 37 patients received an intraoperative blood transfusion, but 4 received postoperative transfusions. Postoperative blood transfusions ranged from 42 to 80 cc with a mean of 60 cc.

The preoperative hematocrit ranged from 24% to 39% for a mean of 32.3%. The immediate postoperative hematocrit ranged from 12% to 33% with a mean of 25.57%. The hematocrit on the first postoperative day ranged from 18.2% to 31% with a mean of 24.1%

The width of the strip of bone removed ranged from 0.5 to 2 cm with a mean of 0.78 cm. The length of the strip ranged from 7.5 to 11 cm with a mean of 9.46 cm. Operative time ranged from 30 to 90 minutes with a mean of 61.06 minutes.

Infants with metopic synostosis who underwent endoscopic strip craniectomy are shown pre- and postoperatively in Fig 7-8.

[FIGURES 7-8 OMITTED]

Lambdoid Synostosis

The estimated blood loss ranged from 5 to 100 cc with a mean of 33.57 cc (Table 2). The infant with the 100-cc blood loss had a strip of bone removed containing the sagittal suture, as well as strips of bone with partially stenosed bilateral lambdoid sutures. This baby received 75 cc of blood intraoperativel)5 although his condition was not life-threatening. No infants received postoperative transfusions. The width of the bone strip removed ranged from 1 to 5.5 cm with a mean of 2.43 cm. The length of the strip ranged from 4.5 to 13 cm. Operating time ranged from 45 to 150 minutes with a mean of 82.43 minutes.

The preoperative hematocrit ranged from 25% to 46% with a mean of 35.1%. The immediate postoperative hematocrit ranged from 24% to 38% with a mean of 30.1%. The hematocrit on the first postoperative day ranged from 18.7% to 32.2% with a mean of 28.9%. All infants were discharged on the first postoperative day.

Discussion

As anthropomorphic measurements and photographs indicate, all patients improved and a substantial number achieved normocephaly (Fig 3-8). When parents arbitrarily decided to discontinue the helmet before ossification was complete, the CI in the infants with sagittal synostosis dropped, as the dural forces tried to return the skull to its preoperative scaphocephalic shape. Other suture synostoses also failed to progress if the helmet was removed before complete ossification.

There were no deaths, no infections, and no transfusion reactions. Two patients received intraoperative blood transfusions, and 14 patients received postoperative blood transfusions, none with life-threatening conditions (Table 2). The criteria used for transfusion included a hematocrit of less than 17%, tachycardia, tachypnea, pale skin, capillary refill of more than 2 seconds, lethargy, and poor eating. All but five patients were discharged on the first postoperative day.

A few patients developed a superficial skin breakdown with recurrent scabbing of 0.5-1.5 cm at the incision site. These breakdowns were attributed to subcutaneous absorbable suture exposure and moisture inside the helmet. These were treated by removing the scab and applying a wet-to-dry dressing to the lesion until it healed, by removing the helmet for a few days and allowing the lesion to air dry, or by making a small fenestration in the helmet above the lesion to allow air drying. All healed without sequelae.

Summary

This outcomes analysis has demonstrated the safety and efficacy of the endoscopic strip craniectomy for early correction of craniosynostosis, as well as the need to wear a molding helmet to overcome the dural forces that drive reossification. These techniques have produced excellent results, no mortalities, minimal complications, decreased blood loss, shortened hospital stay, and lower costs. Best results are obtained when the procedure is performed on infants yotmger than 6 months, and preferably before 3 months, because of the rapid brain growth during this period.
Table 1. Caring for Your Baby's Helmet

* Wait until you get home to let your baby wear the helmet so
 you can be in more familiar surroundings.
* Let your baby wear it for half a day at first and by the third
 day, he or she should be wearing it all day.
* Take the helmet off 3-4 times each day for 30-40 minutes to
 check for redness. There should be a spot of redness where
 the helmet is putting pressure on a prominent area. This will
 help reshape the skull. This redness should go away in 30
 minutes. If the redness does not go away in 30 minutes,
 leave the helmet off until the redness goes away and then try
 again. Take the helmet off in 3-4 hours to check for redness.
 Call if the redness lasts longer than 30-40 minutes as we do
 not want the skin to break down.

Cleaning
* Clean the inside of the helmet after every removal using a
 damp rag with soap and water.
* Clean the inside of the helmet once or twice a day with alcohol.

Problems
* If a heat rash develops on your baby's head, leave the helmet
 off for 1-2 days until the rash disappears. Continue to clean
 the helmet with alcohol.
* At first there may be a little slippage of the helmet because of
 the excess space left for your baby's skull to expand. Call if
 this slippage seems excessive and the pressure points are
 not visible when the helmet is removed.
* Cover your baby's head with a hat or bonnet if he or she will
 be out in the sun, especially in a hot climate. The scalp can
 get sunburned if exposed to the sun.
* Babies with sagittal suture synostosis may experience some
 redness over the ears. Call if this occurs.

Table 2. Endoscopic Strip Craniectomy Patient Data

 Mean Mean Mean Mean
 No. of Blood Strip Operating
 Patients Loss (cc) Size (cm) Time (min)

Sagittal 107 30.51 5.3 x 10.1 60.3
Coronal 42 19.7 0.7 x 10.1 56.8
Metopic 37 33.7 0.7 x 9.4 61
Lambdoid 6 33.5 2.4 x 8.4 82.4

 Patients
 Receiving Hematocrit (%)
 Post-op
 Blood Mean Mean Post-op
 Transfusions Pre-op Post-op Day 1

Sagittal 10 32.5 27.3 23.6
Coronal 0 32.9 29.6 27.9
Metopic 4 32.3 25.57 24.1
Lambdoid 0 35.1 30.1 28.9


References

American Medical Association Policies of House of Delegates. (1989, June). Definitions of "cosmetic" and "reconstructive" surgery, H-475.992. Council of Medical Services Annual Meeting.

Cartwright, C.C. (2002). Assessing asymmetrical infant head shapes. The Nurse Practitioner Journal, 27, 33-39.

Cohen, M.M. (Ed.). (1986). Craniosynostosis: Diagnosis, evaluation and management. New York: Raven Press.

Hunter, A.G., & Rudd, N.L. (1977). Craniosynostosis II. Coronal synostosis: Its familial characteristics and associate clinical findings in 109 patients lacking bilateral polysyndactyly or syndactyly. Teratology, 15, 3401-3410.

Hunter, A.G., & Rudd, N.L. (1984). Craniosynostosis I. Sagittal synostosis: Its genetics and associated clinical findings in 214 patients who lacked involvement of the coronal suture. Teratology, 14, 185-194.

Jimenez, D.F., Barone, C.M., Cartwright, C.C., & Baker, L. (2002). Early management of craniosynostosis using endoscopic assisted strip craniectomies and cranial orthotic molding therapy. Pediatrics, 110, 97-104.

Lajeunie, E., Le Merrer, M., Bonaiti-Pellie, C., Marchac, D., & Renier, D. (1995). Genetic study of nonsyndromic coronal synostosis. American Journal of Medical Genetics, 55, 500-504.

Lajeunie, E., Le Merrer, M., Bonaiti-Pellie, C., Marchac, D., & Renier, D. (1996). Genetic study of scaphocephaly. American Journal of Medical Genetics, 62, 282-285.

Lane, L.C. (1892). Pioneer endoscopic strip craniectomy for relief of mental imbecility due to premature sutural closure and microcephalus. Journal of the American Medical Association, 18, 49-50.

Lannelongue, M. (1890). De la craniectomie dans la microcephalie. Comptes rendus de l'academie des science, 110, 1382-1385.

Shillito, J., & Matson, D.D. (1968). Craniosynostosis: A review of 519 surgical patients. Pediatrics, 41, 829.

Sun P, & Persing J. (1999). Craniosynostosis. In A.L. Albright, I.F. Pollack, & P.D. Adelson (Eds.), Principles and Practice of Pediatric Neurosurgery (pp. 219-242). New York: Thieme Medical Publishers, Inc.

Questions or comments about this article may be directed to: Cathy C. Cartwright, MSN RN PCNS, by phone at 573/882-3424 or by e-mail at cartwrightc@health.missouri.edu. She is a pediatric clinical nurse specialist in neurosurgery at University of Missouri Healthcare, Columbia, MO.

David F. Jimenez, MD FAAP FACS, is an associate professor of neurological surgery, director of pediatric neurosurgery, and co-director at the Center for Craniofacial Disorders, University of Missouri Healthcare.

Constance M. Barone, MD FAAP FACS, is an associate professor of plastic surgery and co-director at the Center for Craniofacial Disorders, University of Missouri Healthcare.

Lynette Baker, BSN RN, is a clinical nurse coordinator in plastic and reconstructive surgery at University of Missouri Healthcare.
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Author:Cartwright, Cathy C.; Jimenez, David F.; Barone, Constance M.; Baker, Lynette
Publication:Journal of Neuroscience Nursing
Date:Jun 1, 2003
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