Table Of ContentEENNDDOOSSCCOOPPIICC
CCRRAANNIIOOSSYYNNOOSSTTOOSSIISS
SSUURRGGEERRYY AN ILLUSTRATED GUIDE TO
ENDOSCOPIC TECHNIQUES
David F. Jimenez,
MD, FACS
Director
Pediatric Neurosurgery
El Paso Children’s Hospital
El Paso, Texas
Elsevier
1600 John F. Kennedy Blvd.
Ste 1800
Philadelphia, PA 19103-2899
ENDOSCOPIC CRANIOSYNOSTOSIS SURGERY, FIRST EDITION ISBN: 9780323721752
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This book is dedicated to the parents of all of our patients who have undergone endoscopic
craniosynostosis surgery. After being told of the diagnosis, they embarked on a challenging
and difficult journey. They were often given conflicting and contradicting recommendations
for treatment. Yet, they followed their heart and soul and decided on a road less traveled.
Their courage and commitment have contributed to a seismic change in the management
of these patients.
iii
Contributors
Sam S. Bae, MD, DDS David F. Jimenez, MD, FACS
Oral, Maxillofacial, and Craniofacial Surgeon Director
Pediatric Craniomaxillofacial Surgery Pediatric Neurosurgery
Three Rivers Oral and Facial Surgery El Paso Children’s Hospital
Tualatin, Oregon El Paso, Texas
Cathy Cartwright, DNP, RN-BC, PCNS, FAAN Hiria Limpo, MD
Director of Advanced Practice Professional Development Neurosurgery Resident
Advanced Practice Programs Boston Children’s Hospital
Children’s Mercy Kansas City Boston, Massachusetts
Kansas City, Missouri Neurosurgery Resident
Hospital Fundación Jimenez Diaz
Linda R. Dagi, MD Madrid, Spain
Associate Professor
Department of Ophthalmology Marc A. Orlandi, M.D.
Harvard Medical School; Assistant Professor
Director of Adult Strabismus Texas Tech University Health Science Center
Ophthalmology EL Paso, Texas
Boston Children’s Hospital Anesthesia Department Chief
Boston, Massachusetts El Paso Children’s Hospital
El Paso, Texas
Emily Louise Day, BS
Clinical Research Assistant Mark R. Proctor, MD
Department of Neurosurgery Professor of Neurosurgery
Boston Children’s Hospital Harvard Medical School;
Boston, Massachusetts Boston Children’s Hospital
Boston, Massachusetts
Abdelrahman M. Elhusseiny, MD
Ophthalmology Resident E. Weston Santee, MD, DDS
Boston Children’s Hospital; Cleft and Craniofacial Surgeon
Harvard Medical School Plastics and Oral Maxillofacial Surgery
Boston, Massachusetts The Children’s Hospital of the King’s Daughters
Norfolk, Virginia
Deanna J. Fish, CPO
Clinical Outreach Manager Tina M. Whitman, DNP, CRNA, APN
Orthomerica Products Inc. Chief CRNA
Orlando, Florida El Paso Children’s Hospital
El Paso, Texas
Christina Hinton, CP
Orthomerica Products Inc. David M. Yates, MD, DMD, FACS
Orlando, Florida Division Chief of Cranial and Facial Surgery
Oral and Maxillofacial Surgery
El Paso Children’s Hospital
El Paso, Texas
iv
Acknowledgment
The development and refinement of all of the endoscopic techniques described
in this book could not have come to fruition without the invaluable input,
thoughtful and careful guidance and development of plastic surgeon Con-
stance M. Barone. Her superb technical skills and keen critical thinking, have
contributed significantly to the great success of all of these surgeries.
v
Video Table of Contents
1 Chapter 4. Perioperative Logistics 5 Chapter 10. Metopic Synostosis
Video demonstrates common principles associated with The entire operation for treatment of Metopic synostosis is
endoscopic craniosynostosis operations. Surgical set up, key presented in this video. Patient positioning, incision location,
surgical instrumentation, and surgical suite organization are subgaleal dissection, osteotomy placement, bone removal,
amongst the covered topics. osseous hemostasis and wound closure are shown in detail.
2 Chapter 6. Anesthesia Management 6 Chapter 13. Lambdoid Synostosis
Presented are the underlying principles related to carrying The entire operation for treatment of Lambdoid synostosis is
out a successful operation from the anesthesia standpoint. presented in this video. Patient positioning, incision location,
Endotracheal intubation, how to properly secure the ET subgaleal dissection, osteotomy placement, bone removal,
Tube, corneal protection, patient turning, precordial Doppler osseous hemostasis and wound closure are shown in detail.
placement and other topics are presented.
7 Chapter 15. Postoperative Cranial Orthotic
3 Chapter 8. Sagittal Craniosynostosis Therapy
The entire operation for treatment of sagittal synostosis is Video demonstrates the many steps related to orthotic
presented in this video. Patient positioning, incision location, therapy after surgery. Head scaring, helmet manufacturing,
subgaleal dissection, osteotomy placement, bone removal, helmet padding, grinding, expansion and modifications
osseous hemostasis and wound closure are shown in detail. done to reach custom orthosis for successful management
and correction of the patient’s cranial/facial deformities.
4 Chapter 9. Uni-Coronal Synostosis
The entire operation for treatment of Unicoronal synosto-
sis is presented in this video. Patient positioning, incision
location, subgaleal dissection, osteotomy placement, bone
removal, osseous hemostasis and wound closure are shown
in detail.
vii
1
The History and Evolution
of Craniosynostosis
Surgery
SAM S. BAE, MD, DDS AND E. WESTON SANTEE, MD, DDS
Early Descriptions of Cranial Morphology
One of the earliest illustrations of the cranium and cra-
and Cranial Sutures nial sutures was recorded several centuries later during the
Medieval Period. Avicenna (980–1037), a Persian physician
The first documented report describing the diversity of cra- and polymath, depicted the structural framework of the
nial morphology dates to 440 BCE in Herodotus’ work, cranium and the cranial sutures in his work, al-Qānūn fī
The Histories (Ἱστορίαι Historíai). Herodotus (484–425 al-Ṭibb (Canon of Medicine), the most comprehensive medi-
BCE), an ancient Greek historian, hypothesized through cal textbook of its time (Fig. 1.1). He distinctly named and
a study that environmental factors contributed to the described the different cranial sutures, portraying the coro-
observed variation in cranial thickness between different nal suture as “an arc in whose center a perpendicular line has
human populations.1 Hippocrates of Kos (460–370 BCE), been set up”9; distinguishing the sagittal suture as the suture
the Greek physician who is widely praised as the “Father of partitioning the skull into two halves,10 and regarding the
Medicine,” provided one of the earliest and most compre- squamosal suture as a “false” suture as they “do not pen-
hensive accounts of cranial anatomy and suture morphol- etrate the bone but overlap like fish scales.”6 Additionally,
ogy in the treaties, On Head Wounds (Περι των εν κεφαλη he accurately explained the configuration and articulation
τρωματων). Hippocrates classified four discrete skull pat- of the bones of the cranial vault,11 stating that the “frontal
terns based on suture arrangement, which he likened to bone is located anteriorly; behind it are two parietal bones
the shape of the Greek letters, and proposed the concept of which are above the temporal bones and the occipital bone
anatomic variability. The treaties also emphasized the clini- which is more compact and protects the back of the brain
cal significance of cranial thickness in the management and posteriorly.”12–14
outcome of head injuries.1–4 The knowledge of cranial anatomy and suture deformity
Variation in the shape of the cranium and cranial sutures expanded during the Renaissance through the works of
were later recognized by Galen of Pergamon (130–200 key anatomists. A German physician, Johannes Dryander
BCE), a Roman physician to the gladiators. Through his (1500–60) published the first detailed pictorial textbook
anatomical studies, which were primarily on animals, he on neuroanatomy in 1536, Anatomia Capitis Humani,
identified the cerebral aqueduct, characterized seven cranial which comprised of eleven elegantly engraved woodcuts. In
nerves, and correlated characteristic cranial features with one of his illustrations, he clearly displayed the configura-
the condition hydrocephalus.1,4,5 In his work, De Ossibus tion of specific cranial sutures (Fig. 1.2A) and alluded to
ad Tirones (On the Bones for Novices), he provided descrip- the presence of the metopic suture, which “moved across
tions of the cranial sutures, the number of bones that form the forehead to the nose.”10,15 In 1543, a Flemish physi-
the cranium, and the shape of a normal skull.6 Galen also cian, Andreas Vesalius (1514–64), wrote and illustrated
defined the term oxycephaly, introducing the notion of the monumental textbook of human anatomy (Fig. 1.3A),
craniosynostosis.7,6 Association of cranial deformities with De Humani Corporis Fabrica Libri Septum (On the Fabric
craniofacial abnormalities like palatal defects was written by of the Human Body). Observations made from numerous
Oribasius (320–403), a Greek medical writer and personal dissections of human cadavers allowed Vesalius to appreci-
physician to the Roman emperor Julian the Apostate.8 ate anatomic variability.1,16–18 He described a wide range
1
2 CHAPTER 1 The History and Evolution of Craniosynostosis Surgery
through a combination of isolated growth restriction and
compensatory expansion elsewhere, thereby postulating the
first scientific explanation of the condition.19,23,25–27
In 1851, German pathologist and anthropologist
Rudolph Carl Virchow (1821–1902) (Fig. 1.4) expanded
upon Otto’s theories and published a seminal paper entitled,
Ueber Kretinismus namentlich in Franken und über patholo-
gische Schädelformen (Cretinism, Particularly in Franconia,
and Pathological Skull Forms).28 In his attempt to describe
the demographics, pathology, and etiology of cretinism,
Virchow created a classification system summarizing the
various pathologic skull shapes observed in craniosynosto-
sis.29,30 He concluded that premature closure of a cranial
suture restricted calvarial growth in a perpendicular direc-
tion, resulting in a compensatory overgrowth at the remain-
ing unaffected sutures29,28 thereby allowing the rapidly
developing brain to grow. He also postulated that cranio-
synostosis was associated with disturbance of thyroid func-
tion or inflammation of the meninges.29,28,31
Virchow’s impact was momentous and provided the
impetus for further exploration and dissemination of cranio-
synostosis studies over the next century. Reports of cranio-
synostosis with other congenital anomalies sprouted, leading
to the recognition and classification of syndromic cranio-
synostosis.25 In particular, Eugène Apert (1868–1940), a
French pediatrician, and Octave Crouzon (1874–1938),
a French neurologist, identified syndromes in 190632 and
1912,33 respectively, which continue to bear their names.
Crouzon also asserted that there may be a genetic contribu-
tion to the pathogenesis of craniosynostosis.31
The theory of Virchow’s law was challenged in 1959 by
Melvin Moss (1923–2006), an American orthodontist, who
proposed that premature sutural fusion is a secondary out-
come and not a cause of cranial growth restriction and mal-
• Fig 1.1 Avicenna’s Canon of Medicine (al-Qānūn fī al-Ṭibb). The old- formation.34,35 Moss highlighted that expansion of the brain
est copies of the second volume (1030). (Courtesy the Institute of primarily influences the growth of the calvarium, which is
Manuscripts of Azerbaijan National Academy of Sciences.)
the fundamental basis for his “functional matrix theory.” He
hypothesized that craniosynostosis resulted from aberrant
of morphologic aberration of the shape of the skull and the development of the cranial base, producing altered biome-
arrangement of the sutures. He displayed one “natural” and chanical forces that transmitted to the sutures via the dura
four “unnatural” skulls, demonstrating how absence of cer- mater, causing premature fusion.31,34,35 His opposing pos-
tain sutures can lead to cranial malformations, namely cra- tulation stemmed from the observation that similar cranial
niosynostosis (Fig. 1.3B).1,7,10 vault deformities have been noted in the absence of suture
fusion,36–38 and from his thesis research which demon-
strated that removal of calvarial sutures in growing rats did
Early Insight into Calvarial Growth and not affect neurocranial development.20,39,40 Experimental
Craniosynostosis studies and surgical interventions directed at extirpation of
the fused sutures have since undermined Moss’ theory, dem-
Modern understanding of craniosynostosis arose in the late onstrating that release of the fusion can inhibit the perpetu-
18th century with Samuel von Sömmering’s (1755–1830) ation of the induced abnormal growth pattern and reverse
observations of cranial sutures’ significant role in the growth the deformity of the cranial base and vault.19,38,41,42
of the cranial vault. In 1791, the German physician rec-
ognized the association that failure of growth at a particu- Advancement of Surgical Concepts
lar suture consequently led to cranial deformity.19–22 The
term “craniosynostosis” was first defined by Adolph Wil- Half a century after Virchow’s hypothesis, surgical treatment
helm Otto (1786–1845) soon after in 1831.23,24 Based on of craniosynostosis was first introduced. Although the condi-
Otto’s studies in humans and animals, Sömmering asserted tion of craniosynostosis was well recognized by the late 19th
that premature closure of sutures led to cranial deformity century, it is believed that many of the initial interventions
CHAPTER 1 The History and Evolution of Craniosynostosis Surgery 3
A B
• Fig 1.2 (A) One of the illustrations of the dissection of the head in Dryander’s Anatomiae pars prior, show-
ing the exposed skull with cranial sutures and dissecting instruments. (B) “The total representation of all
parts of the human head with their explanation.” (An adaptation from an illustration in Magnus Hundt’s
Anthropologium, published in 1501.)
were likely performed on nonsynostotic microcephaly.43 The of his time, criticized and warned against these early
concept of craniectomy or craniotomy, was suggested by a attempts,49 and surgery for craniosynostosis was ultimately
Canadian anatomist and surgeon, William Fuller, who was discontinued after Abraham Jacobi (1830–1919), a German
the first to trephine out portions of the skull of a child with physician and a pioneer of the field of pediatrics in America,
“mental imbecility” in 1878.44 In 1888, an American sur- reported high mortality rates in 1894.50
geon, Levi Cooper (L.C.) Lane (1831–1902) performed the Nearly three decades passed until the resurgence of cra-
first cross-shaped craniectomy on a child with mental imbe- niosynostosis surgery. In 1921, Mehner revived the concept
cility and “decidedly microcephalic” cranium, who, unfortu- of suturectomy and published his technique and outcome of
nately died 14 hours later reportedly due to complications craniectomy for complete removal of a synostosed suture.51
with anesthesia.45 Lane performed a second H-shaped crani- More extensive open craniectomies were discussed by Faber
ectomy in 1892 on an “imbecile microcephalic infant” who and Towne in 1927, who recommended decompressive cra-
fared well this time with improvement of the mental status.45 niosynostosis surgery to prevent complications of blindness
A French surgeon (Fig. 1.5), Odilon Marc Lannelongue secondary to elevated intracranial pressure.52 In 1943, Faber
(1840–1911) is credited for performing the first bilateral and Towne proposed the notion of early prophylactic linear
parasagittal linear craniectomies in 1890 for the correction suturectomy, around the age of 1 to 3 months, for preserva-
of sagittal craniosynostosis, which was proposed to mitigate tion of neurologic function and cosmetic improvement.19,53
intracranial pressure and allow for physiologic expansion By the mid-1900s, with the advent of radiological studies to
of the brain.46,47 The patient was a 4-year old child with confirm the diagnosis and progressive refinement of surgical
a severe psychomotor handicap, who reportedly had near techniques, linear suturectomies became the standard treat-
complete neurologic recovery.46 The idea that craniectomy ment of craniosynostosis; however, outcomes were inconsis-
could alleviate imbecility incited initial enthusiasm; how- tent and often plagued by early reossification at craniectomy
ever, microcephaly resulting from primary brain abnor- sites and inadequate correction of the cranial vault.43 Efforts
malities was often misdiagnosed as craniosynostosis, leading to line the craniectomy edges with numerous products (e.g.,
to poor case selection and futile interventions.7,48 Conse- polyethylene film, tantalum foil, silastic strips, and other
quently, the outcomes were disheartening with significant substances) to prevent suture refusion also failed.43,54–56
refusion of the operated sutures and reversion of the cra- By the 1960s, development of anesthesia, blood manage-
nial deformity and growth constriction.19 Harvey Cushing ment, and operative technique provided the opportunity for
(1869–1939), a highly influential American neurosurgeon more complex and safer craniosynostosis surgery, allowing
