Huang M H, Gruss J S, Clarren S K, Mouradian W E, Cunningham M L, Roberts T S, Loeser J D, Cornell C J
Department of Plastic Surgery, Singapore General Hospital, Singapore.
Plast Reconstr Surg. 1996 Oct;98(5):765-74; discussion 775-6. doi: 10.1097/00006534-199610000-00001.
The diagnosis and treatment of posterior plagiocephaly is one of the most controversial aspects of craniofacial surgery. The features of true lambdoid synostosis versus those of deformational plagiocephaly secondary to positional molding are inadequately described in the literature and poorly understood. This has resulted in many infants in several craniofacial centers across the United States undergoing major intracranial procedures for non-synostotic plagiocephaly. The purpose of this study was to describe the detailed clinical, imaging, and operative features of true lambdoid synostosis and contrast them with the features of positional plagiocephaly. During a 4-year period from 1991 to 1994, 102 patients with posterior plagiocephaly were assessed in a large multidisciplinary craniofacial program. During the same period, 130 patients with craniosynostosis received surgical treatment. All patients were examined by a pediatric dysmorphologist, craniofacial surgeon, and pediatric neurosurgeon. Diagnostic imaging was performed where indicated. Patients diagnosed with lambdoid synostosis and severe and progressive positional molding underwent surgical correction using standard craniofacial techniques. Only 4 patients manifested the clinical, imaging, and operative features of unilambdoid synostosis, giving an incidence among all cases of craniosynostosis of 3.1 percent. Only 3 among the 98 patients with positional molding required surgical intervention. All the patients with unilambdoid synostosis had a thick ridge over the fused suture, identical to that found in other forms of craniosynostosis, with compensatory contralateral parietal and frontal bossing and an ipsilateral occipitomastoid bulge. The skull base had an ipsilateral inferior tilt, with a corresponding inferior and posterior displacement of the ipsilateral ear. These characteristics were completely opposite to the findings in the 98 patients who had positional molding with open lambdoid sutures and prove conclusively that true unilambdoid synostosis exists as a specific but rare entity. Awareness of the features of unilambdoid synostosis will allow more accurate diagnosis and appropriate treatment of posterior plagiocephaly in general and in particular will avoid unnecessary surgical intervention in patients with positional molding.
后斜头畸形的诊断与治疗是颅面外科中最具争议的方面之一。文献中对真性人字缝早闭与因体位塑形继发的变形性斜头畸形的特征描述不足，人们对此了解也甚少。这导致美国多个颅面中心的许多婴儿因非缝早闭性斜头畸形而接受重大颅内手术。本研究的目的是描述真性人字缝早闭的详细临床、影像学和手术特征，并将其与体位性斜头畸形的特征进行对比。在1991年至1994年的4年期间，在一个大型多学科颅面项目中对102例后斜头畸形患者进行了评估。同一时期，130例颅缝早闭患者接受了手术治疗。所有患者均由儿科畸形学家、颅面外科医生和儿科神经外科医生进行检查。在必要时进行诊断性影像学检查。被诊断为人字缝早闭以及严重且进行性体位塑形的患者采用标准颅面技术进行手术矫正。只有4例患者表现出单侧人字缝早闭的临床、影像学和手术特征，在所有颅缝早闭病例中的发生率为3.1%。98例体位塑形患者中只有3例需要手术干预。所有单侧人字缝早闭的患者在融合缝上方都有一条增厚的嵴，与其他形式的颅缝早闭中发现的情况相同，伴有对侧顶叶和额叶的代偿性膨隆以及同侧枕乳突膨出。颅底有同侧向下倾斜，同侧耳朵相应地向下和向后移位。这些特征与98例人字缝开放的体位塑形患者的发现完全相反，确凿地证明真性单侧人字缝早闭作为一种特殊但罕见的实体存在。了解单侧人字缝早闭的特征将有助于更准确地诊断和适当治疗一般的后斜头畸形，特别是将避免对体位塑形患者进行不必要的手术干预。
