Medical History

This was the case of a 9-year-old girl with a 12-month history of headache and decreased vision for 1 month. Her height was 115 cm, which is lower than average for her age and sex by 2 SD, and she was under the third percentile of average height for Chinese girls. Endocrinological examination revealed hypo-thyroxine and growth hormone axis and increased levels of prolactin (31 ng/μL).

Analysis Before Surgery

The tumor was a typical type Q tumor, and the patient’s primary complaint was vision disturbance, and she presented with growth retardation.

Neuroimaging

Surgical consideration: Type Q tumor growth began below the diaphragm and arachnoid. The suprasellar part was covered by the diaphragma sellae (DS) and arachnoid above the DS, and therefore this kind of tumor is considered the optimal tumor type for the transsphenoidal approach. However, there are several difficulties associated with selection of the transsphenoidal approach for the management of this type Q tumor (Fig. 6.7).

1. 1.
   
   
   

   The suprasellar part of the tumor breaks through the diaphragma sellae into the anterior cranial fossa, which might present a significant challenge to GTR.

   
   
    
   
   
2. 2.
   
   
   

   The PS always inflates and fuses with the tumoral cystic walls. The absence or interruption of the PS is very often associated with posterior pituitary gland anomalies and hormonal dysfunction. As a result, these tumors are difficult to treat surgically with the transsphenoidal approach with preservation of the PS and pituitary function.

   
   
    

The midline interhemispheric approach was selected for gross total or maximum possible safe resection and to maintain the intactness of normal nervous tissues, especially the infundibulum-pituitary stalk tract. The ultimate goal of surgery is to pursue a certain quality of life while removing the tumor, specifically the endocrine function dominated by the hypothalamic-hypophyseal axis.

The operative steps involving craniotomy utilized for the removal of type Q craniopharyngiomas can be summarized as follows: (1) standardized skull base craniotomy (frontobasal interhemispheric craniotomy), (2) separation of the tumor from suprasellar structures and the stretched, attenuating DS, (3) resection of the bone at the tuberculum sellae to provide direct vision on the intrasellar tumor portion, (4) T-shaped incision of the elevated DS, and (5) tumor dissection along the true capsule while attempting to preserve the neurohypophysis and continuity of the PS.

Intraoperative Findings

Figures 6.8, 6.9, 6.10, 6.11, 6.12, 6.13, 6.14, and 6.15.

Perioperative Treatment

Postoperative MRI (10a–c) obtained after 24 months revealed total tumor removal with preservation of the pituitary stalk (Fig. 6.16). Endocrinological detection performed 6 months after surgery indicated no new hormone deficiencies. The PRL level was normal, although the levels of several sexual hormones remained low.

Long-Term Follow-Up

The patient’s visual symptoms had recovered by the time of the last official follow-up, which was conducted at 24 months postoperatively. Her diabetes insipidus (DI) recovered gradually. She was able to attend school and did not require hormone replacement therapy at the time of the last follow-up study.

Medical History

This was the case of a 6-year-old male patient with a 12-month history of intermittent headache and vomiting. He also exhibited growth failure, emaciation, and partial hypopituitarism. He presented with visual deterioration (right eye: 0.4, left eye: 0.08) and bitemporal hemianopia.

Physical and Experimental Examination

The patient’s routine blood and urine analysis results were normal; however, he had low levels of plasma thyroxine and free thyroxine (fT4). An insulin stimulation test for cortisol and growth hormone (GH) revealed subnormal responses.

Radiological Images Before Surgery

A predominantly cystic intra- and suprasellar mass was identified both on CT and MRI (Fig. 6.17). The anterior communicating (AcoA) and anterior carotid arteries (ACA) formed a snowman shape at the top of the cystic tumor. On enhanced MRI, the cystic tumor wall was significantly enhanced roundly. On a coronal scan, a solid tumor was observed on the right side of the pituitary fossa near the medial wall of the CS. No obstructive hydrocephalus consequent to the tumor growth pattern and expansion was observed.

Analysis Before Surgery

Intraoperative Findings

After general anesthesia induction, the patient was placed in the supine position with the head fixed in a Mayfield three-pin head-holder. The head was extended slightly downward (approximately 15°), allowing the frontal lobe to spontaneously fall downward because of gravity during surgery. A standard frontobasal interhemispheric craniotomy (FIH) was performed (Fig. 6.18). A square of forehead periosteum was maintained for further frontal sinus repair. The bone flap extended beyond the median line, with the lowest margin as near as possible to the nasion. The mucosa inside the frontal sinus was removed carefully after opening the bone window. The bone of the posterior sinus wall was removed and drilled to increase exposure. Next, bone wax with antibiotic powder was used to fill the sinus cavity completely and reduce the risk of intracranial infection. A curved incision was made to open the bilateral dura mater beside the superior sagittal sinus; this incision was made close to the skull base. Subsequently, the anterior part of the superior sagittal sinus was ligated, followed by removal of the cerebral falx. The dural flap was turned rearward.

Dissection of the Interhemispheric Fissure

A self-retaining retractor was used to retract the bilateral frontal lobe. The bilateral frontal lobes were separated along the interhemispheric corridor and the cerebrospinal fluid (CSF) was drained to the extent possible. Occasionally, dissection of the frontal interhemispheric fissure is much more complex than that used in a lateral transsylvian approach, especially in patients with increased intracranial pressure (ICP). Continuous lumbar CSF drainage during dissection is a valid method of ICP release. The planum sphenoidale, optic chiasm, and cisterna lamina terminalis were exposed in a stepwise fashion after allowing the frontal lobe to fall.

Tumor Removal

The enlarged prechiasmatic space was the major corridor for surgical manipulation. Because of diaphragmatic septation, the suprasellar cystic tumor could be separated via slight pulling from the circle of Willis vascular ring. In this case, the suprasellar cystic tumor protruded in front of the AcoA complex to form a snowman sign (Fig. 6.17). However, this type of snowman sign can be separated easily via intracystic decompression while following the cystic wall interface. At the posterosuperior part of the tumor, the proximal PS could be exposed. The distal PS fused with the tumor capsule at the border of the diaphragmatic foramen. Here, the pituitary appeared to be inflated as an umbrella rather than displaced by compression, and thus it was difficult to preserve PS continuity. As a result, the distal segment of the PS was seceded where the stalk and tumor capsule had fused. Upon total separation of the suprasellar tumor cyst, a circular incision was made in the diaphragm at the bony opening of the pituitary fossa to locate the true subdiaphragmatic tumor capsule. Following this interface, the intrasellar part of the tumor was removed in a stepwise manner. When separating bilateral tumor walls, brisk venous bleeding is often encountered from the bilateral CS. This can be readily controlled via gentle packing with a small piece of Gelfoam (Fig. 6.19).

Pathology Study

A typical adamantinomatous CP was identified on the pathological slides.

Postoperative course: The third VF remained intact (Fig. 6.20). Postoperatively, the patient developed mild transient DI. Hyponatremia was the major manifestation of electrolyte disturbance. The symptoms were relieved with fluid and electrolyte replacement. Endocrinological detection revealed evidence of panhypopituitarism. The patient’s visual function recovered significantly.

Medical History

This was the case of an 8-year-old female patient. She had undergone a prior craniotomy via a right pterional approach to address a significant reduction in visual acuity at a local hospital in December 2011. However, a follow-up series of MRIs indicated tumor recurrence. The patient subsequently had intermittent vomiting and constant headache.

Physical and Experimental Examination

No significant positive symptoms were observed except right eye blindness.

Radiological Images Before Surgery

The MRI scan conducted in December 2011 indicated a typical Id-CP in the intra- and suprasellar regions. This lesion was mixed cystic-solid, and the optic chiasm was displaced upward. Postsurgical MRI indicated subtotal tumor removal with residual intrasellar tumor. Follow-up MRI in April 2012 indicated intrasellar tumor recurrence. In September 2012, MRI indicated that this significant recurrent tumor remained an Id-CP with upward displacement of the optic chiasm. However, the tumor was not treated until the patient presented in our department in March 2013. CT indicated an extensive and expanding tumor in the sellar region, anterior basement of the cranial fossa, and upper clivus that also occupied the third ventricular cavity. Broad punctate calcification was observed. MRI revealed a huge mixed cystic-solid tumor in the intra- and suprasellar regions with extensive extension and external growth. The solid part of the tumor was significantly enhanced on T1-weighted enhanced MRI. The ACA and AcoA were entrapped by the cystic tumor. As a result, the tumor exhibited a lobular tumor shape (Fig. 6.21).

Analysis Before Surgery

This was a case of recurrent CP, and the notable morphological features should be emphasized. Before the primary surgery, the tumor was a typical case of Id-CP. However, the natural “protective membranous structures” such as the diaphragm and suprasellar arachnoid were destroyed by surgery so that no such interface obstructed the invagination of the recurrent tumor into the nervous tissue layer. MRI performed before the secondary surgery indicated tumor penetration through the PS and growth toward the nervous tissue layer of the infundibulum and third VF, leading to severe hypothalamic involvement.

Intraoperative Findings

Although the primary surgery destroyed the normal membranous structures that covered the tumor surface, the inner arachnoid layer continued to separate the tumor from the surrounding neurovascular structures. During surgery, this interface was followed to avoid injury. The most important locations were the site of origin and PS. Intrasellar recurrence is the most frequent issue, and therefore identification of the pituitary capsule was necessary to ensure total intrasellar tumor removal. After total tumor removal, the floor of the pituitary fossa was smooth and contained the neurohypophysis. The involved PS was ruptured to avoid additional recurrences (Figs. 6.22 and 6.23).

Perioperative Treatment

The patient developed severe water and electrolyte imbalance disorder and required fluid and sodium substitution and adjustment. Postsurgical endocrinological detection indicated panhypopituitarism. The patient’s right eye was completely blinded.

Long-Term Follow-Up

After a 1.5-year follow-up, the patient’s right eye remained blind. However, vision in the patient’s left eye was restored. She continued receiving oral prednisone, thyroxin, and desmopressin to treat panhypopituitarism and permanent DI. Her BMI increased from a presurgical value of 15.7–20.

Medical History

This was the case of a 5-year-old male patient with a 1-year history of intermittent headaches that had progressively worsened during the last 2 weeks.

Physical and Experimental Examination

The patient exhibited growth retardation and bitemporal hemianopia but no other positive physical signs. Endocrinological detection indicated hypopituitarism. Deficiencies in fT4, T3, sexual hormones, and GH were present. The PRL level was slightly increased.