This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (22)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Jimenez, D. F.
Right arrow Articles by Baker, L.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Jimenez, D. F.
Right arrow Articles by Baker, L.
Related Collections
Right arrow Surgery
PEDIATRICS Vol. 110 No. 1 July 2002, pp. 97-104

Early Management of Craniosynostosis Using Endoscopic-Assisted Strip Craniectomies and Cranial Orthotic Molding Therapy

David F. Jimenez, MD, FAAP, FACS*,{ddagger},#, Constance M. Barone, MD, FAAP, FACS§,#, Cathy C. Cartwright, RN-C, MSN, PCNS|| and Lynette Baker, RN, BSN

* Departments of Neurological Surgery
{ddagger} Pediatric Neurosurgery
§ Plastic Surgery
|| Neurosurgery
Plastic and Reconstructive Surgery
# Center for Craniofacial Disorders, University of Missouri Hospital and Clinics, Columbia, Missouri


   ABSTRACT
TOP
 ABSTRACT
INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Objective. To assess the safety, efficacy, and results of the early treatment of infants with craniosynostosis using minimally invasive endoscopic strip craniectomies and postoperative helmet molding therapy.

Methods. A total of 100 patients with documented diagnosis of craniosynostosis were prospectively studied and treated with endoscopic strip craniectomies. A total of 106 stenosed sutures were operated on with the following distribution: 61 sagittal, 23 coronal, 18 metopic, and 4 lambdoid sutures. Sixty-three patients were treated under 16 weeks of age. After surgery, all patients were treated with custom-made molding helmets for up to 7 months. Follow-up ranged between 4 months and 50 months.

Results. All patients underwent the surgical procedures successfully and without complications. The mean surgical operative time was 52.7 minutes. The mean estimated blood loss was 26.2 mL; only 1 patient underwent intraoperative blood transfusion, and 10 patients had a non- life-threatening postoperative blood transfusion. All but 3 patients were discharged on the first postoperative day. There were no infections, dural sinus tears, cerebrospinal fluid leaks, or neurologic injuries, and there were no significant complications related to the use of helmet therapy. Most patients have achieved or are in the process of reaching normalization of their craniofacial deformities.

Conclusions. The results indicate that the early treatment of craniosynostosis with minimally invasive endoscopic strip craniectomies is a safe, efficacious, and valuable therapeutic alternative to the current extensive surgical treatment modalities. The significantly less blood loss, need for blood transfusions, and length of stay and decreased costs make this procedure an excellent early option for treating infants who present with craniosynostosis.

Key Words: craniosynostosis • craniectomy • endoscopy • cranial suture • deformation • helmets


   INTRODUCTION
TOP
 ABSTRACT
 INTRODUCTION
METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
Abnormal head shape in infants presents a difficult diagnostic dilemma for the treating pediatrician and a therapeutic challenge for the surgeon. Most commonly a result of deformational forces associated with birth, the majority of these cranial abnormalities will correct on their own during the first weeks of life. Persistent and progressive abnormal head shape should be carefully and thoroughly evaluated. Craniosynostosis is a common cause of deformational cranial changes associated with premature closure of the calvarial sutures and has an estimated frequency on the order of 0.4/1000.1 Sagittal synostosis is the most prevalent form; it occurs with an estimated frequency of 1 case per 1000 live births. In order of decreasing frequency, the other sutures involved are the coronal, metopic, and lambdoid sutures. Surgical treatment of craniosynostosis was first undertaken by Lannelongue in 1892 for the correction of sagittal synostosis.2 Since then, a myriad of surgical procedures have been developed and used for the treatment of this condition. The surgical approach has varied from simple suturectomies to extensive calvarial vault remodeling. Presented herein is our experience with treating patients using minimally invasive endoscopic techniques followed by cranial helmet molding therapy in infants with craniosynostosis.


   METHODS
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 
A total of 103 consecutive patients who were referred to our craniofacial center with the diagnosis of craniosynostosis were treated between May 1996 and October 2000. There were 72 boys and 28 girls. Their ages ranged between 2 weeks and 9.5 months with a mean of 3.1 months. Of the 61 patients with sagittal synostosis, there were 48 boys and 13 girls. Their mean age at the time of surgery was 3.2 months. Twenty patients presented with coronal synostosis. In this group, there were 12 girls and 8 boys, with a mean age of 3.2 months. Involvement of the metopic suture was seen in 18 patients; 12 were boys and 6 were girls, and their mean age was 4.0 months. Lambdoid synostosis, the least common of all synostosis, was seen in only 4 patients, all boys. Their mean age was 4.1 months (Table 1).


View this table:
[in this window]
[in a new window]
  		TABLE 1. Demographics and Distribution of Patients Treated With Endoscopic-Assisted Craniectomies (n = 100)

 
Surgical Technique
Sagittal Synostosis
After induction under general endotracheal anesthesia, the patient was placed in a modified prone position with the head hyperextended and supported in a padded bean bag (sphinx position). After appropriate skin preparation and draping, a 2- to 3-cm midline transverse incision was made behind the anterior fontanelle. Dissection between the scalp and the skull was undertaken with the aid of a 0° rigid rod lens endoscope (Codman and Shurtleff, Randolph, MA). Needle-tip monopolar electrocautery was used to dissect the areolar plane in a bloodless manner toward the occipital region. A second 2- to 3-cm midline transverse incision was made immediately anterior to the {lambda}. The subgaleal plane dissection was then fully extended between the anterior and posterior incisions.

Transverse midline osteotomies were made with a pediatric 7-mm craniotome and 3-mm Kerrison rongeurs. A 30° rigid endoscope was used to dissect the dura freely off the overlying calvarial bone along the sagittal suture, also extending from anterior to posterior osteotomies. Once the bone was fully dissected, bone cutting scissors were used to make paramedian osteotomies, which allowed the removal of a strip of bone containing the stenosed sagittal suture. The width of the strip varied inversely with the patient’s age. Finally, wedge and barrel stave osteotomies were made bilaterally to allow for bitemporal and biparietal cranial lateral expansion. Hemostasis was obtained with thrombin spray, and the scalp was closed with absorbable galeal sutures and steristrips.

Coronal Suture
The patient was placed supine, and the forehead was prepped appropriately with providone-iodine solution. A 2-cm incision was made at the stephanion (junction of the coronal suture and superior temporal line). Subgaleal dissection was carried inferiorly toward the pterion and the zygomatic areas with the aid of a 0° endoscope and extended medially, toward the anterior fontanelle. A 7-mm burr hole was made and enlarged with Kerrison rongeurs to allow insertion of an endoscope under the bone. The dura was also dissected free from the bone extending from the anterior fontanelle to the lesser wing of the sphenoid bone and temporal fossa. A strip of bone was removed as previously described, and hemostasis was obtained with electrocautery and a generous use of thrombin.

Metopic Suture
The patient’s head was placed supine on a horseshoe headrest. A 2- to 3-cm transverse incision was made immediately behind the hairline over the stenosed metopic suture. Subgaleal dissection was carried down to the nasion and nasofrontal suture with the aid of an endoscope and monopolar electrocautery. A midline osteotomy was made with craniotome and rongeurs to allow passage of the endoscope over the dura, which was also dissected free down to the cribriform plate. Once freely dissected, a 0.5- to 1.0-cm strip of bone was removed, making sure that the removal extended inferiorly to the nasofrontal suture. The bone resection was taken posteriorly to reach the anterior fontanelle. In cases of severe trigonocephaly, the frontal bone osteotomy edges can be smoothed with an electric driven rasper.

Lambdoid Suture
The patient was placed supine with a shoulder roll, and the head was turned sideways in a well-padded horseshoe headrest. In a similar manner, the concept was to remove a strip of bone extending from the {lambda} to the asterion and including the entire stenosed lambdoid suture. Two incisions were made and the first incision was made immediately lateral to the {lambda}. The subgaleal space was dissected as previously described and taken down to the region of the asterion, and the second incision was made immediately medial to the asterion. A craniotome was used to create burr holes at each incision followed by medial and lateral osteotomies overlying the stenosed lambdoid suture. A 30° rigid endoscope was then used to dissect the dura free from the overlying bone, and bone cutting scissors were used to remove a strip of bone containing the stenosed lambdoid suture. Wounds were closed with absorbable suture, and skin steristrips were placed over the scalp. No drains were used in either of the previously described operations.


   RESULTS
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
DISCUSSION
 CONCLUSION
 REFERENCES
 
Sagittal Synostosis
The estimated blood loss ranged between 5 mL and 150 mL, with a mean of 31.6 mL. One patient required an intraoperative blood transfusion of 160 mL, and 9 patients required postoperative blood transfusions (range: 50–230) with a mean of 89 mL. Five of the postoperative blood transfusions occurred in the first 9 patients. None of the postoperative transfusions was associated with life-threatening or unstable clinical states. The preoperative hematocrit ranged between 24% and 37% with a mean of 32.9%. Postoperative hematocrit in the recovery room ranged between 10% and 37% with a mean of 26.1%, for a 23% drop from preoperative levels. The size of the strip craniectomy varied inversely with the patient’s age, and the width ranged between 1.2 cm and 7.5 cm (mean: 4.7 cm) and the length ranged between 4.5 cm and 13 cm (mean: 9.8 cm). The estimated percentage of blood volume loss ranged between 1.3% and 27.1% with a mean of 6.1%. The maximum body temperature on the first postoperative day ranged between 36.4°C and 38.9°C with a mean of 37.8°C. All 61 patients were discharged on the first postoperative day except for 2 patients who were discharged on postoperative day 2 and 1 patient who was discharged on postoperative day 3. The last patient was premature and had developed an apneic episode the evening after surgery, requiring intubation and ultimately respiratory weaning. There were no complications as a result of this episode, and he was monitored for 1 extra day (Figs 1–3).


Figure 1
View larger version (78K):
[in this window]
[in a new window]
  		Fig 1. A, Preoperative AP view of newborn with sagittal synostosis demonstrating bifrontal bossing and bipterional narrowing. B, Eight-month postoperative AP view after an endoscopic wide vertex craniectomy and barrel stave osteotomies shows resolution of frontal bossing and widening of pterional diameter.

 

Figure 3
View larger version (108K):
[in this window]
[in a new window]
  		Fig 3. A, Preoperative lateral view demonstrates marked increased glabella to opisthocranion (AP) distance and significant occipital bossing. B, Eight months postoperatively, the scaphocephalic shape and fully corrected with appropriate foreshortening of the AP distance. The marked frontal and occipital bossing have fully corrected.

 
Coronal Synostosis
The estimated blood loss of patients who underwent coronal craniectomies ranged between 10 mL and 60 mL with a mean of 24.2 mL. No patient required intra- or postoperative blood transfusions. The preoperative hematocrit ranged between 26% and 39% with a mean of 32.2%. Postoperative recovery room hematocrit ranged between 24% and 38% with a mean of 27.1% for a drop of 16.1%. The width of the craniectomy ranged between 0.4 cm and 2 cm with a mean of 0.9 cm. The length of the craniectomy ranged between 5.5 cm and 12 cm with a mean of 9.4 cm. The estimated percentage blood volume loss ranged between 2.1% and 14.9% with a mean 5.0%. The maximum body temperature on the first postoperative day ranged between 36.4°C and 38.4°C with a mean of 37.5°C. All patients were discharged on the first postoperative day (Fig 4).


Figure 4
View larger version (92K):
[in this window]
[in a new window]
  		Fig 4. A, Preoperative AP view of 3.5-month-old girl who presented with right coronal craniosynostosis. Right frontal plagiocephaly is noted with compensatory left frontal bulging. The right orbit is elevated and retracted (vertical dystopia) in relation to the left orbit. Right eye has rounded shape versus oblong shape of left eye. Nasal deviation is noted as well. B, Postoperative view 8.5 months after endoscopic-assisted strip craniectomy of the right coronal suture. All of the deformities described in A have fully resolved. Note correction of vertical dystopia and straightening of the nose.

 
Metopic Synostosis
The estimated blood loss of patients who underwent metopic craniectomy ranged between 10 mL and 150 mL with a mean of 36.5 mL. No patient required an intraoperative blood transfusion. Three patients required postoperative transfusions of 42 mL, 70 mL, and 80 mL, respectively. The preoperative hematocrit ranged between 27% and 38% with a mean of 33.4%. The postoperative recovery room hematocrit ranged between 12% and 33% with a mean of 25%. The craniectomy width ranged between 0.5 cm and 2.0 cm with a mean of 0.9 cm. The length of the craniectomy ranged between 7.0 cm and 10 cm with a mean of 9.0 cm. The estimated percentage blood volume loss ranged between 2.3% and 26.8% with a mean of 7%. The maximum body temperature on the first postoperative day ranged between 36.6°C and 38.1°C with a mean 37.1°C. All patients were discharged on postoperative day 1 (Fig 5).


Figure 5
View larger version (82K):
[in this window]
[in a new window]
  		Fig 5. A, A 4.5-month-old boy presented with metopic suture craniosynostosis and classical findings of trigonocephaly. Triangular forehead with a prominent midline ridge develops secondary to restricted bone growth perpendicular to the metopic suture. Marked bitemporal narrowing is seen with recession of the supraorbital rims and bilateral pseudoproptosis. B, Nine months after endoscopic strip craniectomy of the metopic suture, the patient has achieved normocephalic shape with proper contour of the forehead and correction of the supraorbital rim recession.

 
Lambdoid Synostosis
The estimated blood loss ranged between 5 mL and 20 mL with a mean of 12.5 mL. There were no intra- or postoperative blood transfusions. Preoperative hematocrit ranged between 25% and 38% with a mean of 33.25%. Postoperative hematocrit ranged between 30% and 31.7% with a mean of 31.1%. The width of the craniectomy ranged between 1 cm and 3 cm with a mean of 2 cm, and the length ranged between 4.5 cm and 10 cm with a mean of 7.6 cm. The estimated percentage blood volume loss ranged between 0.9% and 3.17% with a mean of 2.04%. The maximum temperature on the first postoperative day ranged between 37.2°C and 38.2°C with a mean of 37.8°C. All patients were discharged on the first postoperative day(Table 2).


View this table:
[in this window]
[in a new window]
  		TABLE 2. Summary Data for Patients Treated With Endoscopic Strip Craniectomies

 
The small amount of scalp swelling that occurs after surgery is allowed to resolve, and within 5 days a positive plaster-of-Paris mold is made of the patient’s head. A customized helmet made of polypropylene is manufactured to constrict growth in areas of abnormal overgrowth and expansion in areas of undergrowth proportionally. The helmet is worn 20 to 23 hours per day during the extremely rapid brain growth phase that occurs in the first 6 to 12 months of life. At approximately 1 year of age, the helmet therapy is discontinued. Depending on the patient’s age, 2 or 3 helmets may need to be constructed and used by the patient (mean: 2.4).

For all patients, there were no injuries to sagittal, transverse, or sigmoid sinuses. There was 1 dural tear (5 mm) but no cerebral spinal fluid leaks. There were no neural or visual injuries, infections, transfusion-related complications, or postoperative hematomas, or mortalities. The majority of the patients had minor swelling at the operative site, which resolved within 2 to 4 days. One patient had a 0.5-cm full-thickness skin burn with the electrocautery unit, which was primarily repaired and healed without problems. Five patients developed small areas of superficial incisional breakdown and recurrent scabbing (0.5–1.5 cm) and subcutaneous absorbable suture exposure. Treatment included removal of exposed suture, scab removal, and aeration of affected area by temporarily removing the helmet or creating a fenestration over the involved area. Healing has occurred in all cases without untoward effects. All but 6 scaphocephalic patients have achieved normocephaly. Four of those patients were older (>6 months), and 2 had significant compliance problems with the use of the helmet. In patients with metopic synostosis, the hypotelorism corrected after the strip craniectomy and the intracanthal distance increased by an average of 0.5 cm. Bifrontal narrowing improved in all patients. In patients with coronal synostosis, 18 have corrected their vertical dystopia and the deviation of the nose and nasion have also corrected. The last area to improve is the frontal plagiocephaly, which still at 32 months’ follow-up continues to improve slowly.


   DISCUSSION
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
CONCLUSION
 REFERENCES
 
Although clinical descriptions of craniosynostosis date back to Hippocrates and Galen,3 the first modern scientific investigator to describe the anatomic structure of calvarial sutures and the results of their premature closure was Sömmering in 1800.4 However, it was Rudolf Ludwig Carl Virchow, accomplished and prominent German pathologist, who in 1851 first coined the term " craniostenosis."5 That same year, he proposed the classic theory that states that bone growth occurs perpendicular to an open suture and, consequently, premature fusion of the suture will result in calvarial deformities secondary to restrictive bone growth perpendicular to the closed suture and compensatory bone growth in directions parallel to the fused suture. He was also the first to note the association between skull shape and suggested brain pathology as a consequence of abnormal calvarial shape.

Subsequently, American surgeon L.C. Lane performed the first surgical procedure to release a stenosed suture in a 9-month-old with microcephaly.6 French surgeon Lannelogue, in 1890, performed bilateral strip craniectomies for correction of sagittal suture synostosis.2 Jacobi in 1894 described his results in treatment of 33 microcephalic patients presumed to have craniosynostosis. He reported poor results with a high mortality rate, which meant the end of craniosynostosis surgery for the next 30 years.7 Faber and Town by 1927 introduced the idea of surgical treatment for craniosynostosis to prevent blindness and other associated complications, and during the following 14 years, these authors successfully treated patients with low morbidity and mortality, ultimately suggesting that optimal time for surgery was 1 to 3 months of age.8 Consequently, the concept of surgical correction for craniosynostosis became widely accepted, and many surgical techniques have been introduced in the ensuing years.

For the treatment of scaphocephaly secondary to sagittal suture synostosis, these procedures have included bilateral strip craniectomies (resection of strips of bone, of various widths, on either side of the midline extending from the coronal to the lambdoid sutures); wide-strip craniectomy with bilateral wedge parietal craniectomies (resection of large midline section of bone with wedge resections of parietal bones inferolateral to the squamosal sutures bilaterally); sagittal craniectomy with biparietal morcellation (removal of sagittal suture with piecemeal resection and replacement of parietal bones); subtotal calvarectomy, total vertex craniectomy (complete resection of top of calvaria from coronal sutures to lambdoid sutures); midline craniectomy with occiput resection, pi procedure (resection of strips of bone resembling the Greek letter pi ({pi}): bilateral paramedian strip craniectomies and a transverse craniectomy at the level of the coronal sutures); and total calvarial removal and reconstruction with multiple bone flap transpositions1,9–15 (complete resection of frontotemporal parietal and occipital skull; resected skull is then sectioned into multiple fragments, which are replaced and secured in place with plates and screws).5 Likewise, correction of coronal synostosis has involved simple strip craniectomies,16,17 lateral canthal advancement18 (unifrontal craniotomy, greenstick fracture of the supraorbital rim and forward advancement); unilateral tongue-in-groove fronto-orbital advancement19 (unifrontal craniotomy, advancement of supraorbital rim and temporal bone in a tongue-in-groove manner); and, more recently, bifrontal craniotomies with orbital frontal bandeau advancement.10,20 (bifrontal craniotomy and resection of strip of bone containing bilateral supraorbital rims, nasion, and temporal bone; the asymmetric construct is reshaped to fit a normal contour and replaced with plates and screws). The last procedure and variations thereof have also been commonly used for the treatment of metopic craniosynostosis.24–31 Last, lambdoid synostosis has been treated with simple linear craniectomies as well as total occipital calvarial removal and reconstruction.32–36 The majority of these complex procedures are lengthy (6+ hours) and almost universally require blood transfusions with an estimated percentage of blood volume loss ranging from 25% to as high as 500%.37,38 Significant fevers and facial swelling are common during the postoperative period.

Although attempting to improve surgical outcomes, these procedures have increased in complexity and duration. As craniofacial teams have formed (neurosurgeons, plastic surgeons, anesthesiologists, and others), so has the number of procedures as well as the invasiveness and extensiveness of these procedures. Among these are sagittal strip with circular occipital and parietal wedge osteotomies, keyhole craniectomies, bilateral parietal flap craniectomies, total vertex craniectomy, pi and reverse pi procedures, and other types of calvarial vault reconstructions.12–15 Reported blood loss associated with these operations range from a few milliliters to up to 1500 mL, operative surgical times have ranged from 4 to 8 hours, and length of hospitalizations ranged from 4 to 7 days.39,40 Blood transfusions are universal and have ranged from as little as 25% of the estimated blood volume for patients undergoing simple linear strip craniectomies to as high as 500% for calvarial vault reconstructions(Table 3). Needless to say, these surgeries have been associated with significant complications, including coagulopathic states, hypotension, venous air embolisms, dural tears, cardiovascular collapse, sagittal sinus tears, hypophosphatemia, hyponatremia, and residual calvarial defects.38,39,41


View this table:
[in this window]
[in a new window]
  		TABLE 3. Representative Data From Repeated Series Treating Craniosynostosis Using Conventional Surgical Techniques

 
Although many authors and health care providers contend that these operations are done only for "cosmetic purposes," this view is contrary to the American Medical Association’s definition of cosmetic and reconstructive surgery. In the policies of the house of delegates of the American Medical Association, the definition of cosmetic surgery is that of "surgery performed to reshape normal structures of the body to improve the patient’s appearance and self esteem."42 However, "reconstructive surgery is performed on an abnormal structure of the body caused by congenital defects, developmental abnormalities, trauma, infection, tumors, or disease and is generally performed to improve the function but may also be done to approximate normal appearance." The results of an abnormally premature sutural closure, which may include scaphocephaly, plagiocephaly, turricephaly, trigonocephaly, vertical dystopia, and increases in intracranial pressures can hardly be said to be normal. As such, the need for surgical treatment should be recognized as a necessity for these patients.

Not completely satisfied with the final results of such extensive operations, we decided to readdress this problem and proposed the following hypothesis. If surgery could be done very early in life in a safe and rapid manner, then simple release of the stenosed suture should allow normal, rapid brain growth to achieve normocephaly. For counteracting the tendency of the cranial vault to revert to premorbid shape after simple craniectomies, the concept of molding helmet use was also introduced. The rapidly growing brain would expand the skull to fit into the normal shape of the helmet. The ease and simplicity of the surgery coupled with the astonishing results of the early patients encouraged us to treat all craniosynostosis using this technique and to prove that the proposed hypothesis was indeed correct (Fig 6).


Figure 6
View larger version (150K):
[in this window]
[in a new window]
  		Fig 6. An 11-month-old girl wearing a surlyn custom-made helmet. The helmet covers the entire calvaria and allows the brain to grow and fill its shape to obtain normocephaly and prevent the head from returning to premorbid shape.

 
Our results indicate that although extensive and invasive procedures can give very good results in patients who are treated for craniosynostosis, a less invasive approach can give comparable and perhaps better results. Historically, surgeons have requested that infants with craniosynostosis be referred for treatment at a later age (6–12 months). Given the extreme amount of blood loss associated with these procedures, that is a reasonable approach. Difficulties with calvarial vault remodeling include inadequate calvarial shape correction, improper skull reossification, palpable and visible "bumps and lumps," loosening of titanium screws and plates and/or wires, migration of screws into the brain parenchyma through the dura, extensive blood losses, and problems associated with blood transfusion reactions. Surgical times range from 3 to 8 hours, postoperative intensive care unit stays vary from 1 to 4 days, and overall hospital stays may be up to a week in length.

Review of our results with an endoscopic approach to the treatment of craniosynostosis demonstrates that the best results are obtained when patients are referred very early in life. We use the concept of rapid and exponential brain growth that takes place during the first 6 months of life to achieve normocephaly. Our results indicate that the best time to perform this procedure is under 6 months of age, preferably at 3 months. Because our techniques are minimally invasive and surgery can be done quickly, concern for massive blood losses are minimized. Only 1 patient required an intraoperative blood transfusion, and of those patients who received a transfusion after surgery, none was done under life-threatening conditions. Of the 103 patients whom we treated endoscopically, only 4 patients stayed in the hospital longer than 1 day. There were no blood transfusion reactions or electrolyte or metabolic problems. Careful anthropometric and photographic documentation has shown that the majority of the patients have achieved normocephaly or significant correction, as shown in Figs 1 through 5. The overall skull smoothness and contour has been that of normal children with no problems associated with metallic rigid fixation (or lack thereof). Analysis and comparison of hospital charges for patients who undergo endoscopic craniectomy reaches approximately $14 000 (current Midwest hospital charges). This is compared with a cohort of 50 patients who underwent calvarial vault remodeling with an average cost close to $39 000 (current Midwest hospital charges).

We have chosen to use custom-made polypropylene helmets to counteract the dural driven tendency of scaphocephalic patients to revert to presurgical calvarial shapes. In trigonocephalic patients (metopic synostosis), helmets restrict progressive midline central frontal growth while allowing lateral fronto-orbital expansion to achieve a rounded forehead. Similarly, in patients with frontal plagiocephaly (coronal synostosis), helmeting restricts further excessive compensatory contralateral frontal growth while allowing ipsilateral fronto-orbital advancement. We believe that adequate helmet fitting and usage are critical in obtaining superior results. Given cranial growth dynamics, the helmet should be worn to close to 1 year of age. Once the patients have obtained normocephaly at 12 months, that shape will most likely be maintained thereafter. With an approximate cost per helmet of $700, the cost and the length of usage of the helmets far outweigh the need for reoperations.


   CONCLUSION
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
REFERENCES
 
New, less invasive techniques for the early treatment of infants with craniosynostosis have been presented. These techniques have excellent results with extremely low morbidity and no mortalities and are associated with low need for blood transfusions, lower hospitalization costs, and shorter hospital stays. However, early diagnosis of craniosynostosis and prompt referral for surgical evaluation are paramount in obtaining the best possible results for patients who are affected by craniosynostosis.


Figure 2
View larger version (69K):
[in this window]
[in a new window]
  		Fig 2. A, Preoperative aerial view of newborn with sagittal craniosynostosis illustrates the typical findings of scaphocephaly. Noted are marked bifrontal bossing, increased anterior-posterior length, and biparietal narrowing and occipital cupping. B, Postoperative aerial view of 8 months after endoscopic surgery. Significant changes are noted with decreased AP distance, increased biparietal distance (increased cephalic index), and overall normalization of the head shape.

 

   FOOTNOTES
 
Received for publication Aug 30, 2001; Accepted Feb 15, 2002.

Reprint requests to (D.F.J.) Division of Neurological Surgery, University Hospital and Clinics, One Hospital Dr, Columbia, MO 65212. E-mail: jimenezd{at}health.missouri.edu


   REFERENCES
TOP
 ABSTRACT
 INTRODUCTION
 METHODS
 RESULTS
 DISCUSSION
 CONCLUSION
 REFERENCES
 

  1. Hunter AGW, Rudo NL. Craniosynostosis I. Sagittal synostosis: its genetics and associated clinical findings in 214 patients who lacked involvement of the coronal suture(s). Teratology.1976; 14 :185 –193[CrossRef][ISI][Medline]
  2. Lannelongue M. De la craniectomie dans la microcéphalie. Compt Rend Seances Acad Sci.1890; 50 :1382 –1385
  3. Cohen MM, ed. Craniosynostosis: Diagnosis, Evaluation and Management. New York, NY: Raven Press; 1986:1–20
  4. Sömmering ST. Vom Baue des Menschlichen Körpers. 1st ed. Leipzig, Germany: Voss; 1800
  5. Virchow R. Üeber den Cretinismus, namentlich in Franken, und über pathologische Schädelformen. Verh Physikalisch Med Ges Wurzburg.1851; 2 :230
  6. Lane LC. Pioneer craniectomy for relief of mental imbecility due to premature sutural closure and microcephalus. JAMA.1892; 49 –50
  7. Jacobi A. Non nocere. M Rec.1894; 45 :609
  8. Faber HK, Towne EB. Early craniectomy as preventive measure in oxycephaly and allied conditions with special reference to the prevention of blindness. Am J Med Sci.1927; 173 :701
  9. Albright AL. Operative normalization of the skull shape in sagittal synostosis. Neurosurgery.1985; 17 :329 –331[ISI][Medline]
  10. Greene CS Jr, Winston KR. Treatment of scaphocephaly with sagittal craniectomy and biparietal morcellation. Neurosurgery.1988; 23 :196 –202[ISI][Medline]
  11. Epstein N, Epstein F, Newman G. Total vertex craniectomy for the treatment of scaphocephaly. Childs Brain.1982; 9 :309 –316[ISI][Medline]
  12. Venes JL, Sayers MP. Sagittal synostectomy. Technical note. J Neurosurg.1976; 44 :390 –392[ISI][Medline]
  13. Jane JA, Edgerton MJ, Futrell JW, et al. Immediate correction of sagittal synostosis. J Neurosurg.1978; 49 :705 –710[ISI][Medline]
  14. Marchac D, Renier D. Craniofacial Surgery for Craniosynostosis. Boston, MA: Little Brown; 1982:87–92
  15. Rougerie J, Derome P, Anquez L. Craniosténosis et dysmorphies cranio-faciales. Principes d’une nouvelle technique de traitement et ses résultats. Neurochirurgie.1972; 18 :429 –440[ISI][Medline]
  16. McLaurin RL, Matson DD. Importance of early surgical treatment of craniosynostosis. Review of 36 cases treated during the first six months of life. Pediatrics.1952; 10 :637 –652[Abstract/Free Full Text]
  17. Shillito J Jr, Matson DD. Craniosynostosis: a review of 519 surgical patients. Pediatrics.1968; 41 :829 –853[Abstract/Free Full Text]
  18. Hoffman HJ, Mohr G. Lateral canthal advancement of the supraorbital margin. A new corrective technique in the treatment of coronal synostosis. J Neurosurg.1976; 45 :376 –381[ISI][Medline]
  19. McCarthy JG, Glasberg SC, Cutting CB, et al. Twenty-year experience with early surgery for craniosynostosis: I. Isolated craniofacial synostosis: results and unsolved problems. Plast Reconstr Surg.1995; 96 :272 –283[ISI][Medline]
  20. Jane JA, Park TS, Zide BM, Lambruschi P, Persing JA, Edgerton MT. Alternative techniques in the treatment of unilateral coronal synostosis. J Neurosurg.1984; 61 :550 –556[ISI][Medline]
  21. Persing JA, Jane JA, Delashaw JB. Treatment of bilateral coronal synostosis in infancy: a holistic approach. J Neurosurg.1990; 72 :171 –175[ISI][Medline]
  22. Mohr G, Hoffman HJ, Munro IR, Hendrick EB, Humphreys RP. Surgical management of unilateral and bilateral coronal craniosynostosis: 21 years experience. Neurosurgery.1978; 2 :83 –92[Medline]
  23. Sgouros S, Goldin JH, Hockley AD, Wake MJC. Surgery for unilateral coronal synostosis (plagiocephaly) unilateral or bilateral correction. J Craniofac Surg.1996; 7 :284 –289[ISI][Medline]
  24. Cohen SR, Kawamoto HK Jr, Burstein F, Peacock WJ. Advancement-onlay: an improved technique of fronto-orbital remodeling in craniosynostosis. Childs Nerv Syst.1991; 7 :264 –271[CrossRef][ISI][Medline]
  25. Cohen SR, Maher H, Wagner JD, Dauser RC, Newman MH, Muraszko KM. Metopic synostosis: evaluation of aesthetic results. Plast Reconstr Surg.1994; 94 :759 –767[ISI][Medline]
  26. Anderson FM. Treatment of coronal and metopic synostosis: 107 cases. Neurosurgery.1981; 8 :143 –149[ISI][Medline]
  27. Delashaw JB, Persing JA, Park TS, Jane JA. Surgical approaches for the correction of metopic synostosis. Neurosurgery.1986; 19 :228 –234[ISI][Medline]
  28. Howes WJ. Closing wedges: a technique for repair of midline craniosynostosis. Pediatr Neurosurg.1991–92; 17 :21 –22[ISI][Medline]
  29. Shaffrey ME, Persing JA, Delashaw JB, Shaffrey CI, Jane JA. Surgical treatment of metopic synostosis. Neurosurg Clin N Am.1991; 2 :621 –627[Medline]
  30. Czorny A, Forlodou P, Stricker M, Ricbourg B. Les Crânes triangulaires: a propos de 87 cas de trigonocéphalies. Neurochirurgie.1994; 40 :209 –221[ISI][Medline]
  31. Di Rocco C, Marchese E, Velardi F. Craniosinostosi metopica. Minerva Pediatr.1989; 41 :559 –564[Medline]
  32. Persing JA, Delashaw JB, Jane JA, Edgerton MT. Lambdoid synostosis: surgical considerations. Plast Reconstr Surg.1988; 81 :852 –860[ISI][Medline]
  33. Pople IK, Sanford RA, Muhlbauer MS. Clinical presentation and management of 100 infants with occipital plagiocephaly. Pediatr Neurosurg.1996; 25 :1 –6[ISI][Medline]
  34. Vander Kolk CA, Carson BS. Lambdoid synostosis. Clin Plast Surg.1994; 21 :575 –584[ISI][Medline]
  35. Sgouros S, Goldin JF, Hockley AD, Wake MJC. Posterior skull surgery in craniosynostosis. Childs Nerv Syst.1996; 12 :727 –733[CrossRef][ISI][Medline]
  36. Jimenez DF, Barone CM. The Sunrise technique: the correction of occipital plagiocephaly using bandeau occipital plate and radial osteotomies. Pediatr Neurosurg.1994; 22 :162 –166
  37. Meyer P, Rosales JK, Howes WJ. Blood loss during repair of craniosynostosis. Br J Anaesth.1993; 71 :854 –857[Abstract/Free Full Text]
  38. Faberowski LW, Black S, Mahla ME, Mickle JP. Evaluation of blood loss and transfusion practice in the perioperative management of craniosynostosis. J Neurosurg Anesthesiol.1999; 11 :167 –172[ISI][Medline]
  39. Harris MM, Yemen TA, Davidson A, et al. Venous air embolism during craniectomy in a supine infant. Anesthesiology.1987; 67 :816 –819[ISI][Medline]
  40. Moylan S, Collee G, Mackensie A, et al. Anaesthetic management in paediatric craniofacial surgery. A review of 126 cases. Paediatr Anaesth.1993; 3 :275 –281
  41. Imberti R, Locatelli D, Fanzio M, et al. Intra-and postoperative management of craniosynostosis. Can J Anaesth.1989; 36 :948 –989
  42. American Medical Association Policies of House of Delegates. Definitions of "Cosmetic" and "Reconstructive" Surgery, H-475.992. Council Medical Services Annual Meeting, June 1989, Chicago, IL
PEDIATRICS (ISSN 1098-4275). ©2002 by the American Academy of Pediatrics



This article has been cited by other articles:


Home page
 J Pediatr PsycholHome page
M. L. Speltz, K. A. Kapp-Simon, M. Cunningham, J. Marsh, and G. Dawson
Single-Suture Craniosynostosis: A Review of Neurobehavioral Research and Theory
J. Pediatr. Psychol., December 1, 2004; 29(8): 651 - 668.
[Abstract] [Full Text] [PDF]

Home page
 Anesth. Analg.Home page
D. G. Ririe, L. R. David, S. S. Glazier, T. E. Smith, and L. C. Argenta
Surgical Advancement Influences Perioperative Care: A Comparison of Two Surgical Techniques for Sagittal Craniosynostosis Repair
Anesth. Analg., September 1, 2003; 97(3): 699 - 703.
[Abstract] [Full Text] [PDF]

Home page
 PediatricsHome page
J. M. Freeman, B. S. Carson Sr, and T. A. Maugans
Management of Infants With Potentially Misshapen Heads
Pediatrics, April 1, 2003; 111(4): 918 - 918.
[Full Text] [PDF]

Home page
 PediatricsHome page
T. A. Maugans
The Misshapen Head
Pediatrics, July 1, 2002; 110(1): 166 - 167.
[Full Text] [PDF]

This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Right arrow Citation Map
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via HighWire
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (22)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Jimenez, D. F.
Right arrow Articles by Baker, L.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Jimenez, D. F.
Right arrow Articles by Baker, L.
Related Collections
Right arrow Surgery