Craniosynostosis in 2-Month-Old Infant Managed with Minimally Invasive Endoscopically Assisted Remodelation (MEAR)

Abstract

Craniosynostosis is an atypical closure of one or multiple sutures that cause abnormal skull shape. Main complications include intracranial hypertension, alteration of cognitive development, seizures, and blindness, among others. The incidence is 1 in 2000 live births, and it is higher in males. This case report presented a 2-month-old infant with scaphocephaly identified on the physical examination. The early approach was a CT scan and cephalometric assessment to confirm the diagnosis and Schedule the surgery treatment. The minimally invasive endoscopic remodeling was successfully performed with positive outcomes. Literature review was performed on digital bases like PubMed, Web of Science and Scopus. Minimally invasive endoscopically assisted remodelation (MEAR) benefits to correct craniosynostosis are a shorter stay at hospital, less perioperative complications and lower medical cost. Using technologies and innovative techniques the surgical approach could be improved to reduce complications and maximize outcomes. Thus, neurosurgery departments must know and implement this technique.

Resumo

A craniossinostose é um fechamento atípico de uma ou múltiplas suturas que causa formato anormal do crânio. As principais complicações incluem hipertensão intracraniana, alteração do desenvolvimento cognitivo, convulsões e cegueira, entre outras. A incidência é de 1 em 2000 nascidos vivos, sendo maior em homens. Este relato de caso apresentou um bebê de 2 meses com escafocefalia identificada no exame físico. A abordagem inicial foi uma tomografia computadorizada e avaliação cefalométrica para confirmar o diagnóstico e programar o tratamento cirúrgico. A remodelação endoscópica minimamente invasiva foi realizada com sucesso, com resultados positivos. A revisão da literatura foi realizada em bases digitais como PubMed, Web of Science e Scopus. Os benefícios da remodelação assistida endoscópica minimamente invasiva (MEAR) para corrigir a craniossinostose são menos tempo no hospital, menos complicações perioperatórias e menor custo médico. Usando tecnologias e técnicas inovadoras, a abordagem cirúrgica pode ser melhorada para reduzir complicações e maximizar os resultados. Portanto, os departamentos de neurocirurgia devem conhecer e implementar esta técnica.

Keywords

craniosynostosis - minimally invasive - endoscopic - case report

Palavras-chave

craniossinostose - minimamente invasivo - endoscópico - relato de caso

Introduction

Craniosynostosis is an abnormally shaped skull caused by a premature fusion of sutures. Scaphocephaly is the most common form of craniosynostosis, the early closure of the sagittal suture produces a longitudinal skull elongation to compensate for the growth. In severe cases, intracranial pressure could occur with other complications and face malformations.[1] Otherwise, syndromic craniosynostosis includes a fusion of multiple cranial sutures and other abnormalities of the face and limbs.[2]

The prevalence of craniosynostosis ranges from 3.1 to 5.06 per 10,000 births.[3] Scaphocephaly accounts for ∼50% of craniosynostosis cases, with an incidence of 1 in 2000 live births.[4] Nonsyndromic craniosynostosis has an incidence of 0.4 to 1.0 in 1000 births.[5] There is a gender variation in presentation, with boys having a 4:1 prevalence of sagittal craniosynostosis, while girls have a 3:2 prevalence of unicoronal craniosynostosis compared with boys.[5]

According to the mechanisms underlying premature closure of cranial sutures, the abnormal dural attachments drive an excessive tension that arrests bone growth and leads to early suture closure.[6] Fibroblast growth factor (FGF) and transforming growth factor β lead the mechano-transducion signals to stimulate the osteogenic cell proliferation at the suture line.[7] Also, gene mutations and chromosomal alterations such as abnormality in FGF receptors,[2] implicating FGFR2, FGFR3, TWIST1, and EFNB1.[7] Environmental factors that may predispose the disease are oligohydramnios, uterine fetal head constriction, exposures to teratogens, use of epileptic drugs (phenytoin, valproic acid) and maternal smoking.[2]

The diagnosis is primarily based on the physical examination confirmed by the CT scan. Also, three-dimensional surface reconstructions using CT can assist in the classification of craniofacial disorders and planning surgical treatment.[8] The CT scan findings concordance with clinical diagnosis in 98% of the cases, 3D imaging allows planning the surgical osteotomies approach to reduce the operative time, and overall risks associated and simulate the surgical results.[9] Furthermore, cephalometry measurement is a standardized technique to confirm longitudinal head growth with the index cranial (ratio of the maximum width to length of the skull) considered normal 74–80 UI in males, and 73–79 UI in females. Craniosynostosis is contemplated under 70 UI, having scaphocephaly CI between 60–67 UI.[10]

In this case, non-syndromic craniosynostosis involves the closure of just one of the sutures, which could be sagittal, coronal, metopic, or lambdoid. The case report presents a 2-month-old patient who was diagnosed with scaphocephaly, a CT scan confirmed it and assisted in planning the approach using a MEAR technique, which is gradually implemented through this decade in the neurosurgery departments.

Case Study

The case is a 2-month-old infant with a height of 51 cm and a weight of 5.2 kg. Mother healthy, father healthy, pregnancy progress normal, delivery on time, cephalad, birth weight 3770 g, PD 50 cm, normal postpartum adaptation. Conscious, afebrile, no signs of acute infection, hydration good, skin clear.

• Head: scaphocephaly, sclera white, conjunctiva pink, pupils isochoric, ears and nose without discharge, throat normal

• Neck: Filling of jugular veins normal, nodes not enlarged.

• Chest: symmetrical, breathing auscultatory, no side effects, eupnoea, AS regular, the sounds are limited, nonpathological murmur.

• Abdomen: calm, painless, peristalsis audible, palpation without palpable resistance

• Genitalia: male genitalia

• Extremities: no deformities, no swelling

• Indicative neurological findings: Picomotor development corresponds to age, finding - isochoric pupils, no nystagmus, and facial expressions that seem symmetrical. No signs of meningeal irritation.

Diagnosis Assessment

To confirm the scaphocephaly clinical diagnosis, a CT scan and cephalometry were required. Diagnosis process: FOO 431 mm/over metopion = +3.4 SD of norm (nonhereditary value) Dynamics of postnatal brain/cerebral growth - see chart FOO IC 62.0 IU.

• Disproportional cephalogram: biparietal = −1.4SD, neurocranium length =  + 3.6SD, forehead width= −0.1SD of normal, cranial base width −1.7 SD.

A 2-month-old boy with a craniosynostosis diagnosis confirmed by a CT scan and cephalometric markers of a premature obliteration of the sagittal suture. [Figs. 1] and [2]

Fig. 1 CT scan, 3D reconstruction previous surgery.

Fig. 2 Cephalometry with +3.4 SD.

Surgical Intervention

In early diagnosis cases, the indication is a minimally invasive surgical technique with an endoscopic strip craniectomy along the prematurely closed sagittal suture with lateral barrel-stave osteotomies. The endoscope helps to control the extent of craniectomy lateral basal and illuminates the operative field to disclose bony spikes, unevenness, and bumpiness.[11]

The surgery starts with two incisions of 3- to 5-cm length ∼5 cm behind the bregmatic fontanelle and above lambda. Next, the surgeon makes two burr holes in the parietal bones, separates the adjacent dura, connects it across the midline, and dissects the dura with a Penfield dissector. Then, the anterior and posterior burr holes relate to bone-cutting scissors, a 2–5-cm-wide sagittal strip craniectomy, and 5–15-mm-wide lateral craniotomies behind the coronal suture and in front of the lambdoid sutures on both sides. Also, the skull remodelation is performed by unsticking the adhesions of the periosteum and dura mater to widen the posterior parietal regions reshaping the parietooccipital skull. Finally, the periosteum is approximated, and the skin is stitched in layers with intradermal atraumatic suture. [Fig. 3]

Fig. 3 The MEAR technique performed in this craniosynostosis case.

Follow-up and Outcomes

The patient was observed at the ICU overnight with moderate head edema that was resolved quickly, and a 3D head CT scan was performed. The child was followed by the neurosurgery department and an orthodontist with a regular register of cephalometry and a 3D head scan.

Surgical intervention was favorable to prevent complications like intracranial hypertension and its consequences, for instance: blindness, brain damage, seizures, and neurodevelopment affections.[2] [3] [Figs. 4] and [5]

Fig. 4 5 CT scan 3 days after remodalation surgery.

Fig. 5 CT scan 3 days after remodalation surgery.

Discussion

This surgical technique was developed and implemented in 2017 in Motol University Hospital; Neurosurgery Department.[11] The craniosynostosis cases are managed with modern surgical techniques. The emphasis, in this case, is the importance of an early diagnosis using a CT scan and cephalometric parameters to achieve an adequate surgery approach. Furthermore, the endoscope is an excellent tool to control the operative field, especially in laterobasal depths where it is valuable to improve the outcomes and reduce the complication risk.

Fassl V. et. identified the age range indication for endoscopically assisted craniosynostosis surgery in children < 3 months old, even a frame 4–9 months old depending on the deformity case. Mentioning the difficulty of performing the technique in patients older than 6 months.[12] The early imaging diagnosis through a CT scan allows attain the surgical age indications, additionally detecting incidental pathologies.[9] The alternative imaging diagnostic, MRI aids in detecting cerebral pathologies, vascular malformations, cranial neuropathies, and soft-tissue abnormalities. Moreover, ultrasonography could identify the loss of hypoechoic signal from the sutural space, irregularly thickened border, and asymmetry of the fontanels (sensitivity = 96.9%, specificity = 100%).[8] [9]

The minimally invasive surgeries significantly decrease blood loss and reduce rates of transfusion in contrast with the open craniotomies.[13] Indeed, the operative time and length of hospital stay are shorter as well, Yan H. et. describe a medical cost reduction in endoscopic procedures cheaper than open procedures.[13] Other benefits are less morbidity, low postoperative pain, and faster recovery.[14]

Considering the intraoperative complications, the endoscopic assisted surgery appears to lower cardiovascular, pulmonary, and dural adverse situations. Despite this, the difference in postoperative complications, such as surgical wound infection, is not significant compared with open craniotomy.[15] The follow-up could extend even for 13 months, initiating helmet therapy as soon as possible within 24 hours postoperative until 5.0 months to 1 year. Criteria to discontinue the helmet therapy comprised either achieving normal cranial parameters.[12]

Endoscopic-assisted remodeling attributes the successful short and long-term outcomes to the younger age of suturectomy; certainly, the differences in outcomes with this technique remain an important element of this vanguard paradigm.[16] New technologies and innovative techniques improve surgical strategies to offer the best treatment for patients.[17] The neurosurgery departments must know about all these techniques. For sure the surgeons will continue improving techniques, such as virtual surgical planning or 3D printed models, to maximize outcomes and minimize surgical trauma in patients.[17]

Conclusion

The proper early diagnosis of craniosynostosis will permit an adequate surgery intervention by the neurosurgery departments. Hence, the medical system and hospitals should promote innovation in the technological field to enhance medical training with updated techniques to give the best treatment for the patient. Limitations like medical assistance accessibility, limited resources, or surgical skills could be strengthened in many countries, impelling research and institutional cooperation. Finally, highlighting the importance of early contact with patients and clinical cases by medical students might be beneficial to achieve high-level education and the best quality medical attention.

Conflict of Interest

The authors report no conflict of interest.

• References

• 1 Campbell WW. DeJong's The Neurologic Examination. 7th ed. Filadelfia, PA, Estados Unidos de América: Lippincott Williams and Wilkins; 2013.
• 2 Kajdic N, Spazzapan P, Velnar T. Craniosynostosis - recognition, clinical characteristics, and treatment. Bosn J Basic Med Sci 2017; 18 (02) 110-116
• 3 Buchanan E, Xue Y, Xue A, Olshinka A, Lam S. Multidisciplinary care of craniosynostosis. J Multidiscip Healthc 2017; 10: 263-270
• 4 Fearon JA, McLaughlin EB, Kolar JC. Sagittal craniosynostosis: surgical outcomes and long-term growth. Plast Reconstr Surg 2006; 117 (02) 532-541
• 5 Dempsey RF, Monson LA, Maricevich RS. et al. Nonsyndromic Craniosynostosis. Clin Plast Surg 2019; 46 (02) 123-139
• 6 Moss ML. The pathogenesis of premature cranial synostosis in man. Acta Anat (Basel) 1959; 37: 351-370
• 7 Brah TK, Thind R, Abel DE. Craniosynostosis: Clinical Presentation, Genetics, and Prenatal Diagnosis. Obstet Gynecol Surv 2020; 75 (10) 636-644
• 8 Badve CA, K MM. Iyer RS, Ishak GE, Khanna PC. Craniosynostosis: imaging review and primer on computed tomography. Pediatr Radiol 2013; 43 (06) 728-742 , quiz 725–727
• 9 Alnaif N, Zhou M, Galli R, Azzi AJ, Alamri A, Gilardino M. The Role of Preoperative Computed Tomography in Nonsyndromic Craniosynostosis. J Craniofac Surg 2019; 30 (02) 424-428
• 10 Al-Shaqsi SZ, Rai A, Forrest C, Phillips J. Standardization of Cranial Index Measurement in Sagittal Craniosynostosis. J Craniofac Surg 2019; 30 (02) 366-369
• 11 Liby P, Lomachinsky V, Taborsky J. et al. Minimally invasive endoscopically assisted remodelation (MEAR) of sagittal craniosynostosis: an alternative technique to open and endoscopic procedures with cranial orthosis time span reduction. Childs Nerv Syst 2021; 37 (02) 581-586 https://pubmed.ncbi.nlm.nih.gov/32728932/ cited 25thaugust2022 [Internet]
• 12 Fassl V, Ellermann L, Reichelt G. et al. Endoscopic treatment of sagittal suture synostosis - a critical analysis of current management strategies. Neurosurg Rev 2022; 45 (04) 2533-2546
• 13 Yan H, Abel TJ, Alotaibi NM. et al. A systematic review and meta-analysis of endoscopic versus open treatment of craniosynostosis. Part 1: the sagittal suture. J Neurosurg Pediatr 2018; 22 (04) 352-360
• 14 Pendharkar AV, Shahin MN, Cavallo C. et al. Minimally invasive approaches to craniosynostosis. J Neurosurg Sci 2018; 62 (06) 745-764
• 15 Arts S, Delye H, van Lindert EJ. Intraoperative and postoperative complications in the surgical treatment of craniosynostosis: minimally invasive versus open surgical procedures. J Neurosurg Pediatr 2018; 21 (02) 112-118
• 16 Goyal A, Lu VM, Yolcu YU, Elminawy M, Daniels DJ. Endoscopic versus open approach in craniosynostosis repair: a systematic review and meta-analysis of perioperative outcomes. Childs Nerv Syst 2018; 34 (09) 1627-1637
• 17 Proctor MR, Meara JG. A review of the management of single-suture craniosynostosis, past, present, and future. J Neurosurg Pediatr 2019; 24 (06) 622-631

Publikationsverlauf

Eingereicht: 01. Februar 2023

Angenommen: 23. Oktober 2024

Artikel online veröffentlicht:
27. März 2025