Continuing Education Activity

Scaphocephaly is the term used to describe the narrow and long abnormal skull shape in sagittal craniosynostosis due to the premature fusion of the sagittal suture forming an osseous ridge. This activity reviews the evaluation and management of scaphocephaly and highlights the healthcare team's role in evaluating, treating, and improving care for patients with this condition.

Objectives:

• Describe the etiology of scaphocephaly.
• Summarize the common physical exam and typical imaging findings associated with scaphocephaly.
• Identify the indications for scaphocephaly surgery.
• Outline some interprofessional team strategies that can be used by the healthcare team to improve patient outcomes when managing scaphocephaly cases.

Introduction

Scaphocephaly is the term used to describe the narrow and long abnormal skull shape in sagittal craniosynostosis due to the premature fusion of the sagittal suture forming an osseous ridge.[1] The head shape is described as a "boat-shaped" head, which is called dolichocephaly. It is the most common type of craniosynostosis. It is essential to make an early diagnosis since a delay may lead to abnormal growth of the cranial vault and affect brain growth and development.

Etiology

The cause of scaphocephaly is the premature closure of all or most of the sagittal suture. Physiologic sagittal suture closure starts at 22 years of age. Sometimes, a complete fusion may not occur; however, most individuals have it closed between 30 and 50 years old. The specific etiology of craniosynostosis, especially the nonsyndromic type of craniosynostosis, is still not completely understood, but most cases are sporadic. Many factors have been studied and implicated, and these include genetic mutations, metabolic and hematologic syndromes, teratogens (i.e., valproic acid), maternal smoking, and advanced paternal age.

A combination of external factors and genetic mutations is implicated in this condition's etiology. There have been more than 100 mutations associated with nonsyndromic craniosynostosis. The most common and most studied mutation is the fibroblast growth factors (FGFR) gene 1-3.[2] In sagittal craniosynostosis, the most common mutation is in the SMAD6 gene.[3] Other mutations associated are in the TWIST1, TCF12, ERF, MSX2 genes.[3][4][5][6] However, only in a few cases, mutations are identified.[2][7]

Epidemiology

The incidence of craniosynostosis is approximately 1 in 200 to 2500 live births. Single suture involvement is most commonly seen. Craniosynostosis is most frequently seen in the child of a non-Hispanic white. Sagittal craniosynostosis is the most common of all the craniosynostosis. It represents about 53% to 60% of all craniosynostosis. It affects males predominantly, approximately 80%, with a male-to-female ratio of 3.5 to 1.[7] Hispanic ethnicity and non-Hispanic black race are associated with a lower prevalence of craniosynostosis.[8]

Pathophysiology

When the sagittal suture prematurely ossifies, the skull's growth proceeds parallel to the future, and the growth perpendicular to the suture terminates. Thus, when the sagittal suture closes, the head keeps growing in the anteroposterior direction producing an elongated head shape. This is recognized as Virchow’s law.[9][10] In premature single suture fusion, elevated intracranial pressure occurs in approximately 11% of cases. In sagittal craniosynostosis, this can be as high as 44%.[11]

Alterations in the expression of growth factors have been implicated in the pathophysiology of craniosynostosis. Various mutations have been identified in craniosynostosis, including three fibroblast growth factors and other genes such as the twisted family basic helix-loop-helix transcription factor TWIST 1.[4][5][12][13][14] They are more frequently found in syndromic craniosynostosis.[15] In sagittal craniosynostosis, approximately 10% of the patients show genetic alterations.[14]

History and Physical

The parents usually complain that the baby’s head doesn’t look right. Frequently they noted that the deformity continues to worsen as the child’s head continues to grow. Parents are asked for a history of prior cases of craniosynostosis.

Clinical observation is essential. A physical examination will show the elongated head and a palpable bone ridge at the head's vertex. Head measurement shows an increased anteroposterior dimension. The bilateral dimension is shortened, producing biparietal and bitemporal narrowing. Prominent frontal and occipital bossing can be observed. The cephalic index is calculated by measuring the biparietal diameter and the occipitofrontal diameter and obtaining the ratio. The typical average index is approximately 80-83. In scaphocephaly, the index is reduced as the occipitofrontal diameter is larger. There are no visible abnormalities of the cranial base, orbits, or face in nonsyndromic sagittal craniosynostosis.

The baby is evaluated for respiratory difficulty. In severe cases, the head is abnormally flexed when lying supine causing airway compromise. An ophthalmologic examination should be obtained to assess for papilledema, indicating an increased intracranial pressure.[16]

Evaluation

Manual palpation of a bony prominence over the sagittal suture will aid in the initial diagnosis. The radiological finding of the absence of the sagittal suture or a bony ridge across part of the suture confirms the diagnosis. 

Ultrasonography can be used to detect the fused suture and avoid radiation to the patient.[17][18][19][20][21][22]

Plain skull X-rays can also show the fused suture; however, the study of choice is the head computed tomographic (CT) scan with 3D reconstruction.[23][24][25][26][27][28] The CT scan will also show other bone anomalies or hydrocephalus. However, some authors do not recommend the 3D CT scan in single sagittal craniosynostosis.[29][30] Magnetic resonance imaging (MRI) is not necessary except in syndromic cases to evaluate in detail the brain. Black box MRI offers an alternative to CT scan, avoiding radiation in patients with craniosynostosis.[31][32][33]

In patients with rare skeletal metabolic disorders and suspected craniosynostosis, it is essential to screen them at the time of diagnosis and follow-up.[34]

Treatment / Management

Treatment of sagittal craniosynostosis is usually surgical to correct head shape and prevent psychological effects on the parents and patient. In most cases, surgery involves the removal of the affected suture. The width of the craniectomy has to be at least 3 cm. The surgery is preferably done within the first 3–6 months of life. If surgery is performed after 12 months of age, more bone removal and remodeling is required. If surgical intervention is not performed early, suboptimal outcomes can result from the thickened skull and slowed brain growth. Control of bleeding is one of the essentials during the surgery. Preoperative prophylactic antibiotics are used. An adequate initial correction of the skull deformity protects the patient from the additional surgical risk by making a reoperation less likely. Surgery can be performed open or endoscopically. Endoscopic surgery has the advantage of reducing blood loss; however, a helmet is usually required for several months. The optimal duration of helmet use is around 7 to 9 months after surgery.[35][36]

Endoscopic surgery (strip craniectomy): Patients are usually placed in a modified prone position with the cervical spine extended. The modified prone position allows access to the frontal and occipital areas. The procedure incorporates two incisions on the midline. The initial incision is done 2-3 cm behind the anterior fontanelle, and the second incision is done anterior to the lambda. The pericranium is preserved. A subgaleal plane from the anterior fontanelle to the lambda of about 3 cm is done bilaterally. A burr hole at the edge of each incision is made. The dura is separated and then cut with Mayo scissors in a triangular shape towards the anterior fontanelle. A second posterior osteotomy is made at the lambda similarly. An endoscope is inserted and advanced posteriorly toward the lambda. Once the dura has been freed from the overlying bone, bone-cutting or Mayo scissors are used to make lateral osteotomies and remove the fused suture. Barrel stave, wedge osteotomies are created with scissors after the dura is freed from the bone directly behind the coronal suture and then directly in front of the lambdoid sutures. No direct cauterization of the dura is performed to allow bone growth from the osteogenic cells.[37][38][39][40][41]

Open surgery (cranial vault remodeling): Milder cases can be successfully treated with the removal of the fused sagittal suture. In more severe cases, a complete reshaping of the skull may be necessary. Patients with complete sagittal synostosis frequently have deformities in the anterior and posterior skull, which need to be addressed. Treatment is directed at the most salient deformity. After the bicoronal zigzag or sawtooth-shaped scalp flap is completed, exposure of the individual sutures is done. Each suture is removed separately. If the sutures are not removed, they will prevent the reshaping process and will maintain the deformity. Removal of the sagittal suture usually results in spontaneous widening of the entire skull as the restraining force is removed. The sagittal bone strip is reduced in length. Several barrel stave lateral osteotomies are made down to the level of the squamosal suture. The removed sutures are floated over the dura. If used, bone grafts are fixed with absorbable plate and screw fixation to fill the bone gaps. If there is significant frontal bossing, the frontal bone is removed and remolded. Radial barrel stave osteotomies are made, and the bone is flattened to correct the abnormal curvature. The frontal bone is then fixed with absorbable plates and screw fixation. If there is posterior bossing, a similar procedure in the occipital bone using radial barrel stave osteotomies is performed.[37] A suction drain can be placed to reduce the subgaleal blood collection, and a firm head dressing is applied. The patient is monitored overnight at the pediatric intensive care unit. The use of tranexamic acid reduces the need for multiple blood transfusions.[42][43][44][45]

Spring-assisted surgery: Uses a strip craniectomy, and indwelling springs aid in the cranial vault expansion.[46][47][48] This technique offers similar cosmetic results with less operative time and blood loss.[47][49] However, a second surgery is required for spring removal. Spring-assisted surgery had shorter operative time, shorter length of hospital stay, and less blood loss than open strip craniectomy with parietal barrel staving.[49][50] 

Studies have shown that the principal differences between the endoscopic approach and the open approach include shorter surgical time, shorter pediatric intensive care unit length of stay, less intraoperative blood loss, and fewer blood transfusions in the endoscopic approach.[51][52]

Differential Diagnosis

Non-syndromic craniosynostosis has to be differentiated from other sources that cause secondary synostosis.[34][53][54]

• Hyperthyroidism
• Hypercalcemia
• Renal osteodystrophy
• Hypophosphatemia
• Vitamin D deficiency
• Sickle cell anemia
• Thalassemia
• Polycythemia vera
• Microcephaly
• Prematurity
• Shunted hydrocephalus
• Sly syndrome
• Morquio syndrome
• Hurler's syndrome
• Beta-glucuronidase deficiency
• Mucopolysaccharidosis
• Mucolipidosis III
• X-linked hypophosphatemia
• 1-alpha hydroxylase deficiency
• Teratogens (phenytoin, valproic acid, retinoic acid, aminopterin)
• Pseudohypoparathyroidism
• Osteopetrosis

The single suture craniosynostosis has to be differentiated from syndromic craniosynostosis. Most syndromic craniosynostosis involves the coronal suture producing brachycephaly.[53]

• Apert syndrome
• Crouzon syndrome
• Muenke syndrome
• Pfeiffer syndrome
• Saether Chotzen syndrome
• Craniofrontonasal syndrome
• Carpenter syndrome

Prognosis

After surgery, the head will remodel and obtain a rounder shape that, in most cases, is satisfactory.[55] The endoscopic approach and the open approach are both effective in correcting sagittal craniosynostosis deformities.[51][52][56][16] A systematic review showed insufficient information to suggest that any treatment had greater cosmetic improvement over the other.[56] The cephalic index correction in strip craniectomy is less than in cranial vault reconstruction but not cosmetically significant.[57][58] Some surgeons recommended overcorrection to compensate for the reduced postoperative cranial growth.[59][60]

Long-term studies have shown that patients with sagittal craniosynostosis and other nonsyndromic craniosynostosis present difficulties in cognitive, language, and motor domains with mild neuropsychological deficits in infancy and childhood.[61][62] A study showed reading and/or spelling disability in up to 50% of the patients.[63] Sagittal craniosynostosis patients show that 39% of them have cognitive and speech problems. However, patients with sagittal craniosynostosis are less likely to have cognitive and speech problems than any other craniosynostosis type.[64] 

Patients with sagittal craniosynostosis have a normal range for intelligence but showed a higher incidence of learning disabilities.[56][63][65] Patients have a higher verbal IQ than the performance IQ, which can suggest finer problems in achievement.[65] One study found no increase in cognitive impairment.[66] A systematic review showed that operated patients for sagittal craniosynostosis have an increased risk of developing learning disorders, but there is insufficient evidence comparing procedures regarding cognitive and neurological outcomes.[56]

Complications

Several complications may occur from surgical correction of the craniosynostosis:[37][67][68][69]

• Cerebrospinal fluid leak
• Dural tears
• Excessive blood loss
• Consumption coagulopathy
• Hypoactivity
• Hyperthermia
• Wound infection local
• Epidural abscess
• Subcutaneous hematoma
• Epidural hematoma
• Venous air embolism
• Inadequate correction 
• Reoperation
• Scalp erosion
• Scalp ulcer

Postoperative and Rehabilitation Care

The patient is admitted to the pediatric intensive care unit for at least 24 hours. Then initial nursing care focuses on evaluating the neurologic status, fluid and electrolyte balance, and pain management. The hematocrit level is closely monitored, and blood transfusions are given if indicated. Pain control is maintained with alternating acetaminophen and ibuprofen and intermittent intravenous morphine as needed. Prophylactic antibiotics may be continued for 24 hours postoperatively. The child is evaluated for adequate hemostasis and signs of infection. Postoperative scalp swelling can be uncomfortable and can be reduced by elevating the head of the bed. The child is usually transferred to the general pediatric floor on the first or second postoperative day. The face and scalp swelling will resolve over 1 to 2 weeks.

Preoperative and postoperative photographs are taken to serve for outcome assessment. Follow-up for at least five years is ideal for assessing the progressive skull reshaping. Evaluation of the skull reshaping pattern and the child's age will guide the timing for revisional surgery. In children older than 1-2 years, defects >2 cm diameter usually require calvarial split bone grafts or bone substitutes to fill the cranial defects and prevent permanent defects.

Consultations

The following consultations are required:

• Pediatric neurosurgeon
• Craniofacial surgeon
• Plastic surgeon
• Pediatric intensivist
• Geneticist

Deterrence and Patient Education

The diagnosis and treatment of craniosynostosis can produce a significant impact on families.[70] Careful explanation of the diagnosis, treatment modalities, postoperative care, and prognosis can help families deal with the initial struggle after the diagnosis is made. The entire family, including grandparents, are offered the occasion to meet with the surgical team to discuss any concerns with the surgical experience, which will impact all of them.

In non-syndromic cases of sagittal craniosynostosis, it is recommended to perform screening for  SMAD6 mutations, and if present, genetic counseling is given to the parents.[3] If there is no family history for craniosynostoses and no genetic mutations are found, there is a 5% recurrence risk to offsprings for nonsyndromic sagittal craniosynostosis.[71]

Patients who have increased intracranial pressure due to craniosynostosis may not present with typical hydrocephalus symptoms but may develop signs such as papilledema, skull deformities, developmental delay, and developmental milestones underachievement.

Enhancing Healthcare Team Outcomes

An interprofessional team of healthcare professionals includes a pediatric neurosurgeon, craniofacial surgeon, plastic surgeon, psychologists, geneticists, and a pediatric intensivist to work together to obtain the best possible outcomes. It is essential to educate parents about diagnosis, prognosis, complications, potential treatment options, and follow-up. It is important to differentiate between patients and individualize the treatment selection concerning age, weight, degree of deformity, and associated symptoms. Every patient is different, and making the proper selection to obtain the best possible outcome is essential. The identification of genetic mutations is necessary for prognosis and parental counseling. Nursing roles include direct patient care, physician-family liaison, and family educator.

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Disclosure: Jose Sandoval declares no relevant financial relationships with ineligible companies.

Disclosure: Orlando De Jesus declares no relevant financial relationships with ineligible companies.