In 1929, Franc D. Ingraham, Harvey Cushing's protégé, established the first pediatric neurosurgical unit in the world at Boston Children's Hospital and dedicated his career to the neurosurgical care of children. He trained with both Cushing and Dandy and spent 1 year working in Oxford with Sherrington, who considered Ingraham to be the finest operative surgeon ever to work in his laboratory. Ingraham was instrumental in developing novel treatments, which he compiled in his classic book, Neurosurgery of Infancy and Childhood. Although he was modest and shy, Ingraham loved to entertain children with magic and enjoyed photography in and out of the operating room. Unfortunately, his career was plagued by personal illness, and he died young in 1965 at the age of 67. Despite his prolific 36-year neurosurgical career, Ingraham remained an associate professor at Harvard at his retirement. To recognize his remarkable contributions, Harvard established an endowed chair in his name in 1967. Ingraham was a pioneer and a leader in the development of pediatric neurosurgery by virtue of his imagination, intelligence, and ability to lead and inspire others. Cushing has come to be regarded as the founder of neurosurgery. It is fair to conclude that Ingraham, his disciple, is the founder of pediatric neurosurgery.
Subash Lohani and Alan R. Cohen
Roberta Rehder, Subash Lohani and Alan R. Cohen
Donald Darrow Matson made seminal contributions to the field of pediatric neurosurgery. Born in 1913 in Fort Hamilton, New York, Matson was the youngest of four sons of an army colonel. He graduated from Cornell University and, years later, from Harvard Medical School. Matson selected Peter Bent Brigham Hospital for his neurosurgical training, which was interrupted during World War II. As a neurosurgeon, he worked close to the front lines under Brigadier General Elliot Cutler in Europe, earning a Bronze Star. Matson returned to Boston to become Franc Ingraham’s fellow and partner. He was a masterful surgeon and, with Ingraham, published Neurosurgery of Infancy and Childhood in 1954, the first pediatric neurosurgery textbook in the world. Upon Ingraham’s retirement, Matson became chairman of the department of neurosurgery at Boston Children’s Hospital and Peter Bent Brigham. In 1968, he became the inaugural Franc D. Ingraham Professor of Neurological Surgery at Harvard Medical School. Among his neurosurgical accomplishments, Matson served as President of the Harvey Cushing Society, later known as the American Association of Neurological Surgeons. He was unable to preside at the 1969 meeting that marked the 100th anniversary of Cushing’s birth, having contracted Creutzfeldt-Jakob disease. Matson died at the age of 55, surviving his mentor Ingraham by only 4 years.
Subash Lohani, Richard L. Robertson and Mark R. Proctor
Arachnoid cyst is a common congenital anomaly in the pediatric population. The cysts are often asymptomatic, but they can cause headache and other symptoms. Occasionally a cyst may rupture after head trauma producing a subdural hematoma. The authors present the case of an 11-year-old boy who presented after a week of progressive and severe back pain radiating to the back of his thighs. Imaging revealed a spinal subdural blood collection at the L4–S1 level. This finding prompted further cephalad imaging of the spine and the brain, which revealed a sylvian fissure arachnoid cyst with intracystic hemorrhage and frontoparietal subdural hematoma. The child did not have headache at this time, although he had experienced chronic headaches since the age of 4 years. He was treated with a course of oral steroids, which immediately relieved his back and leg pain. Subsequent imaging showed resolution of the cranial and spinal subdural blood collections and diminished size of the arachnoid cyst. No surgical treatment was necessary.
S. Alex Rottgers, Subash Lohani and Mark R. Proctor
Historically, bilateral frontoorbital advancement (FOA) has been the keystone for treatment of turribrachycephaly caused by bilateral coronal synostosis. Early endoscopic suturectomy has become a popular technique for treatment of single-suture synostosis, with acceptable results and minimal perioperative morbidity. Boston Children's Hospital has adopted this method of treating early-presenting cases of bilateral coronal synostosis.
A retrospective review of patients with bilateral coronal craniosynostosis who were treated with endoscopic suturectomy between 2005 and 2012 was completed. Patients were operated on between 1 and 4 months of age. Hospital records were reviewed for perioperative morbidity, length of stay, head circumference and cephalic indices, and the need for further surgery.
Eighteen patients were identified, 8 males and 10 females, with a mean age at surgery of 2.6 months (range 1–4 months). Nine patients had syndromic craniosynostosis. The mean duration of surgery was 73.3 minutes (range 50–93 minutes). The mean blood loss was 40 ml (range 20–100 ml), and 2 patients needed a blood transfusion. The mean duration of hospital stay was 1.2 days (range 1–2 days). There was 1 major complication in the form of a CSF leak. The mean follow-up was 37 months (range 6–102 months). Eleven percent of nonsyndromic patients required a subsequent FOA; 55.6% of syndromic patients underwent FOA. The head circumference percentiles and cephalic indices improved significantly.
Early endoscopic suturectomy successfully treats the majority of patients with bilateral coronal synostosis, and affords a short procedure time, a brief hospital stay, and an expedited recovery. Close follow-up is needed to detect patients who will require a secondary FOA due to progressive suture fusion or resynostosis of the released coronal sutures.
Subash Lohani, Diana P. Rodriguez, Hart G. W. Lidov, R. Michael Scott and Mark R. Proctor
Intrasacral meningoceles are rare cystic lesions that can cause focal compression within the bony sacral canal. Their mechanisms are poorly understood, but most intrasacral meningoceles appear to be intrasacral extradural cysts caused by arachnoid herniating through a small dural defect in the caudal end of the thecal sac. As opposed to perineural cysts, they are not associated with an exiting nerve root. When symptomatic, they can cause sacral pain or sacral nerve root dysfunction due to local compression.
This is a retrospective series from Boston Children's Hospital. All patients in whom symptomatic intrasacral meningocele that required surgical treatment was diagnosed between May 1994 and March 2011 were included in the study. Spine MRI was the diagnostic modality of choice. All patients underwent sacral exploration, with ligation and obliteration of the cyst. Resected cyst wall was subjected to pathological examination.
There were 13 patients (11 boys and 2 girls) who underwent operation for intrasacral meningocele. The median age was 8 years (range 5 months–16 years). The most common presenting symptom was back pain (in 5) often described as deep tail bone pain, followed by urinary incontinence (3) and constipation (2). Three patients had evidence of associated tethered cord on MRI studies. Four patients were asymptomatic and their diagnosis was made following imaging for other reasons; they were surgically treated because of the increasing size of the lesion or association with other congenital lesions. Most patients had symptomatic improvement after surgery.
Intrasacral meningoceles are rare lesions that may result from a congenital dural weakness and a resultant arachnoid diverticulum. They present in childhood either incidentally or with symptoms secondary to nerve root compression. Identification of the point of herniation through the dura mater and ligation of the lesion provides cyst cure and resolution of symptoms in most patients.
Subash Lohani, Joseph R. Madsen, Ann M. Bergin and Edward R. Smith
The combination of moyamoya syndrome and symptomatic mesial temporal sclerosis (MTS) has not previously been reported. The authors present the case of a 5-year-old boy with symptomatic MTS who developed progressive moyamoya syndrome. This combination of progressive moyamoya and a structural seizure focus presented a unique clinical problem, with the natural history of MTS predicting a high likelihood of needing resection in the future, which could be challenging following any type of moyamoya-related revascularization surgery. In anticipation of this problem, the patient underwent resection of the right inferior and mesial temporal lobe followed by right pial synangiosis as a 1-day combined operation. Postoperatively he recovered well without any neurological deficits and had an uneventful hospital stay. This case of moyamoya is unique in its association with MTS, and for the simultaneous operations for pial synangiosis and temporal lobectomy, highlighting the importance of surgical planning in patients with dual pathological processes.